Prader–Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach

Abstract

Purpose of Review Prader–Willi syndrome (PWS) is a rare and complex genetic disorder with multiple effects on the metabolic, endocrine, and neurological systems, as well as behavioral and intellectual difficulties. Despite advances in understanding the genetic basis of obesity in PWS, there are conflicting data on its management. Therefore, the present manuscript aims to provide an update on the nutritional treatment and pharmacological approach in adult patients with PWS. Recent Findings The management of obesity in patients with PWS is challenging and requires the cooperation of an experienced multidisciplinary team, including the nutritionist. An adequate clinical evaluation including nutritional and biochemical parameters should be performed to tailor the best therapeutic strategy. Both lifestyle and pharmacological interventions may represent useful strategies to prevent the high rate of morbidity and mortality related to PWS. The use of bariatric surgery is still controversial. Summary Although it is imperative to adopt an obesity prevention strategy in childhood, there is promising evidence for the treatment of obesity in adulthood with current obesity medications in conjunction with lifestyle interventions

Analysis of the Diet Quality and Nutritional State of Children, Youth, and Young Adults with Prader–Willi Syndrome: A Polish Multiple Case Study

“…These ratios are currently recommended as the standard approach in PWS management. It is also recommended to align the PWS diet with the Mediterranean diet model, incorporating small and frequent meals throughout the day to help control hyperphagia [32,46].…”

Management of obesity in prader-willi syndrome

“…Studies indicate increased satiety after meals in patients with PWS. (Barrea et al, 2022;Éva Erhardt;Dénes Molnár, 2022;Nolan;Proietto;Sumithran, 2022) Liraglutide (GLP-1 analogue) has been shown to reduce BMI in children and adolescents with PWS and obesity. However, there were no significant differences between the children and adolescents who received liraglutide and those who received placebo or no treatment.…”

Positive effects of ketogenic diet on weight control in children with obesity due to Prader–Willi syndrome

“…The long‐term success of diet, physical activity and behavioural interventions could have not been demonstrated due to hyperphagia and lack of appetite control 6 . A low‐calorie diet is the standard recommendation for patients with PWS to achieve weight management, and different dietary approaches including Ketogenic diet (KD) have been suggested for selected adults with PWS 8 . The literature comprises limited information on the outcomes of specific diets used in PWS; further research is required on diet composition for healthy weight gain, which might provide normal growth 7 …”

“…6 A lowcalorie diet is the standard recommendation for patients with PWS to achieve weight management, and different dietary approaches including Ketogenic diet (KD) have been suggested for selected adults with PWS. 8 The literature comprises limited information on the outcomes of specific diets used in PWS; further research is required on diet composition for healthy weight gain, which might provide normal growth. 7 KD is a well-known type of nutrition composed of high-lipid and low-carbohydrate (<50 g/day), and has been preferred for a long time in drug-resistant epilepsy patients.…”