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


CONCLUSIONS
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.
Riedl
Stefan
S
Paediatric Department, Medical University Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria. stefan.riedl@meduniwien.ac.at
Blümel
Peter
P
Zwiauer
Karl
K
Frisch
Herwig
H
D002675:Child, Preschool; D015897:Comorbidity; D017809:Fatal Outcome; D005260:Female; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D009765:Obesity / Q000453:epidemiology; D011218:Prader-Willi Syndrome / Q000188:drug therapy* / Q000453:epidemiology
D019382:Human Growth Hormone