Abstract

Prader–Willi syndrome (PWS) is the most common genetic inherited obesity syndrome. Obesity-related complications, mostly related to chronic low-grade systemic inflammation (LGI), are the commonest cause of mortality and morbidity in PWS adults. Phase angle (PhA) is an easy tool to screen a state of LGI in healthy subjects and in subjects with obesity and is obtained from bioelectrical impedance analysis (BIA). The aim of this study was to validate the PhA in PWS adults as a potential biomarker of LGI. In this single-center, cross-sectional study, fifteen PWS adults (six males, aged 19–41 years, and body mass index (BMI) 31.0–68.0 Kg/m2) and fifteen control subjects matched by gender, age, and BMI were evaluated. PhA values were significantly lower (p < 0.001), while high-sensitivity C-reactive protein (hs-CRP) levels were significantly higher (p < 0.001) in PWS adults compared with controls (p < 0.001), without a gender difference in the latter. After adjustment for gender, BMI, and waist circumference, significant correlation was found between PhA and hs-CRP levels (r = −0.69, p = 0.01). At the ROC analysis, the threshold value of PhA predicting the highest hs-CRP levels above the median value was found at PhA ≤ 4.8° (p = 0.01; AUC, 0.82; standard error, 0.12; 95% CI, 0.58 to 1.00). These results suggest that PWS adults had a significant higher degree of LGI compared with their counterparts. Moreover, our finding suggest that PhA is a valid biomarker of LGI also in PWS adults.

Highlights

  • Prader–Willi syndrome (PWS) is the most common genetic inherited obesity syndrome, due to an imprinting disorder resulting from the loss of paternally derived alleles located on 15q11–q13.PWS prevalence is approximately one in 10,000 to 30,000 births without gender-related differences in incidence [1,2]

  • Ratio were found in PWS adults compared with controls (p < 0.001), while lower values of Xc, and Phase angle (PhA), were detected in PWS adults compared with control group

  • Significant higher values of extracellular water (ECW)/intracellular water (ICW) ratio were found in PWS adults compared with controls (p < 0.001), while lower values of Xc, and PhA, were found in PWS adults compared with control group

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Summary

Introduction

Prader–Willi syndrome (PWS) is the most common genetic inherited obesity syndrome, due to an imprinting disorder resulting from the loss of paternally derived alleles located on 15q11–q13.PWS prevalence is approximately one in 10,000 to 30,000 births without gender-related differences in incidence [1,2]. The PWS subjects presented at birth hypotonia, feeding difficulties, and retarded psychomotor development, with obsessive-compulsive characteristics and hyperphagia starting in early childhood These patients develop during childhood a severe obesity, which is combined with. Very recently, Pacoricona Alfaro DL et al assessed the causes of death (including cardiovascular, severe infection, respiratory, gastrointestinal, sudden death, and other causes) in French patients with PWS (both children and adults) from 2004 to 2014. Over these 11 years of study, the death toll was 104 patients with a median age at death of 30 years. More than 50% of the deaths in PWS patients (both children and adults) were due to respiratory causes [4]

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