Obstructive (Cholestatic) Liver DZ

Question 1

What are the types of cholestatic liver dz?

Answer 1

Intrahepatic, extrahepatic

Question 2

What is the most common cause of intrahepatic cholestasis?

Answer 2

Drugs (OCPs, anabolic steroids)

Question 3

What causes extrahepatic cholestasis?

Answer 3

Blockage of CBD

Question 4

What is the most common cause of extrahepatic cholestasis?

Answer 4

Choledocholithiasis

Question 5

What microscopic findings are seen in cholestatic liver dz?

Answer 5

Bile ducts are distended

Question 6

What are clinical findings of cholestatic liver disease?

Answer 6

Jaundice, pruritus, malabsorption, cholesterol deposits, light stools

Question 7

Why can pregnant women develop cholestasis?

Answer 7

Estrogen inhibition of intrahepatic bile secretion- not dangerous

Question 8

Jaundice present in a newborn at birth should make you think of what condition?

Answer 8

Biliary atresia

Question 9

Primary sclerosing cholangitis has a strong association with what other dz?

Answer 9

Ulcerative colitis

Question 10

What is the characteristic histologic finding in a patient with primary sclerosing cholangitis?

Answer 10

Onion skin- periductal fibrosis

Question 11

What are the complications of sclerosing cholangitis?

Answer 11

Cirrhosis, cholangiocarcinoma

Question 12

Define the onset of physiologic jaundice in the newborn.

Answer 12

First week, but not first 24 hours

Question 13

Is physiologic jaundice due to increased conjugated or unconjugated bilirubin?

Answer 13

Unconjugated

Question 14

What is the mechanism of physiologic jaundice?

Answer 14

Increased RBC turnover, immaturity of bilirubin conjugation system, deconjugating enzymes in breast milk

Question 15

Generally, physiologic jaundice is benign. However, what toxic outcome can occur if left physiologic jaundice does not resolve and is left untreated?

Answer 15

Kernicterus- toxic accumulation of unconjugated bilirubin in the brain

Question 16

Define the onset of pathologic jaundice in the newborn.

Answer 16

Onset in first 24 hours or >14 days after birth

Question 17

What hereditary syndromes result in unconjugated hyperbilirubinemia?

Answer 17

Crigler-Najjar, Gilbert

Question 18

What hereditary syndromes result in conjugated hyperbilirubinemia?

Answer 18

Dubin-Johnson, Rotor

Question 19

What is the defect in Crigler-Najjar syndrome?

Answer 19

Decreased function (AD) or no function (AR) of UGT

Question 20

What is the defect in Gilbert syndrome?

Answer 20

Reduced function of UGT

Question 21

Both Dubin-Johnson and Rotor syndrome have defective excretion of bile into intrahepatic bile ducts. What histologic difference exists between the two?

Answer 21

Dubin-Johnson has black pigment in hepatocytes and Rotor does not

Question 22

What is the classic triad in a pt with choledochal cyst? Most pts present before what age?

Answer 22

Pain, jaundice, RUQ mass. Most present by age 10

Question 23

What is the hallmark finding of neonatal hepatitis?

Answer 23

Giant cell transformation