Intractable & Rare Diseases Research | A Scoping Review of Dietary Interventions for Obesity Management in Prader-Willi Syndrome

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This review systematically summarizes the dietary intervention strategies for obesity management in Prader-Willi Syndrome (PWS) published between 2017 and 2023, including ketogenic diet, modified Atkins diet, and Mediterranean diet, and discusses the effects of these interventions on weight, body fat, and lipid profiles. It also emphasizes the importance of multidisciplinary management in improving adherence and outcomes.

 

Overview of the Literature
The article titled, 'A scoping review of dietary interventions to treat obesity among Prader-Willi syndrome individuals', published in the journal Intractable & Rare Diseases Research, reviews and summarizes recent studies on dietary interventions for obesity management in individuals with PWS. By systematically searching PubMed, Scopus, EBSCOhost, and Cochrane Library databases, the authors selected full-text English articles published between 2017 and 2023. Five studies were included in the analysis, covering intervention strategies such as ketogenic diet, modified Atkins diet, and multidisciplinary metabolic rehabilitation programs combined with physical activity. These studies assessed the impact on weight, body fat, and overall health. The article also discusses the heterogeneity of study designs, sample sizes, geographic distributions, and intervention durations, and suggests future research should focus on standardized protocols and long-term follow-ups.

Background Information
Prader-Willi Syndrome (PWS) is a rare genetic disorder caused by the loss of paternally expressed genes in the 15q11-q13 region of chromosome 15. Its global incidence ranges from 1/15,000 to 1/25,000. Clinical features include pathological obesity, dysregulated appetite, hypotonia, hypogonadism, cognitive impairments, and behavioral issues. Patients often face significant challenges in dietary control and weight management. Nutritional status in PWS typically progresses through multiple stages: feeding difficulties in infancy, increased appetite in early childhood, and persistent hyperphagia and weight gain in adolescence and adulthood. While growth hormone (GH) therapy has been extensively studied, dietary management remains a key strategy for controlling obesity, though there is currently no unified standard for intervention. Most existing studies are small-sample, retrospective, or short-term interventions, limiting the evidence base for long-term management. This study represents the first systematic scoping review of recent dietary interventions for obesity in PWS, aiming to provide a reference framework for clinical practice and future research.

 

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Research Methods and Experimental Design
The study employed the six-stage scoping review framework proposed by Arksey and O'Malley, systematically searching four databases—PubMed, Scopus, EBSCOhost, and Cochrane Library—to identify full-text English articles published between 2017 and 2023. Inclusion criteria were: involvement of individuals with PWS, dietary interventions targeting obesity management, and availability of full text. Exclusion criteria were: interventions involving only hormone therapy, pharmacological or surgical interventions, study protocols, conference abstracts, case reports, reviews, or short communications. The literature selection was conducted independently by the research team, with dual review by two authors to minimize bias. Data extraction included author, year, sample size, age, country, study design, intervention type, control group, adherence, and main outcomes. The PRISMA-ScR flowchart was used to illustrate the literature selection process, and three nutrition experts independently assessed the quality of included studies.

Key Findings and Perspectives

• Of the five studies, three used dietary interventions alone, and two combined diet with physical activity. Ketogenic and Mediterranean diets showed some efficacy in weight and body fat control, but required continuous monitoring.
• Modified Atkins diet (MAD) demonstrated short-term weight maintenance or mild weight loss in children with PWS, but led to significant weight regain and poor adherence after discontinuation.
• Structured dietary education and personalized nutrition plans contributed to weight control, particularly when combined with regular follow-ups and family support.
• Multidisciplinary metabolic rehabilitation programs, such as those combining diet and exercise, showed significant improvements in BMI, weight, and lipid profiles in long-term follow-ups, especially among institutionalized individuals with PWS.
• Ketogenic diet showed reductions in weight and body fat in children with PWS, but its long-term safety and side effects require further investigation.

Implications and Future Directions
This study provides a systematic review of dietary interventions for obesity management in PWS, highlighting their potential while acknowledging current limitations, including small sample sizes, heterogeneous study designs, and lack of long-term follow-up. Future studies should involve larger cohorts, standardized intervention protocols, and uniform outcome measures to enhance the quality of evidence. In addition, personalized dietary strategies, long-term adherence plans, and multidisciplinary collaboration should be explored to optimize nutritional management for individuals with PWS.

 

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Conclusion
Prader-Willi Syndrome (PWS) is a rare but complex genetic disorder associated with severe obesity and eating behavior disorders. Dietary intervention is a critical approach for managing PWS-related obesity, though current evidence is limited, mostly retrospective or small-sample studies. This scoping review analyzed five recent studies and found that ketogenic diet, modified Atkins diet, Mediterranean diet, and multidisciplinary metabolic rehabilitation programs have shown some effectiveness in weight management. However, strict dietary restrictions often result in poor adherence and weight regain. Structured dietary education, personalized nutrition planning, and multidisciplinary supervision can enhance long-term outcomes. Future research should include more randomized controlled trials, standardized study designs, and long-term follow-ups to establish more reliable dietary management guidelines and explore the relationship between genotype and dietary response. This review provides preliminary evidence for obesity management in PWS and offers valuable insights for both clinical practice and future research directions.

 

Marwa Aman, Haslina Abdul Hamid, and Roslee Rajikan. A scoping review of dietary interventions to treat obesity among Prader-Willi syndrome individuals. Intractable & Rare Diseases Research.