Multidisciplinary approach to the diagnosis of disorders of coagulation system in childrenwith various pathologies

A significant problem in pediatrics is the hemostasisdysfunction, especially its anticoagulant system. The deficiency of fibrinolysis factors and the anticoagulant system increases the risk of thrombosis. Children with chronic diseases, such as cardiovascular, nervous systems, in patients with hereditary genetic diseases with a deficiency of anticoagulant factors (congenital or acquired), may have episodes of thrombotic events. Thrombosis in children with genetically determined thrombophilia often occur in the context of risk factors. In the early neonatal period, the deficiency of anticoagulant factors manifests as the form of fulminant purpura, followed by the development of skin necrosis. The genetic factor plays one of the most importantreason in the thrombophilia development, which leads to a deficiency or defect in blood coagulation factors, platelet receptors, or manifests itself in the form of a hereditary predisposition. The current screening of hemostasis does not properly identify the causes of thrombosis. The development of thrombotic complications in children is a multifactorial pathological condition and requires multidisciplinary approach in diagnostic and therapeutic measures. The problem of correct in time diagnostics has the socially significant nature. Currently, international consensus and national protocols for the treatment and prevention of venous thromboembolism are regularly reviewing. In the study, the system of natural anticoagulants was evaluated in children with various pathologies. The study included 33 patients with confirmed diagnoses, including cardiovascular pathology, syndromes of nervous diseases, undifferentiatedconnective tissue dysplasia, hereditary monogenic syndrome. The results obtained indicate a high frequency of occurrence of polymorphisms in coagulation genes (in a third of children with various diseases in the study), in some children a decrease in the activity of natural anticoagulants (NAC) proteins (S,С) (from 12 to 15%) was determined, which confirms the need for the presented diagnostics to predict the condition and assess hemostasis in children.

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