Chronic Cholestatic Biliary Diseases

Question 1

epidemiology of primary biliary cirrhosis

Answer 1

• female:male is 9:1

- most common chronic cholestatic liver disease in adults

Question 2

pathogenesis of PBC

Answer 2

• lymphocyte and plasma cell destruction of small ductules within the liver
• focal duct obliteration with granuloma formation

Question 3

symptoms of PBC

Answer 3

• profound pruritus (bile salts get into periphery and activate histamine)
• fatigue is most common symptom

Question 4

diagnosis of PBC

Answer 4

• increased cholestatic enzymes (BA, GGTP, Alk. phosp)
• cholesterol and IgM increased
• jaundice and low albumin

Question 5

potential risk factors

Answer 5

• anti-mitochondiral antibody M2 similar to E. coli on inner mitochondrial membrane
• complicated by osteodystrophy: inability to absorb fat soluble vitamins

Question 6

associated syndromes with PBC

Answer 6

• Sicca, Sjogrens, CREST, scleroderma, thyroiditis, telangectasia

Question 7

autoimmune cholangitis

Answer 7

• AMA negative PBC
• antinuclear antibody (ANA) and antismooth muscle antibody (ASMA) usually positive
• treat with ursodiol and steroids if necessary

Question 8

primary sclerosing cholangitis

Answer 8

• intra and extrahepatic large ducts involved

- many PSC patients have UC

Question 9

cholangitis is a infection of biliary system defined by Charcot’s triad

Answer 9

• elevated liver enzymes, fever, RUQ pain

Question 10

diagnosis of PSC

Answer 10

• MRCP

Question 11

treatment of PSC

Answer 11

• liver transplant

Question 12

Caroli’s disease

Answer 12

• cystic dilatation of intrahepatic bile ducts
• prone to develop recurrent cholangitis
• slow destruction of liver

Question 13

PTC

Answer 13

• radiologic technique used to visualize the anatomy of the biliary tract
• A contrast medium is injected into a bile duct in the liver, after which X-rays are taken
• It allows access to the biliary tree in cases where endoscopic retrograde cholangiopancreatography (ERCP) has been unsuccessful

Question 14

ERCP

Answer 14

• Through the endoscope, the physician can see the inside of the stomach and duodenum, and inject radiographic contrast into the ducts in the biliary tree and pancreas so they can be seen on X-rays

Question 15

MRCP

Answer 15

• medical imaging technique that uses magnetic resonance imaging to visualise the biliary and pancreatic ducts in a non-invasive manner