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Review of 64 cases of death in children with Prader-Willi syndrome (PWS).

Tauber M, Diene G, Molinas C, Hébert M

Division of Endocrinology, Genetics, Gynecology and Bone Diseases, Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse Cedex 09, France. tauber.mt@chu-toulouse.fr

Several deaths have been reported in children with Prader-Willi syndrome (PWS) following treatment with growth hormone (GH). We collected all of the reports of deaths in PWS children, both in treatment and non-treatment groups, analyzed the causes of the death and compared the two groups. We conducted an exhaustive search for reports using bibliographic databases, toxicology pharmacovigilance databases, and personal communications. Sixty-four PWS children (42M/22F) aged from a few days to 19 years were identified, 28 received GH treatment. Our results show that respiratory disorders were the most common cause of death (respiratory insufficiency or infections) which were reported in 61% of the children (68% in GH-treated and 55.5% in -untreated patients). We found no significant differences in gender, prevalence of obesity or prevalence of sleep apnea, between the patients treated with GH and the untreated patients. Nevertheless, most of the deaths in GH-treated children (75%) occurred during the first 9 months after the initiation of GH treatment. Our analysis shows the high frequency of respiratory infections in both GH-treated and -untreated PWS children. The first 9 months of GH treatment seems to be a high-risk period emphasizing the need for comprehensive care before and during GH treatment.

Published 2 April 2008 in Am J Med Genet A, 146(7): 881-7.
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