Study of Different Factor VII Deficiency Variants in Nine Families from Spain
作者:
A. Pardo,
J.P. Oteyza,
L. Blanco,
M.A. Correa,
J. Cesar,
J.L. Steegman,
M. Tamayo,
I. Fernandez,
L. Escribano,
J.L. Navarro,
期刊:
Pathophysiology of Haemostasis and Thrombosis
(Karger Available online 1987)
卷期:
Volume 17,
issue 5
页码: 268-272
ISSN:1424-8832
年代: 1987
DOI:10.1159/000215754
出版商: S. Karger AG
关键词: Factor VII;Hypoprothrombinemia;Bleeding disorder
数据来源: Karger
摘要:
Twenty-three patients with congenital factor VII (FVII) deficiency, belonging to 9 kindreds were studied. Immunological variants were classified according to the relationship between FVII coagulant activity (FVIIC) and the level of FVII antigen (FVIIAg), considering 3 previously described groups: VII-, VII+ and VIIR. Activation variants were determined by the reactivity pattern with three different thromboplastins. One patient was classified as VII-, 16 as VII+, and 6 as VIIR. Three patients belonging to the same kindred showed a Padua 2 FVII deficiency, and 1 patient showed a Padua 1 variant. There was no correlation between antigenic or activation variants and severity of bleeding tendency. Classical autosomal recessive mode of inheritance was observed in VII- and VII+ families. Nevertheless, a possible autosomal dominant trait was observed in VII+ kindreds.
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