Primary biliary cirrhosis

Rare disease affecting mostly women midlife.

SYMPTOMS

Itching appear insidiously and jaundice occurs with febrile seizures.

The liver is big and hard, the rate increased volume.

Biologically, alkaline phosphatase, total fat, cholesterol and bilirubin are increased.

It seems that the administration of codeine can have a very beneficial effect on pruritus that may be these patients.

The primary biliary cirrhosis carries a very important table CHOLESTASIS. Furthermore, the mitochondrial antibody discovery is a very important diagnostic argument.

The cirrhosis is late, it is preceded by liver fibrosis. There is an obstruction of the intrahepatic bile ducts. There is no obstacle in the bile duct.

The chronic course is dotted with complications:

- Appearance of xanthomas,

- Articular manifestations,

- Hypertension portal vein

- Decalcification.

Gastrointestinal bleeding and a large liver failure can take the sick.

This disease can be stored in AUTOIMMUNE DISEASES. The increase in IgM is constant and the presence of anti-mitochondrial antibodies.

The presence of anti-mitochondrial antibodies would be the earliest sign of disease and would predict the onset of the disease several years before the onset of clinical signs.

. It seems that a human virus until then unknown, may be the cause of the disease in susceptible individuals.

It is a retrovirus of the family of murine betaretrovirus

(MMTV).

This virus was detected by electron microscopy the Ochsner clinic foundation of New Orleans (Xu et al) in biliary epithelial cells from three patients affected with primary biliary cirrhosis.

This discovery will lead to new therapeutic strategies.

Liver transplantation is a good therapeutic indication according to strict criteria.

Pending this transplant can sometimes require a long time because of the difficulty of finding a compatible donor, treatment can be given that seems to slow evolution. This treatment is based on ursodeoxycholic acid. This molecule decreases biliary excretion of cholesterol, perhaps by reducing the hepatic synthesis of this lipid, and perhaps also by reducing its intestinal absorption.

The administration of codeine can have very beneficial effects on pruritus, sometimes fierce these patients.