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Hand-Schüller-Christian syndrome: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Hend-Schüller-Krischen syndrome is a clinical variant of histiccytosis-X - granulomatous disease of unknown etiology. The clinical picture is characterized by symptoms of diabetes insipidus, exophthalmos (usually one-sided, less often bilateral) and bone defects - mainly the bones of the skull, femurs, vertebrae. Defects of bones in radiographic examination are pockets of enlightenment, reminiscent of the geographical map. Characterized by skin symptoms (xanthomatosis, papular exanthema and purpura), painless tooth loss, growth retardation, infantilism, hypercholesterolemia, pathological fractures of tubular bones. For a long time, the only clinical manifestation of the disease may be symptoms of diabetes insipidus. Hypogonadism, growth retardation due to a deficiency of growth hormone and partial or complete hypopituitarism are observed much less often. The course is benign.
The pathogenesis of the Hend-Schuiller-Crischen syndrome. As a result of the formation of histiocytic granulomas with eosinophilic elements in the gray hillock and other parts of the hypothalamus, the development of the releasing factors of the hypothalamus with the outcome of panhypopituitarism and the development of an antidiuretic hormone with the appearance of symptoms of diabetes insipidus are disrupted. Exophthalmos appears as a result of osseous foci of eosinophil granulomas in the eye sockets.
The causes of the Hend-Schuiller-Crischen syndrome are unknown.
Treatment of Hend-Schuiller-Crischen syndrome. Local forms of the disease are treated with scraping in the area of bone changes. Disseminated forms are amenable to large doses of glucocorticoids or chemotherapy, mainly using cyclophosphamide, methotrexate. As a rule, under the influence of such treatment, neuroendocrine manifestations of the disease are not completely normalized. You should try to correct them, using drugs used to treat diabetes insipidus, panhypopituitarism.
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