Characterization of alterations in carbohydrate metabolism in children with Prader-Willi syndrome

J Pediatr Endocrinol Metab. 2006 Jul;19(7):911-8. doi: 10.1515/jpem.2006.19.7.911.

Abstract

Objective: To study carbohydrate metabolism and insulin sensitivity and secretion in children and adolescents with Prader-Willi syndrome (PWS) compared with multifactorial obesity (MO) controls.

Patients and methods: Seventy-five patients with PWS and 395 controls with MO were studied by oral glucose tolerance test. Insulin resistance (IR) and beta-cell function were assessed by homeostasis model assessment (HOMA), insulin glucose index, fasting insulin and insulin sensitivity index.

Results: The incidence of diabetes mellitus was 0% in PWS and 1.5% in MO, while carbohydrate intolerance was 9.3% in the former group and 7.6% in the latter (NS); basal insulin level (12 +/- 8.2 vs 22.3 +/- 25 mU/ml) and HOMA-IR (2.47 +/- 1.6 vs 4.18 +/- 5.05) were lower in PWS (p = 0.004 and 0.04, respectively), whereas HOMA beta-cell index was lower in PWS than in MO (59 +/- 42 vs 102 +/- 119, p = 0.03). ISI Composite was higher in PWS compared to MO (6 +/- 5.7 vs 4.18 +/- 5.05, p = 0.04).

Conclusion: Patients with PWS presented lower insulin resistance and a dissociation between beta-cell secretion and the degree of obesity.

MeSH terms

  • Adolescent
  • Adult
  • Carbohydrate Metabolism*
  • Child
  • Child, Preschool
  • Diabetes Mellitus, Type 2 / etiology
  • Diagnostic Techniques, Endocrine
  • Female
  • Glucose / metabolism*
  • Glucose Tolerance Test
  • Humans
  • Incidence
  • Insulin / metabolism*
  • Insulin Resistance*
  • Male
  • Obesity / genetics
  • Obesity / physiopathology
  • Prader-Willi Syndrome / metabolism*
  • Prader-Willi Syndrome / pathology

Substances

  • Insulin
  • Glucose