Phenotypical aspects of maturity-onset diabetes of the young (MODY diabetes) in comparison with Type 2 diabetes mellitus (T2DM) in children and adolescents: experience from a large multicentre database

Aims To analyse and compare clinical characteristics in young patients with maturity‐onset diabetes of the young (MODY) and Type 2 diabetes mellitus (T2DM). Methods We conducted an observational investigation using the DPV‐Wiss database containing clinical data on 40 757 diabetic patients < 20 ...

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Published in:	Diabetic medicine Vol. 26; no. 5; pp. 466 - 473
Main Authors:	Schober, E., Rami, B., Grabert, M., Thon, A., Kapellen, Th, Reinehr, Th, Holl, R. W.
Format:	Journal Article
Language:	English
Published:	Oxford, UK Blackwell Publishing Ltd 01-05-2009 Blackwell
Subjects:	Adolescent adolescents Age of Onset Austria - epidemiology Biological and medical sciences Body Mass Index Child children clinical aspects Diabetes Mellitus, Type 2 - classification Diabetes Mellitus, Type 2 - epidemiology Diabetes Mellitus, Type 2 - genetics Diabetes Mellitus, Type 2 - physiopathology Diabetes. Impaired glucose tolerance Dyslipidemias - epidemiology Endocrine pancreas. Apud cells (diseases) Endocrinopathies Etiopathogenesis. Screening. Investigations. Target tissue resistance Feeding. Feeding behavior Female Fundamental and applied biological sciences. Psychology Germany - epidemiology Glucokinase - genetics Hepatocyte Nuclear Factor 1-alpha - genetics Hepatocyte Nuclear Factor 4 - genetics Humans Hypertension - epidemiology Male Medical sciences MODY Phenotype Polymorphism, Genetic Type 2 diabetes mellitus Vertebrates: anatomy and physiology, studies on body, several organs or systems Vertebrates: endocrinology Young Adult Austria Germany Endocrinopathy Human Type 2 diabetes Maturity onset diabetes young Obesity Nutrition adolescents Multicenter study Nutrition disorder Clinical form Metabolic diseases Type 2 diabetes mellitus Genetic disease Symptomatology Phenotype MODY children Adolescent Database clinical aspects Child Endocrinology Nutritional status Comparative study
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Summary:	Aims To analyse and compare clinical characteristics in young patients with maturity‐onset diabetes of the young (MODY) and Type 2 diabetes mellitus (T2DM). Methods We conducted an observational investigation using the DPV‐Wiss database containing clinical data on 40 757 diabetic patients < 20 years of age from Germany and Austria. Results Three hundred and thirty‐nine cases were clinically categorized as MODY (0.83%); 562 patients were diagnosed as T2DM (1.4%). In 20% of cases, the diagnosis of MODY was based on clinical findings only. Of the 272 subjects where genetic testing was available, 3% did not carry mutations in the three examined MODY genes. Glucokinase‐MODY was commoner than HNF1A‐MODY and HNF4A‐MODY. Age at diagnosis was younger in MODY patients. The body mass index of T2DM was significantly higher compared with all MODY subgroups. Macrovascular risk factors such as dyslipidaemia and hypertension were commoner in T2DM, but 23% of MODY patients had dyslipidaemia and 10% hypertension. Glycaemic control was within the therapeutic target (HbA1c < 7.5%) in 86% of MODY and 70% of T2DM patients. Conclusions The prevalence of MODY in children and adolescents in Germany and Austria is lower than that of T2DM in this age group. Dyslipidaemia and hypertension are less frequent in MODY compared with T2DM patients, but do occur.
Bibliography:	istex:2922BC68A665F07FE98008AB75B9D2A1B9EA8878 ark:/67375/WNG-DH4LSJ4G-4 ArticleID:DME2720 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0742-3071 1464-5491
DOI:	10.1111/j.1464-5491.2009.02720.x