Thromb Haemost 1983; 49(02): 102-105
DOI: 10.1055/s-0038-1657331
Original Article
Schattauer GmbH Stuttgart

The Half Life of Factor XIII in the Management of Inherited Deficiency

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
› Author Affiliations
Further Information

Publication History

Received 06 December 1982

Accepted 26 January 1983

Publication Date:
18 July 2018 (online)

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.

 
  • 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