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A Case of Gaucher's Disease.

Journal of the Korean Pediatric Society 1987;30(7):784-790.
Published online July 31, 1987.
A Case of Gaucher's Disease.
Hyo Nam Cho1, Myung Cheol Cho1, Hyung Ro Moon1, Je Geun Chi2, Hyo Min Kim2
1Department of Peidiatircs, College of Medicine, Seoul National University
2Department of Pathology, College of Medicine, Seoul National University
급성(유아형〉Gaucher씨 병 1례
조효남1, 조명 철1, 문형로1, 지 제 근2, 김 효 민2
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 병리학 교실
Abstract
We experienced a case of Gaucher’s disease of acute infantile type in a 12 month old male boy. The patient showed hepatosplenomegaly with anemia and thrombocytopenia, developmental delay and frequent infection. There were characteristic Gaucher’s cells in bone marrow aspiration and biopsy of liver, spleen, & lymph node. Splenectomy was done at 25 month old. He expired 3 days after splenectomy. Autopsy was done. A brief review of literature was made.
Key Words: Gaucher’s disease , Hepatosplenomegaly , Anemia , Thrombocytopenia , Splenectomy , Autopsy.


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