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.
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Selected References
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