Racial/ethnic and socioeconomic disparities in chronic kidney disease (CKD) have been documented for decades, yet little progress has been made in mitigating them. Several recent studies offer new insights into the root causes of these disparities, point to areas where future research is warranted and identify opportunities for changes in policy and clinical practice. Recently published evidence suggests that geographic disparities in CKD prevalence exist and vary by race. CKD progression is more rapid for racial/ethnic minority groups as compared to whites and may be largely, but not completely, explained by genetic factors. Stark socioeconomic disparities in outcomes for dialysis patients exist, and vary by race, place of residence and treatment facility. Disparities in access to living kidney donation may be driven primarily by the socioeconomic status of the donor as opposed to recipient factors. Recent studies highlight opportunities to eliminate disparities in CKD, including efforts to direct resources to areas and populations where disparities are most prevalent, efforts to understand how to best use emerging information on the contribution of genetic factors to disparities, and continued work to identify modifiable environmental, social, and behavioral factors for targeted interventions among high-risk populations.

Patients with chronic kidney disease (CKD) are predisposed to heart rhythm disorders, including atrial fibrillation (AF)/atrial flutter, supraventricular tachycardias, ventricular arrhythmias, and sudden cardiac death (SCD). While treatment options, including drug, device, and procedural therapies, are available, their use in the setting of CKD is complex and limited. Patients with CKD and end-stage kidney disease have historically been under-represented or excluded from randomized trials of arrhythmia treatment strategies, 1 although this situation is changing. Cardiovascular society consensus documents have recently identified evidence gaps for treating patients with CKD and heart rhythm disorders. To identify key issues relevant to the optimal prevention, management, and treatment of arrhythmias and their complications in patients with kidney disease, Kidney Disease: Improving Global Outcomes (KDIGO) convened an international, multidisciplinary Controversies Conference in Berlin, Germany, titled CKD and Arrhythmias in October 2016.

The main negative consequences of ischemia-reperfusion of the kidneys are the early developing severe chronic dysfunction of the graft, and in the most severe cases the function of the transplanted kidney is not restored (primary non-functioning graft). As a result of loss of transplant function, the patient usually returns to dialysis. These complications are more common in kidney transplants from “donors with extended criteria,” since these organs are most sensitive to damage resulting from ischemia-reperfusion syndrome (IR syndrome). At the same time, the share of such (suboptimal) donors is gradually increasing in Russia. Cold preservation of the organ in special solutions remains the gold standard for kidney transplantation, however, it is not able to fully protect the organ. The article presents the main promising methods that reduce the severity of ischemic and reperfusion injury: donor conditioning, ischemic preconditioning, various variants of kidney preservation, effects on inflammatory mediators, application of biological target drugs. Nevertheless, the pathogenesis of ischemia-reperfusion syndrome has been studied much better than the methods of its correction. Currently, there are only indirect or experimental evidence that the severity of the syndrome of IR can be reduced due to the pharmacoprotection of the ogran before donation, during preservation, as well as in the early postoperative period. Further research is needed to find ways to reduce the severity of ischemic and reperfusion injury of the graft.

In recent years, many new data have been obtained regarding the tactics and strategy of dialysis therapy, which require a revision of existing clinical guidelines. This review of modern criteria for the adequacy of hemodialysis is built in accordance with the Working groups of the 2018 KDIGO Controversies Conference, which is the basis for the development of the update of future clinical guidelines of the International Society of Nephrology. It should be recognized that the intensification of a certain dialysis session has reached a limit in terms of improving meaningful outcomes. At the same time, the individual choice of dialysis modality, conditions for starting and preparing for it, optimization of the ultrafiltration rate, selection of the composition of dialysis solution, and the use of instrumental methods in correcting the water balance allow expanding the possibilities of treatment. The results of the treatment should be evaluated from a patient-oriented position. The concept of “target efficacy” for elderly and frailty patients should gradually give way to the concept of “target tolerance”, in which adequate dialysis should have minimal side effects. At the same time, more frequent, highly effective dialysis may be beneficial in a group of young patients with high metabolic needs. It is worth paying attention to the discord between assessing the significance of outcomes for patients and doctors: a mutual understanding of goals and desires will lead to an increase in compliance with treatment and satisfaction with its results. The purpose of any treatment (including dialysis) should, first of all, not be in conflict with the imperative "noli nocere!".

THE AIM: to evaluate the effect of allelic variations in the hemostatic system genes on the development and course of lupus nephritis. PATIENTS AND METHODS. The study analyzed 100 patients with SLE Caucasians. 80 women and 20 men aged 16 to 73 years (mean age 37, ± 14 years). The duration of observation was for 73 patients over 5 years, for 18 – from 1 year to 5 years and for 9 – less than 1 year A rise in the level of creatinine in the blood above or equal to 2 mg / dl was considered a significant sign of impaired renal function. RESULTS. Among the patients included in the study, kidney damage was detected in 61 people (61%). In 33 of them (54.1%), a variant of renal pathology was observed according to the type of rapidly progressive lupus nephritis (BPVN). In patients with BH, mutations in the MTHFR (C677T) gene were statistically significantly more frequent (p = 0.033). The OR for the mutant genotype is 6.146 with 95% CI from 1.692 to 22.326. In patients with PWHD, mutations in the MTHFR (C677T) gene were statistically significantly more frequent (p = 0.031). The OR for the mutant genotype is 1.625 with 95% CI from 1.034 to 4.771. The five-year renal survival in carriers of the mutant allele of the MTHFR gene (C677T) is statistically significantly lower (72.8%) than in patients without this mutation (81.9%) (p = 0.027). Ten-year renal survival in carriers of the mutant allele of the MTHFR gene (C677T) is statistically significantly less (55.6%) than in patients without this mutation (70.5%) (p = 0.016). In patients with BH, mutations in the PAI-1 gene (4G / 5G 675) were statistically significantly more frequent (p = 0.046). OR for mutant genotype – 1.766 with 95% CI from 1.061 to 4.758. CONCLUSION. The mutant alleles of the MTHFR (C677T) and PAI-1 (4G / 5G 675) genes are likely to be associated with the development of BH. Polymorphism of the MTHFR gene (C677T) is associated with an unfavorable course of HH.

BACKGROUND. Мonoclonal gammopathy (MG) is not only the state preceding of hematological neoplasms, but also associated with non- hematological diseases, in particular damage of kidneys. Earlier diagnosis of MG represents an important area in treating patients with renal diseases associated with MG. THE AIM: To determine the frequency of MG among therapeutic and nephrological patients for optimization of methods of their diagnosis and treatment. PATIENTS AND METHODS: In common, 11392 patients were analyzed within 4 years (2013-2016). The standard clinical examination was conducted. Method of an electrophoresis of proteins of serum of blood and the 24-hour urine, method of immunofixation of proteins of serum and urine, and method of free light chains definition in serum (Freelite) were used for MG identification. RESULTS: MG is diagnosed in 174 of 11392 patients: 49 % of men and 51 % of women aged from 18 up to 85 years. MG was found 2.1 times more often in nephrological patient than in patients of therapeutic departments. Among patients of this group, AL-amyloidosis with kidney involvement was diagnosed in 41 %, cryoglobulinemic glomerulonephritis – in 18 %, chronic glomerulonephritis – in 35 %, also there was small number of patients with light chain disease and cast-nephropathy. 86 % of nephrological patients had less than 5 g/l of monoclonal protein that corresponds oligo secretory MG, and at 46 % from them – less than 1 g/l, other 10 % had MG of 5-10 g/l, and only in 4.42 % of patients MG more 10g/l was defined. CONCLUSION: We conclude that MG, especially oligo secretory form, play a significant role in pathogenesis of renal damage. It is important to apply sensitive methods – immunofixation of proteins and method «Freelite» for nephrological patients.

BACKGROUND. One of the perspectives of modern Nephrology is the study of the mechanisms of nephrosclerosis in ADPKD. Matrix metalloproteinase system (MMP/TIMP)— enzymes that play a key role in the processes of proteolysis in the kidney. THE AIM: to determine the expression of the urine MMP-2, MMP-3 and MMP-9 and their inhibitors TIMP-1 and 2, PAI-I, to establish their relationship with the volume of the kidney corrected to the surface of the body and the functional state of the kidneys, an additional criterion of progression. PATIENTS AND METHODS. The study included 34 children with ADPKD. The level of MMP-2, MMP-3 and MMP-9 and their inhibitors TIMP-1 and 2, PAI-I were determined in urine by ELISA. RESULTS. eGFR in children with total kidney volume greater than 97‰ was significantly lower than in children with normal total kidney volume. In the group of children with a total volume of the kidneys more than 97 percentile,a statistically significant increase in the level of TIMP-1 and TIMP-2 and PAI-I in the urine, and a statistically significant low level of urinary excretion of MMP-3 and MMP-9, compared with the group of children with ADPKD with normal total volume of the kidneys. In the group of children with ADPKD and total kidney volume of more than 97 percentiles of an inverse correlation relationship between the level of eGFR and TIMP-2 and PAI-I, as well as a direct correlation relationship between the total volume of kidney and the urinary excretion of TIMP-1. CONCLUSION. MMP and its inhibitors play an important role in renal damage in children with ADPKD. These proteolysis factors are promising to use as an indicator of the severity of the accumulation of extracellular matrix, that is, monitoring the process of fibrosis, and used as a predictor of progression.

Hepatocyte nuclear factor 1β (HNF1β)-associated disease is a rare autosomal dominant disease caused by various mutations in the HNF1β gene coding the hepatocyte nuclear factor 1β. HNF1β is a transcription factor that is critical for the development of kidney urogenital tract, pancreas, liver, brain, and parathyroid gland. Renal phenotype or HNF1β- nephropathy appeared to be extremely heterogenic: multicystic renal dysplasia, renal hypoplasia, unilateral renal agenesis, horseshoe kidney, atypical familial juvenile hyperuricemic nephropathy, urinary tract malformations and tubular dysfunction. Extrarenal phenotype of HNF1β-associated disease could be maturity-onset diabetes of the young (MODY), pancreatic atrophy and exocrine pancreatic dysfunction, elevated liver enzymes, neonatal cholestasis, congenital abnormalities of the genital tract, hyperparathyroidism, neurological symptoms. The multisystem phenotype makes clinical verification of the diagnosis extremely difficult. In this article, we present a clinical observation of a child with HNF1β – associated disease. The first clinical presentation of HNF1β-associated disease was ultrasound changes in the kidneys (hyperechogenic kidneys?), which were detected by prenatal ultrasonography in pregnancy. Renal ultrasound revealed polycystic kidney disease in the first days of life and bilateral medullary nephrocalcinosis by the age of three. The clinical examination showed a reduced renal function and developed Fanconi syndrome (glycosuria, low molecular proteinuria, hypophosphatemia, aminoaciduria, hyperuricosuria) in the first year of life. Also the child had a non-constant asymptomatic elevation of liver enzymes, hyperparathyroidism, osteoporosis. The diagnosis was confirmed by the results of next generation sequencing which revealed novel heterozygous mutation in exon 4 of the HNF1b gene (chr17: 36091813C>T), p.Cys273Tyr (c.818G>A). The identified mutation was validated by Sanger sequencing. Validation by Sanger sequencing did not reveal a chr17: 36091813C>T mutation in parents, which suggested the appearance of a mutation in the child de novo.

Pregnancy in patients with an advanced stage of chronic kidney disease (CKD) remains a rather rare situation to date. This observation demonstrates our own experience of successfully management of pregnancy in a patient with chronic kidney disease stage 4. A special feature of this observation is an unclear diagnosis that led to CKD. Based on a combination of advanced CKD in a young patient with no kidney history, no changes in urine tests, increased blood pressure, hyperuricemia, and small cysts of both kidneys, a diagnosis of autosomal dominant tubulo-interstitial kidney disease was suggested, despite the lack of family history of renal disease. Since the kidney disease was first identified during pregnancy, the main areas of care were the correction of complications (anemia, calcium-phosphorus disorders), caused by the advanced stage of CKD and the prevention of pre-eclampsia as one of the most frequent complications of pregnancy in this cohort of patients. In order to timely diagnose preeclampsia, the patient was regularly monitored for angiogenesis markers. Conducting pregnancy was carried out by an interdisciplinary team of specialists (nephrologists, obstetrician-gynecologists). Pregnancy ended with the birth of healthy baby. After childbirth renal failure progressed.

Tamm–Horsfall Protein (uromodulin) is named after Igor Tamm and Franc Horsfall Jr who described it for the first time in 1952. It is a glycoprotein, secreted by the cells in the thick ascending limb of the loop of Henle. This protein will perform a number of important pathophysiological functions, including protection against uroinfections, especially caused by E. Сoli, and protection against formation of calcium concernments in the kidney. Igor Tamm (1922-1995) is an outstanding cytologist, virologist and biochemist. He is one of the pioneers in the study of viral replication. He was born in Estonia and died in the USA. In 1964 he was elected for a professorship in Rockefeller Institute for Medical Research, where has been working continuously. Since 1959, he became a head of the virology lab established by his mentor and co-author Franc Horsfall. In the course of studies on the natural inhibitor of viral replication, Tamm and Horsfall isolated and characterized biochemically a new protein named after their names. Franc Lappin Horsfall Jr (1906-1971) was a well-known clinician and virologist with remarkable achievements in internal medicine. He was born and died in the USA. He worked in the Rockefeller Hospital from 1934 to 1960, then in the Center for Cancer Research at the Sloan-Kettering Institute. Here he was a leader of a research team studying the molecular mechanisms of immunity, the effects of chemotherapy with benzimidazole compounds (together with I. Tamm), coxsackie viruses, herpes simplex virus, etc.