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 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.
Mariani
G
G
Department of Internal Medicine and Public Health, University of L'Aquila, L'Aquila I-6F100, Italy. gmariani@cc.univaq.it
Dolce
A
A
Marchetti
G
G
Bernardi
F
F
D001779:Blood Coagulation Factors / Q000627:therapeutic use; D005167:Factor VII / Q000037:antagonists & inhibitors / Q000627:therapeutic use; D005168:Factor VII Deficiency / Q000151:congenital / Q000175:diagnosis*; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D008297:Male; D010949:Plasma; D019106:Postoperative Hemorrhage / Q000517:prevention & control; D012737:Sex Factors
D001779:Blood Coagulation Factors; C025667:prothrombin complex concentrates; D005167:Factor VII