Biliary cirrhosis

Biliary cirrhosis is the cirrhosis of the liver secondary to prolonged obstruction of biliary system, anywhere between the interlobular bile ducts and the papilla of Vater. Obstruction results in progressive destruction of bile ducts.

Primary biliary cirrhosis.
Occurs in chronic inflammation and obliteration of intrahepatic bike ducts.
Mac predominantly in females in the middle age.
Due to immune reactions resulting in liver damage.

Chronic granulomatous inflammation destroying the interlobular ducts, Resulting in fibrosis and later cirrhosis of the livers and its complications.

Clinical features.
1. Cardinal features are pruritis, hyperpigmentation, and jaundice.
2. Liver involvement.
 Progressive jaundice, later becomes intense.
 Patients acquires a ' bottle green colour'.
 Scratch marks, froger clubbing.
 Hepatocellular failure, portal hypertension and ascitis.
3. Hypercholesterolaemia.
 Xanthelasmas around the eyes.
 Xanthomas over joints, tendons, hand creases, elbows and knees.
 Pain, tingling and numbness over feet and hands due to peripheral neuropathy resulting from lip infiltration of peripheral nerves.
4. Malabsorption
 steatorrhoea and diarrhoea  from malabsorption of fat.
 Easy bruising and ecchymosis from vitamin K deficiency.
 Hepatic osteodystrophy -
 might blindness due to vitamin A deficiency.

 Hyperbilirubinaemia of conjugated type.
 Mile elevation of transaminases.
 Two to five dole rise of serum alkaline phosphatase.
 Marked rise of serum 5'- nucleotidase activity
 More than 90% of the patients have antimitochondrial antibodies and increased levels of cryoproteins consisting of immune complexes.
 Antinuclear, antismooth muscle antibodies.
 Lives biopsy confirms the diagnosis.

Ursodeoxycholic acid(10-15mg/kg) improves bilirubin and aminotransferase values.
 Steroids may improve biochemical and histological disease but may lead to osteoporosis.
 Other therapies azathioprine, colchicine, methottrexate.
 Steatorrhoea is treated by limiting eat intake and substituting long chain triglycerides with medium chain triglycerides in the diet.
 Monthly injections of vitamin K.
 Vitamin D 1mg/day.
 Calcium supplementation in the form of calcium gluconate 2-4 g/day.
 Airpinsignocter to reduce osteoporosis.
 Lives transplantation.
 Management of pruritis.
 *cholestyramine 4-16 g/day.
 *Rifampicin, ondansetron and opiate antagonists.

Secondary biliary cirrhosis.
*results from prolonged obstruction to large bike duct by ;
 Bile duct strictures.
 Sclerosing cholangitis.

Clinical features
1. Recurrent abdominal pain in stones.
2. Fluctuating jaundice in stones.3. Previous history of abdominal surgery in strictures.
4. Chronic cholestasis with episodes of ascending cholangitis and even lives abscess.
5. Right upper quadrant pain due to cholangitis or biliary colic
6. Cirrhosis, ascites and portal hypertension are late features.
1 hyperbilirubinaemia of the conjugated type.
2. Markedly elevated serum alkaline phosphatase activity.
3. Ultrasound and CT of abdomen.
4 .ERCP.
5. Lives biopsy.

1. Relief of obstruction to bike flow by ERCP or surgery.
2. Antibiotics in sclerosing cholangitis.

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