Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients - Sorbonne Université
Journal Articles Journal of Clinical Medicine Year : 2021

Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients

Muriel Coupaye
  • Function : Author
  • PersonId : 1102826
Héléna Mosbah
  • Function : Author
  • PersonId : 1102827
Virginie Laurier
  • Function : Author
  • PersonId : 1118855
Fabien Mourre
  • Function : Author
  • PersonId : 1118856
Christine Merrien
  • Function : Author
  • PersonId : 1118857
Christine Poitou
  • Function : Author
  • PersonId : 1102828

Abstract

Type 2 diabetes mellitus (T2DM) affects 20% of patients with Prader-Willi syndrome (PWS), with many cases diagnosed during the transition period. Our aim was to describe the natural history of T2DM in patients with PWS before the age of 25 years and to develop screening and preventive strategies. Thirty-nine patients followed in the French PWS Reference Center were included (median age 25.6 years [23.7; 31.7]). Twenty-one had been treated with growth hormone (GH), fifteen had not, and three had an unknown status. The median age at T2DM diagnosis was 16.8 years (11–24) and the median BMI was 39 kg/m2 [34.6; 45], with 34/35 patients living with obesity. The patients displayed frequent psychiatric (48.3% hospitalization,) and metabolic (56.4% hypertriglyceridemia,) comorbidities and a parental history of T2DM (35.7%) or overweight (53.6%) compared to the PWS general population. There was no difference in BMI and metabolic complications between the GH-treated and non-GH-treated groups at T2DM diagnosis. Patients with PWS who develop early T2DM have severe obesity, a high frequency of psychiatric and metabolic disorders, and a family history of T2DM and overweight. These results underline the need for early identification of patients at risk, prevention of obesity, and repeated blood glucose monitoring during the transition period.
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Dates and versions

hal-03457276 , version 1 (30-11-2021)

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Alice Clerc, Muriel Coupaye, Héléna Mosbah, Graziella Pinto, Virginie Laurier, et al.. Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients. Journal of Clinical Medicine, 2021, 10 (22), pp.5310. ⟨10.3390/jcm10225310⟩. ⟨hal-03457276⟩
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