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JANUARY 2010Question 1You have decided to apply for a multiyear federal research grant for a study designed to reduce childhood mortality in the United States. Of the following, the area of focus that has the GREATEST potential for absolute mortality reduction isA.early recognition and treatment of sepsisB.firearm safetyC.pedestrian and motor vehicle safetyD.prevention of accidental drowningEreducing sports-related head injuries Correct answer CThe most common cause of death in childhood is traumatic injuries (Table). In the United States, up to 20,000 children are killed each year as a result of traumatic injuries, with motor-vehicle crashes and pedestrians involved in motor-vehicle crashes accounting for up to two thirds of the mortality. The rate is highest for teenagers. Efforts in injury prevention related to pedestrian and motor vehicle safety have the greatest potential for reducing this number.Table: Injury Deaths by Age and Mechanism (United States, 1998) Injury MechanismYounger Than 1 y0 to 4 y 5 to 9 y10 to 14 y15 to 19 yTotalUnintentional???????Motor Vehicle?155?467?604?839?4,823?6,888Pedestrian??7?292?230?197?351?1,077Drowning??63?496?243?201?439?1,442Fire/Burn??43264?197?104?87?695Bicycle??09?70?124?66?269Firearm??019?34?68?141?262Fall??20?45?26?29?90?210Suffocation?376?152?61?72?52?713Other??56?160?67?58?519?860Total??720?1,904?1,532?1,692?6,568?12,416Intentional???????Suicide??0?0?7?317?1,737?2,061Firearm Suicide??(0)?(0)?(1)?(153)?(1,087)(1,241)Homicide??322?399?170?290?2,311?3,492Firearm??(0)?(63)?(60)?(194)?(1,898)?(2,215)Total??322?399?177?607?4,048?5,553Intent Unknown???????Total??75?43?15?51?139?323TOTAL??1,117?2,346?1,724?2,350?10,755?18,292Homicide is the second most common cause of death in childhood, followed by suicide, drowning, and fire. Sepsis and sports-related head injuries are not among the top five causes of death in childhood.According to UNICEF, most childhood deaths in the developing world result from one the following five causes or a combination thereof:Acute respiratory infectionDiarrheaMeaslesMalariaMalnutritionMany of these conditions can be improved only through improvement in infrastructures such as sanitation and vaccination programs. American Board of Pediatrics Content Specification(s):Know common causes of death in childhoodQuestion 2A 6–year–old previously healthy boy presents with 2 days of right–sided scrotal pain. He denies trauma, dysuria, fever, or penile discharge. The pain started slowly and has worsened gradually. He denies nausea or vomiting. He never has had a urinary tract infection or any other significant past history. He denies any episode of abuse. On physical examination, the awake and alert boy appears mildly uncomfortable but generally well. He has a mildly erythematous, mildly enlarged, tender right testicle. The pain in the testicle seems diffuse. Lifting the testicle does not seem to change the quality or intensity of the pain. There is a weakly positive cremasteric reflex on the right and a brisk cremasteric reflex on the left. Color Doppler ultrasonography reveals increased blood flow to the right testicle. Urinalysis shows a specific gravity of 1.005; a pH of 6.5; and negative nitrites, leukocyte esterase, and blood.Of the following, in addition to urinalysis, the MOST appropriate further evaluation and treatment for this patient plete blood count and plete blood count and doxycyclineC.nucleic acid amplification tests for Neisseria gonorrhoeae and Chlamydia trachomatis and analgesicsD.urine culture and analgesicsE.urine culture and doxycycline Correct answer DTesticular torsion, torsion of the appendix testis, and epididymitis/orchitis are the most common causes of acute scrotal pain in prepubertal boys. The initial step in management is to exclude the diagnosis of testicular torsion. If this cannot be accomplished confidently on the basis of physical examination alone, color Doppler ultrasonography is used to examine both testicular anatomy and blood flow. The history and findings on physical examination for the boy described in the vignette as well as the increased blood flow on Doppler ultrasonography make the diagnosis of epididymitis more likely than testicular torsion. Epididymitis is an infection or inflammation of the epididymis. In prepubertal boys, epididymitis may be associated with urinary tract infections and structural urologic abnormalities. However, many case series find anatomic abnormalities and positive bacterial cultures in only a minority of cases. The cremasteric reflex usually is preserved, but it often is difficult to assess in younger patients. Pain relief when the testicle is lifted (Prehn sign) is a less reliable finding in children who have epididymitis than in adults. All prepubertal patients who have epididymitis should have a urinalysis and urine culture obtained. A positive urine culture is associated with a higher likelihood of structural abnormalities. One recent series of patients in Israel showed either microbiologic or serologic evidence of a coexisting viral or bacterial infection (other than a urinary tract infection) in a large percentage of patients. The authors postulated that epididymitis in this age group often is a postinfectious inflammatory process for which antibiotics are not needed in most cases. In infants, presumptive antibiotic therapy for urinary pathogens pending culture results may be prudent because of their higher rate of associated urinary tract infection.Once the diagnosis of torsion is excluded in this age group, treatment is aimed at relief of symptoms. Patients rarely show systemic signs of illness, and blood tests, hospital admission, or intravenous therapy rarely is necessary. Antibiotics can be reserved for patients who have positive findings on urinalysis. In prepubertal patients, antibiotics that provide coverage for coliform bacteria usually associated with urinary tract infections (eg, trimethoprim–sulfamethoxazole) are used most often. Doxycycline should not be administered to patients younger than 8 years of age.Among adolescents who are sexually active, Chlamydia is the most common cause of epididymitis, although other organisms may be found. All adolescents who have epididymitis should undergo testing for sexually transmitted infections, but such testing generally is not necessary in younger patients. Prepubertal boys who have epididymitis and negative findings on urinalysis may be treated with rest, analgesics, and close follow–up.American Board of Pediatrics Content Specification(s):Know how to evaluate and manage epididymitis/orchitisQuestion 3A 6-year-old girl is brought in by her mother because of an initially scaly, mildly pruritic, erythematous rash that started above her left eye. The mother initially believed the rash was an insect bite, so she has been treating it with an over-the-counter topical corticosteroid cream for the past 3 weeks. The topical steroid appeared to help with the erythema and the mild pruritus at first, but now the rash is enlarging and appears more papular and less scaly (Figure). FigureOf the following, the MOST likely diagnosis for this child isA.allergic contact dermatitisB.atopic dermatitisC.erythema multiforme minorD.tinea incognitoE.varicella zosterCorrect answer DThe girl described in the vignette has tinea incognito, a dermatophyte infection that has been modified and even exacerbated by the long-term use of topical corticosteroids. The immunosuppressive effect of topical corticosteroids that can alleviate the initial erythema and pruritus likely is responsible for the atypical transformation and spread of the dermatophyte infection. The characteristic circular, well-defined, scaling edge of tinea corporis can assume a variety of appearances: psoriasislike, eczemalike, or licheniform, with follicular, pustular, or papular characteristics. In general, most infectious dermatologic conditions (Table) are aggravated by topical corticosteroid use. Varicella zoster is an unusual condition in a young immunocompetent child. Pain precedes the appearance of the dermatomal rash, which starts as erythematous papules and progresses to vesicles and confluent blisters, eventually crusting over. Table: Infectious Dermatologic ConditionsBacterialImpetigoFuruncles/CarbunclesParonychiaEcthymaErysipelasCellulitisLymphangitisErythrasma?ViralEczema herpeticumMolluscum contagiosumPrimary varicellaVaricella zosterWartsParasiticScabiesDemodex?FungalDermatophyte rashesCandidal dermatitis?In contrast, topical corticosteroids are common therapies for several dermatologic conditions. Inflammatory-mediated rashes and conditions that feature a significant inflammatory component benefit from topical corticosteroid use. Such conditions tend to be noninfectious and include allergic contact dermatitis, atopic dermatitis/eczema, psoriasis, and seborrheic dermatitis. Allergic contact dermatitis (eg, exposure to poison ivy) is a type IV hypersensitivity reaction that is characterized by erythema and edema, usually about 48 hours after exposure to the offending agent. The development of exudative vesicular or bullous lesions may follow. The hallmark of atopic dermatitis is unremitting pruritus, which leads to the excoriation, lichenification, and crusting of lesions. Xerosis, scaling, and erythema are also common. Topical or oral steroid is particularly beneficial for type IV hypersensitivity reactions.Other dermatologic conditions that can improve with topical corticosteroid use but are somewhat less responsive are: discoid lupus erythematosus, pemphigus, vitiligo, pityriasis rosea, and granuloma annulare. The rash of erythema multiforme minor tends to be symmetrically distributed, is initially maculopapular, and eventually develops into the characteristic “target lesions.” Mild cases do not require any treatment. American Board of Pediatrics Content Specification(s):Differentiate dermatologic conditions that benefit from topical corticosteroids from those aggravated by them.Question 4A mother brings her previously healthy 5-year-old boy to the emergency department because he has developed a rash. She reports that the boy has been active and acting well, but she became concerned about increased bruising over areas of minor trauma, such as the legs and elbows. There is no history of bleeding. On physical examination, the afebrile boy appears well, and his vital signs are normal. He has scattered purpura and petechiae on the extensor surfaces of his legs and forearms. You suspect the diagnosis of idiopathic thrombocytopenic purpura and order laboratory tests. The laboratory reports that the boy’s platelet count is 5x103/mcL (5x109/L).Of the following, the MOST appropriate next step is toA.administer intravenous immune globulinB.consult a hematologist for a bone marrow aspirateC.obtain computed tomography scan of the headD.request an examination of the blood smearE.transfuse 2 units of platelets Correct answer DBecause the child described in the vignette is afebrile, well-appearing, and has no other signs of chronic illness, he most likely has acute idiopathic thrombocytopenic purpura (ITP). However, malignancies and other potential causes of thrombocytopenia must be considered in the differential diagnosis. Awaiting the results of the counts of other cell lines and carefully examining the peripheral blood smear are important steps before verifying the diagnosis of ITP. A blood smear in a child who has ITP should show a reduced number of platelets, which are normal, or larger than normal, in size as well as normal white blood cell and red blood cell morphologies. Intravenous immune globulin or other therapies should be administered only after consultation with a hematologist and the diagnosis of malignancies has been excluded. Bone marrow aspiration is somewhat controversial in children who have presumed ITP and should not precede a careful examination of the peripheral blood smear. Computed tomography scan of the head should be obtained only in children who have signs and symptoms of intracranial bleeding, a rare complication of ITP. Platelet transfusion is not indicated for patients who have ITP and are not actively bleeding. ITP is the most common form of acquired thrombocytopenia in the pediatric patient. Similar to the child in the vignette, children who have acute ITP usually present with increased bruising and bleeding from mucosal surfaces, mouth, vagina, and gastrointestinal tract. Otherwise, the children usually appear well, without lymphadenopathy or hepatosplenomegaly. As the platelet count decreases, the risk of bleeding increases, especially after trauma. Life-threatening complications such as intracranial hemorrhage are extremely rare (<1%), but they are associated with a very high mortality rate (46%). Proposed mechanisms responsible for the pathophysiology of ITP involve autoimmunity against glycoproteins on the surfaces of platelets. The autoantibodies coat the platelets that then are destroyed by the spleen and other organs of the reticuloendothelial system. As with some diseases with proposed autoimmune mechanisms, the antibodies are believed to arise from the immune response to a mild bacterial or viral infection, and subsequently cross-react with platelet antigens. In addition, platelet production in ITP may be decreased by immune mediators.A minority of children who have acute ITP eventually progress to chronic ITP. Such a progression is more common in older children. Occasionally, such children are resistant to pharmacologic treatments (including rituximab) and require splenectomy. For most children who have ITP, the thrombocytopenia resolves within 6 months without any treatment. However, depending on the platelet count and the clinical symptoms, treatment may include: Restriction of physical activitiesWearing protective head-gearIntravenous immune globulinCorticosteroidsAnti-Rho antibody (for patients who are Rh-positive)The threshold for pharmacologic treatment in the child who does not have acute bleeding is unclear, although a platelet count of 20x103/mcL (20x109/L) traditionally has been used. American Board of Pediatrics Content Specification(s): Know the etiology and pathophysiology of idiopathic thrombocytopenic purpuraRecognize signs and symptoms and life-threatening complications of idiopathic thrombocytopenic purpuraPlan the management of acute idiopathic thrombocytopenic purpuraQuestion 5A 4-month-old infant is brought to the emergency department for evaluation of bruising. His mother reports that after the child had intermittent bruising for the past several weeks, she took him to the pediatrician, who referred them to the emergency department for further evaluation. The child has had no other symptoms. The mother offers no explanation for the bruising and denies any trauma, other bleeding, fever, or other infectious symptoms. The child was circumcised without any complications, and the remainder of his past medical history is unremarkable. The child lives with his mother, a 2-year-old sibling, and the mother’s boyfriend. The child’s father is not involved in his care. Physical examination reveals a well-appearing, alert, and interactive child who has bruises of varying color on the right pinna, left cheek, bilateral chest, and right thigh. No other findings of note are apparent on the examination. Results of a complete blood count that includes platelets, measurement of liver and pancreatic enzymes, coagulation studies, skeletal survey, and head computed tomography scan are normal.Of the following, the MOST appropriate next step is toA.call the patient’s pediatrician and allow her to decide if a child abuse report is necessaryB.consult hematology and await formal bleeding evaluation before filing a child abuse reportC.discharge the patient with scheduled routine follow-up visit because no other injuries are identifiedD.file a written and verbal child abuse report and provide a summary of the medical information to the investigating workerE.have the mother removed by security and discharge the patient with his birth father Correct answer DUnexplained bruising in a 4-month-old infant is concerning for nonaccidental trauma. If thrombocytopenia or other coagulopathy is eliminated, a full evaluation for other abusive injuries should be undertaken. Such evaluation should include a skeletal survey to identify occult fractures, assessment of hepatic and pancreatic enzymes to screen for abdominal trauma, and head computed tomography scan to screen for intracranial injuries. Even if no additional injuries are found, as described for the infant in the vignette, unexplained bruising in a preambulatory infant or child should be reported as suspected child abuse. Discharging the child with the recommendation for a routine follow-up visit is not sufficient. Notifying the child’s pediatrician to make her aware of the concerns and the disposition plans is appropriate, but deferring the decision to file a report of suspected abuse is not. Isolated bruising in the presence of a normal platelet count and coagulation studies and no history of other bleeding does not warrant hematologic evaluation. Decisions regarding visitation rights and custody of the child are not made by the treating physician (unless emergency custody is taken because of imminent danger to the child); they are made through the legal system following investigations by child protective services and the police.Since 1967, all states are required to have mandatory child abuse and neglect reporting statutes, although specifics vary from one state to another. Mandatory reporting statutes include a minimum definition of abuse and neglect, a list of individuals mandated to report suspected abuse, provisions for criminal and civil liability for failure to report, protection from criminal or civil liability for reports made in “good faith,” identification of appropriate authorities to whom reports should be made, and procedures and a hotline for reporting. The specific language regarding the necessary level of suspicion before a report is filed varies somewhat, but in general, “reasonable” suspicion or concern for possible abuse or neglect, not clinical certainty, is sufficient reason for a report to be filed. All physicians should be familiar with their state’s specific mandatory reporting statutes. Many children’s hospitals and hospital systems have developed multidisciplinary teams to provide consultation on cases of suspected abuse and neglect. Such teams include physicians from various disciplines, nurses or nurse practitioners, and social workers. They also may include representatives from the medical examiner’s office, law enforcement, psychiatry, and child protective services. Whether functioning as a formal member of such a team or as the patient’s treating physician, physicians play a critical role in the evaluation and management of cases of suspected child abuse or neglect. After recognizing the possibility of abuse or neglect, the physician collects and documents information regarding the initial presenting history and any additions or changes to that history over time, examination findings, and results of laboratory and radiologic tests. The information should be documented completely and legibly in the child’s medical record. Any visible injuries should be documented photographically. At a minimum, the physician should communicate this information to the appropriate child protective services and law enforcement authorities in an initial verbal report; most states also require filing of a written report within 48 hours. The family should be informed of the physician’s concerns and the filing of the report. Hospital admission is required for children who have medical issues requiring inpatient care, those in whom the diagnosis is unclear, or those for whom no clearly safe disposition can be determined. The physician may be asked to provide a verbal or written opinion on the case or provide court testimony regarding the findings. American Board of Pediatrics Content Specification(s):Recognize the need to report physical abuse cases to the proper authoritiesKnow the physician's role in working with a multidisciplinary team concerned with the care of a physically abused childQuestion 6A 7-year-old boy is shot in the left chest while sleeping in his bed during a drive-by shooting. Needle decompression of his chest at the scene does not alleviate his respiratory distress. Emergency medical services personnel endotracheally intubate him with a 5.5 uncuffed tube, place an occlusive dressing over the entrance wound, and establish upper extremity intravenous access. Upon presentation to the emergency department, he is being manually ventilated and has decreased breath sounds on the left, a heart rate of 150 beats/min, and an initial blood pressure of 94/62 mm Hg. His endotracheal tube is the appropriate size and appears to be positioned correctly. You obtain additional intravenous access, continue intravenous fluid resuscitation, and obtain chest radiography (Figure). The respiratory therapist states that it is becoming more difficult to ventilate the boy manually. FigureOf the following, the MOST appropriate next step after reassessing the child is toA.exchange the current endotracheal tube with a cuffed typeB.perform a tracheostomyC.perform a tube thoracostomyD.place a central intravenous lineE.place an arterial line Correct answer CThe boy described in the vignette has symptoms and radiographic evidence of a hemothorax. The most appropriate next step is tube thoracostomy to drain the blood from the pleural space that is producing cardiorespiratory compromise. A chest tube also allows determination of the total volume of blood lost in the thorax and the rate of potential ongoing hemorrhage, which, in turn, helps to define the need for an operative intervention with open thoracotomy. Other indications for tube thoracostomy include: a large, enlarging, or symptomatic pneumothorax; tension pneumothorax; chylothorax; significant pleural effusion; or empyema. The relative contraindications for tube thoracostomy are a bleeding diathesis or skin/soft-tissue infection in the overlying area intended for tube placement. However, these conditions do not preclude performing the procedure in a life-threatening situation. The procedure for tube thoracostomy is as follows:Position the patient supine (in nontraumatic situations, recumbent or upright positioning is an alternative), with the ipsilateral arm placed above the patient’s head and the area prepped and draped in a sterile fashion.Locate the insertion site in the mid to anterior axillary line in the fifth or sixth intercostal space. The nipple is used commonly as an anatomic landmark to help identify the appropriate intercostal space.If the clinical situation permits, use local anesthesia prior to making the overlying skin incision.Use a curved hemostat for blunt dissection of the intercostal muscles and subcutaneous tissue over the rib to avoid damaging the neurovascular bundle.Use the tip of the hemostat to puncture the parietal pleura.Use a finger to explore the subcutaneous track carefully and to exclude adhesions between the lung and the chest wall.Use the largest chest tube that can be accommodated between the ribs for fluid drainage. The chest tube is directed through the track anteriorly and superiorly for a pneumothorax and posteriorly for drainage of blood or other fluids.Needle decompression could be useful if there is an associated pneumothorax, but it usually does not improve a large hemothorax. No absolute contraindications to needle decompression of the chest exist, other than performing it in a situation that does not warrant it originally, thus potentially causing an iatrogenic pneumothorax. The primary indication for needle decompression of the chest is to treat a suspected tension pneumothorax, especially when tube thoracostomy cannot be performed expeditiously. The key steps required to perform needle decompression are to:Use a large-bore 16- or 18-gauge needle/catheter, with a smaller-gauge needle for neonates.Locate the insertion site in the anterior midclavicular line, usually in the second or third intercostal space, advancing over the underlying rib to avoid the neurovascular bundle that runs inferior to the rib.Aspirate air into the syringe as the thoracic cavity is entered and the pressure is released. A three-way stopcock attached to the syringe allows continued, periodic evacuation of air from a reaccumulated tension pneumothorax until tube thoracostomy can be plications from these procedures include pain; bleeding; infection; and misguided placement of the decompressing needle or chest tube, leading to injury of larger blood vessels, the diaphragm, lungs, and other underlying thoracic and abdominal organs. Tube thoracostomy may fail to resolve the pneumothorax or fluid collection on the first attempt, necessitating placement of an additional chest tube. Endotracheal tube exchange to a cuffed tube might be necessary in a more stable patient who demonstrates a significant air leak or if a need for higher peak airway pressures is anticipated. A tracheostomy might be appropriate for the patient who experienced significant facial or upper airway trauma and requires an emergent surgical airway because of difficulty with obtaining an oropharyngeal airway. Central venous access and arterial line placement might be helpful later in the resuscitation and monitoring of this patient, but addressing the condition that threatens his breathing takes precedence.It is important to note that in this vignette, the chest radiograph has already been performed and does show evidence for a hemothorax. Given the information provided, the next step is to perform a tube thoracostomy. However, if?history and physical findings are compatible with hemothorax in a patient in uncompensated shock, it is important to perform the tube thoracostomy immediately, rather than waiting for a chest radiograph to confirm the diagnosis.American Board of Pediatrics Content Specification(s):Discuss the indications and contraindications for tube thoracostomy and needle decompression of the chestDescribe the key steps and potential pitfalls in performing tube thoracostomy and needle decompression of the chestDiscuss the complications associated with tube thoracostomy and needle decompression of the chestKnow the anatomy and pathophysiology relevant to tube thoracostomy and needle decompression of the chestQuestion 7A 22-month-old previously healthy child is brought to the emergency department by his parents because of increasing difficulty breathing over the past 8 hours. He was seen by his pediatrician yesterday because of a 2-day history of rhinorrhea, fever, and cough, and she prescribed amoxicillin. His parents state that he seemed to be agitated, coughing more, and breathing much faster this evening, so they gave him a dose of acetaminophen and brought him in for evaluation. They note his breathing seemed to calm down on the 30-minute trip to the hospital. On physical examination, the boy has a temperature of 101.5°F (38.6°C), heart rate of 180 beats/min, respiratory rate of 34 breaths/min, and blood pressure of 92/64 mm Hg. His initial pulse oximetry reading is 88%. He is sleepy and arouses only briefly with intravenous line placement. Crackles are audible throughout the right lung field, aeration is decreased with tubular breath sounds, he is breathing shallowly, and he has mild flaring and retractions. After you administer intravenous antibiotics and a nebulized albuterol treatment, there is no change in his clinical appearance. His pulse oximetry reading is 92% on 50% FiO2 by face mask. Of the following, the MOST appropriate treatment for this patient is toA.begin 1 hour of continuous albuterol via nebulizer, administer prednisolone if he has a family history of asthma, and prepare to admit him to the inpatient unitB.obtain inspiratory and expiratory chest radiographs and request a consultation for possible acute foreign body aspirationC.obtain posteroanterior and lateral chest radiographs and admit him to the inpatient unit for intravenous antibiotics and oxygen therapyD.perform needle thoracentesis of the right chest and admit him to the inpatient unitEprovide assisted ventilation and admit him to the intensive care unitCorrect answer: EThe child described in the vignette has multiple signs of impending respiratory failure and needs to be admitted to a monitored setting in which ventilatory support can be continued. The history and physical examination findings suggest the presence of a consolidated pneumonia, possibly with an effusion. His respiratory rate of 34 breaths/min in the presence of hypoxia noted at triage is inadequate to maintain his increasing ventilatory needs, and carbon dioxide retention likely is contributing to his altered mental status. Although the child is the right age for a foreign body aspiration, the history of the illness beginning gradually with rhinorrhea, fever, and cough and progressing over several days is not suggestive of this diagnosis. Bronchodilator therapy would be unlikely to improve the child’s ventilatory status and, in fact, may worsen his ventilation/perfusion mismatch and cause further hypoxia. Although this child may have an effusion complicating his pneumonia, there is not sufficient information to warrant a needle thoracentesis at this time. There is no evidence of a tension pneumothorax.Although many physicians define respiratory failure by blood gas parameters: a PCO2 of greater than 50 mm Hg or a PaO2 of less than 60 mm Hg, it is better defined by the presence of inadequate ventilation or oxygenation to meet tissue metabolic demands. Nevertheless, blood gas analysis, preferably arterial, would be helpful in this patient as a baseline to assess his current PaO2 and PCO2 and acidosis and to follow his oxygenation and ventilation over time. The most common causes of respiratory failure in newborns are respiratory distress syndrome, meconium aspiration, and sepsis (Table). Pneumonia is a common cause beginning in the newborn period and continuing throughout childhood. In addition, infants are susceptible to bronchiolitis, bronchopulmonary dysplasia, croup, and sepsis. Among toddlers, asthma and near-drowning become important causes, with foreign body aspiration also playing a role. Trauma and drug ingestions join asthma as the leading causes of respiratory failure in teenagers. Table: Differential Diagnosis of Respiratory Failure Age?Common Uncommon Newborn?Respiratory distress syndrome?Diaphragmatic hernia??Meconium aspiration?Congenital airway abnormalities, such as choanal atresia??Pneumonia/sepsis?Pulmonary hypoplasia??Drug-induced respiratory depression?Cystic adenomatoid malformation???Pulmonary hemorrhage?Infant?Pneumonia?Asthma??Bronchiolitis?Trauma??Bronchopulmonary disease?Epiglottitis??Croup???Sepsis/meningitis???Congestive heart failure?Toddler?AsthmaForeign body aspiration?Pneumonia?Hydrocarbon/caustic ingestions??Near drowning?Epiglottitis?Child Asthma?Chest wall disorder ?Pneumonia?Muscle weakness (eg, Guillain-Barré syndrome)??Trauma??Teenager?Asthma?Chest wall disorders??Trauma???Drug ingestions??Another method of categorizing respiratory failure is by anatomic/physiologic groupings. The Pediatric Advanced Life Support curriculum teaches the need to identify the cause of respiratory failure as either upper airway (eg, croup, epiglottitis, retropharyngeal abscess, foreign body), lower airway (eg, bronchiolitis, asthma, foreign body), lung tissue/parenchymal (eg pneumonia/empyema, interstitial disease, congestive heart failure with pulmonary edema, near-drowning), or disordered control of breathing (metabolic, toxicologic, neuromuscular, and central neurologic causes). Management options differ according to the underlying cause, but the potential need for emergency ventilatory support is common to all.As in this patient, the source of respiratory distress frequently is suggested by findings on history and physical examination alone. A febrile infant who has polyphonic wheezes and crackles accompanied by rhinorrhea is likely to have bronchiolitis. Respiratory compensation for metabolic acidosis should be suspected in the afebrile child who has tachypnea, clear lungs, and adequate chest excursion. Little diagnostic testing is required when the diagnosis seems clear from history and physical examination findings. Chest radiography and pulse oximetry should be obtained for all patients in respiratory failure. Blood gas analysis and chemistry panel should be considered for the patient who has altered mental status and respiratory distress to assess for acidosis, hypercarbia, and anion gap. End-tidal carbon dioxide monitoring is being used increasingly to monitor patients who have respiratory distress and impending respiratory failure and to assess the effectiveness of ventilation over time.American Board of Pediatrics Content Specification(s):Know causes of respiratory failureDifferentiate etiologies of respiratory failure by ageKnow applicable uses of monitoring, laboratory, and radiographic procedures with various types of respiratory failureQuestion 8A 4-year-old boy who has had recurrent epistaxis is referred to the emergency department because of further bleeding. Prior otorhinolaryngology evaluation could not identify an anatomic cause for the bleeding. Several episodes in the past few months have lasted more than 20 minutes. Results of a complete blood count, platelet count, peripheral blood smear, prothrombin time, and partial thromboplastin time are normal.Of the following, the test that is MOST likely to reveal the underlying diagnosis isA.bone marrow examinationB.factor IX activityC.fibrin split productsD.platelet aggregation studiesE.von Willebrand factor panel Correct answer EEpistaxis is common between the ages of 2 and 10 years. It is rare in infancy and becomes less prevalent after puberty. Most children have “benign” epistaxis that requires no further evaluation. Patients who have benign epistaxis have single or occasional episodes of epistaxis that last for a few minutes (usually less than 15 minutes) and are associated with a small amount of blood loss. History or physical examination findings suggestive of allergic rhinitis, upper respiratory tract infection, obvious trauma, or epistaxis digitorum strongly suggest the diagnosis. Benign epistaxis tends to occur more often in winter, when indoor heating dries the mucosae, leading to bleeding from fragile superficial vessels.Benign epistaxis typically emanates from the anterior nasal septal mucosa, an area also referred to as Kiesselbach plexus. This highly vascular area is within reach of a child’s finger during nose picking or rubbing. Examination may reveal a prominent anterior septal blood vessel. More profuse bleeding or recurrent bleeding lasting more than 20 minutes, as described for the boy in the vignette, requires further evaluation. Initial assessment should include complete blood count, peripheral blood smear, platelet count, and coagulation studies. Most epistaxis can be terminated with the use of controlled pressure, ice, or topical instillation of vasoconstrictor medications such as phenylephrine. Pressure is applied to the outside of the nose in a forward leaning position. Another method is to pinch the nostrils and compress the upper lip, which occludes the labial artery. If bleeding persists and no anatomic site or foreign body is identified, nasal packing may be indicated.If the initial laboratory results show no abnormality, referral to a hematologist to evaluate for von Willebrand disease is recommended. von Willebrand disease is the most common coagulopathy, with a prevalence of 1% in childhood. von Willebrand factor (VWF) is a multimeric protein that helps platelets bind to the vessel wall and serves as a carrier protein for factor VIII. Therefore, in its severe form, patients exhibit prolonged prothrombin time and partial thromboplastin time. However, many patients who have von Willebrand disease do not exhibit abnormalities in one or both of these tests. Concentrations of the heat-labile VWF protein may be elevated by stress, trauma, medication, or difficult venipuncture. von Willebrand disease is assessed using a panel that includes VWF antigen concentration, ristocetin cofactor for functional testing of VWF activity, and factor VIII concentrations.Examination of the bone marrow is not warranted in a child who has recurrent epistaxis and a normal platelet count. It may be useful if the complete blood count indicates an abnormality in bone marrow production, such as thrombocytopenia, aplastic anemia, or leukemia. Factor IX is much less important as an initial study in this child because children who have clinically significant disorders of factor IX activity usually exhibit a prolonged partial thromboplastin time. Platelet aggregation studies are complex to perform but may be considered when results of initial studies, including evaluation for von Willebrand disease, are normal. Fibrin split products are a specific marker of disseminated intravascular coagulation, which is not a consideration in this child.If the epistaxis is consistently unilateral, otorhinolaryngology evaluation may be indicated to exclude an unrecognized foreign body, abnormal vessel, polyp, or rarely, a neoplastic lesion such as angiofibroma. Vascular disorders such as hypertension or hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) are among the nonhematologic disorders that can result in epistaxis. Nonaccidental trauma should be considered in children younger than 2 years of age who have unexplained epistaxis.American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of epistaxisPlan diagnostic evaluation and initial intervention for patients with epistaxis.Recognize serious and/or life-threatening causes of epistaxisFEBRUARY 2010Question 1A 2-year-old boy presents to the emergency department with a heart rate of 40 beats/min, respiratory rate of 12 breaths/min, and a blood pressure of 70/30 mm Hg. He is lethargic, has miotic pupils, and has otherwise normal findings on physical examination. Following empiric administration of one dose of naloxone and supplemental oxygen by emergency medical services providers in the field, the boy’s pulse oximetry reading was 100%, and he seemed to have a slight but transient improvement in his mental status. Of the following, the substance that is MOST likely responsible for this patient’s dysrhythmia is A.albuterolB.amitriptylineC.caffeineD.clonidineE.cocaine Correct answer DThe boy described in the vignette is displaying symptoms and a dysrhythmia consistent with clonidine toxicity. Lethargy, bradycardia, and hypotension were the most common symptoms reported in a study examining clonidine-only exposures using the American Association of Poison Control Center’s database from 1993 through 1999 in children younger than 19 years of age. Toxic effects of albuterol, cocaine, and caffeine are more likely to cause sinus or supraventricular tachycardia, not bradycardia. Intoxication from amitriptyline often causes altered mental status, but sinus tachycardia and prolongation of the QRS or QT interval are the more likely dysrhythmias associated with this?agent. Electrocardiographic changes can be seen after the ingestion of a number of medications (Table). Primarily, these involve potassium (K+) channel blockers, sodium (Na+) channel blockers, sodium-potassium-adenosine triphosphatase (Na+/ K+-ATPase) blockers, calcium channel blockers, and beta-adrenergic blockers. The potential dysrhythmias observed with the medications are a reflection of the altered myocardial action potential. Table: Electrocardiographic Changes Associated With Medication IngestionsDysrhythmia?Ingested Substance?QT Interval Prolongation?Class Ia, III antiarrhythmic agentsCalcium channel blockersAntipsychoticsAntidepressantsAntihistaminesAntibioticsAntimalarials Selective serotonin reuptake inhibitorsVenlafaxineSinus/Supraventricular Tachycardia?AmphetaminesAntihistaminesAtomoxetineAtropineBelladonna alkaloidsBeta agonistsBupropion/MirtazapineCaffeineCocaineDigoxinEphedrine/Pseudoephedrine/PhenylephrinePhenothiazines?Ventricular Tachycardia/Torsade de pointes?Chloral hydrateDigoxinTricyclic antidepressantsPhenothiazinesQuinidineProcainamideFlecainideAstemizole/TerfenadineOrganophosphates?Bradycardia?Beta-adrenergic blockersBenzodiazepinesCalcium channel blockersClonidineDigoxinNarcoticsNefazodoneSedative-hypnotic agents?Atrioventricular Block?DigoxinDiltiazemBeta-adrenergic blockers?Atrial Flutter/Fibrillation?Digoxin?Potassium channel blockers cause prolongation of the QT interval because myocardial depolarization is driven by outward K+ movement. Sodium channel blockers prolong the QRS complex and can induce ventricular tachycardia and fibrillation. Cardiac glycosides are the prime example of Na+/ K+-ATPase blockers, which impair movement of both K+ and Na+ ions. They can exhibit a multitude of electrocardiographic changes, including premature ventricular and atrial contractions, sinus bradycardia, bundle branch blocks, atrioventricular (AV) blocks, and accelerated junctional tachydysrhythmias. Calcium channel blockers can cause sinus, junctional, and ventricular bradydysrhythmias as well as varying degrees of AV block due to slowed cardiac conduction and subsequent diminished contractility. Beta-adrenergic blockers decrease myocardial contractility and sinoatrial and AV node function, resulting in bradycardia and varying degrees of AV block. American Board of Pediatrics Content Specification(s):Know which ingestions commonly cause dysrhythmiasQuestion 2An otherwise healthy boy comes to the emergency department, complaining of sore throat, inability to take oral fluids, and a rash. He explains that he was treated with ibuprofen, ice, and elevation for an acute ankle injury 1 week ago. On physical examination, the febrile boy has a diffuse rash involving the face, trunk, palms, and soles. His lips and buccal mucosa are swollen, with blistering and erosions (Fig. 1). A complete blood count reveals a white blood cell count of 16.0x103/mcL (16.0x109/L). Results of a comprehensive metabolic panel and urinalysis are normal. A Tzanck smear of the erosions does not demonstrate multinucleate giant cells. Figure 1Of the following, the MOST appropriate initial steps in the management for this teen are to administer A.intravenous fluids and intravenous acyclovirB.intravenous fluids and intravenous methylprednisoloneC.0.3 mL subcutaneous epinephrine (1:1,000) and intravenous diphenhydramineD.intravenous fluids and discontinue ibuprofenE.intravenous clindamycin and obtain a blood cultureCorrect answer: DThe ill appearance and mucocutaneous rash described for the boy in the vignette are suggestive of Stevens-Johnson Syndrome (SJS) triggered by ibuprofen. SJS is a potentially life-threatening, acute, inflammatory, mucocutaneous disorder affecting the skin and at least two or more mucous membrane surfaces such as eyes, lips, or oral mucosa. Management of SJS includes immediate withdrawal of the offending agent, if known (ibuprofen for this boy), and supportive care. The illness should be managed like a “burn,” with aggressive fluid resuscitation, infection control measures, and nutrition support. SJS can be caused by chemical exposures or infections. Among the medications commonly implicated are sulfonamides, antiepileptic drugs such as phenytoin, and nonsteroidal anti-inflammatory drugs such as ibuprofen. The estimated mortality ranges from 5% to 15%. SJS belongs to same disease spectrum as toxic epidermal necrolysis (TEN). In these conditions, lateral pressure to erythematous areas can result in epidermal detachment (Nikolsky sign). Generally, mucous membrane involvement is preceded by a few days of fever and influenza-like symptoms, followed by development of skin lesions. SJS may evolve to become TEN. The primary difference between the two is the extent of body surface involved: less than 10% for SJS and more than 30% for TEN. Life-threatening complications (eg, sepsis and fluid loss) are related to sloughing of the skin and the resultant loss of the protective cutaneous barrier. There is no universally accepted clinical algorithm for diagnosis of SJS. Up to 85% of cases have conjunctival involvement that can range from hyperemia to pseudomembrane formation and frequently is associated with burning and pain. Photophobia, impaired alimentation, and painful micturition corresponding to involvement of mucous membranes also may be noted. Alternative causes of the symptoms should be excluded, particularly infections that have similar presentations, such as group A streptococcal or staphylococcal toxic shock syndrome, leptospirosis, measles, Kawasaki disease, and staphylococcal scalded skin syndrome. The primary differential diagnosis of SJS is erythema multiforme (EM) major, which formerly was considered to be a limited form of the same disease. More recent literature distinguishes EM major from SJS and places SJS and TEN in the same spectrum. The classic lesions of EM (both minor and major) are discrete, macular or papular, concentric erythematous rings (targetlike appearance) (Fig. 2). EM major involves at least one mucous membrane. Most cases of EM (60%) are associated with herpes simplex infection. Others have been associated with viral or mycoplasma infections. Lip involvement can make it difficult to distinguish from SJS. Figure 2 The clinical progression and negative Tzanck smear of the oral erosions for the boy in the vignette makes herpes infection unlikely. Accordingly, intravenous acyclovir is not indicated. Similarly, intravenous clindamycin is reserved for cases of suspected bacterial superinfection or staphylococcal scalded skin syndrome. Subcutaneous epinephrine and diphenhydramine are reserved for acute urticarial rash with angioedema or anaphylactic reaction. The role of corticosteroids in the treatment of SJS is controversial. Many clinicians use them in severe cases, but they provide no clear benefit and may increase the risk of complications. Intravenous immune globulin in a dose of 1 g/kg for 3 days was associated with rapid cessation of skin and mucosal detachment in 43 of 48 patients in one retrospective study involving adults. Similar results were reported in a case series of seven pediatric patients. Meticulous skin care with minimal debridement is an important part of management and may be accomplished best at a specialized pediatric burn center. Epidermal bacterial invasion is common, and infection is the most frequent cause of death. American Board of Pediatrics Content Specification(s):Recognize life-threatening complications of erythema multiforme major (Stevens-Johnson syndrome)Recognize the signs and symptoms of erythema multiforme major (Stevens-Johnson syndrome)Differentiate between erythema multiforme major (Stevens-Johnson syndrome) and other exfoliative dermatosesPlan the management of erythema multiforme major (Stevens-Johnson syndrome)Question 3An 8-year-old boy is brought to the emergency department after being struck by a car. He is hemodynamically stable and had no loss of consciousness. His cervical spine films appear normal, but he has multiple left rib fractures as well as a pelvic fracture. No blood is visible at the meatus, but he is unable to void when asked for a urine specimen.Of the following, the MOST appropriate next steps in the evaluation of his potential genitourinary tract injuries are toA.catheterize the patient to obtain urine, and if hematuria is present, perform computed tomography (CT) scanB.catheterize the patient to obtain urine, and if only microscopic hematuria is seen, undertake no further imagingC.perform retrograde urethrography and place a Foley catheter if there is no extravasation of contrastD.perform a suprapubic puncture to obtain urine, followed by a CT scan if any degree of hematuria is notedE.place a suprapubic catheter and perform antegrade cystourethrographyCorrect answer: CThe child described in the vignette, who has sustained a pelvic fracture, is at risk for urethral injury. Although there is no blood at the meatus, his inability to void may indicate urethral disruption. Such a possibility must be evaluated before placing a Foley catheter because catheter placement may convert a partial urethral disruption into a complete disruption. Therefore, retrograde urethrography is indicated, in which a catheter is placed into the distal urethra just far enough to allow retrograde injection of contrast. The absence of contrast extravasation indicates no urethral disruption, at which point a Foley catheter may be placed safely. A pelvic hematoma may displace the bladder significantly, making blind percutaneous placement of a suprapubic catheter or attempt at suprapubic puncture difficult. Ultimately, open surgical suprapubic catheter placement may be required for long-term management. Finally, the inability to void may indicate a bladder rupture from blunt force on a full bladder or from laceration by the fractured pelvic bones. This also could make percutaneous placement of a suprapubic catheter or a suprapubic puncture difficult or impossible. Intraperitoneal bladder ruptures require surgical repair, whereas extraperitoneal ruptures may be managed with a Foley catheter.A urine sample is necessary to evaluate for hematuria. All patients who have gross hematuria require further imaging (some investigations in children suggest further imaging for microscopic hematuria of greater than 50 red blood cells per high-power field). Polytrauma with rib fractures may be associated with renal injury. Ureteral injuries occur almost exclusively with penetrating trauma. The presence of any hematuria in the pediatric patient who has polytrauma, most commonly as a result of vehicular and pedestrian crashes, requires further evaluation. Such evaluation typically includes CT scan of the abdomen and pelvis in the stable patient. For patients who have more minor trauma (eg, sports-related injuries, low-impact fall) and do not sustain other serious injuries, microscopic hematuria alone is not an indication to perform CT scan. Almost all such patients have injuries that do not require urologic intervention and, therefore, do not require further imaging unless the hematuria persists for more than 1 month. In adults, the presence of hypotension in association with any degree of hematuria may indicate a serious renal injury and requires further imaging. Hypotension is an unreliable indicator of the severity of renal injury in children; the mechanism of injury in association with polytrauma dictates the need for imaging. Intravenous pyelography, once the study of choice for evaluation of renal injury, no longer is recommended in stable patients, who can undergo CT scan. “Single-shot” intravenous pyelography still is used occasionally for patients who require emergent surgery and do not have time for CT scan.American Board of Pediatrics Content Specification(s):Understand the importance of the mechanism of injury and pelvic trauma in evaluating genitourinary tract damagePlan the evaluation and management of a child with post-traumatic hematuriaRecognize and plan the evaluation of urethral traumaQuestion 4You are evaluating an 11-year-boy who was an unrestrained passenger in a high-speed motor vehicle crash. Vital signs include a heart rate of 130 beats/min, respiratory rate of 16 breaths/min, blood pressure of 90/70 mm Hg, and oxygen saturation of 90% on room air. He is fully immobilized and has stertorous respirations, good pulses, and cool extremities. He is obtunded and moans to noxious stimuli. In addition, he has a briskly bleeding scalp laceration, symmetrically reactive pupils, “raccoon” eyes, and a distended abdomen. You initiate assisted ventilation with bag-mask-valve ventilation and secure the airway with a rapid sequence induction/intubation technique. Of the following, the MOST appropriate combination of medications to facilitate intubation in this patient is A.atropine, thiopental, succinylcholineB.fentanyl, propofol, vecuroniumC.lidocaine, etomidate, rocuroniumD.lidocaine, midazolam, fentanylE.atropine, morphine, midazolam Correct answer CThe patient described in the vignette has sustained significant traumatic brain injury and has the potential for increased intracranial pressure (ICP). His vital signs and cool extremities suggest that he is in a state of compensated shock. His abdominal distention suggests he is at risk for blunt intra-abdominal injury, and his stertorous respirations and obtundation suggest an impaired ability to maintain and? protect his airway. Establishing a definitive airway using rapid sequence induction/intubation (RSI) technique is one of the top priorities for this critically ill patient.RSI involves sequential administration of pharmacologic adjuncts, including sedative and paralytic agents at a minimum, to facilitate intubation. For the boy in the vignette, the combination of lidocaine, etomidate, and rocuronium is the preferred combination and sequence to address these goals. The inclusion of lidocaine is theoretically helpful for this patient, who has evidence of increased ICP. The goals of RSI are to minimize adverse physiologic effects and maximize the success rate of endotracheal tube placement. Sedatives help to avoid the psychological trauma associated with chemical paralysis and placement of an endotracheal tube. Paralysis eliminates resistance to direct laryngoscopy, suppresses cough that may increase ICP during airway manipulation, and facilitates passage of the tube through the glottis.The basic steps of RSI involve the “7 Ps”: preparation, premedication, pre-oxygenation, put to sleep (rapid, deep sedation), pressure on the cricoids (Sellick maneuver), paralysis, passing the tube (intubation), and confirming placement. Bag-valve-mask ventilation should be minimized or avoided, unless hypoxia develops, to limit gastric distention, which may increase the risk of aspiration.Preparation of airway equipment includes alternative airway adjuncts in the event of an inadvertent difficult airway or failed intubation. Preoxygenation is important to maintain oxygenation when apnea is induced by the medications. It maintains normoxia by increasing oxygen reserve and washing out nitrogen from the alveoli. Children have greater oxygen consumption (6 mL/kg per minute) and lower functional residual capacity than do adults. A healthy adult can tolerate apnea without hypoxemia for 8 minutes after preoxygenation for 5 minutes, but an infant cannot tolerate apnea more than a few minutes without developing hypoxia (oxygen saturation <90%). Clinical Scenario?Suggested Sedatives ?Compensated shock/hypovolemia with head injury?Etomidate, midazolam?Compensated shock/hypovolemia without head injury?Ketamine, etomidate, midazolam?Decompensated shock?Ketamine, etomidate, half-dose midazolam?Status asthmaticus?Ketamine, midazolam, propofol?Isolated TBI?Propofol, etomidate, thiopental?Status epilepticus?Midazolam, thiopental, propofol?Euvolemic?Midazolam, thiopental, propofol?Premedication with lidocaine, opiates, atropine, or a defasiculating dose (recalled by the mnemonic “LOAD”) of nondepolarizing agent may be useful, if time permits. Lidocaine is believed to suppress local airway reflexes and blunt elevation in ICP. Fast-acting opiates such as fentanyl provide analgesia during intubation and consequently decrease the sympathetic response. Atropine is a useful adjunct to blunt the vagally mediated bradycardia frequently encountered during airway manipulation in children younger than 2 years of age. A small (1/10th the regular dose) initial defasciculating dose of a competitive neuromuscular blocking agent such as vecuronium or rocuronium can decrease the fasciculations encountered with a depolarizing agent such as succinylcholine. Of note, succinylcholine is relatively contraindicated in patients who have increased ICP. There is also a small risk of malignant hyperthermia and prolonged paralysis with the use of succinylcholine, which is increased in a child who has myopathy. Etomidate is hemodynamically neutral compared with propofol or thiopental and is the drug of choice in head-injured patients who have shock. The Table shows a list of suggested sedatives for selected clinical situationsThe use of ketamine in those who have head injuries is controversial; robust evidence of a detrimental effect on ICP in such patients is lacking. In fact, by increasing mean arterial pressure and consequently cerebral perfusion pressure, ketamine may be beneficial in patients who have concomitant hemodynamic instability. The use of atropine, thiopental, and succinylcholine is not optimal because of the hemodynamic effect of thiopental in this patient, who has compensated shock. The combination of fentanyl, vecuronium, and propofol is inappropriate because of the adverse hemodynamic effect of propofol. The use of midazolam, fentanyl, and lidocaine or morphine, atropine, and midazolam are inappropriate because neither combination includes a paralytic agent.American Board of Pediatrics Content Specification(s):Know the anatomy and/or physiology relevant to rapid sequence induction for intubationDiscuss the indications and contraindications for rapid sequence induction for intubationDescribe the key steps and potential pitfalls in performing rapid sequence induction for intubationDiscuss the complications associated with rapid sequence induction for intubationQuestion 5A 14-year-old boy is brought to the emergency department after experiencing a brief syncopal episode preceded by shortness of breath during basketball practice. On physical examination, he is asymptomatic, alert, and in no distress. His vital signs are within normal parameters. You can hear a midsystolic harsh ejection murmur at the lower left sternal border without any clicks or gallop. Of the following, the MOST appropriate next step is to obtainA.echocardiographyB.stress testingC.tilt table testingD.24-hour ambulatory electrocardiographic monitoringE.urine drug screeningCorrect answer: AThe boy described in the vignette experienced syncope associated with exertion, and his physical examination reveals a murmur whose description is typical for hypertrophic obstructive cardiomyopathy (HOCM). Echocardiography is diagnostic for HOCM. As many as 90% of children have an audible murmur at some point in time, particularly during a febrile illness. However, fewer than 5% of heart murmurs in children denote cardiac pathology (Table 1 and 2). If the patient is asymptomatic, does not have extracardiac congenital malformation or evidence of cardiac abnormality, and has a murmur that demonstrates characteristic features of an innocent murmur, additional evaluation is not warranted. The characteristics of an innocent murmur are described in Table 1. Careful auscultation, concentrating on one segment of the cardiac cycle at a time, is important. Table 1: Innocent MurmursTypeShapeTimingLocationCommentsVibratoryEjectionMidsystolicLower left sternal border-apex≤Grade 2 Venous humDiamondContinuousSubclavicularDisappears in supine positionPulmonary flowFlowSystolicUpper left sternal borderNormal S2 splitPeripheral branch pulmonary stenosisEjectionSystolicEntire chestRadiates to back; disappears by 6 months of ageCarotid bruitEjectionSystolicBase of neckDecreased by carotid compressionTable 2: Organic MurmursLesionTimingLocationCommentsVentricular septal defectHolosystolicLower left sternal border Apical mid-diastolic rumbling murmur with large shuntHypertrophic obstructive cardiomyopathyEjection, systolicLower left sternal border to apexLaterally displaced point of maximal impulse, harshMitral regurgitationHolosystolicApexHigher pitched than ventricular septal defectCoarctationEjection systolicUpper left sternal border to left backPulse disparity between upper and lower limbsPulmonary valve stenosisEjection systolicUpper left sternal borderEjection click, wide split S2Tetralogy of FallotEjection, systolicMiddle left sternal borderCyanosisAtrioseptal defectEjection, systolicUpper left sternal borderPersistent split S2Patent ductus arteriosusContinuous, diamond shapedUpper left sternal borderWide pulse pressureAortic stenosisSystolic, ejectionUpper right sternal borderEjection click with valvular stenosisExercise-induced vasovagal syncope usually occurs immediately following intense exercise. If the patient is allowed to lay supine, he or she usually recovers consciousness promptly. Although the diagnosis of vasovagal syncope can be established with tilt table testing, this maneuver lacks the specificity necessary to exclude life-threatening causes of exercise-induced syncope. Holter monitoring (24-hour ambulatory electrocardiographic monitoring) rarely is indicated in the evaluation of children who have syncope because the likelihood of a clinical event occurring within 24 hours is low. Thirty-day event recorders may capture arrhythmic events in patients who have intermittent symptoms.Stress testing with exercise myocardial perfusion scanning may have a role in risk stratification of patients who have HOCM because ischemia is postulated to be a mechanism of syncope in affected patients. However, stress testing cannot be used to screen for HOCM.A urine drug screen would be helpful in confirming abuse of street drugs. However, it would not be helpful in identifying the cause of the pathologic murmur.HOCM is attributed to multiple gene mutations affecting several proteins of the sarcomere. A family history of HOCM may be elicited in affected patients. Classic HOCM is associated with asymmetric hypertrophy of the interventricular septum that is greater than the left ventricular free wall hypertrophy. Dynamic obstruction of the left ventricular outflow tract, so-called idiopathic hypertrophic subaortic stenosis (Figure), is noted in many patients. Electrocardiography results may be normal initially, but with progression of hypertrophy, evidence of left ventricular hypertrophy and ST segment abnormalities become apparent. Chest radiography does not reliably demonstrate cardiomegaly and may appear normal.Other serious or life-threatening causes of murmurs include valvulitis in association with rheumatic fever and myxoma. Valvulitis in association with acute rheumatic fever most commonly affects the mitral valve and produces a transient short mid-diastolic murmur (Carey Coombs). Untreated rheumatic fever can lead to destruction of the valve and result in mitral or aortic stenosis or insufficiency. A myxoma is the most common cardiac tumor that can occur at any age. The signs and symptoms usually stem from obstruction of the ventricular outflow tract or of the mitral valve as the tumor moves with blood flow.Acute severe mitral regurgitation can occur in other clinical settings. These include infective endocarditis with destruction of the leaflet tissue; papillary muscle rupture as a delayed complication of acute myocardial infarction; blunt chest wall trauma (often seemingly trivial) leading to papillary muscle contusion or rupture or detachment of the chordae; and rupture of the chordae tendineae in the setting of a myxomatous mitral valve disease, usually as part of a generalized connective tissue disorder, as seen in Marfan syndrome.American Board of Pediatrics Content Specification(s):Know the etiology by age and the pathophysiology of cardiac murmursRecognize serious and/or life-threatening causes of cardiac murmursQuestion 6A 19–month–old boy is brought to the emergency department due to rectal bleeding. According to his parents, the boy has passed a large amount of dark red blood three times over the past 2 hours. He had two other episodes of rectal bleeding in the past, but these were of smaller volume and were believed to be due to food allergies. Today he has not vomited, has not had diarrhea, and does not seem to be in pain. He is afebrile. On physical examination, the pale-appearing and quiet boy has a temperature of 99.0°F (37.2°C), heart rate of 148 beats/min, respiratory rate of 32 breaths/min, blood pressure of 72/53 mm Hg, and pulse oximetry of 98% in room air. His mucous membranes are pale but moist; his lungs are clear; and he has no grunting, flaring, or retractions. He has a regular heart rate and rhythm, with a II/VI systolic ejection murmur and capillary refill of 2 to 3 seconds. His abdomen is flat, soft, and nontender. He has increased bowel sounds; there is no hepatosplenomegaly or palpable mass. Genitourinary examination findings are unremarkable. Rectal examination shows dark red blood with some stool that is heme-positive. You obtain intravenous access and send blood for a complete blood count, coagulation studies, and type and cross-matching for blood products. A gastric aspirate (via nasogastric tube) is not bloody and is heme-negative. You administer 20 mL/kg 0.9% sodium chloride solution.Of the following, the MOST appropriate next step is toA.consult gastroenterologyB.consult surgeryC.order abdominal computed tomography scanD.order abdominal ultrasonographyE.order contrast enema Correct answer BInitial priorities in the management of hematochezia are to assess and preserve hemodynamic stability while attempting to localize the site of bleeding. Patients who present with mild signs and symptoms may be managed expectantly and in a stepwise fashion. However, patients who have significant hematochezia, such as the boy described in the vignette, require intravenous access, appropriate laboratory studies, and preparation for possible transfusion. At the same time, the clinician must localize the source of the bleeding to either the upper or lower gastrointestinal tract.? A nasogastric tube should be passed and an aspirate obtained to determine if there is blood in the stomach. The boy in the vignette has no evidence of upper gastrointestinal bleeding, based on the nonbloody, heme-negative gastric aspirate. In this situation, the presence or absence of signs of intestinal obstruction (acute abdominal pain, vomiting, abdominal distention) helps to guide the ensuing diagnostic evaluation. The investigation of lower gastrointestinal bleeding is guided by patient age and clinical presentation. Intussusception and midgut volvulus are the most common causes of intestinal obstruction associated with lower gastrointestinal hemorrhage, but the bleeding experienced by the boy in the vignette is painless, and there are no signs of obstruction. The most common causes for lower gastrointestinal bleeding in a child who has this presentation in this age group are Meckel diverticulum, juvenile polyps, vascular malformation of the gut, and infectious colitis (Table). Inflammatory bowel disease (especially ulcerative colitis) also could have this type of presentation, but it would be unusual in this age group, and brisk, painless lower gastrointestinal bleeding is an uncommon presentation of inflammatory bowel disease.The brisk nature of the bleeding, the young age of the patient, and the history of prior episodes of bleeding reported for this boy make a Meckel diverticulum the leading possibility. Meckel diverticulum is present in 2% of the population at birth, but only 2% of affected people ever have related symptoms. Usually the diverticulum is located about 50 to 75 cm (approximately 2 ft) proximal to the terminal ileum. The diagnostic radiology study of choice is a radionuclide scan, but the sensitivity of the test is decreased in the presence of brisk bleeding. In addition, the brisk bleeding in this patient has produced signs of compensated shock. The location of a Meckel diverticulum reduces the likelihood that endoscopic evaluation can determine the cause of bleeding or treat it. Accordingly, consultation with a gastroenterologist is unlikely be of immediate benefit. Surgical intervention, by either laparotomy or laparoscopy, is indicated in a patient who has had such significant bleeding. Plain radiographs are helpful to investigate for the presence of free air and to exclude other causes associated with obstruction. Computed tomography scan, ultrasonography, and contrast enemas are unlikely to demonstrate the cause of bleeding in this patient and may delay definitive care. American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of rectal bleeding.Plan diagnostic evaluation and initial intervention for patients with rectal bleeding.Question 7A 16-year-old girl presents to the emergency department with a rash (Fig. 1), which appears to be composed of copper-colored macules located on both palms. The only finding of note in her past medical history is the previous diagnosis of a sexually transmitted infection. She has no constitutional symptoms and reports that the rash has developed slowly. Figure 1Of the following, the MOST appropriate evaluation and treatment for this patient isA.blood culture and ceftriaxoneB.gonorrhea/chlamydia ligase chain reaction test and azithromycinC.reassurance and outpatient follow-upD.Rickettsia rickettsii titers and doxycyclineE.serum VDRL and penicillin Correct answer EThe history of a previous sexually transmitted infection (marker for unprotected sexual intercourse) plus the distribution and characteristics of her rash suggest that the girl described in the vignette most likely is suffering from secondary syphilis. Acute syphilis generally is diagnosed by direct demonstration of the spirochete organisms via microscopy or direct fluorescent antibodies. Secondary syphilis is diagnosed through indirect serologic methods. The Venereal Disease Research Laboratory (VDRL) test and the rapid plasma reagin (RPR) test are used most commonly. Such tests also are useful for monitoring responses to treatment. Because the tests measure? immunoglobulin G responses to the infections, results may be false-negative early during the acute infection. In addition, certain medical conditions (eg, systemic lupus erythematosus) are associated with false-positive results. The most specific for the diagnosis of syphilis is the fluorescent treponemal antibody test (FTA), which can be ordered if the diagnosis is in doubt. Secondary syphilis is treated with a single intramuscular dose of 50,000 units/kg benzathine penicillin G.The incidence of syphilis and other sexually transmitted infections has increased during the past 2 decades, possibly because of reduced public caution related to the successful treatment of human immunodeficiency virus infections. A painless genital papule progressing to a chancre (ulcerated lesion) characterizes primary syphilis. Such lesions often are not noticed in females because they are internal. The characteristic skin findings of secondary syphilis include disseminated papules, often on the palms and soles. Constitutional symptoms of fever and malaise may be present. Although the organism can be demonstrated in the cerebrospinal fluid of up to 40% of affected patients during this phase, only 1% to 2% present with clinical signs and symptoms of aseptic meningitis. Without treatment, most patients become asymptomatic during the secondary stage. Prenatal screening of pregnant women is designed to discover those who are chronically infected to prevent the consequences of congenital syphilis. Congenitally acquired syphilis has a wide spectrum of manifestations, ranging from in utero death to “snuffles,” macular papular rash in the neonatal period, and notched upper incisors in older children (Fig. 2). Figure 2The rash associated with disseminated gonorrhea infections usually is red papular or petechial, not copper-colored macular. Blood culture and intravenous antibiotics are appropriate management for disseminated gonococcal disease, which is unlikely in this patient, given the appearance of the rash and the absence of constitutional symptoms. Because syphilis is a reportable disease that requires therapy, outpatient follow-up is not appropriate prior to laboratory evaluation and treatment. Rocky mountain spotted fever (caused by Rickettsia rickettsii) can present with rash that is similar to that of secondary syphilis, but affected patients usually appear ill. American Board of Pediatrics Content Specification(s):Recognize the signs and symptoms of syphilisPlan management including ancillary studies of acute and chronic syphilisQuestion 8A 10-year-old Amish boy presents to the emergency department with complaints of pain and spasms in his right foot. He injured the foot 10 days ago when he stepped on a pitchfork while walking barefoot in the barn. He began having severe pain and episodic spasms in the foot yesterday, which now have progressed to involve the entire leg. The boy also is beginning to experience jaw pain and stiffness and difficulty swallowing. His past medical history offers no findings of note, although he is unimmunized. Physical examination reveals an alert child in obvious discomfort whose temperature is 100.0°F (37.8°C), heart rate is 130 beats/min, respiratory rate is 20 breaths/min, and blood pressure 130/90 mm Hg. He has trismus on head and neck examination, and the right foot has a plantar puncture wound without surrounding erythema. The child has intermittent severe pain with visible muscle spasms. Other aspects of the physical examination are normal.Of the following, the MOST appropriate next steps are A.bone scintigraphy scan, administration of intravenous clindamycin, and orthopedics consultationB.electromyography, administration of intravenous immune globulin, and neurology consultationC.lumbar puncture, administration of intravenous vancomycin and ceftriaxone, and hospital admissionD.wound culture and administration of intravenous penicillin G and botulinum antitoxinE.wound debridement and administration of tetanus immune globulin and intravenous metronidazole Correct answer EThe unimmunized boy described in the vignette displays typical signs and symptoms of generalized tetanus: painful muscle spasms and trismus after suffering a contaminated puncture wound. Tetanus is diagnosed by recognition of the clinical presentation; tests such as electromyography and lumbar puncture rarely aid in ascertaining the diagnosis. Although the causative organism, Clostridium tetani, or its? spores can be isolated from wounds in up to one third of patients, failure to do so in most patients mandates that treatment for suspected tetanus not be based on culture results. Treatment is directed at neutralizing the exotoxin tetanospasmin with tetanus immunoglobulin (TIG), stimulating additional antibody production with tetanus toxoid, eradicating the causative organism with antibiotics (metronidazole or penicillin G) and wound debridement, and supportive care. In areas where TIG is not available, bovine or equine tetanus antitoxin can be used, although there is an increased risk of serum sickness with these products. Bone scintigraphy and intravenous antibiotics are appropriate for suspected bone or joint infection, but the absence of fever or inflammatory changes around the wound makes this diagnosis unlikely for this boy. Electromyography findings typically are normal or nonspecific in cases of tetanus; electromyography, intravenous immune globulin, and neurology consultation are appropriate for suspected Guillain-Barré syndrome. Guillain-Barré syndrome typically presents with ascending flaccid paralysis, loss of deep tendon reflexes, and autonomic instability. Lumbar puncture and intravenous broad-spectrum antibiotics are indicated if bacterial meningitis is suspected, but this diagnosis is unlikely with the absence of fever, neck pain or stiffness, headache, or neurologic signs or symptoms reported for this child. Wound culture and administration of intravenous penicillin G and botulinum antitoxin are appropriate for suspected wound botulism, which typically presents with a descending flaccid paralysis.Tetanus is a neurologic disease caused by the exotoxin tetanospasmin that is produced by the vegetative form of C tetani. C tetani is a spore-forming, anaerobic, gram-positive bacillus found in soil and animal and human intestinal tracts. The organism is present worldwide, with cases of tetanus occurring more commonly in warmer climates or during warm seasons in temperate climates. Once C tetani spores are inoculated into a wound, an incubation period of 2 days to several months precedes the onset of painful muscle spasms that are exacerbated by external stimuli. The organism does not cause tissue destruction and does not elicit an inflammatory response. During the incubation period, spores convert to the vegetative form of the organism, which then elaborates tetanospasmin. Wounds contaminated with dirt or soil and that involve necrotic or devitalized tissue provide an ideal anaerobic environment for spore germination. The heat- and antiseptic-resistant toxin enters local peripheral nerves and travels to lower motor neurons within the brain and spinal cord. The toxin binds to neuronal membranes and blocks the release of neurotransmitters, which results in the loss of inhibitory impulses, leading to muscular spasms and sympathetic overactivity. Toxin bound to neurons no longer can be neutralized by antitoxin. Localized tetanus is an uncommon manifestation of C tetani infection in which localized toxin uptake results in painful muscle spasms isolated to the region of the wound. Such a reaction may occur when there are adequate concentrations of serum antitoxin to prevent systemic spread of the toxin but not to prevent local uptake into the peripheral nerves surrounding the wound. Localized tetanus may progress to the generalized form before recognition of the symptoms. Cephalic tetanus is also uncommon and is characterized by isolated cranial nerve involvement following contamination of a wound to the head or neck region; mortality for this form of tetanus is high. Generalized tetanus is the most common manifestation of C tetani infection (75% to 80% of cases). Fifty percent of patients present with trismus due to masseter and parapharyngeal muscle spasm, followed by progression to more diffuse muscle involvement, including paraspinal and abdominal musculature. Muscle spasms may be so severe as to result in opisthotonic posturing, spinal and other fractures, or potentially fatal laryngeal muscle spasm. The “spatula test” is a simple bedside test that can help diagnose tetanus. When a spatula or tongue blade is used to touch the oropharynx, a gag reflex is normally elicited, except in cases of tetanus, where the stimulus triggers a reflex spasm of the masseter muscles, causing the patient to bite down hard on the tongue blade. Neonatal tetanus is a generalized form of the illness that occurs in newborns born to unimmunized mothers when nonsterile umbilical cord care practices are used. The mortality rate for neonatal tetanus is extremely high and often related to secondary complications such as pneumonia, intracranial hemorrhage, or laryngospasm. The overall mortality rate for patients who have tetanus is 45% to 55% worldwide, with rates closer to 60% for neonatal tetanus and only 1% for localized disease. Severe and potentially life-threatening complications of tetanus include aspiration pneumonia, laryngospasm, hypoxic brain injury due to respiratory compromise, rhabdomyolysis, fractures, deep venous thromboses and pulmonary emboli, and severe autonomic instability. Advances in intensive care and supportive therapies in developed countries have improved patient outcomes; current mortality for tetanus in the United States is less than 30%. Supportive care for affected patients is aimed at controlling the painful muscle spasms (benzodiazepines and neuromuscular blocking agents), supporting respiratory function (suction, oxygen, and mechanical ventilation), maintaining hydration and nutritional status (fluids, parenteral nutrition), preventing and treating secondary pneumonia or other infections, and managing sympathetic overactivity (α- and β-blocking agents). Widespread immunization in developed countries has drastically reduced the incidence of tetanus, with fewer than 50 cases per year in the United States. American Board of Pediatrics Content Specification(s):Know the etiology of tetanusUnderstand pathophysiology of tetanusRecognize signs and symptoms of tetanus Recognize life-threatening complications of tetanusMARCH 2010Question 1A 4 month-old infant is brought to the emergency department for evaluation of difficulty breathing. According to his parents, the baby has had rapid breathing and occasional cough that have been worsening progressively over the past 3 weeks. He has had no fevers, rhinorrhea, or drainage from the eyes or ears. He has had more frequent episodes of vomiting after feedings and has been feeding poorly for the past several days. The parents have noted rapid breathing, retractions, and sweating with feedings but no cyanosis or apnea. On physical examination, the infant’s temperature is 37.0°C, heart rate is 168 beats/min, respiratory rate is 70 breaths/min, blood pressure is 78/60 mm Hg, and PO2 is 94% on room air. The alert infant exhibits mild respiratory distress, and chest examination reveals subcostal retractions as well as fine wheezes and rales throughout both lung fields. Cardiac examination shows a normal S1 and S2 and a prominent S3 but no murmurs. The liver is palpable 4 cm below the right costal margin. The remainder of the examination findings are normal. You obtain a chest radiograph (Fig. 1).Figure 1: Chest radiographOf the following, the MOST appropriate next steps to establish the diagnosis are toA.obtain blood for arterial blood gas and electrolyte assessmentB.obtain respiratory specimens for influenza and respiratory syncytial virus rapid antigen testingC.obtain specimens for blood and urine cultureD.order electrocardiography and echocardiographyE.perform endotracheal intubation and bronchoscopyCorrect answer: DThe infant described in the vignette is displaying signs and symptoms of congestive heart failure, and the chest radiograph reveals cardiomegaly and pulmonary edema. Electrocardiography, although not typically diagnostic for the exact defect, can provide evidence to support the presence or absence of arrhythmia, ischemia or infarction, atrial or ventricular hypertrophy, or conduction abnormalities possibly contributing to the patient’s symptoms. Echocardiography and cardiology consultation should be obtained as early as possible to define the cardiac anatomy and function, likely provide the diagnosis, and guide necessary treatment for this infant. The infant in the vignette had an anomalous left coronary artery arising from the pulmonary artery. Her electrocardiographic tracing showed left ventricular hypertrophy with strain and ischemia (ST segment depression and T wave inversion) (Fig. 2).Figure 2: Electrocardiographic tracing documenting biventricular hypertrophy with strain patternAlthough an acute upper respiratory tract infection may exacerbate symptoms in a child who has underlying cardiac disease, testing for viral pathogens will not aid in diagnosing the disorder causing the child’s symptoms. The infant in the vignette is not demonstrating signs or symptoms of respiratory failure and, therefore, does not require endotracheal intubation. Arterial blood gas and electrolyte assessment may be indicated in the evaluation of such a patient, but neither will establish a diagnosis. The infant has no history of fever to suggest the need for obtaining a blood or urine culture.Heart failure is defined as a clinical syndrome resulting from the inability of the heart to provide adequate blood flow to meet metabolic demands of the tissues. Such inadequacy can be due either to intrinsic cardiac abnormalities or to conditions that place an abnormal load on the heart, leading to cardiac dysfunction. In many cases, both an underlying cardiac abnormality and a precipitating cause such as anemia, viral infection, or hypertension precipitate the condition. The most common causes of congestive heart failure (CHF) vary by age.Causes can be divided into broad categories: primary myocardial dysfunction (ischemic heart disease, cardiomyopathy), increased ventricular loads (volume or pressure), restrictive diseases (pericarditis, endocardial fibroelastosis), and electrical abnormalities (arrhythmia). Structural heart defects (resulting in volume or pressure overload) predominate in neonates and infants; cardiomyopathy related to ischemic or other insults is the most common cause in adults. Regardless of the cause, the primary pathophysiology in CHF relates to myocardial dysfunction. Which symptoms predominate initially depends on the site of primary dysfunction. Left ventricular dysfunction leads to pulmonary congestion, decreased renal and systemic perfusion resulting in fluid retention, and decreased gut and skeletal muscle blood flow. Right ventricular dysfunction leads to peripheral edema and hepatomegaly. As CHF progresses, hormonal and neuronal responses to diminished blood flow activate the renin-angiotensin-aldosterone system and the sympathetic nervous system. Such activation results in altered distribution of blood flow, increased vascular tone, tachycardia, and myocardial remodeling/hypertrophy. These responses to poor cardiac function and decreased blood flow cause further increases in myocardial oxygen consumption and propensity for arrhythmias and, if the CHF is treated inadequately, ultimately lead to further cardiac dysfunction.?? The classic symptom of CHF is exertional dyspnea, which is typically the earliest complaint; progressive fatigue and weakness follow, with peripheral edema and dyspnea at rest coming later. These symptoms are related to the inability of the dysfunctional myocardium to increase blood flow as needed during exertion and decreased skeletal muscle perfusion due to redistribution of blood flow. As CHF progresses and pulmonary edema and diminished lung compliance occur, patients complain of shortness of breath when lying flat (orthopnea) and the sudden onset of severe shortness of breath during sleep (paroxysmal nocturnal dyspnea). Cerebral symptoms related to decreased cerebral blood flow occur later in the course of CHF; are more common in elderly patients; and include confusion, memory loss, and poor concentration. In infants, the activity associated with the highest metabolic demands is feeding. Therefore, poor feeding, sweating, and respiratory distress during feedings; irritability; and poor weight gain are the predominant symptoms in this age group.Vital signs in patients who have CHF initially may be normal, but tachycardia and a narrowed pulse pressure develop over time. Tachypnea develops in patients who have pulmonary edema. Pulse oximetry readings may be normal or reveal hypoxemia, depending on the cause and severity of the heart failure. Cardiac examination may reveal abnormal cardiac sounds, with S3 gallops due to poor ventricular compliance being common and S4 gallops heard less commonly. Murmurs may be present in cases involving structural heart disease or valvular insufficiency related to hypertrophy. Lung examination may reveal only tachypnea and increased work of breathing, especially in infants. In older children and adults and as CHF progresses, rales or wheezes may become prominent. Hepatomegaly is common if right heart failure is present, and the liver may be pulsatile. Jugular venous distension may be appreciated, although this is rare in infants. Hepatojugular reflux is reflected by increased jugular venous distension when pressure is applied over the liver. Peripheral perfusion often is poor due to vasoconstriction and redistributed blood flow. Pallor, poor peripheral pulses, and cool skin with delayed capillary refill reflect increased vascular tone and diminished cardiac output. Peripheral edema is common in adults who have CHF but less likely in infants and young children. For patients whose CHF is related to systemic diseases, examination findings related to the precipitating disorder may predominate.Chest radiography should be performed in all patients suspected of having CHF; cardiomegaly is present in most cases, with rare exceptions including total anomalous pulmonary venous return with obstruction and restrictive pericardial/myocardial disorders. Pulmonary venous congestion, Kerley B lines, and pleural effusions are common in advanced CHF. Electrocardiography may reveal evidence of ischemia/infarction, cardiac hypertrophy, rhythm disturbances, or voltage abnormalities related to the myocardial dysfunction or the underlying cardiac disorder. Laboratory evaluation should include hemoglobin, cardiac enzymes, electrolytes, and renal function measurement. Beta-type natriuretic peptide is a hormone secreted by the distended left ventricle, and its assessment is helpful in diagnosing disease and monitoring response to therapy. Because hypoglycemia and hypocalcemia may precipitate or exacerbate CHF in neonates and infants, glucose and calcium concentrations should be monitored.? Management of acute CHF is directed at identifying and treating any precipitating causes (infection, anemia), correcting the underlying disorder (surgery for congenital heart lesions), and reversing the pathophysiologic changes outlined previously to maximize myocardial function and output. These goals can be accomplished by decreasing myocardial workload, controlling sodium and fluid retention, and enhancing myocardial contractility. Workload reduction includes activity restriction and small, frequent meals. Afterload reduction, particularly if hypertension is present, may be accomplished with nitrates (nitroglycerin, sodium nitroprusside) or angiotensin-converting enzyme inhibitors. Sodium and fluid retention are addressed through fluid restriction and the use of diuretics. Loop diuretics (furosemide, ethacrynic acid) are used most commonly, and potassium-sparing diuretics such as spironolactone may be added to maximize diuresis while preventing hypokalemia. Myocardial contractility can be augmented through the use of inotropic agents such as digitalis glycosides, isoproterenol, dopamine, dobutamine, or milrinone. Acute symptomatic management for CHF includes reverse Trendelenburg positioning, supplemental oxygen, morphine, positive-pressure ventilation in those who have refractory respiratory failure or severe acidosis, and blood transfusion for those who have concomitant anemia. American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of congestive heart failureRecognize signs and symptoms of congestive heart failureBe familiar with ancillary studies relevant to congestive heart failurePlan management of acute congestive heart failureQuestion 2A 9-month-old girl is being transported to your emergency department by county prehospital providers via ambulance with use of lights and sirens. The initial report via radio communication suggests that the family had called 911 for an alleged fall from the bed and seizurelike activity. The emergency medical service (EMS) providers report that upon arrival at the home, the child was pale and dusky and had poor respiratory effort. Her heart rate was 210 beats/min, with strong pulses and a normal blood pressure. She did not respond to painful stimuli, and her eyes were deviated to the right. The EMS providers initiated assist ventilation using bag-mask, established an intravenous (IV) line, and administered 1 mg of midazolam for presumed seizures.Of the following, the component of the EMS system that provides ultimate authority for the prehospital care provider to provide these interventions is theA.local agency protocolB.National Association of EMS PhysiciansC.national EMS scope of practiceD.online regional medical directorE.state office of EMSCorrect answer: EProtocols for prehospital care in the United States, such as the provision of midazolam to a child who is having seizures, are developed at the regional level, under the auspices of a regional director. Ultimate authority rests in the state office of EMS, with delegation of this authority to the regions. Regional directors are responsible for planning protocols, conducting quality improvement activities, and supervising education for the prehospital providers. In addition, each EMS service agency within the region is required to have a medical director who oversees all of these activities at the local level.Model protocols and a suggested curriculum for pediatric prehospital education can be found on the National Association of EMS Physicians web site (). In the vignette, the EMT-paramedic (EMT-P) administered midazolam to control seizures without contacting the receiving emergency physician. The EMS provider was following approved pediatric prehospital protocols established under the auspices of the local EMS medical director. This is an example of “offline” medical control. Direct communication and consultation offered by the receiving physician represents “online” medical direction.The scope of practice for prehospital providers depends on their level of training and qualification. Most states have four levels of EMS providers: first responders, EMT–Basic (EMT-B), EMT-Intermediate (EMT-I), and EMT–Paramedic (EMT-P). In a recent document, the National Highway Traffic Safety Administration has renamed these designations. First responder now is termed an emergency medical responder, EMT-B is termed EMT, EMT-I is termed advanced EMT, and EMT-P is termed a paramedic.First responders can open the airway, initiate basic cardiopulmonary resuscitation, and control hemorrhage. EMT-Bs additionally can initiate assist ventilation using bag-mask, administer oxygen, immobilize the cervical spine, and transport the patient. The EMT-I possesses further skills that include the ability to initiate vascular access (IV/intraosseous [IO]), perform defibrillation, and in some states, intubate the trachea. EMT-P is the highest level of qualification for prehospital providers, and they also can administer medications via inhalation, IV, IO, or rectal routes. In some states, the EMT-P also performs rapid sequence intubation aided by sedative and paralytic medications. Because the EMS provider described in the vignette administered a benzodiazepine anticonvulsant via the IV route, he or she is an EMT-P. An EMT-B or EMT-I does not have the qualifications for such medication administration. Some states have another designation, EMT-Defibrillation (EMT-D), for personnel who can defibrillate using automated external defibrillators or conventional defibrillators, in addition to performing the functions of an EMT-B.American Board of Pediatrics Content Specification(s):Know the components of emergency medical service (EMS) systemsUnderstand the legal authority of emergency medical services (EMS) agencies to plan and regulate EMS systemsQuestion 3An 18-month-old boy is brought to the emergency department at 2 pm on Monday for evaluation of vomiting. His mother reports that he began vomiting yesterday and has vomited a total of four times; there was no blood or bile in the emesis. He also has had one watery diarrhea stool, which contained no blood or mucus. He has not had a fever, is still taking food and fluids well, and is otherwise acting normally. He has no prior history of significant vomiting or diarrhea, dehydration episodes, or significant illnesses. On physical examination, the small-for-age child is active and appears well. His temperature is 37.0°C, heart rate is 88 beats/min, respiratory rate is 20 breaths/min, blood pressure is 86/54 mm Hg, and weight is 8 kg. His mucous membranes are moist. His abdomen is soft, nontender, and nondistended with normal bowel sounds; there is no hepatosplenomegaly. The remainder of his examination findings are normal.Of the following, the MOST appropriate next step(s) in evaluating this child is(are) toA.contact the child’s pediatrician to obtain prior growth parameters and well child care historyB.discharge the child with instructions on oral rehydration and follow-up with his pediatricianC.file a child protective services report and take protective custodyD.measure serum electrolytes and renal function and administer an intravenous fluid bolusE.measure serum growth hormone concentrations and obtain radiographs to determine a bone age Correct answer AThe presenting history and physical examination results described for the child in the vignette are consistent with a mild acute gastroenteritis. His weight, however, is well below the third percentile for age. Evaluation of inadequate weight gain requires a thorough history and physical examination; measurement and plotting of an accurate weight, length, and head circumference on a gender-specific National Center for Health Statistics growth chart; and comparison of these parameters to prior measurements. When concerns of inadequate growth arise in the emergency department, efforts should be made to acquire prior growth parameters and health records. If evidence of prior abuse or neglect, poor weight gain or weight loss, or chronic/recurrent illnesses is found, further evaluation is indicated.Although outpatient management with oral rehydration therapy is appropriate for this child’s acute illness, it would not address the?potential for significant failure to thrive (FTT) and possible abuse or neglect. A single abnormally low weight measurement for a child who has otherwise normal examination findings is not sufficient information to warrant a child protective services referral. To undertake laboratory or radiographic evaluation of poor weight gain in the emergency department in the absence of historical or examination evidence of underlying illness or disorder is unlikely to yield useful information. Intravenous fluid therapy is not indicated in a child who has normal examination findings, normal vital signs, and a history of minimal fluid losses.The diagnosis of FTT cannot be based on a single measurement. FTT is defined as weight less than expected based on a child’s age (gestation corrected until 2 years of age), underlying medical condition(s), and genetic potential. FTT is diagnosed when the weight is below the 3rd percentile on more than one occasion, the growth trajectory crosses two or more major percentile lines on a standard growth chart, the weight is less than 80% of ideal body weight for age, the weight for length is below the 10th percentile, or the rate of daily weight gain is slower than expected for age. Certain patient populations, such as those who have Down syndrome or Turner syndrome, should be plotted on diagnosis-specific growth charts.The pattern of abnormality in the various growth parameters may indicate the underlying cause. Parameters that are below the 3rd percentile but follow the normal trajectory indicate constitutional growth delay. Head circumference more than two major percentile lines below the height and weight percentile lines may indicate an underlying neurologic disorder. Decreased length relative to weight is indicative of an endocrinologic disorder.FTT traditionally has been divided into organic (resulting from underlying illness or genetic condition) and nonorganic (also known as psychosocial, the result of environmental factors such as abuse or neglect, mental illness, poverty). This division is rarely clear, and often FTT is the result of a combination of organic and environmental factors. More than 50% of FTT cases in tertiary care centers are primarily the result of psychosocial factors.Evaluation of the patient who has FTT should start with a thorough history. Symptoms of underlying illness resulting in FTT may include feeding or swallowing difficulties, chronic or recurrent vomiting or diarrhea, unexplained rashes, recurrent serious infections, chronic cough and respiratory symptoms, developmental delays or regression, or seizures. A complete psychosocial history should include household composition, family stressors (divorce, unemployment, poverty), feeding practices (including types of foods, mixing of formula, set meal times), history of physical or mental illnesses in caregivers, prior abuse or neglect, and substance abuse. During the evaluation, observation of the parent-child interaction should be documented. Family history should include heights and weights of parents and siblings and any significant illnesses or disorders.A complete physical examination should be performed, with particular attention to evidence of underlying cardiovascular, respiratory, or immunologic disorders. Abnormal lymphadenopathy or hepatosplenomegaly may indicate human immunodeficiency virus infection or other immunologic disorder. Rashes may result from nutritional deficiencies or immune defects.Unfocused laboratory or radiographic evaluation rarely yields significantly abnormal findings. Basic laboratory evaluation for a patient who has significant FTT should start with electrolyte and renal function panels, calcium concentration, complete blood count, urinalysis, and urine culture. Further laboratory testing and any radiographic evaluation should be based on history, examination, or initial laboratory abnormalities.Management of FTT depends on severity, duration, underlying cause, and family dynamics. Treatment of any contributory medical conditions should coincide with maximization of caloric intake, establishment of structured feeding times, and elimination of any disruptive or detrimental feeding practices. Multidisciplinary input frequently is necessary, including physicians, nurses, social workers, dietitians, child life specialists, and psychiatrists. Child protective services should become involved if abuse or neglect is suspected.In the past, prolonged inpatient stays were the standard of care for FTT. Initial inpatient evaluation still may be indicated to allow for a complete psychosocial and physical evaluation and ensure a safe home environment prior to disposition. Documentation of sufficient weight gain in the hospital is supportive evidence of nonorganic FTT, but continued failure to gain weight may be the result of anxiety related to the hospitalization itself. Outpatient evaluation and management depends on communication with the primary physician and the assurance of close follow-up.American Board of Pediatrics Content Specification(s):Understand evaluation of failure to thriveUnderstand treatment of failure to thriveQuestion 4A 5-month-old previously well infant is referred to the emergency department for evaluation of pallor that was detected by his pediatrician during a routine office visit. The mother states that the infant has been asymptomatic, although he has been feeding less than usual, which she attributed to his runny nose and congestion for the past 2 to 3 days. He has no history of fever, weight loss, rash, refusal to feed, difficulty in feeding, cough, or difficulty in breathing. He was born at 37 weeks of gestation, had a birthweight of 3 kg, and had an uneventful perinatal period. There is no pertinent family history. On physical examination, the infant appears to be playful and in no apparent distress. His heart rate is 140 beats/minute and the rest of his vital signs are normal. He has obvious pallor, no visible icterus, and no clinical evidence of congestive cardiac failure. He has no organomegaly, edema, lymphadenopathy, petechiae, or purpura. He has no congenital defects except for a hypoplastic thumb on his right hand. His initial blood tests reveal:Hemoglobin, 6.5 g/dL (65 g/L)Hematocrit, 18% (0.18)White blood cell count, 12.0x103/mcL (12.0x109/L) with 50% neutrophils, 44% lymphocytes, 4% monocytes, and 2% basophilsPlatelet count, 200x103/mcL (200x109/L)Reticulocyte count, <1% (0.1)Negative direct Coombs testMean corpuscular volume (MCV), 102 fLMean corpuscular hemoglobin concentration (MCHC), 28% (0.28)Of the following, the MOST likely diagnosis for this infant isA.anemia of prematurityB.aplastic anemiaC.Diamond-Blackfan anemiaD.physiologic anemiaE.transient erythroblastopenia of childhoodCorrect answer: C?The infant described in the vignette has severe anemia, reticulocytopenia, macrocytosis, a normal white blood cell count, and a normal??platelet count, which suggest pure red blood cell (RBC) aplasia. In the presence of congenital skeletal abnormalities (the hypoplastic thumb), these findings point to the diagnosis of Diamond-Blackfan anemia (DBA), also known as congenital RBC aplasia (Table 1). Aplastic anemia also includes the findings of leukopenia and thrombocytopenia.Table 1: Red Blood Cell (RBC) Disorders?Clinical Presentation??DiseaseHistoryPhysical FindingsLaboratory DiagnosisTreatmentAlpha- and Beta-thalassemia TraitNonePallorFamily historyNonePallorRBC hypochromic, microcyticMCV ↓↓Basophilic stippling (beta-thalassemia trait)↑ A2 Hemoglobin (Hb) (beta-thalassemia trait)Ratio of MCV/RBC <13None to childTest both parentsGenetic counselingAvoid iron therapyDiamond-Blackfan AnemiaFatiguePallorReticulocyte <0.5%Profound anemiaWhite blood cells + platelets normalElevated MCVGlucocorticoidsTransfusionGlucose-6-Phosphate Dehydrogenase (G6PD) DeficiencyNone without oxidant stress (A-variety)Chronic anemiaNeonatal jaundicePallor, jaundice, orthostasisPrecipitous anemiaReticulocyte % ↑Hemoglobinemia ±hemoglobinuriaG6PD assay?Remove precipitating agentSupportiveTransfusionHereditary SpherocytosisNoneAbdominal fullness, early satietyFamily historyHistory of neonatal jaundicePallor, jaundiceSplenomegalySpherocytesCoombs test negativeReticulocyte % ↑Osmotic fragility increasedMean corpuscular Hb concentration ↑No splenectomy if Hb >10 g/dL (100 g/L) and reticulocyte <10%Folic acid (0.5 mg qd <5 years of age; 1.0 mg qd >5 years of age)Splenectomy and immunizations to pneumococcus, Haemophilus influenzae, and meningococcus and penicillin prophylaxisIron DeficiencyFatigueIrritabilityExcess milk intakePallorRBC hypochromic, microcyticMean cell volume (MCV) ↓Serum iron ↓Total iron-binding capacity ↑% Saturation ↓Ferritin ↓Blood in stool or urineRatio of MCV/RBC >13Correct dietEliminate source of bleedingFerrous SO4 6 mg/kg per day elemental ironSevere Sickle Cell SyndromesNeonatal screenFatigueAbdominal, bone painHeadacheChest pain, cough, dyspneaCentral nervous system complainsHematuriaDactylitisPallor, jaundiceNonspecific abdominal signsJoint pain/?swellingEvidence of pulmonary infiltratePriapismNeurologic signsSplenomegaly–?earlyNeonatal screenSickledex positive in trait and diseaseHb electrophoresisReticulocyte % ↑Irreversibly sickled cellsSymptomatic careFolic acid (0.5 mg qd <5 years of age; 1.0 mg qd >5 years of age)Penicillin prophylaxis (125 mg bid <3 years of age; 250 mg bid >3 years of age)AnalgesiaHydrationTransfusion and chelationHydroxyureaTransient Erythroblastopenia of ChildhoodFatigue, irritabilityPallorReticulocyte <0.5%Profound anemiaWhite blood cells + platelets normalSupportiveTransfusionA progressive decrease in hemoglobin after birth that reaches a nadir by 8 to 12 weeks of age is seen in all term newborns and is termed physiologic anemia of infancy. This is not a true anemia but a physiologic reduction in neonatal hemoglobin in response to higher ambient oxygen concentrations and the increasing percentage of adult hemoglobin that occurs after birth, which downregulates erythropoietin production in the neonate. This nadir is lower and reached earlier in preterm infants due to multiple reasons, including lower iron stores (the bulk of transplacental iron transfer does not occur until the late third trimester), lower erythropoietin concentrations, and blood loss due to repeated phlebotomies. A 5-month-old infant born at 37 weeks of gestation is unlikely to have anemia from these two causes. Transient erythroblastopenia of childhood (TEC) is the most common cause of acquired isolated RBC aplasia in children. TEC typically occurs between 6 months and 3 years of age, with most affected children older than 1 year at the time of diagnosis. A transient immunologic suppression of erythropoiesis following a nonspecific viral illness is responsible for the severe anemia. Although the clinical and laboratory picture is similar to DBA, the presence of a normal MCV and normal RBC adenosine deaminase (ADA) concentrations help to distinguish these two conditions (Table 1).Anemia, defined as a reduction in RBC volume or hemoglobin concentration below normal for age, race, and sex, is encountered commonly in clinical practice. Anemia can be classified on a: 1) physiologic basis of decreased production of RBCs, increased destruction of RBCs, or blood loss or 2) a morphologic basis involving RBC volume (MCV), hemoglobin content (MCH), or hemoglobin concentration (MCHC). Although severe anemia requires involvement of a pediatric hematologist, a detailed history, complete physical examination, and a complete blood cell count plus reticulocyte count and a peripheral smear can aid the clinician in categorizing anemia. Anemia in the presence of reticulocytosis indicates a normally functioning bone marrow, suggesting blood loss or hemolysis as the cause; reticulocytopenia suggests bone marrow failure either due to an isolated suppression of RBC precursors or to suppression of all three cell lines, as seen in aplastic anemia. The RBC indices, particularly MCV, can be used to categorize the anemia further as normocytic, microcytic, or macrocytic in patients who have low reticulocyte counts (Table 2). The subsequent evaluation and management depend on the suspected cause. DBA is a rare disorder causing severe anemia due to isolated RBC aplasia that usually is detected within first 6 postnatal months. Nearly 50% of affected infants have congenital abnormalities that include short stature, craniofacial dysmorphism, and skeletal defects, with thumb abnormalities being most common. Among the genetic abnormalities are defects in chromosome 8, which lead to an increased apoptosis (programmed cell death) of RBC progenitor cells and subsequent severe anemia. The laboratory findings reveal macrocytic RBCs with reticulocytopenia. Absence of neutrophilic hypersegmentation and normal vitamin B12 and folic acid values rule out other causes of macrocytosis. Patients who have DBA have elevated fetal hemoglobin and ADA activity. Corticosteroids are beneficial in 25% of patients. Those who do not respond to steroid therapy require frequent blood transfusions and subsequent iron chelation therapy with deferoxamine. High-dose intravenous methylprednisone, intravenous immune globulin, and stem cell transplantation are options if patients fail steroid therapy. American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of anemia by ageBe familiar with ancillary studies relevant to anemiaPlan the management of anemiaQuestion 5A 13-year-old boy presents to the emergency department (ED) with a 3-day history of intermittent vomiting and abdominal pain. He states that he has been fatigued and not feeling well for the past 6 weeks and has missed a lot of school. His mother brought him to the family physician 2 weeks ago, who ordered viral studies for “mononucleosis” and “hepatitis,” results of which were normal. The boy has been taking no medications. His mother states that his school performance has declined and she thought he was malingering. The school nurse evaluated him today after an episode of vomiting at school. Upon noting that he appeared jaundiced, she sent him to the ED for further evaluation. On physical examination, the boy is alert and responsive but appears tired. His sclerae are mildly icteric, his skin is pale, and he has no rashes. He is afebrile and has a heart rate of 100 beats/min, respiratory rate of 24 breaths/min, and blood pressure of 105/68 mm Hg. Cardiac auscultation reveals no murmur, and his lungs are clear. His liver edge is firm and palpable 3 cm below the right costal margin, and his spleen is palpable 3 below the left costal margin. Laboratory studies document:Hemoglobin, 11.2 g/dL (112 g/L)White blood cell count, 4.8x103/mcL (4.8x109/L)Platelet count, 100x103/mcL (100x109/L)Reticulocyte count, 2.9%Prothrombin time, 34 secondsPartial thromboplastin time, 64 secondsTotal bilirubin, 3.4 mg/dL (58.2 mcmol/L) ?(direct fraction, 1.4 mg/dL [23.9 mcmol/L])Alanine aminotransferase, 78 units/LAspartate aminotransferase, 84 units/L?Alkaline phosphatase, 120 units/LOf the following, the MOST appropriate step to confirm the suspected diagnosis is toA.measure serum fibrinogen, fibrin split products, and D-dimerB.measure urinary copper excretionC.perform abdominal ultrasonographyD.refer the patient for a bone marrow biopsyE.refer the patient for liver biopsyCorrect answer: BThe boy described in the vignette has hepatosplenomegaly. The laboratory evaluation reveals hyperbilirubinemia, a portion of which is?direct, as well as mild anemia, mild thrombocytopenia, and prolongation of clotting times. The prolonged prothrombin time is evidence of liver synthetic dysfunction. His transaminases are mildly elevated, and his alkaline phosphatase concentration is normal. When patients present to the ED with hepatosplenomegaly, the clinician should consider the five primary categories of disease process causing organomegaly: inflammation, inappropriate storage, infiltration, vascular congestion, and biliary obstruction.Inflammation/infectious causes are unlikely for this boy with his recent negative screens for infectious hepatitis and Epstein-Barr virus. Autoimmune hepatitis remains a diagnostic possibility, although splenomegaly is atypical for this condition. Infiltration due to leukemia or lymphoma should be considered, but the boy’s relatively normal complete blood count and smear make this possibility less likely. The normal alkaline phosphatase value makes biliary obstruction extremely unlikely as a cause for the hepatosplenomegaly. Finally, the boy has no evidence of varices or edema of the lower extremities that suggest vascular congestion.Thus, by exclusion (Fig. 1), diseases associated with inappropriate storage are the most likely cause of the boy’s symptoms.Figure 1: Diagnostic algorithm to arrive at the most common diagnoses for a child older than 1 year of age who has hepatomegaly. His presentation is compatible with Wilson disease, an autosomal recessive disease of ceruloplasmin metabolism that results in excessive copper accumulation in the tissues. Accumulation in the liver causes hepatocellular necrosis, and redistribution causes hemolysis. Findings on liver biopsy are nonspecific, and the boy’s coagulation profile is a relative contraindication to immediate liver biopsy. The diagnosis can be made by measuring high concentrations of urinary copper excretion. Serum ceruloplasmin concentrations typically are low in this disease but may be falsely elevated in acute inflammatory states. Kayser-Fleisher rings, thin dark rings around the limbus of the cornea, are associated with Wilson disease, but often are not present prior to the onset of neurologic symptoms.? Ultrasonography is not an inappropriate test in this case, but it would not yield a diagnosis, and the laboratory findings argue against biliary obstruction. In addition, no evidence suggests vascular obstruction, which could be evaluated by Doppler ultrasonography. A primary hematologic malignancy such as leukemia or lymphoma would not cause a direct hyperbilirubinemia, obviating the need for bone marrow biopsy. Serum fibrinogen, fibrin split products, and D-dimer are laboratory tests that are ordered if disseminated intravascular coagulation is suspected, which is unlikely in the patient described in the vignette.Hepatosplenomegaly in infancy also may result from inflammation, inappropriate storage, infiltration, vascular congestion, and biliary obstruction. Notably, congenital biliary tract obstruction and congestive heart failure, as well as congenital infections, are common causes in this age group (Table 2).As with older children, an orderly approach should be undertaken to evaluate the neonate who has hepatomegaly in the presence or absence of splenomegaly (Fig. 2).Figure 2: Diagnostic algorithm to arrive at the most common diagnosis for a neonate who has hepatomegalyLife-threatening causes of hepatosplenomegaly include fulminant infection with multiorgan system failure, fulminant hepatic failure with hepatic encephalopathy and bleeding diathesis, congestive heart failure, and brisk hemolysis with cardiovascular collapse.American Board of Pediatrics Content Specification(s):Know the etiology by age and the pathophysiology of hepatosplenomegalyPlan diagnostic evaluation and initial intervention for patients with hepatosplenomegalyRecognize serious and/or life-threatening causes of hepatosplenomegalyQuestion 6Emergency medical services personnel arrive with a 14-year-old G1P0 girl in active labor. She did not recognize that she was in labor until she felt fluid draining from her vaginal area. The paramedics report that the fluid is dark green. The girl states that she is 1 week from her due date, has no other medical problems, and had normal prenatal ultrasonography results when the pregnancy was discovered at approximately 28 weeks’ gestation. She denies drug or alcohol use.Physical examination reveals a crowning fetus that has obvious meconium staining. Upon delivery, the infant is limp and has a weak cry and occasional gasping respirations.Of the following, the MOST appropriate initial steps are toA.dry, warm, and stimulate the infant and suction the oropharynxB.perform bag-valve-mask ventilation with 100% oxygen and reassess the infantC.suction the infant’s mouth and nose with a bulb syringe prior to stimulationD.suction the infant’s trachea with a meconium aspirator and repeat until clearE.wait 1 minute, then assess the Apgar score to determine the need for resuscitation Correct answer DThe infant described in the vignette is demonstrating respiratory depression and poor tone in conjunction with meconium-stained amniotic fluid (MSAF). He requires endotracheal suctioning to clear the meconium, which is accomplished best by placing an endotracheal tube and suctioning through the tube with a meconium aspirator. Suctioning should be repeated until no further meconium is present or until bradycardia occurs. Stimulation of the infant, including drying and suctioning the upper airway, should be delayed to help prevent aspiration of meconium into the lungs. Common practice has been to attempt to suction the mouth and nose at the perineum after the head is delivered, but clinical evidence does not demonstrate that this reduces the incidence or severity of meconium aspiration. Current recommendations are to perform tracheal suctioning only on nonvigorous infants who have MSAF. If the infant demonstrates a strong cry and respiratory effort, routine resuscitative measures should be instituted and the infant monitored for signs of meconium aspiration.? Meconium is present in the amniotic fluid in 10% to 15% of all deliveries. It is most common in term or postdates deliveries and rarely occurs before 34 weeks’ gestation. Fetuses exposed to various stressors, including asphyxia, pass meconium from their rectum in utero or during delivery. Aspirated meconium, especially when thick and particulate, may cause airway obstruction and chemical pneumonitis, termed meconium aspiration syndrome (MAS). Respiratory distress, including tachypnea, grunting, and hypoxia, results. Pneumothoraces, both spontaneous and due to positive-pressure ventilation, can occur with MAS. Progressively worsening hypoxia, hypercarbia, and acidosis may result in persistent pulmonary hypertension of the newborn (PPHN). PPHN occurs in approximately 30% of infants who have MAS. Treatment of MAS is largely supportive, with provision of supplemental oxygen and support of ventilation as necessary. Sedation, paralysis, and cardiovascular support may be necessary if PPHN occurs.Tracheal suctioning previously was advocated for all newborns who had MSAF. However, studies have failed to demonstrate improved outcomes with this approach in vigorous infants, and no increased incidence of respiratory distress or MAS has been seen in this population after routine neonatal care without tracheal suctioning. In addition, there is the potential for complications, including airway trauma, hypoxia, reflux bradycardia, and pneumothoraces, when attempting intubation and tracheal suctioning in a vigorous infant.? The Apgar score is assessed routinely at 1 and 5 minutes postdelivery as an indication of the cardiorespiratory status of the newborn and can be used to assess response to resuscitation. The Apgar score never should be used to determine the need for resuscitation, and resuscitative measures should not be delayed or interrupted for performance of this assessment.American Board of Pediatrics Content Specification(s):Know the anatomy and pathophysiology relevant to the prevention and management of meconium aspirationDiscuss the indications and contraindications for prevention and management of meconium aspirationDescribe the key steps and potential pitfalls in the prevention and management of meconium aspirationDiscuss the complications associated with the prevention and management of meconium aspirationQuestion 7A 2-year old boy is brought to the emergency department because of concern that his abdomen seems distended, his face around his left eye seems swollen and bruised, and he seems to be complaining about intermittent pain in his legs and arms.? He was seen recently by his primary care clinician, who expressed concern that the child might have metastatic neuroblastoma because of elevated urinary concentrations of homovanillic acid and vanillylmandelic acid. Of the following, the MOST sensitive and specific ancillary radiologic study to help detect metastatic disease in soft tissue in this patient puted tomography of the chest, abdomen, and pelvisB.magnetic resonance imagingC.radioiodinated metaiodobenzylguanidine (MIBG) scintigraphyD.skeletal radiologic surveyE.ultrasonographyCorrect answer: CAncillary studies relevant to neuroblastoma can be divided into general studies that may provide preliminary information regarding the consequences of tumor infiltration in the liver and bone marrow and more specialized studies that can help to establish the diagnosis, determine the extent of metastatic disease, and even assess response of the tumor to therapy.? Radioiodinated metaiodobenzylguanidine (MIBG) scintigraphy is a specialized radiographic study that uses MIBG, a catecholamine analog?that has competitive uptake in catecholaminergic cells. The analog can localize in neuroblastoma cells in primary sites in up to 95% of patients.? The test is highly sensitive and specific for detecting metastatic disease in bones, soft tissue, and lymph nodes. In addition, it is useful in confirming proper staging and assessing response to therapy. However, some neuroblastomas may not display MIBG uptake, and such scintigraphy does involve radiation exposure for patients.? Technetium-99m-methylene diphosphate (99mTc-MDP) scintigraphy can identify primary neuroblastoma sites, particularly in bone, because 99mTc-MDP is taken up by cells active in bone metabolism. However, only about 74% of primary tumors display 99mTc-MDP uptake. Furthermore, normal growth plates and trauma may contribute to false-positive results.? Despite these disadvantages, most sources recommend 99mTc-MDP scintigraphy if MIBG scans yield negative results for bone involvement. Plain radiographs of the chest and abdomen are relatively easy to obtain and might demonstrate tumor calcifications within the posterior mediastinum or abdomen.? A skeletal radiologic survey, another routine test, also might demonstrate lytic lesions within the cortical bone. However, these routine radiologic studies are neither specific to neuroblastoma nor do they provide any information on the primary site of tumor origin.? Computed tomography scan may be helpful to make the initial diagnosis of neuroblastoma. However, because neuroblastoma is the most likely diagnosis for this boy and the goal is to detect metastatic disease in soft tissue, such a scan is not the best choice. Consultation with an oncologist is recommended when planning evaluation for oncologic disease in the emergency department.Ultrasonography is useful as a screening tool to help identify an intra-abdominal or pelvic mass and characterize particular lesions within the liver without the risk of radiation exposure. Its disadvantages include its operator-dependent nature and limited ability to assess lymph nodes and metastatic disease.? Magnetic resonance imaging demonstrates primary neuroblastoma tumor extension into bone and soft tissues, including the spinal cord and diffuse metastatic hepatic disease, without using ionizing radiation. However, because of its long imaging time, younger patients often require procedural sedation to complete the study. Some of the routine laboratory studies that might provide some useful information when neuroblastoma is suspected are a complete blood count (CBC) and a serum chemistry panel.? Nonspecific tumor marker serum tests include measurement of lactate dehydrogenase (LDH), neuron-specific enolase, and ferritin. The CBC could demonstrate anemia or thrombocytopenia from metastatic disease. The presence of peripheral blast cells quickly identifies leukemia as the likely diagnosis. Infiltrative disease of the liver, as well as tumor lysis syndrome (less common in neuroblastoma) could be seen as abnormalities in serum chemistry results. Serum LDH, neuron-specific enolase, and ferritin values may be elevated because these substances are secreted by some neuroblastoma tumor cells in active disease.? However, they are neither particularly sensitive nor specific to neuroblastoma because LDH and ferritin are also acute-phase reactants, and neuron-specific enolase concentrations also are elevated in other tumors such as Wilms tumor, Ewing sarcoma, and non-Hodgkin lymphoma.? Assessment of urinary catecholamine metabolite concentrations is a specific test when neuroblastoma is being considered as a diagnosis. Approximately 90% of neuroblastoma tumors secrete the metabolic byproducts of dihydroxyphenylalanine, dopamine, norepinephrine, and epinephrine. Homovanillic acid and vanillylmandelic acid are the specific catecholamine urinary metabolites that strongly suggest the diagnosis of neuroblastoma when their values are 3 standard deviations above the mean for age. Diagnostic sensitivity can be enhanced with collection of a 24-hour specimen, although this involves a time delay disadvantage compared with a random untimed sample. Some studies suggest that monitoring values may help predict prognosis and subsequent response to therapy.? The major disadvantage of this assessment is that results may not be available for several days to weeks because a specialty laboratory may be required to process the sample.Histopathologic analysis of either a tumor biopsy specimen or a bone marrow sample from metastatic disease obviously is required to make the diagnosis of neuroblastoma.American Board of Pediatrics Content Specification(s):Know the appropriate ancillary studies relevant to neuroblastomaQuestion 8A 9-year-old boy presents to the emergency department with a limp and left hip pain of several days’ duration. There is no history of acute trauma. He has sickle cell disease but has not had major complications. On physical examination, the afebrile boy has normal vital signs, except for a heart rate of 98 beats/min. He complains of left hip pain with external and internal rotation, but he can bear weight. No other findings are abnormal. Of the following, the MOST appropriate next step to evaluate this boy’s condition isA.bone plete blood count and blood puted tomography scan of both hipsD.plain radiographs of the hipsE.serum antistreptolysin O titers Correct answer DThe boy described in the vignette has subacute onset of limp and hip pain. His history of sickle cell disease and lack of constitutional symptoms raises the diagnostic possibility of avascular necrosis (AVN) of the femoral head. In the emergency department, this condition is diagnosed best with plain radiographs of the hips.? AVN of the bone usually is caused by repeated episodes of reversible ischemia, which most commonly involve the femoral head. This?condition often is referred to with the eponym of Legg-Calvé-Perthes disease. Among the many predisposing conditions that increase the likelihood of these occurrences are Gaucher disease and various hemoglobinopathies, especially sickle cell disease.? Children who have AVN of the femoral head typically are between 4 and 9 years of age, with a male predominance. They usually present with a history of insidious onset of hip pain, although acute trauma sometimes can precipitate the emergency department visit. As with many conditions affecting the hip, the pain and discomfort can be referred to the knee and inner thigh. It can be useful to contrast the presentation of this condition with slipped capital femoral epiphysis (SCFE), another common hip condition in children. Although both conditions have a male predominance, SCFE is seen more commonly in older children (during onset of puberty) and often is associated with obesity.??Acute trauma also can lead to AVN of affected bones through disruption of blood supply, such as fracture through the scaphoid bone. This condition occurs mostly in older adolescents and adults. Prompt recognition, appropriate immobilization, and timely referral to orthopedists are keys to prevent AVN in scaphoid fractures. Complications of AVN include chronic pain, arthritis, and long-term disability. Early recognition and appropriate management can minimize all of these complications.Other conditions to consider for the child in the vignette include osteomyelitis (for which a bone scan and complete blood count and blood culture are appropriate), septic arthritis (for which a complete blood count and blood culture and ultrasonography of the hips are appropriate), and postinfectious arthritis (for which serum antistreptolysin O titers are appropriate). However, the lack of fever and other constitutional symptoms reported for this boy make these diagnoses less likely. Computed tomography is not the optimal first step in radiographic evaluation of this child’s disorder.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of avascular necrosisRecognize the signs and symptoms and complications of avascular necrosisKnow the indications for and interpret results of ancillary studies in patients with avascular necrosisAPRIL 2010Question 1A 16-month-old boy who has a history of developmental delay is brought to the emergency department after having generalized seizures. To help determine the cause of the seizures, you ask the parents about the extent and character of the boy’s developmental delay. They explain that their pediatrician diagnosed developmental delay according to the Denver screening tool. Of the following, the result of the Denver screening tool is MOST likely to reveal a delay inA.cognitionB.fine motor skillsC.gross motor skillsD.speechE.unknown until he receives more evaluationCorrect answer: EThe Denver Developmental Screen system revised (1992), commonly referred to as Denver II, is the most common developmental screening test used by pediatricians. It is administered to children 2 weeks to 6 years of age and consists of 125 questions in four broad categories: Personal-socialFine motor – adaptiveLanguageGross motorThe answers are based on parental report and direct observations by the test administrator. A composite score is obtained, but no individual score for each of the four areas is reported. Despite being marketed as a screening tool, the Denver II is believed to be more specific but not sensitive for moderate-to-severe developmental delay. Because the instrument is not intended for use as a diagnostic tool for delays in specific areas, including cognitive, fine motor skills, gross motor skills, and speech, it should be used solely to identify potential risks for developmental delay and to guide further characterization of the delay.??American Board of Pediatrics Content Specification(s):?Know the Denver Developmental Screening systemQuestion 2While you are evaluating a 12-year-old girl for an asthma exacerbation, you note that her blood pressure reading is 140/95 mm Hg. You obtained the reading after administering two albuterol nebulizer treatments and a dose of prednisone to the girl. When she is ready for discharge, you repeat the blood pressure measurement in the right arm, as well as the other extremities, and note similar systolic and diastolic values. The remainder of the findings on her physical examination are normal. The electronic medical record for recent emergency department visits shows that the girl’s blood pressure has been in a similar range on one previous visit for an asthma exacerbation 6 months ago.Of the following, the MOST important next step in the evaluation of the girl’s blood pressure isA.ambulatory blood pressure monitoring as an outpatient to rule out “white coat hypertension,” with a follow-up appointment with a cardiologistB.baseline metabolic profile and urinalysis to assess for underlying renal disease, with a follow-up appointment with her primary pediatricianC.counseling the girl’s parent on the possibility of hypertension and referring the girl to her primary pediatrician for repeated blood pressure measurementsD.electrocardiography to evaluate for evidence of left ventricular hypertrophy, with a follow-up appointment with a cardiologist if the reading is abnormalE.outpatient renal ultrasonography and echocardiography, with a follow-up appointment with her primary pediatricianCorrect answer: CThe blood pressure measured for the girl described in the vignette clearly is in the hypertensive range for a child of any age, height, and sex. However, further evaluation is needed to determine whether the blood pressure is persistently elevated. Hypertension can be diagnosed when the child’s blood pressure exceeds the 95th percentile for age, sex, and height on three or more separate occasions 1 week apart. Accordingly, the child’s parent should be counseled about the possibility of hypertension and directed to have her primary pediatrician measure her blood pressure at least twice more. If repeated measures confirm that her blood pressure is greater than the 95th percentile, ambulatory blood pressure monitoring (to rule out “white coat hypertension”) or further diagnostic evaluation is indicated. Such testing, including metabolic profile and urinalysis, electrocardiography, ultrasonography, and echocardiography, are premature for the girl in the vignette at this time. Prehypertension is diagnosed when the blood pressure is in the 90th to 95th percentile for age, height, and sex. Normative tables of these parameters are available in the Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents. A simplified version of the tables with a single cut-off for a child’s age and sex was published in Pediatrics in 2009 to facilitate recognition of potential hypertension in cases where clinicians do not have access to height or body mass index (BMI).Hypertension can be categorized into primary (essential) or secondary. One proposed algorithm for diagnostic evaluation of pediatric hypertension uses the mnemonic MONSTER: Medications, Obesity, Neonatal history, Signs or symptoms, Trends in the family, Endocrine or Renal causes (Figure).The younger the patient, the more likely the hypertension is due to an underlying abnormality. Common causes of hypertension in the newborn include renal artery thrombosis, renal artery stenosis, congenital renal anomalies, and coarctation of the aorta. Among infants and preschool-age children, coarctation and renal artery stenosis remain important causes, and renal parenchymal disease becomes increasingly common as a cause. The leading cause in 6- to 10-year-olds is essential hypertension (which includes obesity), although renal parenchymal disease and renal artery stenosis still account for a significant number of cases. Elevated intracranial pressure can be a cause of hypertension at any age. When hypertension is accompanied by relative bradycardia and altered mental status, occult head trauma should be considered.Although it is a diagnosis of exclusion, essential hypertension is the primary cause of hypertension through adolescence. Medication-induced hypertension is a possibility in any age group; oral contraceptive pills are responsible for hypertension in many teenage girls. Obesity is increasingly recognized as a cause of hypertension in children. Children whose BMIs are greater than the 95th percentile for age are three to five times more likely to have hypertension than their counterparts who have normal BMIs. Obstructive sleep apnea also is being recognized as a more prevalent contributor to hypertension.The diagnostic evaluation should be guided by a careful history and physical examination to avoid unnecessary testing.? In the absence of severely elevated blood pressure, such evaluation should be undertaken on an outpatient basis. If renal disease is suspected, urinalysis provides evidence of glomerular (protein or blood) as well as tubular dysfunction (pH, specific gravity, glucose). The blood urea nitrogen and creatinine of the basic metabolic profile provide an evaluation of glomerular function. Renal ultrasonography with color Doppler provides an indication of kidney size, location, and echogenicity. Cardiac evaluation involving electrocardiography and echocardiography may be indicated in the presence of a murmur or symptoms of obstructive sleep apnea and always is indicated when signs of cardiac failure are evident on physical examination. More unusual but serious causes of hypertension include a variety of endocrine disorders and pheochromocytoma. A careful evaluation of all the medications the child is taking (prescription, over-the-counter, and illicit) always is indicated.The decision to treat an elevated blood pressure in the emergency department depends on the degree of elevation and the presence or absence of associated symptoms. A hypertensive urgency is defined as a severely elevated blood pressure of greater than the 99th percentile for age, sex, and height that may be harmful but is not accompanied by any evidence of end-organ damage or dysfunction. A hypertensive emergency is a severely elevated blood pressure associated with evidence of secondary organ damage, such as hypertensive encephalopathy, left ventricular failure, or seizure. In the absence of symptoms, the decision to initiate treatment for severely elevated blood pressure is controversial.Generally, mean arterial pressure should be reduced by no more than 25% in the first 2 hours, with the ultimate goal of normalizing the blood pressure over a period of several days. The choice of drug to use in the emergency department treatment of a child who is experiencing a hypertensive crisis depends on the degree of hypertension present, current medication use, known underlying medical conditions, suspected cause of the hypertension, and organs involved. Titratable intravenous medications are recommended for symptomatic patients, beginning at the lowest recommended dosage and titrating up to effect. The Fourth Report on the Diagnosis, Evaluation, and Treatment of High blood Pressure in Children lists esmolol (a beta blocker), labetalol (alpha and beta blocker), nicardipine (calcium channel blocker), and sodium nitroprusside (vasodilator) as the most useful drugs for immediate reduction in blood pressure.Sublingual and oral short-acting nifedipine no longer are recommended routinely for acute reductions in blood pressure because their effects are variable and their use has been associated with an increase in adverse effects in both adults and children.American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of hypertensionPlan the diagnostic evaluation and initial intervention for patients with hypertensionQuestion 3An 18-month-old girl is brought to the emergency department by her mother because of fever and upper respiratory tract symptoms.? She recently was diagnosed with acute otitis media. The pediatrician, in concert with the family, elected to adopt a watchful waiting approach and did not prescribe antibiotics for her treatment. She has remained febrile and has been increasingly irritable. On physical examination, you note displacement of the child’s left ear (Fig. 1).Figure 1: Courtesy of M. Rimsza. Reprinted with permission from PREP Self-Assessment 2003.Of the following, the MOST likely complication of this child’s condition isA.bacteremiaB.facial nerve palsyC.meningitisD.otitis externaE.permanent hearing lossCorrect answer: CMastoiditis is a suppurative complication of acute otitis media that primarily affects children younger than 2 years of age. The incidence ranges from 2 to 4 cases per 100,000 children in the United States. Clinically, it manifests as subperiosteal abscess, characterized as swelling over the mastoid process resulting in lateral and inferior displacement of the pinna (Fig. 2), as described for the child in the vignette.Figure 2: Posterior auricular swelling and forward protrusion of the pinna are signs of mastoiditis. (Courtesy of The Media Lab at Dorenbecher). Reprinted with permission from PREP Self-Assessment 2000.Children who have uncomplicated mastoiditis usually appear nontoxic. Serious complications include sinus vein thrombosis, abducens nerve palsy, brain abscess, and meningitis. Specifically, meningitis is caused by direct extension of the underlying infection, leading to irritability,?variable signs of meningeal irritation, and persistent fever. The bacterial pathogens of mastoiditis usually are the same as those that cause acute otitis media. The most common organism is pneumococcus. Widespread immunization against pneumococcus has resulted in an overall decline in invasive pneumococcal disease, although nonvaccine serotypes have emerged as important causes of disease. Other pathogens include Haemophilus influenzae, Staphylococcus aureus, and Pseudomonas aeruginosa. S aureus tends to be a more common cause in children younger than 2 years of age, whereas P aeruginosa is more common in older children, especially those who have recurrent otitis puted tomography (CT) scan often is used to evaluate the extent of the disease and assess for other complications. Radiologic findings typically include opacification of the mastoid air cells with other attendant signs of inflammation that are seen better with intravenous contrast enhancement. The incidental finding of mastoid cell opacification on cranial CT scan in the absence of clinical signs or symptoms of mastoiditis is nonspecific. CT scan alone should not be used to diagnose the condition. It is reasonable to presume that withholding antibiotics in children who have acute otitis media may lead to an increased incidence of mastoiditis. In fact, recent evidence supports this contention. In a retrospective study in Great Britain, the risk for mastoiditis after otitis media was halved by the use of antibiotics to treat the original infection. Given the low frequency of the complication, however, 5,000 cases of acute otitis media would need to be treated to prevent 1 case of mastoiditis.? Therefore, if a watch-and-wait management approach is used for otitis media, follow-up should be assured.As noted earlier, the signs and symptoms of mastoiditis are related to the duration of infection, extent of abscess formation, and degree of complications. In general, children younger than 2 years of age who have mastoiditis present with systemic symptoms, including irritability, anorexia, high fever, upper respiratory tract illness, and displacement of the pinna on the affected side.? Older children who have mastoiditis typically have a history of previous otologic problems, including placement of myringotomy tubes. Other symptoms in older children include earache and purulent discharge.Consultation with an otolaryngologist is recommended when planning the treatment course for mastoiditis. Uncomplicated infection usually responds to treatment with parenteral antibiotics alone. Treatment should be directed against the most common pathogens. Some reasonable initial antibiotic choices include ampicillin-sulbactam or the combination of ceftriaxone and clindamycin. ?If no clinical improvement is apparent within 48 hours, surgical management, including mastoidectomy, should be considered. Postoperative cultures should direct modification of antibiotic therapy. Mastoiditis represents local extension of infection and, therefore, rarely involves bacteremia. Deafness and facial nerve palsy are rare complications of acute otitis media and not specifically of mastoiditis. Otitis externa is a pathologically distinct condition from otitis media. It manifests as infection of the external ear canal and exquisite tenderness over the tragus but no displacement of the pinna.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of mastoiditis by ageRecognize signs and symptoms and complications of mastoiditisPlan the management of mastoiditisKnow the role of ancillary studies in mastoiditisQuestion 4An 11-month–old girl who has an indwelling catheter for maintenance chemotherapy of acute lymphocytic leukemia is brought to the emergency department because of tactile fever at home. She has had symptoms of a mild upper respiratory tract infection for 1 day. On physical examination, she appears well, her heart rate is 152 beats/min, respiratory rate is 26 breaths/min, and blood pressure is 78/56 mm Hg.Of the following, the MOST safe and accurate method of estimating her body core temperature is to use a(n)A.axillary thermometerB.oral thermometerC.rectal thermometerD.temporal artery thermometerE.tympanic thermometerCorrect answer: ETwenty percent of pediatric visits to emergency departments involve a complaint of fever. In certain populations, such as neonates and people who have neutropenia or other causes of immunodeficiency, accurate temperature measurement is especially important because it dictates management. The current therapy for leukemia and indwelling vascular device reported for the girl in the vignette put her at increased risk for bacterial sepsis. A documented fever should prompt the clinician to obtain urine and blood cultures and institute broad-spectrum antibiotics. It is important that the mode of temperature determination be both safe and accurate.Parental reports of a tactile fever have been associated with a wide range of accuracies in predicting the presence of a measured fever. Mothers have been shown to have an accuracy of as high as 80%. However, documentation of fever is the standard of care in medical settings.Although rectal thermometry has been used most often in medical settings, it is not the modality that is most reflective of core temperature. When compared with temperatures measured by thermistors in indwelling pulmonary artery catheters or other indwelling devices, changes in rectal temperature lag significantly behind both rising and falling core temperatures. In addition, rectal temperatures vary, depending on the presence and amount of stool in the rectum, presence of diarrhea, depth of thermometer insertion, and local blood flow. The risk of inducing bacteremia in patients who have neutropenia or other immunocompromising conditions is a contraindication to the use of a rectal thermometer in this patient. Infrared tympanic thermometers most accurately reflect core temperature. Several studies have shown them to reflect pulmonary artery temperatures even when body temperature is changing rapidly. The probe senses infrared heat rays emitted by the tympanic membrane. The measurement is not affected by environmental temperature, is fast and easy to perform, and is not associated with any risk of transmitting or inducing infectious diseases. Some studies have shown them to be inaccurate in younger patients, possibly due to the smaller auditory canals of infants and toddlers and operator error. However, several studies and some systematic reviews have shown good agreement between tympanic thermometer readings and indwelling devices (pulmonary artery, esophageal, and bladder thermistors). They seem to be a good choice in children older than 3 years of age and in children younger than 3 years in whom taking a rectal temperature is contraindicated, such as the girl in the vignette.Measuring temperatures by the axillary method is safe and easy and does not produce patient discomfort. However, the results are markedly affected by the temperature of the ambient environment and take a long time to obtain, making them impractical in a busy emergency department. Most importantly, they are notoriously inaccurate, with a reported sensitivity for detecting fevers of approximately 30%. However, they continue to be used in some settings, such as for immunocompromised patients in emergency departments. If such a method is used, the thermometer must be in place for a prolonged time.Oral thermometers require patient cooperation, which is not possible in infants and toddlers. Therefore, accuracy is lacking in this age group. This method is adequate in patients older than 5 years of age, but even in older children, hot or cold drinks, mouth breathing, and even tachypnea may lead to inaccurate measurements. Infrared temporal artery thermometers are relatively new devices. Their ease and rapidity of use are attractive characteristics for the busy emergency department. However, they are not sufficiently accurate or reliable in children to replace other modalities. In some studies, they show a minimal increase in accuracy over axillary thermometers. The time savings they offer over axillary thermometers may make them an attractive alternative in patients in whom rectal thermometers are contraindicated.American Board of Pediatrics Content Specification(s):Understand fever measurementQuestion 5A 12-year-old girl presents with a 1-week history of difficulty walking, 2-day history of urinary retention, and 24-hour history of visual impairment. She is otherwise healthy. She has had no recent upper respiratory tract infection, gastrointestinal illness, trauma, tick bite, or travel. She reports no fever, headache, emesis, or loss of consciousness. On physical examination, she is afebrile, lucid, and has no meningeal signs. Cranial nerve examination yields normal results, except for diminished visual acuity in the right eye and no blink response to threat from lateral fields. The right pupil is 3 mm, central, and sluggishly reacting to light. Results of funduscopy are shown in the Figure.FigureThe left pupil is 2 mm and briskly reactive, and the left disc appears normal. She is unable to walk unsupported and has decreased motor strength proximally in both lower extremities. Sensation to light touch is decreased below the level of the umbilicus. Perianal sensation also is diminished. Deep tendon reflexes are suppressed in the lower extremities but normal in the upper extremities.Of the following, the test that is MOST likely to confirm the diagnosis isA.cerebrospinal fluid puted tomography scan of brain and spinal cordC.magnetic resonance imaging of brain and spinal cordD.methanol measurementE.serum ceruloplasmin assessmentCorrect answer: CThe acute onset of paraplegia with sensory and sphincter impairment described for the girl in the vignette suggest spinal cord involvement (transverse myelitis). The rapid loss of peripheral vision, afferent pupillary defect, and blurring of the optic disc margins on funduscopy suggest optic neuritis (ON). This constellation of neurologic findings at multiple anatomic levels is most consistent with a demyelinating process. The child described in the vignette has neuromyelitis optica (Devic disease), a variant of multiple sclerosis (MS). Magnetic resonance imaging (MRI) is the study of choice for detecting demyelination; computed tomography scan does not have the capability to detect the demyelinating process. ON is an inflammation or demyelinization of the optic nerve that results in impaired visual acuity. Pain on palpation or movement of the??globe may be noted on examination. There is a relative afferent pupillary defect, decreased visual acuity, and a normal peripheral retina and macula. When the inflammation involves the opthalmoscopically visible portion of the nerve, it is referred to as papillitis (intraocular ON), in contrast to retrobulbar ON. ON may occur as an isolated condition or as a manifestation of neurologic or systemic disease. More specifically, as in this case, it may be a harbinger of a demyelinating disease such as an MS variant. The diagnosis is confirmed with MRI that reveals at least one area of demyelination. Treatment with high-dose intravenous steroids has been shown to hasten the visual recovery and may prevent progression of MS in young adults at risk. Other causes of ON include idiopathic; viral infections (eg, Epstein-Barr virus, encephalitis, measles, mumps, varicella); granulomatous inflammation (eg, tuberculosis, cryptococcosis, sarcoidosis, syphilis); lupus; contiguous inflammation or infection of meninges, orbit, or sinuses; toxicity (eg, methanol, heavy metals, ethambutol, chloroquine, vincristine, isoniazid); nutritional problems (malnutrition, vitamin B12 deficiency); or postinfectious, as in acute disseminated encephalomyelitis (ADEM).Differentiating a first episode of MS from ADEM can be challenging without further follow-up to assess recurrence or time course. In general, ADEM is a monophasic illness that usually afflicts children younger than 10 years of age. Patients may be febrile and have altered mentation, vomiting, seizures, ataxia, weakness, and bilateral ON. There is usually no family history of MS. The cerebrospinal fluid (CSF) examination frequently shows sterile pleocytosis, and cranial MRI typically reveals multiple enhancing, disseminated, multifocal lesions in the basal ganglia, thalamus, brainstem, and at the cortical gray white junction, consistent with inflammation or demyelination. In childhood MS, MRI shows T2-enhancing isolated lesions in the corpus callosum and periventricular white matter. The CSF in MS often contains oligoclonal bands. In the absence of meningeal signs or a suspicion of ADEM, CSF analysis may serve as an adjunct in the evaluation of the girl in the vignette but would not be helpful in confirming the diagnosis. Methanol toxicity is associated with ON, but patients initially present with inebriation and gastritis. After a latency period of up to 30 hours, severe anion gap metabolic acidosis, seizures, and coma usually accompany the visual loss. The duration of symptoms and clinical findings in the vignette are not consistent with acute methanol ingestion. Serum ceruloplasmin would aid in the diagnosis of Wilson disease. This child does not have signs and symptoms consistent with this diagnosis. The ocular finding of Wilson disease include the presence of a Kayser-Fleischer ring, apparent on external examination of the limbus. The neurologic findings are dominated by extrapyramidal symptoms of chronic duration.American Board of Pediatrics Content Specification(s):Plan the evaluation and management of acute optic neuritisRecognize the signs and symptoms of acute optic neuritisQuestion 6A wheelchair-bound 10-year-old boy who has a seizure disorder and severe developmental delay presents to the emergency department (ED) with respiratory distress. He had a prolonged seizure at school today that eventually stopped after the administration of diazepam rectal gel by the staff.? He vomited immediately after the seizure stopped and began having difficulty breathing about 45 minutes later, just prior to his arrival in the ED. On physical examination, he has a temperature of 37.9°C, heart rate of 110 beats/min, respiratory rate of 32 breaths/min, and blood pressure of 100/60 mm Hg. Pulse oximetry shows a saturation of 90% while breathing room air. He is nonverbal (baseline), sitting up in his chair, and exhibiting mild nasal flaring and retractions. His eyes are open, and his mother states that he is back to baseline, except for the respiratory symptoms. Auscultation of his lungs reveals coarse breath sounds bilaterally with scattered diffuse crackles. You obtain a chest radiograph and administer supplemental oxygen.Of the following, the MOST appropriate treatment for this child at this time isA.albuterol nebulizer treatmentsB.antibiotic administrationC.corticosteroid administrationD.early noninvasive ventilationE.oropharyngeal suctioning as neededCorrect answer: EThe abrupt onset of respiratory symptoms after vomiting described for the neurologically compromised boy in the vignette suggests aspiration pneumonia. Little evidence is available to guide physicians in the acute management of this condition; supportive therapy is the treatment of choice. Airway protection is required in the presence of an ongoing risk of aspiration (continued depressed neurologic status) or impending respiratory failure, neither of which is present at this time for this patient. Suctioning the oropharynx to clear secretions or particulate matter is indicated. Corticosteroids have not been proven to be beneficial early in the course of disease, and their role in the subsequent development of pneumonia is unclear. Although approximately 50% of patients eventually develop bacterial pneumonia, presumptive use of antibiotics also has not been proven to alter the course of disease. Noninvasive ventilation is a poor choice for this boy because it is likely to distend his stomach, and with his baseline neurologic function, repeat aspiration may occur. Beta-2 agonists such as albuterol do not have a role in the treatment of aspiration pneumonia unless clinical signs of bronchoconstriction or wheezing are present.Aspiration pneumonia is characterized by inflammation of the lung tissue following insult caused by inhalation of oropharyngeal or stomach?contents. It is most common in debilitated or institutionalized children who have central nervous system disorders that impair swallowing or protective airway reflexes. It may occur in children who have disorders of esophageal dysmotility, such as tracheoesophageal fistula, or with disorders of delayed gastric emptying. Healthy children who have undergone deep sedation with full stomachs also are at risk for aspiration pneumonia.The underlying lung damage is somewhat dependent on the nature of the aspirated material. “Acidic” aspirations that have a pH of less than 2.5 cause direct injury to the alveolar-capillary membranes, resulting in a hemorrhagic, granulocytic, necrotizing reaction. Hypoxia occurs within minutes and is caused by reflex airway closure, destruction of surfactant with resultant atelectasis, interstitial and alveolar edema, hemorrhage, and consolidation. Severe chemical pneumonitis results. Nonacid or mixed aspirates have a wider range of possible outcomes. Clear liquids, such as saliva and small amounts of water, usually do not cause severe damage and may result in transient symptoms with rapid resolution.? Particulate matter, such as partially digested food, may cause a more prolonged pathologic response. The role of infection is unclear, but it probably is not involved in the initial pulmonary complications. However, the injured lung is potentially vulnerable to bacterial infection, especially in the presence of acid aspiration.? In large acidic aspiration events, aerobic organisms predominate; Staphylococcus aureus and Pseudomonas are common pathogens. Among nonhospitalized patients, anaerobic organisms predominate; facultative anaerobes and aerobic organisms are found in hospitalized patients. A patient who aspirates a heavily infected inoculum, such as a child who has small bowel obstruction, is at higher risk of developing a localized necrotizing bacterial pneumonia and may develop an abscess or empyema. Additional life-threatening complications of aspiration pneumonia are respiratory failure and acute respiratory distress syndrome. Aggressive pulmonary toilet and intubation to prevent further aspiration and facilitate suctioning is recommended in severe cases. Antibiotics often are added to therapy when symptoms continue to progress and a secondary bacterial pneumonia is suspected.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of aspiration pneumoniaRecognize signs and symptoms and life-threatening complications of aspiration pneumoniaPlan the management of acute aspiration pneumoniaQuestion 7You are evaluating a 4-year-old boy who has sickle cell disease and asthma. He recently was hospitalized for an acute asthma exacerbation and acute otitis media. He was discharged 5 days ago with albuterol inhalations, prednisone, and high-dose amoxicillin. Today he presents with fever and dyspnea. On physical examination, the ill-appearing boy is in severe respiratory distress and has a temperature of 40.0°C, heart rate of 160 beats/min, respiratory rate of 40 breaths/min, blood pressure of 95/35 mm Hg, and pulse oximetry of 90% on room air. He is lucid and has good pulses. Breath sounds are diminished on the right, with dullness to percussion and egophony. The remainder of his examination findings are normal. You obtain radiography (Fig. 1).Figure 1: Chest radiographOf the following, the MOST appropriate next step isA.administration of intravenous furosemideB.aggressive chest physiotherapyC.bronchoscopyD.ultrasonographically guided thoracentesisE.video-assisted thoracic surgery Correct answer DThe chest radiograph obtained for the boy described in the vignette reveals near-total opacification of the right hemithorax, with mediastinal shift to the left. Such findings are most consistent with a large pleural effusion. The child’s clinical presentation, including the high temperature, toxic appearance, and severe respiratory distress, plus his comorbid conditions of sickle cell disease and asthma??suggest that he has a right-sided pneumonia with a parapneumonic effusion. Management at this point should focus on relieving his respiratory distress via diagnostic and therapeutic thoracentesis. Pleural effusion can be confirmed by decubitus plain radiography that may cause the fluid to layer in the dependent chest (Fig. 2) or with the aid of ultrasonography.Figure 2: Pleural effusion on right lateral decubitus film. Reprinted with permission from Braner DA, Zenel JA, Goldstein B, Silverberg P, eds. Pediatrics: An Interactive Program. Philadelphia, Pa: WB Saunders Co; 1999.Thoracic ultrasonography also provides information about the consistency of the fluid, localizes the area of maximal collection, and guides thoracentesis. Drainage via thoracentesis or chest tube is necessary when pleural fluid compromises respirations or empyema is suspected, as in this case. Examination of the pleural fluid can distinguish empyema from a sympathetic effusion. Pleural fluid abnormalities in various conditions are shown in the Table.Table: Typical Characteristics of Pleural Fluid AbnormalitiesConditionLeukocytes (Type)pHGlucoseLactate Dehydro-genaseFluid CharacteristicsEmpyema>5x106/L (>5,000/mm3); predominantly polymorphonuclear leukocytes (PMNs)<7.3>3.33 mmol/L (>60 mg/dL)ElevatedThick, purulentParapneumonic effusion<1x106/L (<1,000/mm3); predominantly PMNs>7.3>3.33 mmol/L (>60 mg/dL)NormalNonpurulentTuberculous effusion1 to 2x106/L (1,000 to 2,000/mm3); initially followed by mononuclear leukocyte predominance<7.31.11 to 2.22 mmol/L (20 to 40 mg/dL)ElevatedAcid-fast smear almost never positiveChylothorax>1x106/L (>1,000/mm3); predominantly lymphocytes>7.3>3.33 mmol/L (>60 mg/dL)NormalMilky with an elevated triglyceride levelMalignant effusion1x106/L (>1,000/mm3)<7.3<3.33 mmol/L (<60 mg/dL)ElevatedAbnormal cells on cytologyParapneumonic effusions are a known and expected complication of lower respiratory tract infections. Streptococcus pneumoniae is the most common cause of febrile pneumonia in children and the most commonly implicated pathogen for pleural effusions. Specific pathogens that deserve consideration in this case are penicillin-resistant pneumococci as well as methicillin-sensitive and -resistant Staphylococcus aureus (MRSA). MRSA can be community-acquired or a nosocomial infection acquired during the child’s recent hospitalization. Haemophilus influenzae infections have become rare following the introduction of the H influenzae type b conjugate vaccine. Other agents causing focal pneumonia with sympathetic effusion are Mycoplasma pneumoniae, Chlamydia pneumoniae, Mycobacterium tuberculosis, and fungi. Approximately 20% of older children who are infected with Mycoplasma can have parapneumonic effusions. The clinical picture usually includes low-grade fever and patchy or streaky infiltrates. Mycobacterium infections are less common but usually present as a subacute illness, with fever, night sweats, cough, or weight loss.Empyema is a parapneumonic effusion caused by spread of the inflammatory process to the visceral pleura and pleural space. Early in the process, there is a sympathetic leak of leukocytes, fluid, and protein from the lung parenchyma into the pleural space. Subsequently, microorganisms invade, leukocytes migrate to contain the infection, and a variety of inflammatory mediators are released.Chest physiotherapy is indicated as an adjunct in the management of atelectasis. Significant right-sided atelectasis causes a mediastinal shift to the same side, with volume loss on chest radiography. Video-assisted thoracic surgery (VATS) generally is indicated for proven empyema that has a dense layer of exudates; walled-off cavities containing infected fluid; or persistent symptoms of fever, respiratory distress, or chest pain. Recent data from a small study of 18 pediatric patients suggest that VATS may be superior to primary thoracostomy drainage for evacuation of parapneumonic effusion, with shorter hospital length of stay and fewer subsequent interventional procedures. However, because of the severe respiratory stress with hypoxemia experienced by the child in the vignette, immediate removal of fluid is indicated for diagnostic and therapeutic purposes.? Bronchoscopy is useful to obtain secretions for culture in a child who has chronic lung disease, is immunocompromised, or is critically ill. Bronchoscopy is not the procedure of choice for pneumonia unless other signs or symptoms suggest that the patient is critically ill. Intravenous furosemide typically is reserved for pulmonary edema in the setting of congestive heart failure.American Board of Pediatrics Content Specification(s):Recognize the signs and symptoms and complications of pleural effusion (including acute cardiorespiratory failure with large effusions)Know the indications for and interpret results of ancillary studies in patients with pleural effusionsQuestion 8A 16-year-old boy comes to the emergency department after being hit in the face with a line drive at a baseball game. He did not lose consciousness, but he experienced moderate nasal bleeding that is controlled upon arrival. On physical examination, the boy is lucid and has normal vital signs. He has right periorbital swelling with infraorbital echymosis and tenderness. His pupils are equally reactive to light, and extraocular movements are intact. Sensation over the right cheek is normal. There is tenderness to palpation over the nasal bridge and nasal tip and dried blood in the right external naris. You examine the nasal passages more closely (Figure).Figure: Reprinted with permission from Ginsburg CM. Pediatr Rev. 1998;19:142-143Of the following, the MOST appropriate next step is toA.instill two drops/min 0.25% phenylephrine in each nostrilB.obtain plain radiographsC.place an anterior nasal packD.recommend a follow-up appointment with the primary care physicianE.request otolaryngology consultation Correct answer EThe boy described in the vignette has sustained blunt facial trauma with bilateral nasal septal hematomas and the possibility of an underlying maxillofacial fracture(s). A septal hematoma is an uncommon yet serious complication of nasal trauma. Failure to recognize this entity can result in avascular necrosis of the cartilage, formation of a septal abscess, and risk of permanent nasal deformity. Because no alternative blood supply is available, immediate drainage of the hematoma to restore blood flow from the perichondrium is warranted. Such drainage is accomplished best by prompt evacuation with otolaryngology consultation.??Septal hematomas are believed to occur following rupture of the perichondrial blood vessels and extravasation of blood that strips the?perichondrium from the cartilage, causing a closed-space accumulation. If the cartilage is fractured, blood may accumulate on both sides of the septum. Diagnosis is suggested by severe localized pain, tenderness to palpation of the nasal tip, and a cherrylike swelling of the nasal mucosa. The mass fluctuates when probed and does not change in size with vasoconstrictor application.??Surgical drainage of a septal hematoma involves the following steps:After topical anesthesia, a #11 blade is used to make an L-shaped incision in the most inferior and dependent portion of the hematoma.Using forceps or a small hemostat, the opening is expanded to allow drainage.A rubber drain is placed, followed by an anterior nasal pack for 24 to 48 hours.Antibiotics usually are prescribed to prevent secondary bacterial infection, although clinical evidence to support this practice is lacking.??Computed tomography (CT) scan to rule out maxillofacial fractures involving the nasoorbitoethmoid (NOE) complex may be indicated after the septal hematoma has been evacuated. A common injury pattern associated with the signs and symptoms described for the boy in the vignette is a “blow-out” fracture of the orbit, which involves a fracture of the orbit floor, resulting in marked infraorbital swelling and tenderness. Entrapment of the inferior rectus muscle or injury to the infraorbital nerve is a complication of this fracture. Clinical examination may reveal impairment of upward gaze or hypoesthesia over the cheek, both of which were not present for this boy. CT scan can confirm the diagnosis and the presence of muscle entrapment, globe injury, or intraorbital hematoma. Another important injury to the NOE complex is a fracture of the cribriform plate, which forms the medial part of the roof of the nasal cavity. This fracture may result in a cerebrospinal fluid leak (presenting as clear rhinorrhea) and an increased risk of meningitis or brain abscess.??Plain radiographs are neither sensitive nor specific for evaluation of facial fractures. Therapeutic instillation of vasoconstrictor nasal drops in the absence of ongoing epistaxis is not helpful. When the diagnosis of septal hematoma is in doubt, such nasal drops can be used as an adjunct in the evaluation. Outpatient referral to the primary care physician is not appropriate in the face of a nasal surgical emergency. Nasal packing should be used for uncontrolled hemorrhage, not septal hematoma.??American Board of Pediatrics Content Specification(s):??Recognize urgent complications and describe management of facial, orbital, and nasal fractures (eg, retro?orbital hematoma, cribriform plate fractures, and septal hematoma)May 2010Question 1A 14-year-old girl who has previously diagnosed type 2 diabetes mellitus presents to the emergency department after trying to manage hyperglycemia at home over the past several days. She has had polyuria but very little ketonuria. On physical examination, her temperature is 37.0°C, respiratory rate is 22 breaths/min, heart rate is 160 beats/min, and blood pressure is 92/66 mm Hg. She is slowly responsive to questions but confused. Her initial blood glucose measurement in the emergency department reads 850 mg/dL (47.2 mmol/L). Venous blood gas results show a pH of 7.3.Of the following, the MOST appropriate next step is to administerA.10% dextrose and water solutionB.glucagonC.naloxoneD.0.9% saline solutionE.sodium bicarbonate Correct answer DThe patient described in the vignette is exhibiting signs and symptoms of hyperosmolar hyperglycemic nonketotic syndrome (HHNS or HHNKS). HHNS is a less common complication in the diabetic pediatric population because it presents more frequently in those who have type 2 diabetes mellitus, who are predominantly adults. However, like diabetic ketoacidosis (DKA), HHNS is a critical derangement in glucose metabolism that has been reported in type 1 diabetes mellitus as well. HHNS can present acutely in a similar fashion to DKA, despite some differences in pathophysiology and subsequent laboratory findings (Table).Table: Comparative Characteristics of Hyperosmolar Hyperglycemic Nonketotic Syndrome and Diabetic Ketoacidosis?Hyperosmolar Hyperglycemic Nonketotic SyndromeDiabetic KetoacidosisClinical FeatureTypical AssociationType 2 diabetes mellitusType 1 diabetes mellitusDegree of Dehydration15% to 20%5% to 10%Kussmaul RespirationsNot presentPresentMental StatusStupor/ComaAlert to Stupor/ComaLaboratory ValuesArterial pH>7.3<7.3Serum Glucose>600 mg/dL (33.3 mmol/L)>250 mg/dL (13.9 mmol/L)Urinary or Serum KetonesNone or smallPositiveOsmolality>330 mOsm/kg<320 mOsm/kgBlood Urea Nitrogen<25 mg/dL (8.9 mmol/L)>30 mg/dL (10.7 mmol/L)Anion GapVariable>10The relatively rare incidence of HHNS has contributed to a dearth of randomized, controlled pediatric studies to help provide evidence-based data for decision-making. Thus, recommendations for the initial treatment of HHNS are extrapolated from information based on adult HHNS cases, pediatric DKA studies, and expert panel consensus. The fundamental management priorities are repletion of the intravascular fluid deficit, replacement of ongoing fluid losses, and correction of hyperglycemia and electrolyte imbalances.Patients who have DKA tend to develop acidosis, Kussmaul breathing, and other symptoms that generally prompt them to seek medical attention sooner than those who have HHNS. Patients who have HHNS have a greater degree of hyperglycemia, hyperosmolarity, and dehydration (15% to 20% total body water deficit) without profound ketoacidosis. This requires careful but expeditious repletion with iso-osmolar, isotonic fluids to avoid the complications of cerebral edema as well as to preserve end-organ perfusion and restore hemodynamic stability. Although hyperglycemia decreases with the initial administration of intravenous fluids, insulin (preferably administered by continuous intravenous infusion) should be used to continue gradual lowering of the serum glucose concentration. The required insulin dose generally is lower than that administered to patients who have DKA. The extreme dehydration caused by a protracted osmotic diuresis along with the hyperglycemia can lead to significant metabolic derangements, particularly of sodium and potassium. Calculation of the corrected serum sodium concentration (serum sodium in mEq/L + [1.65 * (glucose in mg/dL – 100)/100]) is important in guiding the sodium content of continuing hydrating fluid. Total body potassium is depleted in those experiencing HHNS, primarily because of prolonged excessive diuresis due to their late presentation. The resulting volume depletion causes increased aldosterone concentrations, further contributing to renal potassium loss. Patients who have HHNS usually have normal serum potassium values on presentation.Naloxone is a reasonable treatment option in suspected narcotic overdose or as an initial step in an undifferentiated, unresponsive patient. Glucagon is indicated for a hypoglycemic episode in a known diabetic patient. Glucose-containing solutions generally are not recommended in HHNS or DKA until the serum glucose value has decreased to 250 to 300 mg/dL (13.9 to 16.7 mmol/L). Finally, sodium bicarbonate is not recommended in HHNS because affected patients do not present with the extreme acidosis from ketosis.American Board of Pediatrics Content Specification(s):Know the management of non-ketotic hyperglycemiaQuestion 2You are evaluating a previously healthy 14-year-old boy who presents to the emergency department complaining of recurrent, nonproductive, severe coughing spells with posttussive emesis for the past 2 weeks. The coughing spells are not related to position, exercise, or time of the day. He denies any history of fever, chills, difficulty in breathing, wheezing, upper respiratory tract infection symptoms, weight loss, exposure to animals/pets, and smoking. The only finding of note on physical examination of the well-appearing adolescent is the presence of few subconjunctival hemorrhages.Of the following, the MOST appropriate next steps in management are toA.administer a purified protein derivative test and start isoniazid therapyB.measure peak air flows and start nebulized albuterol therapyC.obtain a nasopharyngeal swab and start azithromycin therapyD.reassure the boy and start outpatient therapy with dextromethorphanE.start loratadine therapy and refer the boy to an allergist Correct answer CAlthough persistent coughing has many causes, the presence of paroxysms of cough associated with posttussive emesis and the absence of other findings described for the boy in the vignette strongly suggest pertussis. All patients in whom pertussis is suspected must have the diagnosis confirmed for epidemiologic purposes and be treated with appropriate antimicrobials to prevent spread to susceptible individuals.Isolation of Bordetella pertussis by culture is the gold standard for diagnosis. Specimens must be collected from the posterior pharynx using a Dacron? or calcium alginate swab and plated on Regan-Lowe chocolate agar or Stainer-Scholte media. Direct fluorescent antibody has been replaced by tests with better sensitivity and specificity. Polymerase chain reaction tests have shorter turnaround times than culture and high specificity and sensitivity, but they may not be universally available.Antibiotics should be administered to all patients in whom pertussis is suspected clinically, regardless of age, and are most useful when symptoms of cough are less than 4 weeks in duration. Although erythromycin has been the traditional antibiotic of choice, azithromycin and clarithromycin are preferred because of the shorter duration of therapy (3 to 7 days versus 14 days), negligible effects on the QT interval, and lesser gastrointestinal adverse effects. The Centers for Disease Control and Prevention (CDC) recommends a 5-day course (500 mg on day 1, followed by 250 mg once a day for 4 days) of azithromycin or a 7-day course of clarithromycin (500 mg twice a day). A 14-day course of trimethoprim-sulfamethoxazole is an alternative for patients who cannot tolerate macrolide antibiotics. Cough suppressants, bronchodilators, antihistamines, and steroids are not useful. To prevent spread of pertussis, the close contacts of the patient in the vignette (including health-care workers and caregivers) should receive full courses of macrolide antibiotics as prophylaxis regardless of age or vaccination status, and immunization with a single dose of combined tetanus and diphtheria toxoids and acellular pertussis (Tdap) vaccine when indicated.Because vaccine-induced and natural immunity wane with age and adolescents can serve as reservoirs, the Advisory Committee on Immunization Practices of the CDC and the American Academy of Pediatrics recommend a single booster dose of Tdap rather than tetanus and diphtheria toxoid (Td) between 11 and 18 years of age as a part of routine immunization.Pertussis (whooping cough or 100-day cough) is considered a disease of childhood, but adults and adolescents now account for two thirds of cases. Pertussis is a highly contagious respiratory illness, with attack rates as high as 80% to 100% in susceptible individuals. The causative pathogen is the gram-negative coccobacillus B pertussis. Sporadic cases of pertussis can be caused by B parapertussis or B bronchiseptica, especially in immunocompromised children. Classic pertussis is divided into three phases: catarrhal, paroxysmal, and convalescent. The catarrhal phase occurs after an incubation period of 1 to 3 weeks, lasts for 7 to 10 days, and is clinically indistinguishable from a viral upper respiratory tract infection. The subsequent paroxysmal phase lasts from 1 to 6 weeks and is characterized by bursts of numerous rapid coughs followed by an inspiratory gasp that is audible as a whooping sound. Paroxysms of cough become more frequent and may occur spontaneously or may be precipitated by external stimuli. The convalescent phase typically lasts for 2 to 3 weeks as the paroxysms slowly resolve. Such typical features do not occur in infants younger than 3 months of age or in adolescent or adult patients. The whooping sound is infrequent because the infant airway lacks the muscular strength to create sudden negative intrathoracic pressure. Infants are more likely to have cyanosis and apnea following paroxysms. Adolescents and adults are more likely to present with long-lasting paroxysmal cough as the only symptom (average duration of cough, 36 to 48 days) and typically do not demonstrate distinct phases of the illness. The CDC/World Health Organization case definition for clinical diagnosis of pertussis includes cough of at least 14 days’ duration and one of the following associated symptoms: paroxysms, whoop, or posttussive emesis.Clinical manifestations of B pertussis infection are primarily due to pertussis toxin and other toxic proteins, such as pertactin, filamentous hemagglutinin, and tracheal cytotoxin. Leukocytosis (15,000 to 100,000 cells/mm3) due to absolute lymphocytosis is characteristic of the catarrhal stage. The lymphocytes are small and have B- and T-cell origins, unlike the large atypical lymphocytes seen in viral infections. Neutrophil predominance suggests a bacterial coinfection. Chest radiograph may show streaky opacities arising from the hilar region, often described as an irregular (“shaggy”) right heart plications such as apnea, pneumonia, seizures, encephalopathy, and death are more common in infants. Complications due to severe coughing such as pneumothorax, epistaxis, and subconjunctival hemorrhages can occur at all ages.Although adenovirus, parainfluenza, respiratory syncytial virus, influenza A and B, and rhinovirus can cause illnesses with intractable coughing, they are associated with systemic signs and symptoms such as fever, sore throat, and conjunctivitis. The absence of prolonged fever, weight loss, and night sweats makes tuberculosis unlikely. Therefore, a purified protein derivative test and isoniazid therapy are not indicated. The child does not have a history or clinical signs of asthma, such as wheezing, that would require measurement of peak air flows and nebulized albuterol therapy. The duration of severe coughing spells and posttussive emesis for 2 weeks is not consistent with a mild upper respiratory tract infection for which reassurance and dextromethorphan might be considered.? Finally, there is no history of allergies or clinical signs of wheezing, urticaria or swelling that would suggest an allergic reaction.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of pertussisRecognize signs and symptoms of pertussisBe familiar with ancillary studies relevant to pertussisRecognize life-threatening complications of pertussisPlan management of acute pertussisQuestion 3A previously healthy 5-month-old infant is brought to the emergency department because of decreased feeding and appearing “weak.” On further inquiry, the mother states that the child is not as active as usual. She denies any history of fever, vomiting, or upper respiratory tract infection symptoms, but notes that the boy has been constipated over the past 2 weeks, which she has been attributing to his decreased feeding. There is no history of travel, exposure to pets, or any sick contacts. The father is a construction worker, and they live in a very old house with peeling paint. On physical examination, the alert and afebrile infant has a heart rate of 130 beats/min, respiratory rate of 32 breaths/min, blood pressure of 80/59 mm Hg, and oxygen saturation of 98% on room air. He has obvious generalized hypotonia, with a loss of head control, mild ptosis, a weak gag reflex, pooling of secretions in the oral cavity, and weak but elicitable deep tendon jerks. He has no asymmetry of his muscle girth or muscle fasciculations. Findings on complete blood count, serum chemistries, and urinalysis are normal.Of the following, the test that is MOST likely to establish the diagnosis for this child is?A.analysis of cerebrospinal fluid protein concentrationB.assessment of blood lead concentrationC.electromyographyD.muscle biopsyE.stool cultureCorrect answer: EThe constipation, normal sensorium, and lower motor neuron weakness described for the infant in the vignette are characteristic of botulism. The diagnostic test of choice is assessment of stools for Clostridium botulinum or serum for botulinum toxin. Although heavy metal poisoning from lead can present as constipation, this condition is more insidious, less likely in infancy, and often associated with encephalopathy and evidence of peripheral neuropathy. Elevated cerebrospinal fluid protein concentrations without a concomitant elevation of white blood cells is diagnostic of Guillain Barré syndrome (GBS), which can present with acute flaccid paralysis but is uncommon in infancy. Further, constipation is not typical of GBS. Electromyography that demonstrates decremental responses to repeated stimulation is suggestive of myasthenia gravis but is not diagnostic of the condition. Patients who have congenital myopathies such as mitochondrial myopathy, which is diagnosed by muscle biopsy, can present with generalized hypotonia, but this condition is less likely in a previously healthy infant.Botulism is an acute syndrome that presents as flaccid, descending paralysis due to a neurotoxin produced by C botulinum, a gram-positive, spore-forming, obligate anaerobe. Five distinct botulism syndromes are based on the mode of acquisition: 1) Infant botulism due to ingestion of clostridial spores that subsequently colonize the host's gastrointestinal tract and release toxin; 2) Foodborne botulism due to ingestion of food contaminated by preformed botulinum toxin; 3) Wound botulism due to infection of a wound by C botulinum, with subsequent in vivo production of neurotoxin; 4) Adult enteric infectious botulism or adult infectious botulism of unknown source, which is similar to infant botulism; and 5) Inhalational botulism due to release of aerosolized toxin from an act of bioterrorism.The effects of botulism are believed to result from the production of a heat-labile protein, the most poisonous substance known to mankind. This neurotoxin is disseminated widely via the vascular system and produces an irreversible disruption of stimulation-induced acetylcholine release from the presynaptic side of the peripheral cholinergic synapses at the neuromuscular junction. It is important to note that the neurotoxin does not affect adrenergic synapses and does not cross the blood-brain barrier. Recovery from this illness requires formation of new presynaptic terminals, a process that can take 4 weeks to 6 months.Infant botulism is the most common variety seen in the United States, with 95% of cases occurring between 3 weeks and 6 months of age. Although typically associated with ingestion of honey contaminated with clostridial spores, the spores also can gain access from contaminated dust from construction sites. Foodborne botulism occurs when the spores already have germinated and formed the neurotoxin and is seen in home canned foods or poorly processed commercial foods. Wound botulism is extremely rare and seen in traumatic wounds with crush injuries. The clinical features of botulism result from progressive neuromuscular blockade and range from mild to severe. Because the relative blood flow is highest to the bulbar musculature, all forms of this illness are characterized by progressive symmetric descending paralysis, with muscles innervated by the cranial nerves affected first, followed by those of the trunk, extremities, and diaphragm. Infants typically present with constipation and poor feeding, as described for the child in the vignette. This presentation is followed by progressive hypotonia and weakness. Cranial nerve dysfunction is manifested by decreased gag and suck, diminished range of eye movement, pupillary paralysis, and ptosis. Autonomic signs include decreased tearing and salivation, fluctuating heart rate and blood pressure, and flushed skin. Infant botulism may progress to life-threatening respiratory failure, and serious complications may develop during the disease course.Botulism is diagnosed clinically, although it often is missed in early stages in infants because of their inability to verbalize. The classic triad of botulism is the acute-onset symmetric descending paralysis with clear sensorium, no fever, and no paresthesias. The diagnosis is established by documenting the presence of C botulinum in stools or wounds or the presence of toxin in the serum. C botulinum is not a part of resident human gut flora. Many causes of hypotonia in infancy can be classified based on the site of involvement (Table) and should be considered in the differential diagnosis.Table: Causes of Hypotonia in InfancyIllnessSiteExamplesUpper Motor NeuronCortical involvementCerebrovascular accident (stroke) due to trauma, embolus, hemorrhage?Spinal involvementTrauma, infection (epidural abscess), transverse myelitisLower Motor NeuronAnterior horn cellSpinal muscular atrophy (Werdnig Hoffman disease), poliomyelitis?Peripheral nerve diseaseGuillain Barré syndrome, heavy metal poisoning, porphyria?Neuromuscular junctionMyasthenia gravis, tick paralysis, botulism?MuscleMyositis, congenital myopathies, muscular dystrophies, periodic paralysisTreatment of botulism consists of supportive care, management of respiratory emergencies, optimization of respiratory mechanics, provision of adequate nutrition and hydration, administration of stool softeners, and anticipation and treatment of complications such as secondary infections. Human botulism immune globulin is licensed for treatment of infant botulism and should be administered to all affected infants. It can be obtained from the California Department of Health Services. Studies have shown that its administration can decrease days on mechanical ventilation and length of intensive care unit and hospital stay. Older patients who have food, wound, or aerosolized botulism can be treated with equine botulinum antitoxin available from the Centers for Disease Control and Prevention.American Board of Pediatrics Content Specification(s):Recognize the signs and symptoms of foodborne or wound botulismRecognize the life-threatening complications of botulismPlan the evaluation and management of botulismRecognize the signs and symptoms of infant botulismKnow the etiology and pathophysiology of infant botulismKnow the management of infant botulismQuestion 4A 6-year-old boy presents to the emergency department at 11 pm with the complaint of fever. According to his mother, the fever has been present for the past 6 weeks, and the pediatrician has not been able to pinpoint the cause, despite numerous consultations with a variety of specialists. The mother also reports that the boy has multiple joint pain and swelling. Upon reviewing the emergency department medical records, you note that the boy and his mother have visited the emergency department multiple times in the past month. During each visit, despite normal physical examination findings, multiple invasive tests, such as complete blood counts, sedimentation rates, and blood cultures, and multiple imaging studies were undertaken, all of which have yielded normal or unrevealing results. Physical examination today of the well-appearing, afebrile boy shows normal vital signs for age and no significant lymphadenopathy, organomegaly, joint swelling, or tenderness. You suspect a factitious disorder.Of the following, the MOST appropriate next step is toA.admit the patient for evaluation of fever of unknown originB.arrange for a bone scanC.contact the primary care physician to discuss concerns regarding your suspicionsD.discharge the patient with instructions to follow up with the primary care physicianE.refer the patient to infectious diseases Correct answer CMunchausen syndrome by proxy, also known as factitious disorder by proxy, is a condition in which a caregiver produces or reports a set of signs and symptoms that do not conform to any specific disease entity or whose disease course does not respond to medical interventions. The goal usually is the psychological gain from the attention that the patient and parent receive from the respected figures (the medical staff) and others. The pediatrician frequently is a central figure and becomes an unwitting partner by ordering multiple tests and multiple consultations with subspecialists. The condition resolves once the perpetrator and the victims are separated.??Munchausen syndrome by proxy is a rare form of child abuse. “By proxy” refers to the perpetrator of the disorder other than the patient?and often is the mother figure. The common signs and symptoms include gastrointestinal complaints such as diarrhea and abdominal pain, recurrent fevers, recurrent apparent life-threatening events, and joint complaints.??Often the doctor orders multiple tests and subspecialist consultations in pursuit of the apparently elusive rare medical diagnosis, heightening the sense that something must be wrong and abetting the perpetrator’s sense of fulfillment. The perpetrator rarely has overt manifestations of other psychopathologies and, therefore, easily gains the sympathies of the physician. She commonly has some background in medical sciences. Of interest, the parent frequently appears calm out of the proportion to the seriousness of the purported underlying condition.??This is a difficult diagnosis to establish. The physician should be suspicious when:The signs and symptoms are not credible (or reproducible).The patient is receiving medical treatment that is potentially harmful (phlebotomy, long-term antibiotics, multiple imaging studies) with no progress or resolution of the symptoms.One central figure is initiating repeated evaluations.A team-based approach is necessary to establish the diagnosis. Based on the child’s history, normal findings on physical examination, and your suspicion of factitious disorder, the most appropriate next step is to contact the primary pediatrician regarding your concerns and the development of a plan. Admission to the hospital may be appropriate after discussion with the primary care physician. Further testing, such as bone scan, or referral to subspecialists, such as infectious diseases, is not warranted at this point. Enlisting the help of experts experienced in the care of children in whom child abuse is suspected can be useful. The diagnosis often is established after inpatient admission through either direct observation by hospital staff or covert video surveillance. Child abuse is not a diagnosis of exclusion. As in other forms of suspected child abuse, child protective services should be informed, even prior to establishing the diagnosis.??American Board of Pediatrics Content Specification(s):??Understand underlying mechanisms and features of Munchhausen Syndrome by ProxyQuestion 5A 14-year-old girl presents to the emergency department with severe abdominal pain and vaginal bleeding. The pain began several hours ago and has become more frequent and more severe. She denies any possibility of pregnancy. Physical examination reveals an obese female who is crying in pain. External genital examination reveals a crowning fetus and no signs of meconium staining. The patient is unsure of her last normal menses. Upon delivery, the infant is limp, cyanotic, and has poor respiratory effort.Of the following, the MOST appropriate initial steps in the management of this infant are toA.administer epinephrine and perform bag-valve-mask ventilation and chest compressionsB.administer naloxone, place an endotracheal tube, and perform tracheal suctioningC.dry and stimulate the infant, then assess the 1-minute Apgar score to determine the need for further resuscitationD.dry, warm, and stimulate the infant and suction the nose and mouthE.initiate bag-valve-mask ventilation and chest compressions for 1 minute, then reassess the infantCorrect Answer: DThe infant described in the vignette is displaying significant cardiorespiratory depression and requires emergent intervention. In the absence of meconium, the initial steps in neonatal resuscitation are drying, warming, and stimulating the infant; opening the airway; and suctioning the mouth and nose. These steps help to prevent or correct hypothermia, stimulate spontaneous respiratory effort, and remove secretions and fluids that may cause airway obstruction. If the infant does not have spontaneous respiratory effort and improvement in color following such measures, positive-pressure ventilation (PPV) should be provided. Studies have shown that the combination of drying, warming, stimulation, airway positioning and suctioning, and PPV establish normal vital signs in 99% of neonates.At the time of birth, several factors are critical to the infant’s successful transition to extrauterine life. The transition requires expansion of the lungs and clearance of amniotic fluid, initiation of gas exchange across the alveolar membrane, spontaneous respiration, a decrease in pulmonary vascular resistance to promote normal extrauterine blood flow, and closure of right-to-left shunts via the foramen ovale and ductus arteriosus. Many of these events are stimulated by varying degrees of hypoxia, hypercarbia, hypothermia, and stimulation. Drug exposure or severe degrees of acidosis, hypoxia, hypothermia, or hypovolemia, however, inhibit respiratory effort, ductus arteriosus and foramen ovale closure, and adequate cardiovascular function. Neonatal asphyxia has multiple causes (Table). The severity and duration of the insult(s) determine whether the asphyxia and resultant metabolic derangements begin in utero or after delivery.Hypothermia is common in neonates, particular those born preterm, because of their high body surface-to-weight ratio, limited fat stores, and thinner epidermis and dermis. Such propensity for hypothermia is increased further among infants born out of hospital or in the emergency department due to lower ambient temperatures. Hypothermia results in depressed respiratory drive and increased metabolic demands and can worsen acidosis, hypoxia, and hypoglycemia. Drying, removal of any wet bedding or clothing, application of external heat, and the use of warmed, humidified oxygen can help reduce heat loss and correct or prevent hypothermia.As noted previously, if the initial steps in resuscitation do not restore normal respiratory effort, heart rate, and color, PPV should be initiated. Because the lungs are not expanded and are filled with amniotic fluid in utero, the initial breaths require higher pressures (25 to 40 cm H2O) to expand the lungs. Subsequent breaths require less pressure, which should be guided by chest rise. Heart rate and color should be reassessed after 30 seconds of adequate PPV, and chest compressions should be initiated if the heart rate remains less than 60 beats/min. Endotracheal intubation should be considered if prolonged PPV is required; oxygenation or ventilation with bag-valve-mask ventilation is inadequate; or ongoing resuscitation, including medication administration, is required. Epinephrine should be administered only if bradycardia or asystole persists after adequate PPV, oxygenation, and chest compressions for 30 to 60 seconds.Preterm, small-for-gestational-age, or asphyxiated infants are at higher risk for developing symptomatic hypoglycemia. Hypoglycemia can contribute to poor tone and respiratory effort or precipitate seizures. Although the glucose concentration that necessitates treatment in neonates remains controversial, close attention to glucose values and consideration of treatment should be given to high-risk infants. A 2-mL/kg dose of 10% dextrose in water should be used to treat symptomatic hypoglycemia.Naloxone administration should be considered for depressed neonates who have known or suspected narcotic exposure and do not respond to appropriate initial resuscitation measures, as outlined previously. Care should be taken in cases of long-term maternal drug abuse because of the possibility of inducing withdrawal. The Apgar score is used to assess newborns at regular intervals after delivery. It takes into account heart rate, respiratory effort, color, tone, and reflex irritability. Although this score can be used to assess an infant’s response to resuscitation, it never should be used to determine the need for resuscitation. Tracheal suctioning should be undertaken prior to drying, warming, and stimulation in depressed infants who have meconium-stained amniotic fluid.American Board of Pediatrics Content Specification(s):Identify causes necessitating neonatal resuscitationUnderstand unique pathophysiologic processes leading to neonatal cardiopulmonary instability including role of thermal regulationQuestion 6A 17-year-old boy is brought to the emergency department by prehospital personnel after sustaining a single stab wound to the left side of the chest. Because he was breathing spontaneously, prehospital providers administered supplemental oxygen by mask. They also established two large-bore intravenous lines in his upper extremities and administered 50 mL/kg of crystalloid fluids. ?He suddenly has complete cardiopulmonary arrest in the emergency department. You successfully secure his airway.Of the following, the MOST appropriate next step in management isA.external pacingB.femoral central venous catheter placementC.open thoracotomyD.rapid infusion of multiple units of type-specific bloodE.tube thoracostomyCorrect Answer: CThe nature of the injury suffered by the boy described in the vignette and his sudden cardiopulmonary arrest in the emergency department make him a suitable candidate for an emergency thoracotomy. The situation does not allow time for transport to the operating theater; the procedure should be performed in the emergency department. The few pediatric studies that have examined the use of emergency thoracotomy appear to agree with recommendations based on adult literature that this is an arguably heroic, lifesaving measure of limited utility for selected patients. Outcomes appear to be better for patients who have sustained nonballistic, single penetrating injuries compared with blunt trauma.??Ultimately, the purpose of emergency thoracotomy is to provide temporary stabilization of a patient's injuries for subsequent definitive?repair in the operating theater. The particular patient situations for which emergency thoracotomy is indicated are:Sudden cardiopulmonary arrest just prior to arrival or while in the emergency department in a patient who has suffered penetrating injury to the chestSudden cardiopulmonary arrest in the emergency department after blunt trauma (although this indication is controversial); cardiopulmonary arrest in the field after blunt trauma is not an indication for thoracotomy because the outcome is uniformly poorPersistent severe hypotension or shock with suspected intrathoracic hemorrhage or pericardial tamponadeThe pathophysiologic objectives of emergent thoracotomy are to:Alleviate massive pericardial tamponade, especially if there is clotted blood in the pericardium that cannot be drained by pericardiocentesisControl thoracic vascular or cardiac hemorrhage directlyImprove temporary shunting of blood preferentially to the brain and heart by allowing open cardiac massage and cross-clamping of the thoracic aortaReduce further hemorrhage below the diaphragm from intra-abdominal compartment trauma by cross-clamping the thoracic aortaThe procedural technique is as follows:Place the patient supine, with the side to be operated on slightly elevated.After sterile preparation of the entire chest wall, make an opening in the left fifth intercostal space anterolaterally, starting from the sternum, avoiding the pectoralis major muscle and breast tissue in females, and following the line of the rib up to the axilla.The left side is incised more commonly because it allows more immediate exposure of the pericardium and affords easier access if open cardiac massage is necessary. Incision of the intercostal muscles and parietal pleura should occur across the superior margin of the rib to avoid the neurovascular bundle. The left-sided approach can be extended, if necessary, to include the right side, sawing through the sternum in the so-called “clamshell” thoracotomy.??Most investigators agree that emergency thoracotomy is not appropriate for patients who have no signs of life in the field and upon arrival to the emergency department after suffering either penetrating or blunt trauma to the chest. Potential problems associated with emergency thoracotomy include:Injuries to underlying thoracic structures, such as breast tissue, heart, and lungInjuries to intercostal, coronary, and thoracic blood vesselsComplications in survivors, such as bleeding and infections of the pericardium, pleural spaces, sternum, and chest wall; arrhythmias; and postpericardiotomy syndromeExposure of the health-care worker to the patient's bloodExternal pacing is indicated for symptomatic bradycardia, sinus or atrioventricular node dysfunction, or to overdrive a tachydysrhythmia not responding to medications or conventional measures. Intraosseous and femoral central venous catheters are unnecessary in this patient because he already has two large-bore intravenous catheters established. Rapid infusion of blood may be indicated for patients who have penetrating trauma wounds and shock, but it is not appropriate to wait for type-specific blood.?? American Board of Pediatrics Content Specification(s):??Discuss the indications and contraindications for emergency thoracotomy?Describe the key steps and potential pitfalls in performing emergency thoracotomy??Discuss the complications associated with emergency thoracotomy??Know the anatomy and pathophysiologic relevant to emergency thoracotomyQuestion 7The parents of a 2-year-old boy bring him to the emergency department after they found him with an open bottle of lamp oil at home.? They also bring in the two bottles of oil from home (Figure).FigureOn physical examination, the boy has normal vital signs and appears to be asymptomatic.Of the following, the MOST appropriate course of action in the emergency department is toA.discharge the boy with instructions for follow-up careB.discharge the boy with a prescription for beta-agonistsC.observe the boy in the emergency department for 4 to 6 hours and discharge him if he remains asymptomaticD.obtain a chest radiograph in 4 hours and discharge the boy if results are normal and he remains asymptomaticE.obtain arterial blood gas measurements and discharge the boy if results are normal Correct answer DHydrocarbons are, by definition, substances comprised of hydrogen and carbon. They have a range of toxic effects. Systemic absorption can lead to neurologic and hematologic toxicities. In practice, most acute adverse effects are from low-viscosity hydrocarbons that cause local toxic effects. Examples of low-viscosity hydrocarbons include gasoline, kerosene (and its derivatives such as lamp oil), lighter fluid/naphtha, and mineral spirits. These hydrocarbons irritate the gastrointestinal and respiratory tracts. The primary clinical effect of importance is chemical pneumonitis, and the major determinants of the degree of chemical pneumonitis are the volume of liquid aspirated (not ingested). Low-viscosity petroleum distillate hydrocarbon such as lamp oil can spread over large areas of the lining of the airways and alveoli, destroying surfactant and causing widespread alveolar collapse and ventilation-perfusion mismatch.The initial symptoms of hydrocarbon ingestion result from oropharyngeal and gastric irritation, and it frequently is difficult to determine?whether any of the substance has been aspirated. Vomiting prompted by gastric irritation may cause subsequent aspiration of the liquid. Therefore, it is important NOT to induce vomiting in children who have ingested hydrocarbon. Children who have aspirated a petroleum distillate hydrocarbon often demonstrate immediate significant coughing and respiratory distress. The physical examination may reveal a "petroleum" smell on the breath, tachypnea, retractions, wheezing, and rales in the lungs. More ominous signs of significant chemical pneumonitis may include marked shortness of breath and hypoxemia, but these may not develop until several hours later.Asymptomatic patients, such as the boy described in the vignette, should be observed; there is no need for investigations such as arterial blood gas. Symptomatic patients should undergo laboratory testing to evaluate and monitor their respiratory status. Arterial blood gas determinations may reflect hypoxemia, hypercarbia, and respiratory acidosis. Changes in the chest radiograph sometimes lag behind findings on physical examination, with abnormalities not becoming apparent for 4 to 6 hours after the aspiration. Patients who remain asymptomatic during 4 to 6 hours of observation in the emergency department and who have normal chest radiographic findings at that time may be discharged home. Asymptomatic patients who have abnormal chest radiographic findings should be admitted to the hospital because their respiratory status may deteriorate. Patients who are symptomatic also should be admitted for observation and supportive care. No child who has had a hydrocarbon ingestion should be discharged prior to a 4- to 6-hour observation period from the time of ingestion.Although there is no specific treatment for chemical pneumonitis caused by hydrocarbon aspiration, ensuring adequate oxygenation and ventilation is important. Appropriate interventions may range from simple supplemental oxygen to more invasive measures such as endotracheal intubation, nonconventional methods of mechanical ventilation, and even extracorporeal membrane oxygenation. Beta-agonists do not reverse the respiratory difficulties.Most children who have hydrocarbon exposures have ingestions instead of aspirations, which usually result in minor clinical effects. Most of those who develop chemical pneumonitis after hydrocarbon aspiration recover completely.American Board of Pediatrics Content Specification(s):Understand the variability of toxicity in hydrocarbon exposuresUnderstand the clinical findings in hydrocarbon exposuresUnderstand the management of hydrocarbon exposuresQuestion 8A 7-year-old boy is brought to the emergency department after sustaining a cut to his right leg from a piece of glass. The only finding of note in the well-appearing child is a clean, gaping, V-shaped 7-cm laceration (with flap) on his leg (Fig. 1). The wound is not actively bleeding, and there is no evidence of any neurovascular injury.Figure 1Of the following, the MOST appropriate approach to closing the wound after providing appropriate local anesthesia is aA.half-buried horizontal mattress suture for the flap and horizontal mattress sutures for the rest of the lacerationB.simple interrupted sutures for the flap and the rest of the lacerationC.single percutaneous suture for the flap and horizontal mattress sutures for the rest of the lacerationD.single percutaneous suture for the flap and subcuticular running sutures for the rest of the lacerationE.single subcuticular suture for the flap and simple interrupted sutures for the rest of the lacerationCorrect answer: AThe half-buried horizontal mattress stitch, also referred to as a corner stitch, is the preferred stitch for closing a wound flap. Because this stitch passes through the dermis of the flap, it creates less tension and, therefore, preserves the distal flap that may consist only of a thin layer of epidermis with poor vascular supply. The remainder of the wound should be closed with horizontal mattress stitches that help eliminate tension on large or gaping wounds.??The basic principles of wound repair are to restore anatomy, avoid infection, and achieve hemostasis. Most lacerations can be repaired by emergency department physicians. The type of suture material used (absorbable versus nonabsorbable), technique of closure (single versus multiple layer), and type of stitches placed (interrupted, subcuticular, horizontal or vertical mattress) depend on the size, shape, depth, and location of the wound.??In general, more superficial, nongaping lacerations can be closed by simple interrupted sutures.? The needle penetrates the skin at a 90-degree angle (Fig. 2), and the width and depth of the suture loop is the same on both sides of the wound.Figure 2: Needle insertion for eversion technique. For proper healing, the edges of the wound must be everted. To accomplish this, the needle should penetrate the skin at a 90 degree angle to its surface. Reproduced with permission from deLamos D. Closure of skin wounds and sutures. UptoDate, Basow DS (Ed), UptoDate, Waltham, MA 2009. Copyright (c) 2009 UptoDate, Inc. For more information, visit .The number of stitches placed depends on wound length; some authors suggest using bite distance from the wound edge as a guide to distance between the sutures. It is important to evert the edges of the laceration (Fig. 3) to improve cosmetic outcome and reduce unsightly scar formation.Figure 3: Proper technique for wound edge eversion. The proper technique for everting the edges of a wound is illustrated in the panels on the left. A) The needle has been inserted at a 90 degree angle. B) The suture loop is as wide at the base as it is at the skin surface. The width and depth of the suture loop are the same on both sides of the wound. In the panels on the right, improper technique has resulted in inversion of the wound edges, which will interfere with wound healing. C) The needle has entered the skin at an angle. D) The base of the wound is narrower than the skin surface. Reproduced with permission from deLamos D. Closure of skin wounds and sutures. A running suture is used for rapid percutaneous closure of long, uncomplicated wounds that align easily and are at a low risk for infection. This technique provides even tension along the length of the wound and easy suture removal. The first step is to place a single interrupted stitch at the edge of the wound, followed, without cutting the suture after the first knot is tied, with the placement of repeated running sutures at 45-degree angle bites along the entire wound. The loop of the final bite is kept loose and is used as a free end to tie the knot.Subcuticular running sutures generally are placed to close linear lacerations on the face and reduce scarring. Using absorbable sutures, a single anchoring stitch is placed at one end of the wound, followed by multiple sequential mirror-image bites at the same subcutaneous plane taken horizontally for the full length of the laceration.Deep gaping wounds and v-shaped wounds, such as the wound described for the boy in the vignette, are challenging and require appropriate technique to achieve adequate wound closure without complications. Gaping wounds indicate underlying tissue tension that must be reduced to avoid wound dehiscence. Tissue tension can be reduced by:Undermining the wound edges by using scissors to loosen the subdermal fatty tissue (increased risk of increased bleeding or spread of infection in contaminated wounds)Using two-layer closure (increased procedure time)Using horizontal or vertical mattress technique in some instancesBoth vertical and horizontal mattress sutures can be used to reduce wound tension and achieve eversion of wound edges, but these types of sutures are associated with a higher risk of ischemia of the wound edges. A vertical mattress suture is placed by taking a bite at a distance from the wound edge, crossing through the dermal tissue, and exiting through the skin at the opposite side at an equal distance. This is considered the far-far portion of the suture. This is followed by turning the needle 180 degrees to reverse the direction of the suture loop and taking a smaller bite closer to the wound edge on the same side. The needle is brought out at the side of the original stitch, and the knot is tied to approximate the wound edges. This is considered the near-near portion. Both points of needle entry and exit are in the same straight line.??The horizontal mattress suture is started by placing a stitch in the usual manner and bringing it out at the opposite side. The second bite is taken 0.5 cm from the first exit site and brought back to the original starting site at the same distance for the knot to be tied.??The v-shaped laceration with the flap is closed by using a half-buried horizontal mattress suture, where the needle is introduced through the skin of the nonflap portion and passed horizontally through the dermal portion of the flap (buried), with the suture loop completed by bringing the needle out through the skin on the opposite side of the nonflap portion of the wound. ?In some instances, it is advisable to extend the v-shaped laceration to a V-Y laceration to reduce tension at the flap. There is no such thing as a half-buried vertical mattress sutureAmerican Board of Pediatrics Content Specification(s):??Know the proper technique and indications for different methods of suturing (eg, horizontal and vertical mattress stitches, corner stitch)June 2010Question 1A 16-month-old girl who has a history of dilated cardiomyopathy presents to the pediatric emergency department with lethargy. During the evaluation, you note that the child is in shock. Multiple attempts at peripheral venous access are not successful. You are considering using the femoral vein for central venous access, but are concerned about potential problems.Of the following, the BEST reason to consider an alternative site is because femoral vein cannulationsA.are associated more commonly with nerve injuriesB.are associated with higher rates of air embolusC.are associated with higher rates of arterial perforationD.more commonly become infectedE.should be avoided if future cardiac catheterizations are anticipated Correct answer ECentral venous access in the femoral region has similar rates of infection, nerve injuries, and air embolus as other access sites. On the other hand, compared with internal jugular (IJ) access, the femoral route is associated with fewer arterial punctures and is not associated with pneumothoraces. One of the reasons to avoid the femoral site is out of consideration for possible future cardiac catheterization.??Attaining central venous access is an important skill for the pediatric emergency medicine physician. The indications include the need for?fluid resuscitation, monitoring central venous pressure, administration of hypertonic fluids, and inability to obtain peripheral access. There are few absolute contraindications to central venous access, especially in life-threatening situations. Relative contraindications include coagulopathy and known or suspected venous thrombus in the proposed site. Central lines above the diaphragm should be attempted for patients who suffer penetrating chest or abdominal injuries, such as from gunshot wounds.??Two techniques are used for establishing central venous access: catheter-over-needle and Seldinger. Most central line kits employ the Seldinger technique, also known as the catheter-over-the wire technique. Catheter caliber and length are determined according to patient size, site of access, and indication.??Cardiorespiratory monitoring is essential during the process. Some operators prefer having audible signals for QRS complexes, with the pitch corresponding to the oxygen saturation. This allows monitoring for arrhythmias (common in central line placement via the subclavian [SC] or IJ routes) and oxygen desaturation (as a result of pneumothorax), even when the operator does not have constant visual contact with the monitor.??As a relatively invasive procedure, central venous access requires judicious use of sedatives and local anesthesia, taking into account the patient’s clinical status. For example, the use of benzodiazepines, narcotics, and even a hypnotic agent such as etomidate can be considered to facilitate the procedure in a relatively stable patient in whom peripheral venous access has been attained. On the other hand, the procedure should be performed without delay for a hemodynamically unstable child.??When attempting IJ and SC access, efforts must be made to restrain the patient’s head, neck, and chest area securely because any patient movement could cause disastrous complications. If the clinical condition allows, placing the patient in the Trendelenburg position can help to distend the veins. Placing a long board under the buttock and thigh region and using the board and tape for gentle external rotation of the hip joint can be useful for femoral access.??Traditionally, insertion sites for central lines are chosen based on landmarks. Increasing evidence in both adult and pediatric patients points to the benefit of using ultrasonographic guidance to increase success rates and decrease the incidence of complications.? Regardless of the site, the basic steps in the Seldinger technique are to:Administer local anesthetic, prepare the site, and create a sterile field.Flush the catheters.Identify landmarks.Puncture the vein with the needle attached to syringe.Detach the syringe.Thread the J-wire through the needle and into the vessel.? If premature ventricular contractions are triggered, the wire has advanced too far. Occasionally, more sustained arrhythmias can result when the ventricle is “tickled” by the wire tip.? Be prepared with a defibrillator.?Withdraw the needle over the wire while AT ALL TIMES maintaining control of the tip of the wire. Discard the needle.Make a small incision with a scalpel at the skin without cutting the wire.Dilate the soft tissue with dilator over the wire, again always maintaining control of the exposed wire.Withdraw the dilator and thread the catheter over the wire to the desired depth, again maintaining control of the wire.Withdraw the wire and keep pressure on the catheter to prevent air embolus.Draw back and flush all ports to ascertain that all ports are intravascular.Transduce the pressure at the distal port to ascertain venous placement.For IJ and SC lines, obtain a chest radiograph to confirm that the catheter tip is outside of the right atrium and to look for pneumothorax.Secure the catheter to the skin, usually with silk sutures. Do not forget to use local anesthetic if the area was not anesthetized previously.Place a sterile dressing.The femoral vein is the most commonly used site in pediatrics due to easy access, even during active cardiopulmonary resuscitation. The vein is medial and inferior to the femoral artery in the inguinal region. The skin entry point should be 1 to 2 cm below the inguinal ligament, and the needle should be parallel to the course of the femur, pointing to the umbilicus.??The IJ vein offers the most direct route to the superior vena cava (SVC). The course of the IJ is within the carotid sheath and, therefore, in close proximity to the carotid artery and the vagus nerve. This is the site where ultrasonographic guidance (Figs. 1 and 2) consistently has demonstrated increased success rates and decreased complications.Landmarks include the triangle formed by the two heads of the sternocleidomastoid muscle and the clavicle. Obviously, care should be taken to avoid puncturing the carotid artery, which lies medial to the IJ. The nondominant hand should be used for identification of the carotid artery. The needle should be directed toward the IPSILATERAL nipple.The SC vein is perhaps the least commonly used site for central line access in the pediatric patient in the emergency department. The SC vein takes a sharper turn into the SVC, which may make it difficult to advance the catheter. In addition, pneumothorax is more common with this approach. The landmarks for the infraclavicular approach are the middle of the clavicle and the sternal notch. Insertion of the needle is preceded by anesthetizing the periosteum of the clavicle. One technique involves directing the needle toward the sternal notch in the horizontal plane. After hitting the clavicle, the clinician should “walk” the needle down the bone, keeping the needle in the horizontal plane, until it just slips under the bone into the vein.In addition to infection, arterial puncture, arrhythmia, and nerve injury, another complication of central venous cannulation deserves mentioning. There is a risk for the catheter tip to erode through the vascular structure, either the vein, or more disastrously, the right atrium. Therefore, it is important to ascertain the position of the catheter tip immediately after placement and periodically thereafter because the catheter may migrate.American Board of Pediatrics Content Specification(s):Discuss the indications and contraindications for central venous accessDescribe the key steps and potential pitfalls in performing central venous accessDiscuss the complications associated with central venous accessKnow the anatomy and/or pathophysiology relevant to central venous accessQuestion 2A 9-year-old boy is brought to the emergency department after falling on his face while skate boarding. He did not lose consciousness after the fall. On physical examination, the alert child has superficial abrasions over the nose, cheeks, and chin. One maxillary and one mandibular central incisor are chipped, and both incisors have small blood spots in the center. There is minimal bleeding in the gingiva, and palpation of the teeth reveals minimal anteroposterior mobility of the central incisors. Both teeth are tender to percussion. Radiographs show no facial fractures.Of the following, after provision of pain control, the MOST appropriate next step in the management of this injury isA.application of a temporary protective coatingB.extraction of the fractured teethC.outpatient follow-up visit with a cosmetic dentistD.antiseptic mouthwash and reassuranceE.splinting of the fractured teeth Correct answer AThe patient described in the vignette has suffered complicated (Ellis III) dental fractures to his maxillary and mandibular central incisors and requires application of a temporary protective coating to protect the pulp. This can be performed by an emergency department physician or a dentist, if available. Splinting of teeth is appropriate for avulsed or subluxed permanent teeth but not for isolated fractures. Extraction of a fractured tooth rarely is necessary and should be avoided. Outpatient follow-up with a dentist is appropriate after initial management, as described. Antiseptic mouthwash is not useful in this setting.??Approximately 50% of children suffer dental injuries, 90% of which involve central or lateral incisors. Falls are the most common causes of dental trauma in preschool and school-age children; sports-related injuries are more common in adolescents. Early interventions can improve cosmetic results, prevent tooth loss, and decrease the risk of infection, so every attempt should be made to initiate care in the emergency department. Careful and comprehensive examination, including evaluation for injuries such as luxation, intrusion, and avulsion of teeth, should be performed. Intraoral dental radiographs and panoramic views should be obtained.??Trauma to the teeth may be associated with injury to supporting structures such as the alveolar bone and periodontium (Fig. 1).Figure 1: Classification of tooth injuries. Tooth fractures may involve enamel, dentin, or pulp and may occur in the crown or the root. PDL: periodontal ligament. Dental fractures are divided into categories based on the Ellis classification system.Ellis I: This level of injury includes crown fractures that extend through the enamel only. Affected teeth are usually nontender and without visible color change but have rough edges.Ellis II: Injuries in this category are fractures that involve the enamel as well as the dentin layer. Affected teeth typically are tender to the touch and to air exposure. A yellow layer of dentin may be visible on examination (Fig. 2).Ellis III: These fractures involve the enamel, dentin, and pulp layers and often are labeled as complicated dental fractures. Affected teeth are tender (similar to those in the Ellis II category) and have a visible area of pink, red, or even blood at the center of the tooth (Fig. 3).Figure 2: Uncomplicated crown fracture. This patient has fractures of the maxillary central incisors; only the enamel and dentin are involved. ?Figure 3. Complicated crown fracture. This fracture of the upper central incisor exposes the neurovascular pulp ??Management of pediatric dental fractures depends on the age of the child, type of dentition (primary or permanent dentition), and severity of injury (complicated or uncomplicated fractures). Ellis type I fractures of primary dentition require smoothing of edges using a dental drill or an emery board. Ellis type I fractures of permanent dentition may require bonding of the chipped fragment (if available) or covering the fracture with a sealant such as glass ionomer. In emergent settings, 2-octyl cyanoacrylate can be used if no other adhesive is available. The dentin is very porous, especially in children younger than 12 years, which predisposes to pulpitis. Accordingly, Ellis type II and type III fractures must be sealed in the emergency department if possible. Additional management may involve partial pulpotomy or capping of the pulp. Ellis Type III fractures may require tooth extraction, root canal surgery, or even emergent temporary bonding of fracture fragments, with subsequent definitive management. Because the use of injectable local anesthetics at the base of the injured tooth may predispose children to sterile abscess, use of dental blocks in the emergency department is preferred for pain control during the dental procedures. Many dentists recommend the administration of antibiotics for Ellis type II and type III fractures, but evidence to support this practice is lacking. Patients may require tetanus booster. Dietary advice (soft diet), and outpatient follow-up with dentistry (within 24 to 48 hours) are recommended for all patients. Complications of dental fractures include deformity, discoloration, abscess formation, malalignment, and loss of teeth.American Board of Pediatrics Content Specification(s):Know the anatomy and pathophysiology relevant to management of dental fracturesDiscuss the indications and contraindications for management of dental fracturesDescribe the key steps and potential pitfalls in managing dental fracturesDiscuss the complications associated with the management of dental fracturesQuestion 3You are evaluating a 15-year-old boy from a juvenile detention facility who has a history of increasing pain in his right hand. He was involved in an altercation in which he punched another inmate in the face 4 days ago. He had sustained a small wound over his right fourth and fifth knuckles for which he did not seek care. On physical examination, the well-appearing, afebrile adolescent has a healing wound on the dorsal aspect of his fourth and fifth knuckles without any obvious discharge or significant swelling. He reports pain on passive flexion of his fourth finger, with point tenderness on the knuckle. Radiographs do not reveal evidence of fracture or foreign body.Of the following, the MOST appropriate next step in management is toA.admit the boy for administration of intravenous antibioticsB.arrange for outpatient hand surgeryC.arrange for outpatient magnetic resonance imagingD.discharge the boy with a prescription for oral penicillinE.initiate human immunodeficiency virus prophylaxisCorrect answer: AThe boy described in the vignette has a clenched-fist injury with signs suggestive of tenosynovitis or early septic arthritis, necessitating inpatient management with parenteral antibiotics for a likely bacterial infection. Pathogens such as hepatitis B, hepatitis C, and herpes also can be transmitted by human bites. The risk of transmission of human immunodeficiency virus via human bite is very low, and postexposure prophylaxis is not recommended. Hand surgery consultation may be indicated in cases of delayed closure of complex lacerations or for assistance in inpatient management of hand infections but is not necessary for this boy. Magnetic resonance imaging is not useful in the initial evaluation of clenched-fist injuries. Although oral penicillin is effective against Eikenella corrodens, a common organism in the oral flora, it is inappropriate as an single outpatient antibiotic, even in minor wounds, because many such infections are polymicrobial, consisting of aerobes (streptococci, Staphylococcus aureus, E corrodens) and anaerobes (Fusobacterium, Peptostreptococcus, Prevotella, Porphyromonas), many of which produce penicillinase. Human bite injuries can be occlusional, in which the upper and lower teeth come together on a body part, or clenched-fist, in which the?dominant hand typically strikes the teeth of another person. The former are more common in the young preschool child and more likely to occur on the face, upper extremities, or trunk. Child abuse should be suspected if the intercanine distance is greater than 3 cm (suggestive of an adult perpetrator) or occlusional bites are present on the genitals of a young child. Clenched-fist injuries are the most serious of human bite wounds and are more common in adolescents. Abrasions or lacerations occur typically on the fourth and fifth metacarpals. In many instances, the injuries are trivial and ignored by the patients. Clenched-fist injuries are highly prone to infection because of the proximity of the bone, joint capsule, and tendon to the overlying skin. In addition, relaxation of the skin and soft tissue after the fist is unclenched allows the oral microbes to enter a deeper and now effectively closed space, leading to septic arthritis, osteomyelitis, or tenosynovitis.Management of lacerations includes copious irrigation with sterile saline and removal of debris. Surgical evaluation to determine involvement of nerves, muscles, tendons, and bone is important. In general, lacerations should be left open and re-evaluated in a few days for delayed primary closure. Very large lacerations may be candidates for primary closure. Because most human clenched-fist wounds are small, local wound care, elevation of the injured extremity, and splinting and immobilization are indicated.Antibiotic prophylaxis is indicated in clenched-fist bites, deep puncture wounds, wounds requiring surgical repair, and those with associated crush injury. There is no conclusive evidence that antibiotic prophylaxis is beneficial for bites that cause only contusions, abrasions, or superficial lacerations of the skin.Monotherapy with amoxicillin-clavulanic acid is the prophylactic measure of choice. Alternatively, a two-drug regimen composed of an antibiotic active against Eikenella (doxycycline, trimethoprim-sulfamethoxazole, penicillin VK, cefuroxime, ciprofloxacin) and an antibiotic active against anaerobes (metronidazole or clindamycin) may be used. Antibiotics that are ineffective against Eikenella, including dicloxacillin, cephalexin, and erythromycin, should not be prescribed as monotherapy.Individuals who have infected hand wounds, such as described in the vignette, should be hospitalized for evaluation and parenteral antibiotic therapy. Empiric antibiotic therapy with a beta-lactam/beta-lactamase inhibitor combination such as ampicillin-sulbactam, piperacillin-tazobactam, or ticarcillin-clavulanic acid should be initiated pending culture results. Alternate options include a third-generation cephalosporin with metronidazole or fluoroquinolones with metronidazole. Consideration of infection due to methicillin-resistant S aureus also should guide antibiotic choice. The patient’s tetanus status should be determined and updated as appropriate.American Board of Pediatrics Content Specification(s):Recognize complications of human bitesPlan acute therapy of human bitesQuestion 4You are evaluating a 9-month-old infant transported to the emergency department via ambulance for a new-onset seizure. She sustained a 5-minute generalized tonic-clonic seizure associated with a tactile fever and chills of 4 hours’ duration. On physical examination, the fussy infant is consoled in her mother’s lap. ?Her rectal temperature is 40.9°C, heart rate is 172 beats/min, respiratory rate is 50 breaths/min, blood pressure is 90/70 mm Hg, and pulse oximetry is 99% on room air. She demonstrates good eye contact and appropriate stranger anxiety. You cannot identify any source of the fever on examination. No findings on the past medical history contribute to the diagnosis. Her immunizations are up to date. One hour after administration of acetaminophen, her temperature is 38.6°C. The infant remains interactive with her parents.Of the following, a TRUE statement about this infant’s condition is thatA.her core temperature above 40.0°C mandates more aggressive cooling measuresB.her response to antipyretics reliably excludes the presence of serious bacterial infectionC.release of endogenous pyrogens has reset her hypothalamic thermostatD.she requires round-the-clock administration of antipyretics to decrease the risk of recurrence of febrile seizuresE.the height of her fever makes a serious bacterial infection more likely than a self-limiting viral infection Correct answer CThe pathophysiology of fever (Figure) involves release of endogenous pyrogens that reset the hypothalamic thermostat.Figure: The febrile response. Reprinted with permission from Avner JR. Acute fever. Pediatr Rev. 2009;30:5-13In response to some provocation, most commonly a viral infection, macrophages release cytokines, including interleukin-1 and -2 and tumor necrosis factor. The cytokines cause local release of prostaglandin E2 in the anterior hypothalamus, which upregulates the body’s temperature regulatory center. The increased set point causes the body to respond with increased heat production by skeletal muscle contraction and decreased heat loss to the environment by cutaneous vasoconstriction. This is manifested clinically as chills, as noted for the infant described in the vignette.Fever is generally a homeostatic response and a helpful, adaptive component of the body’s acute-phase reaction. No evidence suggests that elevated temperature due to an infectious disease itself poses any threat to an otherwise healthy child. Brain or tissue damage can result from hyperthermia, which is an unregulated increase in core temperature above the set point. Hyperthermia occurs either from overproduction of heat (as in thyroid storm) or from heat stroke related to overexertion on a humid day or inability to dissipate heat (overbundled baby) or a combination of the two. Accordingly, the core temperature of the infant in the vignette does not necessitate more aggressive cooling measures. A survey conducted in 1992 by the American Academy of Pediatrics and a study conducted in two urban hospital-based clinics in 2001 suggested the continued existence of “fever phobia” among parents that is given credence by responding physicians. Pediatricians need to counsel parents that fever is a natural response of the human body and not a threat in itself (Table).Table: Counteracting Fever PhobiaEducate about fever at a health supervision visitFever is a normal response to infectionFever is a symptom not a diseaseFever determination does not always need to be exactParents should treat the child's comfort rather than a specific temperatureFever will persist until the disease process resolvesClinical appearance is importantUse the term "fever therapy" rather than "fever control"The decision to treat with antipyretics should be based on how the patient appears or behaves and should be targeted at keeping the child comfortable. Because fever causes increased insensible fluid losses, maintenance of hydration is an important part of medical management.Neither the magnitude of fever reduction nor the child’s clinical appearance after receiving antipyretics can reliably distinguish serious bacterial infection from less serious infections. There is no evidence that round-the-clock administration of acetaminophen or ibuprofen prevents recurrence of febrile seizures. Aggressive attention to fever control to mitigate the possibility of seizure only heightens the anxiety that caregivers feel about both. Further, the convulsive activity can precede the parents’ recognition of the fever.Although the prevalence of serious bacterial infection is higher in infants and children who have hyperpyrexia (temperature >41.1°C by definition) compared with lower levels of pyrexia, most infections associated with this degree of temperature elevation are still due to viral pathogens.American Board of Pediatrics Content Specification(s):Understand the pathophysiology of feverQuestion 5A 16-month-old boy presents to the emergency department with black stools. He had a similar previous episode at 6 month of age that was attributed to milk protein allergy. He was with his grandmother over the weekend, and his parents are not sure if he ingested any milk-containing food. He has no history of vomiting, diarrhea, abdominal pain, or trauma. On physical examination, the pale-appearing child has a resting heart rate of 130 beats/min, but other vital signs are normal. There is neither abdominal tenderness nor organomegaly. Rectal examination reveals normal tone with soft black stool that is guaiac-positive. Hemoglobin measures 8.0 g/dL (80 g/L). At his 12-month health supervision visit, his hemoglobin was 11.0 g/dL (110 g/L). You place a nasogastric tube and obtain clear aspirate.Of the following, the MOST likely test to reveal a diagnosis for this child plete blood count with red cell puted tomography scan of the abdomen with contrastC.flat and upright radiographs of the abdomenD.technetium-99 scanE.ultrasonography of the abdomen Correct answer DThe most likely cause of the painless rectal bleeding described for the boy in the vignette is Meckel diverticulum, the most common clinically significant intestinal malformation. Lower gastrointestinal tract (LGI) bleeding associated with Meckel diverticulum is caused by erosion and ulceration of intestinal mucosa by the acid-secreting ectopic gastric mucosa. The history often is unrevealing, except for the possible occurrence of previous similar episodes, such as described for the boy in the vignette. Laboratory findings usually indicate iron-deficiency?anemia from chronic blood loss. A nuclear medicine (technetium-99) scan for ectopic gastric mucosa offers the greatest diagnostic accuracy for this malformation. Treatment is surgical excision. Flat and upright abdominal radiographs do not provide definitive diagnostic findings. A complete blood count may reveal iron-deficiency anemia, but it does not aid in determining a definitive diagnosis.??LGI bleeding is a relatively common presenting symptom for children in the emergency department. The priority for the physician is to distinguish life-threatening from less acute conditions. Table 1 lists common causes of LGI bleeding in children, and Table 2 lists the differential diagnosis of GI bleeding in children.Diagnosis starts with a careful history and physical examination. A history of recurrent episodes, such as described for this boy, points to a chronic (or anatomic) condition. Physical examination that focuses on the abdomen and perianal area may be helpful in elucidating the diagnosis. External hemorrhoids and anal fissures usually are readily apparent. A distended or tender abdomen points to more ominous causes of LGI bleeding such as intussusceptions or volvulus. Abnormal pigmentation around the lips suggests Peutz-Jeghers syndrome, or familial intestinal polyposis, which predisposes to intussusception.? Trauma to intestinal polyps also can lead to LGI bleeding.Ischemic colitis can result from compromised gut perfusion due to intestinal obstruction, usually either from intussusception or midgut volvulus. These potentially life-threatening conditions typically are associated with at least intermittent abdominal pain as well as vomiting. The best diagnostic tests are ultrasonography for intussusception and upper gastrointestinal radiographic series for suspected midgut volvulus. Air contrast enema may be both diagnostic and therapeutic for intussusception.Allergic colitis is a common cause of LGI bleeding in the neonate. The most frequent allergen is cow milk protein. Allergic colitis is a diagnosis of exclusion because there is no definitive diagnostic test. Milk protein allergy is unlikely for this boy because he has no cramping or diarrhea. In addition, this condition usually improves and even resolves with time and does not usually manifest with intermittent symptoms.Children who have inflammatory bowel disease may present with acute LGI bleeding. The presentation of inflammatory bowel disease is extremely rare at such a young age. Children who have anal fissures usually present with bright-red blood per rectum, instead of black, tarry stool. In addition, the lesions generally are readily visible upon a careful examination of the perirectal area.Intestinal arteriovenous malformation is a rare cause of LGI bleeding in children.American Board of Pediatrics Content Specification(s):Know the etiology by age and the pathophysiology of lower gastrointestinal bleedingPlan diagnostic evaluation and initial intervention for patients with lower gastrointestinal bleedingRecognize serious and/or life?threatening causes of lower gastrointestinal bleedingQuestion 6A 3-year-old girl presents to the emergency department after household bathroom cleaner containing bleach is accidentally splashed in her eye. She has severe right eye pain, decreased vision in that eye, epiphora, and conjunctival erythema. You administer topical anesthetic eye drops.Of the following, the MOST appropriate next step is toA.administer fluoresceinB.conduct a slitlamp examinationC.irrigate the affected eye copiouslyD.measure intraocular pressure with a tonometerE.obtain computed tomography scan of the orbit Correct answer COcular irrigation and decontamination are essential in the management of chemical exposures to the eye, ocular burns, and contamination of the eye with particulate foreign bodies.? Immediate irrigation is the most appropriate next step for the chemical eye exposure described in the vignette because the prognosis of the resultant injury can depend on the timeliness/timing of initiating irrigation.Several medications or pieces of equipment may aid in the process of ocular irrigation. Anesthetic drops or anxiolysis can ease the?discomfort of the procedure, if their administration does not delay the initiation of irrigation. Lid retractors or specula may provide greater exposure of the ocular surface for irrigation and counteract any blepharospasm. Intravenous tubing, nasal cannula tubing, or specialized commercially available scleral irrigating devices are used to direct the sterile irrigating fluid gently across the eye and flush the conjunctival cul-de-sacs of any pooled substances.No universal agreement on the total volume of irrigation fluid and duration of irrigation exists, but many sources advise starting with at least 1 L for at least 30 minutes. Some controversy surrounds the optimal composition and pH of the irrigating fluid. In general, irrigation with tap water or another pH-neutral liquid is a reasonable starting point. Measurement of the ocular pH is most helpful in guiding the response and continued need for irrigation in chemical exposures.Eyelids should be everted to check for retained foreign bodies in the fornices and tarsal conjunctivae that irrigation may have failed to plications of irrigation include ongoing pain/discomfort associated with the procedure itself, blepharospasm, corneal injury from irrigation fluid striking the cornea directly or with excessive force, and potential worsening of the initial injury based on the pH of the irrigation solution and the type of chemical exposure. Although there are no absolute contraindications for ocular irrigation and decontamination, such procedures should be used cautiously if a ruptured globe is suspected.Fluorescein administration, a slitlamp examination, measurement of intraocular pressure, or computed tomography scan of the orbit might be helpful in the management of ocular emergencies but should not delay initiation of ocular irrigation, which is most important for the patient in the vignette. Fluorescein dye evaluates for corneal epithelium injuries, which is appropriate after a chemical exposure. A slitlamp examination is essential for careful evaluation of the anterior chamber and can be used to assist with removal of corneal foreign bodies. Tonometry is used to measure intraocular pressure in suspected glaucoma and blunt eye trauma. Computed tomography scan of the orbit is a useful ancillary study in suspected intraocular foreign body, penetrating globe injury, or craniofacial trauma.American Board of Pediatrics Content Specification(s):Discuss the indications and contraindications for ocular irrigation and decontaminationDescribe the key steps and potential pitfalls in performing ocular irrigation and decontaminationDiscuss the complications associated with ocular irrigation and decontaminationKnow the anatomy and pathophysiology relevant to ocular irrigation and decontaminationQuestion 7A 13-year-old girl is brought to the emergency department for evaluation of a right hand injury sustained during a fight at school.? The girl’s mother reports that the child has been suspended from school for the fight and other recent behavioral problems. She adds that her daughter also has been having difficulty sleeping and has become more withdrawn and “easily agitated.” When questioned alone, the girl reports that one of her friends was killed recently in a gang-related shooting, and she has been having problems at school since then. She admits to difficulty sleeping and concentrating and frequent thoughts about her friend but denies any thoughts of harm to herself or others. She denies any drug or alcohol use or sexual activity. Her vital signs are within normal limits. The only finding of note on physical examination is bruising and abrasions over her right lateral hand. Urine toxicologic and pregnancy screens yield negative results. You evaluate and treat her hand injury.Of the following, the MOST appropriate next step in this child’s care is toA.admit the child for a 72-hour involuntary psychiatric evaluationB.arrange for outpatient psychiatric evaluationC.assess serum ethanol concentrationsD.make a child protective services referral for suspected child abuseE.perform a pelvic examination and send samples for sexually transmitted infection testingCorrect answer: BThe girl described in the vignette is demonstrating both internalized (depression, withdrawal) and externalized (aggressive behavior) symptoms of trauma exposure. She is exhibiting some depressive signs and symptoms (sleep disturbance, withdrawn affect), which should prompt further screening for depression and particularly for suicidal or homicidal thoughts or behaviors. Even in the absence of imminent danger to herself or others, this child warrants referral for psychiatric evaluation and counseling about her recent exposure to community violence, loss of a close friend, and depression symptoms. Emergent psychiatric custody and evaluation are indicated in the presence of clear suicidal or homicidal behavior. In the absence of symptoms of acute intoxication, there is no need to assess ethanol concentrations. No findings suggest child abuse or neglect that warrant child protective services notification.Various studies have shown that more than 90% of children have personally witnessed or been victims of some form of family or?community violence by the time they enter high school. Such exposure can be in the form of child abuse by a parent or caregiver, intimate partner violence (IPV) within the home, physical or psychological bullying at school, or violent acts in the community. Nearly 75% of adolescents report being victims of a violent crime. Community violence is a serious public health concern in the United States, where the rate of violent death far exceeds that in other developed countries. Firearm-related crimes account for nearly 50% of such violent deaths. More than 1.5 million emergency department visits each year are related to violent injury. The victims of such crimes are more than twice as likely as nonvictims to use health-care services, but they may not relate their symptoms to the prior victimization. Older child and adolescent victims of violence are more likely to become perpetrators of violent crime in the future. Violence is more common in lower socioeconomic groups, among those who own or carry firearms, and among those who have a history of drug or alcohol abuse.Between 25% and 50% of adult women report having been victims of IPV at some time, and many of these women have one or more children at the time of the incident. IPV coexists with child abuse and neglect in a significant percentage of cases, as do drug and alcohol abuse and psychiatric illness. Both IPV and community violence occur with greater frequency in lower socioeconomic groups, making the coexistence of violence inside and outside of the home a common problem.The signs and symptoms related to exposure to violence differ somewhat by age. Infants and young children are likely to demonstrate more subtle and nonspecific symptoms, such as sleep and feeding difficulties, regression in developmental milestones, fear of being left alone, and increased crying. School-age children also develop sleep disturbances such as night terrors or nightmares, inability to fall asleep or sleep through the night, and fear of sleeping alone. Cognitive dysfunction may manifest as school avoidance and poor academic performance. Aggressive behavior, anxiety, depression, or true posttraumatic stress disorder symptoms (re-experiencing the traumatic event, avoidance, intrusive thoughts, and exaggerated responses to certain stimuli) also may occur. Depression symptoms, poor academic performance, and aggressive behavior predominate during adolescence. Drug and alcohol abuse are common manifestations of violence exposure in adolescents and adults. Risk-taking behavior leads to an increased incidence of recurrent sexually transmitted or genitourinary infections. Somatic symptoms can occur in all age groups; chronic pain, constipation or diarrhea, headaches, and fatigue are common complaints.The type, severity, and duration of symptoms vary and may be mediated by the patient’s age and sex, duration and severity of the exposure, how recent the trauma was, and the presence and stability of a support system (family, peer, school, or community). Coexisting trauma in multiple realms (eg, both at home and school) is likely to result in more severe and persistent symptomatology. An infant or child is more likely to display trauma symptoms if the parent or caregiver is experiencing symptoms related to violence.American Board of Pediatrics Content Specification(s):Recognize signs and symptoms of exposure to violence in children, adolescents, and adultsUnderstand the dynamics of family violence as it impacts on children and adolescentsUnderstand the dynamics of community violence as it impacts on children and adolescentsQuestion 8Emergency medical services brings in a 16-year-old boy who was struck by multiple bullets while crossing the street. Physical examination reveals spontaneous respirations, normal oxygen saturation, and normal heart rate and blood pressure. His Glasgow Coma Scale (GCS) score is 9. You place two large-bore intravenous lines. You discover a small wound in the right parietal region that is not actively bleeding. There are no other obvious wounds to the head. His pupils are sluggishly reactive. Two other wounds on his right leg also are not actively bleeding.Of the following, the MOST appropriate next step in the management of this boy’s injury is toA.administer a tetanus booster and antibioticsB.obtain a chest radiographC.obtain focused bedside ultrasonography to look for thoracic and abdominal injuriesD.perform endotracheal intubationE.transport the patient to the radiology suite for immediate head computed tomography scan Correct answer DJudging the extent of injury in gunshot wounds (GSWs) to the head is difficult, although the boy described in the vignette has obvious signs of significant neurologic damage. Early airway management is important in this situation because penetrating injuries to the head often lead rapidly to cerebral edema, apnea, and hypoxemia. Accordingly, immediate endotracheal intubation should be performed to decrease further injuries from impending respiratory failure and hypoxemia. Other therapeutic and imaging modalities, including administration of a tetanus booster and antibiotics, bedside ultrasonography, chest radiography, or computed tomography scan, should follow securing of the airway. Surgical interventions include debridement and control of any sites of bleeding.??Penetrating head injuries can be divided into those involving missiles (usually with high kinetic energy) and those involving impalement? (lower kinetic energy). GSWs are the most common cause of the former injuries in the United States. In fact, the United States has the highest rate of GSW-associated head injuries in the developed world. These injuries are comparatively more common in older adolescents, and the increased rate persists through young adulthood. Centers for Disease Control and Prevention data show that firearm injury rates have declined in the past decade, but they still are the second most common cause of mortality in children older than 12 years of age.??The amount of energy transfer from the missile to the body is more important than just the kinetic energy of the missile.? Factors related to energy transfer are listed in the Table.Table: Factors Related to the Transfer of Energy of a Missile to the BodyThe kinetic energy of the missile at the time of impact (related to the initial speed and the distance from the body)The angle (or yaw) at which the missile strikes the bodyThe size, type and weight of the missileThe distance the missile travels in the bodyWhether the missile exits the body (an exiting missile transfers less energy than a missile that does not exit the body)The characteristics of the injured tissue (which type of tissue and whether the injury was due to crushing, tearing, etc.)A GSW to the head is usually devastating, with on-the-scene mortality seen in two thirds of cases. Some predictors for survival include the initial GCS score and pupillary responses. For example, the mortality is 100% among patients whose GCS scores are less than 5 and who have no pupillary response. In contrast, the survival rate is 75% among those who have GCS scores greater than 8 and pupillary response.The path of any projectile that strikes a person’s head can be erratic. High-velocity and small projectiles may glance off the skull, leaving little external signs of trauma. However, the shock to the skull can cause fractures, cerebral contusions, and bleeding. When the missile penetrates the skull, its intracranial trajectory often is difficult to predict. Projectiles can bounce around the inner skull, be redirected, tumble, yaw, and rotate. Any missile that crosses the midline or invades the posterior fossa usually is associated with significant damage. If the missile exits the skull, there is often a large exit wound, causing major damage. In addition to direct tissue damage, the missile can lacerate vessels, lodge in ventricles and cause hydrocephalus, and introduce infections into deep spaces of the brain. Missiles that have significant kinetic energy can cause thermal injuries, even to sites remote to the actual trajectory. A large number of victims suffer seizures soon after the injury and, if they survive, days to months later.Impalements with common objects such as pencils are more common than GSWs, but they are associated with lower mortality. Similar complications, such as bleeding, infection, and local tissue destruction, occur. It is important not to remove the object outside the operating room so as not to precipitate hemorrhage.American Board of Pediatrics Content Specification(s):Understand the most common and most dangerous causes of penetrating head injuries in childrenUnderstand the significance of the penetrating object in evaluating a penetrating injury to the central nervous systemUnderstand the relationship between ballistics and penetrating injury to the brainJULY 2010Question 1A 4? -year-old boy presents to the emergency department with “pink eye.” His mother states that his right eye has been injected and irritated for the last 24 hours. It was crusted shut this morning and has been producing mucoid discharge throughout the day. He has otherwise been well, without fever, runny nose, or cough. Of note, his 2-year-old sister was ill last week with high fever for 5 days and Streptococcus-negative pharyngitis. The boy is enrolled in summer swim lessons, and the mother needs a note indicating when he can return to the pool. The only finding of note on his physical examination is conjunctival injection of the right eye, with minimal discharge noted at the inner canthus.Of the following, the MOST appropriate treatment for this boy isA.artificial tears, with return to swim lessons when symptoms resolveB.culture of discharge and treatment with sulfacetamide ophthalmic drops four times daily if Gram stain is positive, with return to swim lessons after 5 days of treatment or resolution of symptomsC.erythromycin ointment four times daily, with return to swim lessons in 48 ical glucocorticoid eye drops, with return to pool when conjunctiva are clearE.3% vidarabine ophthalmologic drops four times daily, with return to swim lessons in 48 hoursCorrect answer: AAcute conjunctivitis is a benign, self-limited condition characterized by inflammation and injection of the bulbar (portion covering globe) and/or tarsal (portion lining lids) conjunctiva. It can be caused by infections, allergies, or irritants.? The boy described in the vignette most likely has viral conjunctivitis caused by adenovirus. The combination of his sister’s illness 1 week ago, the patient’s current conjunctivitis, and the season of the year are highly suggestive of the diagnosis. Morning crusting does not differentiate viral from bacterial illness; it is common in both. However, bacterial conjunctivitis more classically involves continuous discharge throughout the day that may be white, yellow, or green. It is described as copious and recurring within minutes of clearing the eye. In viral conjunctivitis, the discharge is less prominent throughout the day, and tearing or mucuslike discharge is more common. Patients frequently describe burning or a feeling of “sand in the eye.” Both viral and bacterial conjunctivitis are contagious, with direct spread occurring after touching contaminated eye discharge. The infection is spread easily in pools and child care centers. The common practice of prescribing antimicrobial drops to children who attend child care to facilitate a faster return to care (most centers allow return after 24 hours of treatment) is not supported by evidence.? Treatment for presumed viral conjunctivitis is supportive, with artificial tears or lubricants providing symptomatic relief from eye irritation. To prevent spread, children should be isolated until symptoms have resolved, especially for a recreational activity such as swimming. The decision to treat a child in child care is more complex; cost/benefit analysis may take into account lost time from work for the parent as well as the cost of medication. Although newer generations of topical antimicrobials, including fluoroquinolones and azithromycin, have been shown to be effective in the treatment of acute infectious conjunctivitis, they probably are not necessary and may promote the emergence of resistant organisms. Fluoroquinolones are specifically indicated in the treatment of conjunctivitis in contact lens wearers, in whom the rate of pseudomonal infection is high. Topical steroids have no role in the treatment of acute conjunctivitis by emergency care clinicians. Corticosteroids have been associated with bacterial superinfection, masking serious viral infection due to herpes simplex virus, and increasing the duration of adenoviral shedding. Prolonged use can lead to the development of glaucoma and cataracts. Culture and Gram stain are indicated for gonococcal conjunctivitis, and erythromycin ophthalmic ointment is the treatment of choice for bacterial conjunctivitis in infants and toddlers.Differentiating between bacterial and viral conjunctivitis can be difficult based on symptoms alone. Many sources indicate that bacterial infection is more common in early childhood, although case series often are biased toward more symptomatic patients and, thus, may overrepresent bacterial disease. Even bacterial conjunctivitis is self-limited, and a recent blinded, randomized, placebo-controlled trial comparing topical chloramphenicol to placebo in children 6 months to 12 years of age who had acute infective conjunctivitis showed no significant difference in outcome. The cure rate was 86% in the chloramphenicol group and 83% in the placebo group at day 7. A 2006 meta-analysis from the Cochrane Database of Systematic Reviews, which included both pediatric and adult studies, demonstrated significantly improved rates of clinical and microbiological remission in patients treated with a heterogeneous group of topical antibiotics. Most cases, however, resolved spontaneously, with clinical remission achieved in 65% of cases by days 2 to 5 among patients receiving placebo.? The cause of conjunctivitis varies by age. In the first 24 hours after birth, chemical conjunctivitis caused by silver nitrate ocular prophylaxis is most common, and no treatment is required. At 3 to 5 days after birth, Neisseria gonorrhoeae becomes a serious concern. Gonococcal conjunctivitis is characterized by sudden, severe, grossly purulent conjunctivitis. Such infection is an ocular emergency because it can progress to ulceration and perforation of the globe within 24 hours if untreated. Inpatient treatment with aggressive eye irrigation and systemic coverage with ceftriaxone is the treatment of choice. The incidence of gonococcal conjunctivitis has decreased dramatically with the near-universal use of ocular prophylaxis at birth. Less severe eye discharge beginning at 1 to 2 weeks of age frequently is caused by Chlamydia trachomatis. Systemic treatment with erythromycin is necessary and treats concomitant chlamydial pneumonia, which may become symptomatic at 4 to 6 weeks of age. In the newborn period, gonococcal conjunctivitis always should be ruled out with an immediate Gram stain and culture of eye discharge. If the stain reveals gram-negative diplococci, immediate aggressive treatment should be initiated pending culture results. Bacterial conjunctivitis is more common among infants and toddlers. The most frequently isolated pathogen is Haemophilus influenzae, followed by Streptococcus pneumoniae and, less commonly, Branhamella catarrhalis. Haemophilus infection often is associated with the conjunctivitis-otitis syndrome, and older literature recommends a short course of oral therapy for a toddler suspected of having Haemophilus infection.? More recent conservative antibiotic prescribing patterns for otitis media, including the use of “wait and see” antibiotic prescriptions, may suggest that the oral therapy could be falling out of favor. Bacterial conjunctivitis in those who do not wear contact lenses can be treated with inexpensive topical antibiotics, such as erythromycin ophthalmic ointment for infants and toddlers or combination polymyxin-trimethoprim ophthalmic drops for older children. Failure of response within 1 to 2 days is an indication for ophthalmologic referral.Severe pain in a dermatomal distribution is typical for conjunctivitis caused by herpes simplex virus or varicella zoster. The infection can be severe and is characterized by a dendritic pattern on fluorescein staining of the eye. Affected patients should receive antiviral therapy as well as close follow-up and treatment in consultation with an ophthalmologist.? Allergic conjunctivitis is characterized by itchy eyes and clear discharge. Eyes may be crusted shut in the morning. It is more common in school-age children and adolescents and frequently is associated with seasonal allergies. If accompanied by other systemic allergic symptoms, the conjunctivitis can be managed with oral antihistamines. Eye involvement alone may be treated with topical antihistamines, mast cell stabilizers, or nonsteroidal anti-inflammatory agents.American?Board of Pediatrics Content Specification(s):Know the etiology of conjunctivitisRecognize the signs and symptoms of conjunctivitisProvide appropriate management for a child with conjunctivitisQuestion 2A 1-year-old boy is brought to the emergency department by his mother because she found him drinking from an open bottle of oil of wintergreen approximately 2 hours ago. The bottle had not been used previously and more than 25% of the fluid was missing. The boy’s breath and clothing smell like oil of wintergreen. Of the following, the MOST likely acid-base disturbance expected in this boy at this time bined respiratory and metabolic acidosisB.metabolic acidosisC.metabolic alkalosisD.mixed respiratory acidosis and metabolic alkalosisE.respiratory acidosisCorrect Answer: BOil of wintergreen contains 98% methyl salicylate, which can be highly toxic, even in small doses, when ingested by young children such as the boy described in the vignette. One teaspoon of oil of wintergreen is equivalent to approximately 7,000 mg of salicylate. The boy in the vignette is most likely to manifest clinical signs of acute salicylate toxicity.?HYPERLINK "javascript:showModal('/subscriber/modal/july/july-q2-content-specifications')"???? The signs and symptoms of salicylate poisoning result from a combination of its complex effects on the body’s acid-base status and a number of organ systems (Table). Affected systems include the cardiovascular, central nervous, gastrointestinal/hepatic, hematologic, metabolic, renal, and respiratory.Table: Systemic Effects of Salicylate PoisoningSystemClinical ManifestationsCardiovascularTachycardia, hypotension, dysrhythmiasCentral nervousTinnitus, hearing loss, lethargy, disorientation, agitation, seizures, comaGastrointestinal/HepaticNausea, vomiting, epigastric pain, hepatitis, pylorospasm, decreased intestinal motilityHematologicDecreased prothrombin concentrations, platelet dysfunctionMetabolicDehydration, hypokalemia, hypo/hypernatremia, hypo/hyperglycemia, hypo/hypercalcemiaRenalRenal insufficiency/failureRespiratoryRespiratory alkalosis, tachypnea, hyperpnea, noncardiac pulmonary edemaMiscellaneousHyperpyrexia, leukocytosis, rhabdomyolysisBlood gas chemistries are more useful tests to help differentiate the various phases in salicylate poisoning and assess the patient's metabolic stability. The initial phase consists of pure respiratory alkalosis due to direct central stimulation on the brain's respiratory center. Adults often present with a mixed respiratory alkalosis and metabolic acidosis. Infants do not respond with the same degree of sustained hyperventilation as adults; their respiratory alkalosis may be transient, with metabolic acidosis manifesting earlier in the course. Accordingly, metabolic acidosis would be the expected early manifestation for the child in the vignette.???? ??A widened anion gap metabolic acidosis is seen due to several processes:Lactate from interruption of oxidative phosphorylationKetones from increased fatty acid metabolismRetention of phosphoric and sulfuric acids due to renal??insufficiencyA normal anion gap metabolic acidosis may be present early after an ingestion and does not rule out salicylate poisoning. ??In the later stages, as the metabolic acidosis worsens and compensatory metabolic mechanisms no longer can respond, myocardial dysfunction, hypotension, shock, cerebral and pulmonary edema, and ultimately death ensues.? ??Classic symptoms of acute salicylate poisoning include diaphoresis, hyperventilation, tinnitus or hearing loss, and gastrointestinal irritation (nausea and vomiting). However, children may not manifest all of these symptoms.?? ??Among the helpful ancillary studies in salicylate poisoning are ferric chloride testing, serum salicylate assessment, blood gases, urine pH, and abdominal radiographs. Several drops of a 10% ferric chloride solution can be added to 1 mL of urine to detect the presence of salicylates. A positive result is indicated by the solution changing to a brown-purple color and necessitates determination of a quantitative serum salicylate concentration.??? ??Salicylate in the serum can help confirm the diagnosis of salicylate poisoning. However, recent data reveal that the Done nomogram is not useful in clinical management because specific concentrations do not correlate well with severity of poisoning. Many toxicologists now discourage its use. Furthermore, values can be in the therapeutic range in chronic toxicity. Studies recommend assessing serum concentrations at least 4 to 6 hours after the ingestion, which should be the time of peak concentrations. A 6-hour value of greater than 100 mg/dL is considered potentially lethal and an indication for hemodialysis. In cases of chronic salicylism, hemodialysis may be indicated at a lower serum concentration, depending on clinical symptoms. Values should be monitored until there is an obvious trend in decreasing concentrations, which should be accompanied by improvement in the patient’s clinical status.? ??Alkalinization of the urine is recommended to enhance renal excretion of salicylates; the pH should be maintained between 7.5 and 8.5 for optimal results. Patients who do not respond to alkalinization alone should receive hemodialysis. Abdominal radiographs should be obtained to search for large collections or bezoars of enteric-coated tablets, especially in intentional overdoses. ?? ??American?Board of Pediatrics Content Specification(s):??Recognize signs and symptoms of salicylate poisoning??Understand the usefulness of ancillary studies in salicylate poisoning??Plan the management of acute salicylate toxicityQuestion 3The manufacturer of a new rapid urine dipstick test for diagnosing urinary tract infection (UTI) claims that this test will eliminate the need for obtaining urine cultures, which represent the gold standard, because the results will be available immediately and are as accurate and reliable. Prior to implementing the test in your emergency department, you conduct a study to verify the test characteristics. You enroll and obtain catheterized urine samples from 100 febrile infants who are being screened for UTI. The rapid test is conducted in addition to urine cultures, and the results reveal a 10% rate of UTI in the tested population. The rapid test yields positive results in eight of the infants who have positive urine cultures. Unfortunately, the test also yields positive results in five infants who do not have UTI. Of the following, the respective sensitivity and specificity of the new test is CLOSEST to ?A.62% and 97%B.80% and 6%C.80% and 94%D.87% and 94%E.Cannot be calculated Correct answer CSensitivity is defined as the ability to identify correctly those who have disease (true positives=proportion of people who have disease and positive test results). Specificity is the ability to identify correctly those who do not have disease (true negatives=proportion of people who do not have the disease and have negative test results). Of the population described in the vignette, 10% (10 of 100) infants had UTI?based on the urine culture. Eight of the infants had positive rapid test results, indicating a sensitivity of 8/10 or 80%. In addition, five of the 90 infants who had negative urine cultures had false-positive rapid test results, indicating a specificity of 85/90 or 94%.Sensitivity and specificity traditionally are used to evaluate the usefulness of a screening test compared with the reference standard, and the clinician should understand how they are calculated. The first step is to populate a 2x2 table in which, by convention, the disease status is placed in the columns and the results of the test (positive or negative) are placed in rows (Table).Table: 2x2 Table for the Vignette?With DiseaseWithout DiseaseTotalTest positiveTrue-positives (a=8)False-positives (b=5)a+b=13Test negativeFalse-negatives (c=2)True-negatives (d =85)c+d=87?a+c=10b+d=90Total=a+b+c+d=100Sensitivity=a/a+c Specificity=d/b+dPositive predictive value=a/a+bNegative predictive value=d/c+d It is important to note that sensitivity and specificity are inherent characteristics of the assay and are independent of the prevalence of the disease in the population of interest. In contrast, positive and negative predictive values vary, depending on the prevalence of the disease in the study population. American Board of Pediatrics Content Specification(s):Define and calculate sensitivityDefine and calculate specificityQuestion 4A previously well 15-year-old girl is brought to the emergency department with complaints of tingling and numbness around her mouth for the past 2 days. She is complaining that she is more tired lately, but it has not restricted her activities. She has no history of fever, change in appetite, exposure to any medications, sick contacts, bowel or bladder incontinence, weakness in extremities, or headache. She is a well-adjusted teenager who is otherwise doing well at school. On physical examination, she is awake, alert, and responds appropriately to questions. Her vital signs are normal, as are findings on her systemic examination. Funduscopic examination results are normal, and her central nervous system examination does not reveal any focal neurologic deficit. While her blood pressure is being taken, she complains of some tingling in her arm, followed by painful flexion of her wrist and thumb. This resolves when the sphygmomanometer cuff is deflated. Of the following, the MOST likely diagnosis isA.conversion reactionB.drug overdoseC.hypocalcemiaD.seizureE.transient ischemic episode Correct answer CThe patient described in the vignette is exhibiting carpopedal spasm induced by transient ischemia due to the inflated blood pressure cuff. This sign, also called the Trousseau sign, is characteristic of latent tetany due to hypocalcemia. Another less easily elicited sign of hypocalcemia is the Chvostek sign, which is characterized by ipsilateral contraction of the facial muscles when the facial nerve is tapped anterior to the ear. The generalized weakness and perioral tingling and numbness reported by this girl, combined with the positive Trousseau sign, are highly suggestive of hypocalcemia. Conversion reaction is unlikely in the presence of overt spasm of her arm muscles. No information elicited in the history or physical examination suggests drug overdose, seizure, or transient ischemic episode. Among the many causes of hypocalcemia (Table), autoimmune hypoparathyroidism is likely in an otherwise previously healthy adolescent who has new-onset hypocalcemia.?Table: Potential Causes of HypocalcemiaNeonatal HypocalcemiaTransient or early neonatal hypocalcemia1. Maternal: hyperparathyroidism, use of anticonvulsants, vitamin D deficiency, diabetes mellitus2. Neonatal: prematurity, intrauterine growth restriction, neonatal hypoxia, exogenous phosphate or lipids in parenteral nutritionHypoparathyroidism1. Genetic: autosomal recessive or X-linked2. Syndromic: DiGeorge, mitochondrial disorders (MELAS [mitochondrial encephalopathy, lactic acidosis and strokelike episodes], Kearns Sayre syndrome)3. Autoimmune: autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED)4. Acquired: following neck surgery, iron or copper deposition5. Calcium-sensing receptor defect6. Pseudohypoparathyroidism: end-organ resistanceHypovitaminosis D1. Decreased intake and nutritional deficiency2. Decreased absorption: postgastrointestinal surgery, malabsorption, cystic fibrosis3. Decreased hydroxylation in liver and kidney (vitamin D-dependent rickets)4. Vitamin D resistance: vitamin D-dependent rickets (type 2)Miscellaneous1. Hypomagnesemia2. Pancreatitis3. Hyperphosphatemia4. Fluoride poisoningHypocalcemia is defined as a total serum calcium concentration less than 9.0 mg/dL (2.25 mmol/L) in children, 8.0 mg/dL (2.0 mmol/L) in term infants, and 7.0 mg/dL (1.75 mmol/L) in preterm infants. Serum calcium is regulated in a narrow range by parathyroid hormone (PTH) and calcitonin. Calcium is present in the bones, and less than 1% of the total body calcium is present in the serum and extracellular spaces. Approximately 50% is in the active or ionized form, 40% is bound to albumin, and 10% is present in the chelated form. Even minimal reductions in serum ionized calcium stimulate the calcium-sensing receptors that, in turn, trigger the release of PTH. PTH increases serum calcium concentrations by increasing renal calcium reabsorption, mobilizing bone calcium, and increasing 1,25-dihydroxyvitamin D (active form of vitamin D) that, in turn, increases renal and intestinal calcium absorption. Calcitonin is secreted in response to an elevated serum calcium concentration and reduces serum calcium by reducing bone reabsorption. The characteristic of hypocalcemia is neuromuscular irritability, which manifests as perioral numbness, paresthesias, and muscle cramps in mild cases and carpopedal spasms and seizures in severe cases. Signs and symptoms of hypocalcemia vary along a continuum. Mild, nonspecific systemic symptoms can include vomiting, muscle weakness, and irritability. At the other end of the spectrum are life-threatening, anticonvulsant-resistant seizures; arrhythmias (QT prolongation, ST prolongation that leads to supraventricular tachycardia, complete heart block, and torsades de pointes ventricular tachycardia); and laryngospasm. Neonates may present only with nonspecific symptoms such as apnea, tachycardia, lethargy, poor feeding, vomiting, and abdominal distension. In addition, patients may show signs of underlying disease such as rachitic changes in bones due to vitamin D deficiency or resistance, dental abnormalities (failure of tooth eruption, dental hypoplasia), developmental delay (mitochondrial disorders that include mitochondrial encephalopathy, lactic acidosis and strokelike episodes [MELAS] syndrome and Kearns Sayre syndrome), or cardiac abnormalities (DiGeorge syndrome). Evaluation of the patient in whom hypocalcemia is suspected varies according to the potential cause. Minimum studies in the emergency department should include measurements of serum calcium (total and ionized), total protein, albumin, magnesium, phosphorous, alkaline phosphatase, and creatinine; blood gas for pH; and urinary calcium and urinary creatinine assessment. Blood for assessment of serum PTH, vitamin D, PTH antibodies, and calcium-sensing receptor antibodies may be obtained after consultation with the endocrinologist. Serum ionized calcium values correlate with signs and symptoms of hypocalcemia and must be measured directly or calculated from the total serum calcium values after adjusting for serum albumin. As a rough estimate, the calcium concentration decreases 0.8 mg/dL (0.2 mmol/L) for every 1.0-g/dL (100-g/L) decrease in albumin concentration. Calcium binding to albumin is pH-dependent, with acidemia increasing ionized calcium concentrations and alkalosis increasing albumin binding, thus effectively reducing serum ionized calcium concentrations. For every change of 0.1 units in pH, ionized calcium changes by 10% without altering the total calcium concentration.??Emergency management of symptomatic hypocalcemia requires administration of intravenous calcium. Suggested intravenous doses are 2-mL/kg of 10% calcium gluconate or 0.7 mL/kg of 10% calcium chloride. Each provides 20 mg/kg elemental calcium and should be administered over 10 to 20 minutes with careful monitoring for cardiac arrhythmias. Care should be taken to avoid coadministration with other solutions that result in calcium precipitation, such as sodium bicarbonate. Repeat bolus doses may be provided if the patient continues to be symptomatic. Following the bolus, calcium concentrations should be monitored and maintained either by continuous intravenous infusion (200 to 500 mg/kg per day) or via oral replacement. American Board of Pediatrics Content Specification(s):Recognize the signs and symptoms of hypocalcemiaBe familiar with ancillary studies relevant to hypocalcemiaRecognize the life-threatening complications of hypocalcemiaPlan management of acute hypocalcemiaQuestion 5Upon responding to a call from a party, emergency medical services find a 14-year-old girl who is unresponsive, apneic, and has pinpoint pupils. After they administer naloxone in the field, she regains spontaneous respiratory effort. Friends at the party state that she was previously well and had no apparent comorbidity. She also had no preceding head trauma, vomiting, or recent illness. On physical examination in the emergency department, the girl’s temperature is 37.2°C, respiratory rate is 28 breaths/min with nasal flaring, heart rate is 108 beats/min, blood pressure is 110/78 mm Hg, and pulse oximetry reading is 87% on room air. Her breath sounds are equal, with moderate wheezing and good air exchange. Cardiovascular examination reveals sinus tachycardia without murmur or gallop, 2+ pulses, and warm extremities. She is somnolent but arouses to vigorous stimulation and follows commands. Her pupils are pinpoint and reactive. With supplemental oxygen, the pulse oximetry reading increases to 93%. You obtain chest radiography (Figure).FigureOf the following, the MOST appropriate next step in management is intravenous administration ofA.ceftriaxone 50 mg/kgB.clindamycin 10 mg/kgC.dopamine 10 mcg/kg per minuteD.flumazenil 0.01 mg/kgE.furosemide 1 mg/kgCorrect answer: EThe symptom triad of altered mental status, respiratory depression, and pinpoint pupils, with a therapeutic response to naloxone, described for the girl in the vignette is strongly suggestive of opiate overdose. The chest radiograph (Figure) reveals bilateral airspace disease with a normal cardiac silhouette, consistent with noncardiogenic pulmonary edema (NCPE), most likely due to opiate toxicity.NCPE related to opiate overdose manifests as extensive patchy, bilateral airspace consolidations; indistinct vessels; and peribronchial??cuffing. The exact mechanism of opiate-induced NCPE is unclear. It frequently occurs after reversal with naloxone. When it is not associated with renal insufficiency or other complications such as aspiration of gastric contents, rapid resolution is noted within 1 or 2 days, with no parenchymal sequelae. Careful attention to the airway, ensuring adequate oxygenation and ventilation, and supportive care, usually is all that is necessary. A list of causes of NCPE can be recalled using the mnemonic “NOT CARDIAC” (Table).Table: Causes of Noncardiogenic Pulmonary Edema ? Near drowning ?? Oxygen therapy ?? Transfusion or Trauma (fat embolism) ?? Central nervous system disorder (neurogenic pulmonary edema)?? Allergic alveolitis ?? Renal failure or Re-expansion or negative pressure pulmonary edema ?? Drugs (such as opiates [heroin, cocaine], aspirin, chemotherapeutic agents) ?? Inhaled toxins (ammonia, chlorine, smoke inhalation)?? Aspiration or Acute respiratory distress syndrome or Altitude sickness ?? ContusionIntravenous furosemide may decrease lung water and improve oxygenation for the girl described in the vignette. Because her blood pressure and perfusion are normal, inotropic support is not indicated. ?Intravenous clindamycin may be considered for aspiration pneumonia, but the acute onset of bilateral airspace disease is more suggestive of NCPE. Although ceftriaxone may be indicated for the treatment of pneumonia, this diagnosis is unlikely in this patient. Flumazenil is a reversal agent for benzodiazepines but never should be administered for an unknown overdose. Given the short half-life of naloxone, repeat doses should be considered in the presence of respiratory depression or depressed mentation with pinpoint pupils.??Pulmonary edema is defined as an abnormal accumulation of fluid in the extravascular compartments of the lung. Three mechanisms lead to pulmonary edema:Increased hydrostatic gradient??Diminished oncotic pressure?Increased capillary permeability due to endothelial damageThe causes of pulmonary edema generally are categorized as cardiac or noncardiac. Reduced cardiac function causes increased hydrostatic pressures in the pulmonary capillary bed, resulting in cardiogenic pulmonary edema. NCPE can develop from volume overload due to renal failure, diminished oncotic pressure in a patient who has liver failure, or endothelial injury, as in a patient suffering from a heroin overdose. The chest film may play a role in distinguishing cardiac versus noncardiac forms of pulmonary edema.??Cardiogenic pulmonary edema usually is characterized by cardiac enlargement, pleural effusions, pulmonary vascular redistribution to the upper lobes, Kerley B lines, and peribronchial cuffing. NCPE causes diffuse airspace opacities that are not central in distribution without pleural effusions. The heart size typically is normal. In addition, the clinical scenario generally is substantially different in patients who have cardiogenic versus NCPE. ??Ancillary tests such as assessment of brain natriuretic peptide (BNP) may be helpful. Values of less than 500 pg/dL suggest a diagnosis of cardiogenic pulmonary edema; concentrations less than 100 pg/dL suggest NCPE. These ranges account for the variability of the tests and existence of an indeterminate zone. Elevated values may be observed in other conditions not related to acute heart failure, such as pulmonary thromboembolism and renal insufficiency. Other baseline tests in patients who have “flash” pulmonary edema should include complete blood count to rule out significant anemia, electrolyte assessment, electrocardiography to exclude ischemia, measurement of cardiac markers, and toxicologic screening.??Patients who have acute pulmonary edema and present in extremis, with imminent respiratory failure, must be treated with noninvasive ventilation or endotracheal intubation. Those who have evidence of end-organ hypoperfusion and hypotension (shock) require judicious fluid boluses and intravenous inotropes such as milrinone, dobutamine, and dopamine, along with invasive hemodynamic monitoring??American Board of Pediatrics Content Specification(s):??Recognize signs and symptoms of pulmonary edema??Know the appropriate ancillary studies relevant to pulmonary edema??Plan management of acute pulmonary edema, including the life-threatening complicationsQuestion 6You are receiving a 27 weeks’ gestation, 750-g newborn from a small emergency department (ED). The referring facility’s ED physician endotracheally intubated the infant and a nurse established peripheral venous access. However, that intravenous line infiltrated, and attempts at establishing peripheral venous access elsewhere were unsuccessful. The ED physician successfully inserted a 3.5-French umbilical venous catheter (UVC) and arranged transfer for the infant. The infant is being ventilated via bag-valve-mask and has maintenance intravenous fluids infusing through the catheter. When asked how he determined the proper depth of insertion for the umbilical venous catheter, the ED physician stated he had used an estimation formula. You order a film to confirm proper placement of the UVC.Of the following, the BEST position for the UVC isA.at the level of the ductus venosusB.in the pelvisC.just above the diaphragmD.within the liverE.within the right atriumCorrect answer: CThe correct position of the UVC is just above the diaphragm or, in acute resuscitations, just proximal to the umbilical stump. A variety of formulas and graphs are available to help estimate the initial optimal position of both an umbilical artery catheter (UAC) and a UVC. These methods rely on knowledge of the infant’s birthweight, overall length (cm), or the shoulder-to-umbilicus length measurement.??Umbilical vessel catheterization is a critical procedural skill to obtain emergent vascular access in neonates, especially preterm infants. The indications for umbilical vessel catheterization can be categorized by the type of vessel catheterized (Table 1).Table 1: Indications for Umbilical CatheterizationUmbilical Vein Catheter (UVC):?????????????Gaining vascular access for emergency resuscitation (preferred route for administration of resuscitation medications and fluids) Central venous pressure monitoringVenous blood gas and other blood sample monitoring? Exchange transfusionsAdministration of blood or blood products?Umbilical Artery Catheter (UAC):? ??Gaining vascular access for emergency resuscitation, although umbilical vein catheterization is technically easier to performContinuous blood pressure monitoringArterial blood gas and other blood sample monitoring Contraindications for umbilical vessel catheterization include omphalitis, omphalocele, peritonitis, and necrotizing enterocolitis. Relative contraindications are malformations that distort vascular anatomy; umbilical cord anomalies; bleeding or prothrombotic disorders; or evidence of vascular compromise to the kidneys, buttocks, or lower extremities.??Several anatomic and pathophysiologic details are important to consider when undertaking umbilical vessel catheterization:There are usually two umbilical arteries and one vein; two-vessel cords are rareThe vein is usually in the 11 or 12 o’clock position in the cord, but that position can vary, depending on where the cord is cutUmbilical arteries tend to be smaller in diameter compared with the umbilical veinUmbilical arteries have thicker, more muscular vessel walls compared with the umbilical veinThe umbilical vein may continue to bleed slowly after the cord is cut; the arteries are less likely to bleed because of vasospasmUmbilical vessel catheterization is a sterile procedure, and the initial steps are the same for both umbilical artery and vein (Table 2). Differences in the steps for catheterizing the umbilical artery and vein include the technique for dilating each vessel, catheter advancement, some potential pitfalls, and determination of the proper placement. An umbilical artery is technically more difficult to cannulate because it is smaller, thicker-walled, and usually vasoconstricted compared to the umbilical vein. The umbilical vein needs to be cannulated only to the minimum distance that establishes adequate blood return (usually approximately 2 to 4 cm past the skin) in the setting of cardiopulmonary arrest.Table 2: Umbilical Vessel Catheterization Initial Procedure:1.??? Establish a sterile field, including the umbilical stump and surrounding abdomen, using a bactericidal solution and sterile drapes/towels.2.??? Place a purse-string suture, a cord tie, or umbilical tape around the base of the umbilical stump to provide necessary hemostasis and an anchor point for the catheter after proper positioning.3.??? Attach stopcocks/syringes to the flushed and filled catheters.4.??? Cut the cord horizontally using a scalpel, leaving at least 1 to 2 cm of remaining cord from the skin.5.??? Identify the vessel to be cannulated.??Cannulation of the Umbilical Artery:??1.??? Introduce small curved hemostats/forceps or iris forceps carefully into the lumen of the artery, initially using one point of the instruments, then both points, to dilate the vessel gently.2.??? Repeat the technique as necessary to dilate the artery adequately.3.??? Once the lumen appears sufficiently dilated to accept the catheter, hold it about 1 cm from the tip and slowly introduce it into the lumen.4.??? Slowly advance the catheter to about a depth of 2 cm; do not force the catheter because this may create a false tract.5.??? If there is resistance, loosen the umbilical tie or apply gentle traction to the umbilical cord toward the infant’s head while advancing the catheter.?6.??? After advancement of about 5 cm, check for easy blood aspiration and pulsatile arterial flow, which generally indicates the catheter is still within the vessel. If this is not seen, the catheter likely is in a false track outside of the vessel. Remove the catheter and attempt cannulation of the other artery.7.??? Advance the umbilical artery catheter to the predetermined depth once it appears to still be in the vessel.8.??? Debate exists over the proper positioning of the UAC, using either the “low” or “high” position.?????? The UAC catheter tip lies at the level of the 3rd and 4th lumbar vertebrae for the low position?????? The catheter tip is above the diaphragm at the level between the 6th and 9th thoracic vertebrae for the high position. A 2000 Cochrane Review suggested that the high position is the preferred placement because it is associated with fewer clinical vascular complications and longer duration of service.9.??? Several methods can be used to help estimate the proper insertion depth for the UAC:?????? The shoulder-to-umbilicus length and appropriate reference graph?????? The infant’s birthweight and the following formula:???????????????????UAC distance (cm) = 3 x birthweight (kg) + 9?10. Verify catheter position with plain radiographs: The UAC course should start at the umbilicus and travel inferiorly to join the internal iliac artery, then curve cephalad to connect with the aorta, along the left of the vertebral column, above the diaphragm, with the catheter tip between T6 and T9. ??Cannulation of the Umbilical Vein:1.??? Dilate the lumen of the vein with small curved hemostats/forceps or iris forceps; occasionally, clots may need to be removed.2.??? Introduce the catheter into the lumen and advance to the predetermined depth, confirming patency with good blood return.3.??? If advancing the UVC through the ductus venosus becomes difficult, pull the catheter back about 4 to 5 cm, rotate it clockwise, and re-advance it.??4.??? A number of methods can be used to estimate the proper insertion depth for the UVC:??????? The umbilical stump-to-xiphoid process distance?????? The shoulder-to-umbilicus length and appropriate reference graph?????? One half of the UAC length + 1 cm?????? The infant’s birthweight and the following formulas:???????????? UVC distance (cm) = [3 x birthweight (kg) + 9]/2 + 1???????? ??????? UVC distance (cm) = 1.5 x birthweight (kg) + 5.5????5.??? Verify proper placement by plain radiographs: The tip of the UVC should be in the inferior vena cava, above the level of the ductus venosus and hepatic veins (above the diaphragm), below the level of the right atrium, with the catheter tip between T8-T9.UVCs should not be advanced or frequently manipulated once secured in place to avoid catheter-related bloodstream infections. Blood loss also can occur due to vessel perforation, umbilical cord site bleeding, or separation of the catheter at any connection point in the plications specific to the umbilical blood vessel are usually the result of a malpositioned catheter or thromboembolic complications.Problems with UVCs can be categorized as gastrointestinal (hepatic abscess, hepatic necrosis, portal venous thrombosis, necrotizing enterocolitis, colon perforation), air or catheter tip emboli, or cardiac (dysrhythmias, pericardial effusion/tamponade, thrombotic endocarditis, hemorrhagic pulmonary infarction).Vascular compromise, including vasospasm and thromboembolic effects to particular organs or extremities, is one of the most frequent complications of UACs. Clinical signs depend on the organ system or extremity involved: renal (renal failure, hematuria, oliguria, anuria), cardiovascular (congestive heart failure, hypertension, narrow pulse pressure), extremity (limb color changes or blanching, mottling, tissue necrosis), musculoskeletal (decreased or absent lower extremity movement), or metabolic (hypoglycemia, metabolic acidosis).American Board of Pediatrics Content Specification(s):Discuss the indications and contraindications for umbilical vessel catheterizationDescribe the key steps and potential pitfalls in performing umbilical vessel catheterizationDiscuss the complications associated with umbilical vessel catheterizationKnow the anatomy and pathophysiology relevant to umbilical vessel catheterizationQuestion 7A 3-year-old boy presents to the emergency department with a fever and decreased activity for 12 hours. Physical examination of the lethargic boy, who is poorly responsive to voice, reveals a temperature of 39.9?C, heart rate of 187 beats/min, respiratory rate of 32 breaths/min, blood pressure of 82/36 mm Hg, and pulse oximetry reading of 91%. After rapid evaluation, you determine he is in shock and potentially septic. His airway is intact, he is breathing mildly fast, and his central capillary refill is 4 seconds. You place an intravenous line, draw specimens for laboratory studies, and start a 20-mL/kg normal saline bolus. Because it appears that the boy will require a level of care not available at your facility, you plan to transfer him to one of the local regional pediatric centers, which are located 17, 22, and 35 miles away. Of the following, the MOST appropriate sequence of events for such transfer is toA.call 911 and arrange for immediate advanced life support transport to the closest tertiary care facilityB.call each center, identify which can respond to your request the fastest, and engage that service while continuing evaluation and stabilization of the patientC.continue evaluation and treatment until all radiographic and laboratory data are available, then call the local pediatric intensive care unit for further advice and arrange transportD.notify the local helicopter company and request immediate critical care transportE.notify the predetermined tertiary care center, request advice regarding stabilization and management from a medical representative, and, with their guidance, determine and arrange appropriate transport Correct answer EThe initial step in transfer of an ill or injured patient is to care for and stabilize him or her as much as possible. Transfer of an unstable patient who can be stabilized at the initial site of care is a direct violation of the Emergency Medical Treatment and Labor Act (EMTALA). However, rapid recognition of the need for a higher level of care or resources not available at the initial hospital is critical. Once the clinician recognizes that the ability to care for the patient exceeds the facility’s capability, a definitive care site should be contacted for further advice and arrangement for transport at the first opportunity. ?Ideally, the ultimate site of care is an institution whose personnel are well versed and experienced in the care of critically ill children. It is preferable that referral guidelines include communication with a predetermined pediatric tertiary care center so that a single call from the referring hospital can initiate a standard protocol for specialized consultation and transport. This allows an organized approach to care that spans initial management through transport to the intensive care unit. Clinicians should avoid placing the child in less experienced hands at any point of the transfer process. Placing the patient in the care of a local 911 emergency medical technician or paramedic service may not provide the highest level of care available for the critically ill child described in the vignette, placing both the child and the providers at risk. It is likely that pediatric tertiary care centers in close proximity will have established pediatric critical care transport systems.? One aspect of transfer that often is overlooked is the value of communication with the pediatric transport center team and especially a medical command physician. Most sophisticated transport systems have pediatric experts immediately available for discussion regarding the patient. A concise summary of patient presentation, existing or confounding issues, interventions, and responses to those interventions enables the receiving team to visualize the patient and prepare for continued or additional care. If possible, the calls to the receiving center should be through an established transport communication center to enable the transport team (care team members and transport personnel) to be aware of the existing need and prepare while the medical discussions are ongoing. The transport team should be experienced and skilled in critical care pediatrics; outcomes are improved when transport is accomplished by a specialty (pediatric) compared with general (nonpediatric-specific) transport team.Once the transport has been arranged, care should continue to the best of the clinician’s and institution's abilities. If suggestions are made with which the referring team is uncomfortable or does not agree, they should be discussed with the transport/receiving team to offer them an opportunity to identify other options and have a real-time awareness of the level of illness and potential need for transport team intervention at the referring site. Ideally, all required interventions, stabilization, and preparations should be made prior to transport team arrival, using guidelines and best practices where possible. Time spent by the transport team placing or securing clinical adjuncts (eg, lines, airways) or copying medical records/radiographic materials may be critical to the patient receiving definitive care at the receiving center. Clinical assessment and intervention for the patient should not stop when the transport team is notified and has accepted the patient. Specific noncritical procedures, however, may be postponed as long as treatment is appropriate and needed studies are planned for at the receiving center. For example, lumbar puncture (LP) need not be performed for the septic-appearing child in the vignette before transport if such a procedure would delay the transport process. Treatment with appropriate antibiotics can be started and the LP performed at the receiving hospital. The initial care team should be visible and available to the transport team when they arrive to help ensure a seamless transition of care and appropriate medical handoff. It is important to realize that most transport teams do not have medical or nursing privileges at the referring hospital and are working as consultants to the initial team, so referring clinician presence and collegial care is imperative. American Board of Pediatrics Content Specification(s):??Know indications and procedures for transport to a higher level facilityQuestion 8A 4-year-old boy who recently emigrated from Senegal presents to the emergency department with a history of fever, lethargy, tachypnea, and poor perfusion. On physical examination, his temperature is 39.5?C, respiratory rate is 43 breaths/min, heart rate is 182 beats/min, blood pressure is 92/45 mm Hg, pulse oximetry reading is 88% in room air, and capillary refill is 3 seconds. His airway is intact. He is arousable and oriented with no signs of meningitis, although he does appear ill.Of the following, the signs and symptoms are MOST suggestive ofA.carbamate toxicityB.cocaine toxicityC.filariasisD.sickle cell diseaseE.thalassemia minor Correct answer DThe constellation of symptoms reported for the boy described in the vignette should lead to consideration of acute chest syndrome and the infectious complications associated with sickle cell disease. Immediate care involves oxygen administration, intravenous (IV) access, laboratory assessment (including complete blood count, blood cultures, chemistry and renal function tests, and a blood gas analysis), and chest radiography. Antibiotics should be administered, as should IV fluids to address shock and dehydration. Pain medication and bronchodilators may be required. Admission to the hospital, preferably a pediatric intensive care unit, and involvement of hematology specialists would be prudent. Assistance of airway support and ventilation may need to be addressed if the boy does not improve or deteriorates, and transfusion may need to be considered to replace sickled cells with normal red blood cells.?HYPERLINK "javascript:showModal('/subscriber/modal/july/july-q8-content-specifications')"????Filariasis is common in West Africa and usually presents with adenopathy and intense pruritus; overwhelming sepsis is not a common complication. Cocaine intoxication is unlikely for this child due to his normal blood pressure and sensorium. Having thalassemia minor does not increase the risk of medical complications, and thalassemia has a lower prevalence than sickle cell disease on the African continent. Carbamate toxicity is likely to present with cholinergic symptoms (salivation, lacrimation, urination, defecation, gastrointestinal cramping, emesis, miosis, and muscle fasciculations), none of which are present in this boy.??Sickle cell disease includes a number of potential disorders, from homozygous SS disease to heterozygous conditions such as hemoglobin SC, SD, SO, S/beta-thalassemia, and S/fetal hemoglobin. Sickle cell anemia (HbSS) is the most common, with HbS/beta-thalassemia having similar complications but less severe anemia.??Complications of sickle cell disease and the variants can be acute or chronic and include:AnemiaAcute febrile illness and infection (most common primary cause of death in children who have SSD), often with encapsulated organisms (eg, pneumococcus. Salmonella)Splenic sequestrationAcute chest syndrome (infection, fat emboli, infarction)Vaso-occlusive crisis (painful crisis)StrokeEye complicationsTransient aplastic crisis (parvovirus infection)PriapismAmong the more chronic complications are:Growth disturbancesAvascular necrosis (hip)CholelithiasisHepatic infarctionTransfusion-related hepatitisLearning difficultiesSkin ulcerationsRepeated priapismGlomerular or tubular renal disease (hematuria)EnuresisCardiomegalyLeft ventricular hypertrophyPulmonary hypertensionManagement for such potentially life-threatening complications should be determined in conjunction with experts and awareness of national guidelines. Anemia is evident in patients who have homozygous disease (after the first few postnatal months, when the protective fetal hemoglobin has disappeared), increasing from approximately 6% of 6-month-old infants to 96% of patients by the time they reach 8 years of age. Young children often present with dactylitis (swollen hands), vaso-occlusive episode (painful crisis), splenic sequestration (before splenic fibrosis due to multiple episodes of splenic infarction noted in older children), or acute chest syndrome. Meningitis is seen more often in infants and young children. Older children may demonstrate cerebrovascular accident, priapism, or splenic dysfunction due to infarction. ??In the United States, sickle trait is evident in approximately 8% of African Americans and 0.05% of Caucasians. Within the various expressions of sickle trait are variable amounts of hemoglobin S. Although hemoglobin values and life spans are similar in patients with and without sickle trait, complications still can lead to significant morbidity and mortality. Reported complications in children who have sickle cell trait ?include renal medullary carcinoma, hematuria, hyposthenuria (loss of urinary concentrating ability), renal papillary necrosis, splenic infarction, rhabdomyolysis, and unexpected exercise-related sudden death. There may be a relationship as well with venous thromboembolic events, pregnancy-related complications, hyphema, retinopathy, acute chest syndrome, and asymptomatic bacteriuria.?American Board of Pediatrics Content Specification(s):??Recognize acute complications of sickle hemoglobin disorders by age??Recognize life?threatening complications of sickle hemoglobin disordersAUGUST 2010Question 1A 2-year-old child presents to the emergency department with a 7-hour history of rash. Her mother states that the toddler has had a runny nose and congestion for the past 2 days, and this evening she broke out in the pruritic rash (Figure).FigureShe applied 0.5% hydrocortisone cream, which allowed the girl to sleep, but she awoke several hours later and has been unable to fall back to sleep. There is no history of ingestion of new foods, use of new detergents, or wearing of new clothing. On physical examination, the uncomfortable child is continuously scratching, the rash covers her entire body and blanches with pressure, and she has crusty rhinorrhea. Her temperature is 38.0°C, heart rate is 120 beats/min, respiratory rate is 22 breaths/min, and blood pressure is 90/50 mm Hg. She is not in any respiratory distress and is not drooling. Her lungs are clear, findings on cardiac examination are normal, and she has good perfusion.Of the following, the MOST appropriate treatment for this girl isA.epinephrine intramuscularly in the lateral thighB.epinephrine subcutaneously in the lateral thighC.hydroxyzine hydrochloride and prednisone orallyD.hydroxyzine hydrochloride orallyE.prednisone orally Correct answer DThe child described in the vignette has acute urticaria, probably due to a viral upper respiratory tract illness. The treatment of urticaria associated with viral infection is purely symptomatic. The mainstay of treatment is histamine-1 blockers to mitigate the release of histamine and treat the resulting profound pruritus. Sedating antihistamines can be helpful when pruritus prevents the child from sleeping, such as described for this girl. Diphenhydramine and hydroxyzine hydrochloride belong to this class. Children often experience a better symptomatic response from hydroxyzine, which is why many allergists recommend this as first-line treatment for uncomplicated urticaria. Nonsedating histamine-1-blocking antihistamines (loratadine, cetirizine, and fexofenadine) are helpful with management of daytime pruritus and are administered on a 12- to 24-hour schedule. The combination of a nonsedating antihistamine in the morning with a sedating antihistamine at bedtime may optimize therapeutic response, although there is a paucity of comparative data on this approach.The role of corticosteroids in acute uncomplicated urticaria is controversial. Most authors do not recommend their use unless more severe symptoms, such as angioedema of the face, mouth, and tongue, occur or the patient’s symptoms are uncontrolled with a combination of antihistamines. Although epinephrine can improve the urticaria and may help differentiate urticaria from erythema multiforme in unclear cases, it is not necessary for a child who has no associated respiratory distress, angioedema, or cardiovascular or neurologic complaints that suggest anaphylaxis.Urticaria is usually a self-limited reaction to an allergen.? In an immunoglobulin (Ig)E-mediated reaction, allergen activates mast cells, which release histamine, causing the appearance of transient, pruritic, erythematous, raised wheals with flat tops in the epidermis. Such wheals may coalesce to form large anular or serpiginous lesions. Angioedema occurs in the deeper subcutaneous tissues of the eyelids, lips, tongue, genitals, and dorsum of the hands and feet and frequently is painful rather than pruritic. Foods, medications, latex, insect stings, and blood product administration can trigger IgE-mediated urticaria.Urticaria also can result from non-IgE-mediated stimulation of mast cells. Such a reaction occurs with radiocontrast agents, opiates, nonsteroidal anti-inflammatory agents, viral agents (eg, hepatitis B and Epstein Barr virus) and bacterial pathogens (eg, Streptococcus and Mycoplasma). A summary of common causes of urticaria in children is listed in the Table.Table: Common Causes of UrticariaInfectious Agents Associated With Transient Urticaria? Streptococcus? Infectious mononucleosis (Epstein-Barr virus)? Hepatitis A, B, and C? Adenovirus? Enterovirus? Parasites?Bites and Stings Associated With Transient Common Urticaria? Bees? Wasps? Scorpions? Spiders? Jellyfish?Drugs Associated With Transient Common Urticaria? Penicillin? Cephalosporins? Salicylates? Morphine, codeine, and other opiates? Nonsteroidal anti-inflammatory drugs? Barbiturates? Amphetamines? Atropine? Hydralazine? Insulin? Blood and blood products?Foods Associated With Transient Common Urticaria? Nuts? Eggs? Shellfish? Strawberries? Tomatoes?Systemic Diseases Associated With Transient Common UrticariaCollagen Vascular Disease? Lupus erythematosus? Juvenile rheumatoid arthritis? Polyarteritis nodosa? Dermatomyositis? Neonatal lupus syndrome? Sj?gren syndrome? Rheumatic feverInflammatory Bowel Disease? Crohn disease? Ulcerative colitisMiscellaneous? Aphthous stomatitis? Beh?et disease? ThyroiditisPhysical urticaria can be induced by environmental factors such as change in temperature; direct stimulation of the skin with pressure, stroke, or vibration; or cold. Rarely, solar exposure can induce it in susceptible individuals. Dermatographism may occur in such physical urticarias.For children exhibiting urticaria in whom angioedema of the airway is suspected (enlarged tongue or uvula, drooling, stridor, dysphonia, or other symptoms suggestive of potential airway compromise), epinephrine administered intramuscularly in the lateral thigh is the immediate treatment of choice, in conjunction with histamine-1- and -2-blocking antihistamines and intravenous corticosteroids. Anaphylaxis, rather than uncomplicated urticaria, should be considered in children who have associated vomiting, dizziness, hypotension, poor perfusion, altered mental status, syncope, or subjective or objective complaints of respiratory distress. When in doubt, the child should be treated presumptively for anaphylaxis.The pruritic, raised wheals of urticaria are evanescent.? Individual wheals usually last fewer than 6 hours but may remain up to 24 hours in some cases. They may enlarge and coalesce as symptoms progress and can cover the entire body. Frequently, they are accentuated in areas of pressure, such as waist bands and sock lines. Parents often are frustrated that urticarial lesions re-emerge after the antihistaminic effect dissipates and may return to the emergency department unless provided with careful anticipatory guidance explaining the usual course of viral-associated urticaria (can last 1 to 2 weeks) and the symptomatic nature of treatment. If urticaria is caused by food, drug, or contact exposure, the offending agent should be removed. A careful history, as well as a lack of infectious symptoms, often identifies the underlying cause in these cases. In many cases, the offending agent is not identified, but urticaria resolves.Laboratory investigation is not necessary in acute urticaria, unless indicated for a specific suspected underlying cause such as hepatitis or parasitic infection. Even in cases of chronic urticaria, laboratory tests frequently are not revealing and should not be undertaken in the emergency department. When urticaria is accompanied by other systemic symptoms, such as arthritis or arthralgias or ill appearance, a diagnostic evaluation may be warranted. In addition, individual lesions that remain for more than 24 hours or lesions that burn more than itch, do not blanch, or are associated with purpura suggest urticarial vasculitis. Skin biopsy and further systemic evaluation is indicated in these cases.American Board of Pediatrics Content Specification(s):Know the common etiologies of urticariaRecognize signs and symptoms of urticariaRecognize conditions associated with urticaria that may be life-threateningPlan management of acute urticariaKnow the etiology by age and pathophysiology of urticaria rashesPlan diagnostic evaluation and initial intervention for patients with urticarial rashesRecognize serious and/or life-threatening causes of urticarial rashesQuestion 2A 9-year-old previously healthy girl presents to the emergency department because of rectal bleeding and a possible mass. Her mother states that she saw bright red blood in the toilet bowl after a bowel movement, and when doing the laundry today, found blood staining on the girl’s underpants. She initially thought her daughter was beginning her menstrual cycle, but when she looked at her vaginal and perianal area this afternoon, she saw a dark red mass that she believed was protruding from the girl’s rectum. On physical examination, you do not see any mass, but you do note blood staining her underpants. Her rectal tone seems normal, and a fecal occult blood test performed after digital rectal examination is positive for blood. Her genitalia are at Sexual Maturity Rating 1, and her external urethral and vaginal areas look grossly normal. On further questioning, the girl admits to seeing bright red blood in the toilet bowl after bowel movements on many occasions in the last month but states that there was never anything “sticking out of me” until today. She denies abdominal pain, cramping, vomiting, diarrhea, constipation, or weight loss.Of the following, the MOST likely explanation for the girl’s symptomsA.ileocecal intussusception prolapsed through the anusB.internal hemorrhoidC.prolapsed rectal polypD.rectal prolapseE.urethral prolapseCorrect answer: CThe girl described in the vignette has painless, bright red rectal bleeding in conjunction with or following bowel movements as well as a history of what may be prolapse of a perivaginal or perianal structure. The blood on rectal examination and the mother’s description suggest that tissue prolapsed through the rectum. This combination of findings strongly suggests acute prolapse of a juvenile colonic polyp through the rectum followed by spontaneous reduction.The girl has no other risk factors for rectal prolapse, such as repeated episodes of increased intra-abdominal pressure (chronic constipation), acute or chronic diarrhea, parasitic infection, cystic fibrosis, or conditions predisposing to pelvic floor weakness, such as myelomeningocele. In addition, most rectal prolapse occurs in children younger than 4 years of age; an initial presentation at age 9 is highly unusual. Rarely, an ileocecal intussusception prolapses through the rectum, but it is less likely to reduce spontaneously. Polyps can act as lead points for intussusception, although the absence of pain and cramping makes intussusception extremely unlikely. A patient who has a prolapsed urethra should not have blood in the rectal vault, and spontaneous resolution of urethral prolapse is uncommon. Internal hemorrhoids are very rare in a child of this age and do not cause prolapse.Juvenile colonic polyps (also termed retention or inflammatory polyps) are erythematous, friable, pedunculated masses ranging in size from a few millimeters to 3 cm (Figure).Histologically, they are characterized by benign hamartomatous proliferation of mucus-filled glandular and stromal infiltrates. They are the most common type of childhood bowel tumor, with a peak incidence at 2 to 10 years of age and rare occurrences before the ages of 1 year or after 15 years. They usually present as solitary lesions, although two or more may occur in some children. In the absence of adenomatous changes on biopsy, such lesions are considered to have no malignant potential. The usual presentation is intermittent bright red, painless rectal bleeding accompanying or immediately following defecation. Bleeding usually stops spontaneously, and exsanguinating hemorrhage is rare. Iron deficiency anemia, although relatively common (30%), rarely is the chief complaint. Spontaneous infarction with self-amputation is common. Most polyps are located within 25 cm of the anus and, therefore, may be detected on digital rectal examination or barium enema. If prolapsed through the rectum at the time of examination, the examiner should manually reduce it with gentle pressure via a well-lubricated, gloved hand. Follow-up with a gastroenterologist for diagnosis by colonoscopy, with removal for pathologic confirmation, is required. Juvenile polyps occasionally recur.Other conditions associated with painless rectal bleeding include juvenile polyposis (JP), an autosomal dominant disorder of multiple gastrointestinal tract polyps that sometimes is associated with other congenital anomalies. Twenty percent of cases result from sporadic mutation. In general, children who have JP have an increased risk of gastrointestinal malignancy, although the risk is low if two or fewer polyps are present and there is no family history of JP. Three or more polyps or a family history significantly increases the lifetime risk of malignancy. Every-other-year whole gastrointestinal tract screening with upper endoscopy, colonoscopy, and upper gastrointestinal tract contrast radiography and small bowel follow-through is recommended in such patients. Inactivating mutations causing interruption of cellular signaling of transforming growth factor-beta have been found in some afflicted families. Rarely, the entire gastrointestinal tract is involved; affected patients may present with failure to thrive, malabsorption, anemia, and hypoalbuminemia.Peutz-Jeghers syndrome is another rare autosomal dominant condition characterized by mucosal pigmentation of the lips and gums (deeply pigmented freckled lips) and hamartomas of the gastrointestinal tract. It has an incidence of 1 in 120,000; 50% of individuals have no affected family member. Extensive intestinal involvement is the rule, and disease is so widespread that removal of all hamartomas is not practical.? Most gastrointestinal polyps occur in the jejunum and ileum, with fewer in the rectum, colon, stomach, and duodenum. Recurrent episodes of intussusception are common, especially involving the small bowel. Episodes of painful cramping suggest obstruction from small bowel intussusception. Patients who have Peutz-Jeghers syndrome have a 50% lifetime risk of gastrointestinal malignancy.Other rare disorders of polyposis include Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome, both of which are autosomal dominant disorders characterized by hamartomas of the skin, breast, thyroid, endometrium, brain, and gastrointestinal tract. The development of the hamartomas is caused by mutations in tumor suppressor genes.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of polypsPlan management of prolapsed rectal polypsQuestion 3A 2-year-old child is brought to the emergency department after sustaining a burn (Fig. 1).Figure 1: Burn sustained by the child.His mother states that he grabbed a hot curling iron from the bathroom counter when her back was turned. The child has no other visible injuries and is crying vigorously. You suspect nonaccidental injury because of the location of the burn on the dorsal surface of the hand and call social work to see the child and interview the family. The social worker states that before she calls the Department of Family Protective Services, she needs to know how severe the burn is, what percent total body surface area (TBSA) has been burned, and what the child’s disposition will be.Of the following, the BEST description of this child’s burn and most likely disposition isA.deep partial-thickness, >5% TBSA, and transfer to burn centerB.deep partial-thickness, <5% TBSA, and admit to inpatient wardC.full-thickness, 5% TBSA, and transfer to burn centerD.superficial, 5% TBSA, and schedule outpatient follow-upE.superficial partial-thickness, <5% TBSA, and schedule outpatient follow-up Correct answer BThe burn sustained by the child described in the vignette is predominantly white, but there is some area of redness superiorly. It is difficult to discern by inspection whether this is a deep partial-thickness or full-thickness burn. The presence of pain makes it more consistent with a deep partial-thickness burn. Burns are classified into superficial, superficial partial-thickness, deep partial-thickness, and full-thickness according to the depth of injury within the dermis and epidermis (Table 1). Assessing the full extent of burn depth within the first 24 hours may be difficult; the burn ultimately may prove deeper than originally thought.Table 1: Burn Classification and CharacteristicsBurn ClassificationTissue InvolvedCharacteristicsHealing Time and OutcomeSuperficialEpidermis onlyPainful, dry, red, blanches with pressure3 to 6 days No scarSuperficial partial-thicknessEpidermis and superficial dermisPainful, red, weeping, usually forms blisters, blanches7 to 21 days Scarring unusualMay have pigment changeDeep partial-thicknessEpidermis and deeper dermisLess painful, always blisters, wet or waxy-dry, variable color of patchy white to red, no blanching>21 daysSevere scarring Full-thicknessDermis destroyedPainless; waxy-white, leathery gray, or charred; dry; inelastic skin; no blanchingNo spontaneous healingSkin grafting necessarySevere scarringDetermining the percentage of TBSA burned in a child differs from measurement in an adult. The “Rule of Nines” must be modified to account for the greater surface area represented in a child’s head and lower surface area of the lower extremities. Figure 2 shows a representative bodygram for percent TBSA in children of various ages compared with adults.Figure 2: The “Rule of Nines” is used to determine total body surface area (TBSA) of injury. Only areas of partial- and full-thickness injury are included in the estimate. Compared with an adult, the head of a child is relatively larger in percent surface area, and the lower extremities are relatively smallerThe size of the child’s palm roughly approximates 1% TBSA. Using this rule, the child in the vignette has a burn of less than 5% TBSA.The decision about whether to treat this child as an outpatient or an inpatient is controversial. By official American Burn Association criteria (Table 2), this patient could be treated as an outpatient. He does not need to be admitted for fluid resuscitation because of the small size of his burn, but the emergency department physician may choose to admit him for pain control, close observation, and dressing changes. The social situation alone may warrant admission.Table 2: Recommended Categorization and Disposition per the American Burn AssociationBurn Category% TBSA1% Full-ThicknessOtherPotential FeaturesDispositionMinor<5% in young child2?<10% if older child or adolescent3<2%?Outpatient follow-upModerate5% to 10% in young child?10% to 20% if older child or adolescent?2% to 5%High-voltage injurySuspected inhalation injuryCircumferential burnUnderlying illness4Inpatient managementMajor>10% in young child?>20% if older child or adolescent>5%High-voltage burnKnown inhalation injurySignificant burn to face, eyes, ears, genitalia, or jointBurn center referral1TBSA = Total Body Surface Area?2Young child of ≤10 years old3Older child of >10 years old4Sickle Cell Disease or DiabetesWounds that cross joint lines are at high risk for impaired functional outcome due to scarring. The wound needs to be cleansed with a mild soap and water solution. Further treatment varies by center. One option is to apply a topical antibiotic cream such as silver sulfadiazine (if no sulfa allergy) and cover with a nonadherent sterile dressing. For burns to the face, bacitracin or bacitracin/neomycin/polymyxin B is preferred because silver sulfadiazine can cause staining of the facial epithelium. Alternatively, a silver-impregnated dressing of carboxymethylcellulose or rayon/polyester fibers may be used. This obviates the need for daily dressing changes. When tissue is burned, the thermal energy denatures protein and causes coagulation, resulting in irreversible tissue destruction.? Immediately surrounding the zone of coagulation is an area of decreased tissue perfusion. Tissue in this zone has the potential for salvage if resuscitation is adequate to restore perfusion. The outer margins of the burn have increased perfusion, and tissue recovery is likely unless there is a prolonged period of hypoperfusion. The depth of the wound is determined by the intensity and duration of thermal exposure. Young children’s thinner skin is more susceptible to deeper burns. Vasoactive mediators, such as cytokines, prostaglandins, and oxygen radicals, are released when tissue is burned. The subsequent increase in capillary permeability leads to extravasation of fluid locally. When large amounts of skin are burned (>15% TBSA in young children, >20% TBSA in older children and adolescents), such capillary leak occurs systemically. If more than 40% of TBSA is burned, myocardial depression may ensue. Capillary leak persists for 18 to 24 hours. A hypermetabolic state with increased caloric expenditure and protein metabolism is mediated by catecholamine, glucagon, and cortisol release.Initial rapid assessment with attention to the airway, breathing, and circulation is indicated with any major burn. Children who have flame burns should be evaluated for smoke inhalation or carbon monoxide exposure. Chemical burns require aggressive irrigation. Fluid resuscitation is critically important for children who have burns greater than 10% TBSA or any child who presents with circulatory compromise. Intravenous access should be obtained, preferably through nonburned skin.Fluid resuscitation should be calculated based on TBSA of burn. Most individuals still use the Parkland Formula, which is weight-based. Resuscitation should consist of 4 mL/kg per percent TBSA. Half the fluid should be administered in the first 8 hours; the remainder should be administered over the following 16 hours. Maintenance fluid volume should be added for children younger than 5 years. Lactated Ringer solution, with 5% dextrose added for younger children, is the fluid of choice. There is no evidence of improved outcomes when colloid is administered. Urine output should be monitored by Foley catheter in moderate-to-severe burns, and intravenous fluid therapy should be titrated to maintain a urine output of 1 to 2 mL/kg per hour for children who weigh less than 30 kg and 0.5 to 1 mL/kg per hour for heavier children. Serum glucose should be monitored and fluids titrated or insulin administered to achieve euglycemia. Narcotic pain control usually is required and should be administered promptly and consistently.Debridement of devitalized tissue is recommended and is believed to decrease the rate of infection. Whether to lance intact blisters is controversial, but many experts recommend debridement for large blisters, ones likely to rupture, and those that are painful. After cleansing, a sterile dressing should be applied as outlined previously. Systemic prophylactic antibiotics are not indicated, even in large burns, but the patient must be observed closely for signs and symptoms of secondary infection and antibiotics begun promptly if there are any changes in condition.Circumferential burns are particularly problematic and may cause compartment syndrome from constriction when located on an extremity or restriction of breathing when encircling the chest. Prompt escharotomy is the treatment of choice. Other complications include respiratory failure, sepsis, multiorgan system failure, and death.? Mortality has decreased with the advent of aggressive care for pediatric patients. Severe scarring and contractures remain problematic consequences of major burns.American Board of Pediatrics Content Specification(s):Describe the key steps and potential pitfalls of burn managementKnow the anatomy and pathophysiology relevant to burn managementDiscuss the indications and contraindications for burn managementDiscuss the complications associated with burn managementQuestion 4A 14-year-old boy is ejected from a car after the car strikes a tree. He is appropriately immobilized by prehospital providers, and upon arrival at the emergency department, he has normal mental status and vital signs. He has midline cervical spine tenderness. Careful secondary survey reveals no proprioception in his extremities but overall good strength.Of the following, the MOST likely injury to his cervical spinal cord isA.anterior segment plete transectionC.hemisectionD.posterior segment injuryE.transient concussion Correct answer DWhen treating children who have potential cervical spine trauma, it is important to recognize the various patterns of injuries. Complete transection (Fig. 1) results in immediate and complete loss of function below the level of transection.Figure 1: Complete spinal cord transectionBrown-Sequard syndrome (Fig. 2) results from hemisection of the cervical spinal cord that results in crossed sensory and motor deficit.?The? pattern of injury is characterized by loss of contralateral pain and temperature (spinothalamic tract) and loss of ipsilateral motor function (lateral corticospinal tract). Concussion of the cervical spinal cord refers to transient traumatic paralysis that may recover in hours or longer.Figure 2: Brown-Sequard syndromeInjury patterns also relate to the area of the spinal cord that is affected. Simplistically, the anterior cord encompasses motor tracts, the exit of ventral motor fibers, and pain and temperature tracts. The posterior compartment contains dorsal columns (pain and proprioception) and sensory fibers.Injury to the anterior compartment (Fig. 3) is more common, either through direct mass effect or disruption of the blood supply through the anterior spinal artery.Figure 3: Anterior cord syndromeThe pattern of injury involves loss of motor function and pain sensation below the level of injury but preservation of touch sensation and proprioception.Injuries to the central region (Fig. 4) result in upper extremity weakness greater than lower.Figure 4: Central cord syndromeThis is a consequence of the configuration of the lateral corticospinal tract where cervical motor fibers lie medially and the thoracic, lumbar, and sacral fibers lie progressively more laterally. Finally, injury to the posterior region (Fig. 5) is the least common, resulting predictably in loss of proprioception and pain sensation, as described for the boy in the vignette.Figure 5: Posterior cord syndromeAmerican Board of Pediatrics Content Specification(s):Recognize signs of cord injury syndromes (anterior, central, complete, posterior) in children with cervical spine injuriesQuestion 5A 6-year-old girl is brought to the emergency department because of concerns about neck swelling in her right neck that initially developed more than 1 month ago. She has been seen by her pediatrician on several occasions. She was treated initially with 10 days of amoxicillin-clavulanate without any improvement and has just finished a 2-week course of clindamycin. The swelling has continued to increase, and the skin in the area now is discolored. The child has not had any fever, vomiting, diarrhea, other rash, or any other symptoms. On physical examination, her vital signs, including her temperature, are within normal parameters. Examination of her head, ears, eyes, nose, and throat yields normal results. Neck examination shows a 6x4-cm firm, slightly tender mass in the right submandibular region. The skin overlying the mass is thin and has a red-purple discoloration.Of the following, the MOST appropriate next step in the evaluation of this patient is:A.continued conservative management with outpatient follow-upB.incision, drainage, and packing of the mass and discharge without antibioticsC.obtaining blood samples for measurement of Epstein-Barr virus and cytomegalovirus antibodyD.percutaneous aspiration of the mass and discharge with a prescription for cephalexinE.placement of a purified protein derivative skin test and surgical consultation Correct answer EThe child described in the vignette is experiencing chronic unilateral cervical lymphadenitis that is consistent with infection with nontuberculous mycobacteria (NTM). Definitive diagnosis and treatment are accomplished best with complete excision of the lesion to allow microscopic and microbiologic evaluation of the infected tissue. Excision is typically curative, although antibiotic therapy with azithromycin or clarithromycin in combination with ethambutol, rifabutin, or rifampin may be indicated in some cases. Purified protein derivative (PPD) skin tests are helpful to exclude tuberculosis as a cause; negative or weakly positive results are expected in NTM infection. The length of time and severity of this child’s symptoms argue against continued conservative management. Both incision and drainage and percutaneous aspiration are contraindicated in suspected NTM infection or tuberculous adenitis due to the risk of seeding the adjacent tissues and causing chronic sinus tract or fistula formation. Epstein-Barr virus and cytomegalovirus are frequent causes of febrile illnesses associated with regional or generalized lymphadenopathy in children. However, this child’s presentation of chronic unilateral lymphadenitis associated with overlying skin changes is not consistent with infection from either agent.Cervical lymphadenitis is defined as enlarged, tender, inflamed lymph nodes in the neck. It is extremely common in young children, with?most cases involving the submandibular or deep cervical chains that drain most of the head and neck region. Lymphadenitis results when microorganisms are transported to the involved lymph node(s) via lymphatic channels draining the respective anatomic area. The area drained by the involved node may or may not show signs of local infection. Lymphocyte proliferation as a result of antigen or infectious agent drainage causes lymph node enlargement; pyogenic organism drainage results in acute enlargement of the node associated with warmth, erythema, and tenderness. Neutrophil proliferation within the node may result in abscess formation. The number and location of involved nodes depends on the site, severity, and causative organism of the primary infection.The most common cause of cervical lymphadenitis is viral infections of the head and neck, with common pathogens including enterovirus, adenovirus, influenza viruses, and rhinovirus. Viral infections typically cause acute bilateral lymphadenitis, with mildly tender nodes that usually are 1 to 2 cm in diameter persisting for several weeks before resolution.Bacterial head and neck infections, including impetigo, pharyngitis, otitis, cellulitis of the face or scalp, and periodontal infections, also can progress to cervical lymphadenitis. Typical bacterial causes of cervical lymphadenitis are Streptococcus pyogenes and Staphylococcus aureus (>80% of cases). Group B Streptococcus is a frequent cause in neonates. Lymphadenitis as a result of dental infection may be caused by oral anaerobic bacteria. Bacterial infection most commonly is associated with acute unilateral lymphadenitis. Bacterial lymphadenitis may progress to suppuration, with formation of an abscess within the involved node.Cat-scratch disease results from infection by Bartonella henselae and typically presents with regional lymphadenitis proximal to the site of inoculation. There is often a history of exposure to kittens or dogs, with a scratch leading to the development of pustules or papules at the site. This is followed within days to weeks by the development of lymphadenitis. Fever, malaise, and other systemic symptoms are common. Less common causes of acute unilateral cervical lymphadenitis are Francisella tularensis, Brucella, and Actinomyces.Chronic lymphadenitis persists for weeks or months and can have infectious, inflammatory, and malignant causes. Infectious causes of chronic cervical lymphadenitis include tuberculosis, NTM infections (most commonly, M avium, M fortuitum, M abscessus, and M marinum), human immunodeficiency virus infection, and toxoplasmosis. Among the other causes are Kawasaki disease, collagen vascular disease, lymphoma, and leukemia, although these typically cause enlargement of involved nodes without inflammation (lymphadenopathy) rather than lymphadenitis.In most cases of cervical lymphadenitis, the diagnosis is made on a clinical basis alone and therapy is empiric. If bacterial adenitis is suspected, antibiotic therapy should be aimed at the most likely causative organism according to the patient’s age and presenting signs and symptoms as well as local antimicrobial sensitivities. Patients who have severe disease or fail appropriate initial therapy require further evaluation. Complete blood count and inflammatory marker measurement may be helpful in gauging response to therapy or likelihood of acute bacterial infection as a cause of the lymphadenitis. Nodes that have evidence of acute suppuration or abscess formation should undergo aspiration or incision and drainage for diagnosis and management. Culture results help guide appropriate antibiotic therapy. Aspiration with culture and microscopic examination may aid in the diagnosis of cat-scratch disease and other less common causes of cervical lymphadenitis. Cat-scratch disease may respond to therapy with azithromycin, trimethoprim-sulfamethoxazole, rifampin, or gentamicin, although most cases are self-limited and resolve without treatment. Either needle aspiration or excision can be used to obtain specimens for biopsy in cases of suspected malignancy.Imaging studies are not necessary in most cases of cervical lymphadenitis. Ultrasonography may be helpful in identifying potentially drainable fluid collections as well as the size and extent of the mass. Computed tomography scan may be indicated if deeper infection such as retropharyngeal or deep neck abscess is suspected.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of cervical lymphadenitisRecognize signs and symptoms of cervical lymphadenitisBe familiar with ancillary studies relevant to cervical lymphadenitisPlan management of acute cervical lymphadenitisQuestion 6A 4-year-old girl is brought to the emergency department for evaluation of joint pain. She has had swelling and pain of her left knee, right ankle, and right elbow over the past 2 months, occasionally accompanied by redness and warmth of the involved joints. The pain is worst in the morning and after prolonged rest. She has not had any fever, rash, vomiting, diarrhea, or other systemic symptoms. There is no history of travel, tick exposure, or prior episodes of joint swelling. She has been seen by her family doctor and an immediate care center physician for these complaints, and a complete blood count revealed only mild anemia and radiographs were reportedly normal. On physical examination, the alert and well-appearing child has a temperature of 37.0°C, heart rate of 100 beats/min, respiratory rate of 22 breaths/min, and blood pressure of 94/58 mm Hg. The only abnormalities of note are swelling, mild warmth, and limited range of motion of her left knee, right elbow, and right ankle. She ambulates with a mildly antalgic gait.Of the following, the MOST likely contributor(s) to this child’s disorder is (are)A.exaggerated histamine responseB.genetic and environmental factorsC.lead exposureD.spirochetal infectionE.leukemia Correct answer BThe child described in the vignette meets diagnostic criteria for juvenile rheumatoid arthritis (now termed juvenile idiopathic arthritis [JIA]): younger than 16 years of age, arthritis in one or more joints, duration of 6 weeks or longer, and absence of any other cause of arthritis. The cause of JIA is unknown, although both genetic and environmental factors are believed to play roles. Children who have an exaggerated histamine response would be expected to show evidence of degranulated mast cells, such as urticaria, angioedema, and wheezing. Clinical characteristics of symptomatic lead toxicity include lethargy, abdominal pain, and encephalopathy. The child has no fever or rash to suggest infection with common spirochetes. Leukemia might be associated with arthralgia or bone pain but not arthritis. In addition, the normal results of the complete blood count point away from this diagnosis.The three broad categories of JIA include systemic arthritis, articular arthritis, and symptomatic arthritis. Systemic and articular arthritis comprise the diseases formerly grouped together as juvenile rheumatoid arthritis. Symptomatic arthritis includes psoriatic arthritis (associated dactylitis, nail abnormalities, and personal or family history of psoriasis), enthesitis-related arthritis, and unknown chronic arthritis that does not fit into the other categories.JIA is subdivided further based on the type of disease present during the first 6 months of illness. Oligoarthritis (pauciarticular JIA) (50% of cases) is defined as involvement of fewer than five joints, as in this child. Affected patients typically have mild-to-moderate pain and joint inflammation. Pauciarticular JIA is subdivided into early onset (before 5 years of age) and late onset (5 years or older). Early-onset pauciarticular JIA (as seen in the patient in the vignette) has a female predominance, positive antinuclear antibody (ANA) titer in up to 60% of cases, rare hip or sacroiliac joint involvement, and association with chronic iridocyclitis in nearly 50% of cases. Late-onset pauciarticular JIA has a male predominance, negative ANA titer, frequent involvement of the hip and sacroiliac joints, and occasional association with uveitis. The joint involvement in pauciarticular disease frequently is asymmetric.Polyarticular JIA (35% of cases) is defined as involvement of five or more joints within the first 6 months of symptoms and is subdivided into rheumatoid factor (RF)-positive and RF-negative disease. Polyarticular disease may be associated with fever, rash, and other systemic symptoms, particularly early in the course. RF-positive polyarticular JIA has a female predominance, generally occurs after 8 years of age, is associated with a positive ANA in up to 75% of patients, is not associated with uveitis, but is associated with severe and destructive arthritis. Subcutaneous rheumatoid nodules, particularly over extensor surfaces such as the elbows, are seen frequently in RF-positive disease. This form of JIA is closest in symptomatology to adult rheumatoid arthritis. RF-negative polyarticular JIA also has a female predominance, typically presents before 8 years of age, is associated with a positive ANA in only 25% of patients, rarely is associated with uveitis, and progresses to severe arthritis in approximately 10% of patients. The joint involvement in polyarticular JIA is typically symmetric and more severe than that seen in pauciarticular disease.Systemic JIA (15% of cases) (also know as Still disease) presents with spiking fevers (often twice daily), an evanescent salmon-pink rash, hepatosplenomegaly, myalgias, and serositis. The arthritis may not be present during the initial disease onset, making the diagnosis difficult. Cardiac involvement, including pericarditis or myocarditis, is most common in the systemic form of the disease. Patients who have systemic JIA more frequently are male, typically present before the age of 5 years, have negative ANA and RF status, do not develop uveitis, and develop severe arthritis in approximately 25% of cases. A feared and potentially fatal complication of systemic JIA is macrophage activation syndrome, which can present with unrelenting fevers, hepatosplenomegaly, pancytopenia, hepatic dysfunction, elevated ferritin values, and disseminated intravascular coagulation.Multiple human leukocyte antigen (HLA) genes have been associated with the various forms of JIA, and other non-HLA genes also have been implicated. Concentrations of multiple inflammatory markers, including tumor necrosis factor (TNF), interleukin-2, interferon-gamma, and other proinflammatory cytokines, are elevated in the serum and synovium of JIA patients. Although the exact genetic basis is unclear, immune dysregulation resulting in chronic synovial inflammation and thickening result. There is cellular infiltration and vasculitis involving the synovium, proliferation and thickening of the synovial tissues, and increased fluid production resulting in joint effusions. Radiographs of involved joints reveal soft-tissue swelling, periarticular osteopenia, progressive narrowing of joint spaces and bony erosions, and ultimately subluxation and ankylosis in severe cases. Systemic inflammation is evidenced by elevated white blood cell counts, mild-to-moderate anemia, elevated inflammatory markers, elevated complement and immunoglobulin concentrations, and variable elevation of ANA and RF values, as outlined previously. Because there are no specific markers for JIA and many of the serum markers vary according to disease duration and severity as well as subtype, the diagnosis is largely based on clinical factors.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of juvenile rheumatoid arthritisRecognize the signs and symptoms of juvenile rheumatoid arthritis by ageQuestion 7A 2-year-old boy is transported to the emergency department with a primary complaint of progressive lethargy and fever over the preceding 8 hours. He was previously well and had no known sick contacts. His immunizations are up to date. He attends child care. On physical examination, the ill-appearing boy has a temperature of 39.0°C, heart rate of 180 beats/min, respiratory rate of 55 breaths/min, blood pressure of 75/40 mm Hg, and capillary refill time of 3 seconds. He is somnolent and responds with nonpurposeful, symmetric withdrawal to painful stimulus. The left pupil reacts sluggishly to light. He has a nonblanching rash on his left lower extremity. Point of care blood glucose measures 62 mg/dL (3.4 mmol/L).Of the following, the MOST appropriate sequence of therapeutic interventions that should be undertaken for this patient are:A.blood culture → computed tomography (CT) scan → lumbar puncture (LP) (if CT results are normal) → intravenous (IV) ceftriaxoneB.blood culture → IV ceftriaxone + vancomycin → head CT scanC.blood culture → LP → IV ceftriaxone + vancomycinD.CT scan → IV dexamethasone + ceftriaxone → LP (if CT results are normal)E.CT → LP (if CT results are normal) → blood culture → IV ceftriaxone + vancomycin Correct answer BThe boy described in the vignette is in septic shock, with elevated intracranial pressure (ICP) due to bacterial meningitis. The petechial rash suggests meningococcus as the probable cause. The appropriate course of action after initial stabilization is to obtain a blood culture and administer broad-spectrum IV antibiotics in doses appropriate for meningitis. Empiric antimicrobial coverage should include a third-generation cephalosporin and vancomycin. The role of steroids in meningitis is controversial; they may decrease morbidity associated with meningitis due to Haemophilus influenzae, but there is not good evidence to support their use in other forms of bacterial meningitis in children. If the decision is made to administer dexamethasone, it should be administered with or just before the first dose of antibiotic.Cranial CT scan is warranted because of the abnormal results of his neurologic examination. Cranial CT can help to rule out intracranial complications such as brain abscess or cerebral edema. However, antibiotic therapy should not be delayed while waiting for a CT scan. ?Although obtaining a CT scan is appropriate, it may yield normal results early in the course of illness and is not helpful in most children who have suspected meningitis. Further, normal results do not exclude increased ICP. Clinical decision-making is preferred over a universal neuroimaging strategy before performing lumbar puncture (LP) because herniation may occur in the presence of normal CT findings. The clinical signs of coma and asymmetric pupils for this boy indicate cerebral herniation. In general, if children with suspected meningitis have no focal abnormalities on neurologic examination, cranial CT is not necessary prior to the performance of LP.The boy in the vignette possibly has disseminated intravascular coagulopathy with fulminant sepsis, and the presence of increased ICP and coagulopathy are relative contraindications to performing an LP.? In these cases, it is prudent to delay the LP for 24 to 48 hours until the patient’s clinical condition improves and coagulopathy is excluded. The diagnosis of bacterial meningitis does not rest solely on the results of cerebrospinal fluid culture. The blood culture yields the bacterial pathogen in meningitis in up to 80% of cases. Although culture of cerebrospinal fluid is not likely to yield the bacterial pathogen 12 to 24 hours after antibiotics are administered, the fluid still will show marked abnormalities with regard to protein, glucose, and presence of white blood cells.The interspinous space that corresponds to the level of the iliac crest is L3-L4 and is the commonly recommended site for LP (Fig. 1).Failure to obtain cerebrospinal fluid (CSF) may necessitate reinsertion of the needle one space above or below this site. Because the spinal cord ends at the level of L1, choosing a level higher than the L1-L2 interspace is contraindicated. Of note, because the conus medullaris descends down to the level L2 in preterm neonates, selecting a level caudad to the L2-L3 space can minimize the risk of inadvertent cord injury in this age group.? The needle traverses through the two layers of resistance (interspinous ligament and ligamentum flavum) before entering the subarachnoid space (Fig. 2). The “pop” experienced just before obtaining CSF is the resistance offered by the ligamentum flavum.Figure 2: The needle traverses through the two layers of resistance (interspinous ligament and ligamentum flavum) before entering the subarachnoid space. Illustration courtesy of Jenny Wang.Local anesthetic is strongly advocated before LP in all age groups. Lidocaine use has not been associated with decreased success rates or a higher incidence of traumatic LPs. For a dynamic review of the specific steps involved in performing a LP, please refer to the link to the video posted on the Royal Melbourne Children’s hospital web site.The most common reason for failure to obtain a specimen with LP is incorrect needle placement. Ensuring that the path of the needle is in the midline and at the appropriate depth usually resolves the issue. Occasionally, flow through the needle may be impeded by an interposed dural flap. Incremental, gentle twisting of the needle can facilitate CSF flow. For obese patients, ultrasonography- or fluoroscopy-guided LP may be helpful.A traumatic LP results from inadvertent puncture of the internal vertebral plexus of veins. Reinsertion of the needle at a different level or a reattempt in a different direction usually obviates this problem. Interpretation of the CSF using observed versus predicted red blood cell:white blood cell count ratio may be helpful in diagnosing meningitis in the face of a traumatic LP, but this is controversial.Postdural puncture headache occurs in up to 15% of patients undergoing LP. Use of a smaller-gauge, atraumatic needle, with reinsertion of the stylet before removal, was associated with a lower incidence of spinal headaches in a review of adult-based studies. The purported mechanism is inadvertent pulling of a string of arachnoid into the needle if the stylet is not fully reinserted before removal. Other potential complications of LP include: spinal epidural hematoma (rare), epidural abscess (rare), respiratory arrest from positioning (may occur in neonates or sick infants from excessive neck flexion), and cerebellar tonsillar herniation (rare).American Board of Pediatrics Content Specification(s):Discuss the indications and contraindications for lumbar punctureDescribe the key steps and potential pitfalls in performing lumbar punctureDiscuss the complications associated with lumbar punctureKnow the anatomy and pathophysiology relevant to lumbar punctureQuestion 8A 14-year-old boy is brought to the emergency department by his parents, who found him having a generalized seizure in his bedroom. Physical examination of the lethargic boy shows a temperature of 39.5°C, heart rate of 140 beats/min, respiratory rate of 20 breaths/min, blood pressure of 150/110 mm Hg, and oxygen saturation of 100% on supplemental oxygen via face mask. His pupils are dilated and reactive. His parents state that he has lost weight over the past few months and estimate his current weight at 40 kg. They report no antecedent history of trauma or illness or significant findings on his past medical history. Computed tomography scan of the head yields normal results, and electrocardiography reveals ST segment depressions and premature ventricular contractions.Of the following, the MOST likely substance responsible for this boy’s symptoms isA.amphetamineB.cocaineC.diazepamD.oxycodoneE.phencyclidine Correct answer BThe clinical findings of seizures, arrhythmia, myocardial ischemia, and hyperthermia described for the boy in the vignette are consistent with cocaine overdose. Cocaine is a drug that is abused to increase a sense of well-being. Use also decreases fatigue and hunger. “Crack” is a form of cocaine that is ready for smoking, thereby obviating the need for chemical manipulations to prepare cocaine hydrochloride. Between 6% and 8% of teenagers in the United States report having used crack cocaine. An additional worrisome trend is the declining age of initial use.The effects of cocaine use range from mild elation to coma. In general, there is a dose-dependent relationship. At higher doses, elation,HYPERLINK "javascript:showModal('/subscriber/modal/august/aug-q8-content-specifications')"? euphoria, and overconfidence become agitation, anxiety, and irritability. At even higher doses, euphoria occurs, rapidly alternating with violent and aggressive behavior, which may be accompanied by paranoia and toxic psychosis. Overdose is manifested by convulsions, cardiac arrhythmias, hyperthermia, coma, and ultimately, death.Ulcerations of the nose and perforation of the septum are seen in chronic cocaine use, although crack cocaine use is not associated with these physical findings. Because cocaine is an appetite suppressant, chronic use may lead to weight loss and nutritional deficiencies, as seen in this young man.Benzodiazepines such as diazepam can cause depressed mental status but not other signs and symptoms of sympathomimetic discharge described for this boy Phencyclidine often is used to “cut” crack cocaine. When abused as the principal agent, it causes physiologic changes similar to those of cocaine, but it is not associated with cardiac ischemia.Amphetamines, when used at doses that cause euphoria, manifest very few physiologic adverse effects. Most patients who present to emergency departments with amphetamine overdose manifest anxiety/paranoia, tachycardia, and mild hypertension. They usually do not require pharmacologic intervention or inpatient treatment. A series of emergency department visits for cocaine toxicity showed a higher incidence of undesirable adverse effects, including seizures, stroke, myocardial infarction/arrhythmia, and hyperthermia compared with amphetamines. Opioids, such as oxycodone, cause respiratory depression, pinpoint pupils, and hypothermia.American Board of Pediatrics Content Specification(s):Recognize signs and symptoms of abuse of cocaineSeptember 2010Question 1A 10-year-old girl is brought to the emergency department with a 4-day history of increasing weakness in her lower extremities. Today she was unable to get up from bed or bear weight. She had a viral infection 2 weeks ago that resolved without any medications. She experienced tingling and numbness in the lower extremities 4 days ago, which was followed by weakness. She has had some difficulty in initiating urination for the past 2 days, and today she had an episode of bowel incontinence. There is no history of trauma, travel, or exposure to any sick contacts. Her pets include exotic tropical fish and two turtles. On physical examination, the girl has normal development for her age, is alert, and appears comfortable, but she unable to move her lower extremities. Her temperature is 37.8°C, heart rate is 90 beats/min, respiratory rate is 20 breaths/min, and blood pressure is 118/60 mm Hg. Her cranial nerves are intact, but deep tendon reflexes are absent in the legs and diminished in the arms. Her strength is 1/5 in the legs and 5/5 in the arms. She has loss of rectal tone and pain and touch sensation in the lower extremities. There is no spinal tenderness or deformity. Cerebellar signs are difficult to elicit, but there is no nystagmus. Other findings on her physical examination are normal. Of the following, the test that is MOST likely to establish the diagnosis isA.cerebrospinal fluid analysisB.mouse bioassay for fish toxins?C.magnetic resonance imaging of the spineD.serum assay for acetylcholine receptor antibodiesE.urine testing for porphobilinCorrect answer: CThe patient described in the vignette has acute flaccid paralysis with sensory loss, bowel and bladder incontinence, and loss of rectal tone, which are suggestive of acute spinal cord pathology. The most appropriate diagnostic test is magnetic resonance imaging (MRI) of the spine. Elevated cerebrospinal fluid protein concentrations without significant cytologic response are diagnostic of Guillain Barré syndrome (GBS), acetylcholine receptor antibodies in the serum are indicative of myasthenia gravis, mouse bioassays for marine toxins are used to diagnose ciguatera, and porphobilins in the urine are characteristic of acute porphyria. The presence of an objective loss of sensation below a specific spinal cord level (sensory level) for this girl rules out GBS, myasthenia gravis, ciguatera, and porphyria.????The history, symptoms, and physical examination findings described for the girl in the vignette are suggestive of acute transverse myelitis. This demyelinating disorder is characterized by an abrupt onset of progressive weakness in the lower extremities in conjunction with sensory loss and loss of bladder and bowel function that usually is preceded by a viral or gastrointestinal illness. Symptoms result from motor and sensory deficits attributable to involvement of one or both sides of the spinal cord. Paresthesias typically precede sensory loss and motor loss. Often there is a well-defined level of change in touch and pain sensation, and patients can experience a band of unpleasant hyperesthesia at the upper portion of the level of sensory change. The changes develop over hours or days. Tenderness to percussion of the spinal column can be elicited over the affected segment of spinal cord. Deep tendon reflexes are diminished or absent. A hyperacute presentation sometimes occurs that involves rapid onset of paraplegia, sensory abnormalities, and urinary retention and often is associated with significant back pain.????The exact cause of transverse myelitis is not known. Pathologic examination of the spinal cord in affected individuals reveals perivascular cuffing with lymphocytes. The pathogenesis possibly is due to direct viral invasion of the spinal cord, a cell-mediated autoimmune response, autoimmune vasculitis, or a combination of these actions. Several organisms, including viruses such as Epstein-Barr virus, herpesvirus, influenza, rubella, mumps, and varicella and other infectious agents such as Mycoplasma and Borrelia burgdorferi, have been implicated. Although most cases are idiopathic, transverse myelitis can occur in acute disseminated encephalomyelitis, multiple sclerosis, and neuromyelitis optica (Devic disease).??MRI of the spine and brain is the investigation of choice for diagnosis and follow-up in patients who have transverse myelitis. MRI typically reveals a variable degree of swelling of the cord and diffuse or multifocal areas of abnormal bright signal on T2-weighted sequences, a finding consistent with demyelinization. In addition, imaging can rule out a cord-compressing lesion, which also can present as acute flaccid paralysis. When transverse myelitis or other demyelinating disease is suspected, brain MRI should be performed simultaneously to rule out multifocal areas of demyelinization, which may be indicative of multiple sclerosis. ??Corticosteroids in the form of high-dose methylprednisone with or without intravenous immune globulin are used to treat transverse myelitis, although efficacy remains unproven. Critical supportive therapy involves maintenance of hydration and nutrition; bladder catheterization; and anticipation and prompt treatment of urinary tract infections, pressure sores, pneumonia, and venous thrombosis. Physical therapy for the patient and psychological support for both the patient and family are of great value in facilitating recovery. Recovery can occur over weeks to months and is complete in 60% of cases. Residual deficits include bowel and bladder dysfunction and weakness in lower extremities. ??GBS is the most common cause of acute flaccid paralysis that often follows an antecedent viral or gastrointestinal illness. Nearly 50% of affected patients have autonomic dysfunction in the form of cardiac dysrhythmias, orthostatic hypotension, and bladder dysfunction. In contrast to transverse myelitis, the paralysis typically is ascending and does not involve any sensory loss in spite of paresthesias. ??Acute intermittent porphyria, an autosomal dominant disorder resulting from a partial deficiency of porphobilinogen deaminase activity, can present with a multitude of neuropsychiatric manifestations, such as proximal muscle weakness and bulbar palsy due to motor neuropathy, sensory impairment, and such psychiatric complaints as hysteria, phobias, psychosis, agitation, and delirium. In addition, gastrointestinal symptoms, primarily abdominal pain with or without nausea, vomiting, diarrhea, and abdominal distension, frequently comprise the presenting symptoms and are indicative of an acute attack. ??Marine poisons such as ciguatera can be detected by testing the fish on mouse bioassays, but the diagnosis is clinical, and these tests are not available easily. Ciguatera toxin is associated with consumption of large reef fish such as grouper, red snapper, amber jack, and barracuda. The fish can become contaminated during consumption of algae containing dinoflagellates, which produce the heat-stable ciguatoxin. The toxin becomes concentrated as it moves up the food chain. This disease begins with nausea, vomiting, abdominal cramping, and diarrhea, followed in 2 to 5 days by paresthesia, painful teeth, painful urination, blurred vision, nerve palsies, weakness or reversal of hot or cold sensation, and cardiovascular abnormalities (heart blocks, hypotension). ??Myasthenia gravis, the most common disorder of the neuromuscular junction, is characterized by a fluctuating degree of weakness, typically more in the evening, involving the ocular, bulbar, limb, and respiratory muscles. It is an autoimmune illness due to production of antiacetylcholine receptor antibodies. Although bedside tests with edrophonium chloride (Tensilon?) are helpful, diagnosis is confirmed with serologic testing for autoantibodies against the acetylcholine receptor or a receptor-associated protein, muscle-specific tyrosine kinase. ??Tick paralysis caused by Dermacentor ticks usually begins with paresthesias, followed by unsteady gait and eventual ascending paralysis. Patients do not have fever, altered sensorium, a sensory level, or bowel or bladder dysfunction. A meticulous examination almost always reveals a tick. Botulism and poliomyelitis are other conditions that should be considered in the differential diagnosis of flaccid paralysis, but they can be distinguished clinically. The paralysis in botulism typically is descending, involves bulbar musculature, and has no sensory signs or symptoms. Polio, which is rare, is associated with muscle pain that precedes asymmetric paralysis. American Board of Pediatrics Content Specification(s):??Know the etiology and pathophysiology of sudden flaccid paralysis ?Plan the diagnostic evaluation and initial intervention for patients with sudden flaccid paralysis??Recognize the serious and/or life-threatening causes of sudden flaccid paralysisQuestion 2An 11-month-old infant who attends a child care facility presents to the emergency department with a 16-hour history of vomiting, watery diarrhea, low-grade fever, and oliguria. On physical examination, the listless infant has sunken eyes, diminished tears, skin tenting, and thready pulses. His temperature is 38.4°C, heart rate is 158 beats/min, respiratory rate is 48 breaths/min, blood pressure is 90/70 mm Hg, and capillary refill time is 4 seconds. His abdomen is soft and nontender with mild hyperactive bowel sounds. The remainder of the physical findings are within normal parameters. You administer an intravenous bolus of 20-mL/kg saline and obtain a blood specimen for point-of-care glucose testing and assessment of serum electrolytes. Of the following, the laboratory test that is MOST predictive of the severity of this infant’s dehydration is measurement ofA.bicarbonateB.blood urea nitrogenC.glucoseD.potassiumE.urine specific gravity Correct answer AThe infant described in the vignette is severely dehydrated from an acute infectious diarrheal illness. A variety of laboratory tests have been assessed for predicting the severity of dehydration in children who have gastroenteritis. Serum bicarbonate is the most useful in children. Several studies found that low serum bicarbonate concentrations combined with certain clinical parameters predict dehydration. Vega and Avner found that a serum bicarbonate value of 17 mEq/L (17 mmol/L) was 77% sensitive for moderate dehydration and 94% sensitive for severe dehydration. When the clinical impression of dehydration was combined with a bicarbonate concentration of 17 mEq/L (17 mmol/L) or less, sensitivity for prediction of severe dehydration increased to 100%. A value below 17 mEq/L (17 mmol/L) differentiated children who had moderate and severe hypovolemia from those who had mild hypovolemia. Similarly, the findings of Yilmaz and associates suggested that a serum bicarbonate of less than 15 mEq/L (15 mmol/L), coupled with an elevated serum urea concentration, may be a valuable adjunct to clinical evaluation in predicting the degree of dehydration. Study results are conflicting on whether blood urea nitrogen (BUN) or BUN:creatinine ratio correlates with the severity of dehydration in children. In general, the BUN rises with increasing severity of hypovolemia, reflecting the decline in glomerular filtration rate and increase in sodium and water reabsorption and urea recycling. However, the sensitivity of BUN elevation is not sufficient to be clinically useful in diarrheal dehydration. Furthermore, it may be increased by other factors, such as bleeding or catabolic tissue breakdown.??Urine specific gravity is unreliable in judging dehydration status in the acute-care setting for the following reasons. First, the dehydrated child frequently does not urinate until rehydration has begun. Second, the value of urine specific gravity tends to lag behind actual hydration status. Third, the use of urine specific gravity in the neonate and young infant may not reflect the degree of dehydration because the concentrating ability of the kidney does not reach adult values until approximately 1 year of age. ??Serum potassium may be falsely elevated in dehydration due to metabolic acidosis or may be low due to diarrheal losses. Similarly, hypoglycemia may be noted in approximately 9% of infants and young children who have diarrheal dehydration. Therefore, neither glucose nor potassium is a good predictor of dehydration. ??Most cases of gastroenteritis in children have viral causes.? The virus typically is passed from child to child by the fecal-oral route. Outbreaks in areas of close contact, such as child care centers, are common and reflect the ability of these viruses to survive for prolonged periods on toys, bedrails, countertops, and other surfaces. Rotavirus is a common viral cause of gastroenteritis in the fall and winter months, especially in children younger than 4 years of age. The diarrhea may be severe and prolonged, causing dehydration and hospitalization in children who cannot maintain adequate oral intake. Other viral causes include adenovirus (children younger than 2 years), norovirus (children 3 months to 6 years), and astrovirus (children younger than 7 years, usually associated with contaminated water and shellfish). In addition to viruses, gastroenteritis in children may be due to a variety of other causes. One approach to the differential diagnosis of acute diarrhea is to divide the causes by the presence or absence of blood in the stool (Table 1). The degree of dehydration can be assessed using clinical parameters (Table 2). The American Academy of Pediatrics recommends measuring electrolytes in cases of acute gastroenteritis associated with altered mental status, moderate-to-severe dehydration, or clinical signs of hypokalemia or hypernatremia; for infants younger than 6 months of age; and for suspicious presentations.?Table 2: Degree of DehydrationParameterDegree of DehydrationMildModerateSevereWeight loss—infants5%10%15%Weight loss—children3%6%>9%Skin colorPaleGrayMottledSkin turgorMay be normalDecreasedTentingMucous membranesSlightly dryDryDry, parched, collapse of sublingual veinsEyesNormalDecreased tearsSunken, absence of tearsCentral nervous systemAlert but thirstyIrritableLethargic, grunting, comaPulseNormal and strongRapid and slightly weakSignificantly tachycardic and very weak to not palpableCapillary refillNormal (<2 sec)2 to 4 sec>4 secBlood pressureNo changeOrthostatic decreaseShockUrineNormal to mildly reducedSignificantly reducedAnuriaVolume of deficit—infants50 mL/kg60 mL/kg150 mL/kgVolume of deficit—children30 mL/kg100 mL/kg>90 mL/kgOral rehydration solution (ORS) is recommended as first-line therapy for mild-to-moderate dehydration. Parenteral hydration is suggested for children demonstrating signs of shock. ORS is not contraindicated in the presence of emesis or electrolyte abnormalities. When vascular access is difficult to obtain in a patient who has severe dehydration, a nasogastric tube can be placed to deliver the ORS. Recent literature suggests that success rates with ORS can be enhanced with the additional use of oral ondansetron. Specific contraindications to ORS include intractable emesis, paralytic ileus with abdominal distention, an acute abdomen, altered mentation, and shock. An appropriate ORS should contain 70 to 90 mEq/L sodium and no more than 25 g/L of glucose (Table 3). The underlying physiologic principle for the use of ORS is active cotransport of solute (sodium) with organic substrate (glucose) and water across the intestinal epithelium.Table 3: Solutions Commonly Used in Children Who Have DiarrheaSolutionGlucose/Phenol (g/L)Sodium (mEq/L)Base (mEq/L; Citrate or Bicarbonate)Potassium (mEq/L)Osmolality (mMol/L)Physiologically Appropriate SolutionsOral Rehydration Solutions?????Rehydralyte25753020310WHO and UNICEF oral replacement solution20903020310Oral Maintenance Solutions?????Pedialyte?*25453020270Ricelyte??30*503025200Physiologically Inappropriate SolutionsCola7002130.1750Apple juice6903032730Gatorade??2552033330 American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of viral gastroenteritisRecognize signs and symptoms of viral gastroenteritisPlan management of acute viral gastroenteritisQuestion 3A 15-year-old boy is in the emergency department undergoing an evaluation for behavioral problems and possible drug abuse. His toxicologic screen is positive only for marijuana. His vital signs and other aspects of his physical examination are normal. While awaiting psychiatric evaluation, he becomes increasingly agitated, escalating from being verbally abusive to his parents and staff to throwing objects at staff and punching a hole in the wall. Attempts by his parents, staff, and security to calm him have been unsuccessful. Security officers have placed the patient in 4-point restraints.Of the following, the MOST appropriate next steps areA.administration of an intramuscular antipsychotic and maintenance of chemical and physical restraint until the patient is transferred to a psychiatric facilityB.attempt to obtain the patient’s consent for restraints, and if unsuccessful, remove restraints and discharge himC.continuous face-to-face monitoring of the patient, regular reassessment of the need for restraints, and removal of restraints as soon as it is deemed safeD.immediate removal of restraints to allow the physician to assess the level of agitation prior to ordering patient restraintE.routine monitoring of vital signs and physical examination and continuation of restraints until transfer to a psychiatric facility is completed Correct answer CThe young man described in the vignette has displayed escalating agitation and has failed attempts at de-escalation. His behavior has become dangerous to himself, emergency department staff, and other patients. The Joint Commission requires all health-care facilities to have formal protocols in place regarding the use of physical and chemical restraints. Such protocols should include guidelines for when restraints should be used as well as for removal of restraints, patient observation, and monitoring. All patients in restraints require continuous face-to-face monitoring of their medical and behavioral status and vital signs by trained medical personnel as well as regular offering of food, water, and access to bathroom facilities. An assessment of the patient and formal order for restraint must be provided by a Licensed Independent Practitioner within 1 hour of initiation of restraints. Repeat assessment and renewal of the order is required every 1 hour for children younger than 9 years of age, every 2 hours for children from 9 to 17 years of age, and every 4 hours for adults. Consent is not required when patients are deemed to be incompetent to provide that consent or a danger to themselves or others.HYPERLINK "javascript:showModal('/subscriber/modal/september/september-q3-content-specifications')"???Chemical or physical restraints should be considered only after all efforts have been made to control the patient through other measures. Removal of family members who contribute to the patient’s agitation; discussion with the patient about the reasons for his or her agitation and possible solutions; and provision of a quiet, calm environment may prevent the need for restraint. If these measures fail and the patient is deemed to be dangerous to him- or herself or others, restraints are indicated. ??The decision regarding physical versus chemical restraint depends on the patient’s underlying medical and psychiatric conditions, current medications and allergies, and careful consideration of the risks and benefits of the various techniques. Because intravenous access may be difficult or impossible in an agitated patient, intramuscular medication administration may be required. Classes of medications commonly used for chemical restraint include antihistamines (eg, diphenhydramine, hydroxyzine), benzodiazepines (eg, diazepam, lorazepam), and antipsychotics (eg, haloperidol, ziprasidone, chlorpromazine, olanzapine). Physical restraint involves the use of devices, usually leather or Velcro? extremity straps, to control the patient’s limbs, restrict their mobility, and prevent injury to the patient or others. Most patients are restrained in the supine position with 4-point extremity straps; extremely violent or agitated patients are controlled better in the prone position.? ??Prone restraint is associated with a higher rate of complications, particularly if restraints or pressure are applied to the trunk or sheets/towels are used around the head to control spitting or biting. Deaths and complications occurring during patient restraint led to the development of the previously cited Joint Commission requirements. Complications of restraints include soft-tissue injury or vascular compromise at the site of restraint, fractures, asphyxia from chest or neck compression, aspiration of vomitus and secretions, hyperthermia, and rhabdomyolysis.??Restraints should be removed as soon as safely possible, and restraints never should be placed or continued for the convenience of the medical staff (eg, to hold the patient until transfer to a psychiatric facility) or to punish the patient. Techniques for removal vary from complete removal of all restraints simultaneously to stepwise removal of restraints one limb at a time. As with application of restraints, the most appropriate method of removal depends on the individual patient.All aspects of patient restraint, including the steps taken to avoid restraints, reasons for initiating them, orders with appropriate time-limited reassessment and renewal, assessment of the patient’s status and vital signs, and removal of restraints, should be documented clearly in the patient’s medical record.??American Board of Pediatrics Content Specification(s):??Discuss the indications and contraindications for restraint techniques??Describe the key steps and potential pitfalls in performing restraint techniques??Discuss the complications associated with restraint techniquesQuestion 4A 2-month-old boy is brought to the emergency department because of increased work of breathing that has been present for 3 days. He initially had nasal congestion and cough with a low-grade fever for 1 day. Over the past 3 days, he has had worsening cough, rapid respiratory rate, and noisy breathing. He is not feeding well, and his urine output is decreased. He was born at 30 weeks’ gestation and was in the neonatal intensive care unit for about 5 weeks. On physical examination, the tired-appearing infant exhibits moderate-to-severe work of breathing and has a temperature of 38.6°C, respiratory rate of 54 breaths/min, heart rate of 159 beats/min, blood pressure of 72/42 mm Hg, and pulse oximetry reading of 90% in room air. He has diffuse inspiratory and expiratory wheezing bilaterally, with marked intercostal, subcostal, and supraclavicular retractions. Other than tachycardia, findings on his cardiac examination appear to be normal, and his capillary refill is 2 seconds. He has no hepatomegaly. You administer inhaled racemic epinephrine due to his work of breathing. A few minutes later, the nurse alerts you that his respiratory rate is 24 breaths/min, heart rate is 108 beats/min, and pulse oximetry reading is 84%, despite receiving oxygen via face mask with an approximate Fio2 of 40%.Of the following, the MOST appropriate next step in management is toA.begin positive-pressure ventilation with a bag–valve–maskB.increase the delivered oxygen concentration by face maskC.intubate with a 4.5-mm cuffed endotracheal tubeD.obtain an arterial blood gas to assess for hypercarbiaE.perform nasal suctioning and repeat the racemic epinephrine Correct answer AThe infant described in the vignette has bronchiolitis and has progressed from moderate-to-severe respiratory distress to respiratory failure. His fatigue or worsening ventilation/perfusion mismatch has produced hypercarbia and worsening hypoxia, and the decreasing respiratory rate and heart rate are signs of the onset of respiratory failure. Ventilation and oxygenation both must be addressed via immediate intervention with bag-valve-mask ventilation. Simply increasing the inhaled oxygen concentration or administering additional medications is unlikely to reverse the respiratory failure, and delays in initiating assisted ventilations may lead to the onset of respiratory or even cardiopulmonary arrest. Endotracheal intubation and monitoring of arterial blood gases may be required, but the child first should be stabilized as best possible with immediate positive-pressure ventilation via a bag-valve-mask apparatus to prevent further worsening of his condition.?HYPERLINK "javascript:showModal('/subscriber/modal/september/september-q4-content-specifications')"Bronchiolitis is caused by viral infections in children younger than 2 years of age. There is usually a preceding upper respiratory tract infection. Bronchiolitis most commonly is caused by respiratory syncytial virus (RSV), but other agents may be responsible. Children who have histories of prematurity are at risk for worse outcomes. With spread to the cells lining the lower airways, infection of the respiratory epithelium by RSV or other agents causes airway edema, increased mucous production, and sloughing of necrotized epithelial cells. Increased edema and an inflammatory response follow. Debris in the airways leads to air-trapping, atelectasis, and ventilation-perfusion mismatching. Tachypnea and increased work of breathing are evident as the child attempts to maintain both normocarbia and normal oxygen concentrations.Young infants are particularly prone to the development of respiratory failure due to the anatomy of their respiratory system.? Their airways are smaller in diameter, making obstruction more likely. Infants lack the “pores of Kohn” and “canals of Lambert,” which provide connections between alveoli attached to neighboring terminal bronchioles and other distal airways, respectively. The lack of these connections makes it more difficult for infants to clear mucus from the distal airways and leads to an increased tendency to develop atelectasis. In turn, diffusion capacity is diminished, and there is a marked mismatch between areas that are ventilated and areas that are well perfused (ventilation-perfusion mismatch). Hypoxia and hypercarbia result.In young children, decreased respiratory muscle mass and strength as well as a less rigid chest wall (due to incompletely calcified ribs) lead to a relative inefficiency of the respiratory apparatus, causing a propensity to fatigue and a rapid onset of respiratory failure. See-saw or marked abdominal breathing, deep retractions, and use of accessory muscles are evidence of respiratory distress. When the child’s compensatory mechanisms no longer can meet metabolic demands, hypoxia and hypercarbia result as respiratory failure develops.American Board of Pediatrics Content Specification(s):Understand the anatomy of the respiratory system.Understand the progression of respiratory failure to arrest.Understand the pathophysiology of respiratory failure.Question 5While working in a small community emergency department, you treat a 7-year-old boy who presents with a laceration of his chin. He fell while running at a playground. He did not lose consciousness and appears to have no other injuries. As you begin to repair the laceration, a colleague suggests obtaining mandible films to be sure there is no fracture because of a study that showed 30% of children who have chin lacerations have associated mandible fractures. The authors recommended mandibular radiographs for all children who have chin lacerations. You point out that the study was performed at a pediatric trauma center and that most of the patients studied had head injuries that required computed tomography (CT) scan as part of their evaluation. Of the following, the PRIMARY reason that the findings may not apply in this case is the possibility ofA.confoundingB.misclassification biasC.recall biasD.reporting biasE.selection bias Correct answer E“Bias is defined as any process that leads to the systematic deviation of study results or inferences from the truth.” (Neuspiel DR, 2008 and Porta M, 2008) It does not refer to a conscious or unconscious bias on the part of investigators to steer the results of a study in one direction; rather, it denotes errors caused by suboptimal study design, reporting, or analysis. Many types of bias have been identified in the biomedical sciences, and it is almost impossible to design a study that does not have the potential to be affected by some bias. Researchers and practitioners reading research results must look for potential sources of bias, recognize that this potential exists, and account for its possibility in the design and interpretation of studies.?HYPERLINK "javascript:showModal('/subscriber/modal/september/september-q5-content-specifications')"? When individuals who participate in a study differ in some way from the population to whom the results of the study are applied, erroneous conclusions may be made. This is the meaning of a selection bias and is a source of possible erroneous conclusions in the study mentioned by the colleague in the vignette. Such differences may be due to how subjects are recruited or selected by the investigators or how subjects choose whether to enroll in a study. In case-control studies, selection bias occurs when the control group selected differs by some systematic factor from the subjects classified as cases. In the vignette, the study described was conducted at a trauma center and most of the patients also had injuries that required evaluation with head CT scans. Individuals who have more severe injuries may be referred preferentially to the trauma center and those included in the study seem to have suffered significant injuries in addition to chin lacerations. Therefore, the range of the injuries in the study sample may be more extensive than that seen in the general population. Although the study highlights the importance of looking for mandibular fractures in children who have chin lacerations, it may be an overestimate of the prevalence of mandibular fractures in all children who have such injuries. The results of the study are not generalizable to all children who have chin lacerations.Publication bias is related to the tendency of both investigators (authors) and editors to publish studies that have positive results and to reject studies that do not. Confounding occurs when a factor is associated with both an exposure and an outcome and that factor is identified mistakenly as being linked to the development of the outcome. Misclassification occurs when subjects are misclassified erroneously as either having the exposure or the outcome of interest. In this study, the presence of a chin laceration (exposure) is unlikely to be assigned mistakenly. Misclassification related to the outcome of interest could be present if systematic errors were made in assigning the presence or absence of a mandibular fracture.Recall bias is seen more frequently in case-control or retrospective studies. Subjects who are cases are more likely to “remember” past exposures than controls who have not suffered the outcome of interest. Reporting bias may be seen when different groups selectively report information based on concerns about how that information may be used or what it reveals about them (eg, information about substance abuse or sexual practices). This also may be a problem in unblinded clinical studies, where either the investigators or subjects are aware of a treatment that has been administered and this influences how either group reports the presence of the outcome of interest.American Board of Pediatrics Content Specification(s):Understand some common sources of bias in research (e.g., ascertainment bias, recruitment bias, recall bias).Question 6A 7-year-old previously healthy boy presents to the emergency department with jaundice. There is no family history of liver disease. ?The boy has no history of fever, vomiting, or abdominal pain. On physical examination, his temperature is 37.0°C, heart rate is 120 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 100/60 mm Hg. He has scleral icterus but no organomegaly, signs of bleeding, or any neurologic deficits.? Of the following, in addition to measuring fractionated bilirubin, the MOST important test to perform in the emergency department isA.ceruloplasmin plete blood and reticulocyte countsC.Epstein-Barr virus serologiesD.hepatitis B serologiesE.hepatobiliary iminodiacetic acid (HIDA)?scan Correct answer BThe child described in the vignette, who has jaundice and mild tachycardia in the absence of hepatomegaly, fever, vomiting, or abdominal pain, most likely has hemolytic anemia. Jaundice is defined as the presence of a yellow or yellow-greenish hue to the skin, sclera, and mucous membranes due to elevated concentrations of serum bilirubin (hyperbilirubinemia). Jaundice can be detected readily clinically when the total serum bilirubin is greater than 5 mg/dL (85 mcmol/L). Clinical jaundice occurs much less frequently in older children and adolescents than in neonates.HYPERLINK "javascript:showModal('/subscriber/modal/september/september-q6-content-specifications')"???The causes of hyperbilirubinemia differ by age and affect the laboratory investigations that should be undertaken. (For a useful algorithm of investigations, see Figure 14.1 in Mandl KD. Jaundice—unconjugated hyperbilirubinemia. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 5th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2006:399-408.) The serious and life-threatening causes of hyperbilirubinemia in neonates include sepsis, hemolytic disease of the newborn, metabolic diseases such as galactosemia, and structural lesions such as biliary atresia. In older children, serious and life-threatening causes (Table) include those in which hyperbilirubinemia indicates hepatic failure, such as viral and drug-induced hepatitides.Metabolic diseases such as Wilson disease and Reye syndrome also can present with hyperbilirubinemia. Hyperbilirubinemia also is a marker for hemolysis, which occurs in hemoglobinopathies (eg, hereditary spherocytosis), enzyme deficiencies (eg, glucose-6-phosphate dehydrogenase deficiency), and immune-mediated hemolysis (eg, autoimmune hemolytic anemia). For children who have known hemoglobinopathies such as sickle cell disease, hyperbilirubinemia frequently is a sign of hemolytic crisis.? Laboratory investigations should be dictated by clinical history and findings on physical examination. A reasonable approach involves measurement of fractionated bilirubin, complete blood and reticulocyte counts (including microscopic examination of red cell morphologies), transaminases, prothrombin time, activated partial thromboplastin time, and albumin. More detailed investigation should be based on the results of initial studies and may include imaging of the biliary tract, more detailed biochemical analysis, and liver biopsy.? Infections due to viruses such as hepatitis A and B and Epstein-Barr virus are ?possible but not likely in this patient because of the lack of additional symptoms. Assessment for their presence may be dictated by results of initial laboratory investigations. HIDA scans usually are performed after ultrasonography fails to reveal an anatomic cause of obstructive (conjugated) hyperbilirubinemia. Ceruloplasmin values often are decreased in children who have Wilson disease, although the diagnosis rarely is made in the emergency department, where the priority is to identify life-threatening causes of hyperbilirubinemia that can be addressed acutely.? ?American Board of Pediatrics Content Specification(s):??Know the etiology by age and the pathophysiology of jaundice??Plan diagnostic evaluation and initial intervention for patients with jaundice.??Recognize serious and/or life?threatening causes of jaundiceQuestion 7A 15-year-old girl presents to the emergency department after sustaining an injury while doing a handspring in gymnastics. On physical examination, she has an obvious deformity of her left elbow. Radial pulses and intact sensory and motor functions of her forearm are present. You obtain a radiograph of her injury (Fig. 1).Figure 1After procedural sedation and analgesia, the on-call orthopedic surgeon reduces the injury.Of the following, the MOST common associated injury to expect for this girl isA.coronoid fractureB.medial epicondyle fractureC.radial head subluxationD.radial neck fractureE.ulnar nerve injuryCorrect answer: BThe girl described in the vignette has dislocated her elbow. The peak incidence of this injury occurs in adolescents between 11 and 15 years.Posterior and posteriolateral dislocations of the forearm (ulna) are most common types (95%). The usual mechanism of injury is a fall on outstretched hand with the forearm supinated, in contrast to supracondylar fractures of the distal humerus, in which the forearm typically is pronated. Physical examination reveals an obvious deformity of the elbow, with prominence of the olecranon process. There may be associated neurovascular compromise, similar to injuries to the distal humerus. Associated injuries in decreasing order of frequency include:Avulsion fracture of the medial epicondyle (most common), with entrapment of the medial epicondyle (a possible complication of reduction that requires open reduction)Coronoid process fractureRadial neck fractureInjury to brachial vessels or ulnar and median nervesIntra-articular entrapment of median nerve following close reduction of elbow dislocations in childrenReduction usually is undertaken as soon as possible. Under procedural sedation or general anesthesia, the physician encircles the patient’s upper arm with his or her hands, at the same time pushing the olecranon downward and anteriorly with the thumbs (Fig. 2). It is important to avoid hyperextension of the elbow, which may stress the neurovasculature further.Figure 2: Reduction of an elbow dislocationAfter successful reduction, the pulse, sensory, and motor functions of the forearm should be evaluated. Operative management, including open reduction, should be undertaken if there is residual deficit. Postreduction films should be examined carefully for evidence of bony injuries that were obscured on the initial radiographs. In addition, the joint should be immobilized. Due to swelling and potential development of compartment syndrome, inpatient observation may be warranted.? Radial head subluxation (nursemaid elbow) does not present with an elbow deformity and generally occurs in children much younger than the girl described in the vignette.American Board of Pediatrics Content Specification(s):Understand the mechanism of injury in dislocation of the elbowRecognize dislocation of the elbowProvide appropriate management for a child with dislocation of the elbowQuestion 8A 6-year-old girl is brought to the emergency department because of concerns about neck swelling. She has had several episodes of swelling in the same location, but this episode has persisted for 36 hours and is associated with redness and pain. She also has had a low-grade fever. She has had no vomiting, diarrhea, rash, or other associated symptoms. Further, she has not had any difficulty speaking, swallowing, or breathing. On physical examination, her temperature is 38.0°C, heart rate is 108 beats/min, respiratory rate is 20 breaths/min, and blood pressure is 92/60 mm Hg. The girl appears well and in no acute distress. Results of her head, ears, eyes, nose, and throat examination are normal. She has a 2x-4-cm cystic swelling just anterior to the mid-portion of the sternocleidomastoid muscle (SCM) with mild overlying erythema, and the mass is tender. A small punctum distally has scant fluid drainage.Of the following, the MOST likely cause of this mass is:?A.congenital lesion with superimposed infectionB.local neoplastic diseaseC.metastatic disease from distal malignancyD.regional infection with reactive lymphadenopathyE.traumatic injury with compression of local tissues Correct answer AThe location of the lesion, prior episodes in the same location, patient age, and signs of secondary infection described for the girl in the vignette support the diagnosis of an infected branchial cleft cyst. Malignant causes are unlikely given the patient’s age, recurrent episodes, nature and location of the mass, and lack of associated signs and symptoms. Lymphadenopathy and lymphadenitis are the most common causes of neck masses in young children, but the prior episodes of similar swelling, associated punctum, and absence of evidence of head or neck infection argue against this diagnosis. There is no history of trauma to suggest traumatic injury, and the examination findings are not consistent with a hematoma or local tissue compression.??Neck masses can be classified according to location (midline, anterior or posterior to SCM) or by cause (congenital, infectious/inflammatory, traumatic, neoplastic) (Tables 1 and 2). The age of the patient also can aid in narrowing the diagnostic possibilities.Table 1: Classification of Neck Masses by LocationMidline:Thyroglossal duct cystDermoid tumorThyromegaly (goiter, nodule, thyroiditis, cancer)Ectopic thyroid tissueSubmental lymphadenopathy or lymphadenitisLaryngoceleThymic cystAnterior to Sternocleidomastoid (Anterior Triangle):Lymphadenopathy or lymphadenitisBranchial cleft cystLipomaParotid (parotitis, hemangioma, tumor)HemangiomaCystic hygroma (10%)Posterior to Sternocleidomastoid (Posterior Triangle):Lymphadenitis/lymphadenopathyCystic hygroma (90%)Neoplasm, including metastasesMetastases (multiple primary sites including nasopharyngeal)Cervical ribCongenital lesions are found most commonly in infants and young children, although some remain clinically silent until adolescence or adulthood. Fibrous dysplasia of the SCM usually presents in infancy or early childhood as a firm mass within the SCM, and the patient’s head is tilted toward the affected side, with the chin turned in the opposite direction.??Malignant causes of neck mass are unusual in childhood, representing only 2% of those biopsied. Malignancies that are more likely in a pediatric patient include leukemia or neuroblastoma in infancy or early childhood and lymphoma, thyroid cancer, or rhabdomyosarcoma in older children and adolescents.??Lymphadenitis due to head and neck infection is most common in young children. Significant trauma to the head and neck region is more likely in adolescents and adults.???Common congenital lesions presenting as neck masses include thyroglossal duct cysts, branchial cleft cysts, cystic hygromas, and hemangiomas. Thyroglossal duct cysts are the most common congenital neck mass. These lesions represent remnants of the thyroglossal duct, which follows the course of the embryonic thyroid as it descends from the foramen cecum of the posterior tongue past the hyoid bone to its normal postnatal location. This duct typically atrophies prior to birth; failure of a portion of the duct to involute completely can result in a sinus, fistula, or cyst anywhere along its path in the midline neck. Due to the connection to the base of the tongue, such lesions rise or retract with tongue protrusion. Treatment is surgical excision of the cyst and the entire tract.? ??Branchial cleft cysts also are relatively common and represent remnants of any of the embryonic branchial pouches that fail to involute completely. The exact location of the cyst depends on the pouch involved; they can occur anywhere from the external auditory meatus to the anterior chest wall along the anterior border of the SCM. Second branchial cleft cysts are most common, occurring around the middle third of the SCM. Fistula tracts occasionally are associated with the cyst, leading to a visible punctum and fluid drainage, as described in the vignette. Both thyroglossal duct cysts and branchial cleft cysts are diagnosed commonly only after they become infected, leading to increased swelling, pain, redness, or pus drainage.? ??Cystic hygromas (also known as cystic lymphangiomas) arise from lymphatic sacs and 90% occur in the posterior triangle of the neck. These lesions are often large, soft, multicystic, and compressible. Unlike hemangiomas, cystic hygromas do not resolve spontaneously and require surgical excision. Hemangiomas are common congenital lesions that can occur anywhere, but most frequently are seen in the head and neck region. They have a characteristic red or bluish coloration and domed appearance. The lesions expand throughout the first 2 postnatal years but then regress spontaneously over several years in most cases.? ??Most congenital neck masses are diagnosed on clinical features alone, although imaging such as computed tomography scan or magnetic resonance imaging may be used to clarify the exact course and extent of the lesion. Ultrasonography can be useful in identifying fluid collections or clarifying relationships to vascular structures. ??In addition to the surgical management discussed previously, treatment of superimposed infection should be initiated with appropriate antibiotic therapy. Fibrous SCM dysplasia may be present at birth and is treated with aggressive physical therapy to avoid long-term cosmetic deformity.??When assessing a patient who has a neck mass, the clinician should exclude potentially life-threatening conditions or those that require emergent evaluation and treatment. Lesions that are large or in critical areas may compress airway or vascular structures and require urgent intervention. Traumatic neck lesions may result in rapidly expanding hematomas that can compress adjacent structures or can be associated with traumatic injuries to the airway, gastrointestinal tract, or major vessels. Evaluation with either computed tomography scan or surgical exploration should be considered. Unrecognized or inadequately treated infection in the neck can result in local spread to the mediastinum or prevertebral spaces or progression to sepsis. Malignancy is uncommon in pediatric neck masses; malignant lesions most commonly are firm, nontender, fixed to underlying tissues, progressively enlarging, and located in the posterior triangle or supraclavicular region. Malignancy also should be considered in any neck mass that fails to resolve in the expected time frame. Evidence of distant disease should be sought. Such evidence may include abdominal or chest mass; abnormal lymphadenopathy in other regions; or concerning skin, soft-tissue, or mucous membrane lesions.? ??Ancillary studies are not necessary in all cases of neck mass because many causes can be diagnosed on clinical grounds alone. As noted previously, imaging studies may be helpful in delineating the cause, extent, and exact course of the underlying lesion. Laboratory evaluation may be indicated if the diagnosis is unclear. Complete blood count and inflammatory marker measurement can be used for initial diagnosis or to assess response to therapy of suspected infections. Evaluation for possible malignant causes may include complete blood count, lactate dehydrogenase, uric acid, or specific tumor marker measurement. If malignant causes cannot be excluded based on examination, imaging studies, and laboratory evaluation, then the suspicious mass should be biopsied.? ?? ??American Board of Pediatrics Content Specification(s):??Know the etiology by age and the pathophysiology of neck masses??Plan diagnostic evaluation and initial intervention for patients with neck masses??Recognize serious and/or life-threatening causes of neck masses??Know the differential diagnosis of neck mass by locationOCTOBER 2010Question 1A 17-year-old boy who underwent craniopharyngioma resection 2 years ago presents to the emergency department with irritability and lethargy. Findings on physical examination include a temperature of 37.0°C, heart rate of 110 beats/min, blood pressure of 90/40 mm Hg, and oxygen saturation of 100% on room air. He has no recent history of trauma or illness. The bedside urinalysis by dipstick reveals a specific gravity of 1.005 but otherwise normal findings. Other laboratory findings of note are sodium of 160 mEq/L (160 mmol/L) and glucose of 120 mg/dL (6.7 mmol/L).Of the following, the MOST appropriate next step is to administerA.bolus infusion of 0.45% salineB.bolus infusion of 0.9% salineC.intranasal antidiuretic hormoneD.intravenous broad-spectrum antibioticsE.intravenous hydrocortisoneCorrect answer: BDilute urine in the face of intravascular volume depletion points to diabetes insipidus as the likely cause of hypernatremia and circulatory compromise for the child described in the vignette. Normal (0.9%) saline should be infused in the event of circulatory compromise. Subsequent rehydration should follow calculations of free water deficits and aim for correction over 48 to 72 hours. The volume needed to change an elevated serum sodium concentration is approximately 4 mL/kg of free water for each 1-mEq/L reduction in serum sodium. This should be accomplished over 48 hours. After restoring intravascular volume with normal saline, the usual rehydration fluid is 0.2% saline to 0.45% saline with 5% glucose. Care must be taken not to administer hypotonic fluid at too fast a rate because water will equilibrate across the cerebral blood-brain barrier almost immediately (long before the sodium is corrected), causing cellular edema and increased intracranial pressure.Although infusion of hydrocortisone and broad-spectrum antibiotics may be reasonable options in a child in adrenal crisis and septic shock, the urinary findings for this boy suggest deficiency of antidiuretic hormone (ADH) as the underlying cause of shock. Administration of ADH via the intranasal route is unlikely to restore intravascular volume in the face of circulatory compromise.Hypernatremia is defined as a serum sodium concentration greater than 145 mEq/L (145 mmol/L). Two primary mechanisms maintain sodium homeostasis: ADH and the thirst drive. Infants and other children who are dependent on others to provide them with fluids are at high risk for developing hypernatremia because of impaired access to free water. One cause of hypernatremia is poor feeding due to lack of human milk supply (free water deficit). The other is increased salt load from incorrect formula mixing, with the infant kidneys unable to excrete the increased sodium load due to ADH insensitivity. Other scenarios leading to hypernatremia include severe gastroenteritis and diabetes insipidus. In older children, both mechanisms usually must be affected for symptomatic hypernatremia to develop.Hypernatremia causes an efflux of fluid from the intracellular space to the extracellular space to maintain osmotic equilibrium. This leads to transient cerebral dehydration and cell shrinkage. If severe hypernatremia develops acutely, physical separation of the brain from the meninges may result, leading to rupture of the bridging veins and consequent intracranial or intracerebral hemorrhages. Venous sinus thrombosis can develop. Acute hypernatremia also has been shown to cause cerebral demyelinating lesions, the mechanism of which is unclear.Because most dehydration is from intracellular water loss, initial signs of intravascular volume depletion are less overt despite large free water deficits. Signs and symptoms of hypernatremia include doughy skin and altered central nervous system function (irritability, seizures, high-pitched cry). Agitation, confusion, and progression to coma may result.Urine volume should be measured and compared with fluid intake, and the urine osmolality and electrolyte values should be determined to assess the patient’s renal concentrating ability. Dilute urine (<800 mOsm/kg [800 mmol/L]) in the face of hypernatremia distinguishes urinary concentrating defects from intravascular depletion.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of hypernatremiaRecognize the signs and symptoms of hypernatremiaBe familiar with ancillary studies relevant to hypernatremiaRecognize the life-threatening complications of hypernatremiaPlan management of acute hypernatremiaQuestion 2A 3-month-old infant presents to the emergency department (ED) with fever of 1 day’s duration and three episodes of nonbilious and nonprojectile vomiting. He was born at 28 weeks’ gestation, developed hydrocephalus following an intraventricular hemorrhage, and had a ventriculoperitoneal shunt inserted. He is receiving phenobarbital for neonatal seizures that are well controlled. He has no other complications related to his prematurity. On physical examination, the alert and interactive infant has a temperature of 39.0°C, but all other vital signs are normal. As a part of his evaluation, the neurosurgery consultant aspirates the ventriculoperitoneal shunt reservoir. You perform a successful lumbar puncture after application of EMLA. Three hours later, you are asked to re-evaluate the infant because he appears “blue.” The infant definitely appears cyanotic, but his vital signs and clinical appearance are unchanged. His pulse oximetry reading is 94% on room air.Of the following, the MOST likely cause for this infant’s cyanosis isA.a cyanotic spell due to undiagnosed congenital heart diseaseB.impending respiratory failure due to cerebral herniationC.methemoglobinemia due to topical anestheticsD.septic shock due to meningitis and shunt infectionE.vasomotor instability of prematurity Correct answer CAlthough rare, methemoglobinemia has been described as a complication after the use of topical anesthetics in very young infants. This is the most likely cause of cyanosis for the infant described in the vignette. The normal pulse oximetry readings, alert appearance, and absence of change in vital signs in the presence of cyanosis for the infant rule out congenital cyanotic heart disease, respiratory failure due to cerebral herniation, and septic shock. Vasomotor instability is common in preterm infants and newborns but is not associated with central ical anesthetics are being used increasingly in the ED and have two primary advantages over infiltrative anesthetics: their application is painless and they do not distort the tissue. Major limitations are the slow onset of anesthesia, inadequate anesthesia, cost, and local and systemic adverse effects. The most commonly used topical anesthetic, EMLA (eutectic mixture of local anesthetics), is an oil-in-water emulsion of 2.5% pilocarpine and 2.5% lidocaine. EMLA is particularly useful for procedures such as intravenous catheterization, lumbar puncture, venipuncture, vaccinations, and other intramuscular injections. When applied over intact skin under an occlusive dressing for 45 to 60 minutes, it provides anesthesia to a depth of 5 mm. The newer topical anesthetics 4% liposomal lidocaine and 4% tetracaine gel offer some advantages over EMLA. The liposomal vehicle provides better skin penetration and tetracaine provides vasodilatation instead of vasoconstriction, which is useful during attempts at venous access. Oxygen-activated heating patch, iontophoresis, liposomal vehicles, laser devices to cause dermabrasion, and low-frequency ultrasonography are also being used for local topical ical anesthetics also are used over interrupted skin, especially during laceration repair. Two commonly used topical anesthetics for this indication are LET (4% lidocaine, 0.1% epinephrine, and tetracaine 0.5%) and TAC (0.5% tetracaine, 0.05% epinephrine, and 11.8% cocaine). TAC is used less frequently because of its higher incidence of systemic toxicity, primarily cardiac and central nervous system adverse effects related to the cocaine component. LET gel can be applied directly to wounds for 20 to 30 minutes; blanching around the wound is indicative of effect. Both LET and TAC are contraindicated in areas of end-arterial supply such as fingers.Methemoglobinemia, a potentially serious adverse effect of topical anesthetics, is due to the oxidant effect of lidocaine and prilocaine. Infants younger than 3 months of age; preterm infants; and children receiving other methemoglobin-producing agents such as nitrous oxide, sulfonamides, phenytoin, phenobarbital, and acetaminophen are at particular risk. Patients may present hours after anesthetic application with cyanosis, mottling, pallor, and in some instances, cardiorespiratory failure. The more frequent adverse effects of topical anesthetics are transient and include pallor, erythema, altered temperature sensation, and rash. Eye exposure can result in corneal abrasion, and systemic absorption can lead to cardiac and central nervous system toxicity. These effects are seen when the topical anesthetics are applied for long duration or over a large surface area. Cardiac toxicity results in arrhythmias and cardiac arrest; central nervous system toxicity is characterized by lightheadedness, headache, metallic taste, circumoral numbness or tingling, and seizures.American Board of Pediatrics Content Specification(s):Discuss the indications and contraindications for local and regional anesthesiaDiscuss the complications associated with local and regional anesthesiaQuestion 3A 10-day-old male infant presents to the emergency department with a 5-day history of vomiting. His parents report that the vomiting has become more frequent over the past day, initially occurring only with feedings and now occurring every hour. There is no blood or bile in the emesis. He had watery diarrhea three to four times a day for the first 2 days, but this has resolved; his last bowel movement was 2 days ago. The infant has not had a fever or any respiratory symptoms. He has become increasingly lethargic today and no longer is waking to feed or consuming his normal amount. He has not had a wet diaper in the past 12 hours. The infant was born at term to a G2P1-2 28-year-old woman. His Apgar scores were 8 at 1 minute and 9 at 5 minutes, and he went home 2 days after birth. He had been doing well, consuming 2 oz every 2 to 3 hours of a cow milk protein-based formula. His 2-year-old brother is healthy. On physical examination, the infant is lethargic and hypotonic. His temperature is 37.0°C rectally, heart rate is 190 beats/min, respiratory rate is 60 breaths/min, blood pressure is 68/50 mm Hg, and pulse oximetry reading is 98% on room air. His fontanelle is sunken, skin turgor is poor, and capillary refill is 4 seconds. Cardiovascular examination reveals tachycardia and diminished peripheral pulses but no murmurs or gallops. The remainder of his examination findings are normal. You obtain intravenous access and administer a rapid infusion of normal saline. Results of point-of-care electrolyte panel testing reveal:Sodium, 120 mEq/L (120 mmol/L)?Potassium, 7.2 mEq/L (7.2 mmol/L)?Chloride, 96 mEq/L (96 mmol/L)?Bicarbonate, 8 mEq/L (8 mmol/L)?Blood urea nitrogen, 28 mg/dL (10.0 mmol/L)?Glucose, 38 mg/dL (2.1 mmol/L)Of the following, the test that is MOST likely to reveal the diagnosis is:A.cranial computed tomography scanB.pyloric ultrasonographyC.serum insulin and C-peptide measurementD.serum 17-hydroxyprogesterone measurementE.sweat sodium and chloride concentration analysis Correct answer DThe infant described in the vignette is displaying signs, symptoms, and laboratory abnormalities consistent with an acute salt-losing crisis. For an infant of this age, the most likely cause is congenital adrenal hyperplasia (CAH), and measurement of adrenal hormones, including 17-hydroxyprogesterone, is the key to diagnosing the specific defect.? Analysis of sweat electrolytes is the diagnostic test of choice for cystic fibrosis, which would not be expected to present acutely in an infant of this age or cause hyponatremia and hyperkalemia, as?described in the vignette. Serum insulin and C-peptide measurement is appropriate in a case of unexplained hypoglycemia to identify excess endogenous or exogenous insulin. Head computed tomography scan may be indicated for an infant or child who presents with vomiting and lethargy to exclude intracranial pathology, but this child’s age, constellation of symptoms, and laboratory abnormalities suggest an endocrine cause.? Pyloric ultrasonography aids in the diagnosis of hypertrophic pyloric stenosis, which typically presents with progressive vomiting in an infant after 2 to 3 weeks of age and results in hyponatremia, hypokalemia, and metabolic alkalosis.CAH is a group of disorders resulting from inborn errors of adrenal cortical steroid synthesis. Multiple genetic defects, inherited in an autosomal recessive pattern, can result in various enzymatic deficiencies. Most of the adrenal steroidogenic enzymes are members of the cytochrome pigment 450 (P450) group of oxidases. The specific enzyme involved determines what steroid(s) are deficient or absent as well as what precursors proximal to the defect accumulate in higher than normal concentrations. The Figure outlines the adrenal steroid synthesis pathways and various enzyme defects.The most common form of CAH, accounting for nearly 95% of cases, results from a deficiency of the enzyme 21-hydroxylase, which occurs in approximately 1 in 15,000 live births in the United States. This enzyme catalyzes the hydroxylation of both progesterone (converted to deoxycorticosterone) and 17-hydroxyprogesterone (converted to 11-deoxycortisol). As seen in the Figure, this enzymatic defect blocks production of aldosterone and cortisol, and steroids immediately proximal to the blockade, including 17-hydroxyprogesterone, accumulate. In addition, accumulated precursors are shunted preferentially through unblocked pathways, leading to increased production of androstenedione and testosterone. Due to reduced concentrations of cortisol, lack of negative feedback to the hypothalamic-pituitary axis results in increased production of adrenocorticotropic hormone (ACTH). The increased ACTH concentrations further accelerate the unblocked adrenal steroid pathways, resulting in increased concentrations of testosterone and the aforementioned precursors to cortisol and aldosterone.The pathophysiology of CAH is based upon a combination of excessive concentrations of some adrenal steroids and deficiencies of others. In complete 21-hydroxylase deficiency, inadequate aldosterone results in hyponatremia, hyperkalemia, and acidosis. If left untreated, severe dehydration may progress to hypotension and cardiovascular collapse. This occurs most commonly in the second postnatal week. Insufficient cortisol results in impaired carbohydrate metabolism and blunted catecholamine response to stress. The increased concentrations of testosterone result in ambiguous genitalia in female infants, allowing earlier diagnosis. Males who are not diagnosed in infancy may present in later childhood with precocious puberty. Partial enzymatic defects (simple virilizing CAH) cause milder symptoms. Similar salt wasting is seen with deficiencies of 3 beta-hydroxysteroid dehydrogenase and 20,22-desmolase. Deficiency of 11 beta-hydroxylase and 17 alpha-hydroxylase do not result in salt wasting because of increased concentrations of the potent mineralocorticoid deoxycorticosterone; affected children often present with hypertension.The diagnosis of CAH is based on measurement of various adrenal cortical steroids to aid with identification of the specific enzymatic defect. Such measurement should include serum concentrations of 17-hydroxyprogesterone, dehydroepiandrosterone, androstenedione, and testosterone. Urinary 17-ketosteroids also can be measured. Whenever possible, these measurements should be obtained prior to initiation of therapy, although in severe cases of salt-wasting crisis, this may not be feasible, and resuscitative treatment should not be delayed. Expanded newborn screens now allow identification of some forms of CAH, but these results may not be available before the development of a salt-wasting crisis and falsely abnormal screening results may prompt emergency department referral for definitive testing. Prenatal diagnosis is possible based on elevated amniotic fluid concentrations of 17-hydroxyprogesterone.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of adrenal hyperplasiaRecognize signs and symptoms of adrenal hyperplasiaBe familiar with ancillary studies relevant to adrenal hyperplasiaQuestion 4A 27-day-old infant is referred from her pediatrician’s office to the emergency department for evaluation of fever. She was apparently well until 2 days ago, when she developed a tactile fever and runny nose. The maximum temperature measured at the pediatrician’s office rectally was 38.8°C. The only other complaint mentioned by the mother is decreased oral feeding for 1 day. The infant was born at term via normal vaginal delivery, and the perinatal period was uneventful. There is a strong family history of asthma in both parents. The pediatrician’s office had tested for respiratory syncytial virus (RSV) and influenza A and influenza B viruses; the RSV test result was positive. On physical examination, the well-appearing, alert infant has a rectal temperature of 39.0°C, heart rate of 158 beats/min, and respiratory rate of 32 breaths/min. Clear rhinorrhea is present, and chest examination reveals mild subcostal retractions. There is no other evidence of respiratory distress, and the infant has taken oral feedings in the emergency department.Of the following, the MOST appropriate next step isA.administration of intramuscular ceftriaxone and outpatient follow-up appointmentB.administration of nebulized albuterol and oral steroidsC.admission for intravenous RSV immunoglobulin plete sepsis evaluation, including urine, blood, and cerebrospinal fluid culturesE.reassurance, antipyretics, and outpatient follow-up appointment Correct answer DAlthough the infant described in the vignette has RSV infection, she has a small but not insignificant risk for occult bacteremia and serious bacterial infection, which necessitates a complete sepsis evaluation. Intravenous immunoglobulin against RSV has not been proven beneficial in the treatment of RSV, and there is no indication for a bronchodilator or corticosteroids. Despite the child’s well appearance, the use of parenteral antibiotics without a comprehensive evaluation for the source of fever is inappropriate. Because of the risk for bacterial infection, reassurance and an outpatient follow-up appointment are not appropriate.Fever accounts for up to 17% of all pediatric emergency department visits, and 20% of patients have fever without localizing signs. Most?febrile children (fever defined as rectal temperature ≥38.0°C if 3 months of age and younger or >39.0°C if 3 months to 3 years of age) have nonbacterial causes of fever and benign outcomes. A small but significant group has serious bacterial infection such as bacteremia, urinary tract infection, or meningitis.Prior to the 1990s, 3% to 12% of children who had fever without localizing signs had occult (or unsuspected) bacteremia, a term coined to describe the presence of bacteria in the blood of a child who does not appear to be clinically septic or toxic. Approximately 80% of occult bacteremia was caused by Streptococcus pneumoniae, 10% to 20% by Haemophilus influenzae type b, and the rest by Neisseria meningitidis and Salmonella. Patients who have occult bacteremia potentially could develop other serious bacterial infections, including meningitis, pneumonia, osteomyelitis, or septic arthritis. The risk of developing these outcomes varied with the pathogen. For example, the risk of developing meningitis was 2.7% to 6% with S pneumoniae but substantially higher for H influenzae type b and N meningitidis. Apart from the pathogen, other risk factors for developing serious bacterial infections were age of the child (risk increases as age decreases); height of temperature (risk increases as temperature increases); and presence of predisposing conditions such as prematurity, immune deficiency states, and certain congenital conditions such as Down syndrome.The successful introduction of two conjugate bacterial vaccines (Haemophilus influenzae type b [Hib] and heptavalent conjugate pneumococcal [PCV7]) has dramatically altered the evaluation of febrile children older than 3 months of age. Since the introduction of the Hib vaccine, the incidence of infection due to all types of Haemophilus has decreased by 96%, and more importantly, invasive disease due to Hib has decreased by 99%. Since 2000, when the American Academy of Pediatrics and Advisory Council for Immunization Practices recommended routine use of PCV7 pneumococcal vaccine, the incidence of occult bacteremia has decreased to less than 1%.Evaluation and treatment of children who have fever without localizing signs is particularly difficult because clinical examination by itself is not discriminatory. Research has revealed that relying on clinical scores, including a validated Yale Observation Score, is insufficient, especially in the very young febrile infant. To minimize the risk of missing occult bacteremia and serious bacterial infections, experts recommend a variety of management strategies. Traditional risk stratification strategies (including those that involve clinical and laboratory evaluation) have been derived primarily from three prospective studies, commonly referred to as the Rochester, Philadelphia, and Boston criteria. These strategies have been evaluated extensively, and components of each were endorsed for incorporation into guidelines for the evaluation of febrile infants by a consensus panel of experts in the early 1990s. These guidelines recommend laboratory evaluation (blood tests, urine tests, chest radiographs, and lumbar puncture for cerebrospinal fluid analysis), hospitalization, and empiric treatment with antibiotics for a subset of the youngest infants at highest risk for serious bacterial infections until bacterial cultures are reliably negative (minimum of 48 hours).The risk stratification strategies categorize febrile children into high-risk and low-risk groups based on initial screening tests, especially complete blood count (CBC), band count, neutrophil count, band-to-neutrophil ratio, and urinalysis. Although screening tests are integral in differentiating between febrile infants at low versus high risk for serious bacterial infection, many investigators have demonstrated that the test characteristics (eg, sensitivity, specificity) are too variable and the confidence intervals too wide to be clinically meaningful. For example, the most commonly performed screening test, the white blood cell count with a 15.0x103/mcL (15.0x109/L) cutoff, has been shown to have sensitivity ranging from 45% to 80% in detecting serious bacterial infection. Similar wide ranges have been demonstrated for absolute neutrophil counts and band counts.The addition of biomarkers for serious bacterial infection, such as C-reactive protein, procalcitonin, and interleukins, to routine screening tests has improved the test characteristics in some studies, but other investigators have failed to demonstrate similar results, leading many to question the applicability of specific biomarkers as indicators for serious bacterial infection. Preliminary evidence from small, single-center observational studies suggests that elevation of procalcitonin may be a better marker than white blood cell count in febrile infants. These studies need to be interpreted with caution and the results validated in a multicenter investigation.Advances in rapid viral detection technologies have led to their increasing use in the evaluation of young febrile infants, which is changing management strategies. Recent studies on the effect of detecting a viral cause for fever in well-appearing infants revealed that infants who had proven diagnoses of viral infection were less likely to receive empiric antibiotics, were less likely to undergo laboratory screening tests, and spent less time in the emergency department. Coinfection with a virus has been shown to decrease, but not eliminate, the risk of serious bacterial infection in young febrile infants. In a multicenter prospective study of 1,248 febrile infants 60 days of age and younger, the prevalence of serious bacterial infection in those who had positive tests for RSV was significantly less than in those who did not have evidence of RSV infection (7% versus 12.5%). The investigators concluded that although the prevalence of bacteremia and meningitis was lower in the RSV-infected group than in the RSV-negative group (1.1% versus 2.3%), the risk of these serious bacterial infections was still not inconsequential.Although evaluation of the febrile child continues to be controversial, a standardized algorithmic approach for the well-appearing child 3 months to 36 months of age no longer can be recommended because 1) the impact of the conjugate vaccines has lowered the prevalence in this age group to well below 1%, making screening strategies less cost effective; and 2) the lack of agreement with published guidelines and poor test characteristics of screening tests as well as the wide availability of multipanel viral assays has changed practitioner behavior. The impact of these vaccines, however, is less substantial in the very young infant due to the timing of administration.The risk of serious bacterial infection in febrile infants younger than 60 days of age (estimated range of 6% to 10%) is high due to an immature immune system. Because of this significant risk, experts in pediatric infectious diseases continue to recommend the use of laboratory tests to screen for occult bacteremia (risk estimated between 0.5% and 3%) and serious bacterial infection. In addition, in very young febrile infants, especially those 60 days of age and younger, the organisms responsible for occult bacteremia are different and reflect perinatal transmission of maternal flora (Escherichia coli, Klebsiella, Pseudomonas, Listeria monocytogenes, group B Streptococcus, Enterococcus, occasionally Enterobacter, Citrobacter, and Serratia). This age group is particularly difficult to evaluate, and the risks are higher because of relative immaturity of the immune system and variable impact of the conjugate vaccines.Based on the present state of literature on the evaluation of the febrile child, the following may be a reasonable approach to evaluation. Apart from a detailed history and physical examination, well-appearing febrile infants younger than 1 month of age need a comprehensive sepsis evaluation, admission to the hospital, and treatment with empiric antibiotics effective against the previously mentioned organisms (ampicillin plus cefotaxime). Well-appearing febrile infants 29 to 60 days of age also should undergo a comprehensive sepsis evaluation. Some practitioners may consider not performing a lumbar puncture in a small subset of well-appearing febrile infants, especially those who have positive viral tests. This approach is recommended if reliable follow-up within 24 hours can be assured and the results from the screening tests suggest the infant is at low risk for serious bacterial infection. If a decision is made to treat with outpatient parenteral antibiotics, a cerebrospinal fluid study must be performed. Well-appearing infants 60 to 90 days of age may be treated less conservatively, without lumbar puncture, on an outpatient basis. At the minimum, urine studies, including urine culture, should be obtained in this age group.Finally, a small but concerning increase in non-vaccine serotype pneumococcal disease among well-immunized population, especially serotype 19A, must be recognized. Community-based seroprevalence data should be used in the decision-making process for evaluation and treatment of febrile children. Clinicians should be aware of the potential changing epidemiology of occult bacteremia with both the increased incidence of non-vaccine serotypes and the pending use of PCV-13 vaccine.American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of occult bacteremiaRecognize signs and symptoms of occult bacteremiaBe familiar with ancillary studies relevant to occult bacteremiaPlan management of occult bacteremiaQuestion 5A 4-year-old boy who has a history of acute lymphoblastic leukemia (ALL) is brought to the emergency department with a 1-day history of high-grade fever without chills or rigors. He has come directly from the oncology clinic where he had a follow-up visit. His last chemotherapy was 2 days ago, and according to his oncologist, he has neutropenia, normal findings on chest radiography, and no other systemic symptoms. Apart from the ALL, he has been previously well. Physical examination in the emergency department reveals an alert but tired-appearing, well-hydrated child whose temperature is 39.0°C, heart rate is 155 beats/min, respiratory rate is 27 breaths/min, and blood pressure is 70/45 mm Hg. He has warm extremities, his pulses appear bounding, and his capillary refill is 2 to 3 seconds. He does not have any meningeal signs, and the rest of the physical examination findings are normal. His central venous site is clean and nontender and does not reveal any signs of infection. You order blood cultures and intravenous cefepime and vancomycin. While awaiting the antibiotics, the resident orders a 20-mL/kg normal saline bolus to run over 1 hour. The boy’s capillary blood gas reveals a pH of 7.4, PCO2 of 30 mm Hg, lactate of 3 mg/dL (0.3 mmol/L), and glucose of 82 mg/dL (4.4 mmol/L).Of the following, the MOST appropriate next step in management is toA.add intravenous amphotericin BB.administer 5 mL/kg packed red blood cellsC.administer glucocorticoidsD.increase the rate of normal saline bolus infusionE.start a dopamine infusionCorrect Answer: DAny febrile neutropenic patient is at a high risk for developing septic shock. The boy described in the vignette has a rapid heart rate, slightly prolonged capillary refill time, elevated lactate, and low blood pressure for age, suggesting the early “warm” phase of septic shock. Early goal-directed therapy has been shown to improve outcomes in sepsis and septic shock. The guidelines recommend aggressive intravenous use of isotonic crystalloids as fast as possible. Each 20-mL/kg aliquot of normal saline can be infused over 5 to 10 minutes, and up to 40 to 60 mL/kg can be infused rapidly over 1 hour. If patients fail to show any response to such aggressive fluid management, vasopressors (eg, dopamine) may be indicated. Packed red blood cells may be administered to improve the oxygen-carrying capacity. Hypoglycemia is a known complication of sepsis, but this boy’s serum glucose concentrations are normal. Antifungal agents may be useful if patients fail to respond to broad-spectrum antimicrobial therapy, if fever persists, or if there are any clinical signs suggestive of a fungal infection. Glucocorticoid use in septic shock is controversial and definitely not indicated before intravascular volume is restored.??Shock is defined as a pathologic state characterized by an inability of the body to meet its metabolic demands, namely, oxygen and?nutrients. Normally, tissue metabolic demands are maintained by a complex interplay between the central pump (heart and cardiac output), balance between blood flow and metabolic demand at the tissue level, and the oxygen content of the blood. The oxygen content of the blood is determined by the hemoglobin concentration and its oxygen-carrying capacity. Cardiac output is the product of stroke volume and heart rate. Stroke volume is determined by preload (filling volume of the ventricle), cardiac contractility (Starling curve), and afterload (resistance against which the heart is pumping blood into the systemic circulation). Children have relatively fixed stroke volumes and, hence, are dependent on heart rate to increase cardiac output. Consequently, tachycardia is an early sign of shock as the compensatory mechanisms try to maintain tissue perfusion. In the initial stages of shock, increased vascular resistance maintains perfusion pressure (blood pressure) as a compensatory mechanism while blood is shunted away from the skin, kidneys, muscle, and splanchnic organs to the vital organs, namely, the heart, adrenal cortex, and brain. This stage of compensated shock is characterized by a normal blood pressure in the presence of decreased tissue perfusion and manifests clinically as cool extremities, thready pulse, and oliguria. Failure of compensatory homeostatic mechanisms leads to decompensated shock, which is characterized by a drop in blood pressure. If this state remains untreated, the patient progresses to refractory or irreversible shock.??Shock can be classified as hypovolemic (due to loss of intravascular volume following fluid or blood loss), cardiogenic (alteration of myocardial contractility due to conditions such as myocarditis and cardiomyopathy), and distributive (due to vasodilatation and pooling of blood in the peripheral vasculature). Distributive or vasodilatory shock results from a decrease in systemic vascular resistance, with abnormal distribution of blood flow within the microcirculation and inadequate tissue perfusion. It can lead to functional hypovolemia, which decreases the preload. Distributive shock generally is associated with a normal or increased cardiac output. Although sepsis is the most common cause of distributive shock in children, other causes include anaphylaxis and neurogenic shock. Neurogenic shock is a rare, usually transient disorder that follows acute injury to the spinal cord or central nervous system and results in loss of sympathetic venous tone. It is important to note that reflex tachycardia is absent in patients who have neurogenic shock.??The Society of Critical Care Medicine has clearly defined criteria for systemic inflammatory response syndrome (SIRS), sepsis, severe sepsis, and septic shock. Infection is characterized by an inflammatory response to microorganisms or the invasion of normally sterile host tissue by those organisms; bacteremia is defined as the presence of viable bacteria in the blood. SIRS follows a dysregulated host inflammatory response and is characterized by the presence of two or more of the following:Temperature >38.5?C or <35.0?CHeart rate >90 beats/minRespiratory rate >20 breaths/min or PaCO2 <32 mm HgWhite blood cell count >12.0x103/mcL (12.0x109/L), <4.0x103/mcL (4.0x109/L), or >10% immature (band) formsSIRS may result from infection or have noninfectious causes such as trauma, pancreatitis, or burns. Patients can progress from SIRS to sepsis, septic shock, and severe septic shock. A patient is said to have sepsis if there are clinical signs of SIRS in the presence of a culture-proven infection or an infection identified by visual inspection. Severe sepsis exists if there is sepsis plus at least one of the following signs of organ hypoperfusion or dysfunction:Mottled skinCapillary refill >3 secondsUrine output <0.5 mL/kg for at least 1 hourLactate >18 mg/dL (2 mmol/L)Abrupt change in mental statusAbnormal electroencephalographic (EEG) findingsPlatelet count <100x103/mL (100x109/L)Disseminated intravascular coagulationAcute lung injury or acute respiratory distress syndromeCardiac dysfunction, as defined by echocardiography or direct measurement of the cardiac indexSeptic shock exists if there is severe sepsis plus one or both of the following:Systemic mean blood pressure <60 mm Hg (or <80 mm Hg if the patient has baseline hypertension) despite adequate fluid resuscitationMaintaining the systemic mean blood pressure >60 mm Hg (or >80 mm Hg if the patient has baseline hypertension) requires dopamine?>5 mcg/kg per minute, norepinephrine?<0.25 mcg/kg per minute, or epinephrine?<0.25 mcg/kg per minute despite adequate fluid resuscitationMultiple organ dysfunction syndrome is seen in late stages of septic shock.??Sepsis results from an exaggerated systemic inflammatory response induced by infecting organisms, and inflammatory mediators are the key players in the pathogenesis. The cell wall components lipopolysaccharide (gram-negative bacteria), peptidoglycan (gram-positive and gram-negative bacteria), and lipoteichoic acid (gram-positive bacteria) induce a variety of proinflammatory mediators, including cytokines such as interleukins, tumor necrosis factor, interferon-gamma, macrophage migration inhibition factor, granulocyte colony-stimulating factor, and granulocyte macrophage colony-stimulating factor. Release of such mediators leads to neutrophil and macrophage activation, increase in vascular permeability, direct endothelial injury, and pulmonary epithelial injury. Simultaneous activation of the complement system aids in clearance of the infecting microorganisms but also enhances tissue damage. Hypotension, the cardinal manifestation of sepsis, occurs via induction of nitric oxide. The arterial vasodilation-induced hypotension in septic shock is insufficiently compensated by an increase in cardiac output, and patients may deteriorate rapidly from compensated to decompensated shock. In addition, endotoxins and cytokines suppress cardiac output by causing myocardial dysfunction, pulmonary hypertension, coronary blood flow abnormalities, and downregulation of beta receptors. Endothelial damage activates the coagulation pathway while simultaneously increasing activity of protein C, which functions as an antithrombotic agent in the microvasculature. The imbalance between inflammation, coagulation, and fibrinolysis results in widespread coagulopathy and microvascular thrombosis, ultimately leading to multiple organ dysfunction and death. A dysregulation of peripheral circulation leads to disparity in oxygen extraction at the tissue level or lactate acidemia that, in turn, results in cell death, apoptosis, and organ failure. As the condition progresses, multiple organ failure is seen in the form of renal failure, hepatic dysfunction, acute respiratory distress syndrome, central nervous system manifestations in the form of encephalopathy, myocardial depression, immunosuppression, ileus, and gastrointestinal bleeding. Readers are directed to the goal-directed management of sepsis and distributive shock in the consensus guidelines produced by the Society of Critical Care Medicine and other participating organizationsAmerican Board of Pediatrics Content Specification(s):?Understand the pathophysiology of distributive shockQuestion 6Emergency medical services personnel bring in a 2-year-old child who has acute myelogenous leukemia and was found unconscious at his home 5 minutes from the hospital. The paramedics intubated him on the scene and began cardiopulmonary resuscitation (CPR). On arrival to the emergency department, the nurse verifies that there is no pulse and begins chest compressions at 100 times/min. You confirm endotracheal tube placement and take over ventilation.Of the following, the American Heart Association (AHA) recommended guideline for ventilation for this patient is CLOSEST toA.5:1 ventilation-to-compression ratio, synchronizedB.15:2 ventilation-to-compression ratio, synchronizedC.30:2 ventilation-to-compression ratio, synchronizedD.8 to 10 ventilations/min, unsynchronizedE.12 to 20 ventilations/min, unsynchronizedCorrect answer: DBasic Life Support (BLS) should be begun on patients who have an acute event that leads to life-threatening derangements in cardiac or pulmonary function. BLS refers to the recommended first interventions in these patients. This term is often used synonymously with cardiopulmonary resuscitation (CPR), although CPR is also used in advanced forms of life support. The primary difference between basic life support and advanced life support is the addition of medications and a variety of medical equipment to the advanced life support algorithms.The 2005 AHA guidelines for CPR for health-care clinicians recommend that ventilation be provided 8 to 10 times/min in patients in full cardiac arrest and in whom an advanced airway is in place, such as the boy described in the vignette. There is no recommendation to synchronize breaths to compressions in cycles once an advanced airway is in place. This recommendation was designed to maximize the number of compressions per minute delivered to the child, while minimizing the chance of overventilation. Excessive ventilation leads to increases in intrathoracic pressure that impedes venous return to the heart. Such impedance can cause a reduction in cardiac output, cerebral blood flow, and coronary perfusion. In addition, overventilation can lead to air trapping and barotrauma, including pneumothorax, especially in patients who have small airway obstruction. Finally, it can increase the risk of regurgitation and aspiration. Interruptions in chest compressions are associated with a decreased rate of return of spontaneous circulation.In the absence of an advanced airway, if two clinicians are present, a ratio of 15 compressions to 2 ventilations is recommended. This increased compression-to-ventilation ratio compared with guidelines before 2005 was made because of evidence that a number of chest compressions are necessary to increase coronary perfusion pressure. With every pause in compressions, the coronary perfusion pressure drops. When only a single clinician rescuer is available, the recommended compression-to-ventilation ratio is even higher at 30:2. The Neonatal Resuscitation Program recommends a compression-to-ventilation ratio of 3:1 for newborns in full cardiac arrest because they have greater ventilatory needs.The 2005 AHA guidelines for basic life support also emphasize the delivery of high-quality chest compressions. For infants, chest compressions should be delivered with a two-finger technique, just below the intermammary line. With two clinicians present, the two-thumb-encircling the chest technique provides better coronary perfusion. For toddlers and children, the heel of either one or two hands may be used. The lower half of the sternum should be compressed, taking care not to compress the xiphoid, which may cause liver or splenic injury. New guidelines emphasize “push hard” (? to ? the anteroposterior diameter of the chest in infants), “push fast” (100 times/min in all age groups, minimizing interruptions), and release the chest completely to allow full recoil. If the patient is not on a firm surface, a back board should be used to improve the efficacy of chest compressions.American Board of Pediatrics Content Specification(s):Discuss the indications for basic life support proceduresDescribe the key steps and potential pitfalls in performing basic life support proceduresDiscuss the complications associated with basic life support proceduresKnow the anatomy and/or pathophysiology relevant to basic life support proceduresQuestion 7A 3-year-old girl is brought to the emergency department (ED) by her parents after ingesting an unknown quantity of her mother’s clonidine. The child apparently ingested the pills before being put down for a nap. The mother discovered the open pill bottle 3 hours later when the girl did not awaken as usual from her nap. She is now intermittently sleepy. When awoken, she fights with all clinicians and is difficult to calm. She rips off her monitor leads and screams, “I want to go home.”Of the following, the BEST approach to gastrointestinal decontamination in the ED for this patient isA.no gastrointestinal decontaminationB.physical restraint, followed by nasogastric administration of activated charcoalC.physical restraint, followed by whole bowel irrigationD.rapid sequence intubation, followed by nasogastric administration of activated charcoalE.rapid sequence intubation, followed by nasogastric lavage and administration of activated charcoalCorrect Answer: AThe child described in the vignette has ingested a potentially toxic dose of clonidine, a centrally acting alpha-agonist. Expected physiologic effects include bradycardia, hypo- or hypertension, respiratory depression, and depressed mental status. The risks and benefits of gastric decontamination must be balanced in this situation. The current joint recommendation from the American Academy of Clinical Toxicology (AACT) and European Association of Poisons Centres and Clinical Toxicologists (EAPCCT) is to use activated charcoal only when a potentially toxic substance known to be adsorbed by charcoal has been ingested within 1 hour of presentation to the ED. They state in their 2006 position statement that there continue to be? “… no satisfactorily designed clinical studies assessing benefit from single-dose activated charcoal to guide use of this therapy.” Despite these guidelines, regional poison center data show that patients continue to receive activated charcoal multiple hours after ingestion in United States emergency departments. No data show that this improves outcomes in poisoned patients. Expert toxicologic consultation is recommended if a substance that delays gastric emptying has been ingested. In this situation, there may be a role for administration of charcoal more than 1 hour after the ingestion.The most common adverse effect after administration of activated charcoal to children who have toxic ingestions is vomiting, which occurs in about 20% of patients. Its occurrence appears to increase when activated charcoal is coadministered with sorbitol. Aspiration of charcoal has been documented, even in alert patients. Finally, some evidence suggests that charcoal aspiration is potentially more toxic to pulmonary epithelium than gastric contents alone.The use of activated charcoal is specifically contraindicated in the absence of an intact or protected airway. The child in the vignette ingested the medication at least 3 hours before presentation, long past the window during which activated charcoal is most effective. Further, her waxing and waning mental status make charcoal administration without protection of the airway potentially inadvisable. Prophylactic intubation at this point would be undertaken solely to administer charcoal. In this setting, the risks probably exceed the benefits, and gastrointestinal decontamination should not be attempted. Treatment should be primarily symptomatic, supporting respiration, heart rate, and blood pressure, as needed, until the effects of the ingestion wear off. Naloxone can be administered to treat the central nervous system and respiratory depression that accompany clonidine toxicity, although its therapeutic effect is variable and large doses sometimes are needed.According to the most recent position statement in 2004 from the AACT and EAPCCT, based on available evidence, gastric lavage should not be used routinely, if ever, in the management of poisonings. Results from clinical outcome data point more strongly to lack of benefit. Adverse effects include vomiting, gastrointestinal perforation, and aspiration.Whole bowel irrigation (WBI) is a gastric decontamination technique involving introduction of large volumes and flow rates of polyethylene glycol balanced electrolyte solution into the gastrointestinal tract either orally or by nasogastric tube. There are no reported controlled trials evaluating the efficacy of WBI. Based on volunteer data, the AACT and EAPCCT acknowledge that it has potential benefit to patients who present with toxic ingestions of iron (for which there are no other gastrointestinal decontamination options) or sustained-release or enteric-coated medications, especially if the patient presents more than 2 hours after ingestion. In addition, some toxicologists recommend that its use be considered after the ingestion of arsenic, zinc, packets of illicit drugs, or transdermal patches.Activated charcoal adsorbs drug onto binding sites, thereby minimizing its systemic absorption. The usual dose is 1 g/kg, with 50 or 100 g recommended for an adolescent or adult. Sometimes it is coadministered with a cathartic, although the AACT and EAPCCT indicate that little evidence shows that such coadministration improves outcome. They recommend that, if used at all, only a single dose of activated charcoal with sorbitol be administered, even when a multidose activated charcoal regimen is appropriate. Toxic substances not well adsorbed by charcoal include iron, lithium, alcohols, cyanide, most solvents, and most hydrocarbons. Multidose activated charcoal, used as a form of gastrointestinal dialysis, should be reserved for drugs for which this approach has proven efficacy. The AACT and EAPCCT recommend that its use be considered only for toxic ingestions of phenobarbital, carbamazepine, phenytoin, digoxin, salicylates, and theophylline.American Board of Pediatrics Content Specification(s):Know the role of activated charcoal, including substances not adsorbed by charcoalQuestion 8A 7-year-old boy presents to the emergency department because he is not sleeping well. His guardian states that the boy?frequently wakes?up crying and has?difficulty going back to sleep. There is a vague history of constipation. He has been clingier lately and appears to be less engaged with his friends. He denies fevers, rash, vomiting, and medication use. An older sibling has a history of depression. The physical examination yields no findings of note. Results of laboratory studies provided by the child’s pediatrician include normal complete blood count, electrolytes, C-reactive protein, and urine culture.Of the following, the MOST appropriate potential cause to consider for this boy’s presentation isA.acute lymphoblastic leukemiaB.acute psychosisC.Addison diseaseD.botulism exposureE.sexual abuse Correct answer ENormal electrolyte values would be unusual in Addison disease, the age and presentation for the boy described in the vignette are not appropriate for botulism, there is no evidence of psychosis in the child, and normal complete blood count findings rule out acute lymphoblastic leukemia. Accordingly, of the options given, sexual abuse would be the most likely potential cause of the symptoms reported for this boy. Clinicians should be aware that child sexual abuse is more prevalent than many might suspect. In 2006, of the 3.6 million child abuse or neglect investigations reported to child protective services, 900,000 were substantiated and 79,000 were classified as sexual abuse. An estimated 25% of females and 16% of males are sexually abused before the age of 18. A recently released study from the Department of Health and Human Services (DHHS) notes that the incidence of sexual abuse has declined (38% decrease in cases) between 1993 (~218,000) and 2005/2006 (~135,000), with sexual abuse accounting for approximately 24% of the total child abuse volume. It is important to note, however, that not all allegations or caregiver suspicions of sexual abuse are grounded in fact.Sexual abuse implies contact of a sexual nature that may not be comprehended or consensual. It can include genital, anal, or oral contact, fondling, penetration, or masturbation as well as exhibitionism, pornography, and voyeurism. Some children who have disabilities may be at higher risk for sexual abuse due to their limited mobility, increased number of adult caregivers, and decreased ability to provide details of the events, although this was not evident in the 2006 DHHS report.Concern for sexual abuse is heightened when allegations are part of the history or there is evidence of genital or other sexually related trauma. However, much sexual abuse is not that evident on history or physical examination. Clinicians must be attuned to more subtle information. Clues to potential abuse include disclosures made to others, suspicion of abuse by a caregiver (in or out of the home), and behavioral or physical clues during an evaluation performed primarily for a seemingly unrelated issue.Patients who have acute sexual abuse complaints or concerns should receive a complete medical evaluation, including history and physical examination. Evidence gathering and specialty assessment may be required (eg, social services, sexual abuse team, child protective services, police). Clinicians should recognize that disclosure of sexual abuse can be very difficult and perhaps painful to the patient and may be very disruptive to the home environment. Discussion with patients and families should include language about “doing the right thing” and avoid potential escalation of guilt or concern about causation by the patient. Discussions should not be described as, or promised to be, “confidential” because the information will need to be disclosed if the findings suggest abuse.The history obtained from the patient may be the most important initial component of a potential sexual abuse evaluation. Suspicions of abuse may be noted by specific disclosure (“I was touched by …”); concerns of others; specific complaints of genital, anal, urinary, or other problems; encopresis; fecal retention; sexual history information; behavioral-related complaints such as sleeping abnormalities; mood changes such as depression or withdrawal; suicidal ideation; changes in peer relationships; sexualized behaviors; and dietary changes. Questions should be asked in a nonleading format, using age- and culturally specific techniques. Information should be recorded in a clear and detailed format because such documentation may be used in many different areas over time. Audio and video recording can be useful to ensure appropriate documentation of information. The strongest evidence in the history often is a consistent recollection of events by the abused patient, preferably without a caretaker present, if age permits (>~3 years of age).Emergency department physical examination should be undertaken to ensure there is no overt or dangerous physical condition and to obtain evidentiary materials if the assault has taken place within the last approximately 72 hours (varies by local protocols). Use of local/state protocols should help guide triage, ideal timing and location for full sexual assault examinations, and for the evidence chain. Findings on the physical examination, although often normal even with a strong history of sexual abuse, may raise the consideration of sexual abuse in those who have no overt complaints (eg, acute or healed anogenital injury, vaginal discharge [often profuse yellow-green], odors or bleeding, urethral discharge, abnormal behavioral presentations, pregnancy). Of note, many physical examination findings can mimic abuse, including, but not limited to, urethral prolapse, straddle injuries, labial adhesions, anal fissures, self-inflicted or accidental trauma, local dermatitis, and vulvovaginitis.Certain laboratory results may raise suspicion of sexual abuse. These include evidence of sexually transmitted infections such as Neisseria gonorrhoeae, Chlamydia, human immunodeficiency virus, trichomonas, syphilis, genital warts, and genital herpes.All states require reporting of suspected child abuse or neglect. It is important to recognize that this need not be a proven or certain event, just suspected.American Board of Pediatrics Content Specification(s):Recognize the ways in which children reveal sexual abuseNOVEMBER 2010Question 1A 4-month-old girl presents to the emergency department after a reported fall at home. Her mother states that the girl rolled off the bed and fell about 0.6 m to a carpeted floor approximately 8 hours ago. She cried immediately and has had repeated episodes of emesis since then. Her mother is concerned because she has become more sleepy and irritable since the injury, and now her arms and legs seem stiff. On physical examination, the alert but irritable child seems to have increased tone in both arms and legs. She has a small abrasion over her left supraorbital ridge but no other signs of trauma. There is no evidence of a seizure either by history or on physical examination. Her temperature is 37.2°C, respiratory rate is 34 breaths/min, heart rate is 124 beats/min, blood pressure is 84/52 mm Hg, and pulse oximetry reading is 99% in room air. You obtain cranial computed tomography (CT) scan (Fig. 1).Figure: CT scanComplete blood count, including platelets, prothrombin time (PT), and partial thromboplastin (PTT) time, and electrolyte evaluation yield normal results. Glucose measures 72 mg/dL (4.0 mmol/L). Skeletal survey does not reveal any fractures. You consider contacting child protective services.Of the following, the laboratory test that is MOST likely to yield a diagnosis is assessment ofA.fibrinogen and fibrin degradation products (FDP)B.serum ammonia and urine organic acidsC.serum and urine toxicologyD.serum pyruvate and lactateE.urinalysis and urine cultureCorrect answer: BThe child described in the vignette has bilateral frontal subdural fluid collections and a signal density and brightness most consistent with an acute hemorrhage in the high left parietal area. Although the fall seems minor, less force may be needed in the setting of chronic dilatation of the subdural space to cause bleeding from the bridging veins that can lead to an “acute on chronic” picture of the subdural collections. Whenever the history does not fit the findings on physical examination or the nature of the injuries discovered during the evaluation, nonaccidental injury must be considered. The CT findings shown for this girl may be consistent with “acute on chronic” abuse, but most children who have hypertonia in the setting of acute nonaccidental injury are not alert on examination. This should prompt the physician to consider a broader differential diagnosis, including inborn errors of metabolism. For this child, the subdural collections and clinical picture of increasing fussiness and increased tone are consistent with an acute decompensation in glutaric aciduria type 1 (GA1).GA1 is one of the organic acidurias that typically manifests later in infancy. Organic acidurias (also known as organic acidemias) are?diagnosed by finding increased excretion of organic acids in the urine due to deficiencies in the metabolic pathways that are responsible for the breakdown of amino acids. Assessment of serum ammonia and urine organic acids is most helpful in diagnosing GA1.A bleeding diathesis and abnormal fibrinogen and FDP findings would be unusual in the presence of normal PT and PTT results. Serum and urine toxicology screens may be considered, but they are unlikely to lead to an underlying diagnosis in this infant. Pyruvate and lactate abnormalities are not associated with subdural fluid collections. This afebrile patient does not exhibit any signs of urinary tract infection.Most organic acidurias (eg, methylmalonic aciduria, propionic acidemia, and isovaleric acidemia) become evident in the neonatal period. Children who initially appear well at 1 to 2 weeks of age develop poor feeding, vomiting, hypotonia, and lethargy that may progress to coma. They develop a severe anion gap metabolic acidosis and may appear septic. Hyperammonemia, hypoglycemia, and electrolyte abnormalities due to volume depletion occur. Often, affected children have bone marrow suppression and may have pancytopenia. Intravenous fluid rehydration and the provision of adequate glucose to stop catabolism and sodium bicarbonate to correct acidosis should be provided. Treatment of the hyperammonemia with sodium benzoate, sodium phenylacetate, or intravenous arginine may be indicated. Consultation with a metabolic specialist is recommended.GA1 is caused by a deficiency in glutaryl-CoA dehydrogenase, which is required for the breakdown of lysine, hydroxylysine, and tryptophan. GA1 rarely presents in the newborn period. Affected children usually have an episode of decompensation characterized by ketoacidosis, hyperammonemia, hypoglycemia, and encephalopathy. Typically, the crises are triggered by an intercurrent illness, although there have been reports of episodes associated with or the diagnosis being made after a minor traumatic event.Children who have GA1 exhibit macrocephaly, and as many as 30% have subdural hemorrhages or effusions. Acute bleeding into the areas of subdural effusion may be mistaken for nonaccidental injury. In the absence of additional abuse findings (eg, bruises, fractures) in a young infant, metabolic disorders need to be excluded. It is believed that the widening of the subdural spaces by the pre-existing effusion stretches the bridging veins and results in acute hemorrhages with relatively minor trauma. The most devastating manifestation of this disease is acute symmetric necrosis of the striatum and basal ganglia, which is irreversible and results in severe dystonia. Many, but not all, children have cognitive defects, and seizures are common. Over time, disease progression leads to further cerebral atrophy and a characteristic picture on central nervous system imaging of a marked widening of the Sylvian fissure and worsening subdural fluid collections (Fig. 2).Figure 2: Noncontrast CT scan shows progressive loss of cerebral volume, with marked increase in all cerebrospinal fluid-containing spaces. Changes are most notable at temporal lobes bilaterally. Fluid and calorie restriction in children who have GA1 may worsen the metabolic crisis and hasten the onset of the necrosis within the basal ganglia and the accompanying dystonia. Intravenous fluids, appropriate glucose concentrations to stop catabolism, and treatment of hyperammonemia are indicated. Thorough evaluation for nonaccidental injury should be undertaken, but a broad differential diagnosis and consideration of other entities must not be omitted.American Board of Pediatrics Content Specification(s):Recognize the signs and symptoms of clinical conditions? characterized by the excessive accumulation of one or more organic aciduriaQuestion 2A 6-year-old boy presents to the emergency department in the late evening with abdominal pain. The emergency department physician is concerned for appendicitis and consults the on-call surgeon. The surgeon, who is home, instructs the emergency department physician to order a complete blood count and ultrasonography. Both results are normal, but the emergency department physician remains concerned for appendicitis and requests the surgeon to evaluate the patient.Of the following, the STRONGEST argument to compel the surgeon to come examine the patient is thatA.he/she has a duty to treat the patientB.the laboratory and imaging test results are not specificC.the on-call surgeon at home is not held to the same standard of careD.the patient is likely to experience appendiceal perforation before the morningE.the standard of care includes a computed tomography scanCorrect answer: ADuty to treat is defined as a mutual agreement that medical care has been requested by the patient and offered by the physician. Specialists on call to an emergency department are not exempt from this duty, even though they may not meet the patient face-to-face. This duty provides the strongest argument for the surgeon described in the vignette to come to the hospital to examine the patient. In addition, the Emergency Medical Treatment and Active Labor Act (EMTALA) mandates that an on-call practitioner must respond to a request for on-site evaluation of patients in the emergency department within a reasonable period of time.??Although the laboratory and imaging tests for appendicitis may not be specific, they do not influence the requirement for the on-call?surgeon to present to the emergency department when requested. ?Computed tomography scan may be a useful adjunct, but without more data about local resources, it is difficult to determine whether it is the standard of care. Patients may have bad outcomes (appendiceal perforation) despite physicians meeting the standard of care.??For nearly 2 decades, data from the American Academy of Pediatrics (AAP) Periodic Survey of Fellows has pointed to an increasing trend in the number of malpractice claims, with nearly one third of all pediatricians having been involved in a lawsuit at some point in their career and one tenth while still in training. It is essential for the practicing physician to be aware of issues surrounding potential malpractice claims.??Malpractice law in the United States is based on the concept of negligence. For a physician to be found negligent, four conditions must be met:There must be a duty to treat, with the physician required to have established some relationship with, and responsibility to, the patient.?Failure to provide therapy consistent with the standard of care.Injury to the patient.Causal relationship between the injury and the breach in standard of care.Standard of care is defined as: “that degree of care which a reasonably prudent person with similar training should exercise in similar circumstances.” Today, courts generally have held that residents in training have the same responsibility to provide standard of care as attending physicians.??Establishment of duty to treat for on-call physicians is a somewhat controversial issue. "Professionals do not owe a duty to exercise their particular talents, knowledge, and skill on behalf of every person they encounter in the course of the day ... It is only with a physician's consent, whether express or implied, that the doctor–patient relationship comes into being." (St. John v. Pope, Texas Supreme Court, 1995) It is generally accepted, however, that when a physician voluntarily takes call for a hospital, he or she has a duty to treat the patient when his or her service is called upon. When this duty is breached, the physician is potentially liable to any damages that result from such neglect.??American Board of Pediatrics Content Specification(s):??Understand the concept of duty to treat??Understand the concepts regarding medical malpractice: negligence, standard of care, harm??Understand the ramifications of failure to uphold the principle of duty to treatQuestion 3A 12-year-old boy who has a long-standing seizure disorder that is being treated with valproic acid presents to the emergency department with the complaint of unrelenting abdominal pain. He has been vomiting intermittently for 2 days. He describes the pain as constant. He has had no fever, ill contacts, or breakthrough seizures. On physical examination, the thin, anicteric boy is in obvious discomfort, his abdomen is tender to palpation in the upper quadrants with some voluntary guarding, and he prefers to sit up.? Other findings are within normal parameters.Of the following, the study(ies) indicated at this time that are MOST likely to reveal the underlying cause of this boy’s findings areA.abdominal computed tomography scanB.aspartate aminotransferase, alanine aminotransferase, and coagulation profileC.magnetic resonance cholangiopancreatography (MRCP)D.serum amylase and lipase concentrationsE.serum valproic acid concentrationCorrect answer: DThe boy described in the vignette has signs and symptoms of pancreatitis. The classic signs and symptoms include abdominal pain, nausea, vomiting, and mid-epigastric or upper quadrant pain that sometimes radiates to the back, shoulder, or flank. Although he does not have fever, 50% of patients who have pancreatitis may have low-grade fever of less than 38.5°C. In addition, this boy is taking a medication known to trigger pancreatitis. Individual measurement of serum amylase and lipase concentrations can yield false-negative and false-positive results in this clinical condition, but the combination has a reported sensitivity of 94% for detecting pancreatitis. Elevations in amylase can be seen in myriad disorders, including salivary gland pathology, other intra-abdominal pathology, or systemic illness. Lipase elevations are seen with perforated peptic ulcer and bone fracture with pulmonary fat embolism. A threshold of three times the normal values for amylase and lipase provides increased specificity, without a substantial impact on sensitivity.Aspartate aminotransferase and alanine aminotransferase are more nonspecific assessments than amylase and lipase; they can be elevated in a greater variety of conditions. Valproic acid causes pancreatitis idiosyncratically, so knowing this boy’s valproic acid concentration will not help with the diagnosis. Further, he has no other signs of valproate toxicity.? Therefore, while a valproic acid level may be part of this child’s initial assessment, it will not contribute to the diagnosis. Abdominal ultrasonography is indicated to evaluate for biliary or pancreatic obstruction or gallstone pancreatitis, but this is unlikely in a thin 12-year-old boy who has no jaundice or underlying risk factors. Computed tomography (CT) scan, although useful in predicting severity of illness, should be performed only to rule out other potential causes in unclear cases, to predict need for surgical or endoscopic intervention in critically ill children, or to assess the child who worsens despite initial conventional therapy. CT scan does not need to be performed routinely in children who have typical mild pancreatitis.? In complicated cases, magnetic resonance MRCP is being used increasingly for better imaging of the biliary and pancreatic ducts as well as looking for evidence of pseudocyst formation and anatomic abnormalities. Because of its higher rate of complications and greater invasiveness, endoscopic retrograde cholangiopancreatography (ERCP) now is reserved for cases that are believed to require therapeutic intervention, such as stone removal and sphincterotomy.In contrast to adults, in whom alcoholic pancreatitis is the leading cause, trauma (motor vehicle collision, bicycle handle, abuse) is the most common underlying cause of pancreatitis (14%) in children, closely followed by drug-induced (11%) and gallstone-associated (11%). Familial pancreatitis, an autosomal dominant genetic disorder whose onset occurs from infancy to adolescence, accounts for approximately 5% of cases. Structural and mechanical disorders of the pancreas can cause acute pancreatitis but more commonly cause chronic pancreatitis. They include ductal stenosis, pancreas divisum, choledochal cysts, and tumors. Pancreatic-sufficient cystic fibrosis is another cause of acute pancreatitis that has been described in children who have unrecognized cystic fibrosis and become dehydrated. Hyperparathyroidism leading to hypercalcemia as well as hypertriglyceridemia are other known triggers of pancreatic obstruction and acute pancreatitis. A wide variety of medications (corticosteroids, l-asparaginase, carbamazepine, valproic acid, isoniazid) and infectious illnesses (mumps, group B coxsackieviruses, Salmonella, hepatitis A and B, human immunodeficiency virus, influenza, and hemolytic-uremic syndrome-producing Escherichia coli strains) can precipitate pancreatitis. Parasitic and fungal infections are more common infectious causes worldwide. Many other systemic and autoimmune illnesses (lupus and periarteritis nodosa) also are associated with pancreatitis. Finally, idiopathic, which represents a group of as yet to be determined causes, comprises at least 25% of diagnoses in pediatric series.Regardless of whether the inciting event is traumatic, infectious, or metabolic, damage to the acinar cells is precipitated by release of digestive enzymes in fully active form inside the pancreas. Enzymes normally are released as precursors that subsequently are activated within the duodenum to aid digestion. Premature activation causes the autodigestion of the pancreatic cells, which results in platelet activation and recruitment of inflammatory cells that, in turn, precipitate cytokine release (tumor necrosis factor-alpha, interleukin-1, nitric oxide, free radicals, vasoactive substances). Cytokines are responsible for the severe systemic symptoms that some patients experience, which include tachycardia, hypotension, hypoxia, and capillary leak syndrome. Severe disease with pancreatic necrosis occurs in fewer than 20% of pediatric patients. Systemic complications include septic shock, acute respiratory distress syndrome, and multiorgan system failure. Among local complications are pseudocyst formation, with or without associated infection, and pancreatic abscess. Surgical intervention is a last resort and is indicated to drain abscesses and debride necrotic tissue. Adult scoring systems to predict severity in pancreatitis (Ranson, APACHE II) have limitations when applied to the pediatric population. Young age, high white blood cell count, high lactate dehydrogenase value at presentation, hypocalcemia, hyperglycemia, clinical shock, and low Pao2 all have been described as poor prognostic factors. Prospective studies to help predict the need for intensive care unit admission and more aggressive therapeutic interventions in pediatric patients are lacking.Most children who have pancreatitis have a mild course that responds to gastrointestinal rest, pain control, and aggressive intravenous hydration. Gastric decompression with a nasogastric tube may be useful in cases where vomiting persists despite cessation of oral intake, although it is no longer standard in all cases. Although meperidine historically has been recommended over morphine for pain control in pancreatitis, all narcotics increase pancreatic sphincter wave frequency and biliary pressure to some extent. In the absence of comparative studies of pain control showing that morphine worsens the outcome in pancreatitis, and given the adverse effect profile of meperidine, many practitioners use morphine. Antibiotic therapy is not indicated in uncomplicated pancreatitis. Evidence is mixed for its benefit in cases of known necrotic pancreatitis without obvious infection. In the presence of abscess, infected pseudocyst, or shock and hyperpyrexia, a multidrug regimen such as ampicillin, gentamicin, and clindamycin is indicated.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of pancreatitisRecognize signs and symptoms of pancreatitisBe familiar with ancillary studies relevant to pancreatitisRecognize life-threatening complications of pancreatitisPlan management of acute pancreatitisQuestion 4A 14-year-old girl presents to the emergency department with a several-week history of lower abdominal and low back pain that has been relieved only partially with the use of ibuprofen. The pain is most intense when she tries to lie down, and she has had difficulty sleeping because of the pain. She has no known history of trauma.? She denies dysuria but has to strain to urinate. She also complains of constipation. She states that she is not sexually active and is premenarchal. On physical examination, the obese adolescent looks visibly uncomfortable, frequently shifting positions. Her body mass index is 35 kg/m2. Her breast development is at Sexual Maturity Rating (SMR) 5 and her pubic hair is at SMR 4. She exhibits diffuse lower abdominal tenderness without point tenderness or guarding. You are unable to feel a discrete abdominal mass, although her obesity makes the examination challenging. She has no point tenderness along her thoracic or lumbar spine. Visual inspection of her perineum reveals the finding in the Figure. The children’s hospital at which you are examining the girl does not have an obstetric and gynecology service.FigureOf the following, the MOST appropriate next step is toA.administer an enemaB.assess urine and serum beta human chorionic gonadotropin concentrationsC.obtain a surgical consultationD.order abdominal computed tomography scan with and without contrastE.transfer the girl to a hospital that has obstetric servicesCorrect answer: CThe girl described in the vignette has an imperforate hymen and is presenting with hematocolpos. The bulging bluish mass present at the introitus on physical examination is characteristic. There is no vaginal opening visible. Although her complaint of back pain is somewhat unusual, it is not inconsistent with the diagnosis. The pain is believed to result from compression of the sacral nerve plexus. Urinary retention due to mechanical compression of the bladder neck may be a presenting complaint, and constipation can be seen due to mechanical compression of the rectum.This girl likely began menstruating several years ago, and her uterus and vagina now are distended with blood. Symptoms may begin at varying times after menarche. A pediatric surgeon can incise the hymen, relieving her physical symptoms by draining the vagina and uterus. Abdominal computed tomography scan is not necessary to make the diagnosis because of the classic physical examination findings. In unclear cases, abdominal ultrasonography is the preferred radiographic study, but her obesity would make the examination more difficult. Although measuring urine beta human chorionic gonadotropin is not unreasonable for this adolescent patient, particularly as a screening tool at triage, there is no reason to obtain a serum study. If you suspected that the findings represented the crowning head of a fetus, you would need to deliver the baby in the emergency department rather than transferring care at this stage of labor. If you believed that the visible mass was sarcoma botryoides, transferring care to the obstetric and gynecologic team would be appropriate. Otherwise, there is no need to transfer this patient to a hospital that has obstetric services. Administration of an enema does not address the primary problem of hematocolpos.Imperforate hymen is a developmental anomaly that is believed to occur at 16 to 20 weeks’ gestation in which the vaginal plate fails to perforate at the junction with the fused Müllerian ducts. It may be recognized in the newborn or infant period by careful physical examination. Infants who have hydrocolpos (vaginal distension with mucus produced due to maternal estrogen) may present at several weeks after birth with an abdominal mass, difficulty urinating, or a visible bulging membrane at the introitus. More commonly, girls who have imperforate hymen present during puberty with abdominal pain, which may or may not be cyclical. Clues to the diagnosis are inconsistency between advanced SMR and the absence of menarche. Imperforate hymen is in the differential diagnosis for causes of primary amenorrhea, as is transverse vaginal septum.Treatment traditionally has consisted of making a cruciate incision of the hymen under general anesthesia. Once the hymen has been perforated, no further treatment is required.American Board of Pediatrics Content Specification(s):Know how to evaluate and manage complications of imperforate hymenQuestion 5There has been an explosion at a train station in the center of the city. You are in the area and run to help, identifying yourself as a physician. A policeman asks you to set up triage and hands you tags to identify victims.Of the following, the MOST appropriate step in performing effective triage in this situation is to:A.begin cardiopulmonary resuscitation on the closest victim who looks to have apnea and no pulseB.identify noninjured victims and direct them from the sceneC.identify the sickest patient who does not require cardiopulmonary resuscitation and provide direct careD.identify those who are dead and segregate their bodies from the anize patients into categories to match care needed with available resources Correct answer EPrinciples involved in disaster management include preparation, communication with first responders and hospitals, assurance of scene safety, stabilization and care delivery, communication with disaster victims and families, documentation, identification of victims, and repatriation with families. Care starts with effective triage, which can be accomplished by using a formal disaster system such as START (Simple Triage and Rapid Treatment). This adult-based triage system identifies four categories of patients and helps direct the resources for care to where they are most useful and effective for the whole. The pediatric version for patients younger than 9 years of age, is called JumpSTART, and incorporates pediatric-specific issues into the START triage plan (Figure).Disaster management can be viewed as an extension or expansion of surge planning. Although the surge response may not be contained in the emergency department or hospital, the general principles of preparation and response are similar. Assurance of knowledgeable, qualified personnel who have specific roles and instructions, alternative sites of care, appropriate equipment and medications, and plans for disposition are imperative. With appropriate planning, disaster response can be effective both in the acute response phase and with a more sustained need. Additional information and training is available through the American Medical Association Basic and Advanced National Disaster Life Support curricula.Awareness of general triage principles and standard response chain of command, as well as the ability to work as a team with medical and nonmedical providers, are key to an effective response. Available resources should be allocated to ensure the most good for the greatest number of patients, rather than the standard emergency medicine paradigm of doing the best for each individual. Such an approach may prevent some of the most critical patients from receiving care that would be otherwise appropriate in a definitive care environment. Disaster triage is a dynamic process that should identify those patients for whom immediate, rapid stabilization and care will improve the likelihood of survival or decreased morbidity. Therefore, once scene safety is assured, the initial action in the disaster described in the vignette is to organize patients into categories to match care needed with available resources. Both under- and overtriage have potential consequences for patients and resources of the system. It is important to reassess patients assigned to various categories as time and resources permit to identify those whose initial triage category has changed. Typical triage systems have four or five, often color-coded, triage categories. Systems that have four instead of five categories may combine expectant and dead into one category.Immediate (Red): Patients require immediate but relatively simple interventions to stabilize until more definitive care can be delivered. These include victims who have airway issues, ongoing hemorrhage, pneumothorax, and open chest wounds. Immediate interventions such as intubation, decompression of a pneumothorax, and beginning an intravenous (IV) infusion for severe hypovolemia can enable the immediacy to be downgraded to a more urgent need.Delayed, Urgent (Yellow or Orange): Patients whose care can be delayed because although they are in need of further intervention, they have relatively stable vital signs and examination findings at time of triage. An example of such a patient is one who has a penetrating abdominal wound with stable vital signs or one who has a spinal injury and is not at risk for further injury.Minimal or Minor (Green): These patients are also referred to as the “walking wounded.” They have relatively minor injuries and primarily require only first aid. They may not require hospitalization, but due to their ability to ambulate, may be the first patients to arrive to a medical facility.Expectant (Blue, possible Black): These patients are alive but have injuries leading to a low likelihood of survival. Care delivered to such patients could deplete resources that might be better allocated to more victims who have less severe injuries and better chances of survival. These patients might have severe head injuries, crushed torsos, or a high likelihood of progressing rapidly to cardiac arrest (not amenable to simple and straightforward interventions such as oxygen, intubation, chest tube, or IV fluids). For pediatric patients, in whom impending cardiac arrest often is a result of airway compromise, rapid assessment and potential airway intervention may be prudent.Dead (Black): The risk of attending to dead patients is the potential diversion of medical resources from those who require care. Resuscitation should not be initiated for patients who are without pulse or respiration unless the cause is determined to be reversible and there are adequate medical providers and equipment to accomplish this task without diverting resources from other patients.Attention to the most critically injured who have low chances of survival may not be possible if personnel, equipment, and care capacity are limited. Identification of patients who can be stabilized quickly enables personnel to be maximally effective for the most patients. Recruitment of patients who are not severely injured or others in the environment to provide basic medical care, such as tamponading bleeding and preventing movement of injured victims, may increase skilled responder capacity to deliver care. In a mass casualty scenario, cardiopulmonary resuscitation (CPR) and prolonged resuscitative attempts may not be effective uses of the limited resources available. Secondary tasks are to identify noninjured victims to direct them away from the scene or to provide care to sick patients who do not require CPR.Patients who have decontamination needs should receive medical care during and after decontamination. Partnership with local emergency medical services and hazardous materials response groups should ensure safety of the clinicians and others in the vicinity and work to decontaminate the victims rapidly and efficiently. This often requires removal of contaminated clothing and cleansing with water showers. In young victims, attention should be paid to the prevention of hypothermia. The same general concept of toxin removal is used for victims who have been in an explosion or fire, in that material, such as smoldering clothing, that could cause further injury is removed from them.Preparation is the most important aspect of the disaster plan. This involves developing a risk assessment, determining scopes and scales of possible events (natural and man-made), and planning for appropriate response. Plans should start with manageable numbers and types of patients to develop the basic principles of triage, personnel requirements, and care delivery. Once basic management plans are established, they can be expanded as a local disaster response and integrated with other medical and nonmedical planning and delivery groups. Use of common planning tools and terminology enables more seamless integration and communication with individuals as well as municipal, state, and federal response teams. Pediatrics should be included in disaster planning.Attention to provider safety is imperative in areas where they may be at risk from continued conflict, toxic exposures, or other environmental issues. In addition, plans should be formulated to offer information to their families. Awareness of family safety enables providers to focus on disaster relief and care delivery, rather than preoccupation with concern over loved ones. A similar concept is important in designing disaster care. It is beneficial for families to stay together if at all possible. In these situations, triage is usually to the category of the most significantly injured member of the family. If both pediatric and adult patients are classified as “red,” preservation of the family unit may not be possible during the acute care processes.American Board of Pediatrics Content Specification(s):Understand principles in providing emergency care in disasters, multi-casualty events, and mass gatheringsKnow the principles of field triage in a disasterQuestion 6An 8-year-old girl presents to the emergency department with a laceration to the sole of her right foot after stepping on “something” outside. She thinks it may have been a piece of wood, but she is not completely sure. Her parents immediately irrigated the wound at home, but it continued to bleed after 10 minutes of direct pressure, so they brought her in for further evaluation and management. On physical examination, the girl is anxious about anyone trying to examine her wound, which is about a 3-cm gaping, linear wound at the base of her second to fourth toes. There is still some bleeding but no obvious visible foreign body. Plain radiographs of her foot do not demonstrate a foreign body, and a foreign body still is not identified once the wound is irrigated thoroughly and explored, after using appropriate local anesthesia. However, the girl states, “It still hurts and feels like something is in my foot.”Of the following, the MOST appropriate next step in wound management is toA.administer a dose of broad-spectrum intravenous antibioticsB.inject the wound with methylene blueC.localize the foreign body with computed tomography scanD.localize the foreign body with ultrasonographyE.soak the wound in concentrated povidone-iodine solutionCorrect answer: DRetained foreign bodies in wounds continue to be a source of liability for emergency medicine practitioners because they are not always identified on initial presentation. A high index of suspicion is necessary to ensure they are identified and removed, if necessary.??A thorough history and careful physical examination, with wound exploration, can be helpful in determining foreign body composition,depth, and orientation in wounds, if visible. If a patient declares that he or she still has a foreign body sensation in the wound, as the girl described in the vignette, the physician should undertake a more thorough search. Relying on the history and physical examination alone is not sufficient to eliminate the possible presence of the foreign body. However, caution should be exercised when using only ancillary imaging studies to make the diagnosis. Using all three approaches provides the optimal strategy for evaluating possible wound foreign bodies.??A number of different radiographic methods can be used in the emergency department to detect foreign bodies Plain radiography is the traditional initial imaging modality used most often due to a number of advantages. However, plain radiographs can only reliably detect radio-opaque foreign bodies, even when using external markers to help pinpoint the position. Their sensitivity can be enhanced somewhat by using an attenuated, underpenetrated soft-tissue technique. This technique may reveal soft-tissue changes that provide indirect evidence of a foreign body, such as trapped air or a radiolucent filling defect. Certain foreign bodies, such as vegetable matter, wood, cactus spines, thorns, and some plastics, are radiolucent and not seen readily, requiring an alternate imaging modality. Glass, which is a very common wound foreign body, can be seen on radiography, if the size is greater than 3 mm.??Ultrasonography is rapidly becoming the evidence-based imaging modality of choice when attempting to locate a radiolucent foreign body. Therefore, ultrasonography should be employed to attempt localization of the foreign body for the girl in the vignette. Her wound may have been caused by wood, which usually is radiolucent on plain radiographs.??Computed tomography (CT) scan and magnetic resonance imaging (MRI) have limited use in the emergency department because most foreign bodies can be documented using plain radiographs and ultrasonography. These modalities can be used when a foreign body is suspected but not found upon wound exploration or other radiographic studies. However, both CT and MRI are more costly, both may fail to detect wooden foreign bodies initially, and CT involves exposing the patient to ionizing radiation.??Once a foreign body is identified, the benefit of its removal must be weighed against the potential of causing greater injury and harm during the removal process. Additional factors to consider include its composition and anatomic location and the patient’s level of discomfort or distress.??Characteristics of foreign bodies that necessitate urgent removal include:Highly reactive substances, such as organic, wood, or other vegetable material, likely to produce an inflammatory reaction and increase the risk of subsequent infectionWounds that are heavily contaminated (bite wounds, embedded dirt/grease)Wounds causing neurovascular compromise, infection, allergic reaction, cosmetic deformity, chronic or persistent pain, or functional impairmentWounds inflicted by parts of venomous animals (sea urchin spines, porcupine quills)Close proximity to or within nerves, vascular structures, joints, tendons, or fractured boneIn contrast, most nonreactive foreign bodies, typically metals, do not usually require emergent or urgent removal, unless they are located near important anatomic structures, the patient is symptomatic, or concomitant infection is present. Of note, numerous case reports exist of previous, innocuous foreign bodies left in the body that later became problematic because of the development of infectious complications, migration to sensitive anatomic sites, or in the case of retained bullet fragments, lead poisoning.??No compelling evidence supports the prophylactic use of antibiotics or soaking of the wound in a concentrated povidone-iodine solution to reduce the incidence of infection caused by foreign bodies. Methylene blue sometimes is injected intra-articularly and observed for evidence of extravasation, which suggests an open joint injury, but that procedure should not precede imaging.??American Board of Pediatrics Content Specification(s):??Understand the radiographic evaluation for foreign bodies in wounds??Differentiate between foreign bodies requiring urgent removal and those that can be left in the bodyQuestion 7A previously well 15-year-old boy is brought in by emergency medical services (EMS) personnel from a spring break party. No parent accompanies the boy, and details of his history are unavailable. According to the EMS personnel, there were 10 other teenagers and young adults at the party, and they were consuming alcohol, smoking marijuana, and drinking homemade herbal tea. The boy appears very anxious, complains of blurry vision, and is responding appropriately to your questions. Physical examination reveals a temperature of 38.5°C, heart rate of 120 beats/min, respiratory rate of 22 breaths/min, and blood pressure of 105/65 mm Hg. His pupils are dilated and sluggishly reacting to light. The only finding of note on systemic examination is dry oral mucous membranes.Of the following, the signs and symptoms exhibited by this boy suggest a toxidrome MOST likely due to ingestion ofA.amphetamineB.carbamateC.ethanolD.Jimson weedE.phenobarbital Correct answer DThe boy described in the vignette has fever, tachycardia, anxiety, dilated pupils, and dry mucous membranes, which are suggestive of an anticholinergic toxidrome. Although mixed ingestions are possible, especially in the scenario described, the major effects appear to be due to anticholinergics seen in homemade herbal tea preparations, which commonly are made from Jimson weed or Datura stramonium (Figure).Figure: Jimson weed seeds, some in tea and pods. Reprinted with permission from Mokalla M, Agarwal S, Bressack M. Index of suspicion: case 3. Pediatr Rev. 2004;25:321-327.Datura is a hearty weed with dark green, pointed leaves that have a characteristic odor. The flower is tubular-shaped; the fruit is round,?contains 50 to 100 black seeds, and is covered by a spiky capsule. All parts of the plant are toxic and contain belladonna alkaloids, including atropine, hyoscyamine, scopolamine, and mandragorine. Symptoms usually begin within 1 to 4 hours of ingestion (drinking tea or smoking the plant) and may not resolve for up to 2 weeks.Toxidromes are constellations of signs and symptoms typically involving the autonomic system that may provide a clue to the type of ingestion and help guide therapy even before confirmatory test results are available. Patients who have sympathomimetic ingestions (amphetamines, cocaine) present with tachycardia, elevated blood pressure, elevated respiratory rate, fever, and dilated pupils, similar to the findings with anticholinergic ingestions. Unlike anticholinergic toxidromes, however, affected patients also have increased diaphoresis and peristalsis. Cholinergic ingestions (organophosphates, carbamates) are not associated with substantial changes in vital signs or pupillary size but are characterized by increased salivation, lacrimation, urination, bronchorrhea, and fasciculations. Sedative-hypnotic (barbiturates, benzodiazepines) and alcohol ingestion are more likely to cause hypothermia, bradycardia, hypotension, shallow respirations, normal pupillary size, and altered mental status.More than 600 compounds have anticholinergic properties. These include:Plants (jimson weed or Datura stramonium, deadly night shade or Atropa belladonna)Mushrooms (Amanita species)Prescription drugs (antihistaminics such as diphenhydramine, meclizine, cyproheptadine, tricyclic antidepressants such as amitriptyline, desipramine, antiparkinsonism drugs such as benztropine, trihexyphenidyl, antispasmodics such as dicyclomine, oxybutynin, and sleep aids such as doxylamine)Over-the-counter medications such as cold preparationsMixed illicit drugs such as heroin combined with scopolamine with anticholinergic properties.American Association of Poison Control Centers data documents more than 2.4 million cases of human poison exposure to anticholinergics in 2007 alone. Thus, it is one of the more common poisonings that require emergent evaluation and management.??Compounds that have anticholinergic properties competitively antagonize acetylcholine muscarinic receptors found on the postganglionic cholinergic nerves in smooth muscles (heart, bronchi, intestines), sweat and lacrimal glands, and ciliary body of the eye. Central nervous system (CNS) effects are due to cortical and subcortical muscarinic receptor antagonism. Because these agents do not antagonize the effect of acetylcholine at the nicotinic receptors, no neuromuscular junction effects such as weakness of muscles are seen. The classic symptoms described for anticholinergic poisoning are: red as a beet (cutaneous vasodilatation), dry as a bone (anhidrosis), hot as a hare (anhydrotic hyperthermia), blind as a bat (nonreactive mydriasis), mad as a hatter (delirium, hallucinations), and full as a flask (bladder relaxation along with urethral sphincter contraction). Tachycardia is one of the earliest and most reliable signs of anticholinergic toxicity. CNS manifestations may include anxiety, agitation, dysarthria, confusion, disorientation, visual hallucinations, bizarre behavior, delirium, psychosis (usually paranoia), coma, and seizures. Hallucinations are often described as "Alice in Wonderland-like" or "Lilliputian type," in which people appear to become larger and smaller. Patients who have altered mental status often present with agitation and may appear to grab at invisible objects in the air.??Substances or conditions that produce an alteration in mental status, tachycardia, urinary retention, or seizure should be included in the differential diagnosis. A wide range of medical conditions and drugs can cause agitated delirium, including hypoglycemia, meningitis, fever, hyperthyroidism, neuroleptic malignant syndrome, and other ingestions such as hallucinogens and stimulants. Salicylate overdose also should be considered for those who exhibit agitation and hyperthermia.??Similar to the approach to any toxic ingestion, clinicians should support the airway and monitor breathing, circulatory status, and circulation, with appropriate interventions as required. In addition, bedside glucose assessment, electrocardiography (widening of the QRS complex, as seen in tricyclic antidepressant ingestion, is an early sign of cardiac toxicity), and serum and urinary drug screens are warranted. Sodium bicarbonate should be used in the treatment of widened QRS complexes, and agitation and seizures should be treated with benzodiazepines. Decontamination for anticholinergic toxicity consists of administration of activated charcoal (1 g/kg, maximum of 50 g), and elective endotracheal intubation should be considered if airway protection is required. Antidotal therapy with physostigmine may be required in isolated cases of pure anticholinergic ingestions to reverse the CNS effects of hallucinations, stupor, coma, or seizures.??Rarely, physostigmine is indicated for fever or severe agitation. Physostigmine should be used under the direction of toxicologists because injudicious use can result in cholinergic syndromes. Most cases of anticholinergic ingestions require monitoring, symptomatic therapy, and supportive care only.?? American Board of Pediatrics Content Specification(s):??Recognize the signs and symptoms of anticholinergic poisoning??Plan the management of acute anticholinergic toxicityQuestion 8A 10-year-old boy is brought in to the emergency department by his mother because she is concerned that the left side of his face is drooping. She first noticed it this morning when he woke up. He has not otherwise been ill. He returned from a month-long camp in northern Wisconsin last week. He initially slept “quite a bit” and complained of mild headache, but he has been “back to himself” for the past 3 days. On physical examination, the awake, alert, and cooperative boy exhibits left-sided facial weakness. The remainder of his cranial nerve and neurologic examination results are normal. The only other finding of note is a nonpruritic erythematous patch about 5 cm in diameter on his back.Of the following, the MOST appropriate treatment for this boy isA.acyclovir and prednisone orally for 14 daysB.amoxicillin orally for 21 daysC.azithromycin orally for 10 daysD.ceftriaxone intravenously/intramuscularly for 21 daysE.doxycycline orally for 21 days Correct answer EThe boy described in the vignette has signs and symptoms of early disseminated Lyme disease. Although his presenting complaint is facial nerve palsy, his physical examination also reveals erythema migrans, the most common clinical manifestation of Lyme disease. As of 2009, recommended treatment for early disseminated Lyme disease with facial nerve palsy in children older than 8 years is a 21-day course of oral doxycycline (Table).Table: Recommended Treatment of Lyme Disease in ChildrenDisease CategoryDrug(s) and DoseaEarly localized diseasea8 y of age or olderDoxycycline, 100 mg, orally, twice a day for 14-21 daysbYounger than 8 y of age or unable to tolerate doxycyclineAmoxicillin, 50 mg/kg per day, orally, divided into 3 doses (maximum 1.5 g/day) for 14-21 days??? ORCefuroxime, 30 mg/kg per day in 2 divided doses (maximum 1000 mg/day) or 1.0 g/day for 14-21 daysEarly disseminated and late diseaseMultiple erythema migransSame oral regimen as for early localized disease, but for 21 daysIsolated facial palsySame oral regimen as for early localized disease, but for 21-28 daysc,dArthritisSame oral regimen as for early localized disease, but for 28 daysPersistent or recurrent arthritiseCeftriaxone sodium, 75-100 mg/kg, IV or IM, once a day (maximum 2 g/day) for 14-28 days??? ORPenicillin, 300 000 U/kg per day, IV, given in divided doses every 4 h (maximum 20 million U/day) for 14-28 days??? ORSame oral regimen as for early diseaseCarditisCeftriaxone or penicillin: see persistent or recurrent arthritisMeningitis or encephalitisCeftriaxonef or penicillin: see persistent or recurrent arthritis, but for 14-28 daysIV indicates intravenously; IM, intramuscularly.aFor patients who are allergic to penicillin, cefuroxime and erythromycin are alternative drugs.bTetracyclines are contraindicated in orticosteroids should not be given.dTreatment has no effect on the resolution of facial nerve palsy; its purpose is to prevent late disease.eArthritis is not considered persistent or recurrent unless objective evidence of synovitis exists at least 2 months after treatment is initiated. Some experts administer a second course of an oral agent before using an IV-administered antimicrobial agent.fCeftriaxone should be administered IV for treatment of meningitis or encephalitisAzithromycin is a less effective antibiotic treatment for Lyme disease. If a viral cause is suspected, antiviral therapy (such as acyclovir for?treatment of herpes simplex infection), when combined with a corticosteroid, may be effective. However, the presence of erythema migrans makes Lyme disease, rather than a virus, the most likely cause of this child’s facial nerve palsy. Prednisone may be useful if started within the first 72 hours of symptoms.Northern Wisconsin is an endemic area for the Ixodes scapularis tick, the most common vector for Borrelia burgdorferi, the causative pathogen for Lyme disease? Although this boy has no known history of tick bite, the presence of erythema migrans in a patient who has traveled to an endemic area is sufficient for diagnosis.? Indeed, only about one third of patients who have Lyme disease have a recognized tick bite in the month preceding diagnosis. In the presence of erythema migrans, no further diagnostic or serologic testing is indicated.Controversy surrounds the need for lumbar puncture for patients who have cranial nerve palsy with suspected Lyme disease. The Infectious Diseases Society of America and the American Academy of Neurology recommend that such children only undergo lumbar puncture if there is clinical evidence of central nervous system infection, such as nuchal rigidity or severe headache, neither of which is present in this boy. The current expert recommendation is that children who have meningitis be treated with intravenous/intramuscular therapy for 14 to 28 days.Early localized Lyme disease, which usually occurs within 7 to 14 days of tick bite, is indicated by the presence of a single lesion of erythema migrans at the site of the bite. Manifestations of early disseminated disease, which occurs after the spirochete has entered the bloodstream and has been carried to other tissues in the body, includes multiple erythema migrans, cranial nerve palsy, meningitis, and carditis. In the absence of erythema migrans, serology is recommended to confirm the diagnosis in a patient who has facial nerve palsy. Multiple erythema migrans is, by far, the most common presenting symptom of early disseminated disease. Cranial nerve palsy occurs in fewer than 5% of cases and meningitis in 1%. Carditis is rare and is manifested by partial or complete heart block that usually is self-limited but occasionally may require temporary cardiac pacing.Late Lyme disease occurs weeks to months after the initial infection in untreated or incompletely treated cases. Arthritis, usually monoarticular or oligoarticular, is the most common manifestation. Large joints, mostly commonly the knee, are predominately affected.Serologic testing for Lyme disease is not recommended in the presence of nonspecific symptoms such as fever, headache, or myalgia or for patients who have erythema migrans. When indicated, the Centers for Disease Control and Prevention recommend a two-step blood testing procedure. First, an enzyme immunoassay (EIA) or immunofluorescent assay (IFA) should be performed in an accredited laboratory. If results are positive, this diagnosis is confirmed with a Western blot, which is more specific. If EIA or IFA results are negative, Lyme disease is ruled out.American Board of Pediatrics Content Specification(s):Know the etiology and pathophysiology of Lyme diseaseRecognize signs and symptoms of Lyme diseaseBe familiar with ancillary studies relevant to Lyme diseasePlan management of early and late Lyme diseaseDECEMBER 2010Question 1An 18-month-old girl presents to the emergency department with a several hour history of crying. The parents report the girl has been crying as if she is in pain for several hours, and they noticed that she developed swelling and tenderness of her right groin. She has had no fever, respiratory symptoms, rash, or diarrhea. She has vomited twice without any blood or bile in the emesis. On physical examination, the child appears uncomfortable. Her temperature is 37.9°C, heart rate is 140 beats/min, respiratory rate is 30 breaths/min, and blood pressure is 100/60 mm Hg. Head, neck, heart, and lung examinations yield normal results. The child’s abdomen is soft and nondistended, but examination is limited by crying and poor cooperation. Genitourinary examination reveals normal female genitalia and a palpable, firm, tender, nonreducible swelling in her right groin inferior to the inguinal ligament and medial to the normal femoral pulse. The area is not erythematous and the mass is not fluctuant.Of the following, the MOST appropriate next step in the evaluation of this patient is:puted tomography scan of the abdomen and pelvisB.nuclear perfusion scanC.percutaneous aspiration of the right inguinal massD.ultrasonography of the groinE.vaginal cultures for Neisseria gonorrhoeae and Chlamydia trachomatis Correct answer DThe signs and symptoms described for the girl in the vignette could be the result of several emergent conditions, including incarcerated femoral hernia, lymphadenitis, or abscess. The absence of fever and signs of inflammation strongly suggest that the girl has an incarcerated femoral hernia rather than an abscess. If incarcerated hernia is suspected, urgent surgical consultation should be obtained. Differentiation of the potential causes can be accomplished with ultrasonography, which also can clarify contents and blood flow within a hernia sac. Computed tomography (CT) scan may identify the underlying cause of the patient’s symptoms, but it is not the test of choice for evaluating inguinal and femoral pathology. In addition, the lack of exposure to ionizing radiation and cost make ultrasonography an?attractive first-line alternative. Percutaneous aspiration or incision and drainage should not be performed on groin masses unless the diagnosis has been clarified by radiologic methods; bowel perforation, vascular injury, or damage to other vital structures may result. Evaluation for sexually transmitted diseases such as gonorrhea or Chlamydia is indicated for a patient who has suspected cervicitis, pelvic inflammatory disease, epididymo-orchitis, prostatitis, or urinary tract infection, but this patient’s age and presenting signs and symptoms make these diagnoses unlikely. Nuclear perfusion scan with technetium-99 pertechnetate can be used in the evaluation of testicular blood flow in cases of suspected testicular torsion, but lack of ready availability and delays in treatment make this modality less appealing than ultrasonography, and nuclear perfusion studies are not helpful in evaluation of hernia, hydrocele, and other diagnostic considerations in a female patient.??The groin region can be divided into two areas: the inguinal canal medially and the femoral triangle laterally. Masses within the groin region can be classified based upon their location (femoral or inguinal) or their cause (congenital, noncongenital hernias, vascular, infectious/inflammatory, and neoplasm) (Table).Age is another important consideration in the differential diagnosis of groin masses. Infants and young children are most likely to present with congenital hernias, cord hydroceles, reactive lymphadenopathy, and lymphadenitis. Undescended or retractile testes are found commonly in the inguinal canal in infants. With increasing age, noncongenital hernias become more common. Vascular lesions are more likely in adults but also result commonly from surgical or vascular procedures in the region, such as femoral catheterization for evaluation and treatment of congenital or acquired heart disease. Varicoceles occur most commonly in adolescents and adults but can accompany abdominal tumors, ascites, or hydronephrosis in infants and young children. Neoplastic disease of the femoral or inguinal region, both primary and metastatic, is more likely in adults than in children.??Inguinal hernias typically are indirect (Figure), and most indirect inguinal hernias are congenital. Such hernias result from failure of the processus vaginalis to involute completely.Figure: Indirect inguinal hernias develop at the internal inguinal ring and are lateral to the inferior epigastric artery. Direct inguinal hernias occur through Hesselbach triangle (outlined in blue) formed by the inguinal ligament inferiorly, the inferior epigastric vessels laterally, and the rectus muscle medially. Femoral hernias develop in the empty space at the medial aspect of the femoral canal, inferior to the inguinal ligament. During fetal development, the processus vaginalis is an outpouching of the peritoneum that follows the gubernaculum in its descent through the inferior abdominal wall into the scrotum or labia majora. The proximal portion of this pouch normally closes completely just before birth; failure of complete closure allows peritoneal contents, including fluid, fat, omentum, ovaries, or bowel, to protrude into the scrotum or labia. Swelling that is exacerbated by the Valsalva maneuver typically is intermittent. If the swelling becomes hard, nonreducible with pressure, discolored, or significantly painful/tender, incarceration and possible strangulation should be suspected. Indirect inguinal hernias are more common in preterm infants, males, and those who have conditions causing increased abdominal pressures. The right side is affected more often than the left. Diagnosis typically is based on history and physical examination; ultrasonography can be helpful in cases where the diagnosis is unclear. CT scan or magnetic resonance imaging (MRI) also aid in the diagnosis but are not usually necessary.Direct inguinal hernias occur in the Hesselbach triangle, formed by the inguinal ligament inferiorly, the inferior epigastric vessels laterally, and the rectus abdominus muscle medially. They can be either congenital or acquired and may be associated with straining or heavy lifting. The direct hernia ruptures across the fibrous abdominal wall.Femoral hernias account for approximately 5% of all hernias, and unlike inguinal hernias, are more common in females. These hernias are much more likely to present with acute incarceration or strangulation (40%). The hernia develops medial to the femoral vein in the femoral canal and is the result of weakness of the muscular floor of the canal. Femoral hernias are much more common in adults, particularly elderly women.Evaluation of the patient who has a groin mass should focus on excluding emergent and potentially serious causes and complications. Incarcerated or strangulated hernias require emergent surgical intervention to prevent bowel or other tissue necrosis or progression to sepsis due to tissue infarction and secondary infection. Torsion of a nondescended testicle or diminished blood flow to an ovary contained in a hernia may result in infarction and loss of the involved gonad. Infection in localized abscess or lymphadenitis, septic hip, or bursitis can progress to more extensive local infection or sepsis if not treated adequately. Malignant causes are uncommon in children, but signs or symptoms of local or regional neoplastic disease should be excluded. Vascular lesions, including true or false aneurysms, put the patient at risk for vascular rupture, thrombosis with embolization, or impaired vascular supply to distal structures. Varicoceles and hernias occasionally can result from increased abdominal pressure; abdominal and pelvic pathology, including mass, ascites, and hydronephrosis, should be excluded.If the diagnosis is unclear after initial history and physical examination, imaging studies should be pursued. Ultrasonography with Doppler flow is the most common first-line test, with CT or MRI used when ultrasonographic findings are nondiagnostic or further delineation of suspected vascular or neoplastic lesions is indicated. Laboratory testing is unnecessary in the evaluation of most groin masses but may be indicated if infectious or malignant causes are suspected.American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of groin massesPlan diagnostic evaluation and initial intervention for patients with groin massesRecognize serious and/or life-threatening causes of groin massesQuestion 2A 5-year-old boy presents to the emergency department with scrotal pain and swelling for a few days without any redness. Both the boy and his parents deny any history of trauma, urinary complaints, or lumps or masses in the groin. He has had fever for 2 days with a temperature of 39.0°C, tiredness, intermittent generalized abdominal pain, and periorbital and ankle swelling. Yesterday he completed a 10-day course of amoxicillin for group A streptococcal pharyngitis. His mother has type 1 diabetes mellitus. Physical examination of the awake and alert boy shows a temperature of 39.1°C; heart rate of 120 beats/min; respiratory rate of 35 breaths/min; blood pressure of 95/60 mm Hg; clear lung sounds; tachycardia without any heart murmurs; a distended abdomen with diffuse tenderness, no guarding, and active bowel sounds; bilateral scrotal swelling; and swelling of both ankles.? Laboratory findings include:White blood cell count of 18.5x103/mcL (18.5x109/L) with 80% neutrophils and 10% bandsPlatelet count of 445x103/mcL (445x109/L)Glucose of 100 mg/dL (5.6 mmol/L)Blood urea nitrogen of ?7 mg/dL (2.5 mmol/L)Creatinine of 0.24 mg/dL (21.2 mcmol/L)Sodium of 139 mEq/L (139 mmol/L)Potassium of 4.4 mEq/L (4.4 mmol/L)Chloride of 108 mEq/L (108 mmol/L)Bicarbonate of 27 mEq/L (27 mmol/L)Total protein of 4.0 g/dL (40 g/L)Albumin of 1.2 g/dL (12 g/L)?Urinary protein of >600 mg/dLUrinary red blood cells, 0 to 2 cells/high-power fieldAbdominal ultrasonography findings are normal except for free fluid.Of the following, the MOST appropriate next steps are administration ofA.intravenous antibiotics and exploratory laparotomyB.intravenous ceftriaxone and admission to the hospitalC.intravenous fluid bolus and admission to the hospitalD.oral corticosteroids and nephrology follow-upE.oral nonsteroidal anti inflammatory drugs and nephrology follow-up Correct answer BThe boy described in the vignette has nephrotic syndrome and bacterial peritonitis. Nephrotic syndrome is a glomerular disorder characterized by hypoalbuminemia (serum albumin <2.5 g/dL [25 g/L]), hyperlipidemia, edema, and proteinuria (>40 mg/m2 per hour in the pediatric population). The most common cause in children is idiopathic (90%), and the most common form is minimal change disease. The disease is caused by increased permeability of glomeruli leading to proteinuria that results in hypoalbuminemia and subsequent diffuse edema. No specific causative agent has been identified.? Males are affected more than females, and nephrotic syndrome occurs most??commonly among children 2 to 6 years of age.The typical presentation of nephrotic syndrome is edema (Figure) that may be considered to be due to an allergic reaction, as for the child described in the vignette, who developed periorbital and ankle swelling immediately after finishing a course of amoxicillin.Figure: Facial edema in a patient who has nephrotic syndromeWith the reduction in serum albumin, patients may develop ascites, pleural effusion, and reduction in urinary output due to reduced intravascular volume. Generalized edema becomes clinically evident as weight gain, abdominal distention, and genital swelling develop. Fatigue and irritability may be seen as intravascular dehydration becomes more pronounced. The diagnosis is based on clinical signs and laboratory findings of marked proteinuria, decreased serum albumin concentrations, and elevated serum lipid values. Nephritic syndrome can be ruled out by the absence of red blood cell casts on urinalysis and normal complement C3 and C4 values. However, some patients may have microscopic hematuria with nephrotic syndrome.Emergency department management of the patient who has nephrotic syndrome involves deciding whether to facilitate outpatient follow-up or undertake an inpatient evaluation and treatment for complications. The most important reason for hospital admission for a patient who has newly diagnosed nephrotic syndrome is a major complication at presentation; otherwise, it can be managed on outpatient basis. Infection is the major complication, and the patient described in the vignette has primary or spontaneous bacterial peritonitis, as evidenced by fever, abdominal distention, and tenderness on palpation. Accordingly, the most appropriate actions for this boy are to obtain a blood culture and a peritoneal aspirate and admit him to the hospital for antibiotic therapy that provides a broad spectrum of coverage for Streptococcus pneumoniae and gram-negative organisms. The antibiotic of choice is an intravenous third-generation cephalosporin such as ceftriaxone. Penicillin plus gentamicin is an alternative. Hypovolemia should be treated with fluid boluses. If the patient is euvolemic, diuretic use may be considered to provide symptomatic relief of pulmonary and abdominal discomfort. Treatment of nephrotic syndrome without complications includes oral corticosteroids in consultation with the nephrology team and outpatient follow-up if the patient’s condition allows. If the patient already is receiving corticosteroids, the dose may be reduced if infection is present, but discontinuation is not required.Although appendicitis should be considered in any abdominal emergency, primary peritonitis is a more appropriate consideration for the boy in the vignette and does not warrant an exploratory laparotomy. An intravenous fluid bolus is not indicated because the boy is clinically well hydrated. Nonsteroidal anti-inflammatory medications are not indicated for this boy.Potential life-threatening complications are the major factors associated with the morbidity and mortality of nephrotic syndrome (Table). Among these, infection is the major cause and primary bacterial peritonitis is the most common form of infection.Table:?Complications of Nephrotic SyndromeInfections: peritonitis, pneumonia, arthritis, urinary tract infectionShock due to hypovolemiaArterial and venous thrombosis: renal venous thrombosis, pulmonary emboliRespiratory and cardiac arrestAcute renal failure, hyponatremia, hypocalcemiaSteroid withdrawalSteroid toxicity: hypertension and encephalopathyThe reasons for infections are loss of serum proteins, medications used for management of nephrotic syndrome, and other immune-mediated mechanisms. Common sites of infection are lungs, abdomen, joints, blood, meninges, urinary tract, and skin. The pathogens include gram-negative and -positive bacteria (most commonly, Streptococcus pneumoniae) and viruses (frequently, varicella). Other potential complications are hypovolemia, shock, renal failure, respiratory compromise, adverse effects of corticosteroid or immune therapy, and thromboembolism (both arterial and venous thrombosis) associated with hypercoagulability. Common sites are renal vein thrombosis, pulmonary emboli, and sagittal sinus thrombosis. Hyperlipidemia may contribute to the rare cardiovascular complications.?American Board of Pediatrics Content Specification(s):Recognize signs and symptoms of nephrotic syndromeBe familiar with ancillary studies relevant to nephrotic syndromeRecognize the life-threatening complications of nephrotic syndromeQuestion 3A 13-year-old boy who has cerebral palsy, cardiomyopathy due to an inborn error of metabolism, seizure disorder, developmental delay, and a gastrostomy tube is brought to the emergency department after he was found unresponsive in his bed. His home medications include oxcarbazepine, diazepam, ranitidine, baclofen, furosemide, and potassium chloride. At the scene, emergency medical services found the boy to be limp, unresponsive, and cyanotic, with grunting and irregular breathing. His heart rate was 90 beats/min and respiratory rate was 14 breaths/min, and he had 3-mm pupils that reacted to light. He had coarse breath sounds with decreased air entry at the bases. The team placed a nonrebreather face mask but could not obtain intravascular access. En route to the emergency department, he developed apnea with no palpable pulses. The team started cardiopulmonary resuscitation (CPR) with bag-mask ventilation and chest compressions to a compression-to-ventilation ratio of 15:2. The patient was intubated endotracheally, and the first dose of 0.1 mL/kg of 1:1,000 epinephrine was administered via the endotracheal route.? Upon arrival at the emergency department, the boy has no spontaneous respiratory effort and no palpable pulses and the cardiac monitor shows asystole. CPR is continued and intravascular access obtained, followed by administration of two more doses of 0.1 mL/kg of 1:10,000 epinephrine intravenously. Physical examination shows no spontaneous respiratory effort; asystole with no palpable pulses; temperature of 35.0°C; pupils unresponsive to light, 6 mm, and dilated; coarse lung sounds; and no obvious trauma. Venous blood gas shows pH of 6.995, PaCO2 of 105 mm Hg, sodium of 129 mEq/L (129 mmol/L), potassium of 8.5 mEq/L (8.5 mmol/L), bicarbonate of less than 10 mEq/L (10 mmol/L), glucose of 65 mg/dL (3.6 mmol/L), and ionized calcium of 1.30 mmol/L (5.2 mg/dL) . CPR continues and a 20-mL/kg bolus of normal saline is administered intravenously.Of the following, the MOST appropriate next step to manage this patient’s cardiac arrest is administration ofA.amiodarone 5 mg/kg intravenouslyB.calcium chloride 20 mg/kg intravenouslyC.defibrillation 4 joules/kgD.epinephrine 1:1,000 0.1 mL/kg ?intravenouslyE.sodium bicarbonate 1 mEq/kg intravenouslyCorrect Answer: BThe patient described in the vignette is experiencing asystole. This may have resulted from either respiratory distress or hyperkalemia and, therefore, requires administration of calcium chloride. Severe hyperkalemia is associated with decreased excitability of heart cells due to reduced activity of sodium channels. On electrocardiography, the progression of changes depends on the concentration of potassium. The readings start with peaked T wave, shortened QT interval, disappearance of P wave, and widening of QRS (sine wave), followed by pulseless electrical activity to flat line due to complete absence of electrical activity. The treatment of life-threatening hyperkalemia includes bicarbonate or insulin with glucose. However, the effects of these agents do not occur as quickly as those of calcium chloride, which counteracts the effects of hyperkalemia on myocardium action potential within minutes.When emergency medicine services personnel arrived at the scene, the patient was in cardiopulmonary failure. Without early recognition?and prompt treatment, cardiopulmonary failure almost always rapidly progresses to cardiopulmonary arrest. Bag-valve-mask ventilation with 100% oxygen should have been administered immediately. Asystole or bradycardia are the more common cardiac rhythms identified in pediatric cardiopulmonary arrest rather than ventricular fibrillation or ventricular tachycardia. In the event of asystole, the sequence of management includes assistance in ventilation and initiation of chest compression. The first step is to administer epinephrine via the intravenous or intraosseous route (1:10,000 at a dose of 0.1 mL/kg), or if vascular access cannot be obtained, via endotracheal tube (1:1,000 at a dose of 0.1 mL/kg). Such administration can be repeated every 3 minutes while CPR is continued. High-dose intravenous epinephrine (1:1,000) is not recommended in pediatric cardiac arrests. Defibrillation is not indicated if asystole is documented. During resuscitation, reversible causes should be identified and include the 4 Ts (Tamponade, Tension pneumothorax, Toxins, Thromboembolism) and the 4 Hs (Hypoxemia, Hypovolemia, Hypothermia, Hyper/Hypokalemia). However, epinephrine is the first-line treatment while possible reversible causes are identified.Once spontaneous circulation is established, the patient’s cardiopulmonary status should be stabilized to avoid recurrence of cardiopulmonary arrest. Important postcardiac arrest complications include acidosis, hypotension, and cardiac instability. Acidosis could be both respiratory and metabolic and can be corrected with adequate ventilation and restoration of circulation. Management strategies include administration of a fluid bolus and use of cardiac inotropic agents (milrinone, inamrinone). Intravenous high-dose epinephrine or high-dose dopamine and norepinephrine for hypotensive shock and low-dose dopamine or low-dose epinephrine or dobutamine for normotensive shock is indicated. Sodium bicarbonate use remains controversial because of the requirement to ventilate out the carbon dioxide generated with its use and the increases it causes in osmolar pressure and lactate production.Amiodarone is indicated for refractory ventricular fibrillation or pulseless ventricular tachycardia. Although an additional dose of epinephrine may be attempted for the boy in the vignette, three doses of epinephrine has not prompted recovery of spontaneous circulation, suggesting that calcium chloride is more immediately appropriate.American Board of Pediatrics Content Specification(s):Understand pathophysiology of progression from cardiopulmonary failure to arrestUnderstand physiologic consequences of cardiopulmonary arrest and post arrest phenomenonQuestion 4A 6-year-old girl is transported by paramedics from the site of a motor vehicle crash to a level 1 trauma center emergency department. She was a back seat unrestrained passenger in a high-speed impact of car against the highway barrier. She was found 15 feet away from the crash site, was unresponsive at the scene, and had sustained injuries to multiple organ systems. She had a respiratory rate of 14 breaths/min before intubation at the scene, heart rate of 140 beats/min, and blood pressure of 80/50 mm Hg with poor perfusion. Her airway was not obstructed, she had a left-sided chest wall contusion and left-sided coarse breath sounds, heart sounds were audible, and her abdomen was distended. She also had bilateral periorbital swelling, left-sided neck contusion, and left femur and left forearm deformity. She was immobilized and received 40 mL/kg of normal saline fluid bolus en route to the emergency department. On arrival at the trauma center, physical examination reveals a temperature of 35.2°C, heart rate of 160 beats/min, systolic blood pressure of 60 mm Hg with poor perfusion, and oxygen saturation of 90% on manual ventilation. Proper placement of the endotracheal tube is verified. Her lung sounds are decreased on the left side, trachea is shifted to the right, and heart sounds are muffled, with palpable but weak distal pulses. Her neck contusion has become a palpable hematoma. Her abdomen is distended, and the pelvis is unstable. Her pupils are responsive to light. She has left forearm and left femur deformity.Of the following, the MOST appropriate next step to treat this patient’s change in hemodynamic status is toA.administer a third intravenous fluid bolus followed by blood transfusionB.obtain neck arteriography followed by surgical explorationC.perform diagnostic peritoneal lavageD.perform pericardiocentesisE.perform thoracic needle decompression Correct answer EThe sequence of management for trauma patients is stabilization of airway (with cervical spine immobilization), breathing, and circulation. Any acute change in the patients’ clinical or hemodynamic status necessitates repetition of the same sequence to identify and treat the cause of deterioration. Examinations in the same sequence should be repeated for those experiencing multiple organ trauma to identify any possible development of life-threatening complications. Thus, for the girl in the vignette, who has several possible causes for her acute hemodynamic deterioration, the most appropriate next step in management is needle thoracic decompression of a likely tension pneumothorax to alleviate the respiratory compromise (Figure).Figure: Needle thoracostomy..After securing adequate ventilation, one should proceed to management of hemodynamic instability with repeated additional intravenous fluids bolus for hypovolemia and pericardiocentesis for possible pericardial tamponade. Thereafter, evaluation of the expanding neck hematoma and the distended abdomen is appropriate.Circulatory status can be assessed by determining perfusion and palpating for distal pulses as well as by monitoring mental status and urinary output. In trauma patients, especially in the pediatric population, tachycardia is the first indication of hypovolemia, followed by hypotension that, if not corrected rapidly, can lead to cardiac arrest.? Therefore, it is imperative to identify the reasons for hypovolemia and immediately take corrective steps. Obtaining large-bore vascular access as soon as possible allows immediate resuscitative measures if the need arises.Patients who have tension pneumothorax exhibit signs of respiratory distress, hypotension, and tachycardia. Approximately 20% of pediatric trauma patients who have pneumothorax develop tension pneumothorax. The patient in the vignette has left-sided chest wall contusion associated with coarse breath sounds, and endotracheal intubation followed by manual bag ventilation can result in deterioration of an existing pneumothorax into tension pneumothorax, followed by acute hypotension and hemodynamic instability. There is no indication to wait for radiography before undertaking needle thoracic decompression. Such decompression should be followed by chest tube insertion to avoid the recurrence of tension pneumothorax.Patients who have pericardial tamponade have clinical signs of hypotension with possible associated findings of distant or muffled heart sounds, neck vein distention, and narrow pulse pressure. If the patients’ condition allows, chest radiograph may show an enlarged heart, electrocardiography may show low-amplitude QRS complexes, and pulsus paradoxus may be documented. Emergency bedside ultrasonography can help diagnose the problem rapidly. Although the girl in the vignette has clinical signs of possible pericardial tamponade, securing her airway and breathing takes the precedence, followed by repeat examination and the possible decision to undertake pericardiocentesis.Blunt abdominal trauma is another cause for hypotension in trauma patients due to solid organ injuries resulting in internal hemorrhage. Splenic injury is the most likely reason for internal hemorrhage, followed by liver and retroperitoneal hematoma in blunt abdominal trauma. The liver is the most likely organ affected by penetrating abdominal trauma. Mechanisms of injury for blunt trauma are crushing injury or deceleration shearing effects on the solid organ attachments. Diagnostic peritoneal lavage may be indicated if the patient exhibits hypotension and there are time or resource constraints in obtaining abdominal ultrasonography (FAST) or computed tomography scan. Lavage is considered 98% sensitive in documenting intraperitoneal bleeding and could be an adjunct if the patient must undergo surgeries for other reasons such as intracranial trauma or extremity trauma. However, if there is an indication for exploratory laparotomy, such as penetrating abdominal trauma with hypotension, there is no indication for diagnostic peritoneal lavage. Another cause of hypotension in a trauma patient is blunt neck trauma. Clinical signs include an expanding neck hematoma, widened mediastinum, first rib fracture, or neurologic deficits. Treatment involves arteriography and surgical exploration. This time-consuming process should be delayed while hemodynamic stability is achieved via intravenous fluids and blood transfusion.Spinal cord injury may cause neurogenic shock in trauma patients, although it is an extremely rare event, especially in the pediatric population. In this injury, the hypotension may be associated with bradycardia and peripheral vasodilatation rather than tachycardia and poor perfusion. Treatment for such hypotension includes phenylephrine or epinephrine.American Board of Pediatrics Content Specification(s):Distinguish causes of shock in the trauma patientQuestion 5A 7-year-old girl who has had? placement of a ventriculoperitoneal shunt for management of hydrocephalus related to myelomeningocele presents with a 2-day history of headaches. She initially complained of a mild headache 2 days ago, which resolved after administration of acetaminophen. The pain returned later that evening and subsequently has worsened despite repeated doses of acetaminophen and ibuprofen. Today she developed vomiting and increased sleepiness. Her parents deny any history of fever, sore throat, respiratory symptoms, or diarrhea. The girl had her initial myelomeningocele repair and shunt placement in the neonatal period. She had one shunt malfunction around 4 years of age, and the parents report those symptoms were very similar to her current complaints. Physical examination reveals a sleeping child who wakes with stimulation and interacts appropriately. Her temperature is 37.5°C, heart rate is 70 beats/min, respiratory rate is 24 breaths/min, and blood pressure is 120/80 mm Hg. Examination of the head and neck reveals an easily palpable shunt without swelling or tenderness. The shunt tubing can be palpated from the head to the abdominal scar. Results of neurologic examination are normal. You perform a shunt series and noncontrast head computed tomography scan, which reveal an intact shunt and ventricular size that is unchanged from a scan 1 year ago.Of the following, the MOST appropriate next step in the evaluation of this child is to:A.discharge her with an outpatient follow-up appointmentB.obtain magnetic resonance imaging of the brainC.obtain neurosurgical consultationD.perform a lumbar punctureE.perform percutaneous aspiration of cerebrospinal fluid through the shunt Correct answer CThe child described in the vignette is displaying symptoms consistent with hydrocephalus, and with her history of ventriculoperitoneal shunt (VPS), such symptoms are suggestive of shunt malfunction or obstruction. Although computed tomography (CT) scan of the brain reveals ventricular dilation in approximately 80% of cases of shunt malfunction, the absence of ventriculomegaly cannot exclude this diagnosis in a patient who has suggestive signs and symptoms. Neurosurgical consultation should be obtained to determine the need for further evaluation of the shunt or shunt revision surgery before discharging the girl from the emergency department. Magnetic resonance imaging is unlikely to provide additional useful information in a case of possible shunt malfunction. Evaluation of cerebrospinal fluid (CSF) pressure, cell counts, chemistries, Gram stain, and culture can be accomplished by either aspiration of the shunt or performance of a lumbar puncture. Such procedures most commonly are performed in suspected shunt infection, which is unlikely for this girl because of the time from last revision and absence of fever or other infectious symptoms. Further, neurosurgical consultation should be obtained before performing either of these tests in a patient who has a VPS and suspected increased intracranial pressure.Hydrocephalus is a disorder characterized by excess CSF in the cerebral ventricular system as a result of imbalance between CSF?production and absorption. Symptoms of hydrocephalus result from the associated increased intracranial pressure (ICP). Headache is the most prominent symptom, often occurring in the morning and improving later in the day. Nausea and vomiting, behavior or personality changes, and progressive alterations in mental status occur as the hydrocephalus worsens. Pressure on the brainstem may cause bradycardia, hypertension, and an altered respiratory pattern (Cushing triad). Compression on the sixth or third cranial nerve may produce extraocular muscle abnormalities and diplopia. In infants, bulging fontanelles, frontal bossing, prominent scalp veins, and impaired upward gaze resulting in the “setting sun” sign may be seen. If untreated, hydrocephalus results in cognitive and developmental abnormalities, seizures, and potentially progression to herniation and death.Definitive treatment of hydrocephalus involves surgical drainage and placement of a mechanical shunt to allow ongoing drainage of excess CSF. A catheter is placed into one of the lateral ventricles and connected to a one-way valve that opens based on pressure within the ventricle and leads to a catheter that drains fluid to a distal site to allow fluid absorption. Typically, a reservoir is placed near the valve to allow access to the shunt and CSF, if needed. Several different types/mechanisms of valves are used in shunts, but all are based on the concept of opening and closing triggered by a pressure differential between the ventricular system and the shunt tubing. Newer shunts also may allow external adjustment of the pressure differential to open the valve and control the rate of fluid drainage. Overdrainage may result in ventricular collapse, shunt tip occlusion, and symptoms similar to shunt malfunction. The most common site of drainage is the peritoneal cavity (VPS), with ventriculopleural and ventriculoatrial shunts used less commonly.The most common complications of VPS are mechanical shunt malfunction and infection. Nearly 40% of shunts fail due to one of these complications within the first year of placement. Shunt malfunction results in recurrence of ventricular dilation and increased ICP, prompting the previously cited symptoms. Shunt malfunction may result from occlusion due to cellular debris, occlusion of the tip against the ventricular wall, shunt tubing fracture, migration of the shunt, or overdrainage. Shunt infection occurs in 5% to 10% of patients, with the highest rates in newborns and infants. Most shunt infections occur within the first 6 months of shunt placement. The most common organisms are skin flora, including Staphylococcus epidermidis, S aureus, diphtheroids, and streptococci. The most common presenting symptom is fever, with vomiting and other symptoms consistent with shunt malfunction following.After a thorough history and physical examination, evaluation of potential shunt failure should include a shunt series and noncontrast CT scan of the brain. Shunt series includes two views of the entire length of the shunt tubing (head, neck, chest, and abdomen with ventriculoperitoneal shunts) to identify any fractures (Fig. 1) or migration of the shunt tubing. CT scan can delineate the shunt catheter position as well as the size of the ventricular system (Fig. 2). Comparison to prior CT scan images is critical to differentiate stable ventriculomegaly from increasing dilation consistent with a malfunctioning shunt.Evaluation of potential shunt infection requires analysis of the CSF for infection. CSF can be obtained either by percutaneous aspiration of the shunt reservoir or lumbar puncture if a communicating hydrocephalus is present. In general, percutaneous aspiration of a shunt should be performed with neurosurgical consultation, except in conditions of extreme urgency such as impending herniation. Typical CSF studies include culture and Gram stain, measurement of glucose and protein, cell count, and differential count. Antibiotics should be initiated promptly if shunt infection is suspected. However, antibiotics alone are not likely to clear a shunt infection, and removal of the shunt with temporary external ventricular drain placement is necessary in most cases. Once the infection is cleared, a new shunt can be placed.American Board of Pediatrics Content Specification(s):Recognize the signs and symptoms and complications of hydrocephalusRecognize the signs and symptoms of ventricular shunt complicationsKnow the indications for and interpret results of ancillary studies in suspected ventricular shunt complicationsQuestion 6A 4-week-old previously well female infant presents to the emergency department with a 24-hour history of “constant” diarrhea and decreased oral intake. She has had no fever, emesis, or exposure to illness. Her past medical history offers no findings of note, and both her newborn and 2-week-old evaluations yielded normal results. On physical examination, the infant weighs 3.3 kg (10th percentile) and her temperature is 37.5°C, heart rate is 190 beats/min, respiratory rate is 80 breaths/min, blood pressure is 78/52 mm Hg, and pulse oximetry reading is 83% on room air with good waveform. She exhibits cyanosis, unlabored respirations, 10% dehydration, a capillary refill time of 3 seconds, diaper rash, and thrush. The lungs are clear to auscultation with good air exchange, and cardiovascular examination reveals no murmur or gallop; radial and femoral pulses are normal. Chest radiography documents clear lungs and normal cardiothymic silhouette. The abdomen is soft and distended without hepatomegaly or tenderness. The stool is negative for occult blood, and a bedside glucose measures 118 mg/dL (6.5 mmol/L).Of the following, the MOST likely cause of this infant’s low reading on the pulse oximeter isA.abnormal hemoglobinB.alveolar hypoventilationC.poor perfusionD.right-to-left shuntE.ventilation-perfusion mismatchCorrect answer: AThe infant described in the vignette has methemoglobinemia due to acidosis and severe diarrheal illness. Methemoglobin formation results from conversion of iron from the ferrous to the ferric state in response to oxidant stress such as dehydration or acidosis. Young infants lack the reductase enzyme that is required to reduce the ferric state and restore normal hemoglobin.The effortless tachypnea with clear lung fields described for the infant suggests respiratory compensation in response to severe acidosis?resulting from the dehydration and diarrheal losses of bicarbonate. Her good air exchange makes alveolar hypoventilation less likely. Further, if this degree of hypoxemia was due to ventilation-perfusion mismatch, the infant probably would exhibit increased work of breathing or pulmonary findings from underlying lung disease.? Acute cyanosis from underlying congenital heart disease (right-to-left shunt) is very unlikely with the physical examination and radiography findings described for this infant. Peripheral vasoconstriction or hypoperfusion can cause a poor signal that results in an abnormal pulse oximetry reading, but the infant had a good waveform on the oximetry monitor.The pulse oximeter measures light absorbance at only two wavelengths: 660 nm and 940 nm. Assessment of the pulsatile and background light absorbance is used to create a pulse-added absorbance at each wavelength. The pulsatile absorbance corresponds to the arteriolar contribution to absorbance above the tissue and venous background and primarily reflects arteriolar hemoglobin absorbance. Both oxy- and deoxyhemoglobin absorb light at 660 and 940 nm, and the ratio of the absorbance at the two wavelengths determines oxygen saturation. The machines are calibrated from empiric data on healthy individuals who had simultaneous co-oximetry measurement of arterial oxygen saturation.A ratio of absorbance (660:940 nm) of 0.43 corresponds to 100% oxygen saturation and a ratio of 3.4 corresponds to 0% oxygen saturation. In the absence of dyshemoglobin, an absorbance ratio of 1.0 (unity) corresponds to an oxygen saturation of 85%. Methemoglobin absorbs light almost equally at both 660 nm and 940 nm. In the presence of 100% methemoglobin, therefore, the absorbance ratio is approximately 1.0 and the pulse oximetry reading is approximately 85% oxygen saturation. With lower concentrations of methemoglobin, oxygen saturation measured by pulse oximetry is slightly lower. However, when concentrations reach 30% to 35%, the light absorbance ratio reaches a plateau, and the pulse oximetry reading becomes stable in the 82% to 85% range, independent of actual methemoglobin values. Only the presence of deoxyhemoglobin can lower the reading below this range.Interpreting the results from a blood gas analyzer without co-oximetry may lead to a misdiagnosis because the oxygen saturation is calculated rather than measured. A co-oximeter is a simplified spectrophotometer, but unlike a pulse oximeter, it measures light absorbance at several different wavelengths. These wavelengths correspond to specific absorbance characteristics of deoxyhemoglobin, oxyhemoglobin, carboxyhemoglobin, and methemoglobin. Hence, co-oximetry yields accurate results when elevated dyshemoglobin concentrations are suspected.Pulse oximetry is a commonly used parameter that some consider an additional vital sign. When an adequate level of oxygenation is the primary concern, pulse oximetry is a reasonable adjunct to evaluate the patient. If further information is required to assess for adequacy of ventilation or abnormal hemoglobin, co-oximetry or blood gases would be more helpful. For example, significantly elevated carboxyhemoglobin concentrations can lead to falsely reassuring (high) readings on the pulse oximeter despite tissue hypoxemia. Severe anemia (hemoglobin <5 g/dL [50 g/L]) also can result in inaccurate pulse oximetry readings because of diminished concentration of oxyhemoglobin.American Board of Pediatrics Content Specification(s):Discuss the indications for pulse oximetryKnow the anatomy and pathophysiology relevant to pulse oximetryDescribe the key steps and potential pitfalls in performing pulse oximetryQuestion 7A 15-year-old boy presents to the emergency department with a 1-week history of penile discharge and burning while urinating. Emergency department records indicate that he has been seen twice in the past for reactive airways disease and once for a minor head injury. He denies fever, vomiting, loose stool, and sexual activity. Physical examination demonstrates a well-appearing, muscular young man whose vital signs are within normal limits. There has been no discharge from his uncircumcised penis, but stains on his underwear provide evidence of previous discharge. His general, genital, abdominal, and rectal examinations yield no findings of note.Of the following, the MOST likely diagnosis is:A.abuseB.Chlamydia infectionC.human immunodeficiency virus infectionD.syphilisE.urinary tract infectionCorrect answer: BChlamydia infection is the most frequently reported bacterial sexually transmitted infection (STI) in the United States. Even this rank likely underestimates the prevalence because the disease is not always clinically evident. Chlamydia can be transmitted during oral, vaginal, and anal sexual activity and can be passed from mother to infant during the birthing process. It is estimated that 75% of infected women and 50% of infected men are symptom-free. Symptoms usually occur within 1 to 3 weeks after exposure. Women may experience mucopurulent vaginal discharge or dysuria if the urethra is involved as well as abdominal pain, fever, painful intercourse, or intercycle bleeding. Up to 40% of affected women can develop pelvic inflammatory disease, with resultant infertility and pain. Men usually present with discharge or dysuria, as described for the boy in the vignette. They also may experience meatal burning or itching. The testicles usually are not involved.Suspicion for chlamydial disease is important, as is treatment of the primary patient and his or her sexual contacts. Diagnosis can be made? from a urine sample collected without prior antiseptic preparation (DNA amplification using polymerase chain reaction) or local specimen collection (culture). It is possible that an affected patient might have a negative test result due to a variant strain. Because Chlamydia is an intracellular organism, adequate culture specimens must contain epithelial cells (Figs. 1 and 2).Figure 1: A photomicrograph revealing McCoy cell monolayers with Chlamydia trachomatis inclusion bodies; magnified 200X. Chlamydia, caused by Chlamydia trachomatis, is the most common bacterial sexually transmitted infection. Using cell cultures from the McCoy cell line is one method implemented in diagnosing Chlamydial infections. Figure 2: Photomicrograph of C trachomatis taken from a urethral scrape. 200x iodine stained inclusions in McCoy cell lineClinicians always should consider sexual activity or abuse in a pediatric patient who has evidence or suspicion of an STI. Child sexual abuse more commonly occurs in younger children. ??Patients who have syphilis often exhibit a primary painless lesion or a rash in the later stages but can present with another STI as well. Testing for other STIs should be considered once an STI is identified. Any person who has an STI also is at increased risk for human immunodeficiency virus (HIV) infection. Consideration of and evaluation for HIV infection might start with evidence of an STI, but the isolated nature of this patient’s presentation suggests a bacterial process rather than a systemic viral infection. Although a urinary tract infection is a possibility, this is less likely without previous urinary complications. Further, most urinary tract infections do not present with urethral discharge.Other issues to address with this young man, besides his sexual activity and partners, are the use of condoms, appropriate presumptive treatment, consideration of other STIs, and possible involvement of his parents/guardians in the assessment and follow-up plans.Oral doxycycline (200 mg/day in two divided doses) for 7 days or azithromycin in a single 1-g oral dose is the recommended treatment for Chlamydia infections in adolescents or adults. Alternative approaches include 7-day regimens of oral erythromycin base (2.0 g/day in four divided doses), erythromycin ethyl-succinate (3.2 g/day in four divided doses), ofloxacin (600 mg/day in two divided doses), or levofloxacin (500 mg orally once per day).Erythromycin or azithromycin is the recommended therapy for children between 6 months and 12 years of age, and erythromycin is recommended for infants younger than 6 months of age. A single 1-g oral dose of azithromycin or amoxicillin administered 1.5 g/day in three divided doses for 7 days are recommended regimens for pregnant women. A 7-day regimen of erythromycin base (2 g/day in four divided doses) is an alternative regimen. Doxycycline, ofloxacin, and levofloxacin are contraindicated during pregnancy. Repeat testing (preferably by culture) should be undertaken 3 weeks after treatment. Because the regimens for pregnant women may not be highly efficacious, a second course of therapy may be required.American Board of Pediatrics Content Specification(s):Know the etiology by age and pathophysiology of Chlamydia infectionsRecognize signs and symptoms of genital Chlamydia infectionsKnow management, including ancillary studies and principles of acute and chronic Chlamydia infectionRecognize the complications of Chlamydia infectionsQuestion 8A 15-year-old boy presents with complaints of fatigue, nausea and vomiting, abdominal pain, and constipation. The symptoms began gradually several weeks ago but have become more severe. He currently vomits several times each day and has not had an appetite. He has lost at least 5 lb. He denies fever, dysuria or hematuria, respiratory symptoms, or sore throat. His urine output has been increased despite the poor oral intake. His past medical history is notable for several episodes of kidney stones; evaluation at that time revealed hypercalciuria, but no further evaluation or treatment was initiated. He fractured his left humerus 6 months ago while pitching during a baseball game without any direct trauma. On physical examination, his temperature is 37.6°C, heart rate is 118 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 110/60 mm Hg. His mucous membranes are dry. Cardiac examination reveals only tachycardia, and his respiratory examination yields normal results. He has mild diffuse tenderness without guarding or rebound on abdominal examination, and there are no masses or hepatosplenomegaly. Results of genitourinary examination are normal. Neurologic examination reveals a slightly sleepy but arousable patient who has no focal neurologic deficits. Initial laboratory results include:Sodium, 135 mEq/L (135 mmol/L)?Potassium, 3.2 mEq/L (3.2 mmol/L)?Chloride, 100 mEq/L (100 mmol/L)Bicarbonate, 16 mEq/L (16 mmol/L)Blood urea nitrogen, 26 mg/dL (9.3 mmol/L)Creatinine, 1 mg/dL ( 88.4 mcmol/L)Glucose, 110 mg/dL (6.1 mmol/L)Calcium, 13.6 mg/dL (3.4 mmol/L)Of the following, the MOST appropriate initial step(s) in management for this patient is(are):A.abdominal computed tomography scanB.administration of chlorothiazide and pamidronateC.aggressive rehydration followed by administration of furosemideD.emergent dialysis and fluid restrictionE.measurement of serum growth hormone and prolactinCorrect answer: CThe boy described in the vignette has severe hypercalcemia and a history of secondary urolithiasis and pathologic fracture. Initial management of symptomatic hypercalcemia consists of aggressive hydration, with initial fluid boluses of normal saline administered to correct dehydration and intravascular fluid depletion. Loop diuretics such as furosemide induce calciuresis but should be administered only after extracellular fluid volume has been restored. Thiazide diuretics such as chlorothiazide increase renal tubular reabsorption of calcium and, therefore, are contraindicated in patients who have hypercalcemia. Pamidronate is a bisphosphonate that can be used to decrease serum calcium concentrations, but it should be reserved for patients who fail initial therapy with aggressive rehydration and loop diuretics. Dialysis is rarely necessary for hypercalcemia and is reserved for severe cases or those who have failed conventional therapy. Fluid restriction is inappropriate in the presence of hypercalcemia.??Hypercalcemia can be a manifestation of multiple endocrine neoplasia (MEN) syndrome, which can be identified through measurement of serum prolactin, growth hormone, insulin-like growth factor-1, gastrin, glucagon, calcitonin, and catecholamines. Although such assessment may be indicated in the evaluation of this patient, they are not part of the initial emergency department evaluation and management. Noncontrast abdominal computed tomography scan can be used to diagnose urolithiasis but is not indicated in the initial evaluation of the patient in the vignette.??Hypercalcemia in the pediatric patient can be the result of an underlying endocrinologic disorder, medication or dietary intake, prolonged immobilization, or malignancy (Table).Evaluation of the patient in whom hypercalcemia is suspected should begin with a thorough personal and family history. Findings suggesting hypercalcemia include a history of urolithiasis, pathologic fractures, and pancreatitis. Dietary history should exclude excess intake of vitamins A or D or calcium. A history of exposure to medications associated with hypercalcemia, including thiazide diuretics, lithium, and alkali, should be sought.??Familial hypocalciuric hypercalcemia (FHH) is an autosomal dominant disorder associated with hypercalcemia, hypermagnesemia, and hypocalciuria. FHH typically is asymptomatic and requires no therapy. Hyperparathyroidism (HPT) can be sporadic or familial; familial forms typically are autosomal dominant and often associated with the MEN syndrome. HPT can be diagnosed by measurement of serum parathyroid hormone (PTH), calcium, and phosphorus and urine calcium. Urine calcium values should be elevated in HPT, in contrast to the low values seen in FHH. Hormones associated with the additional endocrine abnormalities of MEN should be measured, as discussed previously.??Immobilization can lead to excess boney resorption and secondary hypercalcemia; treatment should include mobilization, increased fluid intake, and avoidance of excess dietary vitamin D and calcium. Malignancy is an unusual cause of hypercalcemia in children but occasionally is seen in cases of leukemia, lymphoma, rhabdomyosarcoma, hepatoblastoma, neuroblastoma, Ewing sarcoma, or metastatic disease to bone. Malignancy-associated hypercalcemia can result from secretion of PTH-related peptide, PTH, or calcitriol or from direct invasion and destruction of bone by the tumor. Excess vitamin D concentrations can result either from excess intake or from ectopic production due to neoplasms, chronic inflammatory disorders, or granulomatous diseases. Hypervitaminosis D can be diagnosed from elevated serum and urinary calcium, low PTH, and elevated calcidiol or calcitriol concentrations.??Mild degrees of hypercalcemia (<12 mg/dL [3.0 mmol/L]) are rarely symptomatic in children, and emergent treatment is unnecessary. Evaluation is aimed at identification and treatment of the underlying cause. As calcium concentrations increase, typical symptoms include anorexia, constipation, nonspecific abdominal pain, and nausea. Severe hypercalcemia (>14 mg/dL [3.5 mmol/L]) can be associated with significant neurologic symptoms that range from lethargy and poor concentration to stupor and coma. Infants may present with poor feeding leading to failure to thrive. Complications of hypercalcemia include urolithiasis, pancreatitis, pathologic fractures, gastric ulceration, and severe renal fluid losses due to impaired concentrating ability. Electrocardiography typically reveals a shortened QT interval.??Treatment is indicated for any patient whose symptoms are due to hypercalcemia as well as those whose calcium concentrations are greater than 12 mg/dL (3 mmol/L). Management of symptomatic or severe hypercalcemia begins with hydration. Initial fluid boluses with normal saline should be administered to correct intravascular fluid depletion. Rehydration then should be continued with 1.5 to 2 times maintenance therapy for the first 24 to 48 hours. Hydration improves hypercalcemia by diluting intravascular calcium and promotes calciuresis by increasing glomerular filtration of calcium and decreasing renal tubular calcium reabsorption. Once intravascular volume has been restored, administration of intravenous loop diuretics further increases calcium excretion in the urine by inhibiting resorption of both calcium and sodium in the ascending loop of Henle.??If hypercalcemia is severe or does not improve with these initial measures, medications that inhibit bone resorption by inhibiting osteoclast function can be administered. Bisphosphonate agents, including pamidronate and etidronate, are the currently used medications. They are administered by intravenous infusion over several hours, and their hypocalcemic effect can persist for days to weeks. Calcitonin can be administered subcutaneously as an adjunct to the bisphosphonates at the beginning of treatment to augment the hypocalcemic effect. Glucocorticoids can aid in decreasing serum calcium concentrations in patients who have hypervitaminosis D or malignancy-related hypercalcemia. Dialysis rarely is necessary but can be used for patients who have severe and refractory hypercalcemia.??American Board of Pediatrics Content Specification(s):??Recognize the signs and symptoms of hypercalcemia??Be familiar with ancillary studies relevant to hypercalcemia??Recognize the life-threatening complications of hypercalcemia?Plan management of acute hypercalcemia ................
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