title: Diabetes mellitus in children and adolescents with genetic syndromes.
abstract: BACKGROUND
  Several genetic syndromes are associated with diabetes mellitus (DM). This study aimed to analyse data from the DPV database with regard to frequency, treatment strategies and long-term complications in paediatric DM patients with genetic syndromes, including Turner syndrome (TS), Prader-Willi syndrome (PWS), Friedreich ataxia (FA), Alström syndrome (AS), Klinefelter syndrome (KS), Bardet-Biedl syndrome (BBS), Berardinelli-Seip syndrome (BSS) and Down syndrome (DS).
  
  
  METHODS
  Longitudinal data for 43 521 patients with DM onset at age < 20 years were collected from 309 treatment centres in Germany and Austria using the DPV software. Data included anthropometric parameters, type of diabetes, mean age, age at diabetes onset, daily insulin dose, HbA 1c , micro- and macroalbuminuria, retinopathy and dyslipidaemia. Descriptive statistics and standard statistical tests were used for data analysis.
  
  
  RESULTS
  In total, 205 DM patients had one of the following syndromes: DS (141 patients), TS (24), PWS (23), FA (5), AS (5), KS (4), BBS (2) and BSS (1). Diabetes-specific antibodies were positive in the majority of patients with DS, TS and FA.
  
  
  CONCLUSIONS
  Despite the well-known association between DM and certain syndromic disorders, the number of affected patients in the German and Austrian paediatric diabetic population is very low. Nevertheless, physicians should be aware of syndromic forms of diabetes. Joint multicentre analyses are needed to draw relevant conclusions.
authors.affiliation: Department of Paediatrics, University of Halle-Wittenberg, Halle, Germany. frank.schmidt@uk-halle.de
authors.forename: F
authors.initials: F
authors.lastname: Schmidt
authors.forename: T M
authors.initials: TM
authors.lastname: Kapellen
authors.forename: S
authors.initials: S
authors.lastname: Wiegand
authors.forename: A
authors.initials: A
authors.lastname: Herbst
authors.forename: J
authors.initials: J
authors.lastname: Wolf
authors.forename: E E
authors.initials: EE
authors.lastname: Fröhlich-Reiterer
authors.forename: W
authors.initials: W
authors.lastname: Rabl
authors.forename: T R
authors.initials: TR
authors.lastname: Rohrer
authors.forename: R W
authors.initials: RW
authors.lastname: Holl
mesh_terms: D000293:Adolescent; D001317:Austria / Q000453:epidemiology; D001323:Autoantibodies / Q000032:analysis; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000453:epidemiology / Q000209:etiology* / Q000276:immunology; D003924:Diabetes Mellitus, Type 2 / Q000150:complications / Q000453:epidemiology / Q000209:etiology* / Q000276:immunology; D003928:Diabetic Nephropathies / Q000453:epidemiology / Q000209:etiology; D003930:Diabetic Retinopathy / Q000453:epidemiology / Q000209:etiology; D004314:Down Syndrome / Q000097:blood / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology; D050171:Dyslipidemias / Q000453:epidemiology / Q000209:etiology; D005260:Female; D030342:Genetic Diseases, Inborn / Q000097:blood / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology*; D005858:Germany / Q000453:epidemiology; D006442:Glycated Hemoglobin / Q000032:analysis; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D011218:Prader-Willi Syndrome / Q000097:blood / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology; D015995:Prevalence; D011446:Prospective Studies; D014424:Turner Syndrome / Q000097:blood / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology
chemical_list: D001323:Autoantibodies; D006442:Glycated Hemoglobin; C517652:hemoglobin A1c protein, human