Assessing masked hypertension and ambulatory arterial stiffness index in children congenital kidney malformations.

Background: Patients diagnosed with congenital kidney malformations are at an increased risk of developing hypertension, proteinuria, and progressing to chronic kidney disease (CKD). The present study aimed to determine the frequency of masked hypertension and ambulatory arterial stiffness index (AASI) in patients with congenital kidney malformations.

Methods: The study included 174 patients with congenital kidney malformations (48 patients with unilateral renal agenesis (URA), 40 patients with ectopic kidney (EK), 36 patients with horseshoe kidney (HK), 31 patients with multicystic dysplastic kidney (MCDK), 19 patients with unilateral renal hypoplasia (URH), and 45 healthy controls.

Retrospective evaluation of acute kidney injury in paediatric COVID-19 patients: a tertiary referral hospital experience.

Background: Coronavirus disease 2019 (COVID-19) has been recognised as a risk factor for acute kidney injury (AKI). Our aim was to investigate the risk factors contributing to hospitalised and outpatient paediatric COVID-19-associated AKI.

Methods: A retrospective observational study was conducted on patients aged 1 month to 18 years with diagnosed COVID-19-associated AKI applied to a tertiary paediatric referral hospital between March 1, 2020 and March 1, 2022.

Complications Related to Childhood Idiopathic Nephrotic Syndrome, Its Treatment and the Associated Risks in Patients.

Aim Nephrotic syndrome is the most common childhood glomerular disorder, but data on the associated complications are limited and predisposing risk factors have not been fully defined. The aim of this study was to evaluate disease- and treatment-related acute and chronic complications in patients with childhood idiopathic nephrotic syndrome (INS), and to identify the risk factors involved in the development of complications. Methods This single-center study was performed at the pediatric nephrology department of a tertiary pediatric hospital in Turkey.

Predictors of rapidly progressive glomerulonephritis in acute poststreptococcal glomerulonephritis.

Background: Acute post-streptococcal glomerulonephritis (APSGN) is an immune-mediated inflammatory respsonse in the kidneys caused by nephritogenic strains of group A β-hemolytic streptococcus (GAS). The present study aimed to present a large patient cohort of APSGN patients to determine the factors that can be used for predicting the prognosis and progression to rapidly progressive glomerulonephritis (RPGN).

Methods: The study included 153 children with APSGN that were seen between January 2010 and January 2022.

The relevance of practical laboratory markers in predicting high-grade vesicoureteral reflux and renal scarring.

Introduction: A high vesicoureteral reflux (VUR) grade is among the specific risk factors for febrile urinary tract infection (febrile UTI) and renal scarring. The aim of this study was to examine the predictive value of some potential hematological parameters for high-grade VUR and renal scarring in children 2 to 24 months old with febrile UTI.

Methods: We retrospectively examined the clinical features, laboratory tests, and imaging studies of 163 children 2 to 24 months old with a diagnosis of febrile UTI.

Determining the effectiveness of the immature granulocyte percentage and systemic immune-inflammation index in predicting acute pyelonephritis.

Aim: The most serious form of urinary tract infection (UTI) is acute pyelonephritis (APN), which can result in bacteremia and renal scarring. This study aims to show the roles that the systemic immune-inflammation index (SII) and the immature granulocyte (IG) percentage play in predicting APN in pediatric patients by comparing them with traditional infection markers. By illustrating a significant relationship between APN, the IG percentage, and the SII, the study's contributions to the differential diagnosis of UTI can promote a rapid and appropriate treatment of APN.

The importance of ambulatory blood pressure monitoring for diagnosing masked hypertension in patients with renal parenchymal scarring.

Background: The most well-known and common long-term complication in children with renal parenchymal scarring (RPS) is hypertension (HT). The present study aimed to evaluate the presence of HT in children with RPS based on ambulatory blood pressure monitoring (ABPM) and to compare the patients' blood pressure (BP) to that in healthy controls matched for age, gender, and BMI.

Methods: The study included 55 patients aged < 18 years diagnosed with RPS who were followed up for ≥ 1 year and 48 healthy controls matched for age, gender, and BMI.

Clinically Different Presentations of Family Members With the Same Homozygote Diacylglycerol Kinase Epsilon Mutation: Case Report.

Membranoproliferative glomerulonephritis and renal microangiopathies may manifest similar clinical presentations and histology. Many genetic mutations that cause these diseases have been reported. Studies on mutations in the gene encoding diacylglycerol kinase epsilon identified a novel pathophysiologic mechanism leading to atypical hemolytic uremic syndrome and/or membranoproliferative glomerulonephritis.

Circulating extracellular vesicles of patients with steroid-sensitive nephrotic syndrome have higher RAC1 and induce recapitulation of nephrotic syndrome phenotype in podocytes.

Since previous research suggests a role of a circulating factor in the pathogenesis of steroid-sensitive nephrotic syndrome (NS), we speculated that circulating plasma extracellular vesicles (EVs) are a candidate source of such a soluble mediator. Here, we aimed to characterize and try to delineate the effects of these EVs in vitro. Plasma EVs from 20 children with steroid-sensitive NS in relapse and remission, 10 healthy controls, and 6 disease controls were obtained by serial ultracentrifugation.

An Assessment on the Effectiveness of the Immature Granulocyte Percentage in Predicting Internal Organ Involvement Among Children With Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) is the most common childhood systemic vasculitis. The present study aims to investigate the effectiveness of the immature granulocyte (IG) percentage as a new marker for predicting internal organ involvement in HSP. This study included 75 patients below 18 years old who were diagnosed with HSP.

Spondylenchondrodysplasia mimicking a systemic lupus erythematosus: A diagnostic challenge in a pediatric patient.

Spondyloenchondrodysplasia (SPENCD) is a rare autosomal recessive skeletal dysplasia caused by biallelic mutations in the ACP5 gene that encodes tartrate-resistant acid phosphatase (TRAP). The extra-osseous phenotype of SPENCD is extremely pleiotropic and is characterized by neurological impairment and immune dysfunction. This phenotype can mimic systemic lupus erythematosus.

Retrospective evaluation of children with unilateral renal agenesis.

Background: Children born with unilateral renal agenesis (URA) are thought to have a risk of developing hypertension, proteinuria, and progressive chronic kidney disease (CKD). The present study aimed to evaluate the long-term prognosis and clinical characteristics of children with URA.

Methods: The study included 171 patients aged < 18 years diagnosed as URA who were followed-up for ≥ 1 year and 121 healthy controls matched for age, gender, and BMI.

Comparison of pediatric patients with noninfectious idiopathic uveitis and noninfectious uveitis associated with an underlying systemic disease: from a referral center in Turkey.

: The aim of this study was to determine the demographic, clinical, treatment, and outcome features of pediatric noninfectious uveitis patients at a Turkish tertiary center.: This retrospective cohort study included 101 pediatric patients with noninfectious uveitis. Location of uveitis, laterality, age at onset of uveitis, complications of uveitis, duration of follow-up, associated systemic diseases, laboratory findings, medications used, and status of uveitis at the time of data collection were obtained from the patients' files.

A case of Type 1 Dent disease presenting with isolated persistent proteinuria.

Dent disease is a rare X-linked recessive tubular disorder, characterized by the triad of low molecular-weight proteinuria, hypercalciuria, nephrocalcinosis and/or nephrolithiasis. It is caused by mutations in the gene or gene. Thirty to 80% of affected males develop end-stage kidney disease between the ages of 30 and 50 years.

Clinicopathological Assessment of Kidney Biopsies in Children with Familial Mediterranean Fever: A Single-Center Experience.

Objectives: Familial Mediterranean fever (FMF) is a monogenic auto-inflammatory disease which might rarely cause glomerulopathy in patients. The aim of this study was to determine the clinical, demographic, and genetic characteristics and type of glomerular lesions in pediatric FMF patients who underwent kidney biopsy.

Methods: The data of 30 pediatric FMF patients with biopsy-proven glomerulopathy were retrospectively reviewed.

Tocilizumab for juvenile idiopathic arthritis: a single-center case series.

Background: Juvenile idiopathic arthritis (JIA) is the commonest chronic rheumatic disease among children. When not treated effectively, JIA can lead to functional disability, due to joint damage, along with long-term morbidities.

Objectives: To describe the use of tocilizumab therapy for 11 patients with polyarticular JIA (pJIA) and systemic JIA (sJIA) who presented inadequate response or were refractory to disease-modifying anti-rheumatic drugs (DMARDs) and/or other biological therapies; and to evaluate its benefits, safety and tolerability.

Similarities between pediatric FMF patients with sacroiliitis and pediatric juvenile spondyloarthropathy patients with sacroiliitis: a preliminary study.

: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent, self-limited attacks of fever with serositis. Acute recurrent arthritis is the most common form of musculoskeletal involvement in FMF; however, ≤5% of FMF patients can develop chronic arthritis, including sacroiliitis. It is difficult to determine if sacroiliitis is a musculoskeletal finding of FMF or if they are concomitant diseases-FMF and juvenile spondyloarthropathy (JSpA).