Déficit congénital en facteur VII révélé par une hémorragie post circoncision

摘要

Factor VII formerly known as proconvertin is a vitamin K-dependent coagulation factor involved in exogenous coagulation. Congenital factor VII deficiency is a very rare autosomal recessive hereditary disease. We report the case of a new-born of parents who had first-degree consanguinity, admitted to hospital on D10 of life with post-circumcision haemorrhagic syndrome. Laboratory tests showed decreased levels of prothrombin and normal activated partial thromboplastin time. Coagulation tests showed isolated factor VII deficiency to 18%. The new-born received transfusion of packed red blood cells and fresh frozen plasma in order to stop bleeding with favorable outcome. This study highlights the features of this rare deficit. Prognosis is linked to the risk of severe bleedings, such as brain bleeding occurring in the neonatal period as is the case with our patient. Hence the need to perform blood crasis assessment before circumcision.