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Introduction. Myasthenia gravis (MG) or Erb-Goldflam syndrome is an autoimmune neuromuscular disease based on autoaggression of humoral immunity against elements of neuromuscular synapses and intracellular structures of muscle fibers (antibodies to acetylcholine receptors (AchR), muscle-specific tyrosine kinase (MuSK), protein 4 related to low-density lipoprotein receptors (LRP4), ryanodine receptors (RyR), titin, and skeletal muscle antigens). The exact causes of autoimmune processes in MG are unknown, but abnormalities of the thymus gland (hyperplasia and neoplasia), especially in patients with antibodies to AchR, as well as genetic predisposition are of indisputable importance [1]. Aim. To analyze the researches of pathogenesis, diagnosis and treatment for patients with severe MG from latest literature sources to optimize the treatment of this group of patients. To analyze the own experience of implementing a patient-oriented approach to the treatment and rehabilitation of MG. Materials and methods. Bibliosemantic, comparative and system analysis methods, and clinical and neurological examination of patients with MG. Results. The course of MG is variable and can be represented by episodic muscle weakness, stationary flow, slow or rapid progression [2]. Currently, the complex therapy of patients with MG has a clear tendency to move from symptomatic (acetylcholinesterase inhibitors, potassium preparations, potassium-sparing diuretics) treatment to pathogenetic, mainly etiopathogenetic concepts of therapy are being formed (immunosuppressive therapy, antigen-specific immunotherapy, monoclonal antibodies), and it is also being clarified effectiveness of early thymectomy [3]. Conclusions. Complex therapy of patients with MG is based on the use of a differential diagnostic algorithm for various pathogenetic variants of MG, which forms a mainly etiopathogenetic concept of therapy. Antigen-specific immunotherapy, aimed at restoring tolerance to the attacked autoantigen by targeting only the damaged part of the immune system, while leaving the rest intact, is considered more promising for the treatment of MG. MG treatment should be carried out in the conditions of a specialized neurological center, where a full examination and targeted pathogenetic therapy can be carried out.

We present a case of mechanical hemolysis as a complication of extracorporeal membrane oxygenation (ECMO) occurring in a COVID-19 patient as a result of pump head thrombosis. After emergency extracorporeal circuit replacement, hemoadsorption was initiated to address the negative hemolysis effects and plasma free hemoglobin rise in the setting of rapid clinical deterioration and impaired renal function. During therapy hemolysis severity reduced, the lactate dehydrogenase (LDH) levels decreased, while the P/F ratio increased two-fold. The patient was discharged from hospital on day 54 without the need for either oxygen therapy or dialysis. In the discussion section we addressed frequent issues of choosing therapy for ECMO complications.Conclusion. The timely, properly chosen, and clinically relevant use of hemoadsorption combined with advanced high-technology therapeutic procedures can have a positive impact on the patient's outcome.

Rhabdomyolysis is a syndrome caused by destruction and necrosis of muscle tissue, which is accompanied by the release of intracellular contents into the systemic circulation. The etiology of rhabdomyolysis is multifaceted, however, regardless of the etiological factor, the central element of its pathophysiology is systemic endotoxemia with multiple organ failure syndrome. Acute renal failure is one of the most common manifestations of organ dysfunction. Considering the pathogenetic model of the development of systemic endotoxemia, the timely use of extracorporeal therapy, which reduces mortality in organ failure, seems promising. All the current types of extracorporeal therapy can be divided into convection (hemofiltration), diffusion (hemodialysis), convection/diffusion (hemodiafiltration), sorption (hemoperfusion) and plasma exchange (plasmapheresis, plasma exchange, plasma sorption, etc.) methods based on physical principle.The aim of the review was to summarize the available clinical data on extracorporeal treatments for rhabdomyolysis and to assess the feasibility and best indications for these methods based on the current pathogenetic model of rhabdomyolysis.Material and methods. The search for information was carried out in the Web of Science, Scopus, Medline, PubMed, RSCI, E-library and other databases. Eighty-one sources were identified containing current therapeutic approaches and relevant data of clinical and scientific research on the subject of this review.Results. In this review, the main etiological, epidemiological and pathogenetic models of acute renal injury in rhabdomyolysis have been discussed. The main methods of extracorporeal therapy have been reviewed and evaluated based on current understanding, and latest clinical data on their effectiveness have been summarized.Conclusion. The choice of the optimal extracorporeal treatment method, the time of initiation and duration of the procedure still remain controversial. The solution to this issue can potentially help to better correct the electrolyte disturbances and could protect against organ dysfunction, which would improve the outcome in patients with rhabdomyolysis.

The purpose of the research to create a concept for diagnostics and therapy of hemostasis disorders of patients with severe leptospirosis.Patients and Methods. The study included 474 patients with severe serologically confirmed leptospirosis with a favorable outcome of the disease and 31 patients with fatal outcome (total 505 people). Variant of coagulopathy was determined by using a set of special methods for studying hemostasis. The influence of platelet concentrate transfusion, plasma exchange and various tactics of glucocorticoid therapy on patient survival and correction of hemostasis disorders was evaluated. Survival analysis was made using Kaplan-Meyer method with a Cox proportional intensity model. Relative risk (RR) was calculated with a 95% confidence interval (CI).Results: the use of early diagnosis of coagulopathy variants and the use of a differentiated therapy regimen in the choice of the variant of hemostasis disorder led to a decrease of mortality from 16.45% to 11.5% and decrease in the consumption of platelet concentrate and fresh frozen plasma.Conclusion: patients with severe leptospirosis develop a multivariate hemostatic pathology: isolated thrombocytopenia (38%) with thrombotic microangiopathy (20,5%), disseminated intravascular coagulopathy (37,1%), uremic coagulopathy (4,9%), hepatic coagulopathy (3,4%). Plasma exchange in thrombotic microangiopathy is pathogenetically justified. Also, plasma exchange is pathogenetically justified in order to reduce plasma volume in DIC syndrome with consumption coagulopathy and hepatic coagulopathy. The use of GCS in isolated thrombocytopenia can be effective and safe in both “medium doses” and in the form of “pulse therapy” if the following conditions are met: acute renal injury (AKI) III stage according to AKIN and the absence of renal replacement therapy (RRT). The main indication for platelet concentrate transfusion in severe leptospirosis is extremely severe thrombocytopenia (grade 4) with active life-threatening bleeding at the time of transfusion.

In patients with progressive severe acute pancreatitis (SAP), early surgery is the last opportunity to influence the dismal outcome. The use of extracorporeal detoxification (ECD) methods along with a traditional surgical approach does not improve the outcomes of early surgeries in patients with progressive SAP due to the late elimination of aggression factors, as well as due to constant reabsorption of enzymatic toxic exudate from non-drained areas of the retroperitoneal space, abdominal cavity, and omentum. Preventive use of ECD (plasma exchange) can improve the outcomes of early surgeries in patients with fulminant SAP.

The objective: to provide pathogenetic justification for the need for extracorporeal blood purification in the new coronavirus infection.Results: the article analyzes guidelines and the first experience of using extracorporeal methods in the new coronavirus infection in different countries. It has been demonstrated that methods of extracorporeal blood purification have complex pleiotropic (multiple) effects and can quickly normalize the level of cytokines and other pathogenic factors, thereby preventing/reducing the severity of organ disorders. The article specifies the criteria for choosing an extracorporeal method, as well as indications for its early and late use.

COVID-19, formerly coronavirus infection 2019-nCoV, is a potentially severe acute respiratory infection caused by the SARS-CoV-2 coronavirus (2019-nCoV). It is a dangerous disease that can occur both in the form of a mild acute respiratory viral infection and in a severe form with the development of bilateral polysegmental viral pneumonia, specific complications of which may include acute respiratory distress syndrome, resulting in the respiratory failure with a high risk of death. Due to the absence of etiotropic therapy for the coronavirus infection, pathogentic treatment becomes of primary importance. We present a clinical case of a severe form of COVID-19 in a 33-year-old man to whom we administered a new method of pathogenetic treatment of this disease based on immunosuppresiive therapy followed by therapeutic plasma exchange and infusion of non-specific human immunoglobulin.

Introduction: Myasthenia gravis is an autoimmune disease of the postsynaptic part of the neuromuscular junction. The disease is chronic, manifesting itself in the fatigue of various muscle groups, and the first manifestation of the disease often affects the muscles that move the eyeball and eyelid, although MG can lead to weakness of any muscle group. In turn, myasthenic crisis is a severe worsening of muscle weakness that often results in the urgent need of mechanical ventilation of the patient, due to the respiratory failure. Case reports: The first patient, 64 years old, female was admitted to the Neurology Department due to breathing difficulties and weakness of the limbs, which suddenly worsened within a dozen of hours before admission. During the stay, steroid therapy was used, as well as six times plasma exchange took place at the Nephrology Department with good treatment tolerance. The dosage of pyridostigmine was also modified, with no adverse effects. During the stay, the patient's condition improved - respiratory disorders resolved, ptosis was significantly withdrawn, and limb muscle tension increased globally. The second patient, aged 67, also female, with a history of myasthenia gravis as well, was admitted to the Neurology Department due to increased fatigue, ptosis, dysphagia, and difficulties in speaking and breathing. Steroid therapy was used, but due to high blood glucose, the steroid dose was reduced and a 2-day cladribine treatment was initiated, with good tolerance. Discussion: Myasthenic crisis is often a life-threatening condition that requires immediate treatment. Steroid therapy, pyridostigmine, IVIG and plasma exchange are standard approaches in myasthenic crisis. Cladribine is not an ordinary approach, but can be beneficial in some patients.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous immune-mediated peripheral neuropathy with progressive or relapse-remitting course. Incidence of CIDP ranged between 1 and 8.9/100 000. Recently, most frequent therapies for CIDP treatment was glucocorticosteroids, intravenous immunoglobulin and plasma exchange. In cases of ineffectiveness or lack of effectiveness, cytostatics, monoclonal antibodies and others could be used for CIDP treatment. In the article, authors presented an update data on the use of main methods for CIDP therapy, their mechanisms of action, indication for their use and advantages and disadvantages of each of them.

The article reveals medical and social relevance of extreme familial hypercholesterolemia. It presents the main principles, innovative technologies and an algorithm of diagnosis, diagnostic verification, risk stratification of patients and optimization of the use of aggressive combined cholesterol-lowering drug treatment and precision of indications for plasma exchange and the lack of options.

Due to the fact that acquired myasthenia gravis is an autoimmune disease, so rational treatment of these patients includes the symptomatic therapy (acetylcholinesterase inhibitors, potassium supplements, potassiumsaving diuretics), short-term immunotherapy (intravenous immunoglobulin, methods of extracorporeal hemocorrection), longterm immunotherapy (glucocorticosteroids, cytotoxic agents, targeted therapies), thymectomy, as well as a number of new promising therapeutic agents. The choice of effective therapy depends on identifying the clinical forms of myasthenia gravis and a complete analysis of autoimmune mechanisms underlying the disease because of similar clinical forms of myasthenia gravis may respond to similar therapies.

Aim. To improve the efficiency of treatment of the patients with pancreatic necrosis through correcting the platelet-coagulation potential by methods of combined plasma filtration, selective sorption of cytokines with hemofiltration and plasmapheresis. Methods. The effects of selective sorption of cytokines with hemofiltration and plasmapheresis upon coagulation potential were studied in 70 patients with acute pancreatitis. Plasmapheresis was performed on PCS 2 (Haemonetics, USA); selective sorption of cytokines with hemofiltration – on Lynda ® machine (Bellco, Italy). The method of computer laser phasemeter was used for vital assessing of platelet morphofunctional status. Results. In patients with pancreatic necrosis in the fermentation phase the percentage of resting platelets was 51.3%, which is 12% below the control numbers. The number of activated platelets increased: 25.1% of the cells were represented by platelets with low activation; 15.5% – highly activated cells. The number of degenerative-modified platelets increased by two times – up to 8.1%. In the phase of sequestration and suppurative complications the percentage of resting platelets was only 42.3%, which is 1.5 times lower than the control numbers. 34.2% of the cells were presented by platelets with low activation; 11.0% – highly activated cells. The number of degenerative-modified platelets exceeded 12.5%. The analysis of the morphological structure of the platelets population revealed that due to plasmapheresis inclusion into therapeutic complex in the phase of intoxication of pancreatic necrosis the normalization of platelet activation status of hemostasis was observed. In 5 (20%) patients the normalization of average platelets morphometric parameters was shown after selective sorption of cytokines with hemofiltration and plasmapheresis. These patients demonstrated positive clinical dynamics. In 15 patients morphometric values of platelets either remained at the same value or continued to increase progressively. In this subgroup of the patients purulent complications progressed in 9 patients, mortality was 35%. Conclusion. Thus, the level of thrombocyte hemostasis in different phases of pancreatic necrosis can be quantified using indicators of morphofunctional status of circulating platelets. When dealing with coagulation version of disseminated intravascular coagulation syndrome the number of activated cells is on the rise up to 40% or more. The development of consumption coagulopathy is characterized with progressive increase of degenerative platelets up to 10% or more. Combined plasma filtration, selective adsorption of cytokines with hemofiltration and exchange plasmapheresis are effective methods for adjusting of platelet and coagulation hemostasis in patients with necrotizing pancreatitis.

The review discusses approaches to treatment of congenital thrombotic thrombocytopenic purpura (TTP) or Upshaw-Schulman syndrome. In congenital TTP, plasma transfusions are sufficient. Such treatment options as plasma exchange, administration of clotting factor VIII concentrate, recombinant ADAMTS13, are also used. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction. The pathogenesis of TTP is attributed to the deficiency in the activity of the metalloproteinase ADAMTS13, specific von Willebrand factor cleaving protease. TTP is suspected when detecting microangiopathic hemolytic anemia, thrombocytopenia, damage to various organs. Diagnosis of TTP is confirmed by the detection of ADAMTS13 activity in plasma less than 10%. Plasma samples for the study of ADAMTS13 activity should be taken before the start of plasma transfusions or plasma exchange. In patients with severe ADAMTS-13 deficiency autoantibodies anti-ADAMTS13 and inhibitor ADAMTS13 should be investigated. Anti-ADAMTS13 antibodies belonging to IgG not always have inhibitory effects. The inhibitory effect of anti-ADAMTS13 antibodies is confirmed by mixing test. All patients with the first established diagnosis of TTP should be examined for mutations of the ADAMTS13 gene.

Anticoagulant therapy with vitamin K antagonists (AVK) is an effective treatment and prevention of thrombosis. One of the major disadvantages of the AVK is a risk for serious bleeding. Prothrombin complex concentrates (PCC), fresh frozen plasma (FFP) and vitamin K1 are available for control of these situations. The experience of special team ofthe Scientific Center for Hematology was the basis for presented retrospective study. Three regimens of warfarin-related bleeding were compared: PCC+ VK for several bleeding, FFP+ VK for different clinical situations and VKfor light bleeding. PCC showed himself as effective and safe hemostatic agent. Transfusions of FFP were sometimes not effective, sometimes led to TACO. Supplementation of vitamin K1 for patients of I and II groups provided more stable control of hemostasis. In III group VK vas effective to stop bleeding. Two impotent sings for conclusion: necessary of laboratory monitoring, TEG first of all; individual balance of hemostasis base of bleeding or thrombotic risks.

The structure of surgery of pituitary adenomas has been fundamentally changed over the past decade. The transnasal transsphenoidal approach is currently used to resect more than 95% of adenomas. The wide use of endoscopy has changed the need for perioperative control of intracranial pressure. While controlled intracranial hypertension was previously used during microscopically controlled transnasal surgery to bring the suprasellar capsule and tumor remnants to the field of vision, endoscopically controlled tumor resection implies that controlled intracranial hypotension is required to achieve complete spreading of the suprasellar capsule. External lumbar drains inserted at least for the entire surgery duration are conventionally used to control intracranial pressure. We present the results of the study proving the effectiveness and safety of the HyperHAES hypertonic isooncotic plasma substitute in achieving stable intracranial hypotension according to a large body of clinical data. Our findings give grounds for claiming that the use of the HyperHAES hypertonic isooncotic plasma substitute during the standard adenomectomy allows one to abandon the use of invasive external lumbar drainage, while the surgery quality and risks remain at the same level.

Changes in the hemostasis system in choledocholithiasis, taking into account the obturation jaundice severity and possibility of the correction conduction, using miniinvasive operative interventions, were studied. Dynamic of changes in the hemostasis system in patients preoperatively, in 1 and 3 days after endoscopic papillosphincterotomy were monitored, using the method of a low--rate piezoelectric thromboelastography. Basing on analysis of the results, the changes in hemostasis in obturation jaundice were classified, taking into account its degree of severity, what have permitted to select a correct tactics for prophylaxis of hemorrhagic complications intraoperatively and postoperatively.

We presented the results of our research in comparison with the literature on the etiology, pathogenesis and diagnosis of spontaneous bacterial peritonitis (SBP) at liver cirrhosis (LC), complicated by ascites. Based on these data, was proposed a classification of SBP. Were identified criteria for early diagnosis of SBP on the basis of clinical manifestations of bacterial peritonitis, systemic inflammatory response, results of studies of ascitic fluid (AF) using gas chromatography-mass spectrometry (GC-MS). In accordance with the proposed criteria was proposed a scheme for the diagnosis, treatment and prevention of STDs. Presents data on the effectiveness of therapy and prevention of SBP. Denote the potential major long-term trends in the treatment of SBP in cirrhosis.

The paper describes a case of practically simultaneous development of the hemolytic-uremic syndrome (HUS) and the catastrophic antiphospholipid syndrome (CAPS) complicated by mesenteric vessel thrombosis and small bowel necrosis. Multimodality treatment comprising volume plasmapheresis, fresh frozen plasma transfusion, hemodialysis, anticoagulant and disaggregant therapy could relieve thrombogenic events, such as pulmonary artery thromboembolism and intestinal necrosis.

There were examined 34 children, in whom systemic inflammatory answer syndrome (SIAS) was diagnosed. In 16 children pathogenetically substantiated immunocorrection, using donor's plasma, enhanced by antibodies (AB) to endotoxin (ET), was conducted; in 18 children--the conventional complex therapy. While presence of gram-negative infection in children, suffering SIAS, the activation o the inborn nonspeciphic factors (LBP, sCD14) and speciphic low-affinic AB (anti-ET-IgM, anti-ET-IgA) the antiendotoxin immunity in combination with selective immunodeficiency of anti-ET-IgG were revealed. While the donor's plasm application, enhanced by AB to ET, the titer of anti-ET-IgG, anti-ET-IgM, anti-ET-IgA and LBP have had achieved normal values and did not differ from such in the control on the 14th day.

The paper analyzes the results of treatment in victims with the crush syndrome under the conditions maximally close to those of emergency situations. It is concluded that plasma exchange procedures should be performed within the next few hours after taking the victims from the obstructions, which reduces the risk of acute lung damage and death rates in this group of patients to a considerable extent.

Diminution of reperfusion and re-oxygenation lesions is an important task in the treatment of patients with severe injury and blood loss. From this standpoint the influence of perforan on the condition of the oxidant-antioxidant system was studied in patients with severe combined trauma. The application of perftoran in the early post-trauma period was found to intensify the processes of lipid peroxidation and, simultaneously, to normalize the parameters of the antioxidant-protection system.

Aim: To compare therapeutic effectiveness of cryosupernatant plasma fraction (CSP) and fresh-frozen plasma (FFP) in long-term infectious-septic DIC syndrome arising in acute abscess and gangrene of the lung., Materials and Methods: 106 and 131 patients with infectious-septic DIC syndrome were treated with CSP and FFP, respectively. The results of the treatment were compared clinically and hemotopogically., Results: Clinical response to both treatments was evident from positive changes in XIIa-dependent fibrinolysis, lowering of fibrinogen level and enhanced activity of antithrombin III. CSP treatment brought about a decrease in the number of thromboses, lethal cases, unfavorable outcomes., Conclusion: The cryosupernatant can be used instead of fresh-frozen plasma in combined treatment of long-standing infectious-septic DIC syndrome.

Time course of the immunity system parameters was followed in children suffering from grave endotoxicosis forms treated by discrete plasmapheresis (DPA) as the emergency therapeutic measure. T, T-active, B lymphocytes, O cells, immunoglobulins A, G, M were analyzed, as were the counts of phagocytizing leukocytes and circulating immune complexes. The infectious process was found to lead to a temporary relative secondary immunodeficiency. DPA with perfusion of hyperimmune plasma resulted in rapid correction of all component of the immunity system, paralleled by normalization of the general clinical status of the patients, and prevented chronic transformation of the process.

Extracorporeal detoxication techniques (hemosorption, plasmapheresis and plasma exchange) were used in a complex therapy of 55 patients with hemolytic disorders of different etiology. The results of clinical and laboratory studies confirm the efficacy of plasmapheresis. Skin surfaces and mucosa became less icteric, the size of the spleen and liver diminished, the levels of free hemoglobin, bilirubin and circulating immune complexes decreased, blood toxicity reduced. Unlike hemosorption, plasmapheresis and plasma exchange had no unfavourable effects on blood cells.