Clinical picture and management of congenital factor VII deficiency

In patients with congenital FVII deficiency, bleeding manifestations and clinical presentation vary widely, ranging from asymptomatic subjects to patients with haemorrhages that may cause important handicaps. Owing to menorrhagia, which occurs in about two‐thirds of women of fertile age, bleeding is...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2004-10, Vol.10 (s4), p.180-183
Main Authors: Mariani, G., Dolce, A., Marchetti, G., Bernardi, F.
Format: Article
Language:English
Subjects:
Blood Coagulation Factors - therapeutic use
congenital FVII deficiency
Factor VII - antagonists & inhibitors
Factor VII - therapeutic use
Factor VII Deficiency - congenital
Factor VII Deficiency - diagnosis
Female
haemostatic treatment
Hemorrhage - etiology
Hemorrhage - prevention & control
Humans
Male
Plasma
Postoperative Hemorrhage - prevention & control
Sex Factors
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Summary:In patients with congenital FVII deficiency, bleeding manifestations and clinical presentation vary widely, ranging from asymptomatic subjects to patients with haemorrhages that may cause important handicaps. Owing to menorrhagia, which occurs in about two‐thirds of women of fertile age, bleeding is more frequent in women than in men. Gum bleeding and easy bruising are also more frequent in females. FVII:C levels are not a good predictor of bleeding tendency as there is a wide overlap between bleeders and asymptomatic patients. We propose a three‐grade system of classification based on clinical considerations. Therapy for congenital FVIII bleeding is discussed, with the advantages and disadvantages of each treatment, and the suggested single dose given.
ISSN:1351-8216
1365-2516
DOI:10.1111/j.1365-2516.2004.00990.x