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Growth hormone therapy and respiratory disorders: long-term follow-up in PWS children

Jenny Berini1, Valeria Spica Russotto, Paolo Castelnuovo

  • 1Pediatric Unit, Ospedale Del Ponte, Insubria University, 21100 Varese, Italy.

The Journal of Clinical Endocrinology and Metabolism
|July 30, 2013
PubMed

Insights

Long-term growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS) is safe, but requires annual monitoring for sleep-disordered breathing and adenotonsillar hypertrophy. This study highlights the importance of regular check-ups during treatment.

Area of Science:

  • Pediatric Endocrinology
  • Sleep Medicine
  • Genetics

Background:

  • Short-term growth hormone (GH) treatment in children with Prader-Willi syndrome (PWS) has been linked to adenotonsillar hypertrophy and obstructive sleep apnea.
  • The long-term effects of GH therapy on these conditions in PWS patients remain incompletely understood.

Purpose of the Study:

  • To evaluate the impact of long-term GH therapy on sleep-disordered breathing and adenotonsillar hypertrophy in children with PWS.
  • To assess the safety and efficacy of extended GH treatment in this specific pediatric population.

Main Methods:

  • A longitudinal observational study involving 50 children with genetically confirmed PWS undergoing GH therapy for up to 4 years.
  • Polysomnography was used to assess central apnea index, obstructive apnea hypopnea index (OAHI), respiratory disturbance index, and oxygen saturation.
  • Adenotonsillar size was evaluated using flexible fiberoptic endoscopy at multiple time points throughout the study.

Main Results:

  • The incidence of obstructive sleep apnea (OAHI >1) increased significantly over the 4-year treatment period.
  • Despite an increase in severe adenotonsillar hypertrophy, the respiratory disturbance index and central apnea index showed significant decreases.
  • Treatment suspension was necessary for a small subset of patients due to severe obstructive sleep apnea.

Conclusions:

  • Long-term GH treatment appears to be safe for children with PWS.
  • Annual polysomnography and adenotonsillar evaluations are recommended to monitor for potential complications.
  • Close monitoring is crucial for managing sleep-disordered breathing and airway changes during extended GH therapy.
Abstract

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