Disorders of glucose metabolism in Prader-Willi syndrome: Results of a multicenter Italian cohort study

Nutr Metab Cardiovasc Dis. 2016 Sep;26(9):842-7. doi: 10.1016/j.numecd.2016.05.010. Epub 2016 Jun 3.

Abstract

Background and aims: Prader-Willi syndrome (PWS) is characterized by a high incidence of altered glucose metabolism (AGM). However, epidemiological data on impaired fasting glucose (IFG), impaired glucose tolerance (IGT) and type 2 diabetes mellitus (T2DM) are still discordant.

Methods and results: We performed a multicenter study based on 274 PWS patients [144 females, aged 20.3 ± 10.4 yrs (range: 8.1-50.1 years)] evaluating the prevalence for AGM in the entire group, and according to age (children <10 yrs; adolescents 10-18 yrs, and adults >18 yrs), Body Mass Index (BMI = kg/m(2)), gender, genotypes (deletion or uniparental disomy for chromosome 15), and GH therapy (GHT) (untreated, previously or currently treated). Altogether, AGM was detected in 67 (24.4%) of patients (0.7% IFG, 10.2% IGT, 13.5% T2DM). The prevalence of AGM was correlated to age (p = 0.001), BMI (p = 0.001) and HOMA-IR (p = 0.001). However, gender, genotype, and GHT did not influence AGM development in univariate analysis. These data were confirmed as positive predictors when inserted in a multivariate analysis model.

Conclusion: This study is the first report on the prevalence of AGM in a large population of PWS. Overall, PWS subjects show a high prevalence of AGM that appears more common in obese and adult subjects. Our data confirm the main role of obesity on the individual metabolic risk clustering in PWS, and thus reinforce the concept that improvement in weight control remains the most important goal of any PWS treatment program.

Keywords: Diabetes mellitus; GH therapy; Obesity; Prader–Willi syndrome.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Biomarkers / blood
  • Blood Glucose / metabolism*
  • Chi-Square Distribution
  • Child
  • Female
  • Glucose Metabolism Disorders / blood
  • Glucose Metabolism Disorders / diagnosis
  • Glucose Metabolism Disorders / epidemiology*
  • Human Growth Hormone / therapeutic use
  • Humans
  • Insulin Resistance
  • Italy / epidemiology
  • Linear Models
  • Male
  • Metabolic Syndrome / epidemiology
  • Middle Aged
  • Multivariate Analysis
  • Obesity / epidemiology
  • Prader-Willi Syndrome / blood
  • Prader-Willi Syndrome / diagnosis
  • Prader-Willi Syndrome / drug therapy
  • Prader-Willi Syndrome / epidemiology*
  • Prevalence
  • Retrospective Studies
  • Risk Factors
  • Young Adult

Substances

  • Biomarkers
  • Blood Glucose
  • Human Growth Hormone