Nielsen E W, Johansen H T, Høgåsen K, Wuillemin W, Hack C E, Mollnes T E
Department of Anaesthesiology, Nordland Central Hospital, Bodø, Norway.
Scand J Immunol. 1996 Aug;44(2):185-92. doi: 10.1046/j.1365-3083.1996.d01-298.x.
Five patients with hereditary angioedema (HAE) were studied during attacks and remission as were healthy controls. The high levels of C1/C1-INH complexes, low C4 and high ratio C4 activation products (C4bc)/C4 also differed significantly during remission compared to controls. During attacks C4bc/C4 increased (922-2007; P = 0.022, remission versus attacks, median values throughout), C2 and CH50 dropped (111-31%; P = 0.043 and 110-36%; P = 0.016, respectively), TCC (C5b-9) increased (0.88-1.23 AU/ml; P = 0.028). Cleavage of HK increased to be almost complete during attacks (20-90%; P = 0.009). While factor XIa/serpin-complexes did not increase, a more than twofold rise in thrombin/antithrombin-complexes (0.20-0.50 microgram/l; P = 0.009) and in plasmin/alpha-2-antiplasmin-complexes (7.3-17 nmol/l; P = 0.028) was observed. For the first time cascade activation in HAE was studied simultaneously, and corroborates that attacks lead to activation of the kallikrein-kinin system, fibrinolysis and early part of the classical complement pathway. In addition, the authors present novel data of terminal complement and coagulation activation, the latter apparently not via FXIa.
对5例遗传性血管性水肿（HAE）患者在发作期和缓解期进行了研究，并与健康对照者进行了对比。与对照组相比，缓解期时C1/C1-INH复合物水平较高、C4水平较低以及C4激活产物（C4bc）/C4比值较高，这些差异也非常显著。发作期时，C4bc/C4升高（922 - 2007；缓解期与发作期相比，P = 0.022，均为中位数），C2和CH50下降（分别为111 - 31%；P = 0.043和110 - 36%；P = 0.016），末端补体复合物（TCC，C5b - 9）升高（0.88 - 1.23 AU/ml；P = 0.028）。激肽释放酶原（HK）的裂解在发作期增加至几乎完全裂解（20 - 90%；P = 0.009）。虽然因子XIa/丝氨酸蛋白酶抑制剂复合物未增加，但观察到凝血酶/抗凝血酶复合物（0.20 - 0.50微克/升；P = 0.009）和纤溶酶/α2 - 抗纤溶酶复合物（7.3 - 17纳摩尔/升；P = 0.028）增加了两倍多。首次同时研究了HAE中的级联激活情况，证实发作会导致激肽释放酶 - 激肽系统、纤维蛋白溶解以及经典补体途径早期部分的激活。此外，作者还展示了末端补体和凝血激活的新数据，后者显然不是通过因子XIa激活的。
