Original ArticleLongitudinal Evaluation of Sleep-Disordered Breathing in Children with Prader-Willi Syndrome during 2 Years of Growth Hormone Therapy
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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2023, Encyclopedia of Child and Adolescent Health, First EditionSleep disorders in Prader-Willi syndrome, evidence from animal models and humans
2021, Sleep Medicine ReviewsCitation Excerpt :However, retrospective reviews of sudden death incidents in individuals with PWS on and off GH showed no difference in the rates of death [54]. Moreover, the majority of studies did not confirm the worsening effect of GH on SDB in both adult and pediatric patients with PWS [68,96–98], with some studies showing an improvement of Respiratory disturbance index and Central apnea index (CAI) [98]. However, CAI improvement seems to be independent of GH treatment [10].
Sleep disordered breathing in Silver−Russell syndrome patients: a new outcome
2019, Sleep MedicineCitation Excerpt :Furthermore, 73% of PWS patients have impaired sleep, even without clinical signs of sleep apnea syndrome (SAS) [6]. Some studies have evaluated the impact of recombinant growth hormone (rGH) on sleep features and showed possible worsening of sleep PSG parameters 6–9 months after treatment initiation, but no long-term effect [4,7,11–13]. This could be explained by tonsil hypertrophy subsequent to the introduction of rGH, which rapidly stabilizes.
Obstructive events in children with Prader–Willi syndrome occur predominantly during rapid eye movement sleep
2019, Sleep MedicineCitation Excerpt :Growth hormone treatment has been offered to patients with PWS in an effort to improve linear growth and to delay or inhibit the development of obesity [10]. Nevertheless, severe OSAS may occur in some patients while on treatment and especially during episodes of respiratory infections, and cases of sudden death have been reported [11–13]. Growth hormone does not improve the ventilatory response to hypercapnia during sleep, and there are also concerns that it may actually promote growth of adenotonsillar tissue and upper airway obstruction [32].
The authors declare no conflicts of interest.