Death in two female Prader‐Willi syndrome patients during the early phase of growth hormone treatment

Abstract

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 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.