title: Disorders of glucose metabolism in Prader-Willi syndrome: Results of a multicenter Italian cohort study.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Autoimmune Endocrine Diseases Unit, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy. Electronic address: dfintini@hotmail.com.
authors.forename: D
authors.initials: D
authors.lastname: Fintini
authors.affiliation: Division of Auxology, Italian Auxological Institute, Research Institute, Piancavallo, Verbania, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Grugni
authors.affiliation: Autoimmune Endocrine Diseases Unit, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Bocchini
authors.affiliation: Autoimmune Endocrine Diseases Unit, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Brufani
authors.affiliation: Pediatric Department, S. Raffaele Hospital, Milan, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Di Candia
authors.affiliation: Pediatric Endocrinology, Regina Margherita Hospital, Turin, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Corrias
authors.affiliation: Department of Pediatric Science and Surgery, Pediatric Hospital Giovanni XXIII, Bari, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Delvecchio
authors.affiliation: Pediatric Unit, Insubria University, Varese, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Salvatoni
authors.affiliation: Pediatric Unit, Oasi Maria SS, Research Institute, Troina, Enna, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Ragusa
authors.affiliation: Pediatric Department, University of Padua, Italy.
authors.forename: N
authors.initials: N
authors.lastname: Greggio
authors.affiliation: Department of Translational Sciences, University Federico II, Naples, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Franzese
authors.affiliation: Pediatric Endocrinology and Rare Diseases Unit, University of Bologna, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Scarano
authors.affiliation: Pediatric Unit, Salvini Hospital, Rho, Milan, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Trifirò
authors.affiliation: Pediatric Endocrinology and Rare Diseases Unit, University of Bologna, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Mazzanti
authors.affiliation: Pediatric Department, S. Raffaele Hospital, Milan, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Chiumello
authors.affiliation: Endocrinology and Diabetology Unit, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Cappa
authors.affiliation: Autoimmune Endocrine Diseases Unit, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy. Electronic address: antonino.crino@opbg.net.
authors.forename: A
authors.initials: A
authors.lastname: Crinò
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D015415:Biomarkers / Q000097:blood; D001786:Blood Glucose / Q000378:metabolism*; D016009:Chi-Square Distribution; D002648:Child; D005260:Female; D044882:Glucose Metabolism Disorders / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology*; D019382:Human Growth Hormone / Q000627:therapeutic use; D006801:Humans; D007333:Insulin Resistance; D007558:Italy / Q000453:epidemiology; D016014:Linear Models; D008297:Male; D024821:Metabolic Syndrome / Q000453:epidemiology; D008875:Middle Aged; D015999:Multivariate Analysis; D009765:Obesity / Q000453:epidemiology; D011218:Prader-Willi Syndrome / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
chemical_list: D015415:Biomarkers; D001786:Blood Glucose; D019382:Human Growth Hormone
keywords: Diabetes mellitus; GH therapy; Obesity; Prader–Willi syndrome