Biliary Liver Disease

Question 1

What is biliary disease of the liver?

Answer 1

•Liver disease as a result of an abnormality in the biliary tree/system (bile ducts)

Question 2

What is it characterised by?

Answer 2

cholestatsis

Question 3

Where will the abnormalities be?

Answer 3

1. Intrahepatic biliary system 2. Extrahepatic biliary system

Question 4

Name the 3 major disorders of biliary liver disease?

Answer 4

1. primary biliary cirrhosis 2. secondary biliary cirrhosis 3. primary sclerosing cholangitis

Question 5

What is primary biliary cirrhosis?

Answer 5

•Chronic progressive cholestatic liver disease

Question 6

What is primary biliary cirrhosis characterised by?

Answer 6

1. destruction of medium sized intrahepatic bile ducts
2. Portal inflammation and scarring
3. Eventual cirrhosis and liver failure
4. Elevated canalicular liver enzyme Alkaline phosphatase and cholesterol
5. hepatomegaly

Question 7

Epidemiology of primary billiary cirrhosis?

Answer 7

young women

Question 8

Cause of primary billiary cirrhosis?

Answer 8

autoimmune

Question 9

Insidious onset of primary billiary cirrhosis is with?

Answer 9

pruritus

Question 10

Late onset of primary billiary cirrhosis is with?

Answer 10

jaundice

Question 11

What is secondary biliary cirrhosis?

Answer 11

prolonged obstruction of biliary tree resulting in changes in the liver

Question 12

What are the causes of obstruction in adults?

Answer 12

1. Gallstones (extrahepatic) 2. Malignancies in biliary tree or head of pancreas 3. Strictures from previous surgery

Question 13

What are the causes of obstruction in children?

Answer 13

1. Biliary atresia 2. paucity if bile ductsyndromes 3. cystic fibrosis 4. choledochal cysts

Question 14

Describe the morphology of biliary cirrhosis?

Answer 14

1. Yellow green hard liver with a finely granular surface
2. cholestasis
3. Secondary inflammation due to obstruction
4. Periportal fibrosis
5. Hepatic scarring and nodule formation
6. cirrhosis

Question 15

What is primary sclerosing cholangitis characterised by?

Answer 15

1. inflammation and obliterative fibrosis of intrahepatic and extrahepatic bile ducts
2. Dilatation of preserved segments
3. There is alternating segments of stricture and dilatation
4. Commonly seen with inflammatory bowel disease (ulcerative colitis)

Question 16

Cause of primary sclerosing cholangitis?

Answer 16

unknown

Question 17

Describe the pathogenesis of primary sclerosing cholangitis?

Answer 17

1. secretion of proinflammatory cytokines by hepatic macrophages 2. Portal inflammation with concentric periductal fibrosis (onion skin)

Question 18

Symptoms of asymptomatic patients with primary sclerosing cholangitis?

Answer 18

persistent elevation of alkaline phosphatase

Question 19

Symptoms of symptomatic patients?

Answer 19

fatigue, pruritus and jaundice

Question 20

Describe the morphology of primary sclerosing cholangitis?

Answer 20

1. Fibrosing cholangitis of bile ducts 2. Portal and bile duct lymphocytic infiltrate 3. Progressive atrophy of bile duct epithelium 4. Obliteration of the lumen 5. cholestasis 6. cirrhosis