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Metabolic syndrome in children with Prader-Willi syndrome: the effect of obesity
P Brambilla1, A Crinò, G Bedogni
1ASL Milano 2, Milano, Italy. paolo.brambilla3@tin.it
Insights
Metabolic syndrome (MS) is uncommon in non-obese Prader-Willi syndrome (PWS) patients. Obese PWS individuals show MS rates similar to obese controls, highlighting obesity
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Genetics
Background:
- Prader-Willi syndrome (PWS) is the most common genetic cause of obesity, associated with significant health risks.
- The prevalence and characteristics of metabolic syndrome (MS) in pediatric PWS populations are not well-established.
- Understanding MS in PWS is crucial for managing associated morbidities.
Purpose of the Study:
- To determine the frequency of metabolic syndrome (MS) and its individual components in pediatric patients with Prader-Willi syndrome (PWS).
- To compare the prevalence of MS and its components between obese and non-obese PWS children.
- To evaluate the impact of obesity status on MS development in PWS.
Main Methods:
- A cross-sectional study involving 109 children with PWS (aged 2-18 years), categorized as obese or non-obese.
- Comparison group included 96 simple obese children matched for age, gender, and BMI.
- Assessment included hypertension, HDL-cholesterol, triglycerides, glucose metabolism, insulin, and HOMA-index.
Main Results:
- Non-obese PWS children had significantly lower rates of hypertension, low HDL-cholesterol, and high triglycerides compared to obese PWS children and obese controls.
- While altered glucose metabolism was similar across groups, type 2 diabetes occurred only in obese PWS patients.
- Overall MS prevalence was 7.3% in PWS, with none in non-obese PWS versus 16% in obese PWS and controls.
Conclusions:
- Obesity status is a critical determinant of metabolic syndrome (MS) in Prader-Willi syndrome (PWS).
- Non-obese PWS patients exhibit a low frequency of MS and its components.
- Obese PWS patients have MS prevalence comparable to obese controls, emphasizing obesity prevention and early MS detection in PWS management.
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 2-18 years (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.
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