Aim To evaluate safety of using rivaroxaban in patients with stage 4 chronic kidney disease (CKD) or transient, stable decline of glomerular filtration rate (GFR) to 15–29 ml /min / 1.73 m2 in the presence of atrial fibrillation (AF). Material and methods This multicenter prospective, randomized study included patients admitted to cardiology departments from 2017 through 2019. Of 10 224 admitted patients 109 (3 %) patients with AF and stage 4 CKD or a stable decline of GFR to 15–29 ml /min / 1.73 m2 were randomized at 2:1 ratio to the rivaroxaban 15 mg /day (n=73) treatment group or to the warfarin treatment group (n=36). The primary endpoint was development of BARC and ISTH major, minor, and clinically relevant minor bleeding. Mean follow-up duration was 18 months. Results Patients receiving warfarin had a significantly higher incidence of BARC (n=26 (72.2 %) vs. n=31 (42.4 %), р<0.01) and ISTH (n=22 (61.1 %) vs. n=27 (36.9 %), p<0.01) minor bleeding and all ISTH clinically relevant (minor clinically relevant and major bleedings) n=10 (27.7 %) vs. n=8 (10.9 %), р=0.03]. The number of repeated hospitalizations was 65 (43% of patients) in the rivaroxaban treatment group and 27 (48% of patients) in the warfarin treatment group (р=0.57), including 24 (36.9 %) and 11 (40.7 %) emergency admissions in the rivaroxaban and warfarin treatment groups, respectively (р=0.96). Significant improvement of changes in creatinine clearance and GFR (by CKD-EPI and Cockroft-Gault) was observed in the rivaroxaban treatment group. Conclusion The study provided evidence for a more beneficial safety profile of rivaroxaban compared to warfarin in patients with AF and advanced CKD.

Objective: The aim of this case series is to raise awareness of obstetric-related atypical haemolytic uraemic syndrome (aHUS) amongst obstetricians and gynaecologists. Study design: Data from 20 consecutive patients, aged 19–38, with obstetric-aHUS manifestation during or immediately after pregnancy are reported. Patients were diagnosed and treatment was initiated between 2012 and 2016. Results: Presentation of aHUS was mainly preceded by preeclampsia and/or haemolysis, elevated liver enzymes and low platelet count syndrome, other obstetric complications, or by diarrhoea. Thrombotic microangiopathy (TMA) was evident in all patients with signs of microangiopathic haemolysis (sharp decline in haemoglobin; mean 67 g/L), elevated lactate dehydrogenase (LDH; mean 2953.1 U/L), schistocytosis, thrombocytopenia (mean platelet count 52.5 × 10⁹/µL), and acute kidney injury (AKI) (hypercreatininaemia, mean 456.4 µmol/L; oliguria or anuria). The majority of patients (80%) initially presented with arterial hypertension. Diagnosis of obstetric-aHUS was complicated, as multiple organs were affected. Time taken to make the diagnosis of aHUS delayed the initiation of fresh-frozen plasma infusions and plasma exchange (80% of patients) and subsequent eculizumab treatment (40% of patients). Maternal mortality was high (35%) as was foetal mortality (25%). Conclusions: Obstetric-aHUS is a serious condition characterized by multiple organ failure (MOF) and a high mortality rate. Presentation of obstetric-aHUS is preceded by various precipitating factors, suggesting pregnancy complications, and not the pregnancy per se, often induce aHUS in women with a genetic predisposition to its development. A delay in the correct diagnosis and initiation of the most effective treatment can have serious consequences, reinforcing the need to raise awareness of obstetric-aHUS.

The consensus was developed by experts from the Russian Scientific Medical Society of Internal Medicine, the Russian Society for the Prevention of Noncommunicable Diseases, the National Nephrology Association, the Professional Nephrology Association, the Russian Society of Cardiology, the Russian Medical Society for Arterial Hypertension, the Heart Failure Society, the National Atherosclerosis Society, the Russian Endocrinology Association, and the Russian Gerontology and Geriatrics Association.The expert consensus opinion was compiled on the basis of the relevant sections of national and international clinical guidelines on chronic kidney disease, prevention and treatment of atherosclerosis, hypertension, diabetes and heart failure.

Атипичный гемолитикоуремический синдром (аГУС) ультраредкое (орфанное) заболевание прогрессирующего течения, представляющее собой системную тромботическую микроангиопатию (ТМА) вследствие хронической неконтролируемой активации альтернативного пути комплемента. В настоящее время установлено, что генетические аномалии комплемента, которые раньше рассматривали как основную причину заболевания, являются лишь фактором, предрасполагающим к возникновению ТМА. Классическую триаду клинических проявлений аГУС составляют тромбоцитопения, микроангиопатическая гемолитическая анемия и острое повреждение почек. У большинства пациентов независимо от возраста отмечается артериальная гипертензия, основными причинами которой являются гиперренинемия вследствие ишемии ткани почек, обусловленной ТМА, и перегрузка объемом при наличии олиго/анурии. Атипичный ГУС представляет собой системную ТМА, при которой могут поражаться не только почки, но и другие жизненно важные органы головной мозг, сердце, легкие, пищеварительный тракт, глаза и др. Диагноз аГУС это диагноз исключения. Он устанавливается на основании характерной клинической картины после исключения других форм ТМА, как первичных, так и вторичных. Для исключения тромботической тромбоцитопенической пурпуры (ТТП) всем больным с ТМА необходимо определение активности ADAMTS13: снижение ее до 10 и менее является диагностическим маркером ТТП. У пациентов с аГУС и другими ТМА активность ADAMTS13 может быть снижена, однако всегда превышает 10. Инновационным подходом к лечению аГУС, используемым в клинической практике в течение последнего десятилетия, стало применение экулизумаба препарата группы комплеменингибирующих антител, который является препаратом патогенетической терапии аГУС. Экулизумаб является средством первой линии для лечения детей с установленным диагнозом аГУС, при семейном характере заболевания, наличии экстраренальных проявлений. Совершенствование методов диагностики и внедрение новых подходов к лечению позволяет быстрее заподозрить и купировать проявления аГУС, добиться стойкой ремиссии и улучшить прогноз. Atypical hemolytic uremic syndrome (aHUS) is an ultra rare (orphan) progressive disease that is a systemic thrombotic microangiopathy (TMA) due to chronic uncontrolled activation of the alternative complement pathway. It is currently established that complement genetic abnormalities that were previously considered the main cause of the disease, are only a factor predisposing to TMA occurrence. The classic triad of aHUS clinical manifestations is thrombocytopenia, microangiopathic hemolytic anemia andacute kidney damage. Most patients, regardless of age, have arterial hypertension the main reasons of hypertension are hyperreninemia (due to kidney tissue ischemia because of TMA) and volume overload in the presence of oligo/anuria. Atypical HUS is a systemic TMA that affected not only the kidneys, but also other vital organs brain, heart, lungs, digestive tract, eyes, etc. The diagnosis of aHUS is a diagnosis of exclusion that is established on the basis of specifi c clinical picture after exclusion of other forms of TMA, both primary and secondary. To exclude thrombotic thrombocytopenic purpura (TTP), all patients with TMA need to determine ADAMTS13 activity: its reducing to 10 or less is a diagnostic marker of TTP. In patients with aHUS and other TMA, ADAMTS13 activity may be reduced, but always exceeds 10. The innovative pathogenetic approach to aHUS treatment over the last decade became the application of eculizumab that is a drug from complementinhibitory antibodies group. Eculizumab is a fi rstline treatment for children with proven aHUS diagnosis, with familial disease, with extrarenal manifestations. Progress in diagnostic methods and new treatment approaches allows us to quickly suspect and stop the manifestations of aHUS, achieve stable remission and improve the prognosis.

This review devoted to the nephrotic syndrome (NS) subsequent thrombotic outcomes. The pathogenesis of hypercoagulation disorders that cause venous and arterial vascular system thrombosis are studied. Discussed procoagulant and anticoagulant mechanisms imbalance due to the anticoagulants natural urinal loss, affected by disfunction of the glomerular filter selective permeability, leading to high molecular weight liver-derived proteins (at least of the albumin size) leakage, fibrinolysis depression, excessive liver synthesis of plasma clotting cascade factors and platelet activation. Presented new data on the thrombogenesis at NS concerning the role of endothelial microparticles with high prothrombogenic activity that go from damaged glomerulus endothelial capillary cells into the systemic circulation, which can turn the local renal hypercoagulation (concomitant to the kidney immune inflammation process) into the generalized, working towards the thrombosis development. The most frequent adverse variants of arterial and venous thromboses are studied, specified their basic and general risk factors, as well as individual, varying in different patients. Indications and prophylactic anticoagulant therapy regimen and thrombosis treatment duration in patients with NS are discussed. It also stressed that the decision on time and method of anticoagulant therapy for a NS patients is still a challenge for healthcare providers.

Hyponatremia is the most common electrolyte metabolic abnormality in clinical practice. The unfavorable course of many diseases is associated with hyponatremia. Acute severe hyponatremia is life-threatening because cerebral edema may develop. Less obvious chronic hyponatremia increases the risk of balance problems, falls and fractures, especially in elderly patients. In any occasion, hyponatremia should not be now regarded only as a laboratory phenomenon in critically ill patients, but it necessitates a thorough clinical analysis of each individual case and appropriate therapy. The paper presents approaches to diagnosing and treating hyponatremia in various clinical situations.