Disorders of glucose metabolism in Prader–Willi syndrome

Results of a multicenter Italian cohort study

Claudia Brufani, Sarah Bocchini, Stefania Di Candia, Andrea Corrias, A. Salvatoni, Letizia Ragusa, N. Greggio, A. Franzese, Emanuela Scarano, G. Trifiro, L. Mazzanti, G. Chiumello, Danilo Fintini

Research output: Contribution to journalArticle

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/m2), 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.

Original languageEnglish
Pages (from-to)842-847
Number of pages6
JournalNutrition, Metabolism and Cardiovascular Diseases
Volume26
Issue number9
DOIs
Publication statusPublished - Sep 1 2016

Fingerprint

Glucose Metabolism Disorders
Cohort Studies
Glucose
Glucose Intolerance
Type 2 Diabetes Mellitus
Fasting
Genotype
Chromosomes, Human, Pair 15
Multicenter Studies
Cluster Analysis
Body Mass Index
Therapeutics
Multivariate Analysis
Obesity

Keywords

  • Diabetes mellitus
  • GH therapy
  • Obesity
  • Prader–Willi syndrome

ASJC Scopus subject areas

  • Medicine (miscellaneous)
  • Endocrinology, Diabetes and Metabolism
  • Nutrition and Dietetics
  • Cardiology and Cardiovascular Medicine

Cite this

Disorders of glucose metabolism in Prader–Willi syndrome : Results of a multicenter Italian cohort study. / Brufani, Claudia; Bocchini, Sarah; Di Candia, Stefania; Corrias, Andrea; Salvatoni, A.; Ragusa, Letizia; Greggio, N.; Franzese, A.; Scarano, Emanuela; Trifiro, G.; Mazzanti, L.; Chiumello, G.; Fintini, Danilo.

In: Nutrition, Metabolism and Cardiovascular Diseases, Vol. 26, No. 9, 01.09.2016, p. 842-847.

Research output: Contribution to journalArticle

Brufani, C, Bocchini, S, Di Candia, S, Corrias, A, Salvatoni, A, Ragusa, L, Greggio, N, Franzese, A, Scarano, E, Trifiro, G, Mazzanti, L, Chiumello, G & Fintini, D 2016, 'Disorders of glucose metabolism in Prader–Willi syndrome: Results of a multicenter Italian cohort study', Nutrition, Metabolism and Cardiovascular Diseases, vol. 26, no. 9, pp. 842-847. https://doi.org/10.1016/j.numecd.2016.05.010
Brufani, Claudia ; Bocchini, Sarah ; Di Candia, Stefania ; Corrias, Andrea ; Salvatoni, A. ; Ragusa, Letizia ; Greggio, N. ; Franzese, A. ; Scarano, Emanuela ; Trifiro, G. ; Mazzanti, L. ; Chiumello, G. ; Fintini, Danilo. / Disorders of glucose metabolism in Prader–Willi syndrome : Results of a multicenter Italian cohort study. In: Nutrition, Metabolism and Cardiovascular Diseases. 2016 ; Vol. 26, No. 9. pp. 842-847.
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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/m2), 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.",
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N2 - 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/m2), 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.

AB - 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/m2), 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.

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