Sleep-related breathing disorders in prepubertal children with Prader-Willi syndrome and effects of growth hormone treatment

J Clin Endocrinol Metab. 2006 Dec;91(12):4911-5. doi: 10.1210/jc.2006-0765. Epub 2006 Sep 26.


Context: Recently, several cases of sudden death in GH-treated and non-GH-treated, mainly young Prader-Willi syndrome (PWS), patients were reported. GH treatment in PWS results in a remarkable growth response and an improvement of body composition and muscle strength. Data concerning effects on respiratory parameters, are however, limited.

Objective: The objective of the study was to evaluate effects of GH on respiratory parameters in prepubertal PWS children.

Design: Polysomnography was performed before GH in 53 children and repeated after 6 months of GH treatment in 35 of them.

Patients: Fifty-three prepubertal PWS children (30 boys), with median (interquartile range) age of 5.4 (2.1-7.2) yr and body mass index of +1.0 sd score (-0.1-1.7).

Intervention: Intervention included treatment with GH 1 mg/m2.d.

Results: Apnea hypopnea index (AHI) was 5.1 per hour (2.8-8.7) (normal 0-1 per hour). Of these, 2.8 per hour (1.5-5.4) were central apneas and the rest mainly hypopneas. Duration of apneas was 15.0 sec (13.0-28.0). AHI did not correlate with age and body mass index, but central apneas decreased with age (r = -0.34, P = 0.01). During 6 months of GH treatment, AHI did not significantly change from 4.8 (2.6-7.9) at baseline to 4.0 (2.7-6.2; P = 0.36). One patient died unexpectedly during a mild upper respiratory tract infection, although he had a nearly normal polysomnography.

Conclusions: PWS children have a high AHI, mainly due to central apneas. Six months of GH treatment does not aggravate the sleep-related breathing disorders in young PWS children. Our study also shows that monitoring during upper respiratory tract infection in PWS children should be considered.

Publication types

  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Human Growth Hormone / therapeutic use*
  • Humans
  • Male
  • Polysomnography / methods
  • Prader-Willi Syndrome / complications*
  • Prader-Willi Syndrome / drug therapy*
  • Respiration / drug effects*
  • Sleep Apnea Syndromes / complications*
  • Sleep Apnea Syndromes / drug therapy*


  • Human Growth Hormone