Publication: Outcome of intracranial hemorrhage in infants with congenital factor VII deficiency
2
Issued Date
2002-11-01
Resource Type
ISSN
01252208
Other identifier(s)
2-s2.0-0036881437
Rights
Mahidol University
Rights Holder(s)
SCOPUS
Bibliographic Citation
Journal of the Medical Association of Thailand. Vol.85, No.SUPPL. 4 (2002)
Suggested Citation
Ampaiwan Chuansumrit, Nittaya Visanuyothin, Siriraj Puapunwattana, Anong Chaivisuth, Patcharawalai Rasmidat, Pimlak Charoenkwan, Surang Chiemchanya Outcome of intracranial hemorrhage in infants with congenital factor VII deficiency. Journal of the Medical Association of Thailand. Vol.85, No.SUPPL. 4 (2002). Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/20345
Research Projects
Organizational Units
Authors
Journal Issue
Thesis
Title
Outcome of intracranial hemorrhage in infants with congenital factor VII deficiency
Abstract
The outcome of 8 episodes of intracranial hemorrhage in 7 patients (4 males, 3 females) with congenital factor VII deficiency was evaluated. Their levels of factor VII clotting activity (FVII:C) were less than 1 per cent (n = 3) and ranged from 1.7 to 2.3 per cent (n = 4). The onset varied from the first week (n = 2), first month (n = 3), and at the ages of 6, 11 and 12 months (n = 3). The replacement therapy of 10 ml/kg of fresh frozen plasma (FFP) every 6-12 hours for 5-7 days was given to 6 patients. Only one craniotomy for the removal of hematoma was performed. The seventh patient experienced two episodes of bleeding. First, she received 20 μg/kg of recombinant factor VIIa (rFVIIa) every 6 hours for 4 days (1,200 μg) followed by FFP in one episode. Second, a craniotomy for the removal of a 7 cm diameter hematoma was performed by giving 20 μg/kg of rFVIIa every 6 hours for 12 days (9,600 μg) followed by FFP in another episode. As a result of these treatments, 2 died and 5 survived. with sequelae, except for one who received rFVIIa. The sequelae included seizure disorder (n = 1) and hydrocephalus (n = 3). Subsequently, the surviving patients received 15 ml/kg of lyophilized fresh plasma every 3-5 days as prophylactic treatment. In conclusion, rFVIIa in the dose of 20 μg/kg every 6 hours has been shown to be effective in controlling intracranial hemorrhage in patients with congenital factor VII deficiency.