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A “New” Family with Stuart-Prower Deficiency

1959; Thieme Medical Publishers (Germany); Volume: 03; Issue: 01 Linguagem: Inglês

10.1055/s-0038-1654370

2567-689X

J Roos, C van Arkel, M. C. Verloop, F. L. J. Jordan,

Hemoglobinopathies and Related Disorders

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.