156b - Biliary Tract Disorders

Question 1

Which serum liver chemistry test is specific for bile duct damage?

Answer 1

Alk-phos

Question 2

Which serum liver chemistry tests indicate hepatocyte death?

Answer 2

ALT and AST

• Stored in hepatocyte cytosol
• Released when they die

Question 3

List 3 significant lab findings in primary biliary cholangitis

Answer 3

• Elevated Alk-phos
• Elevated GGT
• (+) AMA

Question 4

Which part of the liver will be most inflamed in primary biliary cholangitis?

Answer 4

Around the portal triad

(small ducts are obstructed)

Question 5

When does UDP-GT activty begin in the newborn?

Answer 5

5 days after birth (regardless of gestational age)

• UGP-GT conjugates bilirubin
• May see some jaundice before UDP-GT activity starts
  
  • Can use UV light to metabolize

Question 6

Which genetic mutation is most likely in people with intermittent jaundice (when sick) and no other symptoms?

What lab findings will be significant?

Answer 6

Mutation in the promoter region of the UDP-GT gene
-> Gilbert’s Disease

(Extra TA in the TATA box)

Decreased UDP-GT transcription
-> mild unconjugated hyperbilirubinemia

Question 7

What is the most likely cause of the patient’s symptoms?

Answer 7

Alcoholic liver disease

• AST > ALT with both elevated => Alcoholic liver disease

Question 8

What mutation is associated with intrahepatic cholesasis of pregnacy?

What is the treatment?

Answer 8

Abcb4 (aka MDR3)

Treat with ursodeoxycholic acid

• Increases hydrophilicity of bile pool
• Stimulates bile flow
• Helps symptoms
  
  • Decreased pruitis, LFTs
  • Better fetal outcomes