Hormonal and metabolic effects of carbohydrate restriction in children with Prader-Willi syndrome.

Journal Article (Journal Article)

OBJECTIVE: Macronutrient regulation of hyperphagia and adiposity in Prader-Willi syndrome (PWS) is poorly understood. We compared fasting and postprandial concentrations of hormones and metabolites in eight PWS children (age 9-18 years) fed, in random order, low carbohydrate, high-fat (LC, 15% carb; 65% fat; 20% protein) and low-fat, high carbohydrate (LF, 65% carb, 15% fat, 20% protein) diets matched for calories and protein. METHODS: Participants were randomized to consume either the LC or LF diet during a first hospital admission and the second diet during a subsequent admission. Blood samples were obtained after overnight fasting and 1 hour after a mixed meal. RESULTS: Relative to subjects consuming the LF diet, subjects consuming the LC diet had: lower postprandial insulin concentrations (P = 0.02); higher fasting GLP-1 AND GIP concentrations and increased postprandial GLP-1 (P < 0.02); reduced ratio of fasting ghrelin to GLP-1 (P = 0.0078); increased FFA and fatty acid oxidation, as assessed by concentrations of even-chain acylcarnitines (P < 0.001); lower fasting TG and TG/HDL ratio (P < 0.01); and higher concentrations of branch chain amino acids (P < 0.01). There were no changes in glucose, PYY, or adiponectin. CRP, AST and ALT were all higher (P < 0.01) on the LC diet. CONCLUSIONS: Increases in GLP-1 with low carbohydrate feeding and reductions in the ratio of ghrelin to GLP-1 might limit food intake and improve glycaemic control in PWS. Other potential benefits of carbohydrate restriction may include fat mobilization and oxidation and reductions in the TG/HDL ratio, a marker of insulin resistance. However, increases in CRP, AST and ALT necessitate longer-term studies of low carbohydrate efficacy and safety.

Full Text

Duke Authors

Cited Authors

  • Irizarry, KA; Mager, DR; Triador, L; Muehlbauer, MJ; Haqq, AM; Freemark, M

Published Date

  • April 2019

Published In

Volume / Issue

  • 90 / 4

Start / End Page

  • 553 - 561

PubMed ID

  • 30614551

Pubmed Central ID

  • PMC6805129

Electronic International Standard Serial Number (EISSN)

  • 1365-2265

Digital Object Identifier (DOI)

  • 10.1111/cen.13933


  • eng

Conference Location

  • England