BACKGROUND: VAVASC study (Validation of Arterio Venous Access Stage Classification) is a multicentre, international, prospective study. The study aims to validate the AVAS classification, which is a classification system describing vascular status of patients indicated for creation of arteriovenous access on the upper limb. METHODS: Observational, prospective, multicentre, international study starting in March 2021. Participant recruitment has commenced. Basic demographic data, risk factors and vascular mapping parameters are collected via an online platform. The outcome measures are class of AVAS, predicted arteriovenous access, final arteriovenous access that has been created and a functionality of the arteriovenous access. Predictive models will be used for statistical analysis. CURRENT STATUS: A total of 140 patients from 4 centres in Great Britain, Czech Republic, Brazil and Slovakia are already included and undergoing evaluation. CONCLUSIONS: The study is registered in the Clinical trials registry (NCT04796558), https://register.clinicaltrials.gov/. Study is still open for collaboration with other centres that can register via www.vavasc.com.
- MeSH
- arteriovenózní zkrat * škodlivé účinky metody MeSH
- chronické selhání ledvin * etiologie MeSH
- dialýza ledvin metody MeSH
- horní končetina krevní zásobení MeSH
- lidé MeSH
- multicentrické studie jako téma MeSH
- pozorovací studie jako téma MeSH
- prospektivní studie MeSH
- průchodnost cév MeSH
- výsledek terapie MeSH
- výzkumný projekt MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- protokol klinické studie MeSH
- MeSH
- bortezomib aplikace a dávkování škodlivé účinky terapeutické užití MeSH
- chronické selhání ledvin etiologie terapie MeSH
- cyklofosfamid aplikace a dávkování škodlivé účinky terapeutické užití MeSH
- dexamethason aplikace a dávkování škodlivé účinky terapeutické užití MeSH
- dialýza ledvin metody MeSH
- lidé středního věku MeSH
- lidé MeSH
- paraproteinemie * diagnóza komplikace terapie MeSH
- proteinurie etiologie MeSH
- renální insuficience diagnóza etiologie terapie MeSH
- senioři MeSH
- transplantace kostní dřeně metody škodlivé účinky MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
- Klíčová slova
- roxadustat,
- MeSH
- anemie * diagnóza etiologie farmakoterapie MeSH
- chronické selhání ledvin etiologie komplikace MeSH
- erytropoéza účinky léků MeSH
- hepcidiny MeSH
- hypoxie etiologie farmakoterapie MeSH
- inhibitory prolylhydroxylas * klasifikace terapeutické užití MeSH
- klinická studie jako téma MeSH
- transkripční faktory MeSH
- Publikační typ
- přehledy 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.
- 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
RATIONALE & OBJECTIVE: There is a dearth of data characterizing patients receiving kidney replacement therapy (KRT) for kidney failure due to systemic lupus erythematosus (SLE) and their clinical outcomes. The aim of this study was to describe trends in incidence and prevalence of KRT among these patients as well as to compare their outcomes versus those of patients treated with KRT for diseases other than SLE. STUDY DESIGN: Retrospective cohort study based on kidney registry data. SETTING & PARTICIPANTS: Patients recorded in 14 registries of patients receiving KRT that provided data to the European Renal Association Registry between 1992 and 2016. PREDICTOR: SLE as cause of kidney failure. OUTCOMES: Incidence and prevalence of KRT, patient survival while receiving KRT, patient and graft survival after kidney transplant, and specific causes of death. ANALYTICAL APPROACH: Kaplan-Meier methods and Cox regression models were fit to compare patient survival between the SLE and non-SLE groups, overall KRT, dialysis, and patient and graft survival after kidney transplant. RESULTS: In total, 1,826 patients commenced KRT for kidney failure due to SLE, representing an incidence of 0.80 per million population (pmp) per year. The incidence remained stable during the study period (annual percent change, 0.1% [95% CI, -0.6% to 0.8%]). Patient survival among patients with SLE receiving KRT was similar to survival in the comparator group (hazard ratio [HR], 1.11 [95% CI, 0.99-1.23]). After kidney transplant, the risk of death was greater among patients with SLE than among patients in the comparator group (HR, 1.25 [95% CI, 1.02-1.53]), whereas the risk of all-cause graft failure was similar (HR, 1.09 [95% CI, 0.95-1.27]). Ten-year patient overall survival during KRT and patient and graft survival after kidney transplant improved over the study period (HRs of 0.71 [95% CI, 0.56-0.91], 0.43 [95% CI, 0.27-0.69], and 0.60 [95% CI, 0.43-0.84], respectively). Patients with SLE receiving KRT were significantly more likely to die of infections (24.8%) than patients in the comparator group (16.9%; P < 0.001). LIMITATIONS: No data were available on extrarenal manifestations of SLE, drug treatments, comorbidities, kidney transplant characteristics, or relapses of SLE. CONCLUSIONS: The prognosis of patients with SLE receiving KRT has improved over time. Survival of patients with SLE who required KRT was similar compared with patients who required KRT for other causes of kidney failure. Survival following kidney transplants was worse among patients with SLE.
- MeSH
- chronické selhání ledvin * epidemiologie etiologie terapie MeSH
- incidence MeSH
- lidé MeSH
- náhrada funkce ledvin metody MeSH
- nefritida při lupus erythematodes * MeSH
- registrace MeSH
- renální insuficience * komplikace MeSH
- retrospektivní studie MeSH
- systémový lupus erythematodes * komplikace epidemiologie terapie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Pacienti s chronickým onemocněním ledvin nejsou často pro minimální obtíže včas podchyceni ani praktickým lékařem ani odborným lékařem a přicházejí k léčbě nahrazující funkci ledvin nepřipraveni a pozdě. Nemají informace o možnostech léčby, včetně preemptivní transplantace ledvin a nemají vytvořený trvalý dialyzační přístup. Tento fakt pak zvyšuje riziko úmrtí a morbiditu pacientů po zařazení do dialyzačního programu. Preventivní vyšetření, jako je kontrola krevního tlaku, biochemické vyšetření séra, vyšetření moči a vyšetření glomerulární filtrace by umožnilo záchyt těchto pacientů praktickými a odbornými lékaři a včasné předání nemocných nefrologům.
Chronic kidney disease patients often present late for dialysis due to minimal clinical symptoms, which are frequently undiscovered by both general practitioners and specialists. They have no information about renal replacement therapy, including preemptive kidney transplantation and do not have a permanent dialysis access. This fact increases the morbidity and mortality of those patients. Common preventive examinations such as blood pressure measurement, serum biochemistry, urinalysis, and glomerular filtration tests, might help with earlier diagnosis of these patients by others specialities and timely referral to nephrologist.
- MeSH
- algoritmy MeSH
- analýza moči MeSH
- chronické selhání ledvin diagnóza etiologie klasifikace patologie prevence a kontrola terapie MeSH
- hematurie diagnóza MeSH
- hodnoty glomerulární filtrace fyziologie MeSH
- ledviny * fyziologie MeSH
- lidé MeSH
- proteinurie diagnóza klasifikace MeSH
- rizikové faktory MeSH
- Check Tag
- lidé MeSH
We have developed an artificial neural network prediction model for end-stage kidney disease (ESKD) in patients with primary immunoglobulin A nephropathy (IgAN) using a retrospective cohort of 948 patients with IgAN. Our tool is based on a two-step procedure of a classifier model that predicts ESKD, and a regression model that predicts development of ESKD over time. The classifier model showed a performance value of 0.82 (area under the receiver operating characteristic curve) in patients with a follow-up of five years, which improved to 0.89 at the ten-year follow-up. Both models had a higher recall rate, which indicated the practicality of the tool. The regression model showed a mean absolute error of 1.78 years and a root mean square error of 2.15 years. Testing in an independent cohort of 167patients with IgAN found successful results for 91% of the patients. Comparison of our system with other mathematical models showed the highest discriminant Harrell C index at five- and ten-years follow-up (81% and 86%, respectively), paralleling the lowest Akaike information criterion values (355.01 and 269.56, respectively). Moreover, our system was the best calibrated model indicating that the predicted and observed outcome probabilities did not significantly differ. Finally, the dynamic discrimination indexes of our artificial neural network, expressed as the weighted average of time-dependent areas under the curve calculated at one and two years, were 0.80 and 0.79, respectively. Similar results were observed over a 25-year follow-up period. Thus, our tool identified individuals who were at a high risk of developing ESKD due to IgAN and predicted the time-to-event endpoint. Accurate prediction is an important step toward introduction of a therapeutic strategy for improving clinical outcomes.
CONTEXT: Fabry disease is a rare X-linked genetic disease due to pathogenic variants in the GLA gene. Classic Fabry disease is characterized by glycosphingolipids accumulation in all organs including the kidney, resulting in end-stage renal disease in a subset of male patients. Fabry disease should therefore be considered in the differential diagnosis of patients with unexplained end-stage renal disease. OBJECTIVE: We performed a prospective screening study in Western France to determine the prevalence of Fabry disease in a large population of dialyzed and transplanted patients. PATIENTS AND METHODS: Patients meeting the inclusion criteria (males, 18-70 years with end-stage renal disease of unknown or vascular origin) were selected from the REIN® registry and the CRISTAL® database. Screening on filter papers was performed after patient consent was obtained during either a dialysis session or a transplantation follow-up visit. RESULTS: One thousand five hundred and sixty-one end-stage renal disease male patients were screened and 819 consented (dialysis: n=242; transplant: n=577). One single patient was found with decreased alpha-galactosidase levels <25%. GLA sequencing identified the p.Phe113Leu variant in favor of an unknown superimposed kidney disease responsible for end-stage renal disease since this GLA pathogenic variant is associated with a later-onset cardiac form of Fabry disease with minimal kidney involvement. Family cascade genotyping revealed a previously undiagnosed affected brother. CONCLUSION: The prevalence of Fabry disease in end-stage renal disease patients was 0.12%, questioning the efficacy of this screening strategy with respect to the low prevalence. However, beside the benefit for the patient and his family, the increased awareness of Fabry disease among participating nephrologists may be of interest for future patients.
Diabetes mellitus 1. a 2. typu vede téměř u poloviny pacientů k onemocnění ledvin. Zhoršení renální funkce zvyšuje kardiovaskulární riziko, a tím i mortalitu pacientů s diabetem. Onemocnění ledvin musí být včas diagnostikováno a léčeno. Prevencí vzniku a progrese renálního postižení je léčba hypertenze, kompenzace diabetu a ovlivnění rizikových faktorů. Cílový glykovaný hemoglobin je od 45 do 60 mmol/mol, cílový krevní tlak pod 130/80 mm Hg. Základem léčby hypertenze jsou inhibitory angiotenzin konvertujícího enzymu nebo blokátory receptoru pro angiotenzin. Léčba diabetiků 2. typu zaznamenala v poslední době významnou změnu - kromě již užívaných léků snižujících inzulínovou rezistenci (metformin, thiazolidindiony) a léků zvyšujících inzulinovou sekreci (deriváty sulfonylurey, inkretiny) je k dispozici nová skupina léků - glifloziny. Glifloziny jsou blokátory sodíko-glukózového kotransportéru snižující zpětné vychytávání glukózy v proximálním tubulu. Glifloziny a agonisté glukagonu podobného peptidu 1 mají nefroprotektivní efekt a významně snižují kardiovaskulární riziko. Nefroprotekce a kardiovaskulární účinek je staví na přední místo mezi perorálními antidiabetiky.
The diabetes mellitus type 1 and 2 leads to kidney disease in almost half of the cases. Impaired renal function increases the cardiovascular risk and thus the mortality of patients with diabetes. Kidney disease must be diagnosed and treated on time. Treatment of hypertension, good compensation of diabetes and dealing with risk factors works as prevention of renal impairment progressing. Requested glycated hemoglobin ranges from 45 to 60 mmol/mol, the target blood pressure is below 130/80 mmHg. The treatment of hypertension is based on angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. The treatment of diabetes type 2 has recently undergone a significant change. In addition to drugs reducing insulin resistance (metformin, thiazolidinediones) that are already in use and drugs that increase the secretion of insulin (sulfonylurea derivatives, glyptines and glucagon-like peptide 1 agonists), a new group of drugs becomes available-gliflozins. Gliflozins are sodium-glucose cotransporter blockers that reduce glucose reuptake in the proximal tubule. Gliflozins and glucagon-like peptide 1 agonists have a nephroprotective effect and significantly reduce cardiovascular risk. That is the reason these drugs are at the forefront of all oral antidiabetics.
- MeSH
- albuminurie diagnóza etiologie komplikace MeSH
- chronické selhání ledvin etiologie farmakoterapie komplikace MeSH
- diabetes mellitus 2. typu farmakoterapie MeSH
- diabetické nefropatie * farmakoterapie klasifikace patofyziologie MeSH
- glifloziny terapeutické užití MeSH
- hodnoty glomerulární filtrace účinky léků MeSH
- hypertenze farmakoterapie komplikace MeSH
- hypoglykemika * terapeutické užití MeSH
- komplikace diabetu diagnóza farmakoterapie prevence a kontrola MeSH
- lidé MeSH
- metformin terapeutické užití MeSH
- náhrada funkce ledvin metody MeSH
- rizikové faktory kardiovaskulárních chorob MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
OBJECTIVE: To update the 2012 EULAR/ERA-EDTA recommendations for the management of lupus nephritis (LN). METHODS: Following the EULAR standardised operating procedures, a systematic literature review was performed. Members of a multidisciplinary Task Force voted independently on their level of agreeement with the formed statements. RESULTS: The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNIs) and management of end-stage kidney disease (ESKD). The target of therapy is complete response (proteinuria <0.5-0.7 g/24 hours with (near-)normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2-3 g/day or mycophenolic acid (MPA) at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500 mg × 6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3-0.5 mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1 g/24 hours despite renin-angiotensin-aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations. Treatment of LN in children follows the same principles as adult disease. CONCLUSIONS: We have updated the EULAR recommendations for the management of LN to facilitate homogenization of patient care.
- MeSH
- antirevmatika terapeutické užití MeSH
- azathioprin terapeutické užití MeSH
- chronické selhání ledvin etiologie terapie MeSH
- glukokortikoidy terapeutické užití MeSH
- hodnoty glomerulární filtrace MeSH
- hydroxychlorochin terapeutické užití MeSH
- imunosupresiva terapeutické užití MeSH
- inhibitory kalcineurinu terapeutické užití MeSH
- kombinovaná farmakoterapie MeSH
- kyselina mykofenolová terapeutické užití MeSH
- lidé MeSH
- nefritida při lupus erythematodes komplikace farmakoterapie patologie patofyziologie MeSH
- proteinurie etiologie terapie MeSH
- společnosti lékařské * MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- směrnice pro lékařskou praxi MeSH
- Geografické názvy
- Evropa MeSH
