TY - JOUR
T1 - Chemoreceptive mechanisms elucidated by studies of congenital central hypoventilation syndrome
AU - Spengler, Christina M.
AU - Gozal, David
AU - Shea, Steven A.
N1 - Funding Information:
This work was supported by a Swiss National Science Foundation grant (31-54071.98) to C.M. Spengler, a National Institutes of Health grant (HL-62149) to S.A. Shea, and by grants from the National Institutes of Health (HL-63912 and HL-65270), and the American Heart Association (AHA-0050442N) to D. Gozal.
PY - 2001
Y1 - 2001
N2 - Humans born with the condition of central hypoventilation during non-rapid eye movement sleep, termed congenital central hypoventilation syndrome (CCHS), invariably have absent or greatly diminished central hypercapnic ventilatory chemosensitivity. Genetic and pathological studies of CCHS may enable identification of the genes or areas of the central nervous system involved in the syndrome and thus implicated in central hypercapnic ventilatory chemosensitivity. Functional studies of CCHS permit a more quantitative assessment of the importance of ventilatory chemosensitivity in the regulation of breathing during wakefulness and sleep. The experimental evidence suggests that central hypercapnic ventilatory chemosensitivity is crucial in regulating alveolar ventilation during non-rapid eye movement sleep but not during rapid eye movement sleep or during many of the behaviors occurring during wakefulness. Presumably, other neural drives to breathe supervene to enable adequate ventilation. However, although physiological studies in CCHS subjects have been greatly instructive, their accurate interpretation will have to await future determination of the potential genetic and/or neuroanatomic basis of the syndrome.
AB - Humans born with the condition of central hypoventilation during non-rapid eye movement sleep, termed congenital central hypoventilation syndrome (CCHS), invariably have absent or greatly diminished central hypercapnic ventilatory chemosensitivity. Genetic and pathological studies of CCHS may enable identification of the genes or areas of the central nervous system involved in the syndrome and thus implicated in central hypercapnic ventilatory chemosensitivity. Functional studies of CCHS permit a more quantitative assessment of the importance of ventilatory chemosensitivity in the regulation of breathing during wakefulness and sleep. The experimental evidence suggests that central hypercapnic ventilatory chemosensitivity is crucial in regulating alveolar ventilation during non-rapid eye movement sleep but not during rapid eye movement sleep or during many of the behaviors occurring during wakefulness. Presumably, other neural drives to breathe supervene to enable adequate ventilation. However, although physiological studies in CCHS subjects have been greatly instructive, their accurate interpretation will have to await future determination of the potential genetic and/or neuroanatomic basis of the syndrome.
KW - Control of breathing, central and peripheral chemosensitivity
KW - Disease, congenital central hypoventilation syndrome
KW - Mammals, humans
KW - Sleep, CCHS
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U2 - 10.1016/S0034-5687(01)00294-8
DO - 10.1016/S0034-5687(01)00294-8
M3 - Article
C2 - 11738658
AN - SCOPUS:0035185102
SN - 0034-5687
VL - 129
SP - 247
EP - 255
JO - Respiration Physiology
JF - Respiration Physiology
IS - 1-2
ER -