Background. Thrombocytopathies are topical issues of pediatrics. Platelet dysfunction clinically manifests as thrombotic and hemorrhagic events of various severity and location. Platelet function can be evaluated via aggregatometry. Specified parameters for aggregatometry can be used in pediatrics as a standard for evaluating platelet function in peripheral blood. There were no similar studies in children in Russian Federation.Objective. The aim of the study is — to justify the significance of reference ranges (RR) development for platelet aggregatometry in pediatrics.Methods. The study included 30 relatively healthy patients aged from 1 to 18 years. Laboratory tests included complete blood count via automatic hematological analyzer; platelet aggregation in whole blood was evaluated via impedance aggregometer; plasmic hemostasis component (Quick prothrombin), activated partial thromboplastin time (APTT), thrombin time (TT), and fibrinogen were evaluated on automatic coagulation analyzer; molecular genetic study of platelet genes polymorphisms was performed via real-time PCR. Several inducers were used to study platelet aggregation: thrombin receptor activating peptide (TRAP), adenosine diphosphate (ADP), arachidonic acid (ASPI). Instrumental methods included electrocardiography, ultrasound of abdomen, urinary system, and thyroid gland.Results. The study included 16 girls and 14 boys. The median age was 9.4, interquartile range (IQR) was 6.5; 14,1. RR for platelet aggregation indicators were determined for all aggregation inducers (thrombin, ADP, arachidonic acid) as a result of the study.Conclusion. The obtained data will allow to improve the diagnosis approach for platelet dysfunction regarding the established reference ranges. The results of further research may contribute in the development of the algorithm for controlling antiplatelet therapy in patients with contraindications or with genetically determined low sensitivity to thrombocyte aggregation inhibitors based on acetylsalicylic acid.

Background. Cardiac surgery performed on pediatric patients with the use of artificial blood circulation (ABC) is accompanied by hemodilution, hypothermia and blood contact with artificial surfaces, as well as surgical trauma. All the above lead to endothelial cell injury, platelet aggregation and degranulation, activation of innate immunity, development of systemic inflammation and consumption of clotting, anti-coagulation and fibrinolytic factors, which is ultimately associated with the occurrence of thrombotic complications.Objective. The study aimed at developing a mathematical model for the prognosis of thrombotic complications in children which had undergone the ABC, based on assessment of their clinical and laboratory parameters.Methods. We have assessed clinical and laboratory data obtained from 153 children (newborn to 11 months 29 days of age) which had undergone cardiac surgery under conditions of ABC due to congenital heart defects (CHD). For all patients the general clinical and laboratory parameters: complete blood count, comprehensive metabolic panel, parameters of screening coagulogram, D-dimer concentration, von Willebrand factor activity, levels of antithrombin III, plasminogen, protein C and protein S, alpha-2-antiplasmin, thrombin activatable fibrinolysis inhibitor (TAFI) and fibrin-monomer have been assessed.Results. In 43 patients (28.1%) post-operative thromboses have been diagnosed. Examination of children revealed the presence of thrombosis of various localization including the intracardiac thrombi, ischemic cerebrovascular events, limb ischemia, etc. Based on logistic regression analysis, a model of development of thrombotic complications has been built which included 4 parameters: activity of lactate dehydrogenase (LDH), TAFI activity, von Willebrand factor activity and protein C activity. Model sensitivity was 95.3%, and its specificity — 96.4%.

Introduction. Warfarin-induced skin necrosis constitutes a rare complication of warfarin treatment.Aim. To present a case study of the occurrence and successful treatment of atypical warfarin-induced skin necrosis in a child with congenital heart disease.General findings. The development of finger skin necrosis in a child with congenital heart disease almost 2 years after the start of warfarin therapy was described. In addition, possible causes of the complication and methods for its treatment were discussed.Conflict of interest: the authors declare no conflict of interest.Financial disclosure: the study had no sponsorship

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