Nephrocalcinosis tendency does not worsen under burosumab treatment for X-linked hypophosphatemic rickets: a multicenter pediatric study.

Background: X-linked hypophosphatemic rickets (XLH) is associated with uninhibited FGF23 activity, which leads to phosphaturia, hypophosphatemia and depressed active vitamin D (1,25OH2D) levels. Conventional treatment with phosphate supplements and vitamin D analogs may lead to hypercalciuria (HC), nephrocalcinosis (NC) and hyperparathyroidism. We investigated the effects of burosumab treatment, an anti-FGF23 monoclonal antibody recently approved for XLH, on these complications.

Childhood onset C3 glomerulopathy: recurrence after kidney transplantation-a case series.

Background: C3 Glomerulopathy (C3G) is a complement-mediated disease, with predominant C3 deposits, where pathogenic genetic variants in complement system components and circulating autoantibodies result in loss of control of the alternative pathway, have been described. A high incidence of disease recurrence including graft failure has been reported after kidney transplantation (KTx). Currently treatment modalities for preventing and treating post KTx C3G recurrence (plasma exchange, rituximab and eculizumab) in adults have yielded inconsistent results.

An innocent bystander or a predisposing culprit? Kidney injury following pediatric liver transplantation.

Background: Survival after pediatric liver transplantation has increased dramatically over the years, revealing extra-hepatic complications including impaired kidney function. We conducted a large single-center retrospective study to evaluate kidney outcomes after pediatric liver transplantation.

Methods: From electronic charts of 121 children who underwent liver transplantation during 2007-2020, we collected pre- and post-transplant data.

Isolated Hydronephrosis and Urinary Tract Infection by Two Years of Age: A Population-Based Study.

Objective: To evaluate the risk for urinary tract infection (UTI) in infants with isolated hydronephrosis (IH).

Study Design: A retrospective, population-based study including all infants insured by Clalit Health Services and followed from birth to age 2 years in 3 regions of central Israel. Infants were divided into 3 groups based on electronic medical record diagnoses by age 6 months: (1) control: no urological diagnosis; (2) IH; and (3) complicated urological diagnosis (CUD): any additional nephrological/urological diagnosis with/without HN.

Linear growth of children with X-linked hypophosphatemia treated with burosumab: a real-life observational study.

Unlabelled: To assess the long-term efficacy of burosumab for pediatric patients with X-linked hypophosphatemia, focusing on linear growth. This multi-center retrospective study included 35 pediatric patients who began treatment with burosumab between January 2018 and January 2021. We collected clinical data, anthropometric measurements, laboratory results, and Rickets Severity Score (RSS), from 2 years prior to treatment initiation and up to 4 years after.

Incremental hemodialysis in pediatric patients.

Background: Incremental hemodialysis follows the concept of adjusting dialysis dose according to residual kidney function. Data on incremental hemodialysis in pediatric patients is lacking.

Methods: We conducted a retrospective analysis of children initiating hemodialysis between January 2015 and July 2020 in a single tertiary center, comparing the characteristics and outcomes of those who commenced with incremental hemodialysis vs with conventional thrice-weekly regimen.

[A NEW TRANSITION PROGRAM FOR KIDNEY TRANSPLANTED YOUNG ADULTS FROM PEDIATRIC TO ADULT CARE SERVICES].

Background: Transition from pediatric to adult care services is a complex process, carrying medical, psychosocial and emotional issues. It is known that, during this period, patients may drop-out from follow-up, lose their adherence and suffer from psychosocial complications. This period is prone to rejection of the transplanted kidney.

Serologic response to COVID-19 infection or vaccination in pediatric kidney transplant recipients compared to healthy children.

Background: Differences in serologic response to COVID-19 infection or vaccination were reported in adult kidney transplant recipients (KTR) compared to non-immunocompromised patients. This study aims to compare the serologic response of naturally infected or vaccinated pediatric KTR to that of controls.

Methods: Thirty-eight KTR and 42 healthy children were included; aged ≤18 years, with a previously confirmed COVID-19 infection or post COVID-19 vaccination.

A Clinical Response-Adjusted Steroid Treatment Protocol for Children With Newly Diagnosed Idiopathic Nephrotic Syndrome.

Rationale & Objective: Prednisone protocols for children with idiopathic nephrotic syndrome (INS) are generally similar in dose and duration, despite wide variations in time to response. We assessed the feasibility of a novel clinical treatment protocol characterized by a shorter duration and lower cumulative dose for children with early clinical response.

Study Design: Nonrandomized pilot clinical trial.

[NEPHROTIC SYNDROME RECURRENCE POST-RENAL TRANSPLANTATION: 10 YEARS' EXPERIENCE AT SCHNEIDER CHILDREN'S MEDICAL CENTER IN ISRAEL].

Introduction: A total of 30-50% of pediatric patients presenting with steroid resistant nephrotic syndrome (SRNS) will reach end stage renal disease (ESRD). In patients with primary SRNS, the risk of post-transplant recurrence is around 60% with poor graft outcomes. In the past decade new treatment modalities have emerged in an attempt to improve graft outcomes.

Childhood Hypercalciuric Hypercalcemia With Elevated Vitamin D and Suppressed Parathyroid Hormone: Long-Term Follow Up.

Hypercalcemia with low parathyroid hormone (PTH) level, hypercalciuria, nephrocalcinosis, or nephrolithiasis, was recently reported as caused by mutations in CYP24A1 and SLC34A genes. These encode for vitamin D-24A-hydroxylase and for the renal phosphate transporters NaPiIIa and NaPiIIc, respectively. We aimed to describe the clinical course of these monogenic disorders in patients with and without found mutations during long-term follow-up.

EBV, CMV, and BK viral infections in pediatric kidney transplantation: Frequency, risk factors, treatment, and outcomes.

Background: Improved short- and long-term outcomes of kidney transplantation have been achieved over the past decades due to improved immunosuppression. This may have increased the risk for infections and, particularly, for the viral infections: cytomegalovirus (CMV), Epstein-Barr virus (EBV), and polyoma BK virus (BKV).

Methods: A retrospective review of viremic CMV, EBV, and BKV infections in pediatric renal transplant recipients treated and followed by a national referral center over a 10-year period.

Children with Intestinal Failure Maintain Their Renal Function on Long-Term Parenteral Nutrition.

Background: Long-term parenteral nutrition (PN) has been associated with renal complications, including hypercalciuria, nephrocalcinosis, proteinuria and reduced glomerular filtration rate (GFR). Pediatric data are scarce and mostly short-term. Our study aimed to evaluate renal complications in children with intestinal failure (IF) receiving long-term PN.

Clinical Outcomes and Antibody Response in COVID-19-Positive Pediatric Solid Organ Transplant Recipients.

We describe the clinical and laboratory manifestations and outcomes of 25 pediatric solid organ transplant recipients who tested positive for severe acute respiratory coronavirus-2. Twenty-one (84%) developed a mild disease; 22 of 23 (96%) had a positive serologic response. Two patients (8%), both kidney transplant recipients with additional comorbidities, developed a severe disease.

Serological Response to the BNT162b2 COVID-19 mRNA Vaccine in Adolescent and Young Adult Kidney Transplant Recipients.

Background: Initial reports in adult kidney transplant recipients (KTR) indicate low immunogenicity after 2 doses of the BNT162b2 COVID-19 mRNA vaccine. We describe the immunogenicity of this vaccine compared to the serologic response in naturally infected COVID-19 positive adolescent and young adult KTR.

Methods: For this prospective observational study, the study group included 38 KTR who received 2 doses of the tested vaccine, and the control group included 14 KTR who had a previous polymerase chain reaction-confirmed COVID-19 infection.

Use of Point-of-Care Ultrasound for Evaluation of Extravascular and Intravascular Fluid Status in Pediatric Patients Maintained on Chronic Hemodialysis.

Aims: Traditional methods that use clinical parameters to determine dry weight in hemodialysis patients are inaccurate. This study aimed to compare clinical assessment of fluid status to sonographic parameters of fluid status in pediatric patients undergoing chronic hemodialysis.

Methods: In a prospective observational study, pediatric patients maintained on chronic hemodialysis (ages 2.

Is the prognosis of congenital single functioning kidney benign? A population-based study.

Background: We investigated the risk of kidney injury among adolescents with and without a congenital single functioning kidney (SFK).

Methods: This retrospective study is based on a medical evaluation database of 17-year-old Israeli conscripts, born during 1989-1999. Those with congenital SFK diagnosis, verified by a pediatric nephrologist's review of the original military medical committee classifications, were compared to the rest of the cohort.