Rubin's Liver (3) Flashcards
“AR inherited defect in ATP mediated hepatocyte copper transport”
Wilson disease
What results from the defect in Wilson disease?
lack of Cu transport into bile and ack of Cu incorporation into ceruloplasmin
Symptoms of Wilson disease?
cirrhosis, neuro manifestations, Keyer- flesher rings in cornea
Does wilson disease increase the risk of liver cancer?
Yes
How does CFTR mutation lead to cirrhosis?
biliary obstruction–> biliary cirrhosis
“AR defect in serine protease inhibitor leading to hepatocytes with PAS droplets”
Alpha-1- antitrypsin deficiency
Most common genetic cause of liver disease?
Alpha-1- antitrypsin deficiency
Does Alpha-1- antitrypsin deficiency increase risk of cancer?
Yes!
“hepatocytes distended with glycogen, no fibrosis, hepatic adenomas in adolescence”
Type I glycogen storage disease
“inherited glycogen storage disease with mild hepatomegaly:
Type II, Pompe
“inherited glycogen storgage disease with severe hepatomegaly, and fibrosis that can progress to cirrhosis”
Type III, cori
“branching enzyme dificiecny that is associated by cirrhosis at a young age”
Type IV, Andersen
Which glycogen storage disease responds to dietary control?
Type I
“infants can not process milk, AR mutation, cataracts, jaundince, hepatosplenomegaly, cirrhosis”
Galactosemia
“AR deficiency of Fructose 1 phosphate aldolase leading to hepatomegaly, jaundice, and cirrhosis”
Hereditary fructose intolerance
“loss of FAH leadig to accumulation of succinyl acdetone and succinyl acetoacetate”
Tyrosinemia
“fatal disorder, micronodular cirrhosis and abundant mallory bodies in indian boys”
Indian childhood cirrhosis
Histologic pattern of acetomenophen liver disease?
Zonal hepatocellular necrosis
centrilobular
Which drugs cause cholestasis?
estrogens and antibiotics`
“cholestasis + inflammation”
cholestatic hepatitis
Which drugs case macrovesicular steatosis?
CCL4, corticosteroids, methotexate and amiodarone
“microvascular steatosis + hepatic failure + encephalopathy”
Reye syndrome
What causes Reye syndrome?
aspirin in the setting of influenza and varicella infectoin
What drugs can cause Budd Chiari?
oral contraceptives
Which drugs cause hepatic adenomas?
estrogen and anabolic steroid use
What exposure can lead to hepatic angiosarcomas?
IV thorium dioxide dye
“photosensitivity and hepatic iron overload in middle aged to elderly patients”
Porphyria cutanea Tarda
“nutmeg liver”
due to chronic passive congestion of the heart from CHF
“liver diffusely speckled with dilated and congested sinusoids and terminal venules”
Acute passive congesion from CHF
“donovan bodies”
Parasitic organisms in the Kupffer cells
Visceral leishmaniasis
“leptospirosis + prolonged fever + jaundice”
Weill syndrome
Most common cause of neonatal hepatitis?
alpha 1 anti-trypsin deficiency
“AD syndrome cauing bile duct atresia from notch pathway mutations”
Algille syndrome
2 risk factors for hepatic adenomas?
oral contraceptives
anabolic steroid use
Major complication of hepatic adenomas?
bleeding into the peritoneal cavity
Most common benign tumor o f the liver?
hemangiomas
“multiple small cystic bile ducts on fibrous stroma”
Bile duct microhamartomas (Von meyenburg complexes)
“liver cysts lined by epithelium associated with PKD”
solitary or multiple simple cysts
“malignant tumor of hepatocytes”
hepatocellular carcinoma
“abd pain + enlarged liver + ascites”
Budd chiari (hepatic vein occlusion)
Increased risk with hepatocelluar carcinoma
“tumor arising from biliary epithelium anywhere along the biliary tree”
Cholangiosarcoma
“rare malignant liver tumor of children”
hepatoblastoma
