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Hypoventilation in patients with Prader-Willi syndrome across the pediatric age

Catherine Chen1,2, Iulia Ioan3, Marine Thieux2,4,5

  • 1Service de pneumologie pédiatrique, Hôpital Femme Mère Enfant, Hospices, Civils de Lyon, Bron, France.

Pediatric Pulmonology
|January 5, 2024
PubMed

Insights

Alveolar hypoventilation affects 35% of children with Prader-Willi syndrome (PWS), often without symptoms. Regular screening is recommended, especially with age and growth hormone (GH) treatment, to monitor respiratory health in PWS patients.

Area of Science:

  • Pediatric Pulmonology
  • Genetics
  • Sleep Medicine

Background:

  • Prader-Willi syndrome (PWS) is a complex genetic disorder with limited data on respiratory complications.
  • Standardized respiratory follow-up for PWS patients is lacking, particularly concerning alveolar hypoventilation.

Purpose of the Study:

  • To determine the prevalence of alveolar hypoventilation in children with PWS.
  • To identify potential risk factors associated with alveolar hypoventilation in this population.

Main Methods:

  • Retrospective polysomnography (PSG) study of children with PWS (2007-2021).
  • Inclusion criteria: PSG with transcutaneous CO2 (PtcCO2) or end-tidal CO2 (ETCO2) measurements.
  • Alveolar hypoventilation defined as pCO2 ≥ 50 mmHg for ≥2% of total sleep time or >5 consecutive minutes.

Main Results:

  • 35% (20/57) of children with PWS exhibited alveolar hypoventilation.
  • Median pCO2 max was 49 mmHg; 25% of hypoventilating children were asymptomatic.
  • Higher median age and growth hormone (GH) treatment were associated with hypoventilation.

Conclusions:

  • Alveolar hypoventilation is a significant concern in pediatric PWS, potentially increasing with age and GH therapy.
  • Regular screening using oximetry-capnography is advised for all PWS patients, irrespective of sex, BMI, or apnea index.
Abstract

Keywords:
Prader‐Willy syndromealveolar hypoventilationchild, polysomnographygrowth hormonesleep‐disordered breathing

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