Nephrotic Syndrome Research Articles

Alport syndrome: Expanding diagnosis and treatment

Alport syndrome (AS) is the second common monogenic cause of end-stage kidney disease (ESKD) worldwide and is caused by defective type 4 collagen due to pathogenic variants of COL4A3, COL4A4, or COL4A5. Type 4 collagen also exists in the eyes and ears, and thus ocular defects and hearing loss occur in AS. The understanding of AS has expanded over the past two decades due to greater availability of genetic testing and research on genotype-phenotype correlation. Patients previously diagnosed with idiopathic steroid resistant nephrotic syndrome or ESKD of unknown etiology may now be diagnosed as AS if pathogenic COL4A3-5 variants are identified. Some carriers of heterozygous COL4A3-5 variants may now be classified into females with X-linked AS or autosomal dominant AS, if there are typical pathologic changes in the glomerular basement membrane or if there is proteinuria and progression of kidney disease. Lastly, it has been recommended that renin-angiotensin-aldosterone system inhibition be started as soon as possible for selected AS patients for its long-term protective effect against kidney function deterioration. The purpose of this review is to introduce these important concepts to general pediatricians and pediatric nephrologists.

Co-localization of IgG with Nephrin in Immune-Mediated Idiopathic Nephrotic Syndrome

Co-localization of IgG with Nephrin in Immune-Mediated Idiopathic Nephrotic Syndrome

Randomized Controlled Trial Comparing Rituximab to Mycophenolate Mofetil in Children and Young Adults with Steroid-Dependent Idiopathic Nephrotic Syndrome

Randomized Controlled Trial Comparing Rituximab to Mycophenolate Mofetil in Children and Young Adults with Steroid-Dependent Idiopathic Nephrotic Syndrome

B Cells Are Essential for Proteinuria Induction in a Mouse Model of Autoimmune Idiopathic Nephrotic Syndrome

B Cells Are Essential for Proteinuria Induction in a Mouse Model of Autoimmune Idiopathic Nephrotic Syndrome

Curcuma longa L. (turmeric) as adjuvant therapy in childhood idiopathic nephrotic syndrome: A double-blind, randomized, placebo-controlled clinical trial

Curcuma longa L. (turmeric) as adjuvant therapy in childhood idiopathic nephrotic syndrome: A double-blind, randomized, placebo-controlled clinical trial

Initial Treatment of Idiopathic Nephrotic Syndrome in Children with Mycophenolate Mofetil vs. Prednisone: A Prospective, Randomized, Controlled, Multicenter, Open, Phase 3, Noninferiority Study

Initial Treatment of Idiopathic Nephrotic Syndrome in Children with Mycophenolate Mofetil vs. Prednisone: A Prospective, Randomized, Controlled, Multicenter, Open, Phase 3, Noninferiority Study

An Increase in TCR/BCR Repertoire Clonality by Prednisolone Therapy and Recovery of the Diversity by Rituximab in Childhood Idiopathic Nephrotic Syndrome

An Increase in TCR/BCR Repertoire Clonality by Prednisolone Therapy and Recovery of the Diversity by Rituximab in Childhood Idiopathic Nephrotic Syndrome

Desialylated IgM by Idiopathic Nephrotic Syndrome Subjects Induce Proteinuria and Podocyte Injury

Desialylated IgM by Idiopathic Nephrotic Syndrome Subjects Induce Proteinuria and Podocyte Injury

Establishing Methods to Predict Responsiveness to Steroid Therapy at Disease Onset of Idiopathic Nephrotic Syndrome in Children

Establishing Methods to Predict Responsiveness to Steroid Therapy at Disease Onset of Idiopathic Nephrotic Syndrome in Children

An international, multi-center study evaluated rituximab therapy in childhood steroid-resistant nephrotic syndrome

The efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS) remains unclear. Therefore, we conducted a retrospective cohort study at 28 pediatric nephrology centers from 19 countries in Asia, Europe, North America and Oceania to evaluate this. Children with SRNS treated with rituximab were analyzed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [6 months or more (CNI-resistant) and under 6 months]. Primary outcome was complete/partial remission (CR/PR) as defined by IPNA/KDIGO guidelines. Secondary outcomes included kidney failure and adverse events. Two-hundred-forty-six children (mean age, 6.9 years; 136 boys; 57% focal segmental glomerulosclerosis, FSGS) were followed a median of 32.4 months after rituximab. All patients were in non-remission before rituximab. (146 and 100 children received CNIs for 6 month or more or under 6 months before rituximab, respectively). In patients with CNI-resistant SRNS, the remission rates (CR/PR) at 3-, 6-, 12- and 24-months were 26% (95% confidence interval 19.3-34.1), 35.6% (28.0-44.0), 35.1% (27.2-43.8) and 39.1% (29.2-49.9), respectively. Twenty-five patients were in PR at 12-months, of which 22 had over 50% reduction in proteinuria from baseline. The remission rates among children treated with CNIs under 6 months before rituximab were 42% (32.3-52.3), 52% (41.8-62.0), 54% (44.3-64.5) and 60% (47.6-71.3) at 3-, 6-, 12-, and 24-months. Upon Kaplan-Meier analysis, non-remission and PR at 12-months after rituximab, compared to CR, were associated with significantly worse kidney survival Adverse events occurred in 30.5% and most were mild. Thus, rituximab enhances remission in a subset of children with SRNS, is generally safe and CR following rituximab is associated with favorable kidney outcome.

Analysis of Peripheral Blood Mononuclear Cells Single-Cell RNA Sequencing Shows IFN Signaling Activation Contributes to the Pathogenesis of Childhood Nephrotic Syndrome

Analysis of Peripheral Blood Mononuclear Cells Single-Cell RNA Sequencing Shows IFN Signaling Activation Contributes to the Pathogenesis of Childhood Nephrotic Syndrome

Role of LDL-Scavenger Receptor CD36-Positive Macrophages in Refractory Nephrotic Syndrome with Hyperlipidemia under High-Dosage Steroid Treatment

Role of LDL-Scavenger Receptor CD36-Positive Macrophages in Refractory Nephrotic Syndrome with Hyperlipidemia under High-Dosage Steroid Treatment

Qualitative Findings for Preparing a Clinical Outcomes Assessment Set (COA) for Nephrotic Syndrome (Prepare-NS)

Qualitative Findings for Preparing a Clinical Outcomes Assessment Set (COA) for Nephrotic Syndrome (Prepare-NS)

Different Dosage Regimens of Rituximab in Primary Membranous Nephropathy Treatment: A Systematic Review.

Primary membranous nephropathy (PMN) is one of the prevalent pathological types of adult primary nephrotic syndrome. Pathogenic autoantibodies targeting podocyte antigens such as phospholipase A2 receptor (PLA2R) lead to the disease. Patients frequently experience notable adverse effects when treated with conventional immunosuppressive therapies. Rituximab (RTX), a mouse/human monoclonal antibody, selectively depletes B cells and leads to a decrease in the antibody levels in the circulation, which helps to alleviate membranous nephropathy. Various RTX dosage regimens have been applied globally in the PMN treatment with satisfactory effects. Nevertheless, the optimal dosage of RTX has yet to be determined. This article reviews the application of different doses of RTX in the management of PMN so far.

Differential Expression of Renal and Hepatic PCSK9 during Development of Hypercholesterolemia in the Passive Heymann Nephritis Rat Model of Nephrotic Syndrome

Differential Expression of Renal and Hepatic PCSK9 during Development of Hypercholesterolemia in the Passive Heymann Nephritis Rat Model of Nephrotic Syndrome

Proteinuria as an Endpoint in Clinical Trials of Focal Segmental Glomerulosclerosis

Focal Segmental Glomerulosclerosis (FSGS) is a characteristic histopathological lesion that is indicative of underlying glomerular dysfunction. It is not a single disease entity but rather a heterogeneous disorder that is an important cause of nephrotic syndrome and kidney failure in children and adults. The aim of this Kidney Health Initiative project was to evaluate potential endpoints for clinical trials in FSGS. This paper focuses on the data supporting proteinuria as a surrogate endpoint. Available data support the use of complete remission of proteinuria in patients with heavy proteinuria as a surrogate endpoint for progression to kidney failure. While substantial treatment effects on proteinuria that are short of a complete remission may also predict the effect of a treatment on progression to kidney failure, further work is needed to determine how such an endpoint should be defined. Fortunately, efforts are underway to bring together patient-level data from randomized controlled trials, observational studies, and registries to address this issue.

Lithium-Induced Nephrotic Syndrome: A Rare Complication of a Known Kidney Offender

Lithium-Induced Nephrotic Syndrome: A Rare Complication of a Known Kidney Offender

A Boy with Steroid-Resistant Nephrotic Syndrome and Cortical Blindness

A Boy with Steroid-Resistant Nephrotic Syndrome and Cortical Blindness

A Novel Pattern of Mutations in Northern Indian Children with Steroid-Resistant Nephrotic Syndrome

A Novel Pattern of Mutations in Northern Indian Children with Steroid-Resistant Nephrotic Syndrome

Kidney Outcomes in APOL1 High-Risk and Mendelian Variant in Patients with Adult Nephrotic Syndrome in Mass General Brigham Biobank

Kidney Outcomes in APOL1 High-Risk and Mendelian Variant in Patients with Adult Nephrotic Syndrome in Mass General Brigham Biobank