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Ann Pediatr Endocrinol Metab > Volume 10(2); 2005 > Article
A Case of Bariatric Surgery in a Patient with Prader-Willi Syndrome and Severe Morbid Obesity.
Sun Woo Lee, Sung Uk Hwang, Seung Ho Choi, Duk Hee Kim
1Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea. DHKim3@yumc.yonsei.ac.kr
2Department of General Surgery, Yonsei University College of Medicine, Seoul, Korea.
Abstract
Prader-Willi syndrome is a well known multi-systemic disorder featuring hypothalamic dysfunction and hypotonia with underlying chromosomal anomaly. High morbidity, early mortality due to complications as diabetes mellitus, hypertension, athero-sclerosis, and cardio-respiratory failure have been reported from poorly tolerated obesity and combined metabolic disorders. We experienced a 20-year- old girl with Prader-Willi syndrome diagnosed at the age of 7. She underwent massive, uncontrolled weight gain despite constant multi-drug therapy including human recombinant growth hormone, orlistat, sibutramine and combined behavioral managements. Her body weight reached 104 kg and she developed dyslipidemia, fatty liver, hypertension, diabetes mellitus and episodes of sleep apnea. She showed no significant response to the supportive treatment because of characteristic involvement in CNS especially hypothalamus and psycho-behavioral disorders. As Bariatric surgery was the most effective and curative method in treatment of severe morbid obesity, Roux-en-Y gastric bypass was performed. After the operation, she showed substantial weight loss and consequent improvements in obesity related complications. She could stop all medications concerning for obesity. We report a case of bariatric surgery on a girl with Prader-Willi syndrome and combined severe morbid obesity who underwent bariatric surgery which resulted in marked weight loss and improvements in associated complications.
Keywords: Prader-Willi syndrome;Obesity;Bariatric surgery


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