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Sudden death in growth hormone-treated children with Prader-Willi syndrome

Guy Van Vliet1, Cheri L Deal, Patricia A Crock

  • 1Departments of Pediatrics and Pathology, Sainte-Justine Hospital and Research Center, University of Montreal, Quebec, Canada. gvanvliet@justine.umontreal.ca

The Journal of Pediatrics
|January 15, 2004
PubMed

Insights

Growth hormone treatment may increase risks for children with Prader-Willi syndrome. This includes obstructive sleep apnea, respiratory infections, and sudden death, warranting careful monitoring during therapy.

Area of Science:

  • Pediatric Endocrinology
  • Genetics
  • Respiratory Medicine

Background:

  • Prader-Willi syndrome is a complex genetic disorder affecting multiple body systems.
  • Growth hormone therapy is commonly used to manage growth deficits in Prader-Willi syndrome.
  • Obstructive sleep apnea is a known complication in Prader-Willi syndrome.

Observation:

  • A 4-year-old boy with Prader-Willi syndrome experienced worsening snoring during growth hormone treatment.
  • The child died suddenly and unexpectedly during sleep on day 67 of treatment.
  • Autopsy revealed multifocal bronchopneumonia.

Findings:

  • This case, along with two other recent reports, suggests a potential association between growth hormone therapy and adverse respiratory events.
  • Growth hormone treatment may exacerbate or be linked to obstructive sleep apnea in Prader-Willi syndrome patients.
  • The combination of obstructive apnea and respiratory infection could contribute to sudden death in this context.

Implications:

  • Clinicians should be vigilant for signs of obstructive sleep apnea and respiratory infections in Prader-Willi syndrome patients receiving growth hormone.
  • Further research is needed to elucidate the mechanisms linking growth hormone, sleep-disordered breathing, and mortality risk.
  • Risk-benefit assessments for growth hormone therapy in Prader-Willi syndrome should consider these potential respiratory complications.

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