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CASE REPORT
Korean J Pediatr 2012 June;55(6) :212-214.
Published online 2012 June 15.        doi:https://doi.org/10.3345/kjp.2012.55.6.212
Pulmonary hypertension due to obstructive sleep apnea in a child with Rubinstein-Taybi syndrome
Hyung Soon Choi (Choi HS)1, Jeong Jin Yu (Yu JJ)2, Young-Hwue Kim (Kim YH)2, Jae-Kon Ko (Ko JK)2, In-Sook Park (Park IS)2
1Department of Pediatrics, Kosin University Gospel Hospital, Busan, Korea
2Division of Pediatric Cardiology, Department of Pediatrics, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Jeong Jin Yu ,Tel: +82-2-3010-3924, Fax: +82-2-473-3725, Email: pediatrist@medimail.co.kr
Copyright © 2012 by The Korean Pediatric Society
ABSTRACT
Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately onethird of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.
Keywords: Rubinstein-Taybi syndrome | Obstructive sleep apnea | Pulmonary hypertension
 
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Treatment of obstructive sleep apnea in children  2010 October;53(10)
Pulmonary hypertension in infants with bronchopulmonary  2010 June;53(6)
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