PT - JOURNAL ARTICLE AU - D I Evans AU - I B Sardharwalla TI - Coagulation defect of congenital tyrosinaemia. AID - 10.1136/adc.59.11.1088 DP - 1984 Nov 01 TA - Archives of Disease in Childhood PG - 1088--1090 VI - 59 IP - 11 4099 - http://adc.bmj.com/content/59/11/1088.short 4100 - http://adc.bmj.com/content/59/11/1088.full SO - Arch Dis Child1984 Nov 01; 59 AB - 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.