Autosomal recessive Classification • Types A and B characterized by acid sphingomyelinase deficiency • Types C and D harbor cholesterol metabolism defect Etiology/Pathogenesis • Inherited lysosomal hydrolase deficiency leads to accumulation of sphingolipid…
Typically 1-3 mm • No fibrous septa between nodules But focal sinusoidal or periportal fibrosis can be seen • Inflammation is absent Bile ducts and hepatic arterioles are normal Top Differential Diagnoses…
Once known etiologies are excluded, then considered idiopathic • By mid-2000s, idiopathic neonatal hepatitis (INH) comprised only 15-30% of neonatal cholestasis due to increased ability to detect and diagnose metabolic and…
Often classified as having high-grade dysplasia given architectural complexity • Neoplastic cells have abundant granular eosinophilic cytoplasm with large nuclei, prominent nucleoli • Both intracytoplasmic and intercellular lumina found, many containing mucin,…
Pale, enlarged hepatocytes may be mistaken for normal or interpreted as fixation artifact • Fatty liver disease Pale, enlarged hepatocytes may be misinterpreted as ballooning hepatocyte degeneration • Glycogen storage disease No…
Characterized by ground-glass or smudged nuclei with margination of chromatin • Multinucleated cells with nuclear molding are less common compared with mucocutaneous HSV infections • Immunohistochemistry with antibodies directed against HSV1/HSV2 confirm…
Infected hepatocytes may be slightly enlarged and multinucleated May show hemorrhagic appearance in areas of necrosis • Varicella-zoster virus hepatitis • CMV hepatitis Gross AppearanceThis liver specimen from an autopsy case shows…
Pseudocyst on CTCT shows a unilocular cyst located in the head of the pancreas, consistent with a pancreatic pseudocyst . Pseudocyst With Hemorrhagic FluidGross photograph shows a pseudocyst in the…
Distinguished clinically • Hereditary fructose intolerance Distinct ultrastructural features • Neonatal hemochromatosis Diagnostic Checklist • Fibrosis with variable amounts of steatosis and cholestasis; requires clinical correlation • High risk of hepatocellular carcinoma Micronodular Cirrhosis…
Marked CongestionIn severe cases, venoocclusive disease is characterized by marked congestion in the sinusoids and can be accompanied by areas of hemorrhage . Congestion and Plate AtrophyEndothelial injury in sinusoids…
