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Ann Pediatr Endocrinol Metab > Volume 15(1); 2010 > Article
Review
Journal of Korean Society of Pediatric Endocrinology 2010;15(1): 1-6.
MODY Syndrome.
Jin Soon Hwang
Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Suwon, Korea. pedhwang@ajou.ac.kr
Abstract
Maturity-onset diabetes of the young (MODY) is a heterogenous form of diabetes characterized by the early onset of diabetes, autosomal dominant inheritance, and impaired insulin secretion. MODY is mostly caused by mutations of the hepatocyte nuclear factor 1-alpha (HNF1-alpha) and glucokinase genes in Caucasians. However most Korean, Japanese, and Chinese patients with MODY do not express known MODY genes. The cause of MODY in Asians has not yet been elucidated clearly. This review focuses on studies on Asian patients with MODY.
Keywords: Maturity onset diabetes of the young (MODY)
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