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 language | English (US) |
|---|---|
| Pages (from-to) | 2224-2230 |
| Number of pages | 7 |
| Journal | Journal of Applied Physiology |
| Volume | 77 |
| Issue number | 5 |
| State | Published - 1994 |
| Externally published | Yes |
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Keywords
- hypercapnia
- hypoxia
- obesity
- ventilatory control
ASJC Scopus subject areas
- Endocrinology
- Physiology
- Orthopedics and Sports Medicine
- Physical Therapy, Sports Therapy and Rehabilitation
Cite this
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 journal › Article
}
TY - JOUR
T1 - Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome
AU - Arens, Raanan
AU - Gozal, D.
AU - Omlin, K. J.
AU - Livingston, F. R.
AU - Liu, J.
AU - Keens, T. G.
AU - Ward, S. L D
PY - 1994
Y1 - 1994
N2 - 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.
AB - 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.
KW - hypercapnia
KW - hypoxia
KW - obesity
KW - ventilatory control
UR - http://www.scopus.com/inward/record.url?scp=0028113590&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0028113590&partnerID=8YFLogxK
M3 - Article
C2 - 7868438
AN - SCOPUS:0028113590
VL - 77
SP - 2224
EP - 2230
JO - Journal of Applied Physiology
JF - Journal of Applied Physiology
SN - 8750-7587
IS - 5
ER -