Montali Anna, Truglio Gessica, Martino Francesco, Ceci Fabrizio, Ferraguti Giampiero, Ciociola Ester, Maranghi Marianna, Gianfagna Francesco, Iacoviello Licia, Strom Roberto, Lucarelli Marco, Arca Marcello
Department of Internal Medicine and Allied Sciences, Atherosclerosis Unit, Sapienza University of Rome, Rome, Italy.
Department of Internal Medicine and Allied Sciences, Atherosclerosis Unit, Sapienza University of Rome, Rome, Italy; Department of Cellular Biotechnologies and Hematology, Sapienza University of Rome, Rome, Italy.
PLoS One. 2015 Apr 21;10(4):e0120099. doi: 10.1371/journal.pone.0120099. eCollection 2015.
The precursors of atherogenic dyslipidemia (AD) are not well defined. Therefore, we investigated 62 non-obese, non-diabetic AD and 221 normolipemic children. Anthropometric parameters, blood pressure and biochemical measures were obtained in index children, their parents and all available siblings. The heritability (h(2)) of anthropometric and biochemical traits was estimated by SOLAR. Rare and common variants in APOA1 and LPL genes were screened by re-sequencing. Compared to normolipemic, AD children showed increased body mass index, waist circumference, plasma glucose, insulin, ApoB, HOMA-IR, hs-CRP and lower adiponectin (p<0.001 for all). Metabolic syndrome was present in 40% of AD while absent in controls. All traits (except adiponectin and hs-CRP) showed a strong familial aggregation, with plasma glucose having the highest heritability (89%). Overall, 4 LPL loss-of-function mutations were detected (p.Asp9Asn, p.Ser45Asn, p.Asn291Ser, p.Leu365Val) and their cumulative prevalence was higher in AD than in control children (0.073 vs. 0.026; P=0.038). The LPL p.S447* gain-of-function mutation, resulted to be less frequent in AD than in control children (0.064 vs. 0.126; P=0.082). No variant in the APOA1 gene was found. Our data indicate that AD is a rather common dyslipidemia in childhood; it associates with metabolic abnormalities typical of insulin resistant state and shows a strong familial aggregation. LPL variants may contribute to the development of AD phenotype.
动脉粥样硬化性血脂异常(AD)的前驱症状尚未明确界定。因此,我们对62名非肥胖、非糖尿病的AD儿童和221名血脂正常的儿童进行了研究。在受试儿童及其父母和所有在世的兄弟姐妹中测量了人体测量参数、血压和生化指标。通过SOLAR估计人体测量和生化特征的遗传率(h(2))。通过重测序筛选APOA1和LPL基因中的罕见和常见变异。与血脂正常的儿童相比,AD儿童的体重指数、腰围、血糖、胰岛素、载脂蛋白B、胰岛素抵抗指数(HOMA-IR)、高敏C反应蛋白(hs-CRP)升高,脂联素降低(所有指标p<0.001)。40%的AD儿童存在代谢综合征,而对照组儿童无此情况。所有特征(脂联素和hs-CRP除外)均显示出强烈的家族聚集性,血糖的遗传率最高(89%)。总体而言,检测到4个LPL功能丧失突变(p.Asp9Asn、p.Ser45Asn、p.Asn291Ser、p.Leu365Val),其在AD儿童中的累积患病率高于对照组儿童(0.073对0.026;P=0.038)。LPL p.S447*功能获得性突变在AD儿童中的发生率低于对照组儿童(0.064对0.126;P=0.082)。未在APOA1基因中发现变异。我们的数据表明,AD是儿童期一种相当常见的血脂异常;它与胰岛素抵抗状态典型的代谢异常相关,并显示出强烈的家族聚集性。LPL变异可能有助于AD表型的发展。