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ACUTE RHEUMATIC FEVER (ARF) & RHEUMATIC HEART DISEASE (RHD)CDNA National Guidelines for Public Health UnitsRevision historyVersionDateRevised byChanges1.030 April 2014Acute Rheumatic Fever & Rheumatic Heart Disease Working groupDeveloped by the Acute Rheumatic Fever & Rheumatic Heart Disease Working GroupThe Series of National Guidelines (‘the Guidelines’) have been developed by the Communicable Disease Network Australia and noted by the Australian Health Protection Principal Committee. Their purpose is to provide nationally consistent guidance to public health units (PHUs) in responding to a notifiable or significant disease event. These guidelines capture the knowledge of experienced professionals, and provide guidance on best practice based upon the best available evidence at the time of completion. Readers should not rely solely on the information contained within these guidelines. Guideline information is not intended to be a substitute for advice from other relevant sources including, but not limited to, the advice from a health professional. Clinical judgment and discretion may be required in the interpretation and application of these guidelines. Wherever possible, local knowledge and community priorities should always be considered when implementing these guidelines. The membership of the CDNA and the Australian Health Protection Principal Committee?(AHPPC), and the Australian Government as represented by the Department of Health do not warrant or represent that the information contained in the Guidelines is accurate, current or complete. The CDNA, the AHPPC and the Australian Government do not accept any legal liability or responsibility for any loss, damages, costs or expenses incurred by the use of, or reliance on, or interpretation of, the information contained in the guidelines.Acute rheumatic fever and rheumatic heart disease are sentinel conditions of poverty and of health inequality; their persistence mark the failure of our health and other systems to address the non-communicable diseases of the disadvantaged. While this document does not attempt to address all the social determinants of health there are important considerations for a public health response which should be taken into consideration when planning public health interventions (including but not limited to Environmental Health) specifically in addressing the Closing the Gap National targets. Recommendations made with regards to the social determinants of health, including Environmental Health may be beyond the scope of the Public Health workforce responding to an individual case or an outbreak, but have been included for consideration. Endorsed by CDNA: 27 November 2017Endorsed by AHPPC: 03 July 2018Release of Health: 01 August 2018ACUTE RHEUMATIC FEVER (ARF) & RHEUMATIC HEART DISEASE (RHD)CDNA National Guidelines for Public Health UnitsContents TOC \o "1-3" \h \z \u 1. Summary PAGEREF _Toc10113115 \h 4Public health priority PAGEREF _Toc10113116 \h 4Routine PAGEREF _Toc10113117 \h 4Case management PAGEREF _Toc10113118 \h 5Contact management PAGEREF _Toc10113119 \h 52. The disease PAGEREF _Toc10113120 \h 5Infectious agent PAGEREF _Toc10113121 \h 5Reservoir PAGEREF _Toc10113122 \h 6Mode of transmission PAGEREF _Toc10113123 \h 6Incubation period PAGEREF _Toc10113124 \h 6Infectious period PAGEREF _Toc10113125 \h 7Clinical presentation and outcome PAGEREF _Toc10113126 \h 7Diagnosis of primary ARF PAGEREF _Toc10113127 \h 7Major manifestations PAGEREF _Toc10113128 \h 7Minor manifestations PAGEREF _Toc10113129 \h 8Evidence of group A streptococcal infection PAGEREF _Toc10113130 \h 9Quality of life impact of an acute episode PAGEREF _Toc10113131 \h 10Outcome PAGEREF _Toc10113132 \h 10Diagnosis of ARF recurrence PAGEREF _Toc10113133 \h 10Persons at increased risk of disease PAGEREF _Toc10113134 \h 11Disease occurrence and public health significance PAGEREF _Toc10113135 \h 113. Routine prevention activities PAGEREF _Toc10113136 \h 124. Surveillance objectives PAGEREF _Toc10113137 \h 15Data management PAGEREF _Toc10113138 \h 15Reporting requirements PAGEREF _Toc10113139 \h 155. Communications PAGEREF _Toc10113140 \h 156. Case definitions PAGEREF _Toc10113141 \h 167. Laboratory testing PAGEREF _Toc10113142 \h 178. Case management PAGEREF _Toc10113143 \h 18Response times PAGEREF _Toc10113144 \h 18Response procedure PAGEREF _Toc10113145 \h 18Case investigation PAGEREF _Toc10113146 \h 18Case treatment PAGEREF _Toc10113147 \h 18Education PAGEREF _Toc10113148 \h 19Isolation and restriction PAGEREF _Toc10113149 \h 19Active case finding PAGEREF _Toc10113150 \h 199. Environmental evaluation PAGEREF _Toc10113151 \h 1910. Contact management PAGEREF _Toc10113152 \h 20Contact definition PAGEREF _Toc10113153 \h 20Management of identified contacts PAGEREF _Toc10113154 \h 20Education PAGEREF _Toc10113155 \h 20Isolation and restriction PAGEREF _Toc10113156 \h 2011. Special situations PAGEREF _Toc10113157 \h 20ARF possible outbreak/clustering response PAGEREF _Toc10113158 \h 2012. References and additional sources of information PAGEREF _Toc10113159 \h 23References PAGEREF _Toc10113160 \h 24Appendices PAGEREF _Toc10113161 \h 26Appendix 1: Sample Acute Rheumatic Fever Notification Form for jurisdictions where ARF is notifiable and an ARF/RHD register exists PAGEREF _Toc10113162 \h 26Appendix 2: Example of an Enhanced Surveillance form for person diagnosed with ARF PAGEREF _Toc10113163 \h 27Appendix 3: World Heart Federation Criteria for the Echocardiographic Diagnosis of RHD in Individuals Aged ≤20 Years PAGEREF _Toc10113164 \h 28Appendix 4: Public Health Unit checklist PAGEREF _Toc10113165 \h 29Jurisdictional specific issues PAGEREF _Toc10113166 \h 301. SummaryThe priorities in the control and prevention of acute rheumatic fever (ARF) are:Timely diagnosis with accurate recognition of clinical symptomsNotification and reporting:For ARF - Notification to Public Health Unit by clinician in Western Australia (WA), Northern Territory (NT), Queensland (QLD), South Australia (SA)* and New South Wales (NSW)For Rheumatic Heart Disease (RHD) - Notification to Public Health Unit by clinician in Western Australia (WA), New South Wales (NSW) and South Australia (SA)*Secondary prevention with regular benzathine penicillin G after ARF diagnosis.The aim of this guideline is to summarise the public health aspects of ARF case management, including mitigation of progression to RHD, the chief complication of ARF. It is based on the existing Australian National Guideline (Second Edition, 2012). ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> Public health priority RoutineARF is a sporadic condition in the majority of instances. It has been estimated that a minority of individuals (approximately 3-5 per cent) infected with the causative organism, i.e. rheumatogenic strains of group A streptococcus (GAS), have an inherent susceptibility to developing the autoimmune sequelae which constitute ARF. Although rare, ARF case clusters or outbreaks can occur and a public health response is required (refer to Section 12 - References and additional sources of information).Case managementBased on the Australian National Guideline ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> and 2015 Revised Jones Criteria,PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5HZXdpdHo8L0F1dGhvcj48WWVhcj4yMDE1PC9ZZWFyPjxS

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ADDIN EN.CITE.DATA 2 case management includes:Assignment of ARF as a primary or recurrent case.Assignment of ARF diagnosis as Definite, Probable or Possible.Assignment of RHD diagnosis based on echocardiogram result as absent, mild, moderate or severe, then assignment of clinical priority status as Priority 1 (most severe), Priority 2 or Priority 3. These are denoted P1, P2, P3.Notification and reporting:There is no ARF-specific diagnostic laboratory test. Notification therefore relies on clinical recognition and clinician-initiated reporting. Laboratory evidence of GAS infection is required for definite diagnosis of non-chorea forms of ARF, but this test is not specific for ARF, hence this is not a laboratory-notifiable condition. Under-reporting is a recognized problem, due both to missed diagnosis, and a lack of recognition of the need to notify.Education for case and household/family by healthcare staff including the notifying clinician about primordial and primary preventive strategies to reduce future GAS infections and the subsequent impacts. Commencement of secondary preventive strategiesBenzathine penicillin G intramuscular injection every 21 - 28 days 450mg for individuals <20kg, 900mg for individuals ≥20kg.Ensuring follow up with appropriately-timed future echocardiograms, specialist and dental reviews, in accordance with established care plans based on priority status. ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> Contact management The secondary attack rate for sporadic ARF is well below the threshold required for instigation of contact treatment, ADDIN EN.CITE <EndNote><Cite><Author>Health Protection Agency</Author><Year>2004</Year><RecNum>235</RecNum><DisplayText><style face="superscript">3</style></DisplayText><record><rec-number>235</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1350254920">235</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Health Protection Agency Group A Streptococcus Working Group,</author></authors></contributors><auth-address>james.stuart@.uk</auth-address><titles><title>Interim UK guidelines for management of close community contacts of invasive group A streptococcal disease</title><secondary-title>Commun Dis Public Health</secondary-title><alt-title>Communicable disease and public health / PHLS</alt-title></titles><periodical><full-title>Commun Dis Public Health</full-title><abbr-1>Communicable disease and public health / PHLS</abbr-1></periodical><alt-periodical><full-title>Commun Dis Public Health</full-title><abbr-1>Communicable disease and public health / PHLS</abbr-1></alt-periodical><pages>354-61</pages><volume>7</volume><number>4</number><edition>2005/03/25</edition><keywords><keyword>*Antibiotic Prophylaxis</keyword><keyword>Great Britain</keyword><keyword>Humans</keyword><keyword>Streptococcal Infections/*prevention &amp; control</keyword><keyword>*Streptococcus pyogenes</keyword></keywords><dates><year>2004</year><pub-dates><date>Dec</date></pub-dates></dates><isbn>1462-1843 (Print)&#xD;1462-1843 (Linking)</isbn><accession-num>15786581</accession-num><work-type>Consensus Development Conference&#xD;Guideline&#xD;Practice Guideline&#xD;Review</work-type><urls><related-urls><url> therefore, treatment of household and/or close contacts is not required in sporadic cases.Group A streptococcal infections cluster in households or other living conditions characterised by over-crowding, hence education for households regarding prevention, and ensuring the availability of adequate health hardware, is strongly recommended.Contact management in potential outbreak scenarios is addressed in - HYPERLINK \l "Sect10" Sections 10 & HYPERLINK \l "Sect11" 11 –?Contact Management and Special Situations. 2. The diseaseARF is a preventable disease of socioeconomic disadvantage. Repeated episodes of ARF lead to cumulative cardiac valve damage, resulting in RHD. RHD is an important cause of premature morbidity and mortality in selected Australian populations, particularly Aboriginal and Torres Strait Islander people, Maori, and migrants from Pacific nations. In addition to cardiac involvement other major manifestations include: carditis, arthritis, Sydenham’s chorea, erythema marginatum, and subcutaneous nodules. Minor manifestations include; arthralgia, fever, and elevated acute-phase reactants.Infectious agent The infectious agent is Group A-haemolytic streptococcus (Streptococcus pyogenes or GAS). The potential for non-A-haemolytic streptococci to cause ARF is acknowledged but remains speculative.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5IYWlkYW48L0F1dGhvcj48WWVhcj4yMDAwPC9ZZWFyPjxS

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ADDIN EN.CITE.DATA HYPERLINK \l "ref4" 4, HYPERLINK \l "ref5" 5 ARF is classically understood to follow GAS pharyngitis; however, circumstantial evidence from Northern Australia demonstrates that GAS skin infections (impetigo, pyoderma, skin sores) may be antecedents of ARF.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5DYXJhcGV0aXM8L0F1dGhvcj48WWVhcj4xOTk2PC9ZZWFy

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ADDIN EN.CITE.DATA 1, HYPERLINK \l "ref6" 6- HYPERLINK \l "ref8" 8An abnormal autoimmune host response to GAS is required for the development of ARF. Immune responses to GAS antigens cross-react with host tissues such as cardiac antigens.This occurs only in people with an inherent susceptibility (estimated to occur in 3?to 5?per?cent of people), which may be at least partly genetically mediated. Understanding genetic susceptibility to ARF/RHD is complex HYPERLINK \l "ref9" 9 but evidence is growing. HYPERLINK \l "ref10" 10 The autoimmune response leading to clinically apparent ARF probably only develops after repeated GAS exposures have occurred, accounting for the absence of ARF in infancy. Reservoir Humans are the sole reservoir for GAS. Mode of transmissionGAS is transmitted human-to-human via: direct contactdroplet spread from people with upper airways colonisation or carriage (pharynx,?tonsils, nasal passages)contaminated fomites or surfaces (less common)The importance of crowded living conditions to facilitate transmission has been well documented; e.g. the risk of GAS pharyngeal infection has been shown to be inversely proportional to the distance between a subject’s bed and that of a colonised or infected case (see review HYPERLINK \l "ref11" 11). Poor skin health and household crowding is thought to contribute to the overall burden of GAS infection and carriage.Poverty, household crowding, poor household and community hygiene as risk factors for ARF has recently been confirmed in a large ecological study of over 1000 rheumatic fever cases in New Zealand. HYPERLINK \l "ref12" PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5MZW5ub248L0F1dGhvcj48WWVhcj4yMDA5PC9ZZWFyPjxS

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ADDIN EN.CITE.DATA 12 HYPERLINK \l "_ENREF_18" \o "Bessen, 2009 #5458" Incubation periodThe interval between GAS exposure and pharyngitis (which may be asymptomatic) or clinically apparent impetigo/pyoderma is 1-10 days. The interval between GAS infection and onset of ARF varies depending on: ARF type which can manifest as one or a combination of carditis, arthritis, chorea, erythema marginatum or subcutaneous nodules;factors such as host immune response and whether the episode is primary or a recurrence. In general, the following applies:Rheumatic carditis or arthritis – 2-3 weeks after GAS infection but can be as early as 1 week in recurrent ARF Sydenham’s chorea – 6-9 weeks after GAS infection. Infectious period The infectious period for GAS infection is 10 to 21 days in untreated, uncomplicated cases.Individuals with untreated streptococcal pharyngitis or asymptomatic carriage may carry the organism for weeks to months. Contagiousness of GAS decreases 2 to 3 weeks after onset of infection. Adequate penicillin therapy reduces the infective period to within 24 hours. Clinical presentation and outcomeDiagnosis of primary ARFA diagnosis of ARF can be made (a) after exclusion of differential diagnoses, such as disseminated gonococcal infection or systemic lupus erythematosis, ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> and (b) if the clinical and laboratory features fulfil the Australian modification of the Jones Criteria (now largely incorporated into the 2015?Revised Jones criteria HYPERLINK \l "ref2" PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5HZXdpdHo8L0F1dGhvcj48WWVhcj4yMDE1PC9ZZWFyPjxS

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ADDIN EN.CITE.DATA 2) (Refer to Table 1).A high index of suspicion for ARF is required, especially in at-risk individuals. The Revised Jones Criteria recognise the need for a lower diagnostic threshold in high-risk groups (see Table 1). In Australia, the high-risk populations are: Aboriginal and Torres Strait Islander people, particularly those from northern Australia People of Maori or Pacific Islander background (Melanesia, Micronesia and Polynesia)Immigrants from developing countries Although the recorded primary ARF episode is by definition the first episode to have been recognised and notified, many individuals have already had previous unrecognised ARF, evidenced by the finding of established RHD (as seen on echocardiogram) at the time of ‘primary’ ARF, or by performing a retrospective chart review and finding presentations consistent with ARF where the diagnosis was missed. Prior episodes may also have been inadequately symptomatic for the individual to have sought medical care. (See Table 1)Major manifestationsCarditis: active inflammation of the myocardium, endocardium and pericardium (i.e.?pericarditis). The predominant manifestation of rheumatic carditis is involvement of the mitral and/or aortic valve endocardium presenting as a valvulitis. Echocardiography is the gold standard diagnostic modality, and echocardiogram criteria for rheumatic carditis diagnosis (and RHD diagnosis) are clearly defined.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5SSERBdXN0cmFsaWEgKEFSRi9SSEQgd3JpdGluZyBncm91

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ADDIN EN.CITE.DATA 1, HYPERLINK \l "ref13" 13 Arthritis: swollen and hot joint(s) with pain on movement. This is the most common presenting symptom of ARF. Rheumatic arthritis may have an abrupt onset and last for a few days to weeks and commonly involves the large joints either as a mono-arthritis (single?joint) or polyarthritis (multiple joints). The inflammation is typically migratory (moves from one joint to another) with possible resolution of pain in one joint before onset in the next. Mono-arthritis is a major manifestation in high risk groups (see below) but lower-risk groups require polyarthritis to be a major manifestation; for them monoarthritis is classified as a minor manifestation HYPERLINK \l "ref14" 14.Sydenham’s chorea: abrupt, jerky, involuntary movements +/- associated muscular weakness and emotional lability. These uncoordinated movements especially affect the hands, feet and facial muscles, are often more severe on one side of the body and disappear during sleep. Chorea has the latest onset of timing of all ARF manifestations, with onset from weeks to months after initial streptococcal infection.Erythema marginatum: rare (<2%); highly specific for ARF (considered pathognomonic), but other rashes can be easily mistaken for it. The rash can be difficult to detect in dark-skinned people. The rash appears early in the course of ARF and appears as bright, pink macules or papules that are non-pruritic, blanch under pressure, spread outwards in a circular pattern and may wax and wane over the course of a day. The rash may be found on the trunk or limbs but not on the face.Subcutaneous nodules: also a rare (<2% of cases) but highly specific manifestation of ARF in Aboriginal people. HYPERLINK \l "ref15" ADDIN EN.CITE <EndNote><Cite><Author>Carapetis</Author><Year>2001</Year><RecNum>101</RecNum><DisplayText><style face="superscript">15</style></DisplayText><record><rec-number>101</rec-number><foreign-keys><key app="EN" db-id="sexww5ww3fdraper0w95d2wewp0w00e0wze2">101</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Carapetis, JR, Currie, BJ</author></authors></contributors><titles><title>Rheumatic fever in a high incidence population: The importance of mono-arthritis and low grade fever</title><secondary-title>Arch Dis Child</secondary-title></titles><pages>223-237</pages><volume>85</volume><dates><year>2001</year></dates><urls></urls></record></Cite></EndNote>15 They are 0.5–2 cm in diameter, round, firm, mobile, painless nodules occurring in crops of up to 12 over the elbows, wrists, knees, ankles, Achilles tendons, occiput and posterior spinal processes of the vertebrae. The nodules usually appear in a symmetric distribution, usually present in the first weeks of illness and may be associated with more severe forms of carditis.Minor manifestationsArthralgia: pain on joint movement without evidence of swelling or heat. It usually occurs in the same pattern as rheumatic polyarthritis (migratory, asymmetrical, affecting large joints). Polyarthralgia is a major manifestation in high risk groups (see below) but classified as a minor manifestation in others. Monoarthralgia is a minor manifestation but only for high risk groups. Fever: accompanies most manifestations of ARF, with the exception of chorea. Elevated acute-phase reactants: e.g. CRP, ESR: typically seen in ARF; less so in chorea. Prolonged P-R interval on electrocardiogram (ECG): transient first-degree heart block or other conduction abnormalities (e.g. junctional rhythm) are hallmarks of rheumatic carditis; however, only P-R prolongation is included as a minor criterion. For P-R interval upper limits of normal for different age groups, see Table 1, last footnote. An ECG is required in all cases of suspected ARF. HYPERLINK \l "T1" Table 1: Australian Guidelines for the diagnosis of ARF (from Table 3.2, National Guideline) ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> High Risk? All other groupsDefinite initial episode of ARF2 major or 1 major and 2 minor manifestations plus evidence of a preceding GAS infection?Definite recurrent episode ofARF in a patient with known past ARF or RHD2 major or 1 major and 1 minor or 3 minor manifestations plus evidence of a preceding GAS infection?Probable ARF (firstepisode or recurrence)A clinical presentation that falls short by either one major or one minormanifestation, or the absence of streptococcal serology results, but one in which ARF is considered the most likely diagnosis. Such cases should be further categorised according to the level of confidence with which the diagnosis is made:Probable (‘highly-suspected ARF’)Possible (‘uncertain ARF’)Major manifestationsCarditis (including subclinical evidence of rheumatic valvulitis of echocardiogram)Polyarthritis?? or asepticmono-arthritis or polyarthralgiaChorea§Erythema marginatumSubcutaneous nodulesCarditis (excluding subclinical evidence of rheumatic valvulitis on echocardiogram)Polyarthritis?? Chorea§Erythema marginatum*Subcutaneous nodulesMinor manifestationsMonoarthralgiaFever??ESR ≥30 mm/h or CRP ≥30 mg/LProlonged P-R interval on ECG§Fever??Polyarthralgia or aseptic monoarthritisESR ≥30 mm/h or CRP ≥30 mg/LProlonged P-R interval onECG§§?High-risk groups are those living in communities with high rates of ARF (incidence >30/100,000 per year in 5?to14?year olds) or RHD (all-age prevalence >2/1000). Aboriginal people and Torres Strait Islanders living in rural or remote settings are known to be at high risk. Data are not available for other populations, but Aboriginal people and Torres Strait Islanders living in urban settings, Maori and Pacific Islanders, and immigrants from developing countries, may also be at high risk. ?Elevated or rising antistreptolysin O or other streptococcal antibody, or a positive throat culture or rapid antigen test for GAS. ??A definite history of arthritis is sufficient to satisfy this manifestation. Note that if polyarthritis is present as a major manifestation, polyarthralgia or aseptic mono-arthritis cannot be considered an additional minor manifestation in the same person. §Chorea does not require other manifestations or evidence of preceding GAS infection, provided other causes of chorea are excluded. *Care should be taken not to label other rashes, particularly non-specific viral exanthemata, as erythema marginatum. ??Oral, tympanic or rectal temperature ≥38°C on admission, or a reliably reported fever documented during the current illness. §Upper limit of normal P-R interval is: ages 2-12 years: 0.16 seconds; ages 13-16 years: 0.18 seconds; ages 17+?years: 0.20 seconds. If carditis is present as a major manifestation, a prolonged P-R interval cannot be considered an additional minor manifestation.Evidence of group A streptococcal infectionThis can comprise positive antistreptolysin O titre (ASOT) or anti-DNAseB titre or isolation of group A-haemolytic streptococcus from throat swab; Refer to Section 8 Case?Management.Quality of life impact of an acute episodeAlthough ARF symptoms may be subtle, and the key importance of ARF is the potential for progression to RHD, ARF itself is often a very burdensome illness. ARF requires hospitalisation, to facilitate diagnosis and commence appropriate management and education for the patient and the family.Joint pain and chorea can be very painful and temporarily disabling.Management is burdensome, comprising intramuscular benzathine penicillin G?injections every 21-28 days for a minimum of 10 years, requiring a high level of ongoing engagement with health services.OutcomeAfter primary ARF, outcomes range from full recovery with no permanent sequelae, to a fulminant disease course resulting in death. A recent NT study demonstrated that after a first ARF diagnosis, 61 per cent of people developed RHD within ten years. After RHD diagnosis, 27 per cent developed heart failure within five years ADDIN EN.CITE <EndNote><Cite><Author>Lawrence</Author><Year>2013</Year><RecNum>400</RecNum><DisplayText><style face="superscript">17</style></DisplayText><record><rec-number>400</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1373933331">400</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Lawrence, J. G.</author><author>Carapetis, J. R.</author><author>Griffiths, K.</author><author>Edwards, K.</author><author>Condon, J. R.</author></authors></contributors><auth-address>1Royal Children&apos;s Hospital, Melbourne, Australia.</auth-address><titles><title>Acute Rheumatic Fever and Rheumatic Heart Disease: Incidence and Progression in the Northern Territory of Australia 1997-2010</title><secondary-title>Circulation</secondary-title><alt-title>Circulation</alt-title></titles><periodical><full-title>Circulation</full-title></periodical><alt-periodical><full-title>Circulation</full-title></alt-periodical><edition>2013/06/25</edition><dates><year>2013</year><pub-dates><date>Jun 21</date></pub-dates></dates><isbn>1524-4539 (Electronic)&#xD;0009-7322 (Linking)</isbn><accession-num>23794730</accession-num><urls><related-urls><url>. Careful pregnancy planning in women with RHD of child-bearing age is an important consideration, given elevated foetal and maternal risks. HYPERLINK \l "ref17" PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5XYXRraW5zPC9BdXRob3I+PFllYXI+MjAxMjwvWWVhcj48

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ADDIN EN.CITE.DATA 17 The range of outcomes after a primary ARF episode includes:Resolution of acute episode with no cardiac involvementA rebound phenomenon (flare or relapse of inflammatory and articular features) can occur on weaning or cessation of salicylate/non-steroidal anti-inflammatory drugs (NSAIDs) Resolution of acute episode with cardiac involvement ranging from mild to severeSubsequent ARF recurrence(s) after resolution of primary episode with development of new RHD or progression of existing RHD leading to the potential for early morbidity/mortality related to RHD complicationsAtrial fibrillationHeart failureEndocarditisThromboembolic or haemorrhagic complications including strokeDeath from fulminant carditisARF outcomes with regards to treatment requirements include:All individuals with definite or probable ARF: intramuscular benzathine penicillin G?every 21-28 days for a minimum 10 years as per HYPERLINK \l "Sect8" Section 8 – Case Management Treatment options for RHD, depending on type and severity, which may include:medical management of heart failure including ongoing echocardiograms and intramuscular Benzathine penicillin G injections requiring high level of ongoing engagement with health servicessurgical management of valvular disease (repair or replacement)life-long anticoagulation for certain prosthetic valve types or for atrial fibrillationDiagnosis of ARF recurrenceDefinition of ARF recurrence: new ARF episode occurring >90 days after a prior episode.Diagnosis of ARF recurrence: there is a lower threshold for diagnosis of a recurrence compared with primary episode, as per line 3 of HYPERLINK \l "Table1" Table 1.Timing of recurrences: most recurrences occur within the first 10 years after the primary ARF diagnosis which is the basis for the recommendation to give benzathine penicillin G for this duration. The recurrence rate is highest in the first year, and drops annually thereafter. HYPERLINK \l "ref16" ADDIN EN.CITE <EndNote><Cite><Author>Lawrence</Author><Year>2013</Year><RecNum>400</RecNum><DisplayText><style face="superscript">17</style></DisplayText><record><rec-number>400</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1373933331">400</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Lawrence, J. G.</author><author>Carapetis, J. R.</author><author>Griffiths, K.</author><author>Edwards, K.</author><author>Condon, J. R.</author></authors></contributors><auth-address>1Royal Children&apos;s Hospital, Melbourne, Australia.</auth-address><titles><title>Acute Rheumatic Fever and Rheumatic Heart Disease: Incidence and Progression in the Northern Territory of Australia 1997-2010</title><secondary-title>Circulation</secondary-title><alt-title>Circulation</alt-title></titles><periodical><full-title>Circulation</full-title></periodical><alt-periodical><full-title>Circulation</full-title></alt-periodical><edition>2013/06/25</edition><dates><year>2013</year><pub-dates><date>Jun 21</date></pub-dates></dates><isbn>1524-4539 (Electronic)&#xD;0009-7322 (Linking)</isbn><accession-num>23794730</accession-num><urls><related-urls><url> Persons at increased risk of diseaseIn Australia, the high-risk populations are: Aboriginal and Torres Strait Islander people, particularly those from northern Australia People of Maori or Pacific Islander backgroundImmigrants from developing countries People at risk of ARF are: Children aged 5-14 years, with a peak at around 8 yearsIt is rare for ARF to occur in <3 year-olds HYPERLINK \l "ref18" ADDIN EN.CITE <EndNote><Cite><Author>Olivier</Author><Year>2000</Year><RecNum>5460</RecNum><DisplayText><style face="superscript">19</style></DisplayText><record><rec-number>5460</rec-number><foreign-keys><key app="EN" db-id="xvpwpwx9usst07et2a7v0tdhra2dpz9xvxzv">5460</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Olivier, C.</author></authors></contributors><titles><title> Rheumatic fever - is it still a problem? .</title><secondary-title>J Antimicrob Chemother.</secondary-title></titles><periodical><full-title>J Antimicrob Chemother.</full-title></periodical><pages>13-21</pages><volume>45</volume><dates><year>2000</year></dates><urls></urls></record></Cite></EndNote>18 or > 40 year-olds, although recurrent ARF can occur beyond 40 years-old. People living in a high-risk communityA high-risk community is one where high rates of ARF (incidence >30/100,000 per year in 5–14 year olds) or RHD (all-age prevalence >2/1000) are present ( HYPERLINK \l "Table2" Table?2)Those with increased risk of exposure to GAS infectionCrowded living conditionsInadequate ‘health hardware’ within homes or communities i.e. the physical equipment necessary for healthy, hygienic livingLower levels of health literacySocioeconomic disadvantage Those with an inherent susceptibility to the autoimmune responseAs indicated in Section 2 (Infectious agent), evidence for genetic susceptibility is growing, and remains under investigation in the Australian context and globally.Disease occurrence and public health significanceAustralian ARF rates are available for jurisdictions in which ARF is notifiable or where specific research on this question has been conducted.There are several reasons why ARF and RHD are leading causes for public health concern. Firstly, ARF and RHD are indicators for socioeconomic disadvantage and highlight the requirement for major improvements in the social determinants of health. Secondly, ARF and RHD cause a major burden of premature morbidity and mortality for young Indigenous people in Australia. In the 2006?Australian Institute of Health and Welfare report on mortality in Australia, rheumatic and other valvular disease had the highest differential mortality ratio between Indigenous and non-Indigenous Australians for any clinical category; 19.1, in comparison to 18.2 for nephritis and nephrosis, 18.1 for diabetes, 4.3 for ischaemic heart disease and overall ratio 3.9. HYPERLINK \l "T2" Table 2: ARF incidence rates in selected Australian jurisdictionsJurisdictionARF annual incidence (primary cases and recurrences)Reference and detailsNon-Indigenous Australian bornpopulationAlmost non-existentNo national data on ARF incidence are available. ADDIN EN.CITE <EndNote><Cite><Author>Australian Institute of Health and Welfare</Author><Year>2011</Year><RecNum>1659</RecNum><DisplayText><style face="superscript">20</style></DisplayText><record><rec-number>1659</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1439357789">1659</key></foreign-keys><ref-type name="Report">27</ref-type><contributors><authors><author>Australian Institute of Health and Welfare,</author></authors></contributors><titles><title>Cardiovascular disease: Australian facts. Cardiovascular disease series. Cat. no. CVD 53. Canberra: AIHW. August 2015</access-date></record></Cite></EndNote>19 Number of casesreported via the Australian Paediatric Surveillance Unit in non-Indigenous Australian children in 2007-10 was 10. HYPERLINK \l "ref20" PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5Ob29uYW48L0F1dGhvcj48WWVhcj4yMDEyPC9ZZWFyPjxS

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ADDIN EN.CITE.DATA 20Northern Territory (whole Top End and Central)150-380 / 100,000ARF incidence for NT-wideAboriginal school age children, 2005-9. HYPERLINK \l "ref19" 19 Northern Territory (single Central Australian community) 815 / 100,000 ARF incidence for children aged 5-14?in one community, 1978-1987. HYPERLINK \l "ref21" ADDIN EN.CITE <EndNote><Cite><Author>Brennan</Author><Year>1990</Year><RecNum>127</RecNum><DisplayText><style face="superscript">22</style></DisplayText><record><rec-number>127</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">127</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Brennan, R. E.</author><author>Patel, M. S.</author></authors></contributors><auth-address>Department of Health and Community Services, Alice Springs, NT.</auth-address><titles><title>Acute rheumatic fever and rheumatic heart disease in a rural central Australian aboriginal community</title><secondary-title>Med J Aust</secondary-title><alt-title>The Medical journal of Australia</alt-title></titles><periodical><full-title>Med J Aust</full-title></periodical><pages>335, 338-9</pages><volume>153</volume><number>6</number><edition>1990/09/17</edition><keywords><keyword>Acute Disease</keyword><keyword>Adolescent</keyword><keyword>Adult</keyword><keyword>Age Factors</keyword><keyword>Cause of Death</keyword><keyword>Child</keyword><keyword>Child, Preschool</keyword><keyword>Female</keyword><keyword>Humans</keyword><keyword>Incidence</keyword><keyword>Male</keyword><keyword>Middle Aged</keyword><keyword>Northern Territory/epidemiology</keyword><keyword>*Oceanic Ancestry Group</keyword><keyword>Penicillins/therapeutic use</keyword><keyword>Prevalence</keyword><keyword>Rheumatic Fever/*epidemiology</keyword><keyword>Rheumatic Heart Disease/*epidemiology</keyword><keyword>Rural Health</keyword></keywords><dates><year>1990</year><pub-dates><date>Sep 17</date></pub-dates></dates><isbn>0025-729X (Print)&#xD;0025-729X (Linking)</isbn><accession-num>2233452</accession-num><urls><related-urls><url>, Western Australia 375 / 100,000ARF incidence for Aboriginal children aged 5-14 in the Kimberley, 1988-1992. HYPERLINK \l "ref22" PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5SaWNobW9uZDwvQXV0aG9yPjxZZWFyPjE5OTg8L1llYXI+

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ADDIN EN.CITE.DATA 22 North Queensland156 to 319 per 100,000ARF incidence in north Queensland, 2004-2009. HYPERLINK \l "ref23" ADDIN EN.CITE <EndNote><Cite><Author>Hanna</Author><Year>2010</Year><RecNum>227</RecNum><DisplayText><style face="superscript">24</style></DisplayText><record><rec-number>227</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1350219071">227</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Hanna, J. N.</author><author>Clark, M. F.</author></authors></contributors><auth-address>Cairns Public Health Unit, Tropical Regional Services, Division of the Chief Health Officer, Queensland Health, Cairns, QLD, Australia. Jeffrey_hanna@health..au</auth-address><titles><title>Acute rheumatic fever in Indigenous people in North Queensland: some good news at last?</title><secondary-title>Med J Aust</secondary-title></titles><periodical><full-title>Med J Aust</full-title></periodical><pages>581-4</pages><volume>192</volume><number>10</number><edition>2010/05/19</edition><keywords><keyword>Acute Disease</keyword><keyword>Child</keyword><keyword>Disease Notification</keyword><keyword>Female</keyword><keyword>Humans</keyword><keyword>Male</keyword><keyword>*Oceanic Ancestry Group</keyword><keyword>Population Surveillance/*methods</keyword><keyword>Queensland/epidemiology</keyword><keyword>Recurrence</keyword><keyword>Rheumatic Fever/*epidemiology</keyword></keywords><dates><year>2010</year><pub-dates><date>May 17</date></pub-dates></dates><isbn>0025-729X (Print)&#xD;0025-729X (Linking)</isbn><accession-num>20477734</accession-num><urls><related-urls><url> [pii]</electronic-resource-num><language>eng</language></record></Cite></EndNote>23NSW1.5-2 per 100,000*ARF in 5 – 14 year olds South Australia 30 to 43 per 100,000ARF incidence for SA Aboriginal Heart and Stroke Plan 2015. HYPERLINK \l "ref24" ADDIN EN.CITE <EndNote><Cite><Author>Hanna</Author><Year>2010</Year><RecNum>227</RecNum><DisplayText><style face="superscript">24</style></DisplayText><record><rec-number>227</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1350219071">227</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Hanna, J. N.</author><author>Clark, M. F.</author></authors></contributors><auth-address>Cairns Public Health Unit, Tropical Regional Services, Division of the Chief Health Officer, Queensland Health, Cairns, QLD, Australia. Jeffrey_hanna@health..au</auth-address><titles><title>Acute rheumatic fever in Indigenous people in North Queensland: some good news at last?</title><secondary-title>Med J Aust</secondary-title></titles><periodical><full-title>Med J Aust</full-title></periodical><pages>581-4</pages><volume>192</volume><number>10</number><edition>2010/05/19</edition><keywords><keyword>Acute Disease</keyword><keyword>Child</keyword><keyword>Disease Notification</keyword><keyword>Female</keyword><keyword>Humans</keyword><keyword>Male</keyword><keyword>*Oceanic Ancestry Group</keyword><keyword>Population Surveillance/*methods</keyword><keyword>Queensland/epidemiology</keyword><keyword>Recurrence</keyword><keyword>Rheumatic Fever/*epidemiology</keyword></keywords><dates><year>2010</year><pub-dates><date>May 17</date></pub-dates></dates><isbn>0025-729X (Print)&#xD;0025-729X (Linking)</isbn><accession-num>20477734</accession-num><urls><related-urls><url> [pii]</electronic-resource-num><language>eng</language></record></Cite></EndNote>24* NSW Admitted Patient Data Collection, 2003 – 2012, ICD-10 AM codes (100-102), excludes repeat admissions for individuals. Source: SaPHaRI Centre for Epidemiology and Evidence, NSW Ministry of Health. (excludes recurrences) 3. Routine prevention activitiesThe current cornerstone of ARF/RHD preventive activities in Australia currently is secondary prophylaxis with an antimicrobial agent effective against GAS for those with diagnosed prior ARF/RHDLevels of prevention for ARF/RHD and its sequelae, shown in Table 3, include: Primordial - improved social determinants of healthPrimary - treatment of GAS infection (no vaccination currently available) Secondary - antimicrobial prophylaxis after ARF e.g. intramuscular benzathine penicillin G every 21 - 28 daysTertiary - medical and surgical management of RHD, and consideration of echocardiographic screening in selected populations such as in pregnant women. Primordial and primary preventive strategies classically refer to disease prevention in unaffected hosts and the general community however, these same messages are equally important to provide to individuals after ARF/RHD diagnosis. To potentially reduce their risk of recurrences; a new episode of ARF is estimated to be 10 times more common in an individual with past ARF than in those of similar age and living in the same community/circumstances, but without prior ARF. 4. Surveillance objectivesTo identify cases of ARF and RHD in a timely manner:To notify cases to jurisdictional public health units in accordance with Table 4 for notification purposes to monitor trends, detect outbreaks and potentially trigger contact tracing/screening. To report cases to jurisdictional disease registers in accordance with HYPERLINK \l "Table4" Table 4 for oversight of delivery of ARF and RHD care coordination, in particular commencement and maintenance of secondary prophylaxis, and timeliness of echocardiographic, medical and dental reviews.Data managementDepending on State/Territory requirements (see Table 4), cases should be notified to the jurisdictional notifiable diseases database and/or the RHD disease register (control program), in a timely fashion. Reporting requirementsARF is notifiable in QLD, NT, WA (2015), NSW (2015) and SA (2016). Possible cases are not notifiable.RHD is notifiable in NSW (2015), WA (2015) and SA (2016).ARF/RHD control programs Jurisdictional ARF/RHD control programs exist in QLD, NT, SA, NSW and WA. Each of these jurisdictions maintains a register of ARF/RHD cases. HYPERLINK \l "T4" Table 4: Australian notification requirements *Notifiable to jurisdictional Health Department or Public Health UnitIncluded in jurisdictional registerQLDNTWASANSWQLDNTWASANSWDefinite ARFYesYesYesYesYesYesYesYesYesYesProbable (highly suspected) ARFNoNoYesYesYesYesYesYesYesYesRHDNoNoYesYesYes<35 yrsYesYesYesYesYes* Nil in Vic/ACT or Tas5. CommunicationsCommunicable Disease Directors (or their nominated delegate) should be notified if an ARF outbreak is suspected (see Section 12).6. Case definitionsARFDefinite (confirmed) caseA confirmed case requires clinical evidence AND laboratory suggestive evidence.Clinical evidenceFor initial episode of ARFTwo major manifestations OR One major and two minor manifestations. ORRheumatic Sydenham’s chorea alonec AND other forms of chorea excluded.For recurrent episode in a patient with known past ARF or RHDTwo major manifestationsOROne major and one minor manifestationOR Three minor manifestationsORRheumatic Sydenham’s chorea alonec AND other forms of chorea excludedLaboratory suggestive evidenceElevated or rising antistreptolysin-O or anti-DNase B or other streptococcal antibodyORPositive group A streptococcal (GAS) throat cultureORPositive rapid antigen test for group A streptococci.ARFProbable case (first episode or recurrence)A clinical presentation that falls short by either one major or one minor manifestation ORClinical evidence (as above) without laboratory suggestive evidenceANDwhere ARF is considered the most likely diagnosis by the treating physician. ARFPossible caseAs for probable case, where the treating clinician has less confidence about ARF as the correct diagnosis, but other differential diagnoses have been excluded. RHDDefinite and probable A diagnosis of RHD is made by a cardiologist based on echocardiographic findings. RHD is defined as the presence of specific echocardiographic features summarised in Appendix 1.’ We provide the definition as an appendix. Refer to Appendix 3.Example: A high-risk child with no prior ARF/RHD with monoarthralgia, fever, P-R prolongation (all?minor criteria) and positive streptococcal serology where other causes have been excluded, may be deemed a probable case. A high-risk child with monoarthralgia, fever and elevated CRP only, where other causes have been excluded, may be deemed to be a possible case. The decision is at the discretion of the ARF specialist consulted, based on history, examination findings, history etc.7. Laboratory testingThere is no diagnostic test for ARF. Required laboratory testing includes:Tests to exclude differential diagnosesFull blood count, C-reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR)Supporting evidence of preceding Group A streptococcal infection as per Table 5Numerous laboratory methods for testing ASOT exist, and results can vary significantly between test types. Laboratories may establish locally-appropriate reference ranges for a given ASOT test type and for their population, but if such data are unavailable, the cut-offs provided below are recommended, with advice to practitioners to be mindful of variability between test types. A rise in titre between paired acute and 14-28-day convalescent serum samples may help but also has limitations.1Table 5: Evidence of antecedent group A streptococcal infection SampleDescriptionTest characteristicsUpper limits of normalBloodAntistreptolysin O titre (ASOT) Positive in 75-80% of pharyngeal streptococcal infectionsTypically follows throat streptococcal infectionCan be positive after group A, C or G streptococcal infectionPEVuZE5vdGU+PENpdGU+PEF1dGhvcj5Kb2huc29uPC9BdXRob3I+PFllYXI+MjAxMDwvWWVhcj48

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ADDIN EN.CITE.DATA 43Peak in titre ~3-6 weeks post infectionReturn to normal range may take6-12 monthsAge1-45-1415-2425-34>=35ULN U/mL)170276238177127BloodAntiDNAseB titreTypically follows throat and skin streptococcal infection More specific than ASOT for Group A streptococcal infectionPeak in titre ~6-8 weeks post infectionReturn to normal range may take 6-12 monthsAge1-45-1415-2425-34>=35ULN U/mL)366499473390265Pharyngeal swab (bacterial in transport medium) Group A ???-haemolytic streptococcus cultured from pharyngeal swabOften culture-negative by the time symptomatic ARF is evident;however a positive result provides the required proof to makethe ARF diagnosis even if serological titres for ASOT/ anti-DNaseB are below cutoff. HYPERLINK \l "Sec8" 8. Case managementResponse timesPeople with suspected ARF should be admitted to hospital within 24 hours or as soon as feasible for people living in remote communities. Admission to hospital is advised to: help facilitate the diagnosis including undertaking an echocardiogramensure that adequate information and support is provided to patient and family, given the serious nature of the diagnosis, and need for long-term adherence to treatment and high-level engagement with health servicesResponse procedureCase investigationExamples of an ARF Case notification form and an Enhanced Surveillance form for person diagnosed with ARF is provided in Appendices 1 and 2. Case treatmentCases should be managed by a doctor with expertise in the management of ARF/RHD. Treating doctors seeking specialist advice can be referred through the jurisdictional RHD control programs to an ARF/RHD specialist. Management is guided by the Australian National Guidelines which provide care plans based on the individuals priority status (P1, P2, P3). ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> Some jurisdictional control programs provide education and adherence support directly to patients and their families; elsewhere this responsibility lies with the primary care team.Admission to hospital for further investigation and follow-up is highly recommended.Important components of case management include:Appropriate specialist treatment of the arthritis, carditis and/or chorea ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> e.g. appropriate dosing and duration of salicylate therapy for arthritis HYPERLINK \l "ref1" ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> Education for the patient, family and carers – see belowCommence secondary antibiotic prophylaxis preferably with BPG injections every 21-?28?days Ensure pain minimisation techniques are used for delivery of BPG injections ADDIN EN.CITE <EndNote><Cite><Author>RHDAustralia (ARF/RHD writing group)</Author><Year>2012</Year><RecNum>121</RecNum><DisplayText><style face="superscript">1</style></DisplayText><record><rec-number>121</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="0">121</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>RHDAustralia (ARF/RHD writing group), </author></authors></contributors><titles><title>The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. []</title></titles><number>October 2012</number><dates><year>2012</year></dates><urls><related-urls><url> Acknowledge the special needs of adolescents newly diagnosed with a chronic conditionAlthough individual ARF episodes are acute, the management is akin to chronic disease management with a requirement for regular medication and regular medical care to prevent the long-term complication of rheumatic heart disease Appropriate discharge * planning that includes: communication with primary health care team setting up chronic disease care plan/recalls* Discharge should be delayed until resolution of carditis and inflammatory markers as early discharge has led to fatal decompensation in the community setting. 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ADDIN EN.CITE.DATA HYPERLINK \l "ref44" 44, HYPERLINK \l "ref45" 45 While a link between pyoderma and ARF has never definitively been established, washing with soap and water is an appropriate primordial preventive message to promote for families and communities affected by ARF. Isolation and restrictionNot required Active case findingNot required 9. Environmental evaluationAn Environmental health assessment may be a useful adjuvant to treatment, to address social/environmental issues where this is appropriate and reasonable to do so. The capacity to conduct environmental evaluation varies by region. The appropriate response depends on the geographical setting and the nature of the affected person’s housing. To assist in advising about strategies to avoid future ARF recurrences, the living conditions experienced by the patient could be assessed by an Environmental Health Officer/Unit and in conjunction with the relevant public housing government department or housing provider/funding agency for private housing. Consent should be provided by the family affected and where possible a community member should be present with the family prior to assessment. ?The following describes a suggested response for remote-dwelling Indigenous Australians living as tenants in public or private housing.Issues to assess include:Number of people in the house / per bedroom / per bed (see above and reference HYPERLINK \l "ref42" ADDIN EN.CITE <EndNote><Cite ExcludeYear="1"><Author>Australian Institute of Health and Welfare</Author><Year>2012</Year><RecNum>1712</RecNum><DisplayText><style face="superscript">42</style></DisplayText><record><rec-number>1712</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1445990491">1712</key></foreign-keys><ref-type name="Web Page">12</ref-type><contributors><authors><author>Australian Institute of Health and Welfare,</author></authors></contributors><titles><title>Canadian National Occupancy Standard</title><secondary-title>Metadata Online Registry</secondary-title></titles><number>23/10/2015</number><dates><year>2012</year></dates><pub-location>Canberra</pub-location><publisher>Austrralian Institute of Health and Welfare,</publisher><urls><related-urls><url> for acceptable numbers of bedroom occupants)All functioning health hardware which enables people to wash adequately, especially children, the ability to wash clothing and beddingAccess to hot and cold running water Access to a functional toiletAccess to a hot water washing machine Access to appropriate community hygiene/human-waste removal (safe rubbish removal and disposal, sewage)Deficiencies in the health hardware which are beyond the capacity of the patient’s family to manage should be referred to the jurisdictional government housing authority if the patient lives in public housing or the housing provider if the patient lives in private housing. HYPERLINK \l "Sec10" 10. Contact managementNot routine. Contact management in potential outbreak scenarios is addressed in Section 11 – Special situations. The secondary attack rate for sporadic ARF is well below the threshold required for instigation of contact treatment (e.g. see reference regarding transmission rates HYPERLINK \l "ref3" ADDIN EN.CITE <EndNote><Cite><Author>Health Protection Agency</Author><Year>2004</Year><RecNum>235</RecNum><DisplayText><style face="superscript">3</style></DisplayText><record><rec-number>235</rec-number><foreign-keys><key app="EN" db-id="0wffxfr2hs2fe6e0dznps90w5zs5pf2xz9z2" timestamp="1350254920">235</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Health Protection Agency Group A Streptococcus Working Group,</author></authors></contributors><auth-address>james.stuart@.uk</auth-address><titles><title>Interim UK guidelines for management of close community contacts of invasive group A streptococcal disease</title><secondary-title>Commun Dis Public Health</secondary-title><alt-title>Communicable disease and public health / PHLS</alt-title></titles><periodical><full-title>Commun Dis Public Health</full-title><abbr-1>Communicable disease and public health / PHLS</abbr-1></periodical><alt-periodical><full-title>Commun Dis Public Health</full-title><abbr-1>Communicable disease and public health / PHLS</abbr-1></alt-periodical><pages>354-61</pages><volume>7</volume><number>4</number><edition>2005/03/25</edition><keywords><keyword>*Antibiotic Prophylaxis</keyword><keyword>Great Britain</keyword><keyword>Humans</keyword><keyword>Streptococcal Infections/*prevention &amp; control</keyword><keyword>*Streptococcus pyogenes</keyword></keywords><dates><year>2004</year><pub-dates><date>Dec</date></pub-dates></dates><isbn>1462-1843 (Print)&#xD;1462-1843 (Linking)</isbn><accession-num>15786581</accession-num><work-type>Consensus Development Conference&#xD;Guideline&#xD;Practice Guideline&#xD;Review</work-type><urls><related-urls><url>). ARF occurs in only a small proportion of individuals infected with GAS, perhaps as few as 3-5%. Identification of contactsNot required. Contact definitionNot applicable.Management of identified contactsNot applicable.EducationContacts living with the case person should be included in education about ARF/RHD, in particular the association of GAS transmission with over-crowding and the importance of skin health to reduce GAS transmission for all those living in a household with a person with ARF/RHD. Additionally, for family contacts, the possibility of genetic susceptibility can be raised. Isolation and restrictionNot required. HYPERLINK \l "Sec11" 11. Special situationsARF possible outbreak/clustering responseARF has been considered to be a sporadic condition in Australia. However, in recent years, a small number of situations consistent with possible outbreaks have been observed in parts of northern Australia with already underlying high rates of ARF. This section therefore provides guidance on management of such possible ARF outbreaks, with the caveats that:There is a current lack of evidence to provide clear guidance on ARF outbreak response strategiesThe appropriate definition of an ARF outbreak (specifically, number of cases or type of clustering) is unclear and may evolve over time, or different definitions may be selected in different States/TerritoriesThe time lag between GAS exposure and onset of ARF means that the opportunities for effective outbreak responses are limited Where research laboratory capacity exists to undertake Streptococcal emm-typing of any GAS isolates obtained from pharyngeal or skin swabs, this would be a valuable adjunct to identify whether dominant rheumatogenic GAS strain(s) is/are circulating. This research aspect may help distinguish between an increase in endemic ARF and a true outbreak due to a newly introduced “rheumatogenic” GAS and add to the limited evidence in this area; it is not an essential component of the public health response. Components of outbreak responseCase definition for ARF As per definition provided above, Section 7 Laboratory testingOutbreak definition A greater than expected number of confirmed or probable ARF cases occurring during an approximately 4-week period within a defined region. The threshold number of cases and timing needs to be defined by Public Health Units at regional levels and will vary by background rate of ARF and community population size. For example, two cases in a community, especially if related (e.g. in same family, household or class) may be enough to trigger scrutinyProvision of information to communityBe on the alert for cases of ARF/RHDBe on the alert for known or suspected antecedents of ARF, i.e. pharyngitis or skin sores. Develop a ‘zero tolerance’ approach to these conditions – i.e. ensure all cases are managed appropriately, and education about preventing spread is provided to affected households Ensure staff, especially new or locum staff, know about ARF/RHD (e.g. have undertaken the online training modules – see Resources)Ensure staff have access to the range of resources available to assist clinicians, individuals, families and communities regarding ARF/RHD, including the public service announcements available in English and Aboriginal languages (/films/important-health-message/ ) Control and prevention of further disease The highest priority is to ensure all known people with ARF/RHD currently receiving penicillin prophylaxis in the affected location are up to date with their benzathine penicillin G injections as they are most at riskContact identification and management. The extent of contact tracing and who will be treated will vary by scenario and should be decided in consultation between the community primary care staff, regional Public Health Unit, relevant hospital specialists (paediatricians, infectious disease physicians) and the regional ARF/RHD Control Program. Identify contacts: family, household and close contacts, defined as those staying in the same house as a case in the 4 weeks preceding onset of the ARF in the index case(s)Examine contacts for skin sores or pharyngitis/tonsillitis and provide appropriate management for these conditions (including covering skin sores see Table 3 above)Treatment of all contacts aged >12 months and <50 years not already treated with anti-GAS antibiotic. If prior treatment was with an oral antibiotic more than 7 days prior, re-treat. If prior treatment was with benzathine penicillin G more than 21 days ago, re-treat. The purpose of treating contacts is to prevent further GAS transmission by removing potential sources of GAS infection and to prevent sequelae of GAS infection such as ARF in those recently infected. Active case finding Examine at-risk contacts (e.g. aged <21 years, depending on resources) for features of ARF. Cardiac auscultation by an experienced clinician but where possible use of a portable echocardiogram will provide best sensitivity for diagnosisPEVuZE5vdGU+PENpdGU+PEF1dGhvcj5Sb2JlcnRzPC9BdXRob3I+PFllYXI+MjAxMzwvWWVhcj48

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ADDIN EN.CITE.DATA 46Collect samples for bacterial culture +/- typing of Group A Streptococcus from all cases (already routine practice), but additionally from all contacts; ideally both throat swabs and swabs of any skin sores should be collected in this setting. Provide community education about ARF and ARF prevention strategiesTable 6: Dosage table for benzathine penicillin G for treatment of ARF contacts??*WeightDose of benzathine penicillin GVolume?3kg to <6kg6 to<10kg10 to <15 kg15 to <20 kg 20 kg or more225mg337.5mg450mg675mg900mg0.6mL0.86mL1.15mL1.73mL2.3mL? Note that doses differ from doses required for ARF secondary prophylaxis, since the latter requires a large depot dose with longer half-life for prevention of future exposures, whereas the strategy for contact treatment is to eradicate any current GAS infection or carriage.? Note that doses in mL differ from the CARPA manual as the 2015 preparation of Bicillin LA comes as 900mg in 2.3ml. This may well change again, so check current formulation being used. *Those allergic to penicillin should receive azithromycin 12mg/kg up to 500mg orally, daily for 5 days. 12. References and additional sources of informationResourcesARF fact sheet: (.au/resources/10-things-you-should-know-about-rheumatic-fever) Control Program Contact numbers for provision of patient education and clinician advice:NT Top End 08 89228454NT Central 08 89516909Queensland 1300135854Western Australia 1300622745South Australia 08 74257146New South Wales 1300 066 055Tasmania 1800 671 738 (No Control program contact Communicable Disease Prevention Unit for suspected cases) Diagnosis calculator App: .au/apps Treatment tracker App (staying on track with penicillin): .au/treatment-tracker-app RHD in Pregnancy resources: .au/pregnant Educational resources for patients:Videos:.au/resources/search/advanced Written materials: .au/resources/what-rheumatic-fever Educational resources for staff: .au/e-learning-discussion-forum References ADDIN EN.REFLIST HYPERLINK \l "Ausguide" 1.RHDAustralia (ARF/RHD writing group). The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. [.au/arf-rhd-guideline (.au/sites/default/files/guideline_0.pdf). 2012; (October 2012)]. HYPERLINK \l "Gewitz" 2.Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation 2015; 131(20): 1806-18. HYPERLINK \l "HPA" 3.Health Protection Agency Group A Streptococcus Working Group. Interim UK guidelines for management of close community contacts of invasive group A streptococcal disease. Communicable disease and public health / PHLS 2004; 7(4): 354-61. HYPERLINK \l "Haidan" 4.Haidan A, Talay SR, Rohde M, Sriprakash KS, Currie BJ and Chhatwal GS. Pharyngeal carriage of group C and group G streptococci and acute rheumatic fever in an Aboriginal population. Lancet 2000; 356(9236): 1167-9. HYPERLINK \l "McDonald" 5.McDonald M, Towers RJ, Andrews RM, Carapetis JR and Currie BJ. Epidemiology of Streptococcus dysgalactiae subsp. equisimilis in tropical communities, Northern Australia. Emerg Infect Dis 2007; 13(11): 1694-700. HYPERLINK \l "Carapetis" 6.Carapetis JR and Currie BJ. Group A streptococcus, pyoderma, and rheumatic fever. Lancet 1996; 347(9010): 1271-2.7.McDonald M, Brown A, Edwards T, et al. Apparent contrasting rates of pharyngitis and pyoderma in regions where rheumatic heart disease is highly prevalent. Heart, lung & circulation 2007; 16(4): 254-9. HYPERLINK \l "McDonaldTowers" 8.McDonald MI, Towers RJ, Andrews RM, Benger N, Currie BJ and Carapetis JR. Low rates of streptococcal pharyngitis and high rates of pyoderma in Australian aboriginal communities where acute rheumatic fever is hyperendemic. Clin Infect Dis 2006; 43(6): 683-9. HYPERLINK \l "Bryant" 9.Bryant PA, Robins-Browne R, Carapetis JR and Curtis N. Some of the people, some of the time: susceptibility to acute rheumatic fever. Circulation 2009; 119(5): 742-53. HYPERLINK \l "deDassel" 10.de Dassel JL, Ralph AP and Carapetis JR. Controlling acute rheumatic fever and rheumatic heart disease in developing countries: are we getting closer? Current opinion in pediatrics 2015; 27(1): 116-23. HYPERLINK \l "Ralph" 11.Ralph AP and Carapetis JR. Group A streptococcal diseases and their global burden. Current topics in microbiology and immunology 2013; 368: 1-27. HYPERLINK \l "Lennon" 12.Lennon D, Stewart J, Farrell E, Palmer A and Mason H. School-based prevention of acute rheumatic fever: a group randomized trial in New Zealand. Pediatr Infect Dis J 2009; 28(9): 787-94. HYPERLINK \l "Remenyi" 13.Remenyi B, Wilson N, Steer A, et al. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease--an evidence-based guideline. Nature reviews Cardiology 2012; 9(5): 297-309. HYPERLINK \l "Carapetis2001" 14.Carapetis J and Currie, BJ. Rheumatic fever in a high incidence population: The importance of mono-arthritis and low grade fever. Arch Dis Child 2001; 85: 223-37. HYPERLINK \l "Carapetis1999" 15.Carapetis JR and Currie BJ. Rheumatic chorea in northern Australia: a clinical and epidemiological study. Archives of disease in childhood 1999; 80(4): 353-8. HYPERLINK \l "Lawrence" 16.Lawrence JG, Carapetis JR, Griffiths K, Edwards K and Condon JR. Acute Rheumatic Fever and Rheumatic Heart Disease: Incidence and Progression in the Northern Territory of Australia 1997-2010. Circulation 2013. HYPERLINK \l "Watkins" 17.Watkins DA, Sebitloane M, Engel ME and Mayosi BM. The burden of antenatal heart disease in South Africa: a systematic review. BMC cardiovascular disorders 2012; 12: 23. HYPERLINK \l "Olivier" 18.Olivier C. Rheumatic fever - is it still a problem? . J Antimicrob Chemother 2000; 45: 13-21. HYPERLINK \l "AIHW" 19.Australian Institute of Health and Welfare. Cardiovascular disease: Australian facts. Cardiovascular disease series. Cat. no. CVD 53. Canberra: AIHW. (.au/WorkArea/DownloadAsset.aspx?id=10737418530), 2011. HYPERLINK \l "Noonan" 20.Noonan S, Zurynski YA, Currie BJ, et al. A National Prospective Surveillance Study of Acute Rheumatic Fever in Australian Children. Pediatr Infect Dis J 2012. HYPERLINK \l "Brennan" 21.Brennan RE and Patel MS. Acute rheumatic fever and rheumatic heart disease in a rural central Australian aboriginal community. Med J Aust 1990; 153(6): 335, 8-9. HYPERLINK \l "Richmond" 22.Richmond P and Harris L. Rheumatic fever in the Kimberley region of Western Australia. Journal of tropical pediatrics 1998; 44(3): 148-52. HYPERLINK \l "Hanna" 23.Hanna JN and Clark MF. Acute rheumatic fever in Indigenous people in North Queensland: some good news at last? Med J Aust 2010; 192(10): 581-4. HYPERLINK \l "Chidgzey" 24.Chidgzey PJ. Applied epidemiology in the Kimberley: acute rheumatic fever increase in Western Australia [master’s thesis].Canberra: Australian National University; 2015 HYPERLINK \l "Kerdemelidis" 25.Kerdemelidis M, Lennon DR, Arroll B, Peat B and Jarman J. The primary prevention of rheumatic fever. J?Paediatr Child Health 2010; 46(9): 534-48. HYPERLINK \l "Jaine" 26.Jaine R, Baker M and Venugopal K. Acute rheumatic fever associated with household crowding in a developed country. Pediatr Infect Dis J 2011; 30(4): 315-9.27.Wannamaker LW. The epidemiology of streptococcal infections. New York, NY: Columbia University Press; 1954.28.Adanja B, Vlajinac H and Jarebinski M. Socioeconomic factors in the etiology of rheumatic fever. J Hyg Epidemiol Microbiol Immunol 1988; 32(3): 329-35.29.Okello E, Kakande B, Sebatta E, et al. Socioeconomic and environmental risk factors among rheumatic heart disease patients in Uganda. PLoS One 2012; 7(8): e43917.30.Zaman MM, Yoshiike N, Chowdhury AH, et al. Socio-economic deprivation associated with acute rheumatic fever. A hospital-based case-control study in Bangladesh. Paediatr Perinat Epidemiol 1997; 11(3): 322-32.31.Lenhart MK. Recruit Medicine. USA: Office of the Surgeon General, Department of the Army; 2006.32.Perry W, Siegel A, Rammelkamp C and Wannamaker L. Transmission of Group A Streptococci I. The Role of Contaminated Bedding. American Journal of Hygiene 1957; 66: 85-95.33.Falck G, Kjellander J and Schwan A. Recurrence rate of streptococcal pharyngitis related to hygienic measures. Scand J Prim Health Care 1998; 16(1): 8-12.34.Aboriginal Environmental Health Unit. Closing the gap: 10 years of Housing for Health in NSW An evaluation of a healthy housing intervention. Sydney: NSW Health, 2010.35.Lennon D and Stewart J. An important investment to control Acute Rheumatic Fever needs to run its course. The New Zealand medical journal 2015; 128(1416): 6-9.36.Irlam J, Mayosi BM, Engel M and Gaziano TA. Primary prevention of acute rheumatic fever and rheumatic heart disease with penicillin in South African children with pharyngitis: a cost-effectiveness analysis. Circulation Cardiovascular quality and outcomes 2013; 6(3): 343-51.37.McDonald M, Currie BJ and Carapetis JR. Acute rheumatic fever: a chink in the chain that links the heart to the throat? The Lancet infectious diseases 2004; 4(4): 240-5.38.Australian Government National Health and Medical Research Council. Staying Healthy - Preventing infectious diseases in early childhood education and care services FIFTH EDITION (Updated June 2013) (.au/_files_nhmrc/publications/attachments/ch55_staying_healthy_5th_edition_150602.pdf), 2012.39.Antibiotic Expert Groups. Therapeutic Guidelines: Antibiotic. Version 15. Melbourne, Australia: Therapeutic Guidelines Limited; 2014.40.Padmavati S, Sharma KB and Jayaram O. Epidemiology and prophylaxis of rheumatic fever in Delhi--a five year follow-up. Singapore medical journal 1973; 14(3): 457-61.41.Manyemba J and Mayosi BM. Penicillin for secondary prevention of rheumatic fever. Cochrane database of systematic reviews 2002; (3): CD002227. HYPERLINK \l "WHO2004" 42.WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 October - 1 November 2001. WHO Technical Report Series 923. 2004. whqlibdoc.who.int/trs/WHO_TRS_923.pdf (accessed October 2012.43.Australian Institute of Health and Welfare. Canadian National Occupancy Standard. 2012. (meteor..au/content/index.phtml/itemId/386254) (accessed 23/10/2015. HYPERLINK \l "Johnson" 44.Johnson DR, Kurlan R, Leckman J and Kaplan EL. The human immune response to streptococcal extracellular antigens: clinical, diagnostic, and potential pathogenetic implications. Clin Infect Dis 2010; 50(4): 481-90. HYPERLINK \l "Luby2002" 45.Luby S, Agboatwalla M, Schnell BM, Hoekstra RM, Rahbar MH and Keswick BH. The effect of antibacterial soap on impetigo incidence, Karachi, Pakistan. Am J Trop Med Hyg 2002; 67(4): 430-5.46.Luby SP, Agboatwalla M, Feikin DR, et al. Effect of handwashing on child health: a randomised controlled trial. Lancet 2005; 366(9481): 225-33.AppendicesAppendix 1: Sample Acute Rheumatic Fever Notification Form for jurisdictions where ARF is notifiable and an ARF/RHD register exists“ARF is a notifiable condition. Report all confirmed and suspected cases to your nearest Public Health Unit, who will inform the ARF/RHD Register & Control Program”Patient detailsHospital/Clinic UR no __________________Surname ____________________________Given names ________________Date of birth _____/_____/_____Sex ________________________________Address: ____________________________Suburb/Town ________________________ Postcode ________Phone number(s):_____________________EthnicityAboriginalTorres Strait IslanderAboriginal and Torres Strait IslanderPacific Islander - MaoriPacific Islander - OtherOther: Please state_______________Not statedCountry of birth__________________Notification Date ____/____/____Notifying clinicianClinician’s name_______________________Clinician’s signature____________________Hospital/Clinic name____________________Contact email and phone_________________Current ARF episode This episode is:Select 1: Initial ARF /Recurrent ARF/ UnknownSelect 1: Confirmed / Probable/ PossibleARF symptom onset date:___/___/___RHD identifiedYES / NO / Echo not performedIf YES: date RHD identified: ___/___/___Severity: Priority 1 (severe) / Priority 2 (moderate) / Priority 3 (mild)Date Bicillin given: _____/_____/______ Hospitalisation: YES / NO If YES: date __/_____ Hospital___________ARF diagnostic process Differs depending on risk level. Groups recognsied as high risk or potentially high risk include Aboriginal and Torres Strait Islander Australians, Maori and Pacific Islander people, and migrants from developing counties. MAJOR manifestations (tick all that apply)Poly-arthralgia (high risk groups)Aseptic mono-arthritis (high risk groups)Poly-arthritisCarditisSub-clinical carditis (high risk groups)Erythema marginatumSydenham’s choreaSubcutaneous nodulesMINOR manifestations (tick all that apply)Poly-arthralgia (low risk groups)Aseptic mono-arthritis (low risk groups)Mono-arthralgia (high risk groups)Fever (≥38 °C): _____ °CProlonged P-R interval on ECG ESR (≥30 mm/hr): _____ Date____/___/____CRP (≥30 mg/L): ______ Date ____/___/____Evidence of preceding Group A Strep (GAS) infections 3Elevated ASOT: Yes / Noresult ______ IU/mL Date ___/___/___ Elevated Anti-DNaseB: Yes / Noresult_______IU/mL Date ___/___/___Positive throat swab culture: Yes / No Positive skin swab culture: Yes / No History of URTI / Strep throat: Yes / NoModified JONES CRITERIA for ARF diagnosis.Initial episode of ARF 2 Major manifestationsor1 Major and 2 Minor manifestationsplus evidence of a preceding GAS infectionRecurrent episode:2 Major manifestationsor1 Major and 1 Minor manifestationsor3 Minor Manifestationsplus evidence of a preceding GAS infectionSydenham’s chorea alone is enough to confirm ARFAppendix 2: Example of an Enhanced Surveillance form for person diagnosed with ARF1. Date completed: ____/____/_____ Name of person completing form: ___________ Contact email: _________________2. Case Code: ______3. Date of Birth: ____/____/________4. Sex: FORMCHECKBOX M FORMCHECKBOX F5. Post code: __________6. Country of birth: _____________7. Place of birth: ___________8. Usual place of residence: Capital city / Large town /Small town /Remote area9. Ethnicity: Aboriginal and/or Torres Strait Islander /Caucasian /Asian /Pacific Islander/Middle Eastern /African/Other 10. Number of other children in the family: FORMCHECKBOX 0 FORMCHECKBOX 1 FORMCHECKBOX 2 FORMCHECKBOX 3 FORMCHECKBOX 4 FORMCHECKBOX 5 FORMCHECKBOX >5 FORMCHECKBOX unknown11. How many people usually sleep in the dwelling (approx)? FORMCHECKBOX 1-4 FORMCHECKBOX 5-9 FORMCHECKBOX >9 FORMCHECKBOX unknown12. How many of these people are children aged <15yrs FORMCHECKBOX 1-4 FORMCHECKBOX 5-9 FORMCHECKBOX >9 FORMCHECKBOX unknown13. Do other family members/residents in same dwelling have Hx of ARF or RHD? FORMCHECKBOX Yes FORMCHECKBOX No FORMCHECKBOX unknown 14. Date of diagnosis for current ARF episode:____/____/________15. Date of symptom onset for current ARF episode:____/____/________16. Has this child been previously diagnosed with ARF? FORMCHECKBOX Yes FORMCHECKBOX No FORMCHECKBOX unknownMonths/years for previous diagnoses: ____/________/________/____17. Please select all diagnostic criteria present in the current episode of ARF:MAJOR criteria: FORMCHECKBOX Carditis FORMCHECKBOX Polyarthritis FORMCHECKBOX Sydenham’s Chorea FORMCHECKBOX Erythema marginatum FORMCHECKBOX Subcutaneous nodules FORMCHECKBOX Polyarthralgia (high risk groups) FORMCHECKBOX Aseptic mono-arthritis (high risk groups)MINOR criteria: FORMCHECKBOX Fever (max temp__ oC) FORMCHECKBOX ESR ≥30mm/hr (highest ESR_______mm/hr) FORMCHECKBOX Prolonged PR interval FORMCHECKBOX CRP ≥30mg/L (highest CRP_______mg/L) FORMCHECKBOX Aseptic mono-arthritis (low risk groups) FORMCHECKBOX Polyarthralgia (low risk groups)18. Diagnosis FORMCHECKBOX Definite ARF FORMCHECKBOX Probable (highly suspected) ARF FORMCHECKBOX Possible (uncertain) ARF19. Were any cardiac valve lesions present? FORMCHECKBOX Yes FORMCHECKBOX No FORMCHECKBOX unknownIf yes: (tick all that apply)19a. FORMCHECKBOX Mitral valve regurgitation:Severity: FORMCHECKBOX None FORMCHECKBOX Mild FORMCHECKBOX Moderate FORMCHECKBOX Severe FORMCHECKBOX unknown19b. FORMCHECKBOX Mitral stenosis Severity: FORMCHECKBOX None FORMCHECKBOX Mild FORMCHECKBOX Moderate FORMCHECKBOX Severe FORMCHECKBOX unknown19c. FORMCHECKBOX Aortic valve regurgitationSeverity: FORMCHECKBOX None FORMCHECKBOX Mild FORMCHECKBOX Moderate FORMCHECKBOX Severe FORMCHECKBOX unknown19d. FORMCHECKBOX Aortic stenosis Severity: FORMCHECKBOX None FORMCHECKBOX Mild FORMCHECKBOX Moderate FORMCHECKBOX Severe FORMCHECKBOX unknown19e. FORMCHECKBOX Tricuspid valve lesion (specify) ______________ FORMCHECKBOX unknown19f. FORMCHECKBOX Pulmonary valve lesion (specify) _____________ FORMCHECKBOX unknown20. Evidence of valve lesions is based on FORMCHECKBOX Echocardiogram FORMCHECKBOX Clinical Assessment21. Was there a sore throat within 3 weeks of ARF symptoms? FORMCHECKBOX Yes FORMCHECKBOX No FORMCHECKBOX unknown22. Was there evidence of skin sores within 3 weeks of ARF? FORMCHECKBOX Yes FORMCHECKBOX No FORMCHECKBOX unknown23. Was there evidence of Group A streptococcal (GAS) infection? FORMCHECKBOX Yes FORMCHECKBOX No FORMCHECKBOX unknownIf yes, please provide the following details: FORMCHECKBOX Culture: FORMCHECKBOX Yes FORMCHECKBOX No FORMCHECKBOX unknown If yes, identify site: throat / skin / other FORMCHECKBOX M type if GAS isolate typed ______________ FORMCHECKBOX ASOT titreResult______________Date ____/____/________ FORMCHECKBOX Anti DNase titre Result______________Date ____/____/________Appendix 3: World Heart Federation Criteria for the Echocardiographic Diagnosis of RHD in Individuals Aged ≤20 YearsEchocardiographic Criteria for RHD*Definite RHD (A, B, C, or D)A) Pathologic MR and at least 2 morphologic features of RHD of the MVB) MS mean gradient ≥4 mm HgC) Pathological AR and at least 2 morphological features of RHD of the AVD) Borderline disease of both the AV and MVBorderline RHD (A, B, or C)A) At least 2 morphologic features of RHD of the MV without pathologic MR or MSB) Pathologic MRC) Pathologic AREchocardiographic criteria for pathologic regurgitation (all 4 Doppler criteria must be met)Pathologic MR1. Seen in 2 viewsPathologic AR1. Seen in 2 views2. In at least 1 view jet length ≥2 cm?2. In at least 1 view jet length ≥1 cm?3. Peak velocity ≥3 m/s for 1 complete envelope3. Peak velocity ≥3 m/s in early diastole4. Pansystolic jet in at least 1 envelope4. Pandiastolic jet in at least 1 envelopeMorphologic features of RHDFeatures in the MV1. AMVL thickening ≥3 mm?Features in the AV1. Irregular or focal thickening2. Chordal thickening2. Coaptation defect3. Restricted leaflet motion3. Restricted leaflet motion4. Excessive leaflet tip motion during systole4. ProlapseAbbreviations: AMVL,anterior mitral valve leaflet; AR, aortic regurgitation; AV, aortic valve; MR, mitral regurgitation; MS, mitral stenosis; MV, mitral valve; and RHD indicates rheumatic heart disease.*?Congenital anomalies must be excluded.??A regurgitant jet length should be measured from the vena contracta to the last pixel of regurgitant color (blue or red) on nonmagnified (nonzoomed) images.??AMVL thickness should be measured during diastole at full excursion. Measurement should be taken at the thickest portion of the leaflet and should be performed on a frame with maximal separation of chordae from the leaflet tissue.Appendix 4: Public Health Unit checklist (details will vary significantly according to jurisdiction). Ensure all criteria are markedContact the patient’s doctor to: Patient ID number: _______________Yes No Not doneCommenced ARF Case notification form and an Enhanced Surveillance form (Appendix 1 & 2)Yes No Not doneCommenced jurisdictional notification formYes No Not doneObtained necessary history from notes or interview of patient or clinicianYes No Not doneConfirmed results of relevant pathology tests or recommend that the tests be doneYes No Not doneEnsure the patient diagnostic workup is completed including ECG, CRP or ESR, serology and/or culture. * Follow-up serology may be required if negative or potentially mistimedYes No Not doneRecommended follow-up echocardiograms, specialist and dental reviews, in accordance with established care plans based on priority statusYes No Not doneCommenced secondary prevention with regular benzathine penicillin G after ARF diagnosisYes No Not doneEnsured treating team is aware of local resources for patient and family education (including information on primordial and primary prevention strategies as well as the details of secondary prophylaxis provision locally)Yes No Not doneEnsured the diagnosis is recorded clearly in the patients fileYes No Not doneProvided feedback to clinicians if prior missed episodes or inadequate workup is identifiedContact the patient (or caregiver) to:Yes No Not doneConfirmed onset date and symptoms of the illnessYes No Not doneEnsured patient is consented for inclusion on the jurisdictional register according to local eligibility criteriaYes No Not doneProvided education for case and household/family about primordial and primary preventive strategies to reduce future GAS infections and the subsequent impactsYes No Not doneEnsured the patient is referred to the best available RHD Control program or service for continuity of education, appropriate pain relief and ability to recall for follow up Contact laboratory to:Yes No Not doneObtain any outstanding results for supporting evidence of preceding Group A streptococcal infection as per Table 5antistreptolysin-O or anti-DNase B or other streptococcal antibody ORPositive group A streptococcal (GAS) throat culture ORPositive rapid antigen test for group A streptococci.Contact local Housing Authority to:Yes No Not doneInform them of case details and environmental/housing informationOther issues:Yes No Not doneUsed the RHDAustralia Diagnosis Calculator App to assist early detection and diagnosis of ARF Yes No Not doneAssessed information against case definition to confirm caseYes No Not doneEntered case data onto notifiable diseases database (process varies in each jurisdiction)Yes No Not doneConsider referral for a housing assessment according to local protocolYes No Not doneMaintained awareness of the possibility of ARF clusters/outbreaks and have a protocol in place to investigate and respond Yes No Not doneConsider alerting local RHD program if there are more cases than usual Yes No Not doneConsider active case finding where appropriate (refer Section 12)Jurisdictional specific issuesARF is not currently notifiable in ACT, Victoria or Tasmania. See Table 4.Cross jurisdictional/border issues: Control programs or treating clinicians should ensure that for patients transferred from one jurisdiction to another, all relevant details, especially benzathine penicillin dosing dates, are provided. No formal transfer of care form is currently in use. This process is guided by the CDNA Cross-border NNDSS Notification Protocol.There is no National register. For information on RHD programs and notification process in each jurisdiction visit: .au ................
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