OBJECTIVES: This cross-sectional analysis from the CZecking Heart Failure in patients with advanced Chronic Kidney Disease trial (ISRCTN18275480) examined pulmonary hypertension and right ventricular-pulmonary arterial coupling in patients on chronic hemodialysis. The aims of this analysis were: 1. To analyze relations between pulmonary hypertension and right ventricular-pulmonary arterial coupling with dialysis access flow and current hydration; 2. To analyze structural heart changes associated with right ventricular-pulmonary arterial uncoupling; 3. To reveal the prevalence, etiology and severity of pulmonary hypertension in the Czech hemodialysis population. METHODS: We performed expert echocardiography, vascular access flow measurements, bioimpedance analysis, and laboratory testing in 336 hemodialysis patients. RESULTS: Pulmonary hypertension was present in 34% (114/336) patients and right ventricular-pulmonary arterial uncoupling was present in 25% of patients with pulmonary hypertension. Only weak associations between the flow of the dialysis arteriovenous access and estimated pulmonary arterial systolic pressure and right ventricular-pulmonary arterial coupling was proved. There was a strong association between hydration status assessed by estimated central venous pressure with pulmonary arterial systolic pressure (Rho 0.6, p < 0.0001) and right ventricular-pulmonary arterial coupling (Rho -0.52, p < 0.0001) and association between overhydration to extracellular water ratio with pulmonary arterial systolic pressure (Rho 0.31, p = 0.0001) and right ventricular-pulmonary arterial coupling (Rho -0.29, p = 0.002). The prevalence of heart failure was significantly higher in patients with right ventricular-pulmonary arterial uncoupling (88% vs. 52%, p = 0.0003). CONCLUSION: These findings suggest that optimizing volume status and treating heart failure should be prioritized in hemodialysis patients to prevent pulmonary hypertension progression and right ventricular-pulmonary arterial uncoupling.

• Klíčová slova
• Right ventricular-pulmonary arterial coupling, arteriovenous access flow, chronic hemodialysis, fluid overload, heart failure, pulmonary hypertension,
• MeSH
• arteria pulmonalis * patofyziologie   diagnostické zobrazování   MeSH
• chronické selhání ledvin * terapie   komplikace   MeSH
• dialýza ledvin * škodlivé účinky   MeSH
• echokardiografie MeSH
• lidé středního věku MeSH
• lidé MeSH
• plicní hypertenze * patofyziologie   epidemiologie   etiologie   MeSH
• prevalence MeSH
• průřezové studie MeSH
• senioři MeSH
• srdeční komory * patofyziologie   diagnostické zobrazování   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika epidemiologie   MeSH

BACKGROUND: The application of international recommendations for paediatric maintenance haemodialysis (HD) could be strengthened by national laws or written recommendations. Our aim was therefore to describe the national rules governing paediatric maintenance HD in European countries. METHODS: A national representative, approved by the president of each paediatric nephrology society, was contacted in all 42 European countries to complete two online questionnaires. RESULTS: Answers were received from 36 countries. The population served by HD centres varies from 83,000 to 1,197,000 residents below 18 years of age and the estimated mean number of children on HD per centre from 0.2 to 13.5. The lowest age at which a child can be dialysed in an adult centre varies from 0 to 18 years. Laws or written national recommendations specifying: this age, the need for a paediatrician as part of medical team in mixed adult-paediatric centres, the minimum number of doctors per centre and the number of patients per nurse or nurse's aide required during sessions exist in only 25, 22, 22, 44 and 8% of the countries, respectively. Similarly, dietitians, social workers, school service, psychologists and play specialists/youth workers are required by law or written national recommendations in 36, 28, 36, 31 and 14% of countries, respectively. CONCLUSION: Laws or written national recommendations for paediatric maintenance HD are rare in European countries and very heterogeneous when they exist. This calls for discussion among paediatric and adult nephrologists and health authorities on the organisation of safe and effective paediatric HD practices.

• Klíčová slova
• Child, Legal status, Maintenance haemodialysis, Recommendations, Survey,
• MeSH
• chronické selhání ledvin * terapie   MeSH
• dialýza ledvin * normy   MeSH
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• nefrologie zákonodárství a právo   MeSH
• novorozenec MeSH
• pediatrie * zákonodárství a právo   normy   MeSH
• předškolní dítě MeSH
• průzkumy a dotazníky MeSH
• směrnice pro lékařskou praxi jako téma MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH

Cancer and its treatment may lead to kidney injury and the need for kidney replacement therapy (KRT). We identified 287 pediatric KRT patients with a history of malignancy from the European Society for Paediatric Nephrology/European Renal Association Registry. Of these, 197 had cancer as a primary cause of KRT (group 1) and 90 had a malignancy diagnosis before KRT (group 2). Two matched controls without malignancy were randomly selected for each patient. Data were complemented with a questionnaire. Median time to kidney transplantation (KT) from KRT initiation was 2.4 (IQR: 1.5-4.7), 1.5 (IQR: 0.4-3.3), 3.6 (IQR: 1.3 to Q3 not reached), and 1.1 (IQR: 0.3-3.6) years for group 1, their controls, group 2, and their controls, respectively. Overall 10-year mortality for those on KRT was higher among cancer patients vs controls in group 1: 16% vs 9% (adjusted hazard ratio 2.02, 95% CI: 1.21-3.37) and in group 2: 23% vs 14% (adjusted hazard ratio 2.32, 95% CI: 1.11-4.85). In contrast, 10-year patient survival after the first KT was comparable to controls (93% vs 96%; 100% vs 94%, in groups 1 and 2, respectively). In summary, childhood cancer survivors' KT was delayed, and their overall mortality when on KRT was increased, but once transplanted, their long-term outcome was similar to other KT recipients.

• Klíčová slova
• cancer, children, kidney replacement therapy, kidney transplantation, malignancy, mortality,
• MeSH
• chronické selhání ledvin * mortalita   etiologie   terapie   MeSH
• dítě MeSH
• hodnoty glomerulární filtrace MeSH
• kojenec MeSH
• lidé MeSH
• míra přežití MeSH
• mladiství MeSH
• nádory * komplikace   mortalita   MeSH
• náhrada funkce ledvin * mortalita   MeSH
• následné studie MeSH
• předškolní dítě MeSH
• přežívání štěpu MeSH
• prognóza MeSH
• registrace * MeSH
• rejekce štěpu * mortalita   etiologie   MeSH
• rizikové faktory MeSH
• studie případů a kontrol MeSH
• transplantace ledvin * mortalita   MeSH
• vyšetření funkce ledvin MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Kidney involvement is common in anti-neutrophil cytoplasm antibody-associated vasculitis (AAV) and the prognosis is determined by the severity of kidney damage. This study focused on long-term kidney outcomes, defining possible risk factors and comparing the performance of three different histological classifications to predict outcomes for patients with AAV. METHODS: The dataset included 848 patients with newly diagnosed AAV who participated in seven randomized controlled trials (RCTs) (1995-2012). Follow-up information was obtained from questionnaires sent to the principal investigators of the original RCTs. RESULTS: The cumulative incidence of end-stage kidney disease (ESKD) at 5 and 10 years was 17% and 22%, respectively. Patients who developed ESKD had reduced patient survival compared with those with preserved kidney function (hazard ratio 2.8, P < .001). Comparing patients with AAV and kidney involvement with a matched general population, patients with AAV had poor survival outcomes, even in early stages of chronic kidney disease. The main cause of death was infection followed by cardiovascular disease in patients developing ESKD and malignancy in those who did not. Some 34% of patients with initial need for dialysis recovered kidney function after treatment. Thirty-five out of 175 in need of kidney replacement therapy (KRT) during follow-up received a kidney transplant with good outcome; there was 86% patient survival at 10 years.In the subcohort of 214 patients with available kidney biopsies, three scoring systems were tested: the Berden classification, the Renal Risk Score and the Mayo Clinic Score. The scores highlighted the importance of normal glomeruli and severe glomerulosclerosis on kidney survival (P < .001 and P = .001, respectively). The Renal Risk Score demonstrated a moderate prediction of kidney survival (area under the curve 0.79; standard error 0.03, 95% confidence interval 0.71-0.83). CONCLUSIONS: Early diagnosis of AAV is extremely important. Even milder forms of kidney involvement have an impact on the prognosis. Patients in need of KRT had the lowest survival rates, but kidney transplantation has shown favorable outcomes for eligible AAV patients. The three histologic scoring systems were all identified as independent prognostic factors for kidney outcome.

• Klíčová slova
• ANCA-associated vasculitis, end-stage kidney disease, kidney histology scores, prognosis, transplantation,
• MeSH
• ANCA-asociované vaskulitidy * komplikace   mortalita   terapie   MeSH
• chronické selhání ledvin * mortalita   terapie   etiologie   MeSH
• dospělí MeSH
• hodnoty glomerulární filtrace MeSH
• lidé středního věku MeSH
• lidé MeSH
• míra přežití MeSH
• následné studie MeSH
• prognóza MeSH
• rizikové faktory MeSH
• senioři MeSH
• vyšetření funkce ledvin MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

INTRODUCTION: Periostin is a matricellular protein. Elevated serum concentrations of periostin have been reported in patients with various cardiovascular diseases, including heart failure. Patients with end-stage renal disease have a substantially increased risk for cardiovascular diseases. However, there is a lack of clinical studies to clarify the prognostic significance of systemic periostin on all-cause mortality in patients with end-stage renal disease on hemodialysis. METHODS: 313 stable end-stage renal disease patients were recruited and followed for 5 years concerning all-cause mortality. At baseline, we collected blood samples and clinical data. Serum periostin concentrations were measured using a certified ELISA. RESULTS: The optimal cut-off value for serum periostin regarding all-cause mortality, calculated through receiver operating characteristic analysis, was 777.5 pmol/L. Kaplan-Meier survival analysis using this cut-off value demonstrated that higher periostin concentrations are linked to higher all-cause mortality (log-rank test: p = 0.002). Subgroup analysis revealed that serum periostin concentrations only affected all-cause mortality in male but not in female patients (p = 0.002 in male patients and p = 0.474 in female patients). Multivariate Cox regression analyses, adjusted for confounding factors, likewise showed that elevated serum periostin concentrations were positively associated with all-cause mortality in male (p = 0.028) but not in female patients on hemodialysis (p = 0.313). CONCLUSION: Baseline serum periostin is an independent risk factor for all-cause mortality in male patients with chronic renal disease on hemodialysis.

• Klíčová slova
• All-cause mortality, Hemodialysis patients, Periostin, Sex dependency,
• MeSH
• biologické markery krev   MeSH
• chronické selhání ledvin * terapie   mortalita   krev   komplikace   MeSH
• dialýza ledvin * MeSH
• Kaplanův-Meierův odhad MeSH
• lidé středního věku MeSH
• lidé MeSH
• molekuly buněčné adheze * krev   MeSH
• periostin MeSH
• příčina smrti trendy   MeSH
• prognóza MeSH
• rizikové faktory MeSH
• ROC křivka MeSH
• senioři MeSH
• sexuální faktory MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• biologické markery MeSH
• molekuly buněčné adheze * MeSH
• periostin MeSH
• POSTN protein, human MeSH Prohlížeč

BACKGROUND: Hemodialyzers should efficiently eliminate small and middle molecular uremic toxins and possess exceptional hemocompatibility to improve well-being of patients with end-stage kidney disease. However, performance and hemocompatibility get compromised during treatment due to adsorption of plasma proteins to the dialyzer membrane. Increased membrane hydrophilicity reduces protein adsorption to the membrane and was implemented in the novel FX CorAL dialyzer. The present randomized controlled trial compares performance and hemocompatibility profiles of the FX CorAL dialyzer to other commonly used dialyzers applied in hemodiafiltration treatments. METHODS: This prospective, open, controlled, multicentric, interventional, crossover study randomized stable patients on post-dilution online hemodiafiltration (HDF) to FX CorAL 600, FX CorDiax 600 (both Fresenius Medical Care) and xevonta Hi 15 (B. Braun) each for 4 weeks. Primary outcome was β2-microglobulin removal rate (β2-m RR). Non-inferiority and superiority of FX CorAL versus comparators were tested. Secondary endpoints were RR and/or clearance of small and middle molecules, and intra- and interdialytic profiles of hemocompatibility markers, with regards to complement activation, cell activation/inflammation, platelet activation and oxidative stress. Further endpoints were patient reported outcomes (PROs) and clinical safety. RESULTS: 82 patients were included and 76 analyzed as intention-to-treat (ITT) population. FX CorAL showed the highest β2-m RR (76.28%), followed by FX CorDiax (75.69%) and xevonta (74.48%). Non-inferiority to both comparators and superiority to xevonta were statistically significant. Secondary endpoints related to middle molecules corroborated these results; performance for small molecules was comparable between dialyzers. Regarding intradialytic hemocompatibility, FX CorAL showed lower complement, white blood cell, and platelet activation. There were no differences in interdialytic hemocompatibility, PROs, or clinical safety. CONCLUSIONS: The novel FX CorAL with increased membrane hydrophilicity showed strong performance and a favorable hemocompatibility profile as compared to other commonly used dialyzers in clinical practice. Further long-term investigations should examine whether the benefits of FX CorAL will translate into improved cardiovascular and mortality endpoints. TRIAL REGISTRATION: eMPORA III registration on 19/01/2021 at ClinicalTrials.gov (NCT04714281).

• Klíčová slova
• Dialyzer, Hemocompatibility, Hemodiafiltration, Hydrophilicity, Membrane, Performance,
• MeSH
• beta-2-mikroglobulin krev   MeSH
• chronické selhání ledvin terapie   MeSH
• hemodiafiltrace * přístrojové vybavení   metody   MeSH
• hydrofobní a hydrofilní interakce * MeSH
• klinické křížové studie * MeSH
• lidé středního věku MeSH
• lidé MeSH
• membrány umělé * MeSH
• prospektivní studie MeSH
• senioři MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• randomizované kontrolované studie MeSH
• Názvy látek
• beta-2-mikroglobulin MeSH
• membrány umělé * MeSH

BACKGROUND: This retrospective real-world study used data from two registries, International Pediatric Peritoneal Dialysis Network (IPPN) and International Pediatric Hemodialysis Network (IPHN), to characterize the efficacy and safety of continuous erythropoietin receptor activator (C.E.R.A.) in pediatric patients with chronic kidney disease (CKD) on peritoneal dialysis (PD) or hemodialysis (HD). METHODS: IPPN and IPHN collect prospective data (baseline and every 6 months) from pediatric PD and HD centers worldwide. Demographics, clinical characteristics, dialysis information, treatment, laboratory parameters, number and causes of hospitalization events, and deaths were extracted for patients on C.E.R.A. treatment (IPPN: 2007-2021; IPHN: 2013-2021). RESULTS: We analyzed 177 patients on PD (median age 10.6 years) and 52 patients on HD (median age 14.1 years) who had ≥ 1 observation while being treated with C.E.R.A. The median (interquartile range [IQR]) observation time under C.E.R.A. exposure was 6 (0-12.5) and 12 (0-18) months, respectively. Hemoglobin concentrations were stable over time; respective means (standard deviation) at last observation were 10.9 (1.7) g/dL and 10.4 (1.7) g/dL. Respective median (IQR) monthly C.E.R.A. doses at last observation were 3.5 (2.3-5.1) µg/kg, or 95 (62-145) µg/m2 and 2.1 (1.2-3.4) µg/kg, or 63 (40-98) µg/m2. Non-elective hospitalizations occurred in 102 (58%) PD and 32 (62%) HD patients. Seven deaths occurred (19.8 deaths per 1000 observation years). CONCLUSIONS: C.E.R.A. was associated with efficient maintenance of hemoglobin concentrations in pediatric patients with CKD on dialysis, and appeared to have a favorable safety profile. The current analysis revealed no safety signals.

• Klíčová slova
• Chronic kidney disease, Continuous erythropoietin receptor activator, Hemodialysis, Peritoneal dialysis, Real-world study,
• MeSH
• chronická renální insuficience * terapie   farmakoterapie   MeSH
• chronické selhání ledvin * terapie   MeSH
• dialýza ledvin škodlivé účinky   MeSH
• dítě MeSH
• erythropoetin * MeSH
• hemoglobiny analýza   MeSH
• lidé MeSH
• mladiství MeSH
• prospektivní studie MeSH
• registrace MeSH
• retrospektivní studie MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• erythropoetin * MeSH
• hemoglobiny MeSH

BACKGROUND: Heart failure (HF) is a frequent cause of morbidity and mortality of end-stage kidney disease (ESKD) patients on hemodialysis. It is not easy to distinguish HF from water overload. The traditional HF definition has low sensitivity and specificity in this population. Moreover, many patients on hemodialysis have exercise limitations unrelated to HF. Therefore, we postulated two new HF definitions ((1) Modified definition of the Acute Dialysis Quality Improvement working group; (2) Hemodynamic definition based on the calculation of the effective cardiac output). We hypothesize that the newer definitions will better identify patients with higher number of endpoints and with more advanced structural heart disease. METHODS: Cohort, observational, longitudinal study with recording predefined endpoints. Patients (n = 300) treated by hemodialysis in six collaborating centers will be examined centrally in a tertiary cardiovascular center every 6-12 months lifelong or till kidney transplantation by detailed expert echocardiography with the calculation of cardiac output, arteriovenous dialysis fistula flow volume calculation, bio-impedance, and basic laboratory analysis including NTproBNP. Effective cardiac output will be measured as the difference between measured total cardiac output and arteriovenous fistula flow volume and systemic vascular resistance will be also assessed non-invasively. In case of water overload during examination, dry weight adjustment will be recommended, and the patient invited for another examination within 6 weeks. A composite major endpoint will consist of (1) Cardiovascular death; (2) HF worsening/new diagnosis of; (3) Non-fatal myocardial infarction or stroke. The two newer HF definitions will be compared with the traditional one in terms of time to major endpoint analysis. DISCUSSION: This trial will differ from others by: (1) detailed repeated hemodynamic assessment including arteriovenous access flow and (2) by careful assessment of adequate hydration to avoid confusion between HF and water overload.

• Klíčová slova
• Hemodialysis, arteriovenous access, effective cardiac output, heart failure,
• MeSH
• chronická renální insuficience * komplikace   MeSH
• chronické selhání ledvin * diagnóza   terapie   komplikace   MeSH
• dialýza ledvin škodlivé účinky   MeSH
• lidé MeSH
• longitudinální studie MeSH
• pozorovací studie jako téma MeSH
• srdeční selhání * diagnóza   etiologie   terapie   MeSH
• voda MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• protokol klinické studie MeSH
• Geografické názvy
• Česká republika MeSH
• Názvy látek
• voda MeSH

• Klíčová slova
• End-stage renal disease, Hemodialysis, Type 1 diabetes, Type 2 diabetes,
• MeSH
• chronické selhání ledvin * terapie   epidemiologie   MeSH
• diabetes mellitus * epidemiologie   MeSH
• dialýza ledvin MeSH
• lidé MeSH
• prevalence MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• dopisy MeSH
• komentáře MeSH

Updated guidelines on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were released in 2021 by the American College of Rheumatology jointly with the Vasculitis Foundation and, subsequently, in 2022 by the European Alliance of Associations for Rheumatology. In addition, in 2021, the Kidney Disease: Improving Global Outcomes had released updated recommendations on the treatment of AAV with glomerulonephritis (AAV-GN). Kidney involvement is particularly relevant in microscopic polyangiitis and granulomatosis with polyangiitis, but is less frequent in eosinophilic granulomatosis with polyangiitis. The management of AAV-GN has been a focus for drug development and change over the past 10 years. Avoidance of progression to end-stage kidney disease (ESKD) or kidney failure is one of the main unmet needs in the management of AAV, with ESKD having a major impact on morbidity, health costs and mortality risk. Relevant changes in AAV-GN management are related to remission-induction treatment of patients with severe kidney disease, the use of glucocorticoids and avacopan, and remission-maintenance treatment. All the documents provide guidance in accordance with the evidence-based standard of care available at the time of their release. With our work we aim to (i) show the progress made and identify the differences between guidelines and recommendations, (ii) discuss the supporting rationale for those, and (iii) identify gaps in knowledge that could benefit from additional research and should be revised in subsequent updates.

• Klíčová slova
• ANCA-associated vasculitis, glomerulonephritis, granulomatosis with polyangiitis, guidelines, microscopic polyangiitis,
• MeSH
• ANCA-asociované vaskulitidy * komplikace   farmakoterapie   MeSH
• chronické selhání ledvin * etiologie   terapie   MeSH
• Churgův-Straussové syndrom * MeSH
• glomerulonefritida * farmakoterapie   MeSH
• granulomatóza s polyangiitidou * terapie   MeSH
• lidé MeSH
• mikroskopická polyangiitida * terapie   MeSH
• protilátky proti cytoplazmě neutrofilů MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• protilátky proti cytoplazmě neutrofilů MeSH