[Modern approaches to the detection of monoclonal gammopathy of undetermined significance (MGUS) in patients with kidney diseases].

Monoclonal gammopathy (MG) is not only the state preceding of hematological neoplasms, but also associated with non - hematological diseases, in particular kidney damage. AIM: To assess the diagnostic value of "Freelite" methods in addition to electrophoresis (EF) and immunofixation (IF) of serum and urine proteins for detecting MG in patients with kidney diseases. MATERIALS AND METHODS: 87 patients with kidney damage, in which MG was established using the method of electrophoresis of serum proteins (EF), immunofixation (IF) and the method of free light chains determination - FLC "Freelite" were selected. The diagnostic value of three - component serum panel was compared with EF and IF. RESULTS AND DISCUSSION: AL-amyloidosis with kidney involvement was diagnosed in 41% patients, cryoglobulinemic glomerulonephritis (cryo GN) - in 18%, chronic glomerulonephritis (CGN) - in 35%, also there was small number of patients with light chain disease and cast - nephropathy. Determination of MG using EP was possible only in 38 (44%). Adding to the serum electrophoretic methods instead of the "Freelite" method, the urine EF and IF reduced the number of missed patients with monoclonal gammopathy from 24 (27%) to 11 (13%), including in the subgroup of patients with AL-amyloidosis but did not reach the sensitivity of the three - component serum screening panel. In 10 (11.5%) MG was represented only by intact mIg with one type of light chain, either κ or λ. Most often - in 25% of patients, intact monoclonal gammopathy was observed in HCV (+) cryo GN. A combination of intact mIgM, mIgG or mIgA with mFLC, was detected in 37 (42.5%). In almost half (46%) of the patients, only mFLC was detected - an abnormal κ/λ ratio. CONCLUSION: The serum screening panel EF + IF + "Freelite" spreads the low - grade monoclonal gammopathy recognition (MGUS) and should be included in the algorithm of examining patients with kidney disease.

[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.

[Complement System Abnormalities in Patients with Atypical Hemolytic Uremic Syndrome and Catastrophic Antiphospholipid Syndrome].

Background: The role of the alternative complement pathway (AP) abnormalities in the pathogenesis of aHUS is well studied. Clinical and morphological manifestations of atypical HUS and catastrophic APS are often similar. However, studies on the state of AP in patients with CAPS are virtually absent. Aims: The aim of our study was to assess the state of AP in patients with CAPS and aHUS. Patients and methods: The study enrolled 67 patients (pts) with a diagnosis of CAPS (28 pts) and aHUS (39 pts). Studies of the complement system are made of 10 pts with CAPS and 20 aHUS. Factor H, I, B, D content, functional activity of factor H, and complement components C3, C4 was determined in serum by ELISA kit. Results: Patients with CAPS and aHUS showed similar changes in complement biomarkers. The factor H level in the serum was significantly higher than the standard value. However, the specific activity of factor H reduced, mean rate 59% for aHUS and 26% for CAPS. The median value of factor D was twice higher than the normal range in both groups, indicating the activation of the AP. Conclusions: There are indications of an AP activation not only in pts with aHUS but in CAPS pts too. We suppose that the activity of factor H is a more sensitive indicator of complement system changes than factor H level. Patients with CAPS and aHUS have similar clinical and laboratory characteristics. However, CAPS is more severe, with the involvement of a larger number of vascular beds. Perhaps this is due to the double damaging effects on the endothelium ­ of antiphospholipid antibodies (aPL) and activated complement. So we hypothesize that CAPS can be called aPL-mediated TMA in pts with a complement system defect.

[Incomplete thrombotic microangiopathy as a variant of hemolytic-uremic syndrome].

The described case illustrates difficulties in diagnosing atypical hemolytic-uremic syndrome (aHUS) in incomplete thrombotic microangiopathy (TMA) in the absence of thrombocytopenia, one of the signs of the classic triad of aHUS, which has resulted in the delayed verification of its diagnosis and in progressive kidney injury. The paper discusses the need to carry out kidney biopsy and to include sHUS in both the presence of a complete set of symptoms of this disease and in the absence of one of them into a range of diagnostic search.

[Prognostic value of renal resistance index in estimating the progression of chronic kidney disease].

AIM: To study a relationship between main renal and intraparenchymal renal arterial resistance indices (RIs) measured by Doppler ultrasonography and clinical and laboratory parameters and to determine their prognostic value in estimating the progression of chronic kidney disease (CKD). SUBJECTS AND METHODS: The investigation enrolled 53 CKD patients divided into groups: glomerular and interstitial diseases. Glomerular filtration rate (GFR) calculated using the CKD-EPI formula, proteinuria (PU) severity, kidney sizes, renal parenchyma thickness, parenchyma/collecting index, and main and intrarenal vessel RIs were determined at the first hospitalization. The mean follow-up was 14 ± 2.64 months. The rate of GFR decline was estimated at the rehospitalization. RESULTS: Main renal and intrarenal vessel RIs depend on patient age and pulse pressure. The RIs are associated with GFR and PU in the group of glomerular diseases and with kidney sizes and structure in that of interstitial diseases. The interlobar arterial RI is the most sensitive predictor for worsening renal function with a threshold of 0.65, which is comparable to the prognostic value of PU. CONCLUSION: The main renal and intrarenal vessel RIs may be considered as a predictor for worsening renal function.

[Alterations of the complement system in patients with catastrophic antiphospholipid syndrome].

AIM: To investigate alterations of the complement system in patients with catastrophic antiphospholipid syndrome (CAPS). SUBJECTS AND METHODS: Four patients (2 men aged 23 and 40 years and 2 women aged 39 and 58 years) diagnosed as having CAPS, including 3 patients with systemic lupus erythematosus and secondary antiphospholipid syndrome (APS) and 1 patient with primary APS, were examined. The activity of the complement components C1-C5 and total hemolytic activity were determined in all the patients at the moment of an acute episode and in 1 patient after treatment. RESULTS: The activity of the studied complement components and total hemolytic complement activity proved to be significantly decreased in all the patients. That of complement components recovered after treatment using fresh frozen plasma. The possibility and mechanisms of complement system activation in the patients with CAPS are discussed. CONCLUSION: The preliminary results obtained by the examination of few cases may lead to the conclusion that the complement system may be involved in the development of CAPS.

[Nephrotic crisis--an urgent condition in patients with nephrotic syndrome].

The lecture considers mechanisms of potassium and water retention underlying nephrotic syndrome, clinical differences between hypo- and hypervolemic variants of nephrotic syndrome, risk factors of nephrotic crisis and its clinical symptoms, current approaches to its treatment.

[Characteristics of the kidney lesion in a patient with Sneddon's syndrome].

This case report draws attention to renal damage in a young patient with Sneddon's syndrome, analyses a course of nephropathy and methods of its diagnosis, shows efficacy of anticoagulant therapy, demonstrates possible development of generalized affection of the microcirculatory bed with involvement not only of the skin and brain vessels suggesting that Sneddon's syndrome is a systemic ischemic pathology the manifestations of which in many cases mask polyorganic impairment.

[Ischemic renal disease: clinical course, prognosis, possibilities of treatment].

The case presented for clinical discussion is a patient suffering from ischemic renal disease underlied by renal arterial artherosclerotic stenosis. The article demonstrates a leading role of diagnostic imaging and radiosurgical therapy (balloon dilatation and stenting), and a low effectiveness of conservative treatment.