A novel dosing approach for rituximab in glomerular diseases based on a population pharmacokinetic analysis.

Objectives: Rituximab is being increasingly prescribed for the treatment of autoimmune glomerular diseases. While it is highly effective for some diseases, the response is less predictable for others, which may be due to differing requirements in terms of the dosing according to the disease type and variations concerning exposure to the drug.

Methods: We compiled novel rituximab dosing schedules according to pharmacokinetic analysis of data gathered from rituximab treated patients in a tertiary referral nephrology centre between May 2020 and June 2023.

The most common founder pathogenic variant c.868G > A (p.Val290Met) in the gene in a representative adult Czech cohort with focal segmental glomerulosclerosis is associated with a milder disease and its underdiagnosis in childhood.

Background: Genetic focal segmental glomerulosclerosis (FSGS) is caused by pathogenic variants in a broad spectrum of genes that have a variable representation based on subjects' ethnicity and/or age. The most frequently mutated autosomal recessive gene in FSGS is . In this study, we analyzed the spectrum of variants and their associated phenotype in Czech adult FSGS patients.

Clinical Features, Outcomes, and Response to Corticosteroid Treatment of Acute Tubulointerstitial Nephritis: A Single-Centre Retrospective Cohort Study in the Czech Republic.

Introduction: Acute tubulointerstitial nephritis (ATIN) is a well-recognized cause of acute kidney injury (AKI) due to the tubulointerstitial inflammation. The aim of this study was to explore the clinical features, outcomes, and responses to corticosteroid treatment in patients with ATIN.

Methods: Patients with biopsy-proven ATIN, who were diagnosed between 1994 and 2016 at the Department of Nephrology, Charles University, First Faculty of Medicine, and General University Hospital in Prague, were included in the study.

Complete remission in children and adolescents with type 1 diabetes mellitus-prevalence and factors.

Little is known about complete remission in Type 1 diabetes mellitus (T1D) with the discontinuance of insulin treatment for a period of time. In this retrospective study we analysed the frequency and factors of onset and duration of 1. remission and 2.

-Glycoprofiling of SLC35A2-CDG: Patient with a Novel Hemizygous Variant.

Congenital disorders of glycosylation (CDG) are a group of rare inherited metabolic disorders caused by a defect in the process of protein glycosylation. In this work, we present a comprehensive glycoprofile analysis of a male patient with a novel missense variant in the gene, coding a galactose transporter that translocates UDP-galactose from the cytosol to the lumen of the endoplasmic reticulum and Golgi apparatus. Isoelectric focusing of serum transferrin, which resulted in a CDG type II pattern, was followed by structural analysis of transferrin and serum -glycans, as well as the analysis of apolipoprotein CIII -glycans by mass spectrometry.

The Bright Side of Skin Autofluorescence Determination in Children and Adolescents with Newly Diagnosed Type 1 Diabetes Mellitus: A Potential Predictor of Remission?

Skin autofluorescence (SAF) is a noninvasive method reflecting tissue accumulation of advanced glycation end products (AGEs). We investigated whether, in newly diagnosed children and adolescents with type 1 diabetes (T1D), this surrogate marker of long-term glycemia is associated with markers of the early manifestation phase, residual secretion capacity of the ß-cells, and the occurrence of remission. SAF was measured in 114 children and adolescents (age: 8.

Chronic kidney disease and pregnancy outcomes.

Pregnancy complicated by CKD is currently not fully understood topic. Outcome of pregnancy in patients with CKD is related to impaired glomerular filtration rate and the degree of proteinuria. In our study we evaluated the association of serum creatinine level and proteinuria with both maternal and fetal outcomes in the cohort of 84 pregnant patients with CKD.

Renal involvement of a patient with Crohn´s disease: A case report.

Inflammatory bowel disease (IBD) is a group of chronic relapsing intestinal inflammatory processes primarily represented by ulcerative colitis (UC) and Crohn´s disease (CD). Nearly half of IBD cases are followed by extraintestinal complications and renal involvement can occur independatly or along with other complications and are described with the patients sufferring from UC or CD. Most frequent renal involvement is nephrolithiasis, tubulointerstinal nephritis, different kinds of glomerulonephritis and AA amyloidosis.

Increasing incidence of type 1 diabetes mellitus in young children in Slovakia.

Objectives: We aimed to study the prevalence of the early onset Type 1 diabetes in Slovakia during the years 1996-2017.

Background: Prevalence of Type 1 diabetes in young children is increasing worldwide. However, recent data from Slovakia are missing.

Muckle-Wells Syndrome Across Four Generations in One Czech Family: Natural Course of the Disease.

Muckle-Wells syndrome (MWS) represents a moderate phenotype of cryopyrinopathies. Sensorineural hearing loss and AA amyloidosis belong to the most severe manifestations of uncontrolled disease. Simultaneous discovery of MWS in four generations of one large kindred has enabled us to document natural evolution of untreated disease and their response to targeted therapy.

Periodic fevers and other autoinflammatory diseases.

Autoinflammatory diseases represent a relatively new and rapidly evolving group of rare disorders associated with mutations of genes encoding proteins with a key regulatory role in inflammatory response. Gradual discovery of mechanisms that link genetic disorder with its biochemical and immunological consequences leading to continuous or episodic inflammatory stimulation has enabled introduction of directed immunotherapies. Periodic fever syndromes belong to the so far best-known entities: familial Mediterranean fever, mevalonate kinase deficiency, cryopyrinopathies and TNF-receptor associated periodic syndrome.

Increased levels of soluble ST2 in patients with active newly diagnosed ANCA-associated vasculitis.

Objective: ST2, a member of the interleukin-1 receptor family, is selectively expressed on Th2 cells and mediates important Th2 functions. IL-33 is a specific ligand of ST2. The aim of the study was to determine whether serum levels of soluble ST2 (sST2) or IL-33 predict activity of the disease in patients with ANCA-associated vasculitides (AAV).

IgA nephropathy in Czech patients--are we able reliably predict the outcome?

Background/aims: The aim of our study was to retrospectively analyse data of 520 Czech patients with IgA nephropathy (IgAN) and to specify the risk factors affecting renal survival of IgAN patients.

Methods: Cox proportional hazards regression model was used to evaluate the effects of different variables on renal survival during a median follow up of six years. McNemar´s test was used to analyse the progression of renal function according to Bartosik´s formula.

Rituximab use in patients with ANCA-associated vasculitis: clinical efficacy and impact on immunological parameters.

Rituximab (RTX) was reported effective in ANCA-associated vasculitis (AAV). We aimed to evaluate clinical efficacy of RTX in AAV along with its impact on immunological parameters. Eighteen RTX-treated AAV patients (M/F 11/7; median age 37.

Nationwide biopsy survey of renal diseases in the Czech Republic during the years 1994-2011.

Background: We describe data on 10,472 renal biopsies gathered by the Czech Registry of Renal Biopsies over a period of 18 years.

Methods: We assessed the main demographic, clinical and histological data of individuals who underwent renal biopsies of native kidneys in 31 centers in the Czech Republic (population 10.3 million) during the period 1994-2011.

Association of systemic lupus erythematosus with low serum bilirubin levels.

Objective: Systemic lupus erythematosus (SLE) is accompanied by severe oxidative stress. Bilirubin has been reported as a strong negative predictor of oxidative stress-mediated diseases, such as atherosclerosis. The objective of our study was to evaluate the association between serum bilirubin levels and SLE manifestation.

Intracellular cytokine production in ANCA-associated vasculitis: low levels of interleukin-10 in remission are associated with a higher relapse rate in the long-term follow-up.

Background And Aims: Dysregulation of cell-mediated immune response likely plays a role in the pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV), but it has not yet been fully established. The aim of this study was to assess the intracellular cytokine production in patients with AAV at different stages of the disease, in particular, in relation to the long-term prognosis.

Methods: We included 69 patients with AAV and 24 healthy controls.

Renal AA amyloidosis: survey of epidemiologic and laboratory data from one nephrology centre.

Renal amyloid involvement results, especially, from AL (primary) or AA (secondary) amyloidosis. The extent of amyloid tissue deposits in the kidneys and the clinical course of amyloidosis not only depend on the type of basic process but also reflect the time of diagnosis and the ability to affect the underlying disease. We analyzed laboratory and clinical data from patients with bioptically proven renal amyloidosis.

T cells in the pathogenesis of ANCA-associated vasculitis: current knowledge.

AAV are a group of systemic immune-mediated diseases with a strong and highly specific association with ANCA. In recent years, there has been increasing evidence that ANCA might play a direct pathogenic role in triggering AAV. Nevertheless, effectors of cell-mediated immunity prevail in the inflammation sites in patients with AAV.

Prevalence and risk of hypertension in renal disease--data from the Czech Registry of Renal Biopsies.

Background/aims: We intended to compare the risk and prevalence of hypertension in patients undergoing renal biopsy with those of the general population and to investigate the possible effects of various independent factors (age, sex and degree of renal insufficiency) on the prevalence of hypertension.

Methods: Data obtained within the Czech Registry of Renal Biopsies over an 8-year period (1995-2002) were statistically evaluated and compared with those of the general population obtained within the Post-MONICA Study conducted in 2000/2001.

Results: Hypertension was present in 1,839 out of a total of 3,601 renal patients (51.

Mycophenolate mofetil in low doses stabilizes and improves antineutrophil cytoplasmic antibody-associated vasculitis and lupus nephritis.

Background: Clinical experience with mycophenolate mofetil (MMF) in glomerulonephritis still remains limited.

Methods: In order to assess the experience of one center with the efficacy and tolerability of MMF in patients with glomerulonephritis, we performed a retrospective 6-year analysis of 68 patients treated by MMF for glomerular disease, mainly anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV: n=34) and systemic lupus erythematosus and lupus nephritis (SLE: n=24). Indications were maintenance treatment in 40% of patients, induction treatment in patients not tolerating cyclophosphamide in 27%, and disease relapse in 33%.