Inhibitor development after liver transplantation in congenital factor VII deficiency.
Congenital factor VII (FVII) deficiency is the commonest type of the rare bleeding disorders. Very few cases of congenital FVII deficiency developed inhibitor and liver transplant is considered as definitive treatment. In the literature, twelve patients with congenital FVII deficiency developed inhibitors. Two had spontaneous resolution of inhibitors and one did not respond to high dose recombinant factor VIIa (rFVIIa) and died. Regarding liver transplant in congenital FVII patients, seven patients underwent liver transplant with good prognosis. We report a 5-year-old girl with confirmed severe congenital FVII deficiency since neonatal period. She suffered from recurrent intracranial bleeding despite rFVIIa replacement. After auxiliary liver transplant at the age of 4, she continued to show persistent deranged clotting profile and was found to have inhibitor towards FVII. Interestingly, she was still responsive to rFVIIa replacement.
['See|W-S Q|WS|', 'Chang|K-O|KO|', 'Cheuk|D K-L|DK|', 'Leung|Y-Y R|YY|', 'Chan|G C-F|GC|', 'Chan|S-C|SC|', 'Ha|S-Y|SY|']
D002675:Child, Preschool D005167:Factor VII D005168:Factor VII Deficiency D005260:Female D006801:Humans D007223:Infant D007231:Infant, Newborn D020300:Intracranial Hemorrhages D016031:Liver Transplantation D011994:Recombinant Proteins
inhibitor liver transplant congenital Factor VII deficiency