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 |
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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.
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Key Words:
Gaucher’s disease , Hepatosplenomegaly , Anemia , Thrombocytopenia , Splenectomy , Autopsy.
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