Congenital central hypoventilation syndrome: an update

Pediatr Pulmonol. 1998 Oct;26(4):273-82. doi: 10.1002/(sici)1099-0496(199810)26:4<273::aid-ppul7>;2-c.


Awareness of the existence of CCHS has led to increasingly frequent reports of such patients from all over the world. However, the exact pathophysiologic mechanisms underlying the clinical manifestations of this congenital disease entity remain unknown. For the respiratory physiologist, CCHS can be viewed as an experiment of nature that provides an important and unique window into central cardiorespiratory regulation. For the pediatrician, CCHS children represent an unique clinical challenge in coordinating the diagnostic and therapeutic procedures required to enhance the patients' quality of life.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Autonomic Nervous System Diseases
  • Central Nervous System / abnormalities
  • Humans
  • Infant
  • Infant, Newborn
  • Sleep Apnea Syndromes / congenital*
  • Sleep Apnea Syndromes / physiopathology
  • Sleep Apnea Syndromes / therapy