СЛУЧАЙ НАСЛЕДСТВЕННОГО НАРУШЕНИЯ СВЕРТЫВАНИЯ КРОВИ В РАННЕМ НЕОНАТАЛЬНОМ ПЕРИОДЕ

Abstract

The article describes a case of bleeding that occurred on the first day of life in an infant with severe hemophilia A, analyzes the directions of clinical and laboratory search for disorders of the primary and secondary blood coagulation, which made it possible to clarify the diagnosis and select appropriate hemostatic therapy. Hemorrhagic disorders in the neonatal period can be caused by a deficiency of vitamin K-dependent factors, intrauterine infection, hereditary coagulopathies, primary and secondary disorders of platelet hemostasis and a number of other reasons. Clinical manifestations of hemorrhagic syndrome in a newborn child are nonspecific; they allow one to suspect the type of bleeding, but not identify the cause of the bleeding. Additional studies reflecting the viscoelastic properties of the clot have made it possible to evaluate the effectiveness of hemostatic therapy. To clarify the true cause of bleeding, in addition to thorombeastography, it became necessary to supplement the examination of the child with factor analysis based on classical clotting tests. In terms of diagnosing blood coagulation disorders, thromboelastography had no advantages over classical factor analysis.