Obstructive (Cholestatic) Liver DZ Flashcards
What are the types of cholestatic liver dz?
Intrahepatic, extrahepatic
What is the most common cause of intrahepatic cholestasis?
Drugs (OCPs, anabolic steroids)
What causes extrahepatic cholestasis?
Blockage of CBD
What is the most common cause of extrahepatic cholestasis?
Choledocholithiasis
What microscopic findings are seen in cholestatic liver dz?
Bile ducts are distended
What are clinical findings of cholestatic liver disease?
Jaundice, pruritus, malabsorption, cholesterol deposits, light stools
Why can pregnant women develop cholestasis?
Estrogen inhibition of intrahepatic bile secretion- not dangerous
Jaundice present in a newborn at birth should make you think of what condition?
Biliary atresia
Primary sclerosing cholangitis has a strong association with what other dz?
Ulcerative colitis
What is the characteristic histologic finding in a patient with primary sclerosing cholangitis?
Onion skin- periductal fibrosis
What are the complications of sclerosing cholangitis?
Cirrhosis, cholangiocarcinoma
Define the onset of physiologic jaundice in the newborn.
First week, but not first 24 hours
Is physiologic jaundice due to increased conjugated or unconjugated bilirubin?
Unconjugated
What is the mechanism of physiologic jaundice?
Increased RBC turnover, immaturity of bilirubin conjugation system, deconjugating enzymes in breast milk
Generally, physiologic jaundice is benign. However, what toxic outcome can occur if left physiologic jaundice does not resolve and is left untreated?
Kernicterus- toxic accumulation of unconjugated bilirubin in the brain
Define the onset of pathologic jaundice in the newborn.
Onset in first 24 hours or >14 days after birth
What hereditary syndromes result in unconjugated hyperbilirubinemia?
Crigler-Najjar, Gilbert
What hereditary syndromes result in conjugated hyperbilirubinemia?
Dubin-Johnson, Rotor
What is the defect in Crigler-Najjar syndrome?
Decreased function (AD) or no function (AR) of UGT
What is the defect in Gilbert syndrome?
Reduced function of UGT
Both Dubin-Johnson and Rotor syndrome have defective excretion of bile into intrahepatic bile ducts. What histologic difference exists between the two?
Dubin-Johnson has black pigment in hepatocytes and Rotor does not
What is the classic triad in a pt with choledochal cyst? Most pts present before what age?
Pain, jaundice, RUQ mass. Most present by age 10
What is the hallmark finding of neonatal hepatitis?
Giant cell transformation
