Volume 2, Issue 6 And 7 (4-1993)                   JGUMS 1993, 2(6 And 7): 35-39 | Back to browse issues page

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Noorsalehi. One Case-report of Hand-Schuller-christian Syndrome in Children . JGUMS 1993; 2 (6 and 7) :35-39
URL: http://journal.gums.ac.ir/article-1-2032-en.html
Abstract:   (2245 Views)
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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Review Paper: Research | Subject: Special
Received: 2019/04/10 | Accepted: 2019/04/10 | Published: 2019/04/10

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