Factor VIIa in patients with C1-inhibitor deficiency.

Abstract:

:In hereditary angioedema (HAE), normal C1-inhibitor (C1-INH) is low and the contact system activated. Recently, the findings of a tissue factor mutant selectively deficient in promoting the conversion of FVII to FVIIa, but with retained cofactor for FVIIa, made it possible to examine reliably the pre-existing content of FVIIa in HAE patients. This was of interest as FXIIa (mainly inhibited by C1-INH) is able to activate FVII directly. FVIIa in 21 remission HAE patients were within normal limits but nearly doubled as compared to their 23 normal siblings (p = 0.0017). Cold promoted activation of FVII (CPA) (common clot assay) was displayed in plasma of all 5 untreated patients (C1-INH function < 35%), but not in plasma of 2 patients treated prophylactically with danazol (C1-INH function about 40%). These results suggest that there is a minute, yet significant activation of FVII in patients with C1-INH deficiency.

journal_name

Thromb Haemost

authors

Nielsen EW,Morrissey J,Olsen JO,Osterud B

subject

Has Abstract

pub_date

1995-10-01 00:00:00

pages

1103-6

issue

4

eissn

0340-6245

issn

2567-689X

journal_volume

74

pub_type

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