The article reviews the current Russian normative statements and international examples of quality criteria in dialysis care - from regulatory authorities, professional organizations and dialysis networks. The main criteria is the achievement of the target ranges of important clinical and laboratory parameters in a certain fraction of the patients by a dialysis unit, or the definite fraction of the patients covered by some diagnostic or treatment measures. Using acquired international and domestic experience the Russian Dialysis society has prepared and accepted (during the Renal Week-2014) the Russian Clinical recommendations concerning quality care criteria in dialysis. The recommendations are published in the current issue of ‘Nephrologia i Dialys’. The proposed criteria had been applied to the subgroup of six dialysis units in Saint-Petersburg (representing municipal hospitals, federal institutions and private units) and included 529 patients with average 6 month long evaluation period. The evaluation demonstrated achievability of the proposed criteria in real practice of different dialysis units. Russian dialysis society encourages its wide practical application in different regions to affirm the adequacy of recommendations (or to work out the proposal for its correction).

Recent epidemiological and clinical studies suggest that drug-induced acute and chronic kidney diseases are closely connected. Multiple risk factors for acute kidney injury are AKI (prerenal kidney injury, postrenal urinary tract obstruction, and intrinsic renal diseases) including advanced age, diabetes mellitus, hypertension and afro-american race. However, the most important risk factor for acute kidney injury is preexisting chronic kidney disease, which increases the risk of AKI in 10 folds compared to case of the absence of chronic kidney disease. AKI increases the risk of progression CKD to terminal uremia аnd may lead to chronic kidney disease regardless the cause of the acute injury. The prognostic importance of a small acute decrement in kidney function is discovered. Association between the acute tubular necrosis and long-term renal and cardiovascular outcomes is discussed. Both AKI and CKD are the risk factors for cardiovascular disease.

Aim. To evaluate the rates of changes in kidney function in CKD register. Method. The analysis of St.-Petersburg City nephrology center data on 2579 patients with eGFR (CKD-EPI) below 60 ml/min/1.73 m2 with five and more follow-up visits evaluates the rates and direction of the GFR changes over the time. Results. 44% of pts showed the rate in range 0-5 ml/min/1.73 m2 per year; 30% had the rate more than 5 ml/min/1.73 m2 per year; 26% of pts revealed slow but stable improvement of kidney function. Overall average values of the GFR change rates were -3.71±0.20 and -2.89±0.24 ml/min/1.73 m2 per year for men and women, respectively; being significantly different for CKD stages: -1.27 (-1.96 ÷ - 0.57); -2.64 (-3.14 ÷ -2.15); -4.83 (-5.31 ÷ -4.36) and -6.28 (-7.24 ÷ -5.33) for CKD 3A, 3B, 4 and 5 stages, respectively. CKD progression rates were different for pts with various diagnoses and were associated with calcium, phosphate (not PTH), anemia or iron deficiency, serum albumin and proteinuria levels. In women, low and high total cholesterol levels were linked with higher progression rates. In the multiple regression model, male gender was linked with a negative slope of GRF by 0.94 ml/min/1.73 m2 per year, as well as reduced by 10 ml/min/1.73 m2 GFR at baseline; the reduced serum albumin by 2 g/l, Hb by 5 g/l, elevated phosphate by 0.1 mmol/l, uric acid by 0.2 mmol/l and proteinuria by 0.3 g/d were associated with increased GFR loss rates (by about 10%). Conclusion. The identifying of the modifiable factors linked to CKD progression gives the opportunity to improve comprehensive nephroprotective therapy.

Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease related to uncontrolled complement activation. Until recently, the management of aHUS consisted of early and intensive plasma exchange, although this was mostly ineffective in protecting from subsequent organ damage. Eculizumab, an inhibitor of the alternative complement pathway, produces a rapid and sustained inhibition of the thrombotic microangiopathy process, with significant improvements in long-term clinical outcomes. Due to the significant improvement achieved, eculizumab has subsequently been approved as the first-line therapy when an unequivocal diagnosis of aHUS has been made. The use of eculizumab has changed the management and the outcome of aHUS, becoming the frontline treatment of the disease. We report a case of a 13 year-old boy with aHUS who got eculizumab because of persistent non-specific clinical symptoms in the absence of laboratory evidence of active thrombotic microangiopathy. The use of eculizumab was associated with a significant decrease in proteinuria, anemia, increased GFR and an improvement in general state, revealing that the subclinical symptoms were caused by unsuspected disease activity.

Aim: The study was performed to show the efficiency of local vitamin D3 injection in parathyroid glands in management of secondary hyperparathyroidism and to reveal optimal conditions for this treatment. Results: Forty-six series of local injection of paricalcitol in the parathyroid gland under ultrasound guidance where performed in 37 patients with CKD 5D, receiving renal replacement therapy (16 - HD, 21 - CAPD) with manifestations of secondary hyperparathyroidism resistant to conservative therapy. As a result of the interventions a statistically significant reduction in PTH levels by 22.7% (190 pg/ml, 95% CI - 87 ÷ 293, p=0.001) was achieved. A significant difference in the trends of PTH before and after the intervention during 6 and 12 months was observed. A significant reduction of the maximal linear dimension (in average by 17.1%) and a downward trend in the estimated volume (in average by 12.5%) were revealed in 6 months after the injections. Regression analysis showed a statistically significant dependence of the gland volume changes upon the calculated gland volume before the intervention in the quadratic regression model. The most significant decrease in the volume was noted at the baseline calculated gland volume from 0.2 to 0.5 cm3. No gland volume reduction was found if the calculated volume was less than 0.1 cm3. The magnitude of the PTH level changes did not depend on the baseline calculated parathyroid gland volume. Conclusion: Repeated vitamin D injections in parathyroid glands under ultrasound control can be efficient treatment additional to medicamentous therapy when the glands size is in range 0.2-0.5 sm3 and the PTH level is lower 1000 pg/ml.

Primary hyperparathyroidism is known to be the third most common endocrine abnormality, yielding only diabetes mellitus and thyroid gland diseases. Its prevalence in adult population is currently estimated as 0.4-1%, with 1-5.2% out of these cases recognized as parathyroid carcinoma, which makes the latter becoming not that rare disease, as it used to be thought previously. However, diagnostics is difficult not least because insufficient information about this disease, leading to the late diagnosis and lack of the adequate timely treatment. We present a case of parathyroid carcinoma with rare intra-thyroid localization, representing many difficulties in differential diagnostics and demanding multiple surgical treatments. The course of the disease during almost 3-year follow-up is characterized by metastatic lesions of regional and mediastinum lymph nodes, cranial bones and esophagus with severe hyperparathyroidism and hypercalcemia, leading originally to acute kidney injury, and subsequently to nephrocalcinosis and chronic kidney disease. Based on current literature we discuss genetics, clinical course, labs and pathology of parathyroid carcinoma, its diagnostics and differential diagnostics, prognosis and outcomes.

Astract. Clinical observation of a 57-year-old woman who underwent cholecystectomy performed for calculous cholecystitis in the age of 46 years is reported. Four years later staghorn stones were found in both kidneys and chronic kidney disease (CKD) was diagnosed (blood creatinine level 272 mM). Then nephrolithotomy, nephrostomy, left nephrectomy were performed. At the age of 54 years, cancer of the left breast (T3N0M0) was diagnosed and operated. In preoperative checkup, the total and serum ionized calcium were 2.47 mM and 1.46 mM, respectively. Parathyroid hormone (PTH) was 1486 pg/ml, albumin 42 g/L. 99mTc-sestamibi scintigraphy shown a zone of hyperfixation of radiopharmaceutical agent in the lower segment of the right lobe (27×20 mm). Five months later the patient was transferred to hemodialysis, however then she was diagnosed with severe hyperparathyroidism (HPT): PTH 1278 pg/ml, serum calcium 2.7 mM, phosphorus 2.2 mM, alkaline phosphatase 152 u/l (norm 30-115). Neck ultrasonography revealed a hypoechoic nodular formation with clear margins (26×16×10 mm, V=2177 mm3) in the projection of the right lower parathyroid gland (PTG). Computer tomography revealed an elongated formation in the posterior surface of the right lobe of the thyroid gland: 27 mm (antero-posterior) x 18 mm (transverse) x 31 mm (cranio-caudal); staghorn stone in the right kidney; calcification of the coronary artery; calcified focuses in liver and in spleen. Bone mineral densitometry revealed osteoporosis in the 1/3 of radial bone (T-score -2.5). Echocardiography have shown a calcification of the aortic and mitral valves. Surgical parathyroidectomy was performed. A single nodal education was detect and removed. Pathologist conclusion: an encapsulated unit (weight 6 g, 3.5×2.5×1.0 cm); histologically - PTG adenoma made of the main type cells with follicular and trabecular growth type. The «hungry bone syndrome» was observed postoperatively with PTH 81 pg/ml. Six months later secondary HPT has developed.

Relevance. Diffuse parathyromatosis is a rare ethiological factor of renal hyperparathyroidism. Its diagnostics and treatment are extremely difficult. Efficiency and feasibility of its surgical treatment are questioned. Several conservative approaches are considered as an alternative to surgery, although their efficiency and economic viability has not been confirmed so far. A review of the literature and our own experience of surgical treatment of this rare disease are provided as well as discussion of the treatment and diagnostic tactics. Materials and Methods. A female patient (age 54, hemodialysis for 12 years) who underwent subtotal parathyroidectomy was followed up. A recurrence of the disease occurred in 2 years after the surgery. The differential diagnosis between cancer and parathyromatosis was performed using computed tomography, scintigraphy, histological examination. The final diagnosis was made after the operation, which included wide excision of involved parathyromatosis tissues. Results. Operation efficiency was confirmed by a decrease in parathyroid hormone from 2350 pg/ml (before operation) to 31 pg/ml. The patient was successfully discharged without surgical complications; 1.5 months after surgery the intact parathyroid hormone level increased to 125 pg/ml in the absence of treatment of hypocalcemia. After 2.5 months of adequate therapy with vitamin D and Ca calcium and phosphorus levels were normalized. Conclusions. A total parathyroidectomy followed by dissection of neck fatty tissue and upper mediastinum can be considered as a way of prevention of secondary parathyromatosis. This approach may become the basis of an effective surgical treatment provided adequate correction of bone mineral metabolism in the postoperative period.