Gaucher's Disease: A Report of Two Cases in Homozygous Twins
10.4055/jkoa.1990.25.3.967
- Author:
Jae Do KANG
;
Kwang Yul KIM
;
Yang Hun LEE
;
Keun Soo LEE
- Publication Type:Case Report
- Keywords:
Gaucher's disease;
Homozygous twins
- MeSH:
African Continental Ancestry Group;
Bone Marrow;
Gaucher Disease;
Humans;
Jews;
Korea;
Liver;
Mononuclear Phagocyte System;
Spleen;
Twins
- From:The Journal of the Korean Orthopaedic Association
1990;25(3):967-972
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Gaucher's disease is an uncommon metabolic disorder, which was first described by Gaucher in 1882, characterized by accumulation of distinctive Gaucher's cells in the reticuloendothelial system such as spleen, liver, and bone marrow. The great majority of cases have been reported in Jews, and others in negros and orientals. We are presenting two cases in homozygous twins in Korea, whose clinical manifestations are hepatosplenomegaly and bone lesions due to expansion of involved bones.