Parenchymatous jaundices at children of chest age - Diseases of digestive organs at children
Page 56 of 73 IDIOPATHIC HEPATITIS OF NEWBORNS
Idiopathic defeat of a parenchyma of a liver makes 35 — 45% of all cholestatic states at children of chest age. The statistics shows that at boys it occurs twice more often than at girls, incidence is higher among premature children, family cases give the grounds to assume an autosomal and recessive mode of inheritance or existence of some environmental factor.
Clinical manifestations. The jaundice caused by hyperproduction of not conjugated bilirubin usually arises on the first week of life (80%), but can develop up to 3-month age at any time. As a rule, jaundice — the only symptom of a disease, but sometimes it is accompanied by slight anemia, a small appetite and vomiting. Dark urine and periodic decolorization a calla indicate an intra hepatic cholestasia. Almost at all patients the liver is increased, edge its equal and dense. In 50% of cases the splenomegaly is observed.
Patomorfologiya. The structure of segments is broken owing to considerable giant-cell transformation. These cells prevail in the central zones of segments. Bile accumulation by colossal cells and formation of clots of bile in tubules testifies to a heavy cholestasia in an acute stage of an illness. In every fifth case in portal zones find the cells inherent in an acute or chronic inflammation (fig. 12-25).
Datas of laboratory. Content of bilirubin in serum reaches 0,08 — 0,12 g/l, and more than 60% it are made by direct bilirubin. Level of aminotransferases increases to 400 ME, but this indicator is extremely variable. The quantity of an alkaline phosphatase is a little increased, and albumine and gamma-globulin — normal; the prothrombin time is slightly increased.
Treatment. There is no specific treatment; corticosteroids are inefficient.
Fig. 12-25. A liver at "hepatitis of newborns" (giant-cell hepatitis). Accumulations of colossal cells in a portal zone. Inflammatory cells surround a bilious channel (shooter), a branch of a portal vein (V), a hepatic arteriole (and). Existence of a pericholangitis and fibrosis indicates forming of a chronic illness of a liver. X 100.
Forecast. By the end of the first year of life the absolute recovery comes at 75% of patients, in 25% of cases the illness passes into a chronic stage from the outcome in mikronodulyarny cirrhosis.