Tailored management of life-threatening complications related to severe obesity in a young adult with Prader-Willi syndrome
- Author:
Min-Sun KIM
1
;
Jiyeon KIM
;
Joongbum CHO
;
Sung Yoon CHO
;
Dong-Kyu JIN
Author Information
- Publication Type:Case report
- From:Annals of Pediatric Endocrinology & Metabolism 2022;27(2):148-152
- CountryRepublic of Korea
- Language:English
- Abstract: 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.
