Primary Biliary Cholangitis
> 90% of patients are female Most common in individuals of North European descent • Insidious onset with pruritus, fatigue, jaundice Often other associated autoimmune disorders • AMA(+) Minority of cases are…
Cat-Scratch Disease
Zoonotic infection caused by small, weakly gram-negative coccobacillus Most cases are attributed to B. henselae Clinical Issues • Most cases are children and young adults with history of cat exposure Usually…
Caroli Disease
May fill with bile sludge, stones, &/or pus May affect entire liver or limited to 1 lobe, more commonly left Microscopic • Dilated ducts may show periductal fibrosis, acute and chronic…
Mucinous Cystic Neoplasm
Lined by mucinous columnar epithelium with focal cuboidal, flattened, or papillary areas May have gastric or intestinal metaplasia Varying degrees of dysplasia may be present Densely cellular ovarian-like stroma positive…
Wilson Disease
Inability to excrete copper in bile leads to its accumulation in liver and other tissues Clinical Issues • Variable presentation Acute liver failure, chronic liver disease with fibrosis/cirrhosis, neurologic/neuropsychiatric signs ±…
Paucity of Intrahepatic Bile Ducts (Nonsyndromic)
Portal TractA portal tract in an infant with nonsyndromic paucity of intrahepatic bile ducts shows arteries and veins but no bile ducts. The hepatocytes are compact and clustered. Centrilobular RegionThe…
Nonalcoholic Steatohepatitis
Elevated transaminases • Mainstay of treatment: Management of metabolic conditions Microscopic • Steatosis, predominantly macrovesicular • Lobular inflammation (lymphocytes and Kupffer cells predominates) • Ballooning • Both fat and ballooning are most prominent in zone…
Xanthogranulomatous Cholecystitis
a.k.a. cholegranulomas or cholecystic granulomas Etiology/Pathogenesis • Usually preceded by rupture of Rokitansky-Aschoff sinus Bile and lipid extruded into gallbladder wall, producing granulomatous reaction Clinical Issues • 4-9% of cholecystectomy specimens Majority…
Cholesterol Polyps and Cholesterolosis
Prevalence of 12% in autopsy studies and from 9-26% in surgical pathology studies • Usually asymptomatic and discovered incidentally at cholecystectomy Clinical significance, if any, is unclear • Peak in 5th and…
Nesidioblastosis
Mutations of ABCC8 or KCNJ11 genes encoding subunits of ATP-sensitive potassium channel in majority of cases Mutations of GCK, GLUD1, HSD17B10 (SCHAD), SLC16A1, HNF1A, HNF4A, or UCP2 genes in minority…
