Butler Merlin G, Bittel Douglas C, Kibiryeva Nataliya, Garg Uttam
Section of Medical Genetics and Molecular Medicine, Children's Mercy Hospitals and Clinics and University of Missouri- Kansas City School of Medicine, Kansas City, MO 64108, USA.
Genet Med. 2006 Apr;8(4):243-8. doi: 10.1097/01.gim.0000204469.30913.67.
Prader-Willi syndrome is characterized by infantile hypotonia, feeding difficulties, hypogonadism, small hands and feet, mental deficiency, behavioral problems, and hyperphagia leading to obesity in early childhood. To date there have been no studies examining the associated risk of cardiovascular disease related to obesity in Prader-Willi syndrome, nor of circulating biomarkers such as C-reactive protein known to be predictive of cardiovascular disease. Therefore, we have measured the levels of C-reactive protein in a descriptive study of a cohort of Prader-Willi syndrome and comparison subjects.
An immunoassay was used to quantify C-reactive protein in plasma samples from subjects with Prader-Willi syndrome and obesity and compared to anthropometric and body composition data.
The mean circulating C-reactive protein concentration for 28 subjects with Prader-Willi syndrome (13 females, 15 males; mean age 24.6 +/- 11.6 years; mean body mass index 35.9 +/- 11.9) was 10.3 +/- 8.8 mg/L. The mean C-reactive protein concentration for 22 nonsyndromic obese subjects (16 females, 6 males; mean age 32.3 +/- 12.2 years; mean body mass index 36.6 +/- 10.7) was 8.8 +/- 10.9 mg/L. The reported mean value for C-reactive protein was 2.6 +/- 3.0 mg/L from 100 healthy adults.
The mean C-reactive protein values were similar between the subjects with Prader-Willi syndrome and obesity but significantly higher in Prader-Willi syndrome and obese subjects relative to normative data. Increased levels of C-reactive protein (>3.0 mg/L) are associated with cardiovascular disease suggesting subjects with Prader-Willi syndrome as well as obese subjects are at a similar increased risk.
普拉德-威利综合征的特征包括婴儿期肌张力减退、喂养困难、性腺功能减退、手足短小、智力缺陷、行为问题以及幼儿期因食欲亢进导致肥胖。迄今为止，尚无研究探讨普拉德-威利综合征中与肥胖相关的心血管疾病风险，也没有研究涉及已知可预测心血管疾病的循环生物标志物，如C反应蛋白。因此，我们在一项对普拉德-威利综合征队列及对照受试者的描述性研究中测量了C反应蛋白水平。
采用免疫测定法对普拉德-威利综合征肥胖受试者的血浆样本中的C反应蛋白进行定量，并与人体测量和身体成分数据进行比较。
28例普拉德-威利综合征受试者（13名女性，15名男性；平均年龄24.6±11.6岁；平均体重指数35.9±11.9）的循环C反应蛋白平均浓度为10.3±8.8mg/L。22例非综合征性肥胖受试者（16名女性，6名男性；平均年龄32.3±12.2岁；平均体重指数36.6±10.7）的C反应蛋白平均浓度为8.8±10.9mg/L。100名健康成年人的C反应蛋白报告平均值为2.6±3.0mg/L。
普拉德-威利综合征肥胖受试者的C反应蛋白平均值相似，但相对于正常数据，普拉德-威利综合征肥胖受试者的C反应蛋白水平显著更高。C反应蛋白水平升高（>3.0mg/L）与心血管疾病相关，这表明普拉德-威利综合征受试者以及肥胖受试者具有相似的心血管疾病风险增加情况。
