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Original ArticleOpen Access
Outcome of Intracranial Hemorrhage in Infants with Congenital Factor VII Deficiency
Chuansumrit A 
,
Visanuyothin N ,
Puapunwattana S ,
Chaivisuth A ,
Rasmidat P ,
Charoenkwan P ,
Chiemchanya S
,
Visanuyothin N ,
Puapunwattana S ,
Chaivisuth A ,
Rasmidat P ,
Charoenkwan P ,
Chiemchanya S 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 I 0 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
VIla (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 em 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 mllkg 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.
Key word : Factor VII Deficiency, CNS Bleeding
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 I 0 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
VIla (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 em 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 mllkg 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.
Key word : Factor VII Deficiency, CNS Bleeding
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