Introduction: before the development and widespread of antenatal ultrasound, most of urologic anomalies were diagnosed only when they were symptomatic or complicated. Since the late 1970s, prenatal screening with ultrasound has become a routine component of care for pregnant women worldwide. Fetal hydronephrosis (FH) is the most common urogenital anomaly detected, suggesting that an obstructive process may be potentially present. Relevance: FH represents a spectrum, with most cases being a trivial and inconsequential finding on fetal ultrasound. Mild FH will often resolve spontaneously, whereas moderate to severe FH is frequently associated with congenital anomalies of the kidney and urinary tract. The differential diagnosis of FH is quite broad. It includes ureteropelvic junction obstruction, vesicoureteral reflux, posterior urethral valves, ureterocele or other anomalies. Purpose of review: over the last decade, recommendations for postnatal evaluation of these abnormalities have been under intense investigation. A large cohort studies have resulted in significant changes in current principles of care. The aim of this review is to provide an overview of the management of children with FH.

Aim: many old and frail patients with CKD reveal significant comorbidity, although progress to the stage 5 for a more prolonged period. This ambiguity makes the decisions concerning dialysis start in these patients rather subjective. By the time of dialysis start, decision-taking the worsening of patient`s somatic and mental condition can arise the question about reasonableness of renal replacement therapy considering nearest prognosis. The scale system REIN has been proposed for short-term prognosis for older patients starting dialysis. Methods: we performed the external validation of the REIN scale in unselected Russian population from city renal replacement therapy register. Results: up to score 11 actual mortality coincided with predicted by REIN. We found rapid growth of mortality with further elevation of the scale up to score 18 where the mortality was 100%. The survival in groups with scale below 12, with 12-16 and >16 dramatically diverged, but predicted risks for score 6 and 11 were higher than actual mortality in our group with scores up to 11, while for others the survival was significantly lower than predicted. C-statistic in ROC analysis was 0.793 (95%CI 0.692-0.894). Three-month mortality in low-risk group (score <12) was 4.4%, in medium risk group (12-16) it was 42.9%, in high risk group (>16) it was 100%. Conclusion: REIN scale is a useful tool for the perspective of treatment evaluation in older and frail patients; it can help in decision-making while starting dialysis or refusal of renal replacement therapy in favor of comprehensive conservative treatment. Perhaps, additional factors should be included in model to improve its prognostic value.

Aim: hyperphosphatemia remains one of the most significant risk factor for mortality of dialysis patient. Its correction is a difficult problem: the capabilities and limitations of different methods are still subject to clarification in real-world practice. Methods: among prevalent patients gathered in a newly organized dialysis center, we assessed the baseline phosphate levels and trends of its changes over the period up to two years while applying all available phosphate lowering measures. After 5 years of follow-up, we evaluated the survival rate and significant risk factors. Results: the fraction of patients with phosphate level within target range (0.9÷1.78 mmol/l) increased from 27.1% to 60.3%. Patients were divided into four groups by phosphate levels: (1) stable patients in target range (53%); (2) within target range with a trend of decreasing -0.62±0.20 mmol/l/year (10%), (3) hyperphosphatemia with a trend of increasing 0.19±0.25 mmol/l/year (17%)and (4) hyperphosphatemia with a trend of decreasing -0.33±0.18 mmol/l/year (20%). In univariate Cox regression model (phosphatemia category) the third group had mortality relative risk RR=3.39; р=0.002, while the forth group did not show a difference from first group (reference): RR=1.24; р=0.67, 95%CI 0.47÷1.26. In multivariable model (beside phosphatemia category: RR=3.52; р=0.003 for third group) the significant factors included PTH <150 pg/ml, increasing PHT trend and age. The distribution of patient’s phosphatemia category significantly depended upon their compliance with dietary consultation (χ2=27.4; р=0.01). Conclusion: the effective measures for hyperphosphatemia correction include low-phosphate diet (with exclusion of inorganic phosphate additives), some dialysis regimen modification (prolong and/or frequent sessions, physical activity during session, acidosis correction), phosphate binders and hyperparathyroidism correction.

The objective of this study was to determine the cost effectiveness of intravenous paricalcitol versus non-selective vitamin D receptor agonists (VDRA) in patients with chronic kidney disease (CKD) and a secondary hyperparathyroidism, although without hypercalcemia and hyperphosphatemia in Russian Federation. Patients and methods: the analysis was carried out for hemodialysis patients with the level of serum parathyroid hormone over 300 pg/ml despite the standard therapy including restriction of phosphorus consumption and phosphate-binding agents, without hypercalcemia and hyperphosphatemia. Markov modeling on the basis of clinical trials and epidemiological data on the Russian Federation taking into account tariffs of compulsory health insurances across St. Petersburg is used. Assessment was carried out from a health care payer perspective with use of the 15-year temporary horizon. Clinical and economic outcomes were discounted at 3.5% a year. Results: according to results of modeling, paricalcitol can provide the increase in average life expectancy in comparison with non-selective VDRA for 0.683 year (without discounting). The cost effectiveness of paricalcitol, taking into account the made assumptions, will be 1820.7 thousand rubles/QALY and 1234.0 thousand rubles /LYG. The dose of a paricalcitol at therapy of this group of patients has significant effect on results of cost-effectiveness assessment. Conclusions: paricalcitol in hemodialysis patients with CKD and a secondary hyperparathyroidism without hypercalcemia and hyperphosphatemia can be considered, taking into account assumptions made during modeling, as economically acceptable for the health care system in Russia.

Patients with end-stage chronic kidney diseases are prone to infectious complications due to suppression of the immune system. The aim of the study was to identify changes in subpopulations of T- and B-cells depending on the duration of renal replacement therapy in patients with chronic kidney disease stage 5 (CKD-5). Materials and methods: twenty-one hemodialysis patients with CKD-5 and without infectious complications were studied (median age 43, m/f ratio was 15/6). The patients were divided into 3 groups depending on the duration of the hemodialysis. The 1st group included patients who were on hemodialysis from 1 to 2 years (n=6); in the 2nd group - from 2 to 3 years (n=8); in the 3rd - 3 and more years (n=7). The 20 healthy blood donors were used as controls. The percentage and absolute numbers of T- and B-lymphocytes' subsets were determined in patients' pre-dialysis peripheral blood samples and in blood samples of healthy donors by flow cytometric analysis (BD FACSCanto II). Results: the changes in subsets of T- and B-cells were not detected in patients who received hemodialysis up to 3 years. In patients with long-term hemodialysis (3 and more years) the number of CD3+, CD4+, CD8+ lymphocytes, NK-cells, B-cells subsets and the ratio of CD4/CD8 did not differ from the control group. But the absolute number of Naive CD4+, and the percentage of Effector memory CD4+ cells, and the total number of Treg cells and of Naive CD8+ cells were decreased. The percentage number of the Сentral memory CD4+ and CD8+ cells and the total number of the Terminal effector CD4+ cells were increased. Conclusion: the data obtained allow us to better understand the pathogenesis of cellular immunity changes in patients with CKD-5. This could help to correct such changes and therapeutic tactics in the future

Therapeutic plasma exchange (PE) is a procedure of removing and replacing a large volume of plasma with various biologically active substances that have a pathological effect on the patient. Plasma exchange in some diseases is one of the components of pathogenetic therapy (ANCA-associated systemic vasculitis, antiphospholipid syndrome, Goodpasture’s syndrome, thrombotic microangiopathy, etc.). PE removes circulating immune complexes damaging tissues and organs and restores the coagulation system. In the practical recommendations of the American Apheresis Society (2016) leading experts in the field of apheresis therapy, based on an analysis of numerous clinical studies, gave clear recommendations to use the apheresis technologies for extracorporeal detoxification. Also 4 categories of indications and contraindications, assessing the benefits of carrying out apheresis procedures for a specific nosology were specified. A number of new diseases have been introduced, such as atopic (neuro) dermatitis (atopic eczema), cardiac neonatal lupus, Hashimoto encephalopathy, HELLP syndrome, in which PE is one of the leading therapeutic approaches as well as cytotoxic and glucocorticosteroid therapy. However, the use of plasma exchange in the treatment of some diseases remains controversial, for example, sepsis. So further research is needed.

Fabry-Anderson disease is an orphan (very rare) hereditary X-linked disease related to lysosomal storage diseases, in which there is a deficiency of the enzyme α-galactosidase-A, which leads to the accumulation of sphingolipids in the tissues of vital organs. The disease is diagnosed by determining the level of α-galactosidase-А in the blood, as well as by identifying the mutation of the GLA gene responsible for the production of α-galactosidase-А. The disease is manifested by the defeat of vital organs: the brain, peripheral nervous system, the kidney, which directly affects both the early disability and mortality of these patients. The prognosis for the life of these patients improved with the advent of enzyme replacement therapy. The article presents 5 clinical observations of patients with Fabry-Anderson's disease. All observed patients are male at the time of diagnosis of Fabry-Anderson's disease from 19 to 42 years old (average age 30 years). Kidney and heart damage were found in all patients, two of them receive renal replacement therapy with hemodialysis. All patients receive enzyme replacement therapy. The detailed description of anamnesis, features of clinical manifestations of the disease, process of diagnostics and treatment of the observed patients is given.

Background: the results of studies on the efficiency and safety of direct-acting antivirals became available only in recent years. Data from two randomized clinical trials (RUBY-1 and C-SURFER) allowed the use of a combination of Grazoprevir-Elbasvir and Ombitasvir-Paritaprevir-Ritonavir in patients with hepatitis C and CKD on hemodialysis. However, because of the high cost and absence of government’s medical insurance, they are not available to most patients. Therefore, generic drugs became an alternative in Kyrgyzstan. One of them is a combination of Sofosbuvir and Ledipasvir. The accumulated data from different single-centered studies suggests safety and effectiveness of its use in the treatment of patients with hepatitis C and End Stage Renal Disease. Aim of this article is to show the result of the safe and effective use of an antiviral drug of direct action in a patient on hemodialysis with systemic vasculitis on the background of maintenance therapy with glucocorticosteroids. Methods: we report on the 55-year female patient on maintenance hemodialysis with Systemic vasculitis and Hepatitis C, who received combination of direct acting anti-viral drugs such as Sofosbuvir and Ledipasvir. Results: HCV RNA was negative after 2 weeks of treatment. Therapy was well tolerated without adverse effects. There was no necessity to correct HD or main disease’s treatment. Sustained virological response was achieved after 24 weeks. Conclusion: full dosage of Sofosbuvir-Ledipasvir therapy for hepatitis C patient on HD with systemic vasculitis was effective and safe without discontinuation or severe side effects. However, final conclusion about safety and efficacy of such treatment must be based on prospective multicenter studies.