Jaundice
Low levels of UGT1A1, responsible for bilirubin glucuronidation
What is physiologic jaundice of newborn?
Right sided heart failure; dilated centrilobular sinusoids with atrophy of centrilobular hepatocytes
What is chronic passive congestion?
Central stellate scar; women; benign
What is focal nodular hyperplasia?
PAS positive globules in hepatocyte cytoplasm; substitution of lysine for glutamine at #342
What is alpha 1 antitrypsin deficiency?
Molecular mimicry
What is the mechanism by which a foreign antigen shares sequence or structural similarities with self-antigen?
Severe UGT1A1 deficiency
What is Crigler-Najjar syndrome type 1
Liver enlargement, abdominal pain, ascites
What is Budd-Chiari syndrome (portal vein thrombosis)?
OCP and/or anabolic steroid use; HNFalpha inactivated; benign
What is hepatocellular adenoma?
Auto. recessive mutation of ATP7B gene; hepatolenticular degeneration; impaired copper in ceruloplasmin
What is Wilson's disease?
Primary biliary cirrhosis
Which patients with liver disease have increased serum antimitochondrial antibodies?
Choledocholithiasis; cholangiocarcinoma; pancreatic adenocarcinoma
What are causes of large bile duct obstruction & intrahepatic cholestasis?
Neonatal umbilical sepsis; umbilical vein catheteriz'n; hypercoagulable state; pancreatic CA associated splenic vein thrombosis
What are causes of portal vein obstruction/thrombosis?
Hepatitis B infection; hepatitis C infection; cirrhosis; AFP positive
What is hepatocellular carcinoma?
Mutation of HFE which encodes an HLA class I-like molecule regulating intestinal iron absorption via hepcidin regulation.
What is adult form of hemochromatosis?
NK-T cells
Which innate(like) immune cells play a major role in autoimmune hepatitis?
Severe hemolytic anemia (RBC destruction); Bru 3.5mg/dL
What is unconjugated hyperbilirubinemia?
Ascites, esophageal varices, incr AST/ALT
What is cirrhosis?
Klatskin tumor
What is perihilar biliary adenocarcinoma (often at junction of right and left hepatic ducts)?
Hepatomegaly, abd pain, incr skin pigment, DM, cardiac issues, arthritis
What is 'bronze diabetes' or hemochromatosis?
p-ANCA, anti-SMA, and ANA
What are the 3 characteristic serologic markers in PSC?
Painless jaundice
What is often adenocarcinoma of head of pancreas?
Hepatic artery thrombosis and portal vein thrombosis
What is a cause of hepatic infarct?
NAFLD, metabolic syndrome, hemochromatosis, alpha 1 antitrypsin
What are non-hepatitis causes of hepatocellular carcinoma?
Steatosis, focal hepatocyte necrosis, cirrhosis, choreoathetosis, Kayser-Fleischer rings
What is Wilson's disease?
PDC-E2
What is the dominant autoantigen of PBC?