The Metabolic Phenotype of Prader-Willi Syndrome (PWS) in Childhood: Heightened Insulin Sensitivity Relative to Body Mass Index
The Metabolic Phenotype of Prader-Willi Syndrome (PWS) in Childhood: Heightened Insulin Sensitivity Relative to Body Mass Index
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DOI：
10.1210/jc.2010-1733
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发表时间：
2011-01-01
影响因子：
5.8
通讯作者：
Freemark, Michael
中科院分区：
文献类型：
--
作者：
Haqq, Andrea M.;Muehlbauer, Michael J.;Freemark, Michael
背景：Prader-Willi综合征（PWS）患者的胰岛素敏感性高于体重指数匹配的肥胖对照（OCs）。导致PWS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OC与14名年龄和性别匹配的瘦儿童（LC）的各种激素，细胞因子，脂质和肝功能检查的空腹水平。我们假设PWS儿童的代谢特征与LC相当，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。各组的葡萄糖水平相当。PWS组空腹胰岛素水平和稳态模型胰岛素抵抗指数均低于OC组（P < 0.05），与LC组相似。OCs组高密度脂蛋白水平较低，甘油三酯水平较高（P < 0.05），而PWS组无显著差异。PWS患者的总脂联素、高分子量脂联素及高分子量脂联素/总脂联素比值均高于OC（P < 0.05），与LC相似。OC组超敏C反应蛋白和IL-6水平高于PWS组和LC组（P < 0.05）。尽管如此，派-1水平在OC和PWS患者中均升高。胰高血糖素样肽-1、巨噬细胞趋化蛋白-1、TNF α、IL-2、IL-8、IL-10、IL-12 p40、IL-18、β-谷氨酰胺、总胆固醇或低密度脂蛋白胆固醇、天冬氨酸转氨酶或丙氨酸转氨酶无组间差异。PWS患者相对于OC的胰岛素敏感性升高与脂联素水平升高和高敏C-肽水平降低相关。反应蛋白和IL-6。未来的研究将确定PWS儿童是否可以避免肥胖合并症，如2型糖尿病，高脂血症和非酒精性脂肪肝。（临床内分泌代谢杂志96：E225-E232，2011）
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, TNF alpha, 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. (J Clin Endocrinol Metab 96: E225-E232, 2011)