Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Pediatric Specialists of Virginia/INOVA Children's Hospital, Fairfax, Virginia, USA.
Orphanet J Rare Dis. 2020 Jun 3;15(1):135. doi: 10.1186/s13023-020-01412-w.
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.
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.
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.
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.
普拉德-威利综合征（PWS）是肥胖症最常见的遗传原因。人们已经采用了各种饮食策略来控制 PWS 患者的体重。
这是一项临床可行性研究，旨在测试改良阿特金斯饮食（低碳水化合物、高脂肪）在超重/肥胖的 6-12 岁 PWS 儿童中的应用。参与者接受改良阿特金斯饮食 4 个月，然后返回进行人体测量学重复，包括重复实验室检查和行为问卷。
7 名儿童（6-12 岁）入组该研究。4 名参与者完成了为期 4 个月的饮食试验；2 名无法遵守饮食并提前停止。一名患者体重减轻了 2.9 公斤；其余患者体重保持不变。一名患者的 LDL 升高（基于更大规模的研究预期）和高钙尿症（无肾结石），这是预料之中的不良反应。家庭主观上注意到饮食对暴食和行为的积极影响。
改良阿特金斯饮食可能是 PWS 儿童体重管理的一种可行的低碳水化合物选择。需要进一步研究 PWS 患者长期使用该饮食的情况。
