Based on significant progress in understanding the disease pathogenesis in the past decade hemolytic-uremic syndrome is appear to be heterogeneous group of diseases from the class of thrombotic microangiopathy with different etiology and pathogenesis. This review is dedicated to the most common form of hemolytic-uremic syndrome associated with Shiga toxin-producing E.coli (STEC-HUS). We summarized the latest data about etiology, pathogenesis and therapy of typical hemolytic-uremic syndrome with a focus on the mechanisms of the pathological process and new therapy approaches.

More than three decades have passed since the global study ISKDC (1981) of nephrotic syndrome in children allowed to make certain recommendations such as not to biopsy children at the onset of the disease and in the case of a sensitive to steroid therapy in a patient to recognize the presence of minimal change disease. However, in recent years there has been an increase FSGS in children with primary NS, in the onset of which steroid sensitivity is possible. Therefore, perception of primary nephrotic syndrome in children as a benign, with a favorable outcome should be revised. Moreover, minimal change disease, the pathogenesis of which is traditionally considered the only cell-mediated mechanism can be developed for other violations such as the dysregulation of CD80 expression in podocytes or podocyte destruction due to its structural proteins genes mutation.

The literature review summarizes information about connective tissue dysplasia in children. The influence and the relationship of pathogenic factors acting on the bladder dysfunction in connective tissue dysplasia is discussed.

Renal disorder is associated with high frequency of cardiovascular complications. The review summarizes data of a new entity of cardiorenal syndrome - pulmonary hypertension development in patients with chronic kidney disease (CKD). Its prevalence, potential pathogenic mechanisms, prognostic value and possible treatment in patients with CKD are discussed.

RELEVANCE. Determination of nephropathy progression rate and reveal of early nephrosclerosis development clinical markers are extremely important for evaluation of chronic kidney disease prognosis and use of preventive therapeutic possibilities. THE AIM: to define regularities of progression to the CKD 3 stage in children with immune and non-immune nephropathies. PATIENTS AND METHODS. Due to retrospective analysis of 145 cases of children with immune CKD (steroid resistant nephrotic syndrome, n=75) and non-immune CKD (congenital nephritis, reflux-nephropathy, cystic disease, congenital kidney abnormalities, n=70) GFR dynamics was analyzed by Kaplan-Meier method from the onset of disease till age of CKD 3 stage. RESULTS. Established period for achievement CKD 3 stage depends on degree of proteinuria and arterial hypertension. Efficacy of selective inmmusupresive therapy in SRNS provides no 5-years GFR decline in 100% cases. In patients with non-immune nephropathies the highest rate of progression (to GFR lower 60 ml/min) was found in children with 2 stage arterial hypertension combined with low (less than 1 g/days), and moderated (1-2 g/days) proteinuria. CONCLUSION. Therefore, possibility of adverse outcomes progression rate evaluation in children with CKD promotes the optimization of therapeutic tactics and significantly influences on delay of kidney functions declining.

INTRODUCTION: Fibroblast growth factor-23 (FGF-23) is a phosphaturic hormone, synthesized by osteocytes and involved in the pathogenesis of several hypophosphatemic diseases. THE AIM OF STUDYwas to evaluate influence of intact (iFGF-23) on mineral metabolism in children with different nephropathies. PATIENTS AND METHODS: 87 children, age from 7 months to 17 years 10 months were included in the study. Level of intact FGF-23 was measured (with ELISA) in children with hypophosphatemic rickets (n=15), Fanconi syndrome (n=9), chronic kidney disease stage 3-5 (n=35), steroid osteoporosis (n=10) and in children without nephropathies (n=18). Routine biochemistry markers of phosphate metabolism were measured in children with kidney diseases. RESULTS: FGF-23 level in comparison group was 22,92 pg/ml (5,91-54,37), 5 children were excluded because of undetectable level (<5 pg/ml). All children with kidney diseases had significantly elevated FGF-23 level (in comparison with healthy children): in children with hypophosphatemic rickets FGF-23 level was 73,11 pg/ml (40,08-125,71), p<0,01, in Fanconi syndrome patients - 141,56 pg/ml (34,27-302,64), p<0,01, CKD 3-5 stage patients - 613,71 pg/ml (20,15-6230,77), p<0,01, osteoporosis patients - 106,65 pg/ml (61,64-147,74), p<0,01. Correlation between FGF-23 level and eGFR (using Schwartz formula) in CKD 3-5 stage was found, r= -0,678, p<0,01. Children with Fanconi syndrome on phosphate and vitamin D supplementation had elevated FGF-23 level and we found the strong correlation between FGF-23 level and serum phosphate level (r=0,843, p<0,05) despite of hypophosphatemia. CONCLUSIONS: Our results suggest that FGF-23 is involved in pathogenesis of mineral disorders in different nephropathies. Besides, FGF-23 is rising with the progression of CKD, reaching maximum level in 5-th stage of CKD in pediatric patients as in adult ones.

Atypical hemolytic uremic syndrome (AHUs) in 12-31% of cases is associated with pregnancy or delivery, and may determine the prognosis of both mother and child. AIM: Analysis of clinical manifestations, features of course and outcomes of obstetric aHUS. PATIENTS AND METHODS: From 2012 to 2015 were observed 17 patients aged 19 to 38 years, in whom aHUS developed during pregnancy or directly after delivery. RESULTS: The development of aHUS was preceded by a mainly preeclampsia and/or HELLP syndrome and other pregnancy complications, diarrhea. All patients had complete symptom complex of thrombotic microangiopathy (TMA): sharp decrease of hemoglobin level (67,7 ± 15,9 g/l) with signs of microangiopathic hemolysis (increased LDH levels 2737,3 ± 3276,7 U/l, schistocytosis), thrombocytopenia (55,9±30.9 thousand in mcl.), acute kidney injury (hypercreatininemia 447.6 ±of 226.7 mg/DL, oliguria or anuria). Most patients (82%, 14 of 17) had first time mentioned arterial hypertension. In all patients the TMA was of a systemic nature: the signs of damage of liver, central nervous system, lungs had 73% of women (13 of 17), heart - 41% (7 of 17). In the analysis of flow aHUS depending on the stages of pregnancy at the time of the disease development clinical and laboratory parameters were more severe in women with manifestations of AHUs in the third trimester and after delivery. Treatment included plasma exchange and/or plasma infusions (14 of 17, 82%) with following Eculizumab treatment in 7 patients. Maternal mortality was 29%. CONCLUSION. Obstetric aHUS is characterized by the development of multiple organ failure in most patients. Development aHUS preceded by various complement-activating state. It seems that complications of pregnancy, not the pregnancy per se, are triggers of aHUS in women with a genetic predisposition to its development.

AIM: to evaluate the influence of essential amino acids ketoanalogs (EAAK) and low protein diet (LPD) to production of phosphoric calcium metabolism regulators - morphogenetic proteins FGF-23 and Klotho in patients with chronic kidney disease (CKD) stages 3B-4. PATIENTS AND METHODS: The study included 50 nondiabetic CKD stage 3B-4 patients which were divided into 2 groups depending on the diet type. Group 1 (n=25) got LPD-0.6 grams/kg /day and took EAAK - Ketosteril 1 tab./5 kg/day within 12 observation months; Group 2 (n=25) was comparable to the 1st group by age, sex and GFR reduction degree, also held LPD, but did not take EAAK. FGF-23, alpha - Klotho, phosphorus, total calcium and parathyroid hormone (PTH) serum levels were examined, bioimpedance analysis, echocardiography, abdominal aorta radiography in lateral projection and pulse wave velocity were performed in all patients at the screening time and after 12 months follow. RESULTS: None patient of the Group 1 were recorded nutritional status disorders, while in Group 2 five patients marked nutritive disorders: decrease in muscle mass and body mass index. In addition, CKD patients in Group 1 had lower serum PTH, phosphorus and FGF-23 levels (p<0,05) and higher alpha-Klotho serum levels, than patients in Group 2. Heart and aorta calcification, as well as blood vessels damping function violation were detected significantly more [8% versus 16%, p <0,05 and 8% versus 20%, p <0,05, respectively] in 2nd Group patients than in the 1st. Wherein, there was a significant concentric left ventricular hypertrophy degree increase (16% vs. 32%) in Group 2 patients, that was inversely correlated with GFR (r=-0,540; p<0,01). CONCLUSIONS: EAAK application in CKD stages 3B-4 patients receiving LPD provides not only prevention of nutritional status violations, but also contributes to a more effective correction of hyperphosphatemia, hypocalcemia, decrease hyperproduction of FGF-23 and increase production of alpha-Klotho. Increase of alpha-Klotho production in serum led to reduction in both heart and blood vessels calcification and concentric remodeling of left ventricle myocardium.

World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD) in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.