| Two Cases of Gaucher's Disease in Brothers. |
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Jeong Sick Min1, Il Whan Kim1, Dae Young Hwang1, Hyun Gi Jeong1, Jae Sun Park1, In Sun Jun2, Man Ha Huh2 |
1Department of Pediatrics,Kosin Medical College, Pusan Korea
2Department of Pathology, Kosin Medical College, Pusan Korea |
| Gaucher씨 병 2례 |
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민정식1, 김일환1, 황대영1, 정현기1, 박재선1, 전인선2, 허만하2 |
1고신의대 소아과학교실
2고신의대 병리학교실 |
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| Abstract |
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We experienced 2 cases of Gaucher' s disease of adult type in brothers when their ages were 4 year 1 month and 3 year 10 month respectively. They showed remarkable hepatosplenomegaly with mild hematologic complications and characteristic histiocytoid cells in the spleen and liver biopsies. Splenectomy was followed by improvement of anemia and labored respiration in both patients. In case 1, however, two episode of fever and leg pain with suspected aseptic osteomyelitis and increased hepatomegaly was noticed after splenectomy. A brief review of literature was made.
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| Key Words:
Hepatosplenomegaly, Anemia, Gaucher' s disease, Splenectomy |
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