Prader-Willi syndrome is a disorder of genetic imprinting characterized by common features including early feeding problems and failure to thrive followed by gradual weight gain and development of hyperphagia. Long-term practice has recommended use of dietary restriction using a variety of approaches for weight control/obesity prevention of obesity with some recent anecdotal data suggesting benefits of carbohydrate restricted diets upon hyperphagia/anxiety. A pilot study of implementation of a modified Atkins diet in school aged children with Prader-Willi syndrome is proposed to assess the impact upon behavior and hyperphagia.
*Funded by FPWR-Canada
Research Outcomes: Public Summary
Background: Prader-Willi Syndrome (PWS) is the most common genetic cause of obesity. Various dietary strategies have been used for weight management for people with PWS. Methods: This was a clinical feasibility study to test the use of the Modified Atkins Diet (low carbohydrate and high fat) for children with PWS ages 6-12 years who were overweight/obese. Participants went on the Modified Atkins Diet for 4 months and then returned to have anthropometry repeated including repeat labs and behavior questionnaires. Results: Seven children (ages 6-12) were enrolled in the study. Four participants completed the 4-month diet trial; two were unable to comply with the diet and stopped prematurely. One patient lost 2.9 kg; the others maintained their weight. Adverse effects were increases in LDL (expected based on larger studies) and hypercalciuria (with no renal stones) for one patient. Positive effects on hyperphagia and behavior were noted subjectively by families. Conclusion: The Modified Atkins Diet can be a feasible low carbohydrate option for children with Prader-Willi Syndrome for weight management. Long-term use of the diet in patients with Prader-Willi Syndrome needs to be studied further.
Research Outcomes: Publications
The modified Atkins diet in children with Prader-Willi syndrome. Felix G, Kossoff E, Barron B, Krekel C, Getzoff Testa E, Scheimann A. Orphanet Journal of Rare Diseases (2020) 15:135. doi: 10.1186/s13023-020-01412-w.
Ann Scheimann, MD
Johns Hopkins University