Liver



Liver

Dr. Oliver followed his notes very closely so I am just going to include the additional information. He also stated that he will finish the remaining liver slides on the 31st following the breast lectures.

Pediatric Liver Disease:

The most famous one is Reye's syndrome.

• This is only seen in kids 90% of patients with PBC

• Chronic, progressive cholestatic disease seen in middle aged women (remember that most autoimmune diseases are seen more in women)

• Pathology- cholestasis, destruction of bile ducts by non-necrotizing granulomatous inflammation called florid duct lesion, hepatocyte necrosis from portal inflammation called piecemeal necrosis, scarring and eventually ending up with micronodular cirrhosis

• Slide- portal triad, granuloma (similar to Robbins 19-29)

• Slide-piecemeal necrosis

• Slide- liver with cirrhosis, greenish color from cholestasis (Robbins 19-28)

3. Primary Sclerosing Cholangitis

• Seen in middle aged patients (important distinction is M:F is 2:1)

• Famous person that died from this- Walter Payton

• Etiology- thought to be some kind of autoimmune disease

• 70% of those with PSC have ulcerative colitis but only 4% of those with ulcerative colitis develop PSC

• Pathology- bile duct degeneration, “onion skin” concentric periductal fibrosis obliteration and scarring of ducts caused by cholestasis and biliary cirrhosis

• Slide- ulcerative colitis without PSC (Curran 5.42, p. 97)

• Slide- onion skin fibrosis with bile duct in the middle (Robbins 19-30)

• Slide- Xray of dye injected into biliary tract

Anomalies of the Biliary Tract

1. Von Meyenburg Complexes:

• Most common, clusters of dilated portal bile ducts in the fibrous stroma under the capsule

2. Polycystic Liver Disease

• Multiple cysts in the liver lined by biliary epithelium

3. Congenital hepatic fibrosis (Curran 5.1)

• More fibrosis: fibrotic portal tracts and fibrous septa

4. Caroli’s Disease

• Multiple ducts communicating with the biliary tree

Circulatory Disorders or Vascular Liver Diseases

1. Liver Infarcts (Robbins 19-33)

• Dual blood supply so infarcts are rare and localized unless main hepatic artery thrombosis

• Infarcts of Zahn, occlusion of branches of portal vein, secondary atrophy with portal HTN

2. Portal Vein Obstruction/Thrombosis

• Extrahepatic (infectious pylephlebitis or inflammation of vein)

• Intrahepatic (cirrhosis, tumors, ab pain, ascites, esophageal varices)

• Banti’s syndrome- subclinical occlusion of portal vein causing idiopathic portal hypertension + splenomegaly

• Slide- infarct of liver, pale wedge shaped infarct

3. Passive congestion

• Rt. Sided heart failure causes backflow of blood down inferior vena cava, and liver is the first thing it goes to so you get congestion of liver

• Nutmeg liver, centrolobular area is where it backs up

4. Centrilobular Necrosis (Robbins 19-34)

• Lt. Sided heart failure, shock, vascular insuffiency

• Centrolobular area is the furthest from the blood supply so it is the most susceptible to infarct

5. Cardiac Sclerosis (Cirrhosis)

• Centrolobular liver fibrosis due to chronic CHF

• Just scarring not true fibrosis

6. Peliosis Hepatitis

• Irregular blood filled cystic spaces, usually with no endothelial lining

• Just pooled blood

• Associated with ananbolic steroids, oral contraceptives, cat scratch fever

7. Hepatic Vein Thrombosis (Budd-Chiari Syndrome) Robbins 19-35

• Acute or chronic

• Tremendous congestion of the liver, can involve the entire liver

• Due to: Pregnancy (can cause stasis), postpartum, oral contraceptives (thrombotic), PNH (paroxysmal nocturnal hemoglobinuria), HCC(hepatocellular carcinoma), webs in hepatic veins

• 30% idiopathic

Pregnancy and Hepatic Disease

1. Preeclampsia/ Eclampsia (Robbins 19-37)

• Hemorrhagic disease, hemorrhage under capsule

• HEELP (hemorrhage, elevated liver enzymes and low platelets)

2. Acute fatty liver of pregnancy

• Usually 3rd trimester, mild to severe hepatic failure

• Looks like Reye’s syndrome-microvesicular steatosis

• If severe enough, termination of pregnancy might be needed to save the woman

3. Intrahepatic Cholestasis of Pregnancy

• Benign condition due to estrogen related derangement of bile acid metabolism

• Mild cholestasis and increased risk of gallstones

Liver and Transplantation

1. Drug Toxicity

• Soon after transplant administered

• Nonspecific cholestasis/ necrosis

2. Graft vs. Host disease

• Not from liver transplant but from bone marrow transplant

• Acute GVH 10-50 days after BM transplant, signs of acute hepatitis

• Chronic GVH >100 days and limited to portal tracts

3. Rejection of implanted livers

• Opposite of GVH- patient’s immune system rejects the graft and fights it

• Acute-portal inflammation, hepatocyte injury

• Chronic- vascular problem

4. Veno-occlusive disease nodular regenerative hyperplasia

• Follows BM transplant (more common in allografts)

• Subendothelial deposition of collagen in central vein

5. Nonimmunologic damage to liver allograft

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