BILIARY DISORDERS

Question 1

A neonate with jaundice, dark urine, and pale stools is likely to have which condition? Which investigation would you do and what would you expect to see?

Answer 1

Biliary atresia

Ultrasound will show an absent or abnormal gallbladder and narrow/absence of common bile duct

Question 2

What is primary biliary cirrhosis?

what causes it? What type of cells are involved?

Answer 2

Biliary cirrhosis without extrahepatic obstruction

→ Autoimmune disorder: T-cell attack small intralobular bile ducts IN LIVER ONLY, causing granulomatous inflammation

Question 3

What are the two main presenting symptoms of primary biliary cirrhosis? Explain why these are caused, and which gender this condition is more common in

Answer 3

Fatigue and pruritus (often worse at night)

​→ caused by increased bile acids in skin

condition is more common in women

Question 4

Which investigations would you do in someone with suspected primary biliary cirrhosis? 5 listed

what indicates a poor prognosis?

Answer 4

• Alkaline phosphatase: markedly elevated
• Antimitochondrial antibodies: hallmark of PBC, in 95% of patients
• Antinuclear antibodies: in 70% of patients
• Serum lipids: markedly elevated cholesterol (unable to get lipids out in bile)

► Increased bilirubin occurs late and indicates a poor prognosis

Question 5

What will you see you on imaging in primary biliary cirrhosis?

Answer 5

Absence of biliary obstruction

Question 6

What is the only effective treatment for primary biliary cholangitis (cirrhosis)?

Answer 6

Ursodeoxycholic acid - replaces endogenous bile acids and improves LFTs and disease progression

Question 7

Primary biliary cirrhosis is often associated with other autoimmune disorders. Which is the most common?

Answer 7

Sjogren’s syndrome

Question 8

Which condition is associated with inflammation, fibrosis and strictures in the biliary tree?

which condition is strongly associated with this?

Answer 8

Primary sclerosing cholangitis

strongly associated with ulcerative colitis: 90% of patients with PSC have IBD

Question 9

Primary sclerosing cholangitis vs primary biliary obstruction: ducts affected

Answer 9

• PSC: intra + extrahepatic bile ducts
• PBO: intrahepatic bile ducts only

Question 10

What symptoms do you expect in primary sclerosing cholangitis? 3 listed

Answer 10

• Right upper quadrant pain
• Fatigue
• Jaundice

Question 11

What two things do you expect to be elevated in primary sclerosing cholangitis LFTs?

which antibody is present in 80% of his patients?

Answer 11

• ALP
• Conjugated bilirubin
• Positive p-ANCA

Question 12

A patient with a history of ulcerative colitis presents with right upper quadrant pain and jaundice. What should you suspect and how would you diagnose?

what is the treatment for this condition?

Answer 12

Primary sclerosing cholangitis

Cholangiogram: ERCP or MRCP → will see ‘beading’

Treatment: Stenting, may need liver transplant

Question 13

What should people with primary sclerosing cholangitis be screened for yearly?

Answer 13

Cholangiocarcinoma: bile duct cancer

Risk factors:

• PSC
• Clonorchis sinensis (Chinese liver fluke)