Successful Early Dietary Intervention Avoids Obesity in Patients with Prader-Willi Syndrome: A Ten-Year Follow-up

Abstract

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. To investigate whether early diagnosis of PWS and strict dietary intervention prevents excessive weight gain in patients with PWS. 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. 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). 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.