The review provides a definition of the concept of «patient-reported outcomes (PRO)». The main components of PRO include health-related quality of life (QoL), symptoms assessed by the patient, functional status, satisfaction with treatment. The review presents a description of the most frequently used questionnaires for assessing QoL in patients with chronic kidney disease (CKD). It has been shown that the physical component of QoL decreases in predialysis stages of CKD. As CKD progresses, the Physical Component Summary (PCS) of the SF-36 questionnaire deteriorates, as well as the scale scores of Physical functioning, Pain, General health, and Role limitations caused by Physical problems (RP). Among the CKD-specific QoL scales, a deterioration in the scores of Symptoms/Problems, Burden of Kidney Disease, Effects of kidney disease on daily life is observed. There are no convincing data in the literature in favor of a decrease in psychosocial aspects of QoL in patients with CKD. The review presents the definition of «comprehensive conservative care» proposed by the experts convened at the 2013 KDIGO Controversies Conference on Supportive Care. It has been shown that comprehensive conservative care can be a fairly effective method of choice for elderly patients with Stage 5 CKD. It has been demonstrated that a decrease in PCS is independently associated with CKD progression and an increased risk of death. However, the information on QoL in predialysis CKD is insufficient. The use of different questionnaires for QoL assessment complicates data comparison. Randomized clinical trials are practically absent, which reduces the value of the results obtained.

INTRODUCTION AND AIM. Monoclonal gammopathy of renal significance (MGRS), heterogeneous nosological entity, is caused by monoclonal immunoglobulin (IG) produced by a "small" B-cell lineage clone. The analysis of long-term renal prognosis in various clinical and morphological types of disease and in different degrees of hematological response (HR) in the Russian patient cohort became the goal of this study. PATIENTS AND METHODS. Patients met the criteria of MGRS (morphologically verified monoclonal IG related kidney damage, an aberrant clone in the bone marrow and/or the level of serum/urine paraprotein not met the oncohematological criteria for treatment initiation) were enrolled in this one-center prospective study from 01.01.2011 till 01.03.2020. The morphological spectrum of MGRS, types of therapy, hematological and renal responses (RR) were analyzed. HR was assessed depending on the type of monoclonal IG according to the accepted criteria. The presence of RR was considered as a decrease in daily proteinuria> 30 % from the initial level or less than 0.5 g in the absence of a decrease in eGFR> 25 % at the time of the end of follow-up. The progression of renal dysfunction was documented with a decrease in eGFR> 25 % from baseline. Renal outcome (initiation of renal replacement therapy or eGFR <15 ml/min/1.73m2  at the end of follow-up) and death were determined. Long-term renal survival was assessed by the Kaplan-Meier method. The median follow-up period was 18 (4; 38) months. RESULTS. The incidence of MGRS was 4.9 % (n = 102) of all performed kidney biopsies (n = 2042), the majority of cases represented with AL amyloidosis (AL, n = 73; 71.6 %). In cases of non-amyloid variant of MGRS (non-AL MGRS, n=29, 28.4 %) the most common types of renal injury were light chain deposition disease (27,6 %) and proliferative glomerulonephritis with deposition of monoclonal IG (27.6 %). The majority of patients (80.4 %) was treated with clone-specific agents, autologous bone marrow transplantation was performed in 13 cases. HR was achieved in 74 % and 80 % of the treated patients with AL and non-AL MGRS, respectively. RR was obtained in 42 % of patients with AL and in 62 % of patients with non-AL MGRS. The five-year cumulative renal survival did not differ significantly between the groups: 66 % in AL and 44 % in non-AL MGRS (р=0,78). Cumulative renal survival in patients who did not achieve HR was significantly lower (42 %) than in cases with complete HR (86 %), p=0.0014. CONCLUSION. MGRS is a clinically and morphologically heterogeneous nosological entity characterized by a poor renal prognosis, especially in the absence of clone-specific therapy. Treatment in MGRS should be carried out in a timely manner with the participation of a hematologist and nephrologist in order to prevent loss of kidney function and increase life expectancy.

INTRODUCTION. From 15 to 35% of cases of acute coronary syndrome (ACS) are complicated by the development of acute kidney injury (AKI), prevention and early intervention remain the most effective strategy for managing patients with AKI in ACS. THE AIM: This study aimed to explore a risk factors and biomarkers for predictive and early diagnostic of AKI in ACS. PATIENTS AND METHODS. The study included patients hospitalized with a diagnosis of ACS in Pavlov First Saint Petersburg State Medical University. In case of exclusion of ACS, patients were determined in the comparison group, in case of confirmation of the diagnosis of ACS – in the study group. Biomaterial (blood) was taken at admission (T1), 1 day after admission (T2) and 2 days after admission (T3). For the diagnosis of AKI, KDIGO 2012 criteria were used. The measured biomarkers at each point were sST2, troponin I, NTproBNP. RESULTS. The study included 132 patients, the diagnosis of ACS was confirmed in 91 patients and AKI development was in 30 patients, all from the ACS group. The most significant for predictive diagnosis was the assessment of GRACE score> 133 points (AUC=0.760, p=0.001), sST2 level> 27.2 ng / ml (AUC=0.737, p=0.001), Mehran score> 5 (AUC=0.916, p=0.001), modification of the Mehran score (adding 2 points if the patient has sST2> 27.2) increases the predictive ability of AKI, Mehran+sST2> 7 points – AUC=0.928, p=0.001. CONCLUSIONS. The use of a combination of clinical data (hemodynamic parameters, presence of heart failure, routine laboratory data, presence of AKI risk factors) and assessment of biomarkers level, in particular the sST2 level, seems to be an effective method for predictive diagnosis of AKI and requires further research.

THE AIM: Analysis of clinical and morphological manifestations of lupus nephritis (LN) and their correlations. PATIENTS AND METHODS. A retrospective cross-sectional study included 157 patients (pts) with clinical signs of LN (females 87 %, age 39±13 years). The diagnosis was confirmed morphologically in 133 cases. Clinical (including SELENA-SLEDAI score), immunological and histological parameters were analyzed at the time of kidney biopsy. Clinical factors associated with the development of proliferative and necrotizing LN phenotype were determined in multivariate logistic regression models. RESULTS. Forty eight percents of patients with LN had the estimated glomerular filtration rate (eGFR) ≤59 ml/min/1.73 m². Acute kidney injury (AKI) was found in 14 % of cases. Nephrotic syndrome was detected in 33 % of cases, while immunological activity of systemic lupus erythematosus (SLE) was obvious in 87 %. Dominant morphological class of LN was IV (31.1 %) and 68.2 % of all LN cases were represented by proliferative LN classes. Lupus podocytopathy was diagnosed in three patients (2,3 %). eGFR was mainly associated with the severity of acute and chronic changes in tubulointerstitium and vessels and, to a lesser degree, with glomerular alterations. Proteinuria and hematuria, immunological activity of SLE correlated with active glomerular inflammatory injuries. Proliferative classes LN were able to be identified in patients who did not have significant proteinuria at the time of kidney biopsy. In logistic regression analysis the risk of developing proliferative LN was associated with the level of antibodies to double-stranded DNA (anti-dsDNA) >130 IU/ml (hazard ratio (HR) 3.36 95 % confidence interval (CI) 1.18-9.59) and systolic blood pressure (BP) (НR 1.28 95 % CI 1.01-1.55 (for every +10 mm Hg). Focal necrotizing glomerular lesions with cellular crescents formation was detected in 27 % of LN (71 % of them were classes III or IV). These cases had significantly more pronounced clinical and histological manifestations (glomerular and tubulointerstitial inflammation). Independent clinical factors associated with necrotizing LN were the presence of AKI at the time of kidney biopsy (HR 6.10 95 % CI 1.57-23.75) and a combination of abnormal anti-dsDNA with the decrease of serum complement (C3 and/or C4) (НR 9.99 95 % CI 2.10-47.25). CONCLUSION. Glomerular injury mediated by immune complexes and local activation of complement in SLE is heterogeneous being represented by proliferative, non-proliferative classes of LN or their combinations, as well as special variants of glomerular damage – lupus podocytopathy and necrotizing LN. A precise clinicomorphological diagnosis of LN and associated vascular and tubulointerstitial alterations should be the basis for assessing the disease severity and prognosis and for a choice of personalized therapy.

INTRODUCTION. The risk of femoral neck fractures in hemodialysis patients is estimated at 25.6 per 1000 people per year. Hip arthroplasty is the treatment choice in these patients. One of the causes of general somatic complications is the significantly more frequent formation of blood clots. PATIENTS AND METHODS. The material for the study was data on 96 hemodialysis patients who underwent primary hip arthroplasty in the period from 2016 to 2019. THE AIM. Сomparison of various algorithms for the use of fractionated and unfractionated heparins after primary hip and knee arthroplasty in patients receiving programmed hemodialysis. RESULTS. The largest number of thrombosis was noted in group 1 (heparin was used only during hemodialysis procedures): in 4 patients (16.6 %), in group 4 (using fractionated heparin both during hemodialysis procedures and on interdialysis days): in 2 patients (8 %). When comparing the volume of the evacuated hematoma, the statistically significant volume was in group 2 (with the use of un fractionated heparin both during dialysis and on interdialysis days), on average 98 ± 52 ml. In the same group, the greatest number of infectious complications was noted: in 5 patients (21.8 %); in group 4: in 2 patients (8 %). CONCLUSION. Conducting thromboprophylaxis in hemodialysis patients after arthroplasty of major joints with fractionated heparins, both during the hemodialysis procedure and on interdialysis days, can reduce the number of thromboembolic complications to 8 % (16.6 % with the traditional method). Affect on the volume of blood loss and blood transfusion not statistically significant. At the same time, there is a significant decrease in the level of potassium by 8.8 %, total cholesterol by 7.3 %, triglycerides by 11.7 % with the daily use of fractionated heparins.

INTRODUCTION. Methoxy polyethylene glycol-epoetin beta (PEG-EPO) is a representative of a third-generation erythropoiesis-stimulating agent with a continuous erythropoietin receptor activator (CERA). Although the effectiveness of methoxy polyethylene glycol-epoetin beta has been confirmed in multicenter randomized clinical trials, research continues to examine the safety and tolerability in patients with chronic renal anemia previously treated with recombinant human erythropoietins (rhEpo) of the first generation. AIMS. Evaluation of the efficacy and safety during the transition from rhEpo of first generation epoetin alpha (Epocrin, Aeprin, Eralfon) to third generation continuous activator erythropoietin receptor (CERA), methoxy polyethyleneglycol-epoetin beta (Mircera) administered intravenously every 4 weeks to maintain a stable hemoglobin (Hb) in patients with EPO-dependent anemia in programed hemodialysis (PhD). PATIENTS AND METHODS. A retrospective study was conducted enrolling 116 patients with terminal stage renal failure. They all were undergoing the renal replacement therapy with programmed hemodialysis, and were all being transferred from rhEpo of the first generation (months from -12 to -1) to CERA (Mircera) (months from1 to 12). As rhEpo first generation drugs with epoetin alpha (Alpha-EPO) were used Epocrin, Aeprin, Eralfon. RESULTS. 1. The use of CERA allows one to maintain a stable level of hemoglobin, with smooth dynamics. In sharp contrast, the short-acting epoetins induce drastic fluctuations. 2. The dose modification in the CERA (Mircera) therapy was half as frequent, compared to the frequency of dose modification in the alpha-EPO therapy (1.4±0.15 and 2.1 ± 0.3 episodes/year, respectively). 3. Adverse events associated with the use of CERA (Mircera) were rare. An increase in blood pressure indicators was revealed in CERA (Mircera) therapy, compared with the use of alpha-EPO. CONCLUSION. The use of CERA (Mircera) allows for the effective and safe maintaince of stable hemoglobin level in patients receiving treatment with program hemodialysis.

THE AIM. Comparison of indirect immunofluorescence (IIFT) and enzyme-linked immunosorbent assay (ELISA) methods for the determination of circulating antibodies to the podocytic transmembrane receptor of M-type secreted phospholipase A₂ (PLA₂R-AB) in patients with primary membranous nephropathy (PMN). PATIENTS AND METHODS. The study included 54 patients with PMN (M: F [33:21]) aged 55 (40-63) years and 10 apparently healthy individuals of the corresponding gender and age. Proteinuria and glomerular filtration rate (eGFR) was determined in all patients. The anti-PLA₂R-AB levels were measured by indirect immunofluorescence (IIFT) and enzyme-linked immunosorbent assay (ELISA) using EURUIMMUN AG test systems (Lubeck, Germany). RESULTS. The levels of anti-PLA₂R-АB determined by the two investigated methods were changed in the same direction (r = 0.82, p < 0.005). The ELISA method made it possible to detect a larger number of anti-PLA₂R-positive cases, both in the group of patients before treatment (ELISA – up to 80 %, IIFT – up to 67 %) and among patients receiving immunosuppressive therapy (ELISA – up to 63 %, IIFT – up to 50 %). The concentration of autoantibodies determined by ELISA (both quantitative and semi- quantitative) correlated with proteinuria (r = 0.69, p = 0.001) and eGFR (r = -0.38, p = 0.035). The response to therapy was more clearly traced when the study was performed by quantitative ELISA. CONCLUSION. The ELISA method is more effective in identifying PLA₂R-positive patients with PMN, allows obtaining quantitative results and assessing the dynamics of the concentration of autoantibodies, which contributes to the early correction of the therapy and decisionmaking on immunosuppressive treatment.

AIM: The aim was to determine the significance of blood pressure level on progression of renal failure in children with X-linked Alport syndrome. PATIENTS AND METHODS. eGFR, urine proteine (mg/m²/day), mean day blood pressure – MBP (normalized for sex, age, height) were assessed in retrospective single center study. RESULTS. A 69 children (age 9.49±4.18 years, М/ F=47/22, eGFR=109±17.36 ml/min/1,73m²) were followed for 4[3;5] years. The 27 pts (М/F=13/14) had MBP<50‰, the 33 children (М/F=25/8) – MBP 50-90‰ and pts (М/F=9/0) – MBP≥90‰. Rate of eGFR decline was higher in male (-3[-0.8;-4.7] vs 0.2[-0.4;2]; χ² = 21.15587, p = 0.0067), in pts with MBP ≥90‰ (0.65[-2.65;2] vs -2.3[-6;2.3] vs -3[-7.5;-1.8] in 1st, 2 nd and 3 groups, respectively; р_1,3 =0.0091) and proteinuria (-2.5[-7;0.5] vs 0.2[-4;3]; p=0.0018). Patients with blood hypertension had high risk of renal failure (OR=5.33, 95 % CI 1.75;16.22), especially in cases of proteinuria (OR=7.08, 95 % CI 2.46;20.39). MBP<50‰ associated with low risk of renal disease progression (OR=0.83, 95 % CI 0.31;2.2), especially in boys and proteinuric patients. The male gender and proteinuria are independent risk factors for eGFR decline. CONCLUSIONS. MBP≥90‰ is associated with renal disease progression, especially in case of proteinuria; the MBP<50‰ reduced the risk of eGFR decline in male and proteinuric patients with Alport syndrome; the male gender and proteinuria are independent risk factors for renal disease progression in children with Alport syndrome.

INTRODUCTION. Beta-catenin is a structural protein of adhering junction and intercalated discs of cardiomyocytes as well as the main intracellular messenger of the canonical WNT (cWNT) signaling pathway. The dysregulation of the cWNT signaling and the rearrangement of the cardiomyocyte cytoskeleton accompany the cardiovascular disorders in chronic kidney disease (CKD). THE AIM: to investigate the expression and distribution of β-catenin, calcineurin A, and TGF-β1 in the myocardium of spontaneously hypertensive rats (SHR) with CKD, sham operated SHR and Wistar Kyoto rats of the corresponding age. MATERIAL AND METHODS. Systolic blood pressure (BP), heart rate (HR), myocardial mass index (MMI), creatinine concentration (Cr), myocardial beta-catenin expression and renal Klotho expression, morphological light-optical study of kidney and myocardium tissues was performed in sham operated (SO) Wistar Kyoto rats (WKY), spontaneously hypertensive rats (SHR) and SHR with 5/6 nephrectomy (Nx). RESULTS. SHR rats showed higher values of BP, MMI, cardiomyocyte diameter, myocardial fibrosis area, and lower Klotho levels compared to WKY rats. Nx SHR had lower kidney function and renal Klotho expression, higher BP and MMI compared to SHR. An increase in the cardiomyocytes diameter and the area of myocardial fibrosis was accompanied by the overexpression of β-catenin, calcineurin A, and TGF-β1 in the myocardium. CONCLUSION. The upregulation of canonical Wnt signaling and cellular programs of cardiomyocyte hypertrophy mediated by Klotho deficiency can be involved in myocardial remodeling in chronic renal dysfunction.

INTRODUCTION. High dietary sodium chloride intake has traditionally been considered an important factor in cardiovascular risk and an increase in blood pressure (BP). However, much remains unclear about the relationship between dietary sodium and cardiovascular health. In particular, it was shown that in rats a high content of salt in the diet can lead to miocardial remodeling/damage without increasing BP. It is possible whether this phenomenon in primates remains unknown. The AIM of the study was to trace the dynamics of BP and some echocardiographic (EchoCG) parameters in cynomolgus macaques (Macaca fascicularis), which have been on a high sodium chloride diet for a long time. MATERIAL AND METHODS. 12 male cynomolgus macaques 6-8 years with a body weight of 5,15-9,7 kg were studied. The animals were divided into two groups (six individuals each). The first (control) received a standard diet, the second – a diet high in sodium chloride (8 g NaCl/kg feed). After four and fourteen months, in the animals were measured blood pressure and conducted echocardiographic examination (EchoCG). RESULTS. During the follow-up period in the control group, no significant changes in the studied parameters were recorded. Increased salt intake at four months resulted in a significant decrease in left ventricular end-systolic dimension and an increase in ejection fraction values. After fourteen months, both of these indices returned, practically, to the initial level, but there was a significant increase in the thickness of the posterior wall of the left ventricle in systole and a decrease in systolic excursion of the tricuspid valve annulus compared with the basal data. BP in the salt group also did not change significantly. CONCLUSION. Cynomolgus macaques are able to resist an increase in blood pressure induced by a long stay on a high-salt diet, but in primates under such conditions, the initial manifestations of functional and structural rearrangement of the heart can be observed.

The article is devoted to the problem of treatment of secondary hyperparathyroidism (SHPT) in patients with chronic kidney disease (CKD) on renal replacement therapy with programmed hemodialysis. The pathogenesis of VHPT is based on vitamin D deficiency and the associated launch of a cascade of complications of mineral metabolism, which subsequently leads to significant changes in the morphology and density of bone tissue, as well as cardiovascular complications. According to the main clinical guidelines, the goals of treating IHPT in patients with CKD are aimed at preventing the progression of the disease and suppressing the activity of the parathyroid glands by modulating vitamin D receptors and calcium-sensitive receptors. Maintaining the level of parathyroid hormone within the target values improves the quality of life of patients, reduces the incidence of cardiovascular and bone complications. The article presents the result of our own clinical observation on the correction of alfacalcidol-resistant IHPT with hypercalcemia and hyperphosphatemia in a patient on programmed hemodialysis using a combination therapy with a calcimimetic – cinacalcet, colecalciferol and a selective activator of vitamin D receptors – paricalcitol. On the example of a clinical case, the compensation of IHPT, an improvement in the condition of the altered parathyroid glands, bone tissue without the risk of developing hypo-, hypercalcemia and hyperphosphatemia during long-term treatment was demonstrated.