普拉德-威利综合征成人患者的身体成分、脂肪因子、骨矿物质密度和骨重塑标志物与胰岛素样生长因子-1水平的关系
Body composition, adipokines, bone mineral density and bone remodeling markers in relation to IGF-1 levels in adults with Prader-Willi syndrome.
作者信息
van Nieuwpoort I Caroline, Twisk Jos W R, Curfs Leopold M G, Lips Paul, Drent Madeleine L
机构信息
1Department of Internal Medicine, Section Endocrinology, VU University Medical Center and Amsterdam Neuroscience, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
2Department of Clinical Epidemiology and Biostatistics and EMGO Institute, VU University Medical Center and Institute of Health Sciences, VU University, Amsterdam, The Netherlands.
出版信息
Int J Pediatr Endocrinol. 2018;2018:1. doi: 10.1186/s13633-018-0055-4. Epub 2018 Jan 16.
BACKGROUND
In patients with Prader-Willi syndrome (PWS) body composition is abnormal and alterations in appetite regulating factors, bone mineral density and insulin-like growth factor-1 (IGF-1) levels have been described. Studies in PWS adults are limited. In this study, we investigated body composition, appetite regulating peptides, bone mineral density and markers of bone remodeling in an adult PWS population. Furthermore, we investigated the association between these different parameters and IGF-1 levels because of the described similarities with growth hormone deficient patients.
METHODS
In this cross-sectional observational cohort study in a university hospital setting we studied fifteen adult PWS patients. Anthropometric and metabolic parameters, IGF-1 levels, bone mineral density and bone metabolism were evaluated. The homeostasis model assessment of insulin resistance (HOMA2-IR) was calculated. Fourteen healthy siblings served as a control group for part of the measurements.
RESULTS
In the adult PWS patients, height, fat free mass, IGF-1 and bone mineral content were significantly lower when compared to controls; body mass index (BMI), waist, waist-to-hip ratio and fat mass were higher. There was a high prevalence of osteopenia and osteoporosis in the PWS patients. Also, appetite regulating peptides and bone remodelling markers were aberrant when compared to reference values. Measurements of body composition were significantly correlated to appetite regulating peptides and high-sensitive C-reactive protein (hs-CRP), furthermore HOMA was correlated to BMI and adipokines.
CONCLUSION
In adults with Prader-Willi syndrome alterations in body composition, adipokines, hs-CRP and bone mineral density were demonstrated but these were not associated with IGF-1 levels. Further investigations are warranted to gain more insight into the exact pathophysiology and the role of these alterations in the metabolic and cardiovascular complications seen in PWS, so these complications can be prevented or treated as early as possible.
背景
普拉德-威利综合征（PWS）患者的身体成分异常，且已发现食欲调节因子、骨矿物质密度和胰岛素样生长因子-1（IGF-1）水平存在改变。针对PWS成年患者的研究有限。在本研究中，我们调查了成年PWS患者群体的身体成分、食欲调节肽、骨矿物质密度和骨重塑标志物。此外，鉴于PWS患者与生长激素缺乏患者存在相似之处，我们还研究了这些不同参数与IGF-1水平之间的关联。
方法
在一所大学医院进行的这项横断面观察性队列研究中，我们对15名成年PWS患者进行了研究。评估了人体测量和代谢参数、IGF-1水平、骨矿物质密度和骨代谢情况。计算了胰岛素抵抗的稳态模型评估值（HOMA2-IR）。14名健康的兄弟姐妹作为部分测量的对照组。
结果
与对照组相比，成年PWS患者的身高、去脂体重、IGF-1和骨矿物质含量显著降低；体重指数（BMI）、腰围、腰臀比和脂肪量则更高。PWS患者中骨质疏松症和骨质减少的患病率很高。此外，与参考值相比，食欲调节肽和骨重塑标志物也存在异常。身体成分测量值与食欲调节肽和高敏C反应蛋白（hs-CRP）显著相关，此外，HOMA与BMI和脂肪因子相关。
结论
在患有普拉德-威利综合征的成年人中，已证实其身体成分、脂肪因子、hs-CRP和骨矿物质密度存在改变，但这些改变与IGF-1水平无关。有必要进行进一步研究，以更深入了解PWS患者代谢和心血管并发症的确切病理生理学以及这些改变所起的作用，从而尽早预防或治疗这些并发症。
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