One Case-report of Hand-Schuller-christian Syndrome in Children

نویسنده

  • Noorsalehi,
چکیده مقاله:

SUMMARY Histiocytosis X is associated with idiopathic Proliferation of histiocytes and infiltration of eosinophils in bone marrow Spleen, Liver and other Soft tissues. Clinically there are three distinct forms:  1 - Eosinophilic granuloma is the most benign and Localized form of the disease.  2- Letterer- Siwe is the most malignant, generalized and fatal form of the disease.  3- Hand- Schuller-Christian syndrome is the most Chronic and Variable form of the disease. This can be diagnosed by the following third:  a: One or more destructive foci in the bones.  b: Uni or bilateral exophthalmus. c: Diabetes insipidus with or without other symptoms of Pituitary deficiency.  This form of disease is seen more in children but can occur in any age group. The reported Case is a 3- Year old boy who was Presented with following Clinical Signs and symptoms: Polyuria, polydipsia and low weight.  A Skin biopsy revealed histiocytosis X (Hand - SchullerChristian) and drugs used are Vinblastine,6 MP, Prednisolone and D.D.A.V.P  

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Hand--schuller--christian Disease.

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Hand schuller christian disease: a rare case report with oral manifestation.

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عنوان ژورنال

دوره 2  شماره 6

صفحات  35- 39

تاریخ انتشار 1993-04

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