The Indian juvenile cirrhosis - Diseases of digestive organs at children
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The Indian juvenile cirrhosis - it the family disease of children of preschool age is widespread on the Indian subcontinent, but meets also on the Middle East, in the Western Africa and Central America. The peak of incidence is the share of age 1 — 5 flying, boys and girls are ill equally often. The mode of inheritance is not established yet; it is possible that the pathogeny is defined by action of the external factors accumulating on genetic predisposition.
Clinical and datas of laboratory. The beginning is usually gradual, but also rapid development of process is possible. The first sign — an asymptomatic hepatomegalia, then there are symptoms of an acute viral hepatitis, such as fever, anorexia, decoloured by kcal, dark urine, jaundice. In one cases the illness quickly passes into cirrhosis, in others — spontaneously stops at any stage. Standard biochemical indicators of functions of a liver are changed, however the only sign specific to this illness — sharp increase of level of serumal immunoglobulins.
Patomorfologiya. In the serial bioptata taken in process of a course of a disease transition from the acute inflammatory process reminding a viral hepatitis to the step necrosis and development of cicatricial fabric characteristic of chronic active hepatitis comes to light. At quickly progressing (malignant) current in a pathomorphologic picture the necrosis, a cellular degeneration and wrinkling of segments prevail. In the hepatocytes surrounded with inflammatory cells Mallori's little bodies are visible. The centers of a necrosis are substituted with cicatricial fabric therefore mikronodulyarny cirrhosis develops. Coloring by orcein reveals considerable deposits of copper, mainly in lysosomes.
Treatment. Specific therapy does not exist. Corticosteroids can as at chronic active hepatitis to lead to long remission.
Forecast. Most of children perish within one year. At survivors slow cirrhosis forms.