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Respiratory Complications in Children with Prader Willi Syndrome

H-L Tan1, D S Urquhart2

  • 1Department of Paediatric Respiratory and Sleep Medicine, Royal Brompton Hospital, London.

Paediatric Respiratory Reviews
|November 15, 2016
PubMed

Insights

Prader Willi syndrome (PWS) patients face respiratory issues due to genetic factors. This review examines PWS respiratory complications and growth hormone

Area of Science:

  • Genetics and Molecular Biology
  • Pediatrics
  • Pulmonology

Background:

  • Prader Willi syndrome (PWS) is a genetic disorder caused by deletion or lack of expression of paternal chromosome 15 genes.
  • PWS presents with hypotonia, abnormal respiratory/arousal responses to hypoxia/hypercapnia, scoliosis, and obesity, predisposing patients to ventilatory problems.
  • Respiratory complications range from sleep-disordered breathing to aspiration and functional impairment.

Purpose of the Study:

  • To review common respiratory complications in PWS patients.
  • To synthesize literature on the impact of growth hormone (GH) therapy on respiratory parameters in PWS.
  • To guide safe clinical management of PWS patients undergoing GH treatment.

Main Methods:

  • Literature review of published studies.
  • Synthesis of clinical data on PWS respiratory complications.
  • Analysis of growth hormone's effects on respiratory function in PWS.

Main Results:

  • PWS patients exhibit a spectrum of respiratory issues including sleep-disordered breathing and impaired ventilatory responses.
  • Concerns exist regarding sudden death post-growth hormone initiation in PWS patients.
  • Growth hormone's impact on respiratory parameters requires careful consideration.

Conclusions:

  • Comprehensive understanding of PWS respiratory complications is crucial for safe management.
  • Multidisciplinary care and careful monitoring are essential for PWS patients, especially during growth hormone therapy.
  • Further research is needed to clarify the risks and benefits of growth hormone in PWS regarding respiratory health.

Keywords:
Prader Willi syndromeaspirationgrowth hormonesleep disordered breathing

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