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Prevalence of diabetes in Turner syndrome in the French population and clinical characteristics of the association of these two pathologies : P2-d2-598 Turner Syndrome
Title of the conference
47th Annual Meeting of the European Society for Paediatric Endocrinology (ESPE)
Istanbul, Turkey, September 20-23, 2008
Turner syndrome (TS) is due to the complete or partial absence of one of the X chromosomes and results in several developmental abnormalities including short stature and ovarian failure. An increased risk of diabetes has been shown in a Danish population-based study and in a series of 2912 patients treated with GH and registered in the KIGS database. As part of a prospective study on TS, we examined the prevalence and characteristics of diabetes mellitus in France. A questionnaire was sent to all young adults with TS in France who were registered in the population-based registry of GH-treated patients. Detailed phenotypic information on the cases of diabetes was obtained from patient's charts. Diabetes was classified as type 1 (T1D) or type 2 diabetes (T2D) based on autoantibodies and insulin dependence. Of the 568 patients (69% of all) who responded to the questionnaire, 7 reported to have diabetes, indicating a prevalence of 1.2%. Three patients had T1D and 4 had T2D with a prevalence of 0.5% and 0.7% respectively. All 3 patients with T1D had a 46X,i(Xq) karyotype. One case of ketoacidosis was reported. T1D was diagnosed at a mean age of 15.7 years and T2D at 18 years. Mean BMI at the time of diagnosis was similar in patients with T2D (21.6 kg/m2) and in those with T1D (26.5 kg/m2). T1D was not associated with autoimmune thyroid disease defined by the presence of TPO antibodies. Two patients with T1D and none of those with T2D were treated with GH at the time of diagnosis of diabetes. In conclusion, the prevalence of diabetes appears higher in patients with TS than in the general population in France. The association of 46,X,i(Xq) karyotype with T1D, as well as the known ssociation of this chromosomal complement with autoimmune thyroid disease suggest a specific role in autoimmunity.
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