Geggel R L, Carvalho A C, Hoyer L W, Reid L M
Am Rev Respir Dis. 1987 Feb;135(2):294-9. doi: 10.1164/arrd.1987.135.2.294.
In primary pulmonary hypertension of recent clinical onset, pulmonary endothelial cells show injury. To characterize this phenomenon, we measured plasma von Willebrand factor (vWF) by immunologic and ristocetin cofactor assays in 6 patients with primary pulmonary hypertension, 17 patients with secondary pulmonary artery hypertension associated with congenital heart disease or cystic fibrosis, and 13 patients with congenital heart disease and normal pulmonary artery pressure. In selected cases, we also determined the vWF multimer pattern. In all 6 cases of primary pulmonary hypertension, the ristocetin cofactor activity was increased relative to the vWF antigen (vWF:Ag) concentration (a ratio of 2.55 +/- 0.36; normal range, 0.8 to 1.4); 4 of the 6 also had a similar and abnormal vWF multimer pattern--an increased proportion of the fastest moving bands. In the other 2, the multimer pattern was normal. Of the other 30 patients, a mild increase in ristocetin cofactor/vWF:Ag was seen in only 2 with secondary pulmonary hypertension and 1 with normal pulmonary artery pressure: these also had an abnormal vWF multimer pattern that was different from that observed in patients with primary pulmonary hypertension. The vWF abnormalities we describe in primary pulmonary hypertension offer a marker of the disease and could be helpful in understanding its pathogenesis.
在近期起病的原发性肺动脉高压中，肺内皮细胞显示出损伤。为了描述这一现象，我们通过免疫测定法和瑞斯托霉素辅因子测定法，对6例原发性肺动脉高压患者、17例与先天性心脏病或囊性纤维化相关的继发性肺动脉高压患者以及13例先天性心脏病且肺动脉压正常的患者进行了血浆血管性血友病因子（vWF）的检测。在选定的病例中，我们还确定了vWF多聚体模式。在所有6例原发性肺动脉高压病例中，瑞斯托霉素辅因子活性相对于vWF抗原（vWF:Ag）浓度升高（比值为2.55±0.36；正常范围为0.8至1.4）；6例中有4例还具有相似且异常的vWF多聚体模式——移动最快的条带比例增加。另外2例的多聚体模式正常。在其他30例患者中，仅2例继发性肺动脉高压患者和1例肺动脉压正常的患者出现瑞斯托霉素辅因子/vWF:Ag轻度升高：这些患者也具有异常的vWF多聚体模式，与原发性肺动脉高压患者中观察到的不同。我们在原发性肺动脉高压中描述的vWF异常为该疾病提供了一个标志物，可能有助于理解其发病机制。
