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Congenital central I42hypoventilation syndrome combined with Hirschsprung disease diagnosed in the neonatal period

Jin Hyun Choi, Jin Hee Oh, Jong-Hyun Kim, Dae Kyun Koh, Seung-Chul Hong

Clin Exp Pediatr. 2006;49(4):446-450. Published online April 15, 2006

Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect...

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