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A Case of Histiocytosis X(Hand-Schuller-Christian Syndrome).

Journal of the Korean Pediatric Society 1983;26(12):1209-1214.
Published online December 31, 1983.
A Case of Histiocytosis X(Hand-Schuller-Christian Syndrome).
Yeong Bong Park, Jin Heon Kim, Jong Myeon Hong, Chang Soo Ra
Department of Pediatrics, College of Medicine, Chosun University, Kwangju Korea
Histiocytosis X (Hand-Schuller-Christian Syndrome) 의 1 례
박영봉, 김진헌, 홍종면, 나창수
조선대학교 의과대학 소아과학교실
Abstract
A case of histiocytosis X (Hand-Schuller-Christian syndrome) in a 3 year old girl is presented. The patients has the triad of exophthalmos, diabetes insipidus and skeletal lesions, plus hepatomegaly, wide spread pulmonary infiltration and growth retardation. The patient was treated with chlorambucil, prednisone, radiation therapy and chlorpropamide therapy for diabetes insipidus with good response. Similar cases reported in literature are briefly reviewed.
Key Words: Histiocytosis X, Hand-Schiiller-Christian syndrome, Diabetes insipidus.


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