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Karoli's disease


Karoli's disease (Karoli's syndrome) — is for the first time described by Karoli in 1958, is a rare hereditary disease which is characterized cystous expanded intra hepatic bilious channels.

Symptoms of the Disease of Karoli:

There are two types of a disease of Karoli, the simple (true), or isolated form where only bilious channels then stones and a cholangitis form are meshkoobrazno expanded. The second, more irregular shape, is usually known as Karoli's Syndrome. At the same time except expansion of intra hepatic bilious channels (which are expanded minimum) there are still portal hypertensia and inborn hepatic fibrosis. Both forms of a disease can be combined with a polycystosis of kidneys.

The disease affects about 1 of 1 000 000 people, and face cases of a syndrome of Karoli than the isolated Karoli's disease more often. Disease symptoms (a hepatomegalia, abdominal pains) are more often shown at adult age.

In biochemical analysis of blood the increased levels of bilirubin, transaminases, an alkaline phosphatase are defined.

The diagnosis is made by means of a computer tomography and a holangiografiya. This disease has continuously progressing character, leads to secondary cirrhosis, an amyloidosis, liver cancer.

Karoli etiologies:

Inheritance on an autosomal retsissivnomu to type, PKHD1 gene mutation.

Treatment of the Disease of Karoli:

Specific treatment does not exist — for reduction of frequency of cholangites antibacterial therapy is carried out. Transplantation of a liver is very perspective.

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