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논문 기본 정보

자료유형
학술저널
저자정보
Kim Min-Sun (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine) Kim Jiyeon (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine) Cho Joongbum (Department of Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Med) Cho Sung Yoon (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine) Jin Dong-Kyu (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine)
저널정보
대한소아내분비학회 Annals of Pediatirc Endocrinology & Metabolism Annals of Pediatric Endocrinology & Metabolism 제27권 제2호
발행연도
2022.6
수록면
148 - 152 (5page)
DOI
10.6065/apem.2142022.011

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초록· 키워드

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Prader-Willi syndrome (PWS) is characterized by hypotonia, distinctive facial features, hyperphagia, obesity, short stature, hypogonadism, intellectual disability, and behavior problems. Uncontrolled hyperphagia can lead to dangerous food-seeking behavior and with life-threatening obesity. Severe obesity is prone to obstructive sleep apnea (OSA) and can lead to cor pulmonale. This study reports on a case involving a 21-year-old man with PWS who developed OSA due to severe obesity, which led to cor pulmonale, a life-threatening complication. Multidisciplinary care provided in the intensive care unit included weight reduction, ventilation support, antipsychotics, sedative drugs, rehabilitation, and meticulous skin care. The patient did recover. To prevent severe obesity in adults with PWS, hyperphagia must be controlled, and the patient must also be managed by an endocrinologist throughout childhood.

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