Coagulation defect of congenital tyrosinaemia. Issue 11 (November 1984)

• Record Type: Journal Article
• Authors: Evans, D I; Sardharwalla, I B
• Is Part Of: Archives of disease in childhood. Volume 59:Issue 11(1984)
• Page Start: 1088
• Page End: 1090
• Publication Date: 1984-11
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/adc.59.11.1088
• Languages: English
• ISSNs: 0003-9888

Abstract

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.