Liver & GIT
All of the following statements are correct, Except:
|A||Antimitochondrial antibodies are positive in primary biliary cirrhosis|
|B||In late stage of PSC treatment is liver transplantation|
|C||In advanced stages of cirrhosis major blood supply to liver comes from Hepatic vein|
|D||Elevated lipoprotein-X in PBC|
Extra Edge: Alcoholic hepatitis: NASH, Wilson, PBC all have hepatomegaly but in post necrotic cirrhosis liver is shrunken.!!!
3. Biliary Cirrhosis: (Ref. H-18thedi, Pg 2595)
Biliary cirrhosis results from injury to or prolonged obstruction of either intrahepatic or extrahepatic biliary system. It is of two types – Primary biliary cirrhosis and secondary biliary cirrhosis.
A. Primary biliary cirrhosis
It is characterized by chronic inflammation and fibrous obliteration of intrahepatic bile ductules.
Pathogenesis –Autoimmune diseases, may be associated with like CREST syndrome, sicca syndrome, auto immune thyroiditis, type I DM and IgA deficiency. Q RTA (Renal tubular acidosis)
Clinical Features – women aged 30-60 years.
i. The earliest symptom is pruritus.
ii. Jaundice and gradual darkening of the exposed areas of the skin occur later, steatorrhea with malabsorption of lipid soluble vitamins occur.
iii. Xanthelasma, Xanthomas
i. Increased levels of serum alkaline phosphatase.Q ( 2 to 5 fold)
ii. Serum 5’ – nucleotidase and γ – glutamyltranspeptidase levels increased.
iii. ALT and AST are minimally increased.
iv. Serum bilirubin is usually normal or slightly raised.
v. Anti mitochondrial antibodies Q (IgG) are seen in 90% cases.
vi. Hyper lipidemia Qis seen.
vii. Abnormal lipoprotein y occur is RBC (it occur in many forms of chronic cholestasis)
i. Ursodiol (Ursodeoxycholic acid)
ii. Pruritus is treated with
1. Antihistamines 2. Narcotic receptor antagonists (naltrexone) 3.Rifampin. 4. Cholestyramine.
5. Plasmapheresis has been used rarely in patients with severe intractable pruritus. (H-18thPg- 2596)
iii. Vitamin A, K, D supplements.
iv. Hepatic transplantation (but reoccur in PBC and autoimmune hepatitis)
v. Colchicine, methotrexate and cyclosporine to slow the progression.
B. Secondary biliary cirrhosis- It is the result of long standing obstruction of the larger extrahepatic ducts. The causes of obstruction are:
i. Postoperative strictures. ii. Gall stones
iii. Chronic pancreatitis iv. Primary sclerosing cholangitis
v. Congenital biliary atresia vi. Cystic fibrosis
vii. Choledochal cyst
i. Jaundice and pruritus are most common symptoms.
ii. Fever and right upper quadrant Q pain is typical.
iii. Treatment – Relief of obstruction results in improvement in both symptoms and survival, even in patients with established cirrhosis.