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The Prevalence of Maturity Onset Diabetes of the Young(MODY) 3 in Children with Type 2 Diabetes Mellitus

Korean Journal of Pediatrics 2004;47(6):641-646.
Published online June 15, 2004.
The Prevalence of Maturity Onset Diabetes of the Young(MODY) 3 in Children with Type 2 Diabetes Mellitus
In Kyoung Choi1, Duk Hee Kim1, Ho-Seong Kim1, Nam Huh2, Sang Hyun Paek2, Seoung Young Jung2
1Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
2Samsung Advanced Institute of Technology, Yongin, Korea
소아 제 2형 당뇨병 환아에서 Maturity Onset Diabetes of the Young(MODY) 3의 유병률에 관한 연구
최인경1, 김덕희1, 김호성1, 허남2, 백상현2, 정성영2
1연세대학교 의과대학 소아과학교실
2삼성종합기술원
Correspondence: 
Duk Hee Kim, Email: dhkim3@yumc.yonsei.ac.kr
Abstract
Purpose
: Maturity-onset diabetes of the young(MODY) is a subtype of type 2 diabetes defined by autosomal dominant mode of inheritance, onset of diabetes usually before the age of 25 yrs, and a primary defect in the function of the beta cells of the pancreas. MODY3 is known as the most common form and is caused by mutations in hepatocyte nuclear factor(HNF)-1α. We examined the prevalence of MODY3 in children with type 2 diabetes mellitus(DM).
Methods
: Children with type 2 DM(N=17) and their family members with type 2 DM(N=5) were enrolled. Inclusion criteria for the children were fasting C-peptide and postprandial C-peptide more than 1.0 ng/mL and 1.5 ng/mL respectively, familial type 2 DM in at least two generations, and body mass index(BMI)(kg/m2) less than 95th percentile. Genomic DNA was extracted from blood samples. We analyzed HNF-1 for mutation by DNA microarray method and direct sequencing.
Results
: We found one case with a mutation of the promoter region of HNF-1α(5'-ctaGGCTAGTGGGGTTTTGCGGGGGCAGTGGGTGCAAGG-3') in one child's family member among 22 children and adult subjects with type 2 DM.
Conclusion
: Although we found a mutation of HNF-1α in an adult family member with type 2 DM, we did not find this mutation in a child with type 2 DM. The further investigation of MODY in children, including other types, is required.
Key Words: Maturity-onset diabetes of the young(MODY), Hepatocyte nuclear factor(HNF)-1α, Children, Diabetes mellitus, Type II, Oligonucleotide Array Sequence analysis, Direct sequencing


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