Outcome of Intracranial Hemorrhage in Infants with Con
genital Factor VII Deficiency
AMPAIWAN CHUANSUMRIT, M.D.*, NITTAYA VISANUYOTHIN, M.D.**,
SIRIRAJ PUAPUNWATTANA, M.D.***, ANONG CHAIVISUTH, M.D.****,
PATCHARAWALAI RASMIDAT,M.D.*****, PIMLAK CHAROENKWA N, M.D.******,
SURANG CHIEMCHANY A, M.D.*
Affiliation : * Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok 10400,
** Maharaj Nakhon Ratchasima Hospital, Nakhon Ratchasima 30000,
*** Chiang Rai Prachanukruk Hospital, Chiang Rai 57000,
**** Paholpolpayuhasena Hospital, Kanchanaburi 71000,
***** Nopparatrajthani Hospital, Bangkok 10230,
****** Faculty of Medicine, Chiang Mai University, Chiang Mai 50000, Thailand.
AbstractThe 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 I0 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 ~-tglkg of recombinant factor
VIla (rFVIIa) every 6 hours for 4 days (1,200 !lg) followed by FFP in one episode. Second, a craniotomy for the removal of a 7 em diameter hematoma was performed by giving 20 jlg/kg of rFVIIa
every 6 hours for 12 days (9,600 jlg) 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 jlg/kg every 6 hours has been shown to be effective in controlling
intracranial hemorrhage in patients with congenital factor VII deficiency.
Keywords : Factor VII Deficiency, CNS Bleeding
All Articles
Download
