Lewis J H, Iammarino R M, Spero J A, Hasiba U
Blood. 1978 Jan;51(1):129-37.
A 10-year-old boy had a severe lifelong hemorrhagic disorder that had necessitated more than 50 hospitalizations. Laboratory examination showed prolonged bleeding, clotting, partial thromboplastin, prothrombin, and thrombin times. These findings were due to a potent inhibitor of the thrombin-fibrinogen reaction. This inhibitor was similar to heparin in that it acted immediately and did not interfere with the coagulant activities of certain venoms. It differed from heparin in not being adsorbed to barium citrate or neutralized by protamine sulfate. The inhibitory effect was found in the alpha1-globulin fraction. It was identified immunologically and functionally as a double-banded alpha1-antitrypsin of a previously unreported phenotype. The inhibitory effects were depressed by trypsin and heterologous anti-alpha1-antitrypsin.
一名10岁男孩患有严重的终身出血性疾病，为此他已住院50多次。实验室检查显示出血时间、凝血时间、部分凝血活酶时间、凝血酶原时间和凝血酶时间延长。这些发现是由于一种强力的凝血酶-纤维蛋白原反应抑制剂所致。这种抑制剂与肝素相似，因为它能立即起作用，且不干扰某些蛇毒的凝血活性。它与肝素的不同之处在于它不被柠檬酸钡吸附，也不被硫酸鱼精蛋白中和。抑制作用存在于α1球蛋白部分。通过免疫学和功能鉴定，它是一种以前未报道过的表型的双条带α1抗胰蛋白酶。胰蛋白酶和异源抗α1抗胰蛋白酶可降低其抑制作用。
