title: Maturity-Onset Diabetes of the Young: What Do Clinicians Need to Know?
abstract: Maturity-onset diabetes of the young (MODY) is a monogenic form of diabetes that is characterized by an early onset, autosomal dominant mode of inheritance and a primary defect in pancreatic β-cell function. MODY represents less than 2% of all diabetes cases and is commonly misdiagnosed as type 1 or type 2 diabetes mellitus. At least 13 MODY subtypes with distinct genetic etiologies have been identified to date. A correct genetic diagnosis is important as it often leads to personalized treatment for those with diabetes and enables predictive genetic testing for their asymptomatic relatives. Next-generation sequencing may provide an efficient method for screening mutations in this form of diabetes as well as identifying new MODY genes. In this review, I discuss a current update on MODY in the literatures and cover the studies that have been performed in Korea.
authors.affiliation: Division of Endocrinology & Metabolism, Department of Medicine, Cheil General Hospital & Women's Healthcare Center, Dankook University College of Medicine, Seoul, Korea. hoonie.kim@cgh.co.kr.
authors.forename: Sung Hoon
authors.initials: SH
authors.lastname: Kim
keywords: Diagnosis; Maturity-onset diabetes of the young; Personalized treatment