KDOQI US Commentary on the KDIGO 2025 Clinical Practice Guideline for the Management of Nephrotic Syndrome in Children.

B Cell Suppression in Primary Glomerular Disease

In childhood nephrotic syndrome, definitions of immunosuppression response, frequent relapses (FRNS), and long-term remission are conflicting and based on limited evidence. Our goal was to define treatment response, FRNS, and long-term remission based on associated disease outcomes. We included children (six months-18 years) diagnosed with nephrotic syndrome between 1993-2023 in combined Canadian cohorts. We evaluated different definitions of 1) immunosuppressive treatment response, 2) FRNS, including Kidney Disease Improving Global Outcomes (KDIGO) 2021 and International Pediatric Nephrology Association (IPNA) 2023 criteria, and 3) long-term remission. Outcomes were time-to-chronic kidney disease (CKD), relapse count throughout follow-up, and time-to-relapse, analyzed by Cox proportional hazards and negative binomial regression. We included 1114 children with nephrotic syndrome (median 3.8-years at diagnosis, 63% male, median 4.7-year follow-up). Of these, 1054 (95%) were steroid-sensitive, 60 (5%) were steroid-resistant (SRNS), and 73% with SRNS achieved complete remission with steroid-sparing immunosuppression. No child with treatment-responsive SRNS developed CKD. Within one-year of diagnosis, 281 steroid-sensitive children (27%) were classified with FRNS by KDIGO and 383 (36%) by IPNA criteria. Children with FRNS by IPNA criteria (vs. KDIGO) had a similar number of relapses (adjusted rate ratio 0.95, 95% confidence interval [CI] 0.81-1.12) and CKD risk (2% each) but less often received steroid-sparing immunosuppression (hazard ratio 0.42, 95% CI 0.32-0.56). Ninety-eight percent of relapses occur within three-years of the initial diagnosis or last relapse event. Children with SRNS that achieve remission have a similar CKD risk as steroid-sensitive children. Children with FRNS by IPNA 2023 and KDIGO 2021 criteria experience similar rates of relapse and CKD. This supports defining treatment resistance by response to any immunosuppressive medication, implementation of the IPNA FRNS criteria, and use of three-year relapse-free survival as a surrogate for long-term remission.

BackgroundNephrotic syndrome is the most common kidney disease in children worldwide. Our aim was to critically appraise the quality of recent Clinical Practice Guidelines (CPGs) for idiopathic steroid-sensitive nephrotic syndrome (SSNS) in children in addition to summarize and compare their recommendations.MethodsSystematic review of CPGs. We identified clinical questions and eligibility criteria and searched and screened for CPGs using bibliographic and CPG databases. Each included CPG was assessed by four independent appraisers using the Appraisal of Guidelines for REsearch & Evaluation II (AGREE-II) instrument. We summarized the recommendations in a comparison practical table.ResultsOur search retrieved 282 citations, of which three CPGs were eligible and appraised: Kidney Disease: Improving Global Outcomes (KDIGO) 2012, Japan Society for Pediatric Nephrology (JSPN) 2014, and American Academy of Pediatrics (AAP) 2009. Among these, the overall assessment of two evidence-based CPGs scored > 70% (KDIGO and JSPN), which was consistent with their higher scores in the six domains of the AGREE II Instrument. In domain 3 (rigor of development), KDIGO, JSPN, and AAP scored 84%, 74%, and 41%, respectively. In domain 5 (applicability), they scored 22%, 16%, and 19%, respectively, and in domain 6 (editorial independence), they scored 94%, 65%, and 88%, respectively.ConclusionsThe methodological quality of the KDIGO CPG was superior, followed by JSPN and AAP CPGs with the relevant recommendations for use in practice.Systematic review registrationThe protocol was registered in the Center for Open Science (OSF) DOI: 10.17605/OSF.IO/6QTMD and in the International prospective register of systematic reviews PROSPERO 2020 CRD42020197511.

Canadian Society of Nephrology Commentary on the 2009 KDIGO Clinical Practice Guideline for the Diagnosis, Evaluation, and Treatment of CKD–Mineral and Bone Disorder (CKD-MBD)

The Dialysis Outcomes and Practice Patterns Study (DOPPS) and the Kidney Disease Outcomes Quality Initiative (K/DOQI): A cooperative initiative to improve outcomes for hemodialysis patients worldwide

A Decade After the KDOQI CKD Guidelines: Impact on CKD Guidelines

Definition and Classification of CKD: The Debate Should Be About Patient Prognosis—A Position Statement From KDOQI and KDIGO

KDOQI US Commentary on the 2012 KDIGO Clinical Practice Guideline for Anemia in CKD

The KDOQI US Commentary on KDIGO Anemia Guideline and Quality of Life

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

An Endorsement of the Removal of Race From GFR Estimation Equations: A Position Statement From the National Kidney Foundation Kidney Disease Outcomes Quality Initiative

The 2012 Kidney Disease: Improving Global Outcomes (KDIGO) clinical practice guideline on glomerulonephritis (GN) is intended to assist the practitioner caring for patients with GN. Two chapters of this guideline focus specifically on nephrotic syndrome in children. Guideline development followed a thorough evidence review, and management recommendations and suggestions were based on the best available evidence. Critical appraisal of the quality of evidence and strength of recommendations followed the Grades of Recommendation Assessment, Development and Evaluation (GRADE) approach. Chapters 3 and 4 of the guideline focus on the management of nephrotic syndrome in children aged 1-18 years. Guideline recommendations for children who have steroid-sensitive nephrotic syndrome (SNSS), defined by their response to corticosteroid therapy with complete remission, are addressed here. Recommendations for those with steroid-resistant nephrotic syndrome (SRNS) (i.e., do not achieve complete remission) are discussed in the companion article. Limitations of the evidence, including the paucity of large-scale randomized controlled trials, are discussed. This article provides a short description of the KDIGO process, the guideline recommendations for treatment of SSNS in children and a brief review of relevant treatment trials related to each recommendation.

Management of idiopathic childhood nephrotic syndrome in sub-Saharan Africa: Ibadan consensus statement

FSGS: Forme Pleine or Forme Fruste

A group of experts from the International Pediatric Nephrology Association (IPNA) has recently updated recommendations for steroid-resistant and steroid-sensitive nephrotic syndrome (SRNS and SSNS) in children [1, 2]. SSNS is now defined as idiopathic nephrotic syndrome [spot urinary protein:creatinine ratio (UPCR) ≥200 mg/mmol and albuminemia <30 g/l] responsive to 4 weeks of corticosteroid therapy with or without a confirmation period of 2 additional weeks (late SSNS). We present a summary of the new definitions and recommendations for diagnosis and management of SSNS in children (Fig. 1). ... ... ... ... ... There is no randomized controlled trial comparing a 4 + 4 to a 6 + 6-week course of treatment for the initial episode of SSNS. Therefore, both are recommended in line with Kidney Disease: Improving Global Outcomes guidelines [2, 4, 5].