von Willebrand factor abnormalities in primary pulmonary hypertension.
von Willebrand factor abnormalities in primary pulmonary hypertension.
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DOI：
10.1164/arrd.1987.135.2.294
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发表时间：
1987-02
期刊：
影响因子：
--
通讯作者：
R. Geggel;A. Carvalho;L. Hoyer;L. Reid
中科院分区：
文献类型：
--
作者：
R. Geggel;A. Carvalho;L. Hoyer;L. Reid
在新近临床发作的原发性肺动脉高压中，肺内皮细胞表现出损伤。为了描述这一现象，我们测定血浆血管性血友病因子（vWF）的免疫和维生素C辅因子测定6例原发性肺动脉高压，17例继发性肺动脉高压与先天性心脏病或囊性纤维化，和13例先天性心脏病和正常肺动脉压。在选定的情况下，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患者中，Ristoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastoclastocl另2例多聚体模式正常。在其他30例患者中，仅2例继发性肺动脉高压患者和1例肺动脉压正常患者的vWF：Ag轻度升高：这些患者也具有与原发性肺动脉高压患者不同的异常vWF多聚体模式。我们所描述的原发性肺动脉高压的vWF异常提供了一个疾病的标志物，可能有助于了解其发病机制。
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.