Inhibitor development after liver transplantation in congenital factor VII deficiency

Autor:	Wing-shan Queenie See, Gcf Chan, Daniel K. L. Cheuk, Y.-Y. R. Leung, S.-C. Chan, Shau-Yin Ha, K.-O Chang
Rok vydání:	2016
Předmět:	medicine.medical_specialty Factor VII Deficiency medicine.medical_treatment Intracranial Hemorrhages 030204 cardiovascular system & hematology Liver transplantation Gastroenterology 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine hemic and lymphatic diseases Internal medicine medicine Humans cardiovascular diseases Factor VII deficiency Genetics (clinical) biology Factor VII business.industry Infant Newborn Infant Hematology General Medicine Recombinant Proteins Liver Transplantation Surgery chemistry Recombinant factor VIIa Child Preschool 030220 oncology & carcinogenesis biology.protein Female Good prognosis business Intracranial bleeding Auxiliary liver transplant
Zdroj:	Haemophilia. 22:e417-e422
ISSN:	1351-8216
Popis:	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.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6c29d2245e1576bc50a27f485d0770d4 https://doi.org/10.1111/hae.13047 Zobrazit plný text záznamu Plný text ve formátu PDF Plný text ve formátu HTML
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