LEADING ARTICLE
Data of diseases accompanied by change of blood serum osmolality are presented, states with a combination of hyponatremia with hyperosmia are characterized. There are presented estimation varieties of renal response to a change of blood volume and extracellular fluid at a change of its osmolality and Na+ concentration. Clinical significance of clearance calculation of solute free water and of the sodium free water is shown. Factors of different reactions of body regulatory systems on hyperosmia caused by hyperglycemia, uremia, or hypernatremia are discussed.
REVIEWS AND LECTURES
Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5–10% of HUS in children, but the majority of HUS in adults. The incidence of complement-aHUS is not known precisely. However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from the neonatal period to the adult age. Most patients present with hemolytic anemia, thrombocytopenia and renal failure and 20% have extra renal manifestations. Two to 10% die and one third progress to end-stage renal failure at first episode. Half of patients have relapses. Mutations in the genes encoding complement regulatory proteins factor H, membrane cofactor protein (MCP), factor I or thrombomodulin have been demonstrated in 20–30%, 5–15%, 4–10% and 3–5% of patients respectively, and mutations in the genes of C3 convertase proteins, C3 and factor B, in 2–10% and 1–4%. In addition, 6–10% of patients have anti-factor H antibodies. Diagnosis of aHUS relies on 1) No associated disease 2) No criteria for Shigatoxin-HUS (stool culture and PCR for Shiga-toxins; serology for anti-lipopolysaccharides antibodies) 3) No criteria for thrombotic thrombocytopenic purpura (serum ADAMTS 13 activity > 10%). Investigation of the complement system is required (C3, C4, factor H and factor I plasma concentration, MCP expression on leukocytes and anti-factor H antibodies; genetic screening to identify risk factors). The disease is familial in approximately 20% of pedigrees, with an autosomal recessive or dominant mode of transmission. As penetrance of the disease is 50%, genetic counseling is difficult. Plasmatherapy has been first line treatment until presently, without unquestionable demonstration of efficiency. There is a high risk of post-transplant recurrence, except in MCP-HUS. Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care. Except for patients treated by intensive plasmatherapy or eculizumab, the worst prognosis is in factor H-HUS, as mortality can reach 20% and 50% of survivors do not recover renal function. Half of factor I-HUS progress to end-stage renal failure. Conversely, most patients with MCP-HUS have preserved renal function. Anti-factor H antibodies-HUS has favourable outcome if treated early.
During last years in medical literature appeared new term “systemic autoinflammatory diseases”. In contrast to autoimmune diseases in autoinflammatory diseases initiation the main role belongs not to mechanisms associated with antigen-induced synthesis of antibodies or T-cells activation but allelically determined reactions of immunity and inflammatory. The group of autoinflammatory diseases includes familiar periodic fevers (Mediterranian fever, Muckle–Wells syndrome etc.) which most frequent complication is recurrent AA-amyloidosis. DNA testing showed that the most important mechanism of autoinflammation is neutrophilic inflammation associated with interleukin-1 hyper production. The main releaser of neutrophilic inflmmation is development of macromolecular complex – NLRP3 inflammasome which is very important in inflammation initiation not only at familiar periodic fevers but also at a number of widespread inflammatory diseases (gout, COPD etc.). In this regard NLRP3 inflammasome can be considered as one of universal mechanisms of neutrophilic inflammation. Chronic inflammatory diseases make persistant background and promote activation of immune reactions with possible development of classical autoimmune diseases that presented clinical studies demonstrate.
ORIGINAL ARTICLES. CLINICAL INVESTIGATIONS
AIM OF RESEARCH. Estimation of cardioprotective effect of dialysis fluid with succinate at patients with end-stage kidney failure receiving chronic hemodialysis treatment. PATIENTS AND METHODS. 90 patients of two dialysis departments randomized to 2 groups took part in crossover study. Active group of research received chronic hemodialysis with dialysis fluid with succinate during 6 months and control group received chronic hemodialysis with standard bicarbonate dialysis fluid with subsequent “decussation” after 3-month’s washout period. Dynamics of blood biochemical measurements, data of ECG 24-hour monitoring during which was carried out the 6-minute walk distance (6MWD), echocardiography data (ECHO) were estimated. RESULTS. After 6 months of treatment with acidosuccinate in comparison with control decrease in level of inorganic phosphates, calcium, sodium, creatinine, urea, uric acid and increase of total protein and albumin in blood was noted. Decrease of aldosterone content in blood was noted at female patients. According to 24-hour cardiomonitoring with control of arterial blood pressure (ABP) was noted decrease of systolic ABP, decrease of heart rate in daytime, decrease of heart rhythm disorder and time duration of ST-segment depression episodes. 6MWD demonstrated increase of exercise tolerance. The tendency to decrease of miocardial hypertrophy was noted at ECHO. CONCLUSION. This research showed positive effect of hemodialysis with dialysis fluid wih succinate on cardio-vascular system of petients received chronic hemodialysis treatment, it also allowed to reveal a number of cardioprotective mechanisms of succinate which were not described before (hypophosphatemic, hyponatremic and hypotensive effect, decrease of protein catabolism, decrease of GGT and aldosterone level in patients’ blood). Administration of acidosuccinate is especially recommended to patients with high risk of cardio-vascular complications.
AIM OF RESEARCH: to define the esimate of seasonal factor on vitamin D (VD) status in patients with early stages of chronic kidney disease (CKD). PATIENTS AND METHODS. There were examined 94 patients with CKD of 1-4 stage, male/female – 45/49, 60,1±12,6 years mainly with diabetic nephropathy (91,2%). Besides usual factors by immunoenzyme method were estimated 25(OH)D and 1,25(OH)2D. RESULTS. Defficiency of 25(OH)D (level is lower 30 nM/l) was diagnosed at 14,9% of patients, insufficiency (30-75 nM/l) – at 55,4% and optimal value (higher than 75 nM/l) – at 29,7%. In 63,8% of cases insufficiency of 1,25(OH)2D was defined (concentration in serum lower than 53nM/l). In autumn level of 25(OH)D (n=30) was higher than in spring (n=64, p=0,002). According to data of multiple regression analysis was defined tendention of value seasonal variations of 1,25(OH)2D (p=0,09) due to escurtion of 25(OH)D. CONCLUSION. Insufficiency and defficiency of 25(OH)D are regular at patients with chronic kidney disease of 1-4 stage. Level of 25(OH)D have serious seasonal variations which provide some seasonal variations of 1,25(OH)2D concentration.
AIM OF RESEARCH. To estimate in prospective study predicate capacity of serum cystatin C (sCysC) in diagnostics of acute kidney injury (AKI). PATIENTS AND METHODS. Serum creatinine (sCr) and sСysС were measured together in 68 patients with undergoing coronary angiography and/or angioplasty and who were at high risk to develop AKI. SCr and sСysС were assessed at baseline and 18–20 hours after contrast media exposure; sCr was detected also after 48 hours. SCysC was quantified by immunoturbidimetry. AKI was defined according to AKIN. The binary logistic regression analysis was undertaken and the ability of sCysC to predict AKI was assessed from the area under the receiver operator characteristic curve (AUROC). RESULTS. Contrast-induced AKI defined according to sCr increase ≥0,026mmol/l or ≥1,5 increase in sCr from baseline occurred in 11 patients. At 18–20 hours after contrast media exposure sCr increase wasn’t detected in anybody but sСysС increase was revealed in 30 patients. Cut off was identified with sCysC concentration ≥ 50% from basic with optimal correlation of sensitivity and specificity (81,8% and 98,2%) for early defining CI-AKI patients. The AUROC for this sCysC level was 0.90 (95% CI, 0.95- 0.97). CONCLUSION. The results of this study support that sCysC is a good biomarker of glomerular filtration decrease at AKI and may detect CI-AKI before a rise in sCr.
JOURNAL IN THE JOURNAL. ACTUAL PROBLEMS OF UROLOGY
AIM OF RESEARCH. To prove priority of native surgical academy in experimental validation and clinical management of mucosa membrane free graft transplantation for urethra plastics in men. MATERIALS AND METHODS. Medical literature of the end of 19-th and the beginning of 20-th century, original articles of K.M.Sapezhko and I.A.Tyrmos about experimental and clinical aspects of transplantation of mouth and gut mucosa membrane and also modern biographic articles devoted to stages of their life were analyzed. RESULTS. First-ever successful replaceable urethroplasty of mouth mucosa membrane was made by K.M. Sapezhko in 1890. His student I.A.Tyrmos for the first time ever executed urethral reconstruction with using of mucose membrane of gut to the patient with vesicointestinal fistula. Before application of mucose membrane autografts K.M.Sapezhko executed his experimental work which provided him to describe stages of transplanted grafts acceptance and also to develop urethral stricture classification and to offer recommendations about patients with urethral cicatrical deformity. CONCLUSION. Priority in theory and practical usage of mucosa membrane free grafts autotransplantation for urethral reconstruction in men belongs to russian surgical academy.
Here presented data about change of status in bilipidic membrane layer at long term dialysis therapy with demonstration of internal relations between most informative indicators received at patients assessment as a result of long-term studying of erythrocytes membrane quality at patients with hemodialysis. It is established that condition of plasmatic erythrocytic membranes of patients with chronic hemodialysis alter considerably according to treatment term. Most significant aberrations are noted at early terms of therapy – at duration of hemodialysis from 1 to 3,8 years, which occurs in most evident destabilization of membranous structures. At longer terms of hemodialysis therapy resistance of erythrocytes to external actions of canaloformer practically doesn’t differ from values typical for people without renal pathology. Factorial analysis of ensemble which associates all clinical, biochemical and biophysical factors of patients with chronic hemodialysis was carried out. It is shown that the general component which mainly contributes into structure of general assembly of factors is only values of integral normalized potassium output at only nystatin effect at erythrocytes of hemodialysis patients. According to received data the conclusion is that membranoprotective therapy must become integral and obligatory part of preventive therapy at all stages of CKD treatment.
PROGRAM ON CONTINUOUS POSTGRADUATE EDUCATION ON NEPHROLOGY
The review provides data, indicating about the role of intrauterine fetal growth conditions in chronic pathology at adults and aged individuals. The significance of body mass at birth, associated with maternal nutrition, placenta, trophic status of fetus in progression of obesity, arterial hypertension and insulin resistance in postnatal period is discussed. Low body mass of fetus is associated with small number of nephrons, and with high risk of kidney diseases in adults.
ISSN 2541-9439 (Online)