The continued growth of the population of HIV-infected individuals is caused by two processes: the high frequency of new cases of HIV infection and the effectiveness of combined antiretroviral therapy which suppresses viral replication, prevents HIV-associated diseases and leads to a better quality of life and increase in the short-term and medium-term survival of HIV-infected patients. To date, over 33 million people worldwide are infected with HIV and more than 2 million AIDS-related deaths are recorded every year. In the Russian Federation only the number of registered HIV-infected people is more than 1 million, and it increases annually by 10%. According to UNAIDS and WHO, HIV-related disease will continue to be one of the leading causes of death in the coming decades, and will play a significant role in global mortality rates. The reason for this is defined by long-term course of HIV infection with a higher risk of developing secondary and opportunistic diseases, clinically associated with a progressive decrease in immunity, as well as the defeat of various organs and systems, including kidneys. Patients with HIV are at risk for both acute kidney injury and chronic kidney disease (CKD), secondary to medication nephrotoxicity, HIV-associated nephropathy, immune complex kidney diseases, and, less commonly, kidney disease in the setting of thrombotic microangiopathy. In addition, HIV-positive patients are at increased risk of CKD associated with coinfection with viral hepatitis B and C, hypertension, diabetes. Despite the lack of randomized controlled trials presented recommendations are based on the general health guidelines and are intended to unify approaches to the detection, diagnosis, management of HIV-positive patients with CKD and create prerequisites for addressing these important health challenges.

Diabetic nephropathy (DN) is currently recognized as one of the main causes of CKD. In vast majority of patients the diagnosis of DN is based on clinical data and labs, however, in some cases pathology diagnostics is strictly needed. We studied retrospectively 103 kidney biopsies, performed in patients with type 2 DM. Indications for kidney biopsy were defined as any clinical data, suggestive for non-diabetic kidney disease in patients with DM type 2. Based on pathology findings we were able to distinguish 3 groups of patients: Group 1 (n=52) with kidney lesions, completely consistent with DN; Group 2 (n=23), showing combination of DN and kidney lesions of non-diabetic origin; and Group 3 (n=28), demonstrating the absence of DN and showing features different non-diabetic kidney diseases. The most often findings of non-diabetic lesions turned to be FSGS, IgAN, MN, crescentic GN and MCD. Neither demographic nor main clinical and lab data had no difference between the groups. We also did not found any association between the duration of DM and the type of kidney damage. In our study the most often indications for biopsy were sudden onset of NS or proteinuria in patients with relatively short duration of DM; renal failure or rapid decline of kidney function, microhematuria or the need of differential diagnostics with systemic diseases. Despite of atypical clinical presentation in those cases, about a half of these patients showed only DN by pathology. More characteristic for DN may be considered the combination of NS and impaired kidney function. Mild microhaematuria do not exclude the possible diagnosis of DN. NS with intact kidney function, as well as marked haematuria or rapidly declining kidney function in the absence of NS strictly demand pathology evaluation to rule out non-diabetic origin.

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children under 5. Aim: to assess the incidence of HUS in children in Belarus, to study its age and sex differences, seasonal variations, incidence of different variants of HUS, as well as hematological and biochemical blood changes and their dynamics in the process of recovery of renal function, the diagnostic significance of markers of acute kidney injury, complement activation, thrombosis and inflammation. Materials and methods: 218 children with HUS were included to a prospective, long-term, uninterrupted study for the 10 year period 2005-2014 in Belarus. Results: HUS more often develops in children under the age of 5 years (89.9%), occurs with equal frequency among both sexes, more often in the urban population. The incidence was 3.9 cases per 100 000 children population of age <5 years and 1.5 at the age of <15 years. The most common type of HUS is diarrhea-associated (96.3%), a link with Shiga-toxin was confirmed in 49.5% of cases. The clinical and laboratory changes of the disease and treatment in those who received renal replacement therapy and without it were described. Peritoneal dialysis has been the method of choice in 77.9% of patients requiring dialysis therapy. Conclusions: the use of a number of organizational, diagnostic and treatment recommendations allowed reducing mortality in children with HUS to 2.3%. The results of treatment of children with atypical HUS remain unsatisfactory.

Objective: to identify the important determinants of the 28-day mortality and to assess their explanatory power in adult patients in undergoing continuous renal replacement therapy (CRRT) for cardiac surgery-associated acute kidney injury AKI (CS-AKI). Material and methods: the retrospective cohort monocenter study covered 162 cardiac surgical patients over 18 years old operated under cardio-pulmonary bypass (CPB) and complicated by the development of AKI treated with CRRT. Study endpoints were 28-day and hospital mortality. Results: it was found that the most important determinants of 28-day mortality in patients undergoing cardiac surgery with the development AKI were the use of extracorporeal membrane oxygenation (ECMO), the duration of days with oliguria and the used intra-aortic balloon pumping (Odd Ratio 7.24; 6.55; 3.75; respectively). It was found that the ratio of the surface area of the haemofilter membrane to the body surface area as a significant determinant of mortality in cardiac patients with AKI (Odd Ratio 1.41). We found that the CRRT dose effluent is not associated with the 28-day mortality. Whereas, the maximum level of lactate during the CRRT had a good explanatory power (AUC 0.79) with sensitivity and specificity of 0.9/0.71. Daily fluid balance and the total balance of body fluids (delta) during CRRT verified as suitable determinants in predicting the 28-day mortality (AUC 0.784, AUC 0.786, respectively). Conclusion: in patients with severe CS-AKI requiring CRRT, 28-day mortality appears strongly associated with extrarenal (cardiovascular) factors, which determine the short-term outcomes. The ratio of surface area of haemofilter membrane/body surface area provided to be a factor modifying short-term mortality in patients with AKI.

Congenital thrombotic thrombocytopenic purpura (TTP), also known as Upshaw-Schulman Syndrome is a rare inherited deficiency of ADAMTS13. Unlike more common acquired TTP which is characterized by an acquired inhibitor of ADAMTS13, patients with congenital TTP have an absolute deficiency of ADAMTS13 without an inhibitor. Congenital TTP generally presents in infancy with repeated episodes of acute hemolysis and evidence of microangiopathy, these episodes are usually triggered by illness or physiological stress. Congenital TTP can be effectively treated with plasma infusion either during acute episodes or on a prophylactic schedule to prevent episodes. We present a case of a 7 year old boy with congenital TTP. He suffered from thrombocytopenia and anemia since infancy, but the correct diagnosis was not made until the age of 7. At presentation to our center the patient was documented to have thrombocytopenia, elevated creatinine, and schistocytes. He was initially treated with plasma infusion at a rate of 10 ml/kg with resolution of his thrombocytopenia and hemolysis. At the time of writing this article he is maintained on a prophylactic schedule of biweekly plasma infusions at 10 mg/kg and is maintaining a normal platelet count with no evidence of hemolysis.

The article represents brief literature review of the problem of high-output heart failure (HOHF) in patients on maintenance hemodialysis (HD) with arteriovenous fistula (AVF) as dialysis access. The possible mechanisms of AVF influence on central hemodynamic parameters and the role of these mechanisms in formation of HOHF are of broad interest. HOHF is usually an under-diagnosed condition in the dialysis population and its real incidence is underestimated. We present here a case of HOHF in a patient on maintenance HD. The signs of HOHF developed after formation of AVF with excessive flow formation. The patient suddenly died from ventricular fibrillation. A procedure of HOHF diagnostics, which include catheterization of right heart accompanied by temporary AVF occlusion is proposed. Autopsy report confirmed the suggestion that HOHF is not an independent heart disease in patients on MHD, but potentially reversible pathophysiological phenomenon.

Atypical haemolytic uremic syndrome (aHUS) associated with complement-mediated thrombotic microangiopathy (TMA) is typically associated with non-immune mechanical haemolytic anemia, thrombocytopenia and renal failure. We report a case of an adolescent incomplete form of athrombocytopenic TMA as a possible phenotype of aHUS. Partial hematologic presentation with the progressive renal failure has led to wrong diagnosis of the rapidly progressing glomerulonephritis and to unnecessary immunosupressive therapy. Subclinical period with persistence of anemia, minimal proteinuria and non-specific symptoms of illness (weaknesses, fatigue) were the features of the disease in the patient. Subclinical course of the disease with the absence of thrombocytopenia was unfavorable prognostic factor that resulted in the delayed diagnosis and poorer renal outcome in the patient. Our clinical case shows that diagnosis of TMA can be suspected if there is a combination of the hemolytic anemia and kidney injury. Careful patient examination, identification of the type of anemia with platelets count are necessary for earlier diagnosis of TMA. Kidney biopsy is required to confirm the diagnosis of aHUS in patients with incomplete clinical pattern.

Introduction: atypical hemolytic uremic syndrome (aHUS) is a rare, life-threating disease, associated with uncontrolled activation of complement system. Frequency of aHUS in population is 1-7 cases per million. Mutations of membrane cofactor protein (MCP) were found in patients with aHUS in 15% of cases. Materials and methods: we present a case of atypical hemolytic uremic syndrome, associated with homozygous mutation of MCP gene. A girl had 6 episodes of aHUS from 2002 till 2014, and had a complete recovery of kidney function. When she was 15 year old, 7th episode has happened and she had prolonged elevation of serum creatinine level (200 μΜ/l) and LDH (360 U/l; upper limit 225 U/l) after it. In our hospital, we performed kidney biopsy, confirmed thrombotic microangiopathy, and used next-generation sequencing of CFH, CFI, CFB, MCP and THBD genes. All mutations and polymorphisms were confirmed with direct sequencing. During observation, we found thrombocytopenia 90×109/l and elevation of creatinine level up to 378 μΜ. Specific treatment with eculizumab was started. Results: homozygous mutation of MCP gene c.307C>T and homozygous polymorphism of CFH gene c.2808G>T were found in a patient with aHUS. A recovery of kidney function was achieved on eculizumab therapy.