Hepatorenal syndrome is a life-threatening complication of liver cirrhosis. 90% of patients with liver cirrhosis die within 2 months since the onset of hepatorenal syndrome development. For many years the hypothesis of the hemodynamic mechanism of hepatorenal syndrome development was accepted as the only true one, according to this hypothesis compensatory systemic vasodilation in response to portal hypertension causes renal ischemia and the development of functional specific acute kidney damage, the so-called “hepatorenal syndrome – acute kidney damage”. In recent years some works were published that substantiate the role of a systemic inflammatory reaction in the development of hepatorenal syndrome; this inflammatory reaction being associated with activation of innate immunity cells in response to a bacterial infection, including that to the microflora of the intestine which is adjacent to the liver. Data has been obtained which indicated that Toll-like receptors (TLRs), in particular TLR4 and TLR9 are involved in the development of hepatorenal syndrome.

Background:Primary immunodeficiency diseases (PID) occur on average with a frequency of 1: 100 000 of the population. In accordance with the classification of PIDS of the International Union of Immunological Societies (IUIS) 9 different groups of innate errors of immunity are distinguished [1]. The polymorphism of the clinical picture, difficulties in recognizing PID cause their late diagnosis and the associated development of irreversible organ damage, complications and high mortality. According to the traditional view, the clinical marker of PID is an infectious syndrome. However, in some cases, PID is detected as a rheumatologic disease.Objectives:The aim of the study is to analyze the frequency of rheumatologic diseases in adult patients with PID living in one of the subjects of Russia –Chuvashia.Methods:The material of the study was patient data obtained during a retrospective analysis of 49 outpatient records included in the Republican Register of PID. Diagnosis of various forms of PIDS was carried out in accordance with the criteria developed by the IUIS [1].Results:During the period from 1993 to January 2020, 49 cases of PID were registered in the adult population of Chuvashia. According to the frequency of PID, сommon variable immunodeficiency (CVID) is the most common in Chuvashia (26 people). In the second place there is selective IgA deficiency (10 people); in third place there are X-linked agammaglobulinemia (4 people) and hereditary angioedema (4 people). The remaining forms of PID account for 5 cases: 2 cases of Louis-Bar syndrome, 1 case of DiGeorge syndrome, 1 case of Wiskott-Aldrich syndrome and 1 case of Hyper IgE syndrome. The main symptom of PID in 35 (71.4%) patients was heightened susceptibility to infection. In 14 (28.6%) patients, the clinical picture was dominated by non-infectious presentations (autoimmune, lymphoproliferative and oncological diseases). In 9 patients (18.3%), it was manifested by rheumatologic diseases (rheumatoid arthritis, psoriatic arthritis, scleroderma-like syndrome). These symptoms were more characteristic of CVID. With selective IgA deficiency and X-linked agammaglobulinemia, rheumatic symptoms were observed only in isolated cases. CVID debuted in 2 cases as rheumatoid arthritis, resulting in the delay in the diagnosis of an average of 5.2 years. Only the detailed immunological and genetic study made it possible to diagnose PID and prescribe adequate treatment – replacement immunoglobulin therapy. This treatment reduced the frequency of infectious manifestations of the disease and it did not significantly improve the course of rheumatological diseases. Therefore, the use of methotrexate and targeted therapy was continued.Conclusion:In the clinical picture of PID, rheumatic symptoms predominate in 28.6% of cases, which requires a thorough immunological and genetic examination of patients with rheumatic diseases in order to diagnose PID in a timely manner.References:[1]Picard C, Bobby Gaspar H, Al-Herz W, et al. J Clin Immunol. 2018;38(1):96-128.Disclosure of Interests:None declared

Despite the fact that the general clinical manifestations of COVID-19 are well known, there remain problems associated with the impact of COVID-19 on human health, in addition to its effects on the respiratory system. Patients with COVID-19 and concomitant cardiovascular diseases are more likely to be hospitalized and to pass treatment in the intensive care units and to have worse prognoses. The article discusses the problems arising from the effect of type 2 coronavirus acute respiratory syndrome (SARS-CoV-2) on the cardiovascular system, starting with the mechanisms associated with angiotensin converting enzyme 2 (ACE2) receptors, as well as discusses cases of major pathological changes in the heart and blood vessels that are detected in these patients. In addition to the known risk factors for severe COVID-19: cardiovascular diseases, diabetes mellitus, chronic lung diseases and old age, even young patients without a history of risk factors may develop myocardial damage. We present a description of a clinical case of acute myocardial infarction against the background of a new coronavirus infection COVID-19 in a patient aged 28 years with a diagnosis of coronary heart disease.

In recent years, primary immunodeficiencies have turned from the class of rare diseases to the category of more common disorders which may be encountered by doctors of any clinical discipline. The first case of primary immunodeficiency disorder (PID) in Chuvashia was detected in 1993. Since that time, the Department of Internal Diseases with the Course of Clinical Immunology at the I. Ulyanov Chuvash State University registered all the cases of PID diagnosed in the region, introducing them into the Republican Registry of PID. The study was aimed for searching epidemiological indexes, clinical and laboratory manifestations of PID in Chuvash region. The study was based on the patient data obtained by retrospective analysis of 85 case histories of PID patients, treated at different departments of the Republican Clinical Hospital, and the City Chuvash Pediatric Clinical Hospital of Public Health Ministry in 2000-2019, as well as on 49 outpatient records of the patients included into the Regional PID Registry. Various forms of PIDs were diagnosed according to the criteria developed by the European Society for Immunodeficiency and the Pan-American Group on Immunodeficiency (1999). The results of this study showed that the incidence of PID in the Chuivash Region is 3.4:100,000. The incidence of common variable immune deficiency (CVID), the most common form of PID in the region, was 1.58 per 100,000 population. The average age at the time of CVID diagnosis in Chuvash patients was 30.4±16.1 years, and the age of CVID debut was 11.3±15.0 years. The delay in proper diagnosis from the moment of clinical manifestation of CVID was, on average, 17.9 years in the region. At the time of CVID diagnosis, the patients showed marked decrease in the levels of 3 or 2 immunoglobulin classes (IgG and IgA), and T-helper cell contents (CD3+CD4+) in peripheral blood. Prevalence of selective IgA deficiency with сlinical symptoms was 0.83 per 100,000 population of the region, and the incidence of the asymptomatic form of this PID was 1 : 167. In patients with selective IgA deficiency, there were also disorders in the T cell system manifesting as decreased relative number of cytotoxic T-cells as well as elevated IgG and IgM levels. The age of diagnosis of X-linked agammaglobulinemia in the region was 3.5±3.0 years. In addition to disturbances of humoral adaptive immunity in children with this disease, a decrease in absolute T cell numbers was detected. In conclusion, the article describes disturbances of postvaccinal immunity in a pregnant patient with CVID, with asymptomatic clinical course, thus leading to false interpretation of the serological markers of TORCH infections and wrong strategy of pregnancy management.

A clinical case of systemic lupus erythematosus (SLE) is presented. Since childhood the patient had preclinical lupus: leukopenia, anemia, photoallergy, vasculitis, and serological changes a-SS-A (+++), but at that time the findings did not meet the criteria for SLE. At the initial examination of the woman at the age of 24 years, a clinical blood test revealed hemoglobin 101 g/l, leukopenia 3.2×109/l, ESR 41 mm/h, hyper-γ-globulinemia 29%. The immunological blood analysis revealed a-DNA (+++), a-SS-A (+++), aSm (+++), aRNP (+++). The diagnosis was "Systemic lupus erythematosus of chronic (at the beginning) course, activity of the 1st degree (2 points by the SLEDA scale): with hematological disorders (leukopenia), immunological disorders (ANA+)". Metypred was administered at a dose of 16 mg/day, followed by the dose reduction to 6 mg/day and Plaquenil 400 mg/day. The woman received inpatient and outpatient treatment, repeatedly consulted in the Federal State Budgetary Institution "V.A. Nasonova Research Institute" under the Russian Academy of Medical Sciences. At the age of 26, the diagnosis was made: "Systemic lupus erythematosus, grade 2 activity with skin lesions of the type of subacute cutaneous lupus, cheilitis, lymphadenopathy", blood tests revealed leukopenia, lymphopenia, anemia, as well as immunological disorders: a-n DNA 93.4 u/ml, aSm > 200 u/ml, aRo > 200 u/ml, C3 0.63 g/l, rheumatoid factor 69.5 mIU/ml, ANA (Нер-2) 1/640 Sp. A complex therapy was performed with the use of Metypred, Cyclophosphane and Azathioprine. Against the background of the therapy, the patient's condition is stable, but immunological disorders are preserved: persistent high positivity for aRo-SS-A and the presence of the rheumatoid factor. The case demonstrates the need for an in-depth examination of women with anemia of unknown origin in combination with leukopenia to exclude systemic diseases of the connective tissue.