先天性酪氨酸血症的凝血缺陷
Coagulation defect of congenital tyrosinaemia.
作者信息
Evans D I, Sardharwalla I B
出版信息
Arch Dis Child. 1984 Nov;59(11):1088-90. doi: 10.1136/adc.59.11.1088.
Abstract
Three infants with hereditary tyrosinaemia had a severe coagulation defect due to a combination of deficient hepatic synthesis of clotting factors and a consumption coagulopathy. In all three plasma failed to clot normally with the venom of Bothrops atrox (Reptilase) and we attribute this to a defect of fibrinogen (dysfibrinogenaemia). Treatment was unsuccessful, and all died.
摘要
三名患有遗传性酪氨酸血症的婴儿出现严重凝血缺陷，原因是肝脏凝血因子合成不足与消耗性凝血病共同作用。在这三名婴儿中，血浆均不能被矛头蝮蛇毒（爬虫酶）正常凝固，我们认为这是纤维蛋白原缺陷（异常纤维蛋白原血症）所致。治疗未成功，三名婴儿均死亡。
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