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Respiratory Depression in Young Prader Willi Syndrome Patients following Clonidine Provocation for Growth Hormone

Gregory A Hollman1, David B Allen, Jens C Eickhoff

  • 1American Family Children's Hospital, Department of Pediatrics, University of Wisconsin, Madison, WI 53792, USA.

International Journal of Pediatric Endocrinology
|April 7, 2010
PubMed

Insights

Clonidine used for growth hormone (GH) testing in children with Prader Willi Syndrome (PWS) caused significant sedation and respiratory depression. Doses often exceeded those for sedation, leading to concerning effects in some patients.

Area of Science:

  • Pediatric Endocrinology
  • Pharmacology
  • Genetics

Background:

  • Prader Willi Syndrome (PWS) is a complex genetic disorder affecting multiple body systems.
  • Growth hormone (GH) deficiency is common in PWS, necessitating accurate assessment.
  • Clonidine is sometimes used to stimulate GH secretion, but its safety profile in PWS requires evaluation.

Purpose of the Study:

  • To assess the sedative and respiratory effects of clonidine when administered for growth hormone (GH) secretion evaluation in children with Prader Willi Syndrome (PWS).

Main Methods:

  • A prospective study involving 17 children with PWS, undergoing up to four clonidine challenge tests over three years.
  • Clonidine was administered at a dose of 0.15 mg/m(2).
  • Vital signs, oxygen saturation, and sedation levels were meticulously recorded at baseline and every five minutes post-administration.

Main Results:

  • All 60 studies resulted in sedation scores of 4-5 (drowsy to asleep).
  • Significant mean declines in respiratory rate (7.5 breaths/min) and oxygen saturation (2.2%) were observed (P < .001).
  • Nine instances of oxygen saturation ≤94% occurred in five patients (29%), with three linked to partial airway obstruction.

Conclusions:

  • Clonidine doses used for GH assessment in PWS frequently surpass typical sedation doses.
  • These higher doses can lead to considerable respiratory depression and oxygen desaturation in some children with PWS.
  • No correlation was found between oxygen desaturation and Body Mass Index (BMI) in this cohort.

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