Introduction. Atypical hemolytic-uremic syndrome (aHUS) is a systemic disease from the group of thrombotic microangiopathies with a progressive course and unfavorable prognosis, which is based on the uncontrolled activation of the alternative complement pathway of hereditary or acquired nature, leading to generalized thrombosis in the microvasculature. The goal is to provide current clinical guidelines for the care of adult patients with aHUS. Basic information. Experts from the National Association of Nephrologists have developed current clinical guidelines for the provision of medical care to adult patients with aHUS. The recommendations discuss in detail the issues of etiology, pathogenesis, epidemiology and clinical manifestations of the disease. Particular attention is paid to the diagnosis, differential diagnosis and treatment of aHUS, based on the principles of evidence-based medicine.

Life participation requiring physical activity and physical function is a key patient-reported outcome for people receiving peritoneal dialysis (PD). Clinician guidance is required from multidisciplinary sources regarding exercise and activity advice to address the specific needs of this group. From August 2020 through to June 2021, the Global Renal Exercise Network and the International Society for Peritoneal Dialysis reviewed the published literature and international clinical experience to develop a set of clinical practice points. A set of questions relevant to physical activity and exercise were developed from the perspective of a person receiving PD and were the basis for the practice point development. The GRADE framework was used to evaluate the quality of evidence and to guide clinical practice points. The review of the literature found sparse quality evidence, and thus the clinical practice points are generally based on the expert consensus of people receiving PD, PD exercise expert clinicians and experienced PD exercise researchers. Clinical practice points address timing of exercise and activity (post-catheter insertion, peritoneal space empty or full), the uptake of specific activities (work, sex, swimming, core exercise), potential adverse outcomes related to activity and exercise (exit site care, perspiration, cardiovascular compromise, fatigue, intra-abdominal pressure), the effect of exercise and activity on conditions of interest (mental health, obesity, frailty, low fitness) and exercise nutrition.

Background: patients with atrial fibrillation (AF) require prescription of an anticoagulant, although this therapy is associated with an increased risk of bleeding, the risk of which is increased in the presence of chronic kidney disease (CKD). Aim: to investigate the level of podocyt damage (nephrin) markers and tubulointerstitial renal tissue damage markers (neutrophil gelatinase-associated lipocalin, NGAL; kidney injury molecule-1, KIM-1, albuminuria level) in urine in patients with AF and CKD stages 3 and 4, receiving apixaban, depending on the presence of bleeding. Methods: 142 patients with AF and CKD stages 3 and 4, receiving apixaban therapy, 58-99 years old (median age 84 84 [76; 90] years) were included in the study. Retrospective and prospective assessment of hemorrhagic complications was performed. The urine levels of nephrin, NGAL, KIM-1, and albumin were determined. Results: during prospective follow-up, patients with AF and CKD stages 3-4 with bleeding had significantly higher urinary nephrin levels compared with patients without bleeding (1.1 [0.8; 1.4] ng/mL vs. 0.9 [0.6; 1.2] ng/mL, respectively; p=0.049). In a retrospective analysis, urinary nephrin levels did not significantly differ in the patients in the presence or absence of bleeding history. Urinary levels of albumin, KIM-1, and NGAL were not significantly different between subgroups with and without bleeding. Conclusion: The results of the study indicate that there is a relationship between the level of a marker of podocyt nephrin damage in urine and the presence of bleeding in patients with AF and CKD stages 3 and 4 receiving apixaban. It can be assumed that this association can be explained by the fact that nephrinuria, which reflects the severity of podocyte damage and correlates with albuminuria, thereby, indirectly reflects an increase in the permeability of glomerular capillaries due to endothelial damage due to protein loss in urine.

Rationale: itching is one of the essential problems in patients with chronic kidney disease (CKD), including those receiving renal replacement therapy (RRT). For a comprehensive assessment of itching and its impact on various aspects of the quality of life in patients with CKD, questionnaires filled out by patients are used. Currently, there are no Russian versions of standardized instruments that have been tested on the domestic population of patients with CKD and are suitable for assessing the skin manifestations and their impact on the quality of life of this patient population. The aim of the study was to test the use of questionnaires for a comprehensive assessment of itching and various aspects of functioning in CKD patients receiving RRT, as well as validation of the Russian versions of the Itch MOS and Skindex-16 questionnaires. Methods: the Skindex-16, Itch MOS, 5D Elman scale, and WI-NRS scale questionnaires were tested in a group of adult patients with CKD who were on RRT during their hospitalization. To test Russian versions of the Skindex-16, Itch MOS, and 5D-Elman questionnaires, patients were interviewed in focus groups (n=5-8) to analyze the clarity and ease of filling them out by patients. Reliability was assessed by internal consistency (Cronbach's alpha coefficient) and by the test-retest method. Content, face, and convergent validity were evaluated. Also, specialists were interviewed to test the questionnaires. Statistical analysis was performed using the Student's t-test, paired t-test, or their nonparametric analogues. Spearman correlations were used to assess the relationship between the values of the questionnaire indicators. Results: the linguistic and cultural adaptation and validation of the Skindex-16 and Itch MOS questionnaires were performed. The Russian versions of the questionnaires were characterized by satisfactory reliability and validity. To test the use of the questionnaires among patients 100 patients with stage 5 CKD receiving HRT hemodialysis for 49.5 (Q1-Q3: 16; 79) months were included. 6 specialists took part in the testing of the questionnaires. All the questionnaires were shown to be convenient, informative, clinically interpretable, and appropriate for joint use. Conclusion: Russian versions of the Skindex-16 and Itch MOS questionnaires have been developed; their reliability and validity for assessing concerns associated with itching in patients with CKD undergoing hemodialysis have been demonstrated. The Russian versions of the Skindex-16, Itch MOS, 5D Elman, and WI-NRS questionnaires could be used in research and clinical practice to analyze various aspects of itching and its impact on quality of life in CKD patients receiving RRT.

Quality of life (QoL) of patients with immune-mediated glomerulopathies (IGP) in pre-dialysis stages of chronic kidney disease is scantily studied. The aim: testing the hypothesis on a relationship between QoL variables, clinical indices of IGP severity, and its “primary” or “secondary” etiology. Patients and methods: the study included 430 patients with primary IGP (IgA nephropathy, minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, membranoproliferative glomerulonephritis/С3 glomerulopathy) and secondary IGP: systemic lupus erythematosus, ANCA-vasculitis, systemic scleroderma, rheumatoid arthritis, plasma cell dyscrasias (amyloidosis/myeloma). All IGP cases were biopsy-proven and immunosuppression naive. For QoL evaluation, we used the Russian version of the Kidney Disease Quality of Life Short Form (KDQOL-SF) questionnaire that comprises general scales and kidney disease-specific scales. Routine clinical and demographic parameters were applied as independent variables in multivariable regression models. Results: QoL indicators were significantly lower in the secondary IGP group compared to the primary IGP. Female gender, an older age, a decrease in serum albumin, estimated glomerular filtration rate, and mean blood pressure as well as secondary IGP etiology and nephrotic syndrome were all independently associated with a decrease in the QoL. Clinical and demographic factors explained 3 to 34% of the variance in QoL in models applied. Conclusion: we confirmed a close association between the etiology of IGP, its clinical features, and QoL indices. Such association should be considered in the planning of personalized management of patients with IGP with baseline evaluation and monitoring of self-assessed QoL.

Thin basement membrane disease (TBMD) and autosomal dominant Alport syndrome (ADAS) are inherited renal diseases caused by structural abnormalities of α-3 and α-4 chains of collagen type 4. The concept that TBMD and ADAS are distinct conditions was initially proposed based on supposed clinical and morphologic differences. Most patients with TBMD have hematuria with little or no proteinuria, normal renal function, and diffuse thinning of the glomerular basement membrane (GBM) on electron microscopy. However, there are patients with TBMD who develop end-stage chronic kidney disease (ESKD) in adulthood. In contrast, Alport syndrome (AS) is defined as progressive nephropathy accompanied by hearing loss and ocular abnormalities, with irregular thickening and lamellation of the GBM. However, many patients with ADAS have no extrarenal manifestations, ESKD does not develop until old age, and the only change in the GBM is thinning. Genetic studies have proven that both TBMD and ADAS are caused by heterozygous pathogenic variants in the COL4A3 and COL4A4 genes. There are difficulties in differentiating between these two conditions and the old concept of two different diseases should be reconsidered. The diagnosis of AS is important because it facilitates close follow-up and early treatment of patients, whereas the diagnosis of TBMD may lead to an underestimation of the risk of developing ESKD. Although ADAS has long been considered an extremely rare condition, recent studies using next-generation sequencing have demonstrated that the number of these patients is much larger than previously thought, leading to much debate over the diagnosis of this condition. We present a review of available publications on TBMD and ADAS and our clinical observation demonstrating the difficulties encountered in diagnosing collagen type 4 pathology.

Introduction: infectious complications of permanent vascular access are a common cause of rehospitalization of patients on hemodialysis and can lead to loss of access in 20% of cases. Due to the limited vascular resources, it is necessary to introduce more modern and safe methods of treating this complication. Vacuum aspiration therapy has already established itself as a reliable and effective way to treat infected wounds in various areas of surgery. The use of vacuum therapy (VAC-system) promotes biological cleansing and reduction of bacterial contamination of the wound surface, has a positive effect on the regeneration capabilities of tissues, reduces intoxication and systemic inflammatory response, prevents secondary contamination of the wound with microbial flora, which reduces the time of wound healing and hospitalization of the patient. Clinical case: the article presents a clinical case of successful treatment of an infected wound of the upper third of the right shoulder in the area of the venous fistula of a vascular prosthesis using VAC therapy. Conclusion: infectious complications after the formation of AVF inevitably increase the duration of treatment and hospitalization and increase the risk of septic complications, thrombosis, and vascular erosion. Our experience has shown that treating infected AVF prostheses with VAC therapy is safe and allows for maintaining vascular access. Further research is needed to develop a more straightforward treatment algorithm.

Abstracts of the competition of the Association of Young Nephrologists, XXII North-Western Nephrology School of the Russian Dialysis Society

Abstracts of the XVIII Conference of the Russian Dialysis Society - Chronic Kidney Disease

Abstracts of the XVIII Conference of the Russian Dialysis Society - Nephrology

Abstracts of the XVIII Conference of the Russian Dialysis Society - Renal Replacement Therapy

Abstracts of the XVIII Conference of the Russian Dialysis Society - Acute Kidney Injury

Abstracts of the XVIII Conference of the Russian Dialysis Society - Kidney transplantation