UNLABELLED: Hyperphagia is a frequent symptom in patients with Prader-Willi syndrome (PWS) and results in marked obesity with the risk of metabolic and cardiovascular complications. AIM: To investigate whether early diagnosis of PWS and strict dietary intervention prevents excessive weight gain in patients with PWS. PATIENTS AND METHODS: A strict fat reduced and modified carbohydrate diet consisting of 10 kcal/ cm height was provided to nine patients (seven female, two male) diagnosed early with PWS (group A). Patients were prospectively followed at our center with follow-up visits every three months. Eight patients with late diagnosis of PWS served as controls (group B). Body mass index (BMI) SDS and height SDS were compared between these two groups over a ten-year period. RESULTS: At the age of two years height SDS and BMI SDS were significantly lower in group A (-2.9 vs -1.2, p <0.05, and BMI SDS -0.1 vs +1.8, p < 0.05). After ten years BMI SDS increased significantly to +1.2 SDS in group A, but was still significantly lower than in group B (BMI SDS +2.4), p <0.005. Patients without restrictive diet were significantly taller than patients on the diet (height SDS group A -2.8 vs group B -1.3, p < 0.05). CONCLUSION: Early dietary treatment starting at the second year of life and continued until the age of ten years is effective in avoiding excessive weight gain in patients with PWS, but results in shorter stature. Therefore growth hormone may be a useful additional treatment in these patients.
UNLABELLED: Hyperphagia is a frequent symptom in patients with Prader-Willi syndrome (PWS) and results in marked obesity with the risk of metabolic and cardiovascular complications. AIM: To investigate whether early diagnosis of PWS and strict dietary intervention prevents excessive weight gain in patients with PWS. PATIENTS AND METHODS: A strict fat reduced and modified carbohydrate diet consisting of 10 kcal/ cm height was provided to nine patients (seven female, two male) diagnosed early with PWS (group A). Patients were prospectively followed at our center with follow-up visits every three months. Eight patients with late diagnosis of PWS served as controls (group B). Body mass index (BMI) SDS and height SDS were compared between these two groups over a ten-year period. RESULTS: At the age of two years height SDS and BMI SDS were significantly lower in group A (-2.9 vs -1.2, p <0.05, and BMI SDS -0.1 vs +1.8, p < 0.05). After ten years BMI SDS increased significantly to +1.2 SDS in group A, but was still significantly lower than in group B (BMI SDS +2.4), p <0.005. Patients without restrictive diet were significantly taller than patients on the diet (height SDS group A -2.8 vs group B -1.3, p < 0.05). CONCLUSION: Early dietary treatment starting at the second year of life and continued until the age of ten years is effective in avoiding excessive weight gain in patients with PWS, but results in shorter stature. Therefore growth hormone may be a useful additional treatment in these patients.
Authors: Ann O Scheimann; Merlin G Butler; Jennifer L Miller; Phillip D K Lee; David A Stevenson; Janalee Heinemann; Daniel J Driscoll Journal: Obes Surg Date: 2012-03 Impact factor: 4.129
Authors: Tie-Lin Yang; Yan Guo; Li-Shu Zhang; Qing Tian; Han Yan; Christopher J Papasian; Robert R Recker; Hong-Wen Deng Journal: J Clin Endocrinol Metab Date: 2010-05-13 Impact factor: 5.958
Authors: William S Garver; Sara B Newman; Diana M Gonzales-Pacheco; Joseph J Castillo; David Jelinek; Randall A Heidenreich; Robert A Orlando Journal: Genes Nutr Date: 2013-03-08 Impact factor: 5.523