[Comparative characteristics of the complement system in patients with C3-glomerulopathy and atypical hemolytic uremic syndrome of chronic course who suffered an acute episode of thrombotic microangiopathy].

AIM: To compare changes in the complement system in C3-glomerulopathy (C3-GP) and atypical hemolytic uremic syndrome (aHUS) after the relief of an acute episode of thrombotic microangiopathy. MATERIALS AND METHODS: The study included 8 patients diagnosed with C3-GP and 8 with aHUS in remission. The blood levels of the complement system components were determined: C3, C4, C3a, C5a, factor H (CFH), factor B (CFB), membrane-attacking complex (MAC), antibodies to C3b (anti-C3b-AT), the level of hemolytic activity (CH50), the content of factor D (CFD) in the urine. RESULTS: C3 and CH50 levels were within the reference range in both groups, however, in the C3-GP group they were at the lower limit, and C3 level was significantly lower than in the aHUS group: 0.56 [0.44; 0.96] vs 1.37 [1.16; 2.52] (p=0.003). CFB increased level was detected in both groups, but in the C3-GP group it was significantly lower than in the aHUS group - 275.1 [222.1; 356.6] vs 438.7 [323.3; 449.3] (p=0.010). C3a, C5a and MAC levels were increased in both groups, but the maximum was in the C3-GP group, and the MAC level in the C3-GP group was 2 times higher than that in aHUS, and these differences reached statistical significance - 123 555±6686 vs 5603±1294 (p=0.036). CFH and CFD levels was increased in both groups, but their highest values was in the aHUS group. CONCLUSION: Alternative complement pathway activation signs were present in both groups of patients with complement-mediated nephropathies, regardless the stage of the disease. In C3-GP, alternative complement pathway activation was more pronounced than in aHUS after the relief of an acute episode of thrombotic microangiopathy.

[Nephrotic syndrome during pregnancy. Is it chronic glomerulonephritis or preeclampsia? Case report].

Nephrotic syndrome (NS) during pregnancy is a fairly rare pathology and its descriptions in the literature are few. For a long time, NS was associated only with an exacerbation of chronic glomerulonephritis or de novo nephritis, however, the experience of recent years has shown that NS can be a manifestation of the classical obstetric pathology - preeclampsia (PE). The appearance of massive proteinuria with the development of NS is most typical for early PE, which, of course, makes diagnosis difficult, especially if PE develops at an unusually early time (up to 20 weeks). To describe PE that does not fit into the classical criteria, the term "atypical" PE is now used, the development of which can be promoted by both obstetric and somatic risk factors. The presented clinical observation describes the development of early (within 14 weeks) severe PE with the NS at the onset of the disease in a patient with the first multiple pregnancy and complete hydatidiform mole (HM) of one of the fetuses. The progression of nephropathy with the addition of thrombotic microangiopathy and HELLP syndrome made it possible to assume the diagnosis of PE with a high probability. The rapid relief of all clinical manifestations after delivery confirmed this assumption. The role of HM as the main trigger of unusually early PE is discussed. Apparently, the patient's trophoblast disease in the form of hydatidiform mole caused the formation of a severe angiogenic imbalance already in the early stages of pregnancy, which led to the development of PE, which manifested NS as a consequence of podocytopathy due to VEGF deficiency. Thus, the development of NS in a pregnant patient without a history of kidney disease dictates, first of all, the exclusion of PE, until proven otherwise.

[Multifocal analysis of the bioelectric potential and light sensitivity of the retina in thrombotic microangiopathy associated with malignant arterial hypertension]. / Mul'tifokal'nyi analiz elektricheskogo biopotentsiala i svetovoi chuvstvitel'nosti setchatki pri tromboticheskoi mikroangiopatii, assotsiirovannoi so zlokachestvennoi arterial'noi gipertenziei.

PURPOSE: This study evaluates the function of the retina according to multifocal electroretinography (mfERG) and its light sensitivity according to microperimetry (MP) in patients with thrombotic microangiopathy (TMA) associated with malignant hypertension (MH). MATERIAL AND METHODS: The study analyzed mfERG and MP data of 20 patients (40 eyes) aged 40.4±7.4 years (18 men, 2 women) with MH-associated TMA. In all patients TMA of the kidneys was verified by nephrobiopsy. The control group consisted of 20 healthy individuals (40 eyes) of the appropriate age. RESULTS AND DISCUSSION: A statistically significant decrease in the response density of P1 mfERG (nV/deg2) of the central retinal zone (0-27.7°) was found in study patients in comparison with the control group (p<0.05), differences in the latency of P1 mfERG (ms) were statistically insignificant (p>0.05). Analysis of MP data in study patients revealed a statistically significant decrease in the mean light sensitivity (dB) of the central field of vision (30°) (p<0.05) compared to the control group. A statistically significant correlation was found between the response density of P1 mfERG (nV/deg2) and mean light sensitivity (dB) in the corresponding quadrants of the visual field (p<0.05). A number of statistically significant correlations were found between the indicators of MP and mfERG and some non-ocular clinical manifestations of TMA in MH. CONCLUSION: A statistically significant decrease in the light sensitivity of the central field of vision caused by marked decrease in retinal function, probably of an ischemic nature, is characteristic for MH-associated TMA. In this disease the response density of P1 mfERG (nV/deg2) is a sensitive indicator of impaired retinal function. With the activation of systemic TMA, increase in blood pressure and deterioration of kidney function in MH, the light sensitivity of the eye also decreases.

[Biomarkers of retinal ischemia in thrombotic microangiopathy associated with malignant arterial hypertension on optical coherence tomography]. / Markery retinal'noi ishemii pri tromboticheskoi mikroangiopatii, assotsiirovannoi so zlokachestvennoi arterial'noi gipertenziei, po dannym opticheskoi kogerentnoi tomografii.

Malignant arterial hypertension is a clinical syndrome characterized by severe diastolic arterial hypertension with signs of ischemic damage to various organs. In some malignant arterial hypertension cases, thrombotic microangiopathy occurs - a rare life-threatening condition characterized by multiple systemic thrombosis of the microvasculature, including in the eyes, which can be clarified by optical scanning of the retina. PURPOSE: To determine markers of retinal ischemia in the eyes with thrombotic microangiopathy associated with malignant arterial hypertension. MATERIAL AND METHODS: The study included 6 patients (12 eyes) with thrombotic microangiopathy associated with malignant arterial hypertension who were examined by optical coherence tomography (OCT) and OCT angiography (OCT-A). All patients suffered from renal dysfunction, which etiology was determined by renal biopsy verifying the presence of renal thrombotic microangiopathy in all cases. RESULTS: According to OCT findings, there were bilateral local foci of thinning of the inner nuclear layer with elevation of the outer plexiform and outer nuclear layers of the retina in 5 out of 6 patients (83%). OCT-A revealed that in most cases (67%), these changes had perivascular localization and corresponded to the areas of attenuation of the deep capillary plexus. A statistically significant thinning of the inner nuclear layer of the retina was found in thrombotic microangiopathy associated with malignant arterial hypertension in comparison with the control group. CONCLUSION: Presence of renal thrombotic microangiopathy confirmed by renal biopsy and the anatomical similarity of the microvasculature of the kidneys and the eyes, give basis to consider the foci of «chronic¼ paracentral acute middle maculopathy detected with OCT in patients with malignant arterial hypertension as biomarkers of thrombotic microangiopathy of the eye.

[The role of optical coherence tomography angiography in the assessment of retinal microcirculation in thrombotic microangiopathy associated with malignant arterial hypertension]. / Rol' opticheskoi kogerentnoi tomografii s funktsiei angiografii v otsenke retinal'noi mikrotsirkulyatsii pri tromboticheskoi mikroangiopatii, assotsiirovannoi so zlokachestvennoi arterial'noi gipertenziei.

Optical coherence tomography angiography (OCT-A) allows detailed assessment of the state of local blood flow in various systemic ischemic diseases, which include thrombotic microangiopathy (TMA) associated with malignant arterial hypertension (MAH). PURPOSE: To evaluate retinal microcirculation using OCT-A imaging data in the group of patients with TMA associated with MAH. MATERIAL AND METHODS: The study consisted of a qualitative and quantitative analysis of OCT-A data from 7 patients (14 eyes) with TMA associated with MAH and verified by renal biopsy. The control group included 7 healthy individuals (14 eyes). The following parameters were analyzed: area size of the foveal avascular zone FAZ, mm, vessel density (VD, %) and vessel skeleton density (VSD, %). The indicators were measured separately in the deep and superficial vascular plexuses and divided by localization into: the total density of the entire measured area, central density, density by quadrants (superior, inferior, nasal and temporal). All quadrants were subdivided into inner (corresponding to parafovea) and outer (corresponding to perifovea). RESULTS: Local foci of vascular attenuation of the deep retinal capillary plexus, pathological tortuosity of blood vessels in the deep and superficial retinal capillary plexuses were found in 100% of study group patients. Per-quadrant analysis of OCT-A finding in the macular zone revealed a statistically significant decrease in VD of the deep retinal capillary plexus (p<0.05). A direct correlation was found between glomerular filtration rate and the VD index in the outer inferior (r=0.567; p=0.034) and inner temporal quadrants (r=0.613; p=0.020) of the deep retinal capillary plexus, as well as the level of hemoglobin in blood serum and VD in inner temporal quadrant of the deep vascular plexus (r=0.596; p=0.025). CONCLUSION: Changes revealed with OCT-A are the symptoms of TMA in MAH patients spreading to the retinal microvasculature, mainly at the level of the deep retinal capillary plexus.

[Ocular ischemic changes in thrombotic microangiopathy associated with malignant arterial hypertension (case study)]. / Ishemicheskie izmeneniya glaza pri tromboticheskoi mikroangiopatii, assotsiirovannoi so zlokachestvennoi arterial'noi gipertenziei (klinicheskoe nablyudenie).

The article presents a clinical observation of visual organ damage as an extrarenal manifestation of thrombotic microangiopathy in malignant arterial hypertension with an analysis of structural and functional changes in the eye by modern examination methods. The presence of renal thrombotic microangiopathy was verified by kidney biopsy. Optical coherence tomography was used to determine structural changes in the inner and outer layers of the retina in the form of their disorganization, areas of thickening of the nerve fiber layer, intraretinal inclusions and cysts, alterations of the ellipsoid zone and retinal pigment epithelium. Optical coherence tomography angiography revealed areas of vascular pattern attenuation in the deep and superficial retinal plexuses corresponding to OCT-detected foci of disorganization of the inner retinal layers. Color Doppler imaging showed absence of the diastolic component of the Doppler spectrum with an increase in the peripheral resistance index RI up to 1.0 in the central retinal artery and posterior short ciliary arteries in both eyes. Functional changes observed with multifocal electroretinography and microperimetry corresponded to the structural changes. Taking into account the similarity of microcirculation in the kidneys and the eyes, the probable pathogenetic mechanism underlying the detected structural and functional symptoms of ischemic damage to the visual organ in malignant arterial hypertension is chronic thrombotic microangiopathy. An interdisciplinary approach to studying thrombotic microangiopathy, including those associated with malignant arterial hypertension, using high-tech examination methods can be useful for timely diagnosis and treatment of this pathology.

[Ocular manifestations of primary thrombotic microangiopathy]. / Glaznye proyavleniya pervichnoi tromboticheskoi mikroangiopatii.

The article reviews literature on ocular manifestations of primary thrombotic microangiopathy. Thrombotic microangiopathy is a clinical and morphological syndrome that characterizes a systemic disseminated lesion of the microcirculatory bed, it is a special type of vascular damage that combines thrombosis and inflammation of the vascular wall leading to occlusion of the vessel lumen, causing ischemic damage to various organs and tissues. The classic types of primary thrombotic microangiopathy are thrombotic thrombocytopenic purpura, shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) and atypical hemolytic uremic syndrome. Thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome are characterized by ischemic damage to the visual organ as a result of suspected thrombotic microangiopathy in the microcirculatory bed of the eye. The clinical picture of ocular manifestations of these diseases is similar, however the damage to the eye in the form of ischemic Purtscher-like retinopathy is more characteristic of atypical hemolytic uremic syndrome, which may be due to chronic uncontrolled activation of the alternative complement pathway in this disorder. Timely initiation of systemic therapy of the underlying disease in most cases leads to complete restoration of visual functions. A thorough ophthalmological examination of patients with these diseases is required.

[Analysis of the course and outcomes of pregnancy in patients with advanced stages chronic kidney disease].

BACKGROUND: Pregnancy in patients with advanced chronic kidney disease (CKD) is associated with a high risk of adverse outcomes for the mother and the fetus, but data on the characteristics of the course of pregnancy in these women is limited. AIM: To analyse of the course and outcomes of pregnancy in patients with CKD stages 3a4. MATERIALS AND METHODS: Thirty five pregnant women with CKD stages 34 were included: 3a 12 (34.3%) patients, stage 3b 10 (28.6%), stage 4 13 (37.1%). RESULTS: Proteinuria, serum creatinine, blood pressure in dynamics, the presence of a physiological response were investigated. Pregnancy management included blood pressure correction, antianemic, antiplatelet, anticoagulant therapy, prevention and treatment of urinary infection, correction of metabolic disorders. All pregnant women had proteinuria of varying severity, which increased towards the end of pregnancy. Seventeen (51.5%) patients had hypertension, successfully corrected with antihypertensive drugs. The average delivery term was 34.6 weeks. Preeclampsia developed in 14 (42.4%) cases, an inverse relationship was found between the presence of a physiological response and preeclampsia (p=0.009; rs=-0.463). All children were born alive and viable. After delivery in patients with CKD 3a creatinine values returned to the pre-gestational level, in patients with grade 3b and 4 progression of CKD was noted. CONCLUSION: A favorable pregnancy outcome in women with late stages of CKD is possible with constant monitoring by a multidisciplinary team of doctors with mandatory monitoring of renal function, proteinuria, blood pressure, coagulation, markers of preeclampsia and indicators of fetal health. It was proposed to consider the physiological response of the kidneys to pregnancy as a predictor of a favorable outcome.

[Thromboembolic complications in nephrotic syndrome].

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.

[Optical coherence tomography angiography in the diagnosis of retinal microangiopathy in atypical hemolytic-uremic syndrome (a case report)]. / Opticheskaya kogerentnaya tomografiya-angiografiya v diagnostike mikroangiopatii setchatki pri atipichnom gemolitiko-uremicheskom sindrome (klinicheskoe nablyudenie).

The article describes a clinical case of acute macular neuroretinopathy and «chronic¼ paracentral acute middle maculopathy in a patient with atypical hemolytic-uremic syndrome - an orphan disease characterized by systemic thrombosis in the vessels of the microcirculatory bed due to chronic uncontrolled activation of the alternative complement pathway. Optical coherence tomography angiography data confirm the ischemic nature of the disease with localization in the deep vascular plexus of the retina in acute macular neuroretinopathy and in the superficial vascular plexus of the retina in «chronic¼ paracentral acute middle maculopathy. The use of modern diagnostic methods, including optical coherence tomography angiography, can help improve detection of the pathology and expand its understanding in severe microangiopathic syndromes, which include atypical hemolytic-uremic syndrome.

[Ophthalmic aspects of vascular and functional changes in malignant arterial hypertension of renal origin]. / Oftal'mologicheskie aspekty sosudistykh i funktsional'nykh izmenenii pri zlokachestvennoi arterial'noi gipertonii pochechnogo geneza.

Malignant arterial hypertension is a clinical syndrome characterized by severe diastolic arterial hypertension with signs of ischemic damage to target organs - kidneys, heart, brain, eyes. Malignant arterial hypertension can be one of the reasons, as well as a consequence of thrombotic microangiopathy - a special type of vascular lesions of the organ microvasculature. Ocular manifestations of arterial hypertension include hypertensive retinopathy of varying severity, choriopathy. The appearance of new diagnostic research methods allows a detailed study of eye damage in malignant arterial hypertension, whether associated or not with thrombotic microangiopathy, and to analyze the relationship of ocular and systemic manifestations of this disease to determine the nature and degree of involvement of the organ of vision in the pathological process and identify prognostic signs of disease progression and its forms.

[Prevalence of Advanced Chronic Kidney Disease in Patients with Nonvalvular Atrial Fibrillation Hospitalized in Cardiology Departments].

OBJECTIVE: To estimate the prevalence of chronic kidney disease (CKD) 3b - 5 stages and the newly diagnosed sustained reduction in glomerular filtration rate (GFR) <30 ml / min / 1.73 m2 in patients with atrial fibrillation (AF) in real clinical practice, as well as the features of their anticoagulant therapy. MATERIALS AND METHODS:  Retrospectively, data of all discharge epicrisis from cardiological departments of five Moscow hospitals from June 1, 2016 to May 31, 2017 were analyzed. Patients over 18 years old with AF were enrolled. At the next stage, patients with CKD 3 b - 5 st and newly diagnosed sustained reduction in GFR <30 ml / min / 1.73 m2 (at least 2 measurements during hospitalization) were selected. RESULTS: Data of 9725 patients were analyzed, AF was diagnosed in 2983 (31 %) cases, of which a decreased GFR <45 ml / min / 1.73 m2 was detected in 27 % (n = 794) cases. Among them, 349 (44 %) were diagnosed with CKD 3b st, 123 (15 %) with CKD 4 st, 44 (6 %) with CKD 5 st, 278 (35 %) had a newly diagnosed sustained reduction in GFR. In 63 % of patients with AF and GFR <45 ml / min / 1.73 m2, anemia was diagnosed, 39 % of them had moderate and severe one. 711 (89 %) patients were prescribed anticoagulants, 53 % were assigned direct oral anticoagulants (DOACs). Patients with CKD 3 b st. more often rivaroxaban 15 mg (29 %) was prescribed, with CKD 4 and CKD 5 - warfarin (48 % and 25 %, respectively), in patients with newly diagnosed sustained reduction in GFR <30 ml / min / 1.73 m2 - apixaban 10 mg / day (16.2 %). CONCLUSION: A quarter of patients with AF revealed a decreased GFR <45 ml / min / 1.73 m2, half of them were recommended DOACs. 42 % of patients with GFR <30 ml / min / 1.72 m2 were prescribed DOACs, 27 % - warfarin. Patients with CKD 5 st DOACs were not assigned; in half of cases, none of the anticoagulants was recommended. Most often, the dose of the prescribed anticoagulant was not counted according to GFR in patients with newly diagnosed sustained reduction in GFR <30 ml / min / 1.73 m2.

[Safety performance of rivaroxaban versus warfarin in patients with atrial fibrillation and advanced chro-nic kidney disease].

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.

[The prognostic value of the method for determining the estimated glomerular filtration rate in chronic heart failure with a preserved left ventricular ejection fraction: focus on cystatin C].

The aim of the present study is to determine the prognostic value of GFR reduction according to the CKD-EPI formula, taking into account blood creatinine and a formula that simultaneously takes into account creatinine and cystatin C in patients who were hospitalized for the first time due to decompensation of chronic heart failure with a preserved left ventricular ejection fraction (HFSA) observation within 24 months. MATERIALS AND METHODS: The study included 117 patients (women - 65.8%, mean age 71.6 ± 9.1 years) hospitalized due to debugging of CHF and having a preserved left ventricular ejection fraction according to echocardiography. The study was a prospective observation for 2 years after the inclusion of each patient. On the first day of hospitalization, all serum samples were taken to determine the level of cystatin C. The estimated glomerular filtration rate (eGFR) was determined using the CKD-EPI formula, taking into account blood creatinine and the combined formula, including creatinine and cystatin C. The combination was used as an end point death and re-hospitalization within two years of follow-up. To determine the effect of a decrease in eGFR on the forecast, the Kaplan-Maer method and the log-rank test were used. Differences were considered statistically significant at p<0.05. The study was approved by the local ethics committee. RESULTS: During the observation period, the mortality rate was almost 12%. At the same time, every third patient was repeatedly hospitalized within two years. In order to determine the effect of reducing GFR on reaching the end points, all patients were divided into groups with eGFR values of more or less than 45 ml/min/1.73 sq.m according to both formulas. When separating patients using the CKD-EPI formula, which includes only creatinine, the groups did not differ in terms of the frequency of reaching the combined end point, as well as its components: death and re-hospitalization. However, patients with eGFR values less than 45 ml/min/1.73 sq. M according to the combined formula data significantly more often reached the combined end point, mainly due to an increase in mortality. CONCLUSION: The data obtained suggest that adding cystatin C to the CKD-EPI formula and appropriately identifying patients with reduced eGFR has a high prognostic value for stratifying the risk of an unfavorable outcome after the first decompensation of HFSSFV.

[Ocular thrombotic microangiopathy in atypical hemolytic-uremic syndrome (a clinical case study)]. / Tromboticheskaia mikroangiopatiia glaza pri atipichnom gemolitiko-uremicheskom sindrome (klinicheskoe nabliudenie).

The article presents a clinical observation of ocular thrombotic microangiopathy in atypical hemolytic-uremic syndrome - a rare genetic disease characterized by systemic thrombosis caused by uncontrolled activation of alternative complement pathway. A typical manifestation of this ocular lesion in this disease is bilateral Purtscher-like retinopathy. Timely diagnostics of atypical hemolytic-uremic syndrome, including ophthalmologic examination, determines the early start of a highly effective pathogenetic therapy with complement inhibitor eculizumab.

Atypical hemolytic-uremic syndrome as one of the causes of acute kidney injury in pregnant women.

AIM: Analysis of clinical manifestations, course and outcomes of obstetric aHUS. MATERIALS AND METHODS: 45 patients with aHUS development during pregnancy or immediately after childbirth were observed between 2011 and 2017, age from 16 to 42 years. RESULTS: All patients had AKI (serum creatinine 521,5±388,0 µmol/l, oliguria or anuria that required initiation of hemodialysis). 93.3% pts had extrarenal manifestations of TMA with the development of multiple organ failure (MOF). The mean number of damage organs was 3,7±1,2. In all patients, the development of aHUS was preceded by obstetric complications, surgery, infection, etc. In the outcome: 53.4% women showed complete recovery of renal function, 11.1% developed CKD 4-5 stages, 35.5% had dialysis-dependent end-stage renal failure (ESDR). Maternal mortality was 23.9%. Perinatal mortality was 32.6%. The early start of eculizumab treatment (within 1-2 weeks from the onset of aHUS), compared with therapy start after 3 weeks, increased the chances of favorable outcome for mother in 5.33 times, and the chances for normalization of renal function in 48.7 times. CONCLUSION: Obstetric aHUS is characterized by the development of AKI in 100% of cases. In most patients, the obstetric aHUS occurs with the development of MOF. Timely diagnosis of aHUS and immediate treatment by eculizumab allows not only to save the life of patients, but also completely restore their health.

[Difficulties in the diagnosis of diarrhea-associated hemolytic uremic syndrome in adults]. / Trudnosti diagnostiki gemolitiko-uremicheskogo sindroma, assotsiirovannogo s diareei, u vzroslykh.

Hemolytic uremic syndrome (HUS) is a rare, but menacing condition registered mainly in children. The paper gives a detailed description and analysis of a clinical case of HUS with a favorable outcome in an adult woman who developed the syndrome in the presence of bloody diarrhea. It considers an update on the etiology, pathogenesis, and clinical features of HUS associated with diarrheal syndrome and discusses differential diagnostic features, diagnostic problems, and characteristics of management tactics for patients.

[Hyponatremia: A clinical approach]. / Giponatriemiia: klinicheskii podkhod.

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.

[Electrophysiological studies of the retina in antiphospholipid syndrome]. / Élektrofiziologicheskoe issledovanie setchatki pri antifosfolipidnom sindrome.

AIM: to evaluate the functional state of the retina in patients with antiphospholipid syndrome (APS) through different types of electroretinography (ERG). MATERIAL AND METHODS: The total ERG (t-ERG), local macular ERG (m-ERG), and oscillatory potentials (OP) were recorded in 56 patients with verified APS. RESULTS: In most patients with APS, ERG was of abnormal appearance, particularly, t-ERG was notable for reliably lower a- and b-wave amplitudes (70% of cases), m-ERG - for lower amplitudes and shorter latency (64% of cases). Pathological changes of t-ERG and m-ERG components were equally common in patients with and without retinal vascular occlusions. Linear dependence was established between the decrease in b-wave amplitude of subnormal t-ERG and the Vs value in the central retinal artery. A decrease in the OP index and OP configuration distortion were found in 82% of patients. At that, OP indices tended to be lower in patients from the retinal vascular occlusions group. CONCLUSION: The use of different types of ERG enable detection of not only local, but also generalized retinal changes in APS. Regardless of the presence of retinal occlusion, APS patients show suppression of biopotential in most of cellular elements of the retina due to ischemia. Despite high visual acuity, the local macular potential gets significantly reduced in more than half of APS patients. Oscillatory potentials recording is a highly sensitive method for diagnosis of retinal ischmia in APS.

[Changes in the complement system in membranoproliferative glomerulonephritis]. / Izmeneniia v sisteme komplementa pri membranoproliferativnom glomerulonefrite.

AIM: To compare the clinical manifestations membranoproliferative glomerulonephritis (MPGN) in its idiopathic variant, lupus nephritis (LN), and C3 glomerulopathy (C3-GP), by comparing them with changes in the complement system. SUBJECTS AND METHODS: The clinic of nephrology followed up 42 patients with different types of MPGN in 2013 to 2015. The study included 35 patients divided into 3 groups: 1) 8 patients with C3-GP, 2) 13 with idiopathic MPGN; 3) 14 with Class IV LN. The investigators studied the blood and urine levels of components and markers for activation of the classical and alternative pathways (C3 and C4, С3а, C5a, CFH, CFB, and CFD) of the terminal complement complex (TCC). RESULTS: The detection rate of C3-GP was 19%. The patients with C3-GP were noted to have the lowest blood concentration of S3 and the highest urinary level of С3а, C5a, TCC, CFH, CFB, and CFD. C3 nephritic factor was detected in 2 patients from the C3-GP (dense deposit disease) group. CONCLUSION: Alternative complement pathway dysregulation caused by genetic or autoimmune factors plays a leading role in the pathogenesis of C3-GP.