Cardin F, Taylor L, Hutton R, McIntyre N, Kernoff P, Burroughs A K
Hepatobiliary and Liver Transplantation Unit, Royal Free Hospital, London, UK.
Blood Coagul Fibrinolysis. 1991 Apr;2(2):267-71. doi: 10.1097/00001721-199104000-00008.
Qualitative abnormalities in von Willebrand Factor (vWF) in patients with cirrhosis have been little studied with contrasting results. We used crossed immunoelectrophoresis (2-DIE) and multimeric analysis of vWF in eight patients with stable hepatic cirrhosis to evaluate abnormalities in vWF before and 1 h following intravenous administration of three doses of desmopressin acetate (0.3 micrograms/kg) given at baseline, 4 and 24 h. We thought that qualitative abnormalities might be more easily detected following desmopressin as this is known to release vWF from storage sites. There was an increased electrophoretic mobility on 2-DIE in all patients with no change following desmopressin. The multimeric analysis did not show an increase in lower molecular weight multimers, but showed a statistically significant increase in higher molecular weight multimers following desmopressin (P less than 0.02). These results suggest that the vWF of cirrhotics has an abnormal charge (not altered by release following desmopressin) which would explain the increased electrophoretic mobility on 2-DIE with a normal pattern of lower molecular weight multimers using multimeric analysis.
肝硬化患者血管性血友病因子(vWF)的定性异常鲜有研究,结果也相互矛盾。我们对8例稳定期肝硬化患者进行了vWF的交叉免疫电泳(二维免疫电泳,2-DIE)和多聚体分析,以评估在基线、4小时和24小时静脉注射三剂醋酸去氨加压素(0.3微克/千克)之前及之后1小时vWF的异常情况。我们认为,在去氨加压素之后可能更容易检测到定性异常,因为已知它能从储存部位释放vWF。所有患者二维免疫电泳的电泳迁移率均增加,去氨加压素后无变化。多聚体分析未显示低分子量多聚体增加,但显示去氨加压素后高分子量多聚体有统计学意义的增加(P<0.02)。这些结果表明,肝硬化患者的vWF电荷异常(去氨加压素释放后未改变),这可以解释二维免疫电泳中电泳迁移率增加,而多聚体分析显示低分子量多聚体模式正常。