Insights from ADPedKD, ERKReg and RaDaR registries provide a multi-national perspective on the presentation of childhood autosomal dominant polycystic kidney disease in high- and middle-income countries.

Data on the presentation of Autosomal Dominant Polycystic Kidney Disease (ADPKD) in children have been based on small/regional cohorts and practices regarding both asymptomatic screening in minors and genetic testing differ greatly between countries. To provide a global perspective, we analyzed over 2100 children and adolescents with ADPKD from 32 countries in six World Health Organization regions: 1060 children from the multi-national ADPedKD registry were compared to 269 pediatric patients from the United Kingdom (RaDaR) and 825 from the European Rare Kidney Disease Registry (ERKReg). Asymptomatic family screening was a common mode of presentation (48% in ADPedKD, 62% in ERKReg) with broad international variability (19%-75%), but fairly stable temporal trends in both registries with no correlation to genetic testing.

Resistance to Epoetin-Stimulating Agents in Children Receiving Renal Replacement Therapy.

The incidence of anemia increases with the stage of chronic kidney disease (CKD). Erythropoietin (EPO) deficiency is a common cause of anemia in CKD. Erythropoietin-stimulating agents (ESAs) are the mainstay of the treatment.

Pediatric crush-related acute kidney injury and risk factors: a single center experience.

Background And Objectives: Crush injury, the most important trauma complication encountered in earthquake victims, occurs as a result of prolonged compression of muscle mass. Crush syndrome, resulting from crush injury, and acute kidney injury (AKI) are the most common causes of in-hospital deaths after earthquakes. The aim of this study is to convey our experience after the devastating Turkey-Syria earthquake and to identify the risk factors of crush syndrome and crush-related AKI.

Magnetic resonance imaging based kidney volume assessment for risk stratification in pediatric autosomal dominant polycystic kidney disease.

Introduction: In the pediatric context, most children with autosomal dominant polycystic kidney disease (ADPKD) maintain a normal glomerular filtration rate (GFR) despite underlying structural kidney damage, highlighting the critical need for early intervention and predictive markers. Due to the inverse relationship between kidney volume and kidney function, risk assessments have been presented on the basis of kidney volume. The aim of this study was to use magnetic resonance imaging (MRI)-based kidney volume assessment for risk stratification in pediatric ADPKD and to investigate clinical and genetic differences among risk groups.

Collapsing Glomerulopathy in a Patient with a TRPC6 Mutation Presenting as Rapidly Progressive Glomerulonephritis: A Case Report and Review of the Literature.

Collapsing glomerulopathy (CG) is a proliferative disease characterized by segmental or global wrinkling of the glomerular basement membrane and the formation of pseudocrescents, whereas focal segmental glomerulosclerosis (FSGS) is characterized by podocytopenia, and focal and segmental sclerosis of the glomeruli. Mutations in NPHS1, NPHS2, WT1, PLCE1, CD2AP, ACTN4, and TRPC6 have been reported in steroid-resistant FSGS patients. The mutations p.

Clinical Predictors of Steroid Resistance in Childhood Nephrotic Syndrome.

We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a single tertiary center. One hundred and sixty-two children (57 girls and 105 boys) with NS who were followed between 1998 and 2018 were analyzed in this retrospective cohort. The median (interquartile range; range) age and follow-up time were 4.

Catastrophic antiphospholipid syndrome accompanied by complement regulatory gene mutation.

Background: Antiphospholipid syndrome (APS), particularly the catastrophic antiphospholipid syndrome (CAPS), is one of the rare causes of thrombotic microangiopathy (TMA). CAPS is the most severe form of APS, especially when accompanied by complement dysregulation, causes progressive microvascular thrombosis and failure in multiple organs. In this report, a case of CAPS with TMA accompanied by a genetic defect in the complement system is presented.

Association between serum biomarkers and oral health status in children with chronic kidney disease: A cross-sectional study.

Objectives: The aim of this study was to investigate the association between serum biomarkers and oral health parameters in children with chronic kidney disease (CKD).

Materials And Methods: Serum hemoglobin, blood urea nitrogen, serum creatinine, calcium, parathormone, magnesium, and phosphorus levels were measured in 62 children with CKD aged between 4 and 17 years. Intraoral examinations of the patients were performed by two different pediatric dentists.

Phenotypic and genotypic characteristics of children with Bartter syndrome.

Introduction: Bartter syndrome (BS) is a group of autosomal-recessive tubular disorders and it is classified into five genetic subtypes. BS can also be classified by phenotype (antenatal, classic). Patients with mutations in the same gene can present different phenotypes.

Clinical and genetic characterization of children with cubilin variants.

Background: Cubilin is one of the receptor proteins responsible for reabsorption of albumin in proximal tubules and is encoded by the CUBN gene. We aimed to evaluate clinical and genetic characterization of six patients with proteinuria who had CUBN mutations.

Methods: Patients' characteristics, serum creatinine, albumin, vitamin B levels, urine analysis, spot urine protein/creatinine, microalbumin/creatinine, beta-2 microglobulin/creatinine ratios, estimated glomerular filtration rates (eGFR), treatments, kidney biopsies, and genetic analyses were evaluated.

Molecular Aspects of Distal Kidney Tubular Acidosis in Children, Its Long-Term Outcome, and Relationship with Hyperammonemia.

Objective: We aimed to present the characteristics, genetic analysis results, long-term progno- sis of our patients with distal kidney tubular acidosis, and the relationship between hyperam- monemia and distal kidney tubular acidosis.

Materials And Methods: Biochemical, clinical, and imaging findings were collected at presen- tation and the last clinic visit, and results of the genetic analysis were recorded.

Results: Our study included 9 patients (3 female, 33%).

Comparison of infants and children with urolithiasis: a large case series.

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed.

A nationwide retrospective study in Turkish children with nephrocalcinosis.

Background: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey.

Methods: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010-2019) were included in the study.

Sclerostin and osteoprotegerin: new markers of chronic kidney disease mediated mineral and bone disease in children.

Background Sclerostin and osteoprotegerin (OPG) are new markers of chronic kidney disease (CKD) mediated mineral bone disease (CKD-MBD) which were extensively evaluated in adult population. We aimed to evaluate the associations between serum levels of sclerostin/OPG and parameters of bone turnover and compare the serum levels of sclerostin/OPG in different stages of CKD in children. Methods 70 children with CKD stage 1-5, aged 2-21 years were examined.

Intravesical hyaluronic acid treatment in recurrent urinary tract infections in children with spina bifida and neurogenic bladder.

Introduction: Damage to the glycosaminoglycan layer of the urothelium, which is composed of hyaluronic acid (HA), may increase the possibility of bacterial adherence and infections. Patients with neurogenic bladder (NB) who perform clean intermittent catheterization (CIC) 4-6 times a day are also under great risk for recurrent urinary tract infections (RUTIs).

Objective: The aim of this study was to assess the efficacy and safety of intravesical HA in reducing the frequency of RUTIs in patients with spina bifida (SB) and NB, who perform CIC.

Invasive aspergillosis in a patient with end stage renal disease.

Invasive aspergillosis caused by A. Fumigatus, almost occurs in immunocompromised hosts and has a poor prognosis. We report a case of invasive Aspergillosis in a 15-year-old boy with ESRD.

Changes in Bacterial Resistance Patterns of Pediatric Urinary Tract Infections and Rationale for Empirical Antibiotic Therapy.

Background: The causative agent spectrum and resistance patterns of urinary tract infections in children are affected by many factors.

Aims: To demonstrate antibiotic resistance in urinary tract infections and changing ratio in antibiotic resistance by years.

Study Design: Retrospective cross-sectional study.

The Comparative Effect of Carrot and Lemon Fiber as a Fat Replacer on Physico-chemical, Textural, and Organoleptic Quality of Low-fat Beef Hamburger.

This study was designed to determine the usability of lemon fiber (LF-2%, 4%, 6%) and carrot fiber (CF-2%, 4%, 6%) to produce low-fat beef hamburgers. To that end, a certain amount of fat was replaced with each fiber. The proximate composition, pH value, cholesterol content, cooking characteristics, color, texture profile, and sensory properties of low-fat beef hamburgers were investigated.