Changing Epidemiology and Outcomes of Hemolytic Uremic Syndrome in Children: A Prospective National Cohort Study from the Polish Pediatric HUS Registry and the Polish Registry of Renal Replacement Therapy in Children.

: Hemolytic uremic syndrome (HUS) is a known cause of acute kidney injury in children, but there are few recent reports on its epidemiology and outcome. We aimed to investigate trends in the incidence and the long-term outcomes of both Shiga toxin-producing -HUS (STEC-HUS) and atypical HUS (aHUS) in Poland over the last 12 years (2012-2023), based on the Polish Pediatric HUS and Pediatric Renal Replacement Therapy (RRT) Registries. : A total of 436 patients (301 with STEC-HUS and 135 with aHUS) were included.

Ambulatory blood pressure parameters and their association with albuminuria in adolescents with type 1 diabetes mellitus.

Background: This study aimed to evaluate the blood pressure (BP) status, including arterial stiffness parameters, hemodynamic indicators, circadian profile, and its association with albuminuria in adolescents with type 1 diabetes mellitus (DM1).

Methods: The analysis included 46 patients, with diabetes duration of 7.38 ± 3.

Liquid chromatography-tandem mass spectrometry method for mycophenolic acid and its glucuronide determination in saliva samples from children with nephrotic syndrome.

Background: Saliva sampling is one of the methods of therapeutic drug monitoring for mycophenolic acid (MPA) and its metabolite, mycophenolic acid glucuronide (MPAG). The study describes the liquid chromatography tandem mass spectrometry (LC-MS/MS) method developed for saliva MPA and MPAG determination in children with nephrotic syndrome.

Methods: The mobile phase consisted of methanol and water at gradient flow, both with 0.

High-performance liquid chromatography with fluorescence detection for mycophenolic acid determination in saliva samples.

Background: For therapeutic drug monitoring (TDM) of mycophenolic acid (MPA), which is frequently proposed, saliva might be a suitable and easy-to-obtain biological matrix. The study aimed to validate an HPLC method with fluorescence detection for determining mycophenolic acid in saliva (sMPA) in children with nephrotic syndrome.

Methods: The mobile phase was composed of methanol and tetrabutylammonium bromide with disodium hydrogen phosphate (pH 8.

Limited sampling strategy to predict free mycophenolic acid area under the concentration-time curve in paediatric patients with nephrotic syndrome.

In paediatric patients, there is no data on the recommended area under the concentration-time curve from 0 to 12 h (AUC ) for free mycophenolic acid (fMPA), which is the active form of the drug, responsible for the pharmacological effect. We decided to establish the limited sampling strategy (LSS) for fMPA for its use in MPA therapeutic monitoring in children with nephrotic syndrome treated with mycophenolate mofetil (MMF). This study included 23 children (aged 11 ± 4 years) from whom eight blood samples were collected within 12 h after MMF administration.

Acute kidney injury and diabetic kidney disease in children with acute complications of diabetes.

Background: Diabetic ketoacidosis (DKA) and hyperglycaemia without ketoacidosis are common acute complications of diabetes. Their association with acute kidney injury (AKI) and diabetic kidney disease (DKD) was studied.

Methods: The study group consisted of 197 children with type 1 diabetes with average diabetes duration of 8.

Inosine monophosphate dehydrogenase activity and mycophenolate pharmacokinetics in children with nephrotic syndrome treated with mycophenolate mofetil.

Some studies have shown that the area under the concentration-time curve (AUC) of mycophenolic acid (MPA) should be higher for children with nephrotic syndrome (NS) than after renal transplantation. The pharmacodynamic aspect of MPA, the activity of inosine monophosphate dehydrogenase (IMPDH), has not been studied in children with NS. The study included 21 children (4-16 years old) with NS treated with mycophenolate mofetil.

Chronic kidney disease predictors in obese adolescents.

Background: Glomerular hyperfiltration, initiating development of obesity-related glomerulopathy, results in an enlargement of the glomeruli and unsealing of the filtration barrier. It can be followed by adaptive focal segmental glomerulosclerosis and chronic kidney disease (CKD). The aim of the study was to determine the expression pattern of lipid metabolism and selected kidney damage markers in obese adolescents and to identify potential factors which can predict CKD.

Relationship between Gd-IgA1 and TNFR1 in IgA nephropathy and IgA vasculitis nephritis in children - multicenter study.

Aim Of The Study: To evaluate the relationship between serum Gd-IgA1 (sGd-IgA1) and serum and urine TNFR1 (sTNFR1, uTNFR1) levels as possible prognostic factors in IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN).

Material And Methods: From 299 patients from the Polish Registry of Pediatric IgAN and IgAVN, 60 children (24 IgAN and 36 IgAVN) were included in the study. The control group consisted of 20 healthy children.

The Role of Complement Component C3 Activation in the Clinical Presentation and Prognosis of IgA Nephropathy-A National Study in Children.

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up.

Differences between Obese and Non-Obese Children and Adolescents Regarding Their Oral Status and Blood Markers of Kidney Diseases.

(1) Background: A rarely discussed effect of obesity-related glomerulopathy (ORG) may slowly lead to irreversible glomerular damage and the development of chronic kidney disease. These patients need to undertake medical care, but whether they should be included in intensive oral care is still not mandatory. The study aimed to assess a relationship between renal, metabolic, and oral health indicators among pediatric patients affected by simple obesity.

The Evaluation of Multiple Linear Regression-Based Limited Sampling Strategies for Mycophenolic Acid in Children with Nephrotic Syndrome.

We evaluated mycophenolic acid (MPA) limited sampling strategies (LSSs) established using multiple linear regression (MLR) in children with nephrotic syndrome treated with mycophenolate mofetil (MMF). MLR-LSS is an easy-to-determine approach of therapeutic drug monitoring (TDM). We assessed the practicability of different LSSs for the estimation of MPA exposure as well as the optimal time points for MPA TDM.

Prediction of steroid resistance and steroid dependence in nephrotic syndrome children.

Background: Steroid resistant (SR) nephrotic syndrome (NS) affects up to 30% of children and is responsible for fast progression to end stage renal disease. Currently there is no early prognostic marker of SR and studied candidate variants and parameters differ highly between distinct ethnic cohorts.

Methods: Here, we analyzed 11polymorphic variants, 6 mutations, SOCS3 promoter methylation and biochemical parameters as prognostic markers in a group of 124 Polish NS children (53 steroid resistant, 71 steroid sensitive including 31 steroid dependent) and 55 controls.

Association of Oral Status and Early Primary Hypertension Biomarkers among Children and Adolescents.

The aim of this case-control study was the evaluation of the association between biomarkers of early primary arterial hypertension (HA) and oral diseases among children and adolescents. Material and methods. Subjects suspected of primary HA (n = 180) underwent a complex evaluation of their vascular status: blood pressure, heart rate, vascular stiffness, sympathetic activity in a 24 h ambulatory examination, followed by measurement of serum uric acid (UA), cystatin C, and creatinine.

Dental caries-related primary hypertension in children and adolescents: Cross-sectional study.

Background: Hypertension is a growing clinical problem in pediatric population. Also, the cause of hypertension is usually unknown and it may result from systemic inflammation related to tooth decay.

Aim: To estimate the potential association in cross-sectional study between tooth decay and hypertension in children and adolescents.

The Application of Inosine 5'-Monophosphate Dehydrogenase Activity Determination in Peripheral Blood Mononuclear Cells for Monitoring Mycophenolate Mofetil Therapy in Children with Nephrotic Syndrome.

In pediatric nephrotic syndrome, recommended mycophenolic acid (MPA) pharmacokinetics are higher than those for transplant recipients. In MPA therapeutic monitoring, inosine-5'-monophosphate dehydrogenase (IMPDH) activity may be useful. We modified the method established for renal transplant recipients and determined IMPDH activity in peripheral blood mononuclear cells (PBMCs) from healthy volunteers and children (4-16 years) with nephrotic syndrome treated with mycophenolate mofetil (MMF).

Early kidney damage in diabetic adolescents with increased blood pressure and glomerular hyperfiltration.

Background: The early impact of type 1 diabetes mellitus (DM1), increased blood pressure and glomerular hyperfiltration (GHF) on kidney damage in adolescents using two urinary markers of kidney injury - neutrophil gelatinase-associated lipocalin (uNGAL) and transferrin (uTransf) - was assessed.

Methods: The study group consisted of 80 adolescents with DM1, of whom 42 were patients with increased blood pressure (IBP), and 38 were patients with normal blood pressure (NBP). Blood pressure was assessed by 24-hour ambulatory blood-pressure monitoring.

Comparison of cuff-based and cuffless continuous blood pressure measurements in children and adolescents.

Objective: In recent times, new methods of blood pressure measurements have been introduced, including cuffless blood pressure (BP) measurement device using pulse transit time (PTT) for calculation of BP values. However, it is still unknown how values obtained with a new cuffless device compare with standard ambulatory measurements in children. The main aim of the study was to investigate whether BP values measured by a cuffless PTT device are comparable with measurements by a standard upper arm cuff-based BP device.

Limited sampling strategy to predict mycophenolic acid area under the curve in pediatric patients with nephrotic syndrome: a retrospective cohort study.

Purpose: Limited sampling strategy (LSS) is a precise and relatively convenient therapeutic drug monitoring method. We evaluated LSSs for mycophenolic acid (MPA) in children with nephrotic syndrome treated with mycophenolic mofetil (MMF) and validated the LSSs using two different approaches.

Methods: We measured MPA plasma concentrations in 31 children using HPLC-UV method and received 37 MPA pharmacokinetic profiles (0-12 h).

Multicenter analysis of the efficacy and safety of a non-standard immunosuppressive therapy with rituximab in children with steroid-resistant nephrotic syndrome.

The aim of the study was a multicenter analysis of the efficacy and safety of a non-standard immunosuppressive therapy with rituximab (Rtx) in children with steroid-resistant nephrotic syndrome (SRNS) with particular emphasis on the possibility of permanent discontinuation or dose reduction of other immunosuppressive drugs such as glucocorticoids and cyclosporine A after 6 months of observation. The study group consisted of 30 children with idiopathic nephrotic syndrome, who were unresponsive to standard immunosuppressive treatment, and hospitalized in the years 2010-2017 in eight paediatric nephrology centres in Poland. The children were administered a single initial infusion of rituximab at the dose of 375 mg/m of the body surface area.

Antihypertensive treatment prescription in pediatric dialysis patients in Poland: A comparison between two nationwide studies 2003/2004-2013.

Background: Blood pressure in pediatric dialyzed patients is under poor control.

Objectives: The aim of the study was to assess the strategy and efficacy of antihypertensive drugs used for the treatment of hypertension in pediatric dialyzed patients in 2013 in comparison with the data collected in 2003/2004. The results have been viewed against present strategies of antihypertensive treatment in children.

Continent catheterizable conduits in pediatric urology: One-center experience.

Background: Clean intermittent catheterization (CIC) is a standard treatment for patients who are unable to empty the bladder. In the absence of the urethra or if catheterization through the urethra is problematic, a continent vesicostomy is used as a catheterizable conduit. The Malone procedure is an established treatment option for children with neurogenic constipation and fecal incontinence.

Early Progression of Xanthogranulomatous Pyelonephritis in Children Might Be Dependent on Vimentin Expression.

BACKGROUND Xanthogranulomatous pyelonephritis (XP) is an extremely rare, severe, atypical form of chronic renal parenchymal inflammation accompanied by hydronephrosis and/or urolithiasis. The pathomechanism of XP is not yet fully understood. Microscopically, XP is indicated by the presence of multinucleated giant cells and lipid-laden macrophages, as well as inflammatory infiltration and intensive renal fibrosis.