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KMID : 1036920220270020148
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Annals of Pediatric Endocrinology & Metabolism
2022 Volume.27 No. 2 p.148 ~ p.152
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Tailored management of life-threatening complications related to severe obesity in a young adult with Prader-Willi syndrome
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Kim Min-Sun
Kim Ji-Yeon
Cho Joong-Bum
Cho Sung-Yoon
Jin Dong-Kyu
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Abstract
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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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KEYWORD
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Prader-Willi syndrome, Hyperphagia, Obesity, Pulmonary heart disease, Heart failure, Obstructive sleep apnea
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