Lopes A A, Maeda N Y, Bydlowski S P
Instituto do Coração, Hospital das Clínicas da FMUSP e Fundação Pró-Sangue Hemocentro de São Paulo.
Arq Bras Cardiol. 1998 Mar;70(3):141-5. doi: 10.1590/s0066-782x1998000300001.
To analyze quantitative and structural changes in circulating von Willebrand factor (vWF) in 40 precapillary pulmonary hypertensive patients, as an attempt to identify possible correlations between endothelial cell dysfunction and patient short-term (one year) survival.
Plasma antigenic activity of vWF (vWF:Ag) was analyzed by immunoelectrophoresis. The relative concentration of vWF low molecular weight multimers (LMWM%) and the composition of vWF subunit were determined by densitometric analysis of Western blots.
vWF:Ag was importantly increased in patients in comparison with normals (p < 0.001). Patients also had increased LMWM% (p < 0.001) and increased degradation of vWF main subunit (p < 0.05). At the beginning of the study, nonsurvivors (N = 11) had higher vWF:Ag (p < 0.001) and LMWM% (p < 0.005) values in comparison with survivors. LMWM% was selected by logistic regression analysis as a predictor of death during the first year of follow-up (p < 0.05).
Marked changes in circulating vWF likely reflect extensive pulmonary vascular endothelial cell dysfunction and are associated with poor short-term prognosis in pulmonary hypertension.
分析40例毛细血管前性肺动脉高压患者循环血管性血友病因子（vWF）的定量和结构变化，以试图确定内皮细胞功能障碍与患者短期（一年）生存率之间的可能相关性。
采用免疫电泳法分析vWF的血浆抗原活性（vWF:Ag）。通过对蛋白质印迹进行光密度分析来测定vWF低分子量多聚体的相对浓度（LMWM%）和vWF亚基的组成。
与正常对照相比，患者的vWF:Ag显著升高（p < 0.001）。患者的LMWM%也升高（p < 0.001），且vWF主要亚基的降解增加（p < 0.05）。在研究开始时，与存活者相比，非存活者（N = 11）的vWF:Ag（p < 0.001）和LMWM%（p < 0.005）值更高。通过逻辑回归分析，LMWM%被选为随访第一年死亡的预测指标（p < 0.05）。
循环vWF的显著变化可能反映了广泛的肺血管内皮细胞功能障碍，并与肺动脉高压患者的短期预后不良相关。
