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Pyridoxine responsive sideroblastic anemia in a boy with mitral valve prolapse

Korean Journal of Pediatrics 2006;49(11):1223-1226.
Published online November 15, 2006.
Pyridoxine responsive sideroblastic anemia in a boy with mitral valve prolapse
June Seung Sung, Ki Hwan Kim, Dong Gyun Han, Mi Jeong Kim, Young Kook Cho, Hae Yul Chung, Hee Jo Baek, Jae Sook Ma, Hoon Kook, Tai Ju Hwang
Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Me
Pyridoxine responsive sideroblastic anemia in a boy with mitral valve prolapse
성준승, 김기환, 한동균, 김미정, 조영국, 정해율, 백희조, 마재숙, 국훈, 황태주
전남대학교 의과대학 소아과학교실
Correspondence: 
Hoon Kook, Email: hoonkook@chonnam.ac.kr
Abstract
Sideroblastic anemia is a rare, heterogeneous group of disorders characterized by hyperferremia, microcytic hypochromic anemia, and bone marrow erythroid hyperplasia with the presence of numerous ringed sideroblasts. We describe herewith the case of a rare coincidence of sideroblastic anemia and mitral valve prolapse with resultant regurgitation in a 2-year-old boy. In addition to the inherent propensity for the development of cardiac dysfunction in sideroblastic anemia due to transfusion-associated myocardial iron overload and chronic anemia, a coincidence of MVP will further increase the likelihood of the morbidity or mortality of th patient. in this patient. After response to pyridoxine, the patient remains in good condition with stable hemoglobin levels.
Key Words: Sideroblastic anemia , Pyridoxine , Mitral valve prolapse


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