G Diene1, E Mimoun, E Feigerlova, S Caula, C Molinas, H Grandjean, M Tauber. 1. Centre de Référence du Syndrome de Prader-Willi, Division of Endocrinology, Genetics, Gynaecology and Bone Diseases, Hôpital des Enfants, and INSERM U558, University Paul Sabatier, Toulouse, France.
Abstract
AIM: The first results from the French National Prader-Willi pediatric database in a cohort of 142 children aged 0.2-18.8 years are reported. This database gathers information about the endocrine dysfunctions traditionally described in Prader-Willi patients. METHODS: Questionnaires were filled in by the patients' practitioners. The coordination team of the reference center performed the statistical analysis. RESULTS: Median BMI Z-score was +1.3 for a median age of 7.1 years, and 40% of the population were overweight or obese (International Obesity Task Force 2000 criteria). Growth hormone deficiency was present in 80% of patients and 86.7% were treated, with a height gain of +1 SD and a BMI reduction of -0.8 Z-score achieved in the first year of treatment. Hypogonadism was present in 49% of patients, and hypothyroidism in 24.4%. Glucose intolerance was found in 4% of patients, but no diabetes mellitus was detected in the 74 patients explored. CONCLUSION: Our report gives an overview of endocrine dysfunctions recorded in a large registry database of French children and adolescents with Prader-Willi syndrome. The database, which now encompasses six southern regions of France, will be further extended to the whole country and to adult patients. Copyright (c) 2010 S. Karger AG, Basel.
AIM: The first results from the French National Prader-Willi pediatric database in a cohort of 142 children aged 0.2-18.8 years are reported. This database gathers information about the endocrine dysfunctions traditionally described in Prader-Willipatients. METHODS: Questionnaires were filled in by the patients' practitioners. The coordination team of the reference center performed the statistical analysis. RESULTS: Median BMI Z-score was +1.3 for a median age of 7.1 years, and 40% of the population were overweight or obese (International Obesity Task Force 2000 criteria). Growth hormone deficiency was present in 80% of patients and 86.7% were treated, with a height gain of +1 SD and a BMI reduction of -0.8 Z-score achieved in the first year of treatment. Hypogonadism was present in 49% of patients, and hypothyroidism in 24.4%. Glucose intolerance was found in 4% of patients, but no diabetes mellitus was detected in the 74 patients explored. CONCLUSION: Our report gives an overview of endocrine dysfunctions recorded in a large registry database of French children and adolescents with Prader-Willi syndrome. The database, which now encompasses six southern regions of France, will be further extended to the whole country and to adult patients. Copyright (c) 2010 S. Karger AG, Basel.
Authors: T Cadoudal; M Buléon; C Sengenès; G Diene; F Desneulin; C Molinas; S Eddiry; F Conte-Auriol; D Daviaud; P G P Martin; A Bouloumié; J-P Salles; M Tauber; P Valet Journal: Int J Obes (Lond) Date: 2014-01-10 Impact factor: 5.095
Authors: Emma K Baker; Sheena Arora; David J Amor; Perrin Date; Meagan Cross; James O'Brien; Chloe Simons; Carolyn Rogers; Stephen Goodall; Jennie Slee; Chris Cahir; David E Godler Journal: J Autism Dev Disord Date: 2021-07-22