КЛИНИЧЕСКИЕ И ГЕМОСТАЗИОЛОГИЧЕСКИЕ ОСОБЕННОСТИ ЦИРРОЗА ПЕЧЕНИ АУТОИММУННОЙ ЭТИОЛОГИИ У ДЕТЕЙ

Abstract

Objective of the study: to determine clinical, hemostasiological features and evaluate the relationship of autoantibodies with hemostasiological disorders in children with liver cirrhosis (LC) class A according to Child–Pugh score in the outcome of autoimmune hepatitis (AIH). Materials and methods of research: a single-center, retrospective, controlled, non-randomized study. From 2017 to 2021, 31 children aged 13 (10; 15.5) years old [15 girls (48%) and 16 boys (52%)] with LC class A according to Child–Pugh classification in the outcome of AIH and 15 children aged 13 (10; 15.5) years [8 girls (53%) and 7 boys (47%)], belonging to health groups I–II. All participants were examined according to the medical standard for specialized medical care for children with LC in accordance with the «Autoimmune hepatitis in children” of the Federal clinical guidelines (Moscow, 2016) and the medical standard for specialized medical care for children with AIH. The parameters of the hemostatic system were determined on the ACL-TOP 500 hemocoagulation analyzer (Instrumentation Laboratory Company, USA) using standard kits: activated partial thromboplastin time (aPTT), Prothrombin time (PT) calculated as % of prothrombin activity according to Quick, thrombin time, fibrinogen content, activity of antithrombin III (ATIII), protein C, plasminogen, alpha2-antiplasmin (α2-AP). To determine the factor XII-dependent fibrinolysis activity (XIIa-DF) and the content of Soluble fibrin monomer complexes (SFMCs), reagent kits from the Tekhnologiya-Standard company (Russia) were used. The platelet count was determined using a Cell-Dyn 610 hematology analyzer (Abbott Diagnostics, USA). The study of platelet aggregation activity was carried out on a platelet aggregation analyzer using adenosine diphosphate (ADP) at a concentration of 1.25 mg/ml and collagen at a concentration of 2.0 microns/ml as an aggregation inducer. Measurement of plasma endothelin-1 was carried out by using Endothelin 1-21 ELISA Assay kit. Homocysteine concentration and VWF activity were determined on an ACL-TOP 500 hemocoagulation analyzer (Instrumentation Laboratory Company, USA) using standard kits. Results: the clinical picture was characterized by asthenoneurotic (100%), abdominal pain (45%), hemorrhagic [ecchymosis (55%), epistaxis (30%), bleeding gums (16%)] syndromes, hepatomegaly (90%), splenomegaly ( 65%). Hemostasiological disorders were characterized by a decrease in the content of platelets (p=0.001), an increase in the degree (ADP inducer, p=0.001; collagen inducer, p=0.023) and speed (ADP inducer, p=0.003; collagen inducer, p=0.032) of their aggregation, inhibition coagulation (p=0.001) and anticoagulant (p=0.002) components of hemostasis in comparison with a group of healthy children. Positive autoantibodies were diagnosed in 97% of patients, in 49% of cases, which is typical for Type 2 AIH. Indicators of vascular-platelet hemostasis can be used to assess autoimmune activity in children with Child–Pugh class A cirrhosis: VWF activity 200.0% is associated with an increase in antinuclear antibodies (ANA) in the blood (sensitivity – 83%, specificity – 69% ). Conclusion: indicators of vascular-platelet hemostasis can be used to assess autoimmune activity in children with class A cirrhosis according to Child–Pugh in the outcome of AIH.