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

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Tauber, M. Diene, G. Molinas, C. Hébert, M.
Spatial Coverage	France
Description	Country affiliation: France Author Affiliation: Tauber 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)
Abstract	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.
File Format	HTM / HTML
ISSN	15524825
Issue Number	7
Volume Number	146A
e-ISSN	15524833
Journal	American Journal of Medical Genetics Part A
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2008-04-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Human Discipline Genetics Prader-willi Syndrome Mortality Cause Of Death Child Female France Epidemiology Humans Male Journal Article Review
Content Type	Text
Resource Type	Article
Subject	Genetics Genetics (clinical)

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1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
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4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
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