Chronic Kidney Disease affects approximately 10% of the world’s adult population: it is within the top 20 causes of death worldwide, and its impact on patients and their families can be devastating. World Kidney Day and International Women’s Day in 2018 coincide, thus offering an opportunity to reflect on the importance of women’s health and specifically their kidney health, on the community, and the next generations, as well as to strive to be more curious about the unique aspects of kidney disease in women so that we may apply those learnings more broadly. Girls and women, who make up approximately 50% of the world’s population, are important contributors to society and their families. Gender differences continue to exist around the world in access to education, medical care, and participation in clinical studies. Pregnancy is a unique state for women, offering an opportunity for diagnosis of kidney disease, but also a state where acute and chronic kidney diseases may manifest, and which may impact future generations with respect to kidney health. There are various autoimmune and other conditions that are more likely to impact women with profound consequences for child bearing, and on the fetus. Women have different complications on dialysis than men, and are more likely to be donors than recipients of kidney transplants. In this editorial, we focus on what we do and do not know about women, kidney health, and kidney disease, and what we might learn in the future to improve outcomes worldwide.

Despite the rarity of end-stage renal disease (ESRD) in children, it is a serious medical and social problem. Aim of the study was to assess the incidence and prevalence of ESRD in children in Belarus, age and sex of these patients, etiology of renal disease leading to ESRD and to determine the influence of the disease and methods of renal replacement therapy (RRT) on growth and anthropometric characteristics of the patients. Materials and methods: the study included 121 children who received RRT on 1 January 2007 and all subsequent new patients who started this therapy before 31 December 2016. Age at start of RRT was 11.4 (6.9; 14.9) years, 62,8% of children were boys. Results: the incidence of ESRD in children in Belarus aged 0-14 years, who started RRT in 2007-2016, was 7 (6; 8) cases per year or (4.8 (4.2; 5.2) per million age related population (pmarp); for the period 2009-2016 the incidence was 11 (9; 12) cases per year (5.6 (4.8; 6.8) pmarp). The prevalence of ESRD in children on RRT during the period from 2007 to 2016 increased by 31.7% in the group of 0-14 years: from 26 patients (18.3 pmarp) to 38 (24,1 pmarp), and by 51.7% aged 0-17 - from 37 (20.3 pmarp) to 57 (30.8 pmarp). The main cause of ESRD was congenital abnormality of kidney and urinary tract (44.6% cases). On December 31, 2016 75.4% of children were transplanted, 15.8% received peritoneal dialysis and 8.8% hemodialysis. 53 (43.8%) patients were transferred to adult service and 83% of them with a functioning graft. Mortality on RRT was 8.3%, 5-year mortality rate was 17.9 deaths per 1000 patient-years. SDS (standard deviation score) of height at the start of RRT was -1,16±1,74, SDS < -2 was found in 28.8% patients, more often at the age of 5-9 years (40.6%) and at 10-14 years (32.5 %). Improvement of growth depended not only on the age of transplantation, but also on the duration of graft functioning (r=0.55, p<0.01). 28% of children on RRT needed therapy with growth hormone. Conclusions: the incidence of ESRD in children in Belarus matches the average of ESPN/ERA-EDTA register, the prevalence is lower, but has a constant tendency to increase. There is a high number of transplants and low mortality of patients on RRT. It is necessary to introduce ESRD to the list of pathologies requiring the appointment of growth hormone in pediatric practice. Key words: end-stage renal disease (ESRD), the incidence, prevalence, mortality, transplantation, peritoneal dialysis, hemodialysis, growth, growth hormone

Atypical hemolytic-uremic syndrome is a rare life-threatening disease from the group of thrombotic microangiopathies, caused by the hyperactivation of the complement system. In the most cases it is associated with genetic disorders in the cluster of complement genes. Currently, a large number of different variants of the complement system genes associated with the development of aHUS are described in different countries. In our country, data on the genetic features of pediatric aHUS patients and obstetric aHUS have been published. Genetic changes in the complement system in adult aHUS patients in Russia were not so far presented. Aim: studying the genetic profile of the complement system in adult patients with aHUS. Materials and methods. The study included 20 patients with aHUS: 9 men (45%) and 11 women (55%). All patients underwent molecular-genetic analysis (search for mutations in the clinically significant part of the human genome - exome) by sequencing (Genotek laboratory). Genes CFH, CFHR1-5, CFB, CFI, DGKE, THBD, MCP, C3, С5, ADAMTS13 were analyzed. Results. Genetic variants (mutations) of the complement system associated with aHUS development were detected in 5 patients (25%). Two patients had one mutation, 2 patients - 2 and one patient - 3 mutations. In 3 patients, different genetic variants of the C3 gene were found. Two patients showed the same changes in the CFHR5 gene. In 3 patients, rare changes in the ADAMTS-13 gene, clinically associated with the development of thrombotic thrombocytopenic purpura were found. In all 20 patients, genetic variants of the complement genes with unknown clinical significance were identified, including rare variants of the C3 gene in 9 patients (45%).

Aim of the study: early renal allograft dysfunction (ERAD) is one of the more common complications of kidney transplantation. The aim of this study was the investigation of the prevalence of ERAD and it influence on the late transplant function and survival rate. Methods: we conducted retrospective, cohort, observational, comparative single center study. 295 renal transplant recipients were included to the study. Results: we found that the prevalence of ERAD was 24.1% (71/295). This complication was associated with decrease of 6 years cumulative kidney transplant survival from 80.75% in control group to 68.75% in study group. Besides, ERAD was associated with a late increase in the serum creatinine level to 130.3 (97-141.5) µmol/l versus 99.4 (80.5-122) µmol/l (р<0.001) and decrease eGFR from 68.48 (53-86.8) ml/min in control group to 50.15 (39.95-66.78) ml/min in study group (р<0.001).

Aim: arterial hypertension is one of the main causes of the left ventricular hypertrophy (LVH) in hemodialysis (HD) patients. The aim of this study was to investigate the correlation between LVH and blood pressure variability in hemodialysis patients. Patients and methods: 62 patients (M/F: 32/30, 55±13 years) after 1 year hemodialysis treatment were studied. Echocardiographic evaluation was performed after dialysis and left ventricular mass index (LVMI) was calculated. All patients underwent 24-hour ambulatory BP monitoring (ABPM), predialysis and postdialysis systolic and diastolic BP was determined and Home Blood Pressure Measurements (HBPM) was performed. Average values for 24-hour BP, daytime BP, and nighttime BP were calculated, average values of HBPM during 30 days including hemodialysis session days and pre- and postdialysis office BP were analysis and variability of BP parameters was calculated. Results: left ventricular hypertrophy was detected in 32 (51.6%) patients. Mean LVMI was 123.41±39.26 g/m2. Pulse blood pressure was higher in patients with LVH with all methods BP measuring. Predialysis systolic BP variability was determined as the independent factor is associated with LVMI in multivariate regression analysis (R2=0.31; β=0.34; t=2.69; P=0.009). Conclusions: our study showed that patients on the long term (more 1 year) hemodialysis treatment have higher pulse BP with all methods of measurement. The predialysis systolic BP variability was associated with the left ventricular hypertrophy independently of the blood pressure level.

Modern approaches to management of pericardial diseases in the context of new version of ESC Guidelines are presented. The necessity for understanding of syndromic approach to pericardial lesions in nephrology practice is emphasized. Pathophysiological mechanism of the pericardial syndromes (PS) formation regardless of etiology of pathological process are briefly presented. The main clinical and instrumental features of different pericardial lesions are described in the context of syndromic approach: inflammatory pericardial syndrome, effusive pericardial syndrome (EPS), constrictive pericardial syndrome (CPS) and effusive-constrictive pericardial syndrome (ECPS). Three medical cases related to different PS which seriously complicated the clinical course of kidney diseases are described in detail. In the first case up to date diagnosis of EPS prevented cardiac tamponade in a patient on program hemodialysis. In the second case the detection of CPS resulted in successful pericardiectomy and complete restoration of renal transplant function. The third case underscore the importance of ECPS diagnostics as a cause of acute renal failure. Described clinical cases are demonstrating approaches to integral evaluation of different critical conditions in urgent nephrology based on cardiorenal interaction.

The review presents current definitions for asymptomatic microscopic hematuria, recommended for clinical application in the Guidelines issued by several associations and societies, and the regulations for urine samples collection for accurate quantitative evaluation of microscopic hematuria. Detailed information about the prevalence of asymptomatic microscopic hematuria in adult patients depending on age and sex are provided according to the data from several large cohorts and screening trials. The causes of non-glomerular and glomerular microscopic hematuria are presented. Different laboratory tests and examination techniques recommended for the evaluation of patients with asymptomatic microscopic hematuria and differential diagnostic are discussed, as well as the indications and contraindications for particular examination techniques application depending on age and sex of the patients. The results of patient’s evaluation with different methods are shown. In addition, the work-up algorithm for the adult patients with asymptomatic microscopic hematuria, considering the need to rule out both urological and glomerular/interstitial disorders, is provided.

The development of thrombotic microangiopathy (TMA) during pregnancy and in postpartum period requires differential diagnosis between classical obstetric TMA (preeclampsia and HELLP-syndrome) and TMA, for which pregnancy can become a trigger of atypical hemolytic-uremic syndrome (аHUS), thrombotic thrombocytopenic purpura (TTP), obstetric sepsis with DIC-syndrome and catastrophic antiphospholipid syndrome (CAPS) in order to determine treatment strategy. Complement blocking treatment with eculizumab requires aHUS diagnosis, in the pathogenesis of which the main role is played by the uncontrolled activation of the alternative complement pathway. Complement dysregulation occurs in TMAs different from primary aHUS. Short treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition. This observation demonstrates the first experience of successful postpartum catastrophic antiphospholipid syndrome (CAPS) treatment with complement-blocking drug ekulizumab in Russia. Underestimation of the importance of detected laboratory changes and information about a burdened obstetric anamnesis led to the mistaken diagnosis of atypical HUS, which served as the basis for the ekulizumab prescription, without a doubt, saved the patient's life. The role of the complement activation in the pathogenesis of CAPS is discussed. A conclusion is made that the use of complement-blocking therapy in combination with anticoagulants primarily is advised in the obstetric CAPS patients.

IgG4-associated disease (IgG4-AD) is a systemic disease characterized by inflammation and fibrosis that affects almost all systems and organs. Humoral and cellular immunity plays some role in the pathophysiology of IgG4-AD, but the mechanism of this process is currently not fully understood. The most frequent targets are the organs of the biliodigestive zone and kidneys. IgG4-associated tubulo-interstitial nephritis (TIN) is the most common variant of kidney damage associated with this disease. The article presents a case of IgG4-AD in a young patient which debuted with diabetes mellitus and acute renal injury. The diagnosis of IgG4-AD was verified morphologically: the characteristic dense interstitial infiltration represented by plasma cells with an increase in the number of IgG4-positive cells were detected in kidney biopsy. Taking into account the obtained data, the patient was diagnosed with the development of IgG4-associated renal lesions in the form of acute TIN and pancreatic gland in the form of autoimmune pancreatitis with the formation of secondary diabetes mellitus. According to the international standards of IgG4-AD therapy, systemic glucocorticoids were prescribed. As the result, there was a complete restoration of renal function, but a normalization of endocrine function of the pancreas was no achieved.

Infectious diseases are often complicate the course of the main kidney disease in nephrological practice. Patients on long-term immunosuppressive therapy or renal replacement therapy are under serious risk of infectious complications. Particular attention is paid to specific infection - tuberculosis. This group of patients often develop mycobacteriosis caused by atypical mycobacteria, which become pathogenic due to immunodeficiency. Diagnosis of mycobacteriosis can be problematic as it requires special laboratory methods which are not available in routine clinical practice. We discuss the difficulties of diagnostics and treatment of tuberculosis in patients with diabetic nephropathy and chronic glomerulonephritis. The clinical cases show specific symptoms of tuberculosis intoxication and respiratory disorders, the lack of non-specific antibacterial therapy with the fatal multi-organ failure and death. A long unclear fevered period in immunocompromised patients is an absolute indication for persistent and probably repeated examination for exclusion of non-specific or specific infec-tious process. The main methods of diagnosis of pulmonary tuberculosis are still x-rays with the obligatory CT and sputum seed.