Arousal and cardiorespiratory responses to hypoxia in Prader-Willi syndrome

Raanan Arens, David Gozal, Brian C. Burrell, Sandra L. Bailey, Daisy B. Bautista, Thomas C. Keens, Sally L. Davidson Ward

Research output: Contribution to journalArticlepeer-review

63 Scopus citations


Ventilatory responses to peripheral chemoreceptor stimuli are absent in patients with Prader-Willi syndrome (PWS) during wakefulness. Because arousal from sleep after rapidly developing hypoxia may require intact peripheral chemoreceptor function, we hypothesized that blunted hypoxic arousal responses during sleep Stage 3/4 would be present in PWS. Thirteen patients with PWS (mean age, 23.4 ± 3.7 ± SEM yr; 46% male; body mass index [BMI], 28.9 ± 1.6 kg/m2) and 11 matched control subjects (mean age 28.0 ± 5.4 yr; 54% male; BMI, 28.8 ± 3.1 kg/m2) were studied. An abrupt decrease in inspired O2 tension to 80 mm Hg was introduced until arousal occurred or for a maximum of 3 min. One of the 13 patients with PWS and seven of the 11 control subjects were aroused by the hypoxic challenge (p < 0.02). During hypoxia, heart rate increased by 9 ± 2% in the PWS group versus 22 ± 4% in the control group (p < 0.005). Respiratory rate did not change in the PWS group (4 ± 2%; p = NS), but it increased by 13 ± 2% in the control group (p < 0.02). We conclude that abnormal arousal and cardiorespiratory responses to hypoxia are frequent in PWS. We postulate that intact peripheral chemoreceptor function is an important component underlying arousal mechanisms to rapidly developing hypoxia during sleep.

Original languageEnglish (US)
Pages (from-to)283-287
Number of pages5
JournalAmerican journal of respiratory and critical care medicine
Issue number1
StatePublished - 1996
Externally publishedYes

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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