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Title: Outcome of intracranial hemorrhage in infants with congenital factor VII deficiency
Authors: Ampaiwan Chuansumrit
Nittaya Visanuyothin
Siriraj Puapunwattana
Anong Chaivisuth
Patcharawalai Rasmidat
Pimlak Charoenkwan
Surang Chiemchanya
Mahidol University
Maharaj Nakhon Ratchasima Hospital
Chiang Rai Prachanukhro Hospital
Paholpolpayuhasena Hospital
Nopparatrajthani Hospital
Chiang Mai University
Keywords: Medicine
Issue Date: 1-Nov-2002
Citation: Journal of the Medical Association of Thailand. Vol.85, No.SUPPL. 4 (2002)
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.
ISSN: 01252208
Appears in Collections:Scopus 2001-2005

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