4.5 Article

Central CO2 chemosensitivity and CO2 controller gain independently contribute to daytime Pco(2) in young subjects with congenital central hypoventilation syndrome

期刊

JOURNAL OF APPLIED PHYSIOLOGY
卷 135, 期 2, 页码 343-351

出版社

AMER PHYSIOLOGICAL SOC
DOI: 10.1152/japplphysiol.00182.2023

关键词

central CO2 response; congenital central hypoventilation syndrome; loop gain; peripheral CO2 response

向作者/读者索取更多资源

This study aimed to evaluate both peripheral and central CO2 chemosensitivity in patients with congenital central hypoventilation syndrome (CCHS) and their correlations with daytime PCO2 and arterial desaturation during exercise. The results showed that peripheral CO2 chemosensitivity is altered in some patients with CCHS, and daytime PETCO2 level is dependent on central and peripheral chemoreceptor responses.
Whether peripheral chemoreceptor response is altered in congenital central hypoventilation syndrome (CCHS) remains debated. Our aim was to prospectively evaluate both peripheral and central CO2 chemosensitivity and to evaluate their correlations with daytime PCO2 and arterial desaturation during exercise in CCHS. To this end, tidal breathing was recorded in patients with CCHS allowing the calculation of loop gain and its components {steady-state controller (assumed to mainly be peripheral chemosensitivity) and plant gains using a bivariate [end-tidal PCO2 (PETCO2) and ventilation] constrained model}, a hyperoxic, hypercapnic ventilatory response test (central chemosensitivity), and a 6-min walk test (arterial desaturation). The results of loop gain were compared with those previously obtained in a healthy group of similar age. The study prospectively included 23 subjects with CCHS, without daytime ventilatory support; the subjects had a median age of 10 (5.6 to 27.4) yr (15 females) with moderate polyalanine repeat mutation (PARM: 20/25, 20/26, n = 11), severe PARM (20/27, 20/33, n = 8), or non-PARM (n = 4). As compared with 23 healthy subjects (4.9-27.0 yr), the subjects with CCHS had a decreased controller gain and an increased plant gain. Mean daytime PETCO2 level of subjects with CCHS correlated negatively to both Log(controller gain) and the slope of CO2 response. Genotype was not related to chemosensitivity. Arterial desaturation on exercise correlated negatively with Log(controller) gain but not with the slope of the CO2 response. In conclusion, we demonstrate that peripheral CO2 chemosensitivity is altered in some patients with CCHS and that the daytime PETCO2 depends on central and peripheral chemoreceptor responses.

作者

我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。

评论

主要评分

4.5
评分不足

次要评分

新颖性
-
重要性
-
科学严谨性
-
评价这篇论文

推荐

暂无数据
暂无数据