Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome

Raanan Arens, D. Gozal, K. J. Omlin, F. R. Livingston, J. Liu, T. G. Keens, S. L D Ward

Research output: Contribution to journalArticle

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Abstract

Abnormalities of ventilatory control may play a significant role in the pathophysiology of sleep-disordered breathing in patients with the Prader- Willi syndrome (PWS). We measured rebreathing hypercapnic and hypoxic ventilatory responses (HCVR and HPVR, respectively) during wakefulness in 8 nonobese PWS (NOB-PWS) and 9 obese PWS (OB-PWS) patients and compared their results with those from 24 healthy nonobese control (NOB-CON) and 10 obese control (OB-CON) subjects. The slope of HCVR was similar in NOB-PWS patients and NOB-CON subjects (NS). However, HCVR was significantly lower in OB-PWS patients than in OB-CON subjects (P < 0.02). In PWS patients, the mean point of origin of the positive slope of HCVR occurred at a significantly higher end-tidal PCO2 than in either control group. During isocapnic hypoxic challenges, six PWS patients had no significant HPVR. In the remainder, mean slopes of HPVR were -0.80 ± 0.06 l · min-1 · %arterial O2 saturation- 1 in five NOB-PWS patients and -0.68 ± 0.15 l · min-1 · %arterial O2 saturation-1 in six OB-PWS patients. These responses were significantly decreased compared with those in the control groups (P < 0.006). We conclude that NOB-PWS patients have normal HCVR, which is blunted in OB-PWS patients. Furthermore, isocapnic HPVR is either absent or markedly reduced in PWS patients. The severity of abnormality of the HPVR is independent of the degree of obesity. We postulate that the primary abnormality of ventilatory control in PWS affects peripheral chemoreceptor pathways.

Original languageEnglish (US)
Pages (from-to)2224-2230
Number of pages7
JournalJournal of Applied Physiology
Volume77
Issue number5
StatePublished - 1994
Externally publishedYes

Fingerprint

Prader-Willi Syndrome
Control Groups
Wakefulness
Sleep Apnea Syndromes
Obesity

Keywords

  • hypercapnia
  • hypoxia
  • obesity
  • ventilatory control

ASJC Scopus subject areas

  • Endocrinology
  • Physiology
  • Orthopedics and Sports Medicine
  • Physical Therapy, Sports Therapy and Rehabilitation

Cite this

Arens, R., Gozal, D., Omlin, K. J., Livingston, F. R., Liu, J., Keens, T. G., & Ward, S. L. D. (1994). Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome. Journal of Applied Physiology, 77(5), 2224-2230.

Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome. / Arens, Raanan; Gozal, D.; Omlin, K. J.; Livingston, F. R.; Liu, J.; Keens, T. G.; Ward, S. L D.

In: Journal of Applied Physiology, Vol. 77, No. 5, 1994, p. 2224-2230.

Research output: Contribution to journalArticle

Arens, R, Gozal, D, Omlin, KJ, Livingston, FR, Liu, J, Keens, TG & Ward, SLD 1994, 'Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome', Journal of Applied Physiology, vol. 77, no. 5, pp. 2224-2230.
Arens R, Gozal D, Omlin KJ, Livingston FR, Liu J, Keens TG et al. Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome. Journal of Applied Physiology. 1994;77(5):2224-2230.
Arens, Raanan ; Gozal, D. ; Omlin, K. J. ; Livingston, F. R. ; Liu, J. ; Keens, T. G. ; Ward, S. L D. / Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome. In: Journal of Applied Physiology. 1994 ; Vol. 77, No. 5. pp. 2224-2230.
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