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Ann Scheimann1, Merlin G. Butler2, Linda Gourash3, William Klish4 

Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD1, Department of Pediatrics, Children’s Mercy Hospital, Kansas City, MO2, Pittsburgh Partners, Pittsburgh, PA3, Department of Pediatrics, Baylor College of Medicine, Houston, TX4 

Prader-Willi syndrome (PWS) is a complex genetic disorder localized to chromosome 15 and considered the most common genetic etiology for the development of obesity.  Although some morbidities associated with PWS including respiratory disturbance/hypoventilation, diabetes and stroke are commonly seen in obesity, others such as osteoporosis i, ii  arising from diet restrictions, growth hormone deficiency and hypogonadism as well as altered pain threshold/inability to vomit pose unique issues.  A variety of bariatric procedures have been utilized to cause gastric stasis,  decrease gastric volume, and  induce malabsorption with poor results in comparison to normal obese. 

 i Kroonen LT, Herman M, Pizzutullo PD, Macewen GD.  Prader-Willi Syndrome:  Clinical Concerns for the Orthopedic Surgeon.   J Pediatr Orthop 2006;26:673-9.

 iiHoybye C, Hilding A, Jacobsson H, Thoren M.  Metabolic Profile and Body Composition in Adults with Prader-Willi Syndrome and Severe Obesity.  J Clin Endocrinol Metab 2002;87:3590-7.

 

edited: 02/09/2012

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