Congenital factor VII deficiency presenting as delayed bleeding following dental extraction. A review of the role of factor VII in coagulation

Pathology ◽  
1997 ◽  
Vol 29 (2) ◽  
pp. 234-237 ◽  
Author(s):  
David C. Wei ◽  
Ricky Wing Kit Wong ◽  
Edwin P. Robertson
Keyword(s):  
Dental Extraction ◽  
Factor Vii ◽  
Delayed Bleeding ◽  
Factor Vii Deficiency ◽  
Congenital Factor

Clinical And Laboratory Observations In Congenital Factor VII Deficiency

1981 ◽  
Author(s):  
L M Kernoff ◽  
J Hughes ◽  
K Denson
Keyword(s):  
Prothrombin Time ◽  
Autosomal Recessive ◽  
Procoagulant Activity ◽  
Dental Extraction ◽  
Factor Vii ◽  
Recessive Character ◽  
Factor Vii Deficiency ◽  
Time Test ◽  
Congenital Factor

Four generations of a kindred suffering from congenital Factor VII deficiency have been studied. The defect is transmitted as an autosomal recessive character with homozygotes having < 1% Factor VII procoagulant activity, and heterozygotes between 20-50%. Homozygotes suffer from epistaxis, menorrhagia, easy bruising, haemarthrosis, and excess bleeding after dental extraction. Heterozygotes are asymptomatic. Antibody neutralisation tests using a monospecific rabbit antihuman Factor VII antibody demonstrated in the three homozygotes and their heterozygous parents the presence of Factor VII antigen in excess of Factor VII procoagulant activity. The abnormality is therefore the result of a functionally abnormal molecule and not a true deficiency of Factor VII. When the homozygous plasmas were tested in the prothrombin time test, a progressive diminution in sensitivity to the Factor VII deficiency was observed with human, rabbit and ox tissue thromboplastins. Differences were however small and the total insensitivity to ox brain thromboplastin characteristic of Factor VII Padua was not observed.

Two Typical Hereditary Charts of Congenital Factor VII Deficiency

1961 ◽  
Vol 05 (01) ◽  
pp. 087-092 ◽  
Author(s):  
F. J Cleton ◽  
E. A Loeliger
Keyword(s):  
Factor Vii ◽  
Autosomal Gene ◽  
Homozygous State ◽  
Recessive Character ◽  
Factor Vii Deficiency ◽  
Complete Penetrance ◽  
Coagulation Defect ◽  
Haemorrhagic Diathesis ◽  
Intermediate Expression ◽  
Congenital Factor

SummaryThe inheritance of congenital factor VII deficiency was investigated in 2 unrelated families. Out of 68 individuals, 4 (3 proven and 1 highly probable) were found to have severe factor VII deficiency (<C 0.1% factor VII), and 29 appeared to be heterozygotes (30—60% factor VII). The coagulation defect is due to an autosomal gene of intermediate expression and complete penetrance. The recessive character of the haemorrhagic diathesis due to the homozygous state for the abnormal gene is clearly demonstrated.

scholarly journals Efficacy and safety of recombinant factor VIIa preparation (NovoSeven) for patients with congenital factor VII deficiency

2006 ◽  
Vol 17 (6) ◽  
pp. 695-705 ◽  
Author(s):  
Hideji HANABUSA ◽  
Kazushige OYAMA ◽  
Satoshi WATANABE ◽  
Yuzuru SAKAKIBARA ◽  
Yuji HIRAMATSU ◽  
...  
Keyword(s):  
Recombinant Factor Viia ◽  
Factor Viia ◽  
Factor Vii ◽  
Efficacy And Safety ◽  
Factor Vii Deficiency ◽  
Congenital Factor

scholarly journals Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

Haemophilia ◽  
2016 ◽  
Vol 22 (5) ◽  
pp. 752-759 ◽  
Author(s):  
M. Napolitano ◽  
M. N. D. Di Minno ◽  
A. Batorova ◽  
A. Dolce ◽  
M. Giansily-Blaizot ◽  
...  
Keyword(s):  
Clinical Phenotype ◽  
Treatment Options ◽  
Factor Vii ◽  
International Studies ◽  
Factor Vii Deficiency ◽  
Congenital Factor

Congenital Factor VII Deficiency

2018 ◽  
pp. 239-259
Author(s):  
Mahmood Shams ◽  
Akbar Dorgalaleh
Keyword(s):  
Factor Vii ◽  
Factor Vii Deficiency ◽  
Congenital Factor

scholarly journals Novel IVS7+1G>T mutation of life-threatening congenital factor VII deficiency in neonates

Medicine ◽  
2019 ◽  
Vol 98 (40) ◽  
pp. e17360
Author(s):  
Juan He ◽  
Wei Zhou ◽  
Hui Lv ◽  
Li Tao ◽  
XiaoWen Chen ◽  
...  
Keyword(s):  
Factor Vii ◽  
Factor Vii Deficiency ◽  
Life Threatening ◽  
Congenital Factor
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