Hypoxic and hypercapnic ventilatory responses in awake children with congenital central hypoventilation syndrome

Paton, J.Y. , Swaminathan, S., Sargent, C.W. and Keens, T.G. (1989) Hypoxic and hypercapnic ventilatory responses in awake children with congenital central hypoventilation syndrome. American Review of Respiratory Disease, 140(2), pp. 368-372. (doi:10.1164/ajrccm/140.2.368)

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Publisher's URL: http://dx.doi.org/10.1164/ajrccm/140.2.368


Congenital central hypoventilation syndrome (CCHS) has been thought to be a disorder of central chemoreceptor responsiveness. Previous studies in CCHS have shown decreased or absent ventilatory responsiveness to both hypercarbia and hypoxia. However, hypoxic responsiveness during wakefulness has not been systematically studied. We studied hypoxic and hypercapnic ventilatory responses during wakefulness in five children with CCHS (6 to 11 yr of age). To measure the hypercapnic response, the children rebreathed a hyperoxic hypercapnic mixture until PaCO2 reached 56 to 69 mm Hg. For the hypoxic response, the children rebreathed a hypoxic gas mixture, at mixed venous PCO2, until SaO2 had fallen to less than 78%. We found that the ventilatory responses to hypercapnia and hypoxia were very variable (linear correlation coefficients ranging from -0.44 to +0.63 for hypercapnic responses and from -0.15 to +0.77 for hypoxic responses), with no significant change from baseline in response to either stimulus. There was no evidence of progressive ventilatory stimulation despite increasing stimulus. Additionally, these children had no subjective sensation of dyspnea or discomfort. This establishes that hypoxic and hypercapnic ventilatory control is absent during wakefulness. Chemoreceptor control (peripheral and central) is, therefore, defective in all states in children with CCHS. We speculate that the defect in CCHS lies in central integration of the central and peripheral chemoreceptor signals.

Item Type:Articles
Additional Information:Journal has been renamed American Journal of Respiratory and Critical Care Medicine (ISSN 1073-449X) from 1994.
Glasgow Author(s) Enlighten ID:Paton, Dr James
Authors: Paton, J.Y., Swaminathan, S., Sargent, C.W., and Keens, T.G.
College/School:College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing > Clinical Specialities
Journal Name:American Review of Respiratory Disease
Journal Abbr.:Am Rev Respir Dis.
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