The incidence of myasthenia gravis which is characterized by progressive muscular weakness on the background of structural disorders of the thymus, has increased. Myasthenia gravis is a multifactorial autoimmune disease, it has a pronounced clinical heterogeneity, and therefore the standard diagnostic and treatment protocol is not always effective. To substantiate an individual approach to the treatment of various clinical forms of myasthenia, we conducted a study of mechanisms and markers of loss of central and peripheral self-tolerance in thymus-independent myasthenia (M) and thymus-dependent myasthenia gravis with thymus hyperplasia (MH) and thymoma (MT), involving a total of 427 patients examined. In patients with different phenotypes of myasthenia, we used the methods of spectrophotometry, flow cytometry, enzyme immunoassay. In patients with MH on the background of lymphofollicular thymus hyperplasia we revealed a pronounced humoral sensitization in comparison with the reference values: the concentration of C4 complement, C-reactive protein, circulating immune complexes and the initiation of an indirect autoimmune reaction a reliable increase in autoantibodies (AAbs) to the α1 and α7 subunit of subunit of nicotinic receptors (nAChR). In M and MT groups a high similar titer of AAbs to other epitopes was revealed: DNA, β2-glycoprotein I, membranes of intestinal and stomach cells, lung, liver, kidney cells. A pronounced blast-transforming response to the presence of the mitogen PHA was revealed in the MT group. In the MT group, a decrease in the content of CD4+ CD28+ co-stimulatory molecules and in the MH group, a decrease in СD4+ CD25+ Treg lymphocytes was revealed. Individual methods for correcting the loss of self-tolerance in patients with different clinical phenotypes of myasthenia were justified taking into account the use of immunosuppression, specific viral-neutralizing immunoglobulins and massive IgG immunoglobulin therapy, and the application of anti-inflammatory recombinant interleukins.

Summary. Goal. Investigation of the interactions of coagulation, anticoagulant and fibrinolytic systems with factors of immunoresistance in hepatosplenomegaly syndrome (SHSM). Materials and methods. Materials — сells and blood serum of 58 patients with SHSM against the background of liver cirrhosis complicated by portal hypertension, with the etiological factor — HCV / HBV virus infection (group I, 22 people) and the etiological factor — CMV / VEB virus infection (group II, 36 people), who were admitted to the hospital for bleeding from esophageal varicose veins. Methods - photometric on a biochemical analyzer Stat Fax 1904 Plus (USA). (C3 and C4 components of complement, antithrombin III and plasminogen, concentration of circulating immune complexes (CIC), determination of the coagulation time of venous blood Lee-White, calculation of the prothrombin index, fibrinogen content by the Rutberg gravimetric method. Protein C activity (PrS) by the clotting method on a coagulometer K 3002 Spectramed (Poland). Peripheral blood platelet counts were performed using immersion microscopy according to the Fonio method. Results. Multidirectional changes in the functions of the hemostasis system were revealed: a decrease in antithrombin III activity, protein C content, fibrinogen concentration, a decrease in plasminogen activity, a decrease in platelet counts, an increase in platelet antibodies, an increase in the concentration of the C3 component and a decrease in the C4 component of complement. Conclusions. Hemorrhagic and thrombotic complications of HCV, life-threatening and affecting the tactics and results of surgical and minimally invasive treatment, can occur both in the HCV group on the background of HBV/HCV viral hepatitis, and in the HCV group on the background of herpes virus CMV/VEB infection, but in group I both hemorrhagic and thrombotic complications were dominated by plasma risk factors for and in group II - platelet and immunological (complement component C3) risk factors for hemorrhagic complications, plasma factors of thrombotic complications.

Summary. Purpose: to identify the features of lipid metabolism in patients with hepatosplenomegaly syndrome (HSS) of various origins to develop an individualized approach to the treatment of patients with this pathology. Materials and methods: blood serum of 73 patients with HSS on the background of liver cirrhosis complicated by portal hypertension, divided into 3 groups 1) with the etiological factor of hepatitis viruses HCV / HBV, 2) with autoimmune hepatitis with the etiological factor of the herpes group viruses CMV / VEB, 3) against the background of liver hepatosis and fermentopathies. Results and its discussion. In patients with HSS, multidirectional changes in the synthesis of lipid fractions were observed, also associated with impaired protein metabolism, namely: - with HSS against the background of viral hepatitis B and C, hypocholesterolemia and hypobetacholesterolemia were observed due to a decrease in cholesterol synthesis in hepatocytes; a decrease in the concentration of HDL is also associated with a violation of the process of cholesterol esterification due to a deficiency of the enzyme lecithin cholesterol acyltransferase, and an increase in the concentration of LDL is associated with a deficiency of the enzyme lipoprotein lipase in viral damage to hepatocytes. - with HSS against the background of autoimmune hepatitis, no significant changes were observed in the synthesis of lipid fractions; - in hepatosis (fermentopathies), type 5 hyperlipidemia with hypercholesterolemia and hypotriglyceridemia was found, which are a consequence of impaired formation and excretion of lipids from the liver, probably as a result of a decrease in the synthesis of apoproteins and indicate the accumulation of fat in hepatocytes and the formation of hepatosis. Conclusion: Surgical or endovascular treatment of HSS against the background of liver cirrhosis or hepatosis needs to be supplemented with individualized metabolic drug therapy, including lipid-lowering therapy. When detecting hypocholesterolemia and hypobetacholesterolemia in the first group, lipid-lowering therapy is not indicated, whereas if type 5 hyperlipidemia with hypercholesterolemia and hypotriglyceridemia is detected in the group of patients with fatty hepatosis, lipid-lowering therapy is necessary.

Summary. Aim. To study the condition of the phagocytic part of immunity in cholangitis. One of the main ways of elimination of bacterial contamination of the body is the work of the immune system, namely phagocytosis. This especially applies to acute and chronic cholangitis. Materials and methods. Depending on the magnitude of pathological changes in the biliary system, we have identified three groups of patients in whom cholangitis occurs in conditions that progress and worsen. Group I – cholangitis in conditions of choledocholithiasis, group II – cholangitis in conditions of iatrogenic strictures of the main bile ducts, and group III – cholangitis in conditions of already performed reconstruction of the duct system. The functional activity of phagocytic cells was studied. Results and their discussion. It was established that the functional capabilities of neutrophils are depleted in proportion to the severity of cholangitis and its duration. In case of chronic undulating cholangitis, there is an insufficiency of the phagocytic link, phagocytes work on the verge of exhausting their reserve capabilities. Adhesion forces and the potential of intracellular lysosomal enzymes are especially affected. Conclusions. The functional capacity of phagocytic cells in cholangitis is characterized by a deficiency, the degree of virulence is to lie down in the severity of inflammation and thrive. The greatest manifestations of changes were revealed in acid-independent mechanisms of phagocytosis, which were characterized by impaired adhesion and the release of intracellular lysosomal enzymes.

Summary. A range of specific biomarkers to evaluate the severity of the condition, the choice of tactics, effectiveness and prognosis of treatment are used. Goal. Evaluation of biomarkers characterizing the etiopathogenetic disorders in hepatosplenomegaly syndrome complicated by recurrent bleeding from esophageal phlebectasis to select individual treatment tactics. Materials and methods. The digestive function of phagocytes is investigated, the concentration of opsonizing, membranotropic cytotoxic factors and the degree of endogenous intoxication in 3 groups of patients with different etiological factors of hepatosplenomegaly, complicated by bleeding are evaluated. Results. The presence of specific trigger factors (viral hepatitis, herpes viruses, hereditary-related disorders of lysosomal enzymes) made it possible to classify patients into three groups. In the first group of patients with viral hepatitis B and C the induction of phagocytic activity of neutrophils, a significant increase in lymphocytotoxicity on the background of disorders of protein and lipid metabolism showed. In the second group of patients with the presence of herpes group viruses and autoimmune component an imbalance of opsonizing factors revealed, in particular proteins of the complement system, which caused impaired clearance of low molecular weight immune complexes and products of their degradation. In the third group of patients with hepatitis a significant decrease in the activity of lysosomal enzymes of phagocytic cells are found. The identified pathogenetic biomarkers indicate the presence in this group of patients with congenital enzymopathy characteristic of accumulation diseases. For patients with a pronounced manifestation of intercellular interactions disorders, as an effective alternative to liver transplantation the use of transfusions of mesenchymal stem cells, which are capable of trans differentiation under the influence of mediator microenvironment factors may be.