title: Inhibitor development after liver transplantation in congenital factor VII deficiency.
abstract: 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.
authors: Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Special Administrative Region, China.
authors: W-S Q
authors: WS
authors: See
authors: Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong, Special Administrative Region, China.
authors: K-O
authors: KO
authors: Chang
authors: Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Special Administrative Region, China.
authors: D K-L
authors: DK
authors: Cheuk
authors: Department of Pathology, The University of Hong Kong, Hong Kong, Special Administrative Region, China.
authors: Y-Y R
authors: YY
authors: Leung
authors: Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Special Administrative Region, China.
authors: G C-F
authors: GC
authors: Chan
authors: Department of Surgery, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Special Administrative Region, China.
authors: S-C
authors: SC
authors: Chan
authors: Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Special Administrative Region, China. syha@hku.hk.
authors: S-Y
authors: SY
authors: Ha
mesh_terms: D002675:Child, Preschool; D005167:Factor VII / Q000627:therapeutic use*; D005168:Factor VII Deficiency / Q000150:complications / Q000628:therapy*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D020300:Intracranial Hemorrhages / Q000209:etiology / Q000517:prevention & control*; D016031:Liver Transplantation; D011994:Recombinant Proteins / Q000627:therapeutic use*
chemical_list: D011994:Recombinant Proteins; D005167:Factor VII
keywords: congenital Factor VII deficiency; inhibitor; liver transplant