Neuenschwander S, Pliska V
Department of Animal Science, ETH-Swiss Federal Institute of Technology, Zürich.
Haemostasis. 1994 Jan-Feb;24(1):27-35. doi: 10.1159/000217077.
The sheep haemophilia A, recently described in a flock of White Alpine sheep, is probably caused by the production of a less potent mutant of factor VIII (F-VIII). Other potential mechanisms, such as drastic reduction (or lack) of F-VIII production, or occurrence of an F-VIII inhibitor, are less likely. This conclusion was based on the analysis of plasma dilution-clotting effect relationships in normal female (anestrous, non-pregnant) sheep, female carriers of haemophilia A, and afflicted male sheep. A response model underlying the analysis was suggested, its validity tested, and a mathematical analysis suitable for routine evaluation procedure recommended.
最近在一群白阿尔卑斯山羊中发现的羊血友病A，可能是由效力较低的VIII因子（F-VIII）突变体产生所致。其他潜在机制，如F-VIII产量的大幅降低（或缺乏），或F-VIII抑制剂的出现，可能性较小。这一结论是基于对正常雌性（发情期、未怀孕）绵羊、血友病A雌性携带者和患病雄性绵羊的血浆稀释-凝血效应关系的分析得出的。提出了分析所依据的反应模型，测试了其有效性，并推荐了适用于常规评估程序的数学分析方法。
