特纳综合征患者血清转化生长因子β的变化

Perturbation of the transforming growth factor β system in Turner syndrome

目的:观察转化生长因子β(transforming growth factor-beta,TGFβ)在特纳综合征(Turner syndrome,TS)患者血清中的变化及其与内分泌和心血管参数的关系。方法:选择TS患者和健康志愿者妇女(对照组)各40例,采用酶联免疫方法测定血清中的TGFβ1、TGFβ2及转化生长因子受体(endoglin)。所有TS患者均行心血管系统磁共振和超声检查。结果:TS患者血清中TGFβ1和endoglin水平均高于对照组(P<0.000 1),而TGFβ2的水平则低于对照组(P<0.000 1)。TGFβ的改变与年龄、血压、血小板、甲状腺功能、体重指数或心血管疾病的分型无关。结论:TS患者血清中TGFβ显著性改变对疾病的研究具有重要意义。

Objective： To measure components of the circulating transforming growth factor β（TGFβ） system in patients with Turner syndrome（TS） compared to relevant controls and to ascertain correlation with endocrine and cardiovascular parameters.Methods： TGFβ1,TGFβ2 and endoglin（a vascular TGF receptor component） were measured using enzyme-linked immunoassays in the sera of 40 subjects with TS and 40 healthy volunteer women.The cardiovascular phenotype in TS subjects was extensively characterized by cardiac magnetic resonance（MR） and echo.Results： TGFβ1 levels were about 3-fold higher in TS while TGFβ2 levels were about 3.5-fold higher in controls（P 0.000 1 for both）.Soluble endoglin levels were 25% higher in TS（P 0.000 1）.Variation in TGFβ system components was not explained by age,blood pressure,platelet count,thyroid function,body proportions or cardiovascular phenotype.Conclusion： There is profound perturbation of the TGFβ system evident in the circulation of individuals with TS.