Article
[Ondine syndrome or central congenital hypoventilation syndrome].
La Revue du praticien - 31 Jan 2006
Trang Ha
Abstract excerpt
Congenital central hypoventilation syndrome (CCHS) is a rare disease due to a severely impaired central autonomic control of breathing and dysfunction of the autonomous nervous system. The incidence is estimated to be at 1 of 200,000 livebirths. A heterozygous mutation of PHOX-2B gene is found in 90% of the patients. Association with a Hirschsprung's disease is observed in 16% of the cases. Despite a high...
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