Elsevier

The Journal of Pediatrics

Volume 162, Issue 2, February 2013, Pages 263-268.e1
The Journal of Pediatrics

Original Article
Longitudinal Evaluation of Sleep-Disordered Breathing in Children with Prader-Willi Syndrome during 2 Years of Growth Hormone Therapy

https://doi.org/10.1016/j.jpeds.2012.07.042Get rights and content

Objective

To review longitudinal polysomnography data to assess sleep-related disordered breathing (SRDB) before and up to 2 years after initiation of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS).

Study design

This was a retrospective review of systematic polysomnography evaluations performed in children with PWS before and at 6 weeks, 6 months, 1 year, and 2 years after initiation of GH therapy.

Results

A total of 15 children with PWS were reviewed. At baseline, the median age was 3.7 years (range, 0.8-15.4 years), and the median body mass index percentile was 82.4 (range, 0-100). GH was discontinued in 2 of these 15 children owing to the occurrence of severe obstructive sleep apnea after 6 weeks of GH therapy. The remaining 13 children who were followed for up to 2 years on GH therapy demonstrated no statistically significant trends over time for any adverse sleep-related outcomes, specifically obstructive or central sleep apnea.

Conclusion

In young children with PWS with known SRDB at baseline, the first few weeks after initiation of GH therapy may represent a vulnerable time for the development of significant SRDB. However, most children with PWS did not show significant changes in SRDB after 2 years of GH therapy. We conclude that long-term GH therapy appears to be safe after an initial period of increased risk in the context of SRDB in children with PWS.

Section snippets

Methods

We reviewed data for 2005-2012 from the PWS program at The Hospital for Sick Children, Toronto. We identified patients with PWS who underwent PSG studies before and after initiation of GH therapy. The primary indication for the PSG referral was to assess SRDB before the start of GH therapy. The current protocol for performing PSG in children with PWS in our institution is: (1) before GH therapy initiation (baseline); (2) 6 weeks after GH initiation; (3) 6 months after GH initiation; (4) 1 year

Results

Between 2005 and 2011, 21 children with genetically confirmed PWS underwent baseline screening PSG to evaluate their suitability for GH therapy. After baseline testing, 6 of these 21 children were excluded from this review for various reasons, including loss to follow-up after the baseline sleep study (n = 2), unexplained delay in GH treatment initiation with no current follow-up PSG data (n = 2), no follow-up PSG performed after GH initiation (n = 1), and oxygen therapy for the treatment of

Discussion

This study was a longitudinal assessment of sleep disorders in patients with PWS receiving GH therapy with multiple PSGs extending up to 2 years after initiation of GH therapy. In this study, 2 of 15 patients discontinued GH therapy after their first PSG at 6 weeks postinitiation secondary to a diagnosis of severe OSA; however, 13 patients were treated with GH therapy with no clinically significant changes in OAHI and CAI over the 2-year period. Furthermore, GH therapy did not negatively affect

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    The authors declare no conflicts of interest.

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