Effect of non-pharmacological intervention on the nutritional status of patients with Prader Willi Syndrome

Abstract

IntroductionObesity is highly prevalent in patients with Prader-Willi syndrome (PWS), particularly among adults. This condition, which can be morbid in many cases, is multifactorial and has a complex management. The purpose of our study was to describe the feasibility of achieving a better nutritional status, including normal weight in individuals diagnosed with PWS, through specific nutritional interventions within the framework of a transdisciplinary treatment and without resorting to pharmacological treatments or growth hormone (GH). MethodologyThis observational study included patients with confirmed genetic diagnosis of PWS, receiving transdisciplinary treatment in a specialized rare diseases institution. Patients under treatment with GH and those under pharmacological treatment with nutritional objectives were excluded from the study. All patients attended our institution regularly on a weekly or fortnightly basis. Anthropometric records, including weight, height, and body mass index (BMI) were evaluated in each visit from treatment onset until the last check-up. ResultsWe included 24 patients with confirmed genetic diagnosis of PWS. At baseline, 9 patients (38%) had obesity grade III, 1 (4%) of obesity grade II, 10 (42%) of obesity grade I, 2 (8%) of overweight, and 2 patients (8%) with normal baseline weight. After a median duration of 52 months (interquartile range 23 - 116 months) of transdisciplinary nutritional treatment, we identified a significant reduction in BMI (baseline 40.2±15.7 kg/m2 vs. follow-up 28.3±6.7 kg/m2, p<0.0001), without significant differences regarding height (baseline 1.45±0.1 meters vs. follow-up 1.48±0.1 meters, p=0.09). ConclusionIn this study, we demonstrated that nutritional nonpharmacologic interventions immersed in a transdisciplinary treatment enabled a consistent and sustainable improvement in BMI and nutritional status among patients with PWS.