Outcome of intracranial hemorrhage in infants with congenital factor VII deficiency.
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 microg/kg of recombinant factor VIIa (rFVIIa) every 6 hours for 4 days (1,200 microg) followed by FFP in one episode. Second, a craniotomy for the removal of a 7 cm diameter hematoma was performed by giving 20 microg/kg of rFVIIa every 6 hours for 12 days (9,600 microg) 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 microg/kg every 6 hours has been shown to be effective in controlling intracranial hemorrhage in patients with congenital factor VII deficiency.
Chuansumrit
Ampaiwan
A
Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
Visanuyothin
Nittaya
N
Puapunwattana
Siriraj
S
Chaivisuth
Anong
A
Rasmidat
Patcharwalai
P
Charoenkwan
Pimlak
P
Chiemchanya
Surang
S
D005168:Factor VII Deficiency / Q000150:complications* / Q000628:therapy*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D020300:Intracranial Hemorrhages / Q000209:etiology* / Q000628:therapy*; D008297:Male; D017063:Outcome Assessment, Health Care