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

Andrea Gabriela Krochik1, Blanca Ozuna, María Torrado

  • 1Nutrition Department, Hospital de Pediatría J P Garrahan, Buenos Aires, Argentina.

Journal of Pediatric Endocrinology & Metabolism : JPEM
|September 26, 2006
PubMed

Insights

Children and adolescents with Prader-Willi syndrome (PWS) show lower insulin resistance and impaired beta-cell function compared to those with multifactorial obesity (MO). This suggests a unique metabolic profile in PWS, impacting carbohydrate metabolism and insulin sensitivity.

Area of Science:

  • Metabolic disorders
  • Pediatric endocrinology
  • Genetics

Background:

  • Prader-Willi syndrome (PWS) is a complex genetic disorder associated with obesity and metabolic complications.
  • Carbohydrate metabolism and insulin sensitivity are frequently affected in individuals with PWS.
  • Understanding these metabolic alterations is crucial for managing PWS patients.

Purpose of the Study:

  • To compare carbohydrate metabolism, insulin sensitivity, and insulin secretion in children and adolescents with PWS versus controls with multifactorial obesity (MO).
  • To investigate the relationship between obesity, insulin resistance, and beta-cell function in PWS.

Main Methods:

  • Oral glucose tolerance test (OGTT) was performed on 75 PWS patients and 395 MO controls.
  • Insulin resistance (IR) and beta-cell function were assessed using Homeostasis Model Assessment (HOMA-IR, HOMA-B), insulin glucose index, and insulin sensitivity index (ISI Composite).

Main Results:

  • The incidence of diabetes mellitus was 0% in PWS and 1.5% in MO.
  • Carbohydrate intolerance was observed in 9.3% of PWS patients and 7.6% of MO controls (not significant).
  • Patients with PWS exhibited lower HOMA-IR (2.47 vs 4.18, p=0.04) and higher ISI Composite (6 vs 4.18, p=0.04), indicating reduced insulin resistance.

Conclusions:

  • Children and adolescents with PWS demonstrate lower insulin resistance compared to those with multifactorial obesity.
  • A dissociation between beta-cell secretion and the degree of obesity was observed in PWS patients.
  • These findings highlight distinct metabolic characteristics in PWS, impacting glucose regulation.
Abstract

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