Fibrin cross-linking in congenital factor XIII deficiency. Issue 5 (May 1980)

• Record Type: Journal Article
• Authors: Rodeghiero, F; Barbui, T
• Is Part Of: Journal of clinical pathology. Volume 33:Issue 5(1980)
• Page Start: 434
• Page End: 437
• Publication Date: 1980-05
• Subjects: Pathology -- Periodicals; Pathology, Molecular -- Periodicals; 616.0705
• DOI: 10.1136/jcp.33.5.434
• Languages: English
• ISSNs: 0021-9746

Abstract

Abstract : Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. Quantitative studies of transamidase activity of the factor are available in only a few cases, and the fibrin cross-linking pattern is not well known. The present paper deals with the quantitative estimation of factor XIII transamidase activity (dansylcadaverine system), factor XIII molecular subunits, and the corresponding fibrin cross-linking pattern in seven homozygous patients with factor XIII deficiency. The results indicate that transamidase activity was present in all patients, and the range was 0.5-1.7%. The pattern of fibrin stabiisation showed an absence of cross-linking in two patients, the presence of gamma-gamma-dimers (traces) in four, and gamma-gamma-dimers plus incomplete alpha-polymers (traces) in one patient. In conclusion, the homozygous patients reported here were not completely devoid of functioning factor XIII.