CONGENITAL CENTRAL HYPOVENTILATION SYNDROME (CURSE OF ONDINE SYNDROME)
Congenital central hypoventilation syndrome (CCHS, «Ondine’s curse») is a rare genetic disorder due to PHOX2B gene mutation. Nearly 1,000 children worldwide have CCHS. Classic CCHS presents as apparent hypoventilation with monotonous respiratory rates and shallow breathing either during sleep only or while awake as well as asleep, autonomic nervous system dysregulation (ANSD); and in some individuals, altered development of neural crest-derived structures (i. e. Hirschsprung disease) and/or tumors of neural crest origin. The article presents a clinical case of CCHS in preterm child with aggravated perinatal anamnesis.