This article presents a clinical case of ocular thrombotic microangiopathy of mixed origin (antiphospholipid syndrome, malignant arterial hypertension, multigenic thrombophilia). Multimodal imaging of the fundus provides a detailed assessment of its structures. Pathological changes in the choroid, the "retinal pigment epithelium - Bruch's membrane" complex, and the neurosensory retina, identified using fundus photography, short-wavelength autofluorescence, optical coherence tomography (OCT), and OCT angiography, are described as nonspecific in nature.
View Article and Find Full Text PDFLupus nephritis (LN) is one of the most common organ-specific manifestations of systemic lupus erythematosus (SLE). Various clinical signs of LN develop in at least 50% of patients with SLE. In addition to LN, the spectrum of renal lesions associated with SLE also includes vascular pathology.
View Article and Find Full Text PDFBackground: The spectrum of diseases characterized by the development of renal thrombotic microangiopathy (TMA) encompasses the malignant hypertension (MHT). TMA in MHT has conventionally been regarded as a variation of secondary TMA, the treatment of which is restricted to the stabilization of blood pressure levels, a measure that frequently fails to prevent the rapid progression to end-stage renal disease in patients. Nevertheless, there exists a rationale to suggest that, in certain instances, endothelial damage in MHT might be rooted in the dysregulation of the complement system (CS), thereby presenting potential opportunities for the implementation of complement-blocking therapy.
View Article and Find Full Text PDFNephrotic 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 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).
View Article and Find Full Text PDFAim: 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.
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.
Unlabelled: 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.
Unlabelled: 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.
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.
View Article and Find Full Text PDFThe 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.
View Article and Find Full Text PDFBackground: 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.
In this work we present a comprehensive study of the domain structure of a nickel oxide single crystal grown by floating zone melting and suggest a correlation between point defects and the observed domain structure. The properties and structure of domains dictate the dynamics of resistive switching, water splitting and gas sensing, to name but a few. Investigating the correlation between point defects and domain structure can provide a deeper understanding of their formation and structure, which potentially allows one to tailor domain structure and the dynamics of the aforementioned applications.
View Article and Find Full Text PDFAim 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.
View Article and Find Full Text PDFThis 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.
View Article and Find Full Text PDFMalignant 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.
View Article and Find Full Text PDFThe article describes a clinical case of acute macular neuroretinopathy and «chronic» 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 «chronic» 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.
View Article and Find Full Text PDFObjective: 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.
Unlabelled: 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.
Determining the prognosis of heart failure with preserved ejection fraction (HFpEF) is problematic, as the ejection fraction cannot be used. Formulae that estimate glomerular filtration rate (eGFR) may be potential prognosticators for this condition, since renal dysfunction is a well-known predictor of poor outcomes of all forms of heart failure. A prospective observational study of 117 HFpEF patients (average age 71.
View Article and Find Full Text PDFAim: 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).
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.
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