• Clinical and Experimental Pediatrics
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• 제55권6호
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• pp.212-214
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• 2012

Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third 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.

• 대한치과교정학회지
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• 제52권4호
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• pp.308-312
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• 2022

Since the emergence of neonatal infant orthodontics for treatments of cleft lip and palate with or without Robin sequence (RS) in Europe in the 1950s, advancements in design and scope of its application have been remarkable. As the first institution to adopt orthodontic airway plate (OAP) treatment in the United States in 2019, we saw a need for innovation of the original design to streamline the most labor-intensive and time-consuming aspects of OAP utilization. A solution is introduced using a systematic split expansion mechanism to re-size the OAP periodically to accommodate the neonate's maxillary growth. To date, seven RS patients have received this modified treatment protocol at our institution. Each patient completed full treatment using only one OAP. This innovative utilization method is aptly named the split orthodontic airway plate (S-OAP). Details of the S-OAP and its modifications from conventional OAP are reported.

• 대한기관식도과학회지
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• 제3권1호
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• pp.128-136
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• 1997

저자들은 Patau증후군에 동반된 후두연화증으로 심폐정지가 유발된 환아에서 $CO_2$레이저를 이용한 aryepiglottoplasty를 시행하여 극적인 호전을 보였기에 문헌고찰과 함께 치료경험을 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제45권3호
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• pp.383-389
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• 2002

저자들은 미숙아에서 청색증과 무호흡증으로 입원 치료 하던 중 저칼슘혈증으로 인한 경련이 나타난 환아에게서 흉부 X선 사진과 MRI상 흉선을 관찰할 수 없으면서 T 세포수의 감소와 부갑상선 홀몬수치 감소를 나타내고 소악증, 어구, 부리모양의 코 등의 안면 기형과 합지증, 코 역류증, 폐쇄성 수면 무호흡증, 구개 범인두 부전증 등의 증상을 보이며 염색체 22q11 극소결실이 FISH검사에서 확진된 부분형 DiGeorge 증후군 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.