Abstract
Congenital combined vitamin K-dependent clotting factors deficiency is an extremely rare bleeding disorder, a few cases have been described worldwide. This coagulopathy requires a differential diagnosis with vitamin K deficiency bleeding of the newborn and coagulopathy, associated with different liver disease. The world practice of treating this hemorrhagic condition is the use of vitamin K 1 (not registered in the Russian Federation), as well as a prothrombin complex concentrate. This article describes the clinical manifestations, diagnosis and specific treatment in 2 patients with this bleeding disorder. The patients' parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.
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