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Inborn fibrosis of a liver


Liver fibrosis inborn - the disease which is shown gepatosplenomegaliy, portal hypertensia at the kept function of a liver. It is observed preferential at children of post-natal and early age.

Symptoms of Inborn fibrosis of a liver:

Significant increase in a liver, sometimes stony density is noted, the splenomegaly takes place. Functional trials of a liver without changes. Portal hypertensia can develop at various age (of 6 months to the adult). Esophageal and gastric bleedings owing to a rupture of varicose veins of a gullet which are observed more often at children are characteristic 3 years are more senior and can be a cause of death. Collateral circulation and ascites are possible.

Reasons of Inborn fibrosis of a liver:

The etiology is not established. More or less widespread portal fibrosis which is accurately separated from a segment takes place; the boundary layer of hepatocytes remains absolutely normal. The architecture of segments is completely kept, there are no nodes of regeneration and damage of hepatic cells, in segments there are no cholestasia signs.

Treatment of Inborn fibrosis of a liver:

Treatment in the period of a decompensation of functions of a liver stationary, includes the same means that at an exacerbation of chronic hepatitis, and also posindromny therapy. At ascites, hypostases sodium is strictly limited, food with enough potassium, the diuretics, albumine, drugs stopping esophageal, gastric and intestinal bleeding is appointed.

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