Metabolic syndrome in children with Prader–Willi syndrome: The effect of obesity

Brambilla, P.; Crinò, A.; Bedogni, G.; Bosio, L.; Cappa, M.; Corrias, A.; Delvecchio, M.; Di Candia, S.; Gargantini, L.; Grechi, E.; Iughetti, L.; Mussa, A.; Ragusa, L.; Sacco, M.; Salvatoni, A.; Chiumello, G.; Grugni, G.

doi:10.1016/j.numecd.2009.10.004

« Previous Next »Nutrition, Metabolism & Cardiovascular Diseases
Volume 21, Issue 4 , Pages 269-276, April 2011

Metabolic syndrome in children with Prader–Willi syndrome: The effect of obesity

P. Brambilla
- ASL Milano 2, Milano, Italy
- Corresponding author at: Via Parada 32, 20057 Vedano al Lambro (MI), Italy. Tel.: +39 3392238772; fax: +39 0295158603.
A. Crinò
- Ospedale Bambino Gesù, Research Institute, Rome, Italy
G. Bedogni
- Clinical Epidemiology Unit, Liver Research Center, Trieste, Italy
L. Bosio
- Department of Pediatrics, S. Raffaele Scientific Institute, Vita-Salute S. Raffaele University, Milan, Italy
M. Cappa
- Ospedale Bambino Gesù, Research Institute, Rome, Italy
A. Corrias
- Ospedale Infantile Regina Margherita, Turin, Italy
M. Delvecchio
- Casa Sollievo della Sofferenza, Research Institute, S. Giovanni Rotondo, Italy
S. Di Candia
- Department of Pediatrics, S. Raffaele Scientific Institute, Vita-Salute S. Raffaele University, Milan, Italy
L. Gargantini
- Azienda Ospedaliera Treviglio, Treviglio (BG), Italy
E. Grechi
- Department of Pediatrics, S. Raffaele Scientific Institute, Vita-Salute S. Raffaele University, Milan, Italy
L. Iughetti
- Università di Modena e Reggio Emilia, Modena, Italy
A. Mussa
- Ospedale Infantile Regina Margherita, Turin, Italy
L. Ragusa
- Oasi Maria SS, Research Institute, Troina (EN), Italy
M. Sacco
- Casa Sollievo della Sofferenza, Research Institute, S. Giovanni Rotondo, Italy
A. Salvatoni
- Università dell’Insubria, Varese, Italy
G. Chiumello
- Department of Pediatrics, S. Raffaele Scientific Institute, Vita-Salute S. Raffaele University, Milan, Italy
G. Grugni
- Istituto Auxologico Italiano, Research Institute, Verbania, Italy
- On behalf of the Genetic Obesity Study Group of the Italian Society of Pediatric Endocrinology and Diabetology (ISPED).

Received 6 June 2009; received in revised form 8 October 2009; accepted 11 October 2009. published online 20 January 2010.

Abstract

Background and aims

Prader–Willi syndrome (PWS), the most frequent syndromic obesity, is associated with elevated morbidity and mortality in pediatric and adult ages. In PWS, the presence of metabolic syndrome (MS) has not yet been established. The aim of the study was to estimate the frequency of MS and its components in pediatric subjects according to obesity status.

Methods and results

A cross-sectional study was performed in 109 PWS children aged 2–18years (50 obese and 59 non-obese) and in 96 simple obese controls matched for age, gender, and also for BMI with obese PWS. Obesity was defined when SDS-BMI was >2.

Non-obese PWS showed significantly lower frequency of hypertension (12%) than obese PWS (32%) and obese controls (35%)(p=0.003). The same was observed for low HDL-cholesterol (3% vs 18% and 24%, p=0.001) and high triglycerides (7% vs 23% and 16%, p=0.026). Frequency of altered glucose metabolism was not different among groups (2% vs 10% and 5%), but type 2 diabetes (four cases) was present only in obese PWS. Non-obese PWS showed lower insulin and HOMA-index respect to obese PWS and obese controls (p≤0.017). Overall MS frequency in PWS was 7.3%. None of the non-obese PWS showed MS compared with 16% of obese PWS and controls (p<0.001). When obesity was excluded from the analysis, a significantly lower frequency for clustering of ≥2 factors was still found in non-obese PWS (p=0.035).

Conclusion

Non-obese PWS showed low frequency of MS and its components, while that observed in obese PWS was very close to those of obese controls, suggesting the crucial role of obesity status. Prevention of obesity onset remains the most important goal of PWS treatment. Early identification of MS could be helpful to improve morbidity and mortality in such patients.

Keywords: Prader–Willi syndrome, Metabolic syndrome, Obesity, Hypertension, Lipids, Insulin

Abbreviations: PWS, Prader–Willi syndrome, UPD, uniparental disomy, DM2, type 2 diabetes mellitus, CVD, cardiovascular disease, MS, metabolic syndrome, GH, growth hormone, BMI, body mass index, CDC, Centre for Disease Control and Prevention, BP, blood pressure, HDL-C, high-density lipoprotein cholesterol, HOMA-index, homeostasis model assessment index, IGT, impaired glucose tolerance, IQR, inter-quartile range, SDS, standard deviation score