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儿童普拉德-威利综合征(PWS)的代谢表型:相对于体重指数,胰岛素敏感性增强。

The metabolic phenotype of Prader-Willi syndrome (PWS) in childhood: heightened insulin sensitivity relative to body mass index.

机构信息

Division of Pediatric Endocrinology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.

出版信息

J Clin Endocrinol Metab. 2011 Jan;96(1):E225-32. doi: 10.1210/jc.2010-1733. Epub 2010 Oct 20.

DOI:10.1210/jc.2010-1733
PMID:20962018
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3038476/
Abstract

CONTEXT

Insulin sensitivity is higher in patients with Prader-Willi syndrome (PWS) than in body mass index-matched obese controls (OCs). Factors contributing to the heightened insulin sensitivity of PWS remain obscure. We compared the fasting levels of various hormones, cytokines, lipids, and liver function tests in 14 PWS patients and 14 OCs with those in 14 age- and gender-matched lean children (LC). We hypothesized that metabolic profiles of children with PWS are comparable with those of LC, but different from those of OCs.

RESULTS

Leptin levels were comparable in PWS patients and OCs, suggesting comparable degrees of adiposity. Glucose levels were comparable among groups. However, fasting insulin concentrations and homeostasis model assessment insulin resistance index were lower in PWS patients than in OCs (P < 0.05) and similar to LC. Moreover, high-density lipoprotein levels were lower and triglycerides higher in OCs (P < 0.05) but not PWS patients. Total adiponectin, high-molecular-weight (HMW) adiponectin and the HMW to total adiponectin ratio were higher in PWS patients (P < 0.05) than in OCs and similar to LC. High-sensitivity C-reactive protein and IL-6 levels were higher in OCs than in PWS patients or LC (P < 0.05). Nevertheless, PAI-1 levels were elevated in both OC and PWS patients. There were no group differences in glucagon-like peptide-1, macrophage chemoattractant protein-1, TNFα, IL-2, IL-8, IL-10, IL-12p40, IL-18, resistin, total or low-density lipoprotein cholesterol, aspartate aminotransferase, or alanine aminotransferase.

CONCLUSIONS

The heightened insulin sensitivity of PWS patients relative to OCs is associated with higher levels of adiponectin and lower levels of high-sensitivity C-reactive protein and IL-6. Future studies will determine whether PWS children are protected from obesity comorbidities such as type 2 diabetes, hyperlipidemia, and nonalcoholic fatty liver disease.

摘要

背景

与体重指数匹配的肥胖对照组(OC)相比,普拉德-威利综合征(PWS)患者的胰岛素敏感性更高。导致 PWS 患者胰岛素敏感性升高的因素仍不清楚。我们比较了 14 名 PWS 患者和 14 名 OCs 与 14 名年龄和性别匹配的瘦儿童(LC)的空腹激素、细胞因子、脂质和肝功能检查水平。我们假设 PWS 患儿的代谢谱与 LC 相似,但与 OC 不同。

结果

PWS 患者和 OC 的瘦素水平相当,表明肥胖程度相当。各组血糖水平相当。然而,PWS 患者的空腹胰岛素浓度和稳态模型评估胰岛素抵抗指数低于 OC(P < 0.05),与 LC 相似。此外,OC 的高密度脂蛋白水平较低,甘油三酯水平较高(P < 0.05),但 PWS 患者则不然。PWS 患者的总脂联素、高分子量(HMW)脂联素和 HMW 与总脂联素的比值较高(P < 0.05),与 OC 相似,与 LC 相似。OC 的高敏 C 反应蛋白和 IL-6 水平高于 PWS 患者或 LC(P < 0.05)。然而,PAI-1 水平在 OC 和 PWS 患者中均升高。各组间胰高血糖素样肽-1、巨噬细胞趋化蛋白-1、TNFα、IL-2、IL-8、IL-10、IL-12p40、IL-18、抵抗素、总胆固醇或低密度脂蛋白胆固醇、天冬氨酸氨基转移酶或丙氨酸氨基转移酶无差异。

结论

与 OC 相比,PWS 患者的胰岛素敏感性升高与脂联素水平升高和高敏 C 反应蛋白及 IL-6 水平降低有关。未来的研究将确定 PWS 儿童是否能免受肥胖相关并发症的影响,如 2 型糖尿病、高脂血症和非酒精性脂肪肝疾病。

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