Thrombosis and Haemostasis, Table of Contents Thromb Haemost 1983; 49(02): 102-105DOI: 10.1055/s-0038-1657331 Original Article Schattauer GmbH Stuttgart The Half Life of Factor XIII in the Management of Inherited Deficiency Authors Author Affiliations J D Fear The Department of Medicine, St. James's University Hospital, Leeds, England K J A Miloszewski The Department of Medicine, St. James's University Hospital, Leeds, England M S Losowsky The Department of Medicine, St. James's University Hospital, Leeds, England Recommend Article Abstract PDF Download(opens in new window) Buy Article(opens in new window) Summary Following the injection of a large dose of factor XIII concentrate the in vivo half life of factor XIII was estimated in a patient with inherited deficiency. Factor XIII activity and enzyme concentration were measured quantitatively, and a qualitative assessment of the crosslinking of fibrin was also made for upto 6 weeks after the injection. The half life was found to be about 9-10 days. This is longer than most previous reports suggest. An explanation for this finding is offered. The relevance of the long half life of factor XIII to the prophylactic treatment of patients with inherited deficiency is demonstrated. Keywords KeywordsFactor XIII - Half life - Prophylaxis PDF (1104 kb) References References 1 Trobisch H, Egbring R. Substitution mit einem neuen Faktor XIII Konzentrat bei kongenitalem Faktor XIII-Mangel. Dtsch Med Wochenschr 1972; 97: 499-502 2 Zimmermann R, Ehlers W, Manz F, Meinrenken W, Egbring R, Gemmeke H. Ein neuer Fall von schwerem Faktor XIII-Mangel. Blut 1977; 35: 457-464 3 Kitchens CS, Newcomb TF. Factor XIII. Medicine 1979; 58: 413-429 4 Miloszewski K, Losowsky MS. Factor XIII Concentrate in Long Term Management of Congenital Factor XIII Deficiency. Thrombos Diathes Haemorrh 1975; 34: 323-324 5 Losowsky MS, Hall R, Goldie W. Congenital Deficiency of Fibrin Stabilising Factor. Lancet 1965; II: 156-158 6 Sheltawy MJ, Miloszewski K, Losowsky MS. Factors Affecting Factor XIII Assay by Dansyl Cadaverine Incorporation. Thrombos Diathesis Haemorrh 1972; 28: 483-488 7 Laurell CB. Electroimmuno assay. Scand J Clin Lab Invest 1972; 29 suppl. (Suppl. 124) 21-37 8 Weber K, Osborn M. The Reliability of Molecular Weight Determination by Dodecyl Sulphate-polyacrylamide Gel Electrophoresis. J Biol Chem 1969; 244: 4406-4412 9 Miloszewski K, Losowsky MS. The Half Life of Factor XIII in vivo . Br J Haematol 1970; 19: 685-690 10 Duckert F, Jung E, Shmerling DH. A Hitherto Undescribed Congenital Haemorrhagic Diathesis Probably Due to Fibrin Stabilizing Factor Deficiency. Thrombos Diathes Haemorrh 1960; 5: 179-186 11 Amris CJ, Ranek L. A Case of Fibrin Stabilizing Factor (FSF) Deficiency. Thrombos Diathes Haemorrh 1965; 14: 332-340 12 Schwartz ML, Pizzo SV, Hill RL, McKee PA. Effect of Fibrin Stabilizing Factor on the Subunit Structure of Human Fibrin. J Clin Invest 1971; 50: 1506-1513 13 Ikkala E, Myllylä G, Nevanlinna HR. Transfusion Therapy in Factor XIII (FSF) Deficiency. Scand J Haematol 1964; 1: 308-312 14 Ottaviani P, Mandelli F. Two cases of haemorrhagic disease arising from fibrin stabilizing factor deficiency. Hémostase 1966; 6: 317-324 15 Bouhasin JD, Altay C. Factor XIII deficiency: Concentrations in relatives of patients and in normal infants. J Pediatr 1968; 72: 336-341 16 Lopaciuk S, Kozlowska J, Uszynski L. Przypadek Wrodzonego Braku Czynnika XIH (FSF). Pol Arch Med Wewn 1969; 42: 255-259 17 Broccia G. Haemorrhagic Disease due to Hereditary Deficiency of Factor XHI. Acta Haematol (Basel) 1970; 44: 177-185 18 Fukui H, Yoshiya H, Katagjri S, Yoshioka A, Mikami S, Okuda T. et al. Congenital Factor XIII (Fibrin Stabilizing Factor) Deficiency: Report of Three Cases. Jpn J Clin Haematol 1974; 15: 191-200 19 Losowsky MS, Miloszewski K. Management of Patients with Congenital Deficiency of Fibrin Stabilising Factor (Factor XIII). In: Proceedings of the International Congress of Haematology. Seno TakakuIrino. (eds).: Excerpta Medica; 1977 Kyoto, Japan: 1976
