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Journal of the Korean Society of Neonatology 2004;11(1):72-76.
Published online May 1, 2004.
A Case of Ondine's Curse with Hirschsprung Disease.
In Ok Hwang, Eun Sil Lee
Department of Pediatrics, College of Medicine, Yeungnam University, Daegu, Korea. les205@med.yu.ac.kr
선천성 거대 결장증을 동반한 Ondine’s Curse 1례
Ondine's curse, also called congenital central alveolar hypoventilation, is a rare disorder involving failed automatic control of respiration in the absence of cardiopulmonary disease, resulting in inadequate ventilation with progressive hypercapnia and hypoxia during sleep. Although the exact pathophysiological mechanism remains unknown, it is thought that congenital defects in central chemoreceptor that originates from the neural crest cells may be responsible. This syndrome is often reported in association with Hirschsprung disease, and their co-occurrence suggests a common etiology, involving abnormal distribution and/or migration of neutral crest cells. We report a case of Ondine's curse with Hirschsprung disease in a 1-day-old neonate required endotracheal intubation and assisted ventilation.
Key Words: Congenital central alveolar hypoventilation; Hirschsprung disease


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