6-year treatment follow-up with an extended-release alkaline formulation (Sibnayal) in primary distal renal tubular acidosis.

Background: Distal renal tubular acidosis (dRTA) is a rare disease characterized by hyperchloremic metabolic acidosis affecting growth, bone and kidney health.

Methods: The aim of B22CS study was to evaluate long-term safety and efficacy (anthropometric/pubertal, tubular damages/kidney function, bone biomarkers, compliance assessments) of Sibnayal, a prolonged-release alkalinizing formulation with twice daily dosing, in children and adults with dRTA. All patients were previously included in the pivotal B21CS study, so were already receiving Sibnayal when included in B22CS open-label follow-up study.

One-Year Outcomes After Belatacept Conversion in Adolescent Kidney Transplant Recipients.

Introduction: Belatacept (CTLA4-Ig) has shown efficacy in adult kidney transplantation (KT) recipients (improved graft and patient survival and reduced donor-specific antibody [DSA]) compared with calcineurin inhibitors (CNIs). Its long-term benefits and monthly i.v.

Management of patients with posterior urethral valves "from the fetus to adolescence": French national diagnostic and care protocol (NDCP).

Posterior urethral valves (PUV) are congenital anomalies characterized by the persistence of mucosal folds in the urethra, leading to various degrees of obstruction. They are the most common cause of lower urinary tract obstruction in fetuses and children, with a severe prognosis, as one-third of affected children develop end-stage renal disease before adulthood. The French National Diagnostic and Care Protocol (NDCP) aim to provide healthcare professionals with guidelines for the optimal diagnostic and therapeutic management of PUV from the fetal stage to adolescence.

Donor-derived Cell-free DNA as a Noninvasive Biomarker of Kidney Allograft Rejection in Pediatric Kidney Transplantation.

Background: Allograft biopsy remains the gold standard to diagnose rejection. New noninvasive biomarkers are needed to avoid unnecessary biopsies and to diagnose early rejection. We studied the performance of donor derived cell-free DNA (dd-cfDNA) to detect rejection in an unselected cohort of pediatric kidney transplant recipients (pKTRs) and determined whether dd-cfDNA could improve standard-of-care monitoring and detection of kidney allograft rejection in children.

Assessment of respiratory function in children after kidney transplantation.

Background: Severe respiratory complications following kidney transplantation have been reported, yet remain poorly understood in the pediatric population. This study aimed to document respiratory disease in this population.

Methods: At annual follow-ups, patients completed a respiratory symptoms questionnaire and underwent pulmonary function tests (PFTs).