Fukui H, Takase T, Ikari H, Murakami Y, Okubo Y, Nakamura K
Br J Haematol. 1979 Aug;42(4):637-46. doi: 10.1111/j.1365-2141.1979.tb01176.x.
The mean level of factor VIII procoagulant acitivity (VIII:C) and factor VIII related antigen (VIIIR:AG) was normal in 100 newborn cord plasmas, whereas that of von Willebrand factor (VIIIR:WF) activity was slightly lower than normal. On crossed immunoelectrophoresis, 20 of 50 newborn infants had an increased anodal mobility of VIIIR:AG. When the cord plasma showing an abnormal electrophoretic pattern was mixed with normal plasma, two precipitation peaks with a broad base were found. Similar mixing experiments with the abnormal cord plasma and plasma from a patient with atypical von Willebrand's disease did not normalize the electrophoretic mobility of VIIIR:AG. Gel filtration of the cord plasma with an abnormal electrophoretic pattern of VIII:AG, showed that the three activities were all detected at the position corresponding to a molecular weight of about 800 000. The results suggest the presence of qualitative abnormalities of the factor VIII molecule in half of full-term newborn cord plasma.
100份新生儿脐带血浆中,凝血因子VIII促凝活性(VIII:C)和因子VIII相关抗原(VIIIR:AG)的平均水平正常,而血管性血友病因子(VIIIR:WF)活性略低于正常水平。在交叉免疫电泳中,50名新生儿中有20名的VIIIR:AG阳极迁移率增加。当显示异常电泳图谱的脐带血浆与正常血浆混合时,发现了两个宽底沉淀峰。将异常脐带血浆与非典型血管性血友病患者的血浆进行类似的混合实验,并未使VIIIR:AG的电泳迁移率恢复正常。对VIII:AG电泳图谱异常的脐带血浆进行凝胶过滤,结果显示三种活性均在对应于分子量约800000的位置被检测到。结果表明,足月新生儿脐带血浆中有一半存在因子VIII分子的质量异常。