Therapeutic plasma exchange (PLEX) is an adjunctive treatment for patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and kidney involvement. Little is known about the effect of PLEX on early changes in kidney function. This post-hoc analysis of the PEXIVAS trial investigated the effects of PLEX on changes in kidney function within 12 months. PEXIVAS was a randomized controlled trial recruiting 691 patients with ANCA-associated glomerulonephritis, of whom 349 underwent PLEX and 342 received no-PLEX. The primary outcomes of this post hoc study of PEXIVAS were change in estimated glomerular filtration rate (eGFR) from baseline and recovery of kidney function (defined as eGFR increase of 15ml/min/1.73m2 or more). Baseline eGFR was 21.7 ± 20.3 and 20.6 ± 18.7 ml/min/1.73m2 in the PLEX and no-PLEX groups, respectively. Mean improvements in eGFR at weeks two, four, and eight after initiation of therapy were greater for the PLEX vs. the no-PLEX groups. The greatest significant difference in recovery of kidney function in the PLEX compared to the no-PLEX groups was at week four (relative risk (RR): 1.41; 95% confidence interval:1.09-1.82). Increased eGFR or recovery of kidney function at week four were significantly associated with lower risk for end-stage kidney disease at week 52 (RR: 0.96: 0.95-0.97, and RR: 0.29: 0.16-0.52; respectively). Neither changes in eGFR nor recovery of kidney function differed by reduced- compared to standard-dose glucocorticoid group. Overall, our study indicates that PLEX improves early kidney function in patients with ANCA-associated glomerulonephritis.
- Klíčová slova
- ANCA, end-stage kidney disease, glomerulonephritis, kidney function, plasma exchange, vasculitis,
- MeSH
- ANCA-asociované vaskulitidy * patofyziologie terapie farmakoterapie komplikace imunologie diagnóza MeSH
- dospělí MeSH
- glomerulonefritida * patofyziologie imunologie terapie krev MeSH
- glukokortikoidy * terapeutické užití aplikace a dávkování MeSH
- hodnoty glomerulární filtrace * MeSH
- ledviny * patofyziologie účinky léků MeSH
- lidé středního věku MeSH
- lidé MeSH
- obnova funkce MeSH
- senioři MeSH
- výměna plazmy * MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- 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
- glukokortikoidy * MeSH
The Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guideline for the Management of Glomerular Diseases is an update to the KDIGO 2012 guideline. The aim is to assist clinicians caring for individuals with glomerulonephritis (GN), both adults and children. The scope includes various glomerular diseases, including IgA nephropathy and IgA vasculitis, membranous nephropathy, nephrotic syndrome, minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), infection-related GN, antineutrophil cytoplasmic antibody (ANCA) vasculitis, lupus nephritis, and anti-glomerular basement membrane antibody GN. In addition, this guideline will be the first to address the subtype of complement-mediated diseases. Each chapter follows the same format providing guidance related to diagnosis, prognosis, treatment, and special situations. The goal of the guideline is to generate a useful resource for clinicians and patients by providing actionable recommendations based on evidence syntheses, with useful infographics incorporating views from experts in the field. Another aim is to propose research recommendations for areas where there are gaps in knowledge. The guideline targets a broad global audience of clinicians treating GN while being mindful of implications for policy and cost. Development of this guideline update followed an explicit process whereby treatment approaches and guideline recommendations are based on systematic reviews of relevant studies, and appraisal of the quality of the evidence and the strength of recommendations followed the "Grading of Recommendations Assessment, Development and Evaluation" (GRADE) approach. Limitations of the evidence are discussed, with areas of future research also presented.
- Klíčová slova
- AAV, ANCA, C3, FSGS, IgA nephropathy, IgA vasculitis, KDIGO, MPGN, anti-GBM, complement, evidence-based, glomerular diseases, glomerulonephritis, guideline, infection-related glomerulonephritis, lupus nephritis, membranous nephropathy, minimal change disease, nephrotic syndrome, systematic review,
- MeSH
- dítě MeSH
- dospělí MeSH
- glomerulonefritida * diagnóza terapie MeSH
- IgA nefropatie * diagnóza terapie MeSH
- ledviny MeSH
- lidé MeSH
- lipoidní nefróza * MeSH
- membranózní glomerulonefritida * diagnóza farmakoterapie MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- směrnice pro lékařskou praxi MeSH
Pulmonary syndrome is defined by occurrence of lung involvement (alveolar haemorrhage) in association with renal failure (with a typical crescentic necrotizing rapidly progressive glomerulonephritis). It is caused by an autoimmune disease, most frequently ANCA-associated vasculitides and anti-GBM (glomerular basement membrane) disease. Early establishment of the right diagnosis and immediate treatment are crucial for favourable prognosis of the patients. First choice therapy includes high-dose corticosteroids and cyclophosphamide, usually with plasma exchange added. Newer therapeutic possibilities include especially rituximab even though there is limited experience with its use in the settings of the most severe cases of pulmonary syndrome.
- Klíčová slova
- ANCA, anti‑glomerular basement membrane (anti‑GBM) antibodies, diffuse alveolar haemorrhage, rapidly progressive glomerulonephritis, rituximab, vasculitis,
- MeSH
- ANCA-asociované vaskulitidy * MeSH
- autoprotilátky MeSH
- glomerulonefritida * terapie MeSH
- krvácení MeSH
- lidé MeSH
- syndrom MeSH
- výměna plazmy MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- autoprotilátky MeSH
Podocytes form an outer aspect of the glomerular capillary wall and play a decisive role in its permeability for macromolecules. The main podocyte surface antigen podocalyxin, a highly electronegative sialoglycoprotein, prevents the podocyte foot processes from collapsing. Podocyte damage in glomerular disease is supposed to be accompanied by podocyte detachment, and shed podocytes and their fragments (marked by podocalyxin) may be identified in the urine. Using anti-podocalyxin monoclonal antibody, PCX+EL were counted by FACS in 38 patients with various types of active glomerulonephritis, 15 patients with chronic glomerulonephritis in long-term remission and 44 healthy controls. Urinary levels of PCX+EL were significantly higher in patients with active glomerulonephritis compared to patients with chronic glomerulonephritis in longterm remission (93 +/- 100 vs. 6.3 +/- 3.2/microl of urine, P < 0.000001) and healthy controls (4.4 +/- 2.6/microl of urine, P < 0.000001 compared to active glomerulonephritis, n.s. compared to chronic glomerulonephritis in longterm remission). These preliminary data suggest the potential of this simple method to monitor the activity of glomerular disease. Further prospective studies of larger cohorts of patients with individual glomerular diseases are clearly warranted.
- MeSH
- analýza moči metody MeSH
- časové faktory MeSH
- dospělí MeSH
- glomerulonefritida patologie terapie moč MeSH
- indukce remise MeSH
- lidé středního věku MeSH
- lidé MeSH
- podocyty patologie MeSH
- průtoková cytometrie MeSH
- senioři MeSH
- sialoglykoproteiny moč MeSH
- studie případů a kontrol MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Názvy látek
- podocalyxin MeSH Prohlížeč
- sialoglykoproteiny MeSH
Authors present a group of patients in the article who were monitored at a nephrology outpatient department of the University Hospital in Martin between years 1997 and 2001 for nephritic or nephrotic syndrome. Indications, contraindications and ways of histology examinations of kidneys in their department are discussed in the beginning of the work. Than prevalence of individual types of glomerulonephritides as well as way and length of therapy based on histology picture are discussed in the monitored group of patients. In the end results of therapeutic response are presented. Among other things, authors came to a conclusion that it is the least possible to manage the disease when histology results show sclerotisation of glomerules and in cases of frequent relapses in prolipherative forms of glomerulonephritides.
- MeSH
- biopsie MeSH
- dospělí MeSH
- glomerulonefritida * klasifikace diagnóza patologie terapie MeSH
- ledviny patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- nefrotický syndrom diagnóza patologie terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
Antineutrophil cytoplasmic antibody (ANCA)-positive renal vasculitis is the most common cause of rapidly progressive (crescentic) glomerulonephritis. Its life-threatening natural course may be modified substantially by current treatment modalities. The European Vasculitis Study Group (EUVAS) developed a subclassification of ANCA-positive vasculitides based on the disease severity at presentation, and have organized (so far) two waves of clinical trials. The first wave of randomized clinical trials had the aim of optimizing the existing therapeutic regimens; the second wave concentrated on testing some newer therapeutic approaches. Here, the design and available results of the first wave and the design of some second wave trials are reviewed briefly. The potential of the new targeted approaches (e.g. anti-tumour necrosis factor therapy) is also briefly mentioned.
- MeSH
- glomerulonefritida komplikace diagnóza terapie MeSH
- imunosupresiva aplikace a dávkování MeSH
- kombinovaná terapie MeSH
- lidé MeSH
- plazmaferéza metody MeSH
- prognóza MeSH
- protilátky proti cytoplazmě neutrofilů analýza MeSH
- randomizované kontrolované studie jako téma MeSH
- stupeň závažnosti nemoci MeSH
- vaskulitida komplikace diagnóza terapie MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Geografické názvy
- Evropa MeSH
- Názvy látek
- imunosupresiva MeSH
- protilátky proti cytoplazmě neutrofilů MeSH
Metabolic acidosis is a known complication of chronic renal failure. Maintenance of the pH within the reference range is important for influencing manifestations of the uraemic syndrome and the mortality of haemodialyzed patients. Intermittent bicarbonate haemodialysis should ensure the acid-base balance within the reference range also during the interval between haemodialyzations. In a short-term prospective study 20 haemodialyzed patients with chronic glomerulonephritis and pyelonephritis were examined. The authors assessed indicators of the acid-base balance (pH, HCO3-, pCO2) at the time of two haemodialyses and during the interval between haemodialyses. The blood flow in the dialyzation monitor was 300 ml/min. and the flow of the dialyzation solution 500 ml/min. The bicarbonate concentration in the dialyzation solution was 34 mmol/l. The duration of haemodialysis was 4 hours three times per week. Bicarbonate haemodialysis with a bicarbonate concentration of 34 mmol/l in the dialyzation solution ensured also during the interval between dialyzations a pH in the reference range in patients with chronic renal failure.
- MeSH
- acidobazická rovnováha * MeSH
- acidóza prevence a kontrola MeSH
- chronická nemoc MeSH
- chronické selhání ledvin komplikace metabolismus terapie MeSH
- dialýza ledvin * MeSH
- glomerulonefritida metabolismus terapie MeSH
- hemodialyzační roztoky MeSH
- hydrogenuhličitany aplikace a dávkování MeSH
- koncentrace vodíkových iontů MeSH
- lidé MeSH
- pyelonefritida metabolismus terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- Názvy látek
- hemodialyzační roztoky MeSH
- hydrogenuhličitany MeSH
BACKGROUND: Anti-neutrophil cytoplasmic antibodies (ANCA) define pathogenetically related group of renal vasculitides and glomerulonephritides mostly with serious prognosis. If unrecognized, these life-threatening diseases may cause loss of independent renal function and other dangerous extrarenal complications (e. g. pulmonary haemorrhage). We concentrated on the diagnosis, treatment and log-term follow-up of these patients. METHODS AND RESULTS: Renal biopsy was performed in 46 ANCA-positive patients. Age and sex distribution, type of ANCA, organ involvement, renal biopsy findings, renal function and effect of therapy were analyzed in these patients. Twenty three patients suffered from renal vasculitis, most commonly Wegener's granulomatosis (14 patients) and microscopic polyarthritis (7 patients). IgA nephropathy (7 patients) and idiopathic necrotizing/crescentic glomerulonephritis (8 patients) prevailed in patients with limited renal involvement. Renal morphology and function was most seriously impaired in patients with Wegener's granulomatosis. Immunosuppressive treatment was able to control the activity of the disease with the negativization of ANCA and improvement or stabilization of renal function in more than 90% of patients.CONCLUSIONS. ANCA-positive renal vasculitis and glomerulonephritis is relatively common. Clinical signs of extrarenal involvement are present in about 50% of patients with ANCA-positive glomerulonephritis. Rapidly introduced immunosuppressive treatment effectively controls renal and extrarenal manifestations of the disease.
- MeSH
- autoprotilátky analýza MeSH
- dospělí MeSH
- glomerulonefritida imunologie terapie MeSH
- granulomatóza s polyangiitidou imunologie terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nemoci ledvin imunologie terapie MeSH
- protilátky proti cytoplazmě neutrofilů MeSH
- senioři MeSH
- vaskulitida imunologie terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- Názvy látek
- autoprotilátky MeSH
- protilátky proti cytoplazmě neutrofilů MeSH
The mechanisms of free-radical injury include reactions with proteins, nucleic acids, and polysaccharides; and covalent binding to membrane components and initiation of lipid peroxidation. Cells have developed antioxidant defense to prevent free-radical injury including superoxide dismutase (SOD) and glutathione peroxidase (GPx). Significantly higher concentrations of total malondialdehyde (MDA) in plasma (1.22 +/- 0.42 vs. 0.64 +/- 0.22 micromol/L, p < 0.0001) as well as erythrocytes (2.56 +/- 1.28 vs. 1.03 +/- 0.44 micromol/L, p < 0.0001) of the CAPD patients were found when compared to the control group. The free MDA in plasma and the erythrocytes do not differ significantly in continuous ambulatory peritoneal dialysis (CAPD) patients and the control group. A significantly lower activity of GPx in erythrocytes of CAPD patients (17.85 +/- 2.63 U/g Hb vs. 23.26 +/- 3.61 U/g Hb, p < 0.0001) was found when compared to the control group, but the SOD activity in erythrocytes is not different (2272.36 +/- 579.92 U/g Hb vs. 2347.13 +/- 502.51 U/g Hg, NS). Our results show an increase of total MDA in erythrocytes and plasma. MDA is the product of lipid peroxidation with decreasing activity of GPx, which is capable of detoxifying peroxides. The activity of SOD did not change in CAPD patients. These results propose a possible role of free radicals with reduced antioxidant activity of GPx in CAPD patients and indicate that they could play some role in other pathological conditions such as atherogenesis and hemolysis.
- MeSH
- analýza rozptylu MeSH
- antioxidancia * MeSH
- chronická nemoc MeSH
- dospělí MeSH
- glomerulonefritida krev terapie MeSH
- glutathionperoxidasa krev MeSH
- hemoglobiny analýza MeSH
- intersticiální nefritida krev terapie MeSH
- kontinuální ambulantní peritoneální dialýza * statistika a číselné údaje MeSH
- lidé středního věku MeSH
- lidé MeSH
- malondialdehyd krev MeSH
- peroxidace lipidů * MeSH
- senioři MeSH
- superoxiddismutasa krev MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- srovnávací studie MeSH
- Názvy látek
- antioxidancia * MeSH
- glutathionperoxidasa MeSH
- hemoglobiny MeSH
- malondialdehyd MeSH
- superoxiddismutasa MeSH
The authors describe the clinical, laboratory and morphological findings, the clinical course and response to treatment in three patients with rapidly progressing glomerulonephritis where positive antibodies against the cytoplasm of neutrophil granulocytes (ANCA) were found. The authors confront their own clinical experience with data in the literature. Examination of ANCA is not only a significant contribution towards a more accurate diagnosis of renal vasculitis, but also an indicator of the activity of the disease and thus of the effectiveness of immunosuppressive treatment.
- MeSH
- autoprotilátky analýza MeSH
- cytoplazma imunologie MeSH
- dospělí MeSH
- glomerulonefritida imunologie patologie terapie MeSH
- ledviny patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- neutrofily imunologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- autoprotilátky MeSH
