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Exp Clin Endocrinol Diabetes 2012; 120(10): 579-585
DOI: 10.1055/s-0032-1306330
Article
© J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York

Diabetes Mellitus in Children and Adolescents with Genetic Syndromes

F. Schmidt
1   Department of Paediatrics, University of Halle-Wittenberg, Halle, Germany
,
T. M. Kapellen
2   Department of Paediatrics, University of Leipzig, Leipzig, Germany
,
S. Wiegand
3   Institute of Experimental Paediatric Endocrinology; Charité, Universitätsmedizin Berlin, Germany
,
A. Herbst
4   Department of Paediatrics, Hospital of Leverkusen, Leverkusen, Germany
,
J. Wolf
5   Department of Paediatrics, St. Vincenz-Hospital, Paderborn, Germany
,
E. E. Fröhlich-Reiterer
6   Department of Paediatrics, Medical University of Graz, Graz, Austria
,
W. Rabl
7   Department of Paediatrics, Technical University of Munich, Munich, Germany
,
T. Rohrer   *
8   Department of Paediatrics and Neonatology, Saarland University Hospital, Homburg/Saar, Germany
,
R. W. Holl   *
9   Institute of Epidemiology and Medical Biometry, University of Ulm, Ulm, Germany
,
for the DPV-Wiss Study Group and the BMBF Competence Network Diabetes. › Author Affiliations
Further Information

Publication History

received 24 January 2012
first decision 24 January 2012

accepted 22 February 2012

Publication Date:
22 March 2012 (online)

Also available at
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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, HbA1c, 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.

Conclusion:

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.

Key words

Turner syndrome - Prader-Willi syndrome - Friedreich ataxia - Alström syndrome - Klinefelter syndrome - Pediatric diabetology
*

*  These authors contributed equally as senior authors.


 

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