title: 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.
abstract: OBJECTIVE
  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 (HbA(1c) < 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.
authors: Paediatric Department, Medical University of Vienna, Vienna, Austria. edith.schober@meduniwien.ac.at
authors: E
authors: E
authors: Schober
authors: B
authors: B
authors: Rami
authors: M
authors: M
authors: Grabert
authors: A
authors: A
authors: Thon
authors: Th
authors: T
authors: Kapellen
authors: Th
authors: T
authors: Reinehr
authors: R W
authors: RW
authors: Holl
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001317:Austria / Q000453:epidemiology; D015992:Body Mass Index; D002648:Child; D003924:Diabetes Mellitus, Type 2 / Q000145:classification / Q000453:epidemiology* / Q000235:genetics* / Q000503:physiopathology; D050171:Dyslipidemias / Q000453:epidemiology; D005260:Female; D005858:Germany / Q000453:epidemiology; D005941:Glucokinase / Q000235:genetics*; D051538:Hepatocyte Nuclear Factor 1-alpha / Q000235:genetics*; D051557:Hepatocyte Nuclear Factor 4 / Q000235:genetics*; D006801:Humans; D006973:Hypertension / Q000453:epidemiology; D008297:Male; D010641:Phenotype; D011110:Polymorphism, Genetic; D055815:Young Adult
chemical_list: D051538:Hepatocyte Nuclear Factor 1-alpha; D051557:Hepatocyte Nuclear Factor 4; D005941:Glucokinase