All issues > Volume 26(12); 1983

Case Report

J Korean Pediatr Soc. 1983;26(12):1209-1214. Published online December 31, 1983.

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

Yeong Bong Park¹, Jin Heon Kim¹, Jong Myeon Hong¹, Chang Soo Ra¹

¹Department of Pediatrics, College of Medicine, Chosun University, Kwangju Korea

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.

Keywords :Histiocytosis X; Hand-Schiiller-Christian syndrome; Diabetes insipidus.