Master Protocols: A Promising Approach to Accelerate Drug Development in Rare Kidney Diseases.

Kidney diseases have been a highly challenging area for new drug development because of traditional requirements for reaching doubling of serum creatinine, dialysis, or transplantation endpoints for regulatory approval, which translates into clinical trials needing several years of follow up and large numbers of study participants to achieve adequate power. In recent years, however, progress in surrogate endpoints (specifically proteinuria reduction and slowing of estimated glomerular filtration rate decline in rare glomerular diseases) has resulted in greatly increased interest by biotechnology and pharmaceutical sponsors in investing in these indications.

Exome analysis links kidney malformations to developmental disorders and reveals causal genes.

Congenital anomalies of the kidneys and urinary tract (CAKUT) are developmental disorders that commonly cause pediatric chronic kidney disease and mortality. We examine here rare coding variants in 248 CAKUT trios and 1742 singleton CAKUT cases and compare them to 22,258 controls. Diagnostic and candidate diagnostic variants are detected in 14.

Erratum to "Advancing Understanding of Mitochondrial Function in Diabetic Kidney Disease" [ Volume 10, Issue 2, February 2025, Pages 285-286].

[This corrects the article DOI: 10.1016/j.ekir.

Prospective Study of a Point-of-Care Diagnostic Test for Acute Kidney Injury in a South Asian Hospital.

Introduction: Serum creatinine (SCr) is used to diagnose acute kidney injury (AKI) but has limited utility to distinguish volume depletion from tubular injury. Urine neutrophil gelatinase-associated lipocalin (uNGAL) distinguishes these causes of elevated SCr.

Methods: We tested a novel point-of-care (POC) dipstick to measure uNGAL at presentation at the emergency medical services department (EMSD) in a tertiary care hospital in Puducherry, India.

Environmental exposure assessment in the international prospective study of Chronic Kidney Disease of UnceRtain Etiology (CKDu) in Agricultural Communities (CURE) research consortium: Design and protocol development.

Background: Chronic kidney disease of unknown etiology (CKDu) is a major health concern among outdoor manual workers in rural Central America and South Asia. The CURE study is a prospective longitudinal cohort designed to investigate CKDu's environmental risk factors through standardized exposure assessments, questionnaires, and biological and environmental sample collection.

Methods: This manuscript details the development of a standardized exposure assessment protocol within the CKDu CURE Consortium.

Executive summary of the KDIGO 2025 Clinical Practice Guideline for the Management of Nephrotic Syndrome in Children.

The Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guideline for the Management of Glomerular Diseases was last updated and published in 2021. KDIGO continues to be committed to the nephrology community to provide periodic updates, based on new developments for each of the glomerular diseases. For children with nephrotic syndrome, the updated guideline now contains a treatment algorithm on when to perform a kidney biopsy and/or genetic testing and which immunosuppressive therapy to use in children with a complete response to glucocorticoids (steroid sensitive), who subsequently become infrequent or frequent relapsers or even steroid dependent.

Podocytopathies.

The podocyte is a critical component of the glomerular filtration barrier. Injury to these specialized cells results in podocytopathies. A kidney biopsy will reveal focal segmental glomerulosclerosis or minimal change disease.

The kidney injury biomarker profile of patients with lupus nephritis remains unchanged with the second-generation calcineurin inhibitor voclosporin.

Objectives: Kidney injury in patients with lupus nephritis (LN) results in pro-fibrotic biomarker expression, a manifestation also observed with calcineurin inhibitor (CNI) therapy. The second-generation CNI, voclosporin, is approved in the United States and Europe for the treatment of patients with active LN in combination with background immunosuppression, based on successful outcomes from the global phase 2 AURA-LV and phase 3 AURORA 1 studies, which demonstrated the efficacy of voclosporin across diverse racial and ethnic populations, and encompassing multiple biopsy classes of LN, alongside a favorable safety profile. This analysis examined changes from baseline levels of serum and urinary biomarkers, including pro-fibrotic biomarkers, in a cohort of patients from the parent AURORA 1 study.

Improved Outcomes of Myeloma Cast Nephropathy in Newly Diagnosed Multiple Myeloma With Modern Anti-Myeloma Therapies.

Myeloma cast nephropathy (MCN) is a driver of renal failure in newly diagnosed multiple myeloma (NDMM) and has been historically associated with increased early mortality. Since patients with moderate to severe renal insufficiency are typically excluded from trials, we performed a retrospective study to characterize modern-era outcomes in MCN. We reviewed 274 consecutive NDMM patients from 2017 to 2023 at an academic center and identified 46 patients (16.

Potential Role of Mineralocorticoid Receptor Antagonists in Nondiabetic Chronic Kidney Disease and Glomerular Disease.

Glomerular disease is a leading cause of CKD and ESKD. Although diabetic kidney disease is the most common cause of glomerular disease, nondiabetic causes include malignancy, systemic autoimmune conditions, drug effects, or genetic conditions. Nondiabetic glomerular diseases are rare diseases, with a paucity of high-quality clinical trials in this area.

Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome produced by dysregulated activation of the immune system. Acute kidney injury (AKI) and proteinuria have been infrequently described in the setting of HLH, and investigations of underlying histopathologic changes in the kidney are limited.

Methods: To characterize kidney pathology in HLH, a retrospective review of 30 patients' clinical and laboratory data, and kidney tissue was performed (18 from autopsy, and 12 biopsied patients).

Infection-Related Glomerulonephritis in Children and Adults.

Infection-related glomerulonephritis is an immunologically mediated glomerular injury after an infection. Glomerulonephritis may occur with the infection or after a variable latent period. Poststreptococcal glomerulonephritis (PSGN) is the prototype of infection-related glomerulonephritis.

International Variability in the Epidemiology, Management, and Outcomes of CKD and ESKD: A Systematic Review.

Key Points: There is dramatic global variability in the prevalence of ESKD. Higher health care spending in each country is associated with increased delivery of care for ESKD.

Background: Approaches to treating ESKD may vary internationally on the basis of the availability of care and other factors.