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Thromb Haemost 1959; 03(01): 059-076
DOI: 10.1055/s-0038-1654370
Originalarbeiten — Original Articles — Travaux Originaux
Schattauer GmbH

A “New” Family with Stuart-Prower Deficiency

J Roos
1   Utrecht/Netherlands
,
C van Arkel
1   Utrecht/Netherlands
,
M. C Verloop
1   Utrecht/Netherlands
,
F. L. J Jordan
1   Utrecht/Netherlands
› Author Affiliations
Further Information

Publication History

Publication Date:
08 June 2018 (online)

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Summary

A description is given of the coagulation disturbances in six patients with a haemorrhagic diathesis due to Stuart-Prower deficiency. Relatives of these patients, if heterozygous for this deficiency, showed no significant haemorrhagic diathesis; in the laboratory, their one-stage “prothrombin” times showed only a slight prolongation. Thromboplastin formation in these relatives was sufficient, but determination of the Stuart-Prower factor revealed lower values. The relatives in question were incapable of giving the same correction of the thromboplastin generation test in their bleeder relatives as normal serum does.

The relation between Stuart-Prower deficiency and Christmas factor is discussed.

One patient is described who combined heterozygotism for Stuart-Prower deficiency with alcaptonuria.

The mode of inheritance of the Stuart-Prower deficiency is discussed.

 

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