Factor VII deficiency: a single-center experience.
Congenital factor VII deficiency is the most common form of rare coagulation factor deficiencies. This article presents a retrospective evaluation of 73 factor VII deficiency cases that had been followed at our center. The study consisted of 48 males and 25 females (2 months-19 years). Thirty-one (42.5%) of them were asymptomatic. Out of symptomatic patients, 17 had severe clinical symptoms, whereas 8 presented with moderate and 17 with mild symptoms. The symptoms listed in order of frequency were as follows: epistaxis, petechia or ecchymose, easy bruising, and oral cavity bleeding. The genotype was determined in 8 patients. Recombinant activated factor VII (rFVIIa) was used to treat 49 bleeding episodes in 8 patients after 2002. In 2 patients with repeated central nervous system bleeding prophylaxis with rFVIIa was administered. No allergic and thrombotic events were observed during both treatment and prophylaxis courses. Antibody occurrence was not detected in the patients during treatment.
Bakirkoy Children's and Maternity Education and Research Hospital, Istanbul, Turkey. zafersalcioglu@yahoo.com
Zafer
Z
Salcioglu
Arzu
A
Akcay
Hulya Sayilan
HS
Sen
Gonul
G
Aydogan
Ferhan
F
Akici
Deniz
D
Tugcu
Nuray Aktay
NA
Ayaz
Zafer
Z
Baslar
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005168:Factor VII Deficiency / Q000097:blood / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D015942:Factor VIIa / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D005838:Genotype; D006470:Hemorrhage / Q000097:blood / Q000235:genetics / Q000517:prevention & control; D006801:Humans; D007223:Infant; D008297:Male; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000009:adverse effects
D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa