Division of Endocrinology, Department of Translational Medicine, University of Piemonte Orientale, Novara, Italy.
Division of General Medicine, Istituto Auxologico Italiano, IRCCS, San Giuseppe Hospital, Piancavallo, Verbania, Italy.
J Endocrinol Invest. 2021 Jul;44(7):1447-1456. doi: 10.1007/s40618-020-01444-w. Epub 2020 Oct 16.
Angiopoietin-like 8 (ANGPTL8) is a liver- and adipose tissue-produced protein that predicts non-alcoholic fatty liver disease (NAFLD) and altered metabolic homeostasis in the general population as well as in persons with common and genetic obesity, including the Prader-Willi syndrome (PWS). However, its metabolic correlate in paediatric patients with respect to PWS is unknown.
This cross-sectional study investigated circulating ANGPTL8 and adipocytokines levels in 28 PWS and 28 age-, sex- and BMI-matched children and adolescents (age, 7.0-17.8y) in relation to NAFLD and metabolic homeostasis assessed by OGTT, paediatric metabolic index (PMI) and fatty liver index (FLI), liver ultrasonography (US), as well as dual-energy X-ray absorptiometry (DEXA) for analysis of fat (FM) and fat-free mass (FFM).
At the set level of significance, PWS children showed lower values of FFM (p < 0.01) but healthier insulin profiles (p < 0.01) and PMI values (p < 0.05) than matched controls. By US, the prevalence of NAFLD was similar between groups but less severe in PWS than controls. Analysis of ANGPTL8 levels showed no difference between groups, yet only in PWS ANGPTL8 levels were associated with ALT levels, FLI values and NAFLD. In stepwise multivariable regression analysis on merged data, ANGPTL8 levels were independently predicted by BMI SDS, leptin levels and NAFLD.
ANGPTL8 levels are similar in PWS and controls and, overall, they are directly associated with the presence and severity of NAFLD in patients with PWS.
血管生成素样蛋白 8(ANGPTL8)是一种由肝脏和脂肪组织产生的蛋白质,它可以预测非酒精性脂肪性肝病(NAFLD)以及普通人群和具有常见和遗传性肥胖症(包括普拉德-威利综合征(PWS))人群的代谢平衡改变。然而,其在患有 PWS 的儿科患者中的代谢相关性尚不清楚。
本横断面研究调查了 28 名 PWS 患者和 28 名年龄、性别和 BMI 匹配的儿童和青少年(年龄 7.0-17.8 岁)的循环 ANGPTL8 和脂肪细胞因子水平,与 NAFLD 和代谢平衡有关,通过 OGTT、儿科代谢指数(PMI)和脂肪肝指数(FLI)、肝脏超声(US)以及双能 X 射线吸收法(DEXA)进行评估,用于分析脂肪(FM)和无脂肪质量(FFM)。
在设定的显著性水平上,PWS 儿童的 FFM 值较低(p<0.01),但胰岛素谱(p<0.01)和 PMI 值(p<0.05)更健康。通过 US,两组之间的 NAFLD 患病率相似,但 PWS 组比对照组更轻。ANGPTL8 水平分析显示两组之间无差异,但仅在 PWS 中 ANGPTL8 水平与 ALT 水平、FLI 值和 NAFLD 相关。在合并数据的逐步多元回归分析中,ANGPTL8 水平独立于 BMI SDS、瘦素水平和 NAFLD 预测。
PWS 患者和对照组的 ANGPTL8 水平相似,总体上与 PWS 患者 NAFLD 的存在和严重程度直接相关。