Prader-Willi Syndrome Macronutrient Study

Duke University

Status: Completed

Conditions: Syndromic Obesity; Childhood Obesity; Prader Willi Syndrome

Treatments: Other: Low Fat diet; Other: Low Carbohydrate diet

Study type: Interventional

Funder types: Other

Identifiers

NCT02011360

Pro00049226

SFR2382 (Other Grant/Funding Number)

Details and patient eligibility

About

The overall objective is to explore the mechanisms by which macronutrients regulate food intake and weight gain in Prader Willi Syndrome (PWS).

Previous studies from the investigators' labs suggest that the increased appetite of PWS may be triggered or maintained by an increase in the levels of ghrelin, an appetite-stimulating hormone produced primarily by the stomach. This study will compare the effects of low carbohydrate diet versus low fat diet on levels of ghrelin, appetite suppressing hormones and markers of insulin sensitivity in patients with PWS.

The investigators hypothesize that the low carbohydrate diet will suppress plasma active ghrelin and increase appetite-suppressing hormones to a greater degree and for longer duration than the low fat diet and will thereby reduce hyperphagia and increase satiety. The investigators also hypothesize that the low carb diet will improve hormonal and metabolic markers (fatty acids, amino acids and organic acids) of insulin sensitivity and inflammatory cytokine profiles of children with PWS.

Enrollment: 10 patients

Sex: All

Ages: 5 to 17 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• diagnosis of Prader Willi syndrome confirmed by chromosome analysis (ie. interstitial deletion of paternally-derived chromosome 15q, uniparental maternal disomy or other chromosome 15 abnormalities)
• age 5 years to 17 years
• written informed consent and assent obtained and willingness to comply with the study schedule and procedures
• free T4, TSH values in the normal range (either endogenous or with thyroxine replacement)
• weight stable (BMI percentile fluctuation of <5 percentiles) over the preceding 2 months prior to the study

Exclusion criteria

• presence of other clinically significant disease that would impact body composition including diabetes mellitus, chronic inflammatory bowel disease, chronic severe liver or kidney disease or neurologic disorders
• concomitant use of medications known to affect body weight or investigational drug in the past year

Trial design

10 participants in 2 patient groups

Low carbohydrate diet

Other group

Description:

Low carbohydrate diet: 15%carb; 65%fat; 20% protein. This will be administered over 72 hour hospital stay.

Treatment:

Other: Low Fat diet

Low Fat diet

Other group

Description:

Low fat diet: 65%carb; 15%fat; 20% protein. This will be administered over a 72 hour hospital stay.

Treatment:

Other: Low Carbohydrate diet