The factor VII deficiency in moroccan children
The Factor VII deficiency is a rare causes of bleeding disorders in children. 12 cases were diagnosed and treated in hematology and oncology service between 2003 and 2016. The clinical manifestations are extremely variable. The severity of bleeding is variable and does not seem related to the importance of the deficit; A deep deficit may remain asymptomatic or result in more or less severe hemorrhagic syndrom. 33% of cases were asymptomatic, 66% of cases had a hemorrhagic syndrom: It's Mucocutaneous bleeding (33%), gastrointestinal bleeding (17%), Urinary bleeding (hematuria) (8%), Articular bleeding (haemarthrosis ankle) (8%). Our patients have low PT raging from 12% to 55%. Family investigation is required, including the dosage of Factor VII in parents and siblings. The Factor VII deficiency is suspected ahead of the combination of an elongated prothrombin time and a normal PTT, it is confirmed by the dosage of factor VII activity, by chronometric method. Replacement therapy of this deficit is not systematic, it is indicated in the case of an acute hemorrhagic stroke, or prophylactically prior to surgery, it is based on the recombinant Factor.
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