Endocrine disorders in children with Prader-Willi syndrome--data from 142 children of the French database.
OBJECTIVE
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.


CONCLUSIONS
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.
Diene
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G
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.
Mimoun
E
E
Feigerlova
E
E
Caula
S
S
Molinas
C
C
Grandjean
H
H
Tauber
M
M
D000293:Adolescent; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D003924:Diabetes Mellitus, Type 2 / Q000150:complications / Q000453:epidemiology; D004393:Dwarfism, Pituitary / Q000150:complications / Q000453:epidemiology; D004700:Endocrine System Diseases / Q000150:complications / Q000453:epidemiology*; D005602:France / Q000453:epidemiology; D018149:Glucose Intolerance / Q000150:complications / Q000453:epidemiology; D006801:Humans; D007006:Hypogonadism / Q000150:complications / Q000453:epidemiology; D007037:Hypothyroidism / Q000150:complications / Q000453:epidemiology; D007223:Infant; D009765:Obesity / Q000150:complications / Q000453:epidemiology; D011218:Prader-Willi Syndrome / Q000150:complications / Q000453:epidemiology*; D015995:Prevalence; D011795:Surveys and Questionnaires; D055815:Young Adult