Christian's disease
Contents:
Description:
Histiocytosis X (X), a langergansokletochny histiocytosis — the term designating group of diseases with an etiology obscure up to the end at which the pathological immune cells called by histiocytes and eosinophils, actively breed, especially in lungs and bones that causes formation of cicatricial fabric.
Christian's disease symptoms:
Distinguish 3 forms of a langergansokletochny histiocytosis:
* Hend's disease — Schueller — Krischena,
* Abt's disease — Letterera — Siwa,
* Taratynov's disease or eosinophilic granuloma.
Diseases differ both on a current, and according to the forecast, but as it is 3 forms of one disease, their mutual transitions can be observed.
Hend's disease — Schueller — Krischena can strike children at any age. Defects of bones of a skull, a basin, an exophthalmos, not diabetes mellitus, obesity, lag in physical development, a hepatomegalia, a lymphadenopathy, petekhialny rash, seborrhea, changes in lungs, stomatitises are characteristic. Consecutive infection can join a basic disease.
Abt's disease — Letterera — Siwa meets at children of early age more often. Develops sharply, with high fever, skin rashes in a breast and a backbone, a gepatosplenomegaliya (increase in a liver and spleen), a generalized hyperadenosis, otitises, mastoidites, damage of lungs, damage of flat bones.
Taratynov's disease is observed preferential at children of school age. Are typical: the general weakness, increased fatigue, a hyporexia, the ostealgia (are surprised both flat, and tubular bones) raised by SOE, sometimes an eosinophilia. In some cases the disease proceeds asymptomatically and comes to an end with spontaneous treatment. On roentgenograms of bones the destruction centers are found, is more often than a rounded or oval shape without sclerosis zones. In insignificant number of cases a clinical picture of a disease brighter: not sugar mocheiznureniye, exophthalmos, gepato-or gepatosplenomegaliya, anemia, various changes of skin, etc.
Christian's disease reasons:
The etiology is not found out. Are assumed that the certain immunopathological process promoting proliferation of histiocytes is the cornerstone of a histiocytosis of X.
Treatment of the Christian's disease:
Treatment is, as a rule, carried out in a hospital. At acute manifestations appoint glucocorticoids in combination with cytostatics, for example, cyclophosphamide though any means is not obviously effective. In all cases the main therapy is combined with symptomatic.
