Acta Paediatr. 2005 July.
Death in two female Prader-Willi syndrome patients during the early phase of growth hormone treatment.
Riedl S, Blumel P, Zwiauer K, Frisch H.
Paediatric Department, Medical University Vienna, Wahringer Gurtel 18-20, 1090 Vienna, Austria.
[ PubMed ]

Abstract: Reports on sudden death in Prader-Willi syndrome (PWS) patients after the start of growth hormone (GH) treatment have been published recently. We observed a 4.7-y-old girl who showed a continuous increase in pulmonary artery pressure and died of cardiorespiratory failure 7 wk after GH therapy had been initiated, and a 9.3-y-old girl with additional trisomy 21 [Down's] who died during a minor respiratory infection 6 mo after GH had been started. Both patients were overweight (weight for height 127% and 224%, respectively). GH-induced fluid retention may have occurred in the younger girl. In contrast to the reported cases, our PWS patients were female. Conclusion: Our cases illustrate the difficulty of differentiation between possible GH side effects and the natural course of disease, in particular with respect to obesity-related comorbidity and mortality.