Serum Lipoprotein(a) and High-Sensitivity C-reactive Protein Correlate With Somatic Parameters Including MLPA Subgroups in Children With Prader-Willi Syndrome.

CONTEXT

Prader-Willi syndrome (PWS) is 1 of the most common monochromosomal (15q11-13) causes of polygenic syndromic childhood obesity.

OBJECTIVES

We primarily compare and correlate serum lipoprotein(a) [Lp(a)], high-sensitivity C-reactive protein (hs-CRP), and baseline clinical characteristics of genetically confirmed children with PWS at their GH treatment-naïve stage to their control groups. Secondary objectives were to correlate serum Lp(a) and hs-CRP concentration to multiplex ligation-dependent probe amplification subgroups, body composition indices, sleep apnea parameters, and hepatic shear-stress by 2-dimensional shear wave elastography in children with PWS.

METHODS

A total of 32 genetically confirmed PWS children (age 5 to 18 years), 20 simple obesity children, and 20 healthy children as age-matched control groups were studied for the primary and secondary study objectives.

RESULTS

Lp(a) was higher in the study group as compared to the control group (   .0001), but we found no difference between the control groups ( = .9680).In addition, no correlation was detected in Lp(a) levels in the study population with respect to their body weight, body mass index, and waist circumference. hs-CRP levels were also higher in the study population compared to both control groups ( = .0962; < .0001); in contrast, Lp(a) differed significantly between the control groups ( = .002). Lower fat-free mass index (FFMI) correlated with higher levels of serum Lp(a) ( = -0.5525; = .001), whereas FFMI was not correlated with hs-CRP levels in PWS children ( = .657). Based on genomic subtypes, patients with PWS were divided into deletion and nondeletion genetic subgroups. We found significantly altered levels of Lp(a), hs-CRP, fat-free mass, and sleep apnea parameters, particularly in the deletion subgroup.

CONCLUSION

Serum Lp(a) as well as hs-CRP stand out to be the core independent risk factors along with their strong correlation with the other study parameters, which necessitates the role of future targeted therapeutics in PWS, especially in deletion pathology. Thus, genetic subtyping during diagnostic confirmation endorses further prognostic elaboration.