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Thromb Haemost 1983; 50(02): 588-590
DOI: 10.1055/s-0038-1665262
Original Article
Schattauer GmbH Stuttgart

Factor XIII Levels in Five Families of Patients with Inherited Factor XIII Deficiency: Support for an Autosomal Recessive Inheritance

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 31 March 1983

Accepted 21 June 1983

Publication Date:
18 July 2018 (online)

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Summary

Using quantitative methods, f.XIII activity and levels of subunits a and b have been measured in 5 families of 6 patients with inherited XIII deficiency, including 2 children of a XIII deficient male. The parents, as a group, and the children, individually, have low XIII activity and low levels of subunit A when compared to controls. These findings provide further support for an autosomal inheritance of f.XIII deficiency. The measurements, however, did not allow confident selection of individual heterozygotes as has been previously suggested and an explanation of this finding is offered.

Keywords

Factor XIII - Activity - Subunits - Deficient families
 

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