G Grugni1, G Guzzaloni, F Morabito. 1. Department of Auxology, IRCCS S. Giuseppe Hospital, Istituto Auxologico Italiano Foundation, Verbania, 28921 Italy. gragru@jumpy.it
Abstract
BACKGROUND: Prader-Willi syndrome (PWS) is the most common genetic obesity. Excessive weight gain follows failure-to-thrive in early infancy; in adolescents and young adults, excess body weight can exceed 100%. The hyperphagia associated with PWS is responsible for the early mortality. Dietary restriction, alone or combined with anorexic drugs, are ineffective to induce a permanent weight loss. Thus, surgical treatment of morbid obesity in PWS has been attempted, but gastric restrictive operations are unable to produce stable weight loss. In a small number of patients, favorable results have been reported with biliopancreatic diversion (BPD). CASE REPORT: A 24-year-old woman with PWS, Pickwickian, at age 21 weighed 80 kg (BMI= 50) and underwent BPD. RESULTS: 3 years after the BPD she regained 21 of the 26 kg lost; somnolence and respiratory difficulties were the same as before surgery. The patient now presents severe reduction of bone mass density, hypochromic anemia, hypoproteinemia, and diarrhea associated with eating. CONCLUSION: The regain of weight following BPD suggests that this procedure alone is not adequate for long-term control of obesity in PWS.
BACKGROUND:Prader-Willi syndrome (PWS) is the most common genetic obesity. Excessive weight gain follows failure-to-thrive in early infancy; in adolescents and young adults, excess body weight can exceed 100%. The hyperphagia associated with PWS is responsible for the early mortality. Dietary restriction, alone or combined with anorexic drugs, are ineffective to induce a permanent weight loss. Thus, surgical treatment of morbid obesity in PWS has been attempted, but gastric restrictive operations are unable to produce stable weight loss. In a small number of patients, favorable results have been reported with biliopancreatic diversion (BPD). CASE REPORT: A 24-year-old woman with PWS, Pickwickian, at age 21 weighed 80 kg (BMI= 50) and underwent BPD. RESULTS: 3 years after the BPD she regained 21 of the 26 kg lost; somnolence and respiratory difficulties were the same as before surgery. The patient now presents severe reduction of bone mass density, hypochromic anemia, hypoproteinemia, and diarrhea associated with eating. CONCLUSION: The regain of weight following BPD suggests that this procedure alone is not adequate for long-term control of obesity in PWS.
Authors: F De Peppo; G Di Giorgio; M Germani; E Ceriati; P Marchetti; C Galli; M G Ubertini; S Spera; G Ferrante; M Cuttini; M Cappa; G Castelli Gattinara; M Rivosecchi; A Crinò Journal: Obes Surg Date: 2008-05-01 Impact factor: 4.129