Scott E. Wenderfer
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View article: Hypertension, proteinuria, and RAAS inhibition use in children with systemic lupus erythematosus: Data from a multi-institutional pediatric learning health system
Hypertension, proteinuria, and RAAS inhibition use in children with systemic lupus erythematosus: Data from a multi-institutional pediatric learning health system Open
Objectives To assess the potential of a multi-institutional pediatric learning health system for comparative effectiveness research in pediatric-onset systemic lupus erythematosus (SLE), we characterized renin angiotensin aldosterone syste…
View article: Aberrant Glycosylation of <scp>IgG</scp> in Children With Active Lupus Nephritis Alters Podocyte Metabolism and Causes Podocyte Injury
Aberrant Glycosylation of <span>IgG</span> in Children With Active Lupus Nephritis Alters Podocyte Metabolism and Causes Podocyte Injury Open
Objective Podocytes are integral to the maintenance of the glomerular filtration barrier. Their injury results in proteinuria and disease progression in lupus nephritis (LN). Aberrant IgG glycosylation drives podocyte injury in LN and lead…
View article: Genome-wide studies define new genetic mechanisms of IgA vasculitis
Genome-wide studies define new genetic mechanisms of IgA vasculitis Open
IgA vasculitis (IgAV) is a pediatric disease with skin and systemic manifestations. Here, we conducted genome, transcriptome, and proteome-wide association studies in 2,170 IgAV cases and 5,928 controls, generated IgAV-specific maps of gen…
View article: Plasma Lycopene and Lutein Concentrations Are Associated With Reduced Adiposity and Improved Metabolic Health in Healthy Infants
Plasma Lycopene and Lutein Concentrations Are Associated With Reduced Adiposity and Improved Metabolic Health in Healthy Infants Open
Objectives: Excess adiposity and metabolic abnormalities in infancy have been linked to future risk of obesity and cardiometabolic disease. Carotenoids are phytochemicals found in fruits, vegetables, and human milk with established metabol…
View article: Histologic and Clinical Factors Associated with Kidney Outcomes in IgA Vasculitis Nephritis
Histologic and Clinical Factors Associated with Kidney Outcomes in IgA Vasculitis Nephritis Open
Background Nephritis is a common manifestation of IgA vasculitis and is morphologically indistinguishable from IgA nephropathy. While MEST-C scores are predictive of kidney outcomes in IgA nephropathy, their value in IgA vasculitis nephrit…
View article: Renal Response Outcomes of the EuroLupus and National Institutes of Health Cyclophosphamide Dosing Regimens in Childhood‐Onset Proliferative Lupus Nephritis
Renal Response Outcomes of the EuroLupus and National Institutes of Health Cyclophosphamide Dosing Regimens in Childhood‐Onset Proliferative Lupus Nephritis Open
OBJECTIVE: We compared clinical characteristics and renal response in patients with childhood-onset proliferative lupus nephritis (LN) treated with the EuroLupus versus National Institutes of Health (NIH) cyclophosphamide (CYC) regimen. ME…
View article: Development of clinical and laboratory biomarkers in an international cohort of 428 children with lupus nephritis
Development of clinical and laboratory biomarkers in an international cohort of 428 children with lupus nephritis Open
Background There is no consensus on which treatment goals should be achieved to protect kidney function in children with lupus nephritis (LN). Methods We retrospectively analyzed trends of commonly used laboratory biomarkers of 428 patient…
View article: Renal Response Outcomes of the <scp>EuroLupus</scp> and National Institutes of Health Cyclophosphamide Dosing Regimens in <scp>Childhood‐Onset</scp> Proliferative Lupus Nephritis
Renal Response Outcomes of the <span>EuroLupus</span> and National Institutes of Health Cyclophosphamide Dosing Regimens in <span>Childhood‐Onset</span> Proliferative Lupus Nephritis Open
Objective We compared clinical characteristics and renal response in patients with childhood‐onset proliferative lupus nephritis (LN) treated with the EuroLupus versus National Institutes of Health (NIH) cyclophosphamide (CYC) regimen. Met…
View article: Association of COVID-19 Versus COVID-19 Vaccination With Kidney Function and Disease Activity in Primary Glomerular Disease: A Report of the Cure Glomerulonephropathy Study
Association of COVID-19 Versus COVID-19 Vaccination With Kidney Function and Disease Activity in Primary Glomerular Disease: A Report of the Cure Glomerulonephropathy Study Open
View article: Genome-wide association analyses define pathogenic signaling pathways and prioritize drug targets for IgA nephropathy
Genome-wide association analyses define pathogenic signaling pathways and prioritize drug targets for IgA nephropathy Open
View article: Urine biomarker score captures response to induction therapy with lupus nephritis
Urine biomarker score captures response to induction therapy with lupus nephritis Open
View article: Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network
Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network Open
View article: How common is chronic kidney disease in children with lupus nephritis?
How common is chronic kidney disease in children with lupus nephritis? Open
View article: 1115 RAIL biomarkers capture response to induction therapy in pediatric patients during induction therapy
1115 RAIL biomarkers capture response to induction therapy in pediatric patients during induction therapy Open
Background Systemic Lupus Erythematosus (SLE) remains a diagnostic and therapeutic challenge, particularly lupus nephritis (LN). We previously described a composite score, the pediatric Renal Activity Index for Lupus (pRAIL), consisting of…
View article: 1108 Repeat kidney biopsy for lupus nephritis after induction: histologic and clinical responses in childhood-onset SLE
1108 Repeat kidney biopsy for lupus nephritis after induction: histologic and clinical responses in childhood-onset SLE Open
Background In children with proliferative lupus nephritis, response to induction treatment is based on clinical parameters. The added benefit of repeat kidney biopsy in defining therapy response remains unclear. Emerging adult data suggest…
View article: IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome Open
Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. App…
View article: Racial and Ethnic Disparities in Acute Care Utilization Among Patients With Glomerular Disease
Racial and Ethnic Disparities in Acute Care Utilization Among Patients With Glomerular Disease Open
View article: Clinical presentation and management of nephrotic syndrome in the first year of life: A report from the Pediatric Nephrology Research Consortium
Clinical presentation and management of nephrotic syndrome in the first year of life: A report from the Pediatric Nephrology Research Consortium Open
Background and objectives Nephrotic syndrome (NS) in the first year of life is called congenital (CNS) if diagnosed between 0–3 months, or infantile (INS) if diagnosed between 3–12 months of age. The aim of this study was to determine if t…
View article: <scp>Childhood‐Onset</scp> Lupus Nephritis in the Childhood Arthritis and Rheumatology Research Alliance Registry: <scp>Short‐Term</scp> Kidney Status and Variation in Care
<span>Childhood‐Onset</span> Lupus Nephritis in the Childhood Arthritis and Rheumatology Research Alliance Registry: <span>Short‐Term</span> Kidney Status and Variation in Care Open
Objective The goal was to characterize short‐term kidney status and describe variation in early care utilization in a multicenter cohort of patients with childhood‐onset systemic lupus erythematosus (cSLE) and nephritis. Methods We analyze…
View article: Urinary CD163 is a marker of active kidney disease in childhood-onset lupus nephritis
Urinary CD163 is a marker of active kidney disease in childhood-onset lupus nephritis Open
Objective The objective of this study was to evaluate the utility of urine CD163 for detecting disease activity in childhood-onset SLE (cSLE) patients. Methods Sixty consecutive pediatric patients fulfilling four or more ACR criteria for S…
View article: Correction to: Utility of the 2018 revised ISN/RPS thresholds for glomerular crescents in childhood-onset lupus nephritis: a Pediatric Nephrology Research Consortium study
Correction to: Utility of the 2018 revised ISN/RPS thresholds for glomerular crescents in childhood-onset lupus nephritis: a Pediatric Nephrology Research Consortium study Open
View article: Urine ALCAM, PF4 and VCAM-1 Surpass Conventional Metrics in Identifying Nephritis Disease Activity in Childhood-Onset Systemic Lupus Erythematosus
Urine ALCAM, PF4 and VCAM-1 Surpass Conventional Metrics in Identifying Nephritis Disease Activity in Childhood-Onset Systemic Lupus Erythematosus Open
Objectives Serial kidney biopsy for repeat evaluation and monitoring of lupus nephritis (LN) in childhood-onset Systemic Lupus Erythematosus (cSLE) remains challenging, thus non-invasive biomarkers are needed. Here, we evaluate the perform…
View article: Use of renin angiotensin aldosterone system inhibitors in children with lupus and time to glucocorticoid discontinuation
Use of renin angiotensin aldosterone system inhibitors in children with lupus and time to glucocorticoid discontinuation Open
View article: Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus
Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus Open
Childhood-onset systemic lupus erythematosus (cSLE) only represents 20% of all SLE patients, and males with SLE only represent 10%. To study this rare SLE subset, males diagnosed with cSLE over a 30-year period were identified. Organ invol…
View article: Clinical measurement of lupus nephritis activity is inferior to biomarker-based activity assessment using the renal activity index for lupus nephritis in childhood-onset systemic lupus erythematosus
Clinical measurement of lupus nephritis activity is inferior to biomarker-based activity assessment using the renal activity index for lupus nephritis in childhood-onset systemic lupus erythematosus Open
Objectives The renal activity index for lupus (RAIL) measures lupus nephritis (LN) activity considering urine levels of 6 biomarkers (neutrophil gelatinase-associated lipocalin, monocyte chemoattractant protein-1, kidney injury molecule-1,…
View article: Multisite Retrospective Review of Outcomes in Renal Replacement Therapy for Neonates with Inborn Errors of Metabolism
Multisite Retrospective Review of Outcomes in Renal Replacement Therapy for Neonates with Inborn Errors of Metabolism Open
View article: Use of EuroLupus Cyclophosphamide Dosing for the Treatment of Lupus Nephritis in Childhood-onset Systemic Lupus Erythematosus in North America
Use of EuroLupus Cyclophosphamide Dosing for the Treatment of Lupus Nephritis in Childhood-onset Systemic Lupus Erythematosus in North America Open
Objective. Childhood-onset systemic lupus erythematosus (cSLE) has higher rates of lupus nephritis (LN) than adult-onset SLE, often requiring intensive immunosuppression. This study examined North American practices and preferences for the…
View article: GWAS defines pathogenic signaling pathways and prioritizes drug targets for IgA nephropathy
GWAS defines pathogenic signaling pathways and prioritizes drug targets for IgA nephropathy Open
IgA nephropathy (IgAN) is a progressive form of kidney disease defined by glomerular deposition of IgA. We performed a genome-wide association study involving 10,146 kidney biopsy-diagnosed IgAN cases and 28,751 matched controls across 17 …
View article: 503 Developing a standardized steroid dosing regimen in pediatric proliferative lupus nephritis
503 Developing a standardized steroid dosing regimen in pediatric proliferative lupus nephritis Open
Background Corticosteroids (CS) remain the mainstay of therapy for childhood-onset systemic lupus erythematosus (cSLE) although there are no widely accepted dosing strategies of oral (PO CS) or intravenous CS (IV CS). We aimed to (1) devel…
View article: Differences in rituximab use between pediatric rheumatologists and nephrologists for the treatment of refractory lupus nephritis and renal flare in childhood-onset SLE
Differences in rituximab use between pediatric rheumatologists and nephrologists for the treatment of refractory lupus nephritis and renal flare in childhood-onset SLE Open
Background Consensus treatment plans have been developed for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in childhood-onset systemic lupus erythematosus. However, patients who do not respond to initial therapy, …