Nephrology Commons^™

Clinical Presentations, Treatments, And Outcomes Of Pediatric Lupus Nephritis: A Prospective Cohort Study From The Pediatric Nephrology Research Consortium., Melonie Phillips, Mahmoud Kallash, Chloe Tang, Steve Rust, Kia Jubert-Bacon, Scott Wenderfer, Smriti Mohan, Neal Blatt, Don Batisky, Alejandro Quiroga, Tetyana L. Vasylyeva, Nilka Dejesus, Jerome C. Lane

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: There are few prospective studies of mycophenolate mofetil (MMF) versus cyclophosphamide (CYC) for pediatric lupus nephritis (pLN) and none evaluating rituximab (RTX).

METHODS: The Prospective Pediatric Lupus Nephritis Registry (ProPeL-R) enrolled patients <  21 years within 4 weeks of an initial kidney biopsy diagnostic of pLN. Demographic, clinical, and laboratory data were collected prospectively at enrollment, 3 months, 6 months, and then every 6 months thereafter for up to 5 years of follow-up. For this study, we compared patients receiving initial therapy with corticosteroids (CS) and either MMF (n = 33) vs. CYC (n = 18), and those treated with CS, either MMF or CYC, with RTX (n = 20) vs. without RTX (n = 51).

RESULTS: Histology consisted of 18% class III; 35% class IV; 25% mixed class; and 22% pure class V. Eighty-two percent were female. No significant differences in response or infection rates were identified between those receiving MMF or CYC. There was a significant increase in combined complete (CR) and partial response (PR) at 24 months with RTX use. Overall CR rates at 6 and 24 months were 35% …

The Epidemiology Of Kidney And Urinary Tract Infections In Pediatric Chronic Kidney Disease: Prevalence, Risk Factors And Association With Gfr Decline In The Chronic Kidney Disease In Children Study., Derek K. Ng, Andrea R. Molino, Siyao Zhang, Emily J. Stonebrook, Craig S. Wong, Pratyush Gyawali, Kimberly J. Reidy, Susan L. Furth, Bradley A. Warady, Jeffrey J. Fadrowski, Ckid Study Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Urinary tract and kidney infections are potential complications in pediatric chronic kidney disease (CKD), especially among patients with congenital anomalies of the kidney and urinary tract (CAKUT). We characterized the prevalence of self-reported infections from childhood through young adulthood; evaluated risk factors; and quantified the association with CKD progression.

METHODS: Infections in the past year were self-reported at annual Chronic Kidney Disease in Children (CKiD) study visits. Age-specific infection prevalences were stratified by sex and CKD diagnoses (glomerular, non-glomerular non-CAKUT, CAKUT low-risk for infections, and three CAKUT high-risk categories: reflux nephropathy, obstructive uropathy, and other high-risk). Repeated measures logistic …

Peritoneal Dialysis Catheter Complications In Neonates With Kidney Failure., Shannon Reinert, Maanasa Yarlagadda, Alexander Bondoc, Donna Claes, Stefanie Riddle, Meredith P. Schuh

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Neonates with kidney failure require placement of a peritoneal dialysis catheter (PD-C) shortly after birth. PD-C malfunction and peritonitis, both common in this population, are associated with PD failure. The aim of this study was to describe our center's PD-C-related practices and outcomes in neonates with kidney failure.

METHODS: We performed a retrospective chart review of neonates who required kidney replacement therapy (KRT) within the first 30 days of life from 01/2018-12/2023. We evaluated for PD-C duration, PD-C replacement or revision, and peritonitis.

RESULTS: Twenty-four neonates had PD-C placed for chronic dialysis at a median of 9.7 days and …

Association Between Estimated Time With Low Glomerular Filtration Rate And Access To Transplant Among Youth With Advanced Chronic Kidney Disease., Elaine Ku, Timothy Copeland, Charles E. Mcculloch, Jonathan D. Savant, Bradley A. Warady, Susan L. Furth, Sarah Kizilbash, Daniel I. Feig, Roshan P. George, Joseph T. Flynn, Judith Jerry-Fluker, Matthew B. Matheson, Sandra Amaral, Ckid Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Slower chronic kidney disease (CKD) progression allows more time for transplant preparation. Whether differences in CKD progression by race/ethnicity associate with preemptive or living donor transplantation in youth has not been well studied.

METHODS: We examined the association between time spent with low eGFR (between 10-30 mL/min/1.73 m²) and odds of preemptive or living donor transplantation among youth with CKD. eGFR was estimated using the bedside Schwartz (if < 18 years) and CKD-EPI 2021 equations (if ≥ 18 years) and the CKiD U25 equation in sensitivity analysis. Time spent with low eGFR was compared by race/ethnicity.

RESULTS: Among 333 youth with CKD (median age 11 years [IQR 7,14]), median time spent with low eGFR was 28.8 months. 77% were preemptively waitlisted, and 45% received preemptive …

Endothelin Receptor Antagonists In Resistant Hypertension And Proteinuric Kidney Disease: Receptor Strategy And Volume Management, Corey Keith Goldman, Jesse M. Goldman

Division of Nephrology Faculty Papers

This narrative review examines how endothelin receptor antagonists (ERAs) can be used at the intersection of difficult-to-control hypertension, chronic kidney disease (CKD), and albuminuria or proteinuria. For clinicians, the central question is not receptor selectivity alone, but whether a specific agent, indication, dose, background therapy, and monitoring plan can deliver meaningful blood-pressure or kidney benefit without unacceptable fluid retention. Pulmonary arterial hypertension established the pharmacology and major safety liabilities of the class; the current implementation questions for hypertension and kidney specialists arise in systemic hypertension and proteinuric kidney disease. Aprocitentan is the first and only ERA approved for hypertension; in …

Are We Winning At Twinning? Persistent Cni Use In Identical Kidney Transplants., Nicholas S. Herrera, Shivani Wadnerkar, Diane M. Cibrik

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Calcineurin inhibitors (CNIs) revolutionized kidney transplantation by reducing acute rejection rates. Identical twin kidney transplantation is the only setting in which the allograft is immunologically indistinguishable from self, yet CNI use persists. We characterized CNI utilization across end-stage kidney disease etiologies and examined whether CNI exposure was associated with reduced rejection or improved death-censored graft survival (DCGS).

METHODS: We performed a retrospective cohort study using scientific registry of transplant recipients data (January 2000-August 2025). Among 255 adult identical twin kidney transplant recipients, discharge regimens were categorized by CNI use; Firth penalized Cox regression was the primary method for 10-year …

Clinicopathologic Spectrum Of Renal Disease In Sars-Cov-2 Infection And Post-Covid-19 Vaccination, Naya Williams, Mohammed S. Razzaque

School of Medicine Publications

Both SARS-CoV-2 infection and COVID-19 vaccination have been associated with renal complications. In the context of SARS-CoV-2 infection, kidney injury is primarily attributed to a complex interplay of systemic immune dysregulation and vascular damage. Severe infection triggers a cytokine-mediated inflammatory response characterized by elevated levels of pro-inflammatory mediators, resulting in systemic hemodynamic instability, increased capillary permeability, and renal hypoperfusion, ultimately leading to acute tubular injury. In addition, virus-induced endothelial activation promotes a prothrombotic state, microvascular injury, and further impairment of renal perfusion. Collectively, these processes converge to produce acute kidney injury (AKI) and, in severe or prolonged cases, may contribute …

Adult-Onset Iga Vasculitis Initially Attributed To A Cutaneous Drug Reaction And Diverticulitis, Leila C. Tou, Catherine Grace P. Hobayan, Shane Devine, Sarah Koraym

Journal of Community Hospital Internal Medicine Perspectives

Immunoglobulin A vasculitis (IgAV), formerly Henoch–Schönlein purpura, is a small-vessel vasculitis characterized by palpable purpura, arthralgias, gastrointestinal involvement, and renal disease. Although IgAV is common in children, adult-onset disease is rare and may be associated with more severe manifestations, particularly renal involvement. In adults, IgAV may be difficult to recognize because of overlap with more common gastrointestinal, infectious, or medication-related conditions. We present a case of adult-onset IgAV initially attributed to presumed diverticulitis and later to a drug eruption following antibiotic exposure. This case highlights diagnostic pitfalls and the importance of early recognition and renal surveillance.

Bridging Pediatric To Adult Care In Cystinosis: A Scoping Review Of Healthcare Transition Of Care Protocols And A Roadmap For Future Practice., Sanat Subhash, Nicholas Pantelakis, Kyle Backston, Ruhaan Bajpai, Amir Safadi, Brandon Petrovich, Bradley A. Warady, Maria Diaz-Gonzalez De Ferris, Rupesh Raina

Manuscripts, Articles, Book Chapters and Other Papers

Cystinosis is a rare lysosomal storage disorder that affects approximately 1 in 100,000 to 200,000 live births worldwide. Long-term graft success in cystinosis depends on cysteamine adherence and structured multidisciplinary transition, supported by protocols developed by both pediatric nephrologists and hospital organizations. This review explores institutional and regional care models, structured programs such as the RISE protocol and Got Transition's Six Core Elements, and major barriers including adult nephrology expertise, psychosocial stressors, and insurance coverage gaps. Additionally, we explore the impact of digital health tools for their role in supporting cysteamine adherence and maintaining continuity of care. Emerging technologies such …

Facilitating Living Kidney Donation: Retrospective Evaluation Of Weight Loss Interventions To Support Kidney Donation, Julia Dumitrescu, Roslyn Mannon, Scott Westphal, Ashley Brabec, Regina Rau, Amy Olsen, Taylor Cidlik

Graduate Medical Education Research Journal

Background. Living kidney donor transplantation provides recipients with kidney failure with an excellent graft function and survival. Donation of a kidney entails a thorough medical and psychosocial evaluation. Obesity is an increasingly prevalent medical condition and a known risk factor for the development of chronic kidney disease post-donation. Accepting overweight and obese individuals as living kidney donors remains controversial. The purpose of this study was to review the impact of obesity on the determination of candidates for living kidney donation at a single transplant center.

Methods. We performed a retrospective chart review of living kidney donors evaluated over 5 …

Polygenic Risk Scores And Hla Class Ii Variants Are Biomarkers Of Corticosteroid Response In Childhood Nephrotic Syndrome., Tiffany Tu, Alejandro Ochoa, Amika Sood, Ashley Dabrik, Megan Chryst-Stangl, Brandon Lane, Guanghong Wu, Frank Donovan, Ursula Harper, Settara Chandrasekharappa, Christopher Esezobor, Adaobi Solarin, David Hooper, Christine Sethna, Sandra Amaral, Mahmoud Kallash, Michelle Rheault, Priya Verghese, Vikas Dharnidharka, Eloise Salmon, Patricia Weng, Tarak Srivastava, Michael E Seifert, Cozumel Pruette, David Selewski, Keisha Gibson, Tracy Hunley, Asiri Abeyagunawardena, Shenal Thalgahagoda, Arvind Bagga, Aditi Sinha, Nicholas Webb, Larry Greenbaum, Ali Gharavi, Krzysztof Kiryluk, Matthias Kretzler, Lisa Guay-Woodford, Simone Sanna-Cherchi, Agnieszka Bierzynska, Ania Koziell, Gavin Welsh, Moin Saleem, Charles Rotimi, Eileen Chambers, Cliburn Chan, Curegn Consortium, Pnrc Glomerular Disease Group, Cibmtr/Nmdp Consortium, Annette Jackson, Adebowale Adeyemo, Rasheed Gbadegesin

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Nephrotic syndrome (NS), a common glomerular disease in children, is classified based on response to corticosteroid therapy as either steroid-sensitive nephrotic syndrome (SSNS), or steroid-resistant nephrotic syndrome (SRNS). However, there are no current reliable predictors of therapy response at initial clinical presentation.

METHODS: To evaluate predictors, we conducted genome-wide association studies, developed polygenic risk scores (PRS) for therapy response and analyzed classical HLA alleles in 1,997 children (994 discovery and 1,003 replication/validation cohorts) previously unstudied children with NS and 3,558 ancestry-matched control individuals.

RESULTS: A significant association with HLA loci defined by variants in HLA-DQB1, HLA-DRB1, and HLA-DQA1 were …

Target Trial Of Glucagon-Like Peptide-1 Agonists Versus Dipeptidyl Peptidase-4 Inhibitors And Mortality In Hemodialysis, Dustin Le, Duncan Kilpatrick, Walter K. Kraft, Morgan E. Grams, Bernard G. Jaar, Jung-Im Shin

Division of Nephrology Faculty Papers

INTRODUCTION: The comparative effectiveness of glucagon-like peptide-1 (GLP-1) agonists (GLP-1s) versus dipeptidyl peptidase-4 (DPP-4) inhibitors (DPP-4s) in hemodialysis is unknown.

METHODS: Using 2011 to 2021 US Renal Data System and Medicare claims data, we emulated a target trial comparing new users of GLP-1s versus DPP-4s with patients who survived ≥ 90 days on in-center hemodialysis before medication initiation. We used inverse probability of treatment weighting (IPTW) and Cox proportional hazards models to estimate hazard ratios (HRs).

RESULTS: We identified 3671 GLP-1 and 12,039 DPP-4 users. After IPTW, mean age was 63 years, 52% male, 63% White, and mean body mass …

Increasing Medication Adherence In Those With Hypertension, Holly Kimiecik

Evidence-Based Practice Project Reports

Hypertension is a chronic condition characterized by persistently elevated blood pressure and remains a significant contributor to morbidity and mortality in the United States. According to the Centers for Disease Control and Prevention (2024), approximately 47.7% of U.S. adults have hypertension, yet only about one in five achieve blood pressure control below 130/80 mm Hg. Medication adherence plays a critical role in managing hypertension and preventing complications. Adults aged 18 years and older prescribed antihypertensive medications from a primary care clinic in Valparaiso, Indiana, participated in this six-week quality improvement intervention. Evidence from a comprehensive literature review supports combining patient …

Diabetic Nephropathy Masquerading As Systemic Disease: The Critical Role Of Renal Biopsy In A Young Uninsured Woman With Nephrotic Syndrome, Yolanda V. Gutierrez, Alexis Vargas Del Toro, Cristina Kochi Yamamoto, Jose Loayza Pintado, Andres Suarez-Parraga

School of Medicine Publications

Nephrotic syndrome can have overlapping features across multiple diseases, making diagnosis difficult without tissue confirmation. We describe a 30-year-old uninsured Hispanic woman with type 1 diabetes, hypertension, and CKD3B who presented with nephrotic syndrome, sepsis, and progressive muscle weakness. Her pleural effusions, periorbital changes, elevated free light chains, and dysphagia raised concern for systemic conditions such as amyloidosis or polymyositis, especially given inconsistent outpatient follow-up. Because her management would differ significantly depending on the underlying cause and due to the high risk of loss to follow-up, a renal biopsy was pursued. Serologic studies were unremarkable, and biopsy ultimately confirmed diabetic …

Significantly Elevated Procalcitonin In Aki Reliable Or Not Reliable?, Sally Othman, Sujata Kambhatla, Chadi Saad

Conference Presentation Abstracts

Introduction Procalcitonin (PCT) is a biomarker that helps distinguish bacterial infections from other causes of infection or inflammation and can be used as a helpful adjunct to clinical judgment to resolve diagnostic uncertainty. However, the reliability of procalcitonin goes down when a patient has Acute kidney injury (AKI). Yet is there any significance for procalcitonin if it is extremely high, in hundreds, same in our patient, despite the patient in AKI and can PCT still be used to help determine mortality along with all other markers, or independent from other markers. Case report 36 years old male with PMH of …

Device And Patient-Related Complications Of Renal Denervation For Resistant Hypertension: A Post-Marketing Analysis Of The Fda Maude Database, Sai Tharun Reddy Gopannagari, Kesava Manikanta Achuta, Divya Ganisetti, Towfiqul Chowdhury, Chadi Saad

Conference Presentation Abstracts

Background: Renal denervation (RDN) has re-emerged as an adjunctive therapy for resistant hypertension following recent FDA approvals of ultrasound- and radiofrequency-based systems. While contemporary randomized trials report a favorable safety profile, real-world post-marketing data may capture a broader spectrum of complications. Methods: We queried the FDA Manufacturer and User Facility Device Experience (MAUDE) database from inception through December 22, 2025, using product code ‘QYI’ and brand names ‘Paradise’ (Recor Medical) and ‘Symplicity Spyral’ (Medtronic). Reports describing RDN procedures for resistant hypertension were included. Events were manually reviewed and categorized as vascular, device-related, hemodynamic/arrhythmic, neurologic, or other. When multiple events occurred …

Dnajb9-Positive Fibrillary Glomerulonephritis In A Patient With Longstanding Discoid Lupus Erythematosus: A Rare Association, Seyedah Simi Daryabari, Abhishek Vadhar, Sally Othman, Brahmani Tata, Towfiqul Chowdhury, Sujata Kambhatla, Chadi Saad

Conference Presentation Abstracts

Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease characterized by the organized deposition of nonamyloid, randomly arranged fibrils within the mesangium and glomerular basement membrane. While FGN is frequently associated with malignancies and chronic infections, its association with the autoimmune spectrum is emerging as a critical clinical entity. Specifically, the relationship between DNAJB9-positive FGN and discoid lupus erythematosus (DLE) is exceedingly rare, as renal manifestations in lupus patients are typically attributed to immune-complex-mediated lupus nephritis. We present the case of a 67-year-old female with a longstanding history of DLE, manifesting as cutaneous lesions on the extremities and scalp with …

The Dietary Management Of Sodium In Children With Kidney Diseases-Clinical Practice Recommendations From The Pediatric Renal Nutrition Taskforce., José Renken-Terhaerdt, An Desloovere, Michiel Js Oosterveld, Nonnie Polderman, Evelien Snauwaert, Stella Stabouli, Johan Vande Walle, Caroline Anderson, Sheridan Collins, Larry A. Greenbaum, Matthew Harmer, Lyndsay Harshman, Christina L. Nelms, Pearl Pugh, Vanessa Shaw, Jetta Tuokkola, Molly R Wong Vega, Bradley A. Warady, Rukshana Shroff, Fabio Paglialonga

Manuscripts, Articles, Book Chapters and Other Papers

Sodium imbalance is a common concern in children with kidney diseases, presenting as either sodium excess or sodium deficit, each with significant clinical implications. Sodium excess contributes to fluid overload and hypertension, while increased sodium losses, particularly via urine or peritoneal fluid, can predispose patients to hypotension and growth failure. Effective sodium management is thus a critical component of care in pediatric kidney diseases, with dietary sodium intake playing a pivotal role in maintaining homeostasis. The Pediatric Renal Nutrition Taskforce (PRNT) has developed clinical practice recommendations (CPRs) for dietary sodium management in children with kidney diseases, addressing key aspects of …

Regional Variation Of Underlying Kidney Diseases In Children Undergoing Chronic Kidney Replacement Therapy Around The Globe., Dagmara Borzych-Dużałka, Marjolein Bonthuis, Uma Ali, Yok-Chin Yap, Michael Manno, Yihui Zhai, Reyner Loza, Seema Hashmi, Naye Choi, Kenza Soulami, Judith Exantus, Mohamed S. Al Riyami, Sameh Mabrouk, Marbella Ma Angeles, Cristina Zelaya-Camacho, Henny Adriani Puspitasari, Syed Saimul Huque, Francisco Cano, Kar Hui Ng, Sevcan A. Bakkaloğlu, Jerome Harambat, Kitty J. Jager, Bradley A. Warady, Franz Schaefer

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: There is a scarcity of information regarding the distribution of the diseases leading to kidney failure (KF) in children living in the emerging world. We used registry data to provide a global overview of the underlying disease spectrum in children commencing kidney replacement therapy (KRT).

METHODS: We analyzed KF causes among 23,620 children and adolescents commencing maintenance KRT in 80 countries, using data from the IPNA Global KRT Registry (including ESPN/ERA Registry), the International Pediatric Dialysis Network (IPDN), the United States Renal Data System (USRDS), and the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA). The analysis considered …

Ai-Powered Insights In Pediatric Nephrology: Current Applications And Future Opportunities., Arwa Nada, Yamen Ahmed, Jieji Hu, Darcy K. Weidemann, Gregory H. Gorman, Eva Glenn Lecea, Ibrahim A. Sandokji, Stephen Cha, Stella Shin, Salar Bani-Hani, Sai Sudha Mannemuddhu, Rebecca L. Ruebner, Aadil Kakajiwala, Rupesh Raina, Roshan George, Rim Elchaki, Michael L. Moritz, The American Society Of Pediatric Nephrology Quality Improvement And Artificial Intelligence (Aspn Qi/Ai) Interest Group

Manuscripts, Articles, Book Chapters and Other Papers

Artificial intelligence (AI) is rapidly emerging as a transformative force in pediatric nephrology, enabling improvements in diagnostic accuracy, therapeutic precision, and operational workflows. By integrating diverse datasets-including patient histories, genomics, imaging, and longitudinal clinical records-AI-driven tools can detect subtle kidney anomalies, predict acute kidney injury, and forecast disease progression. Deep learning models, for instance, have demonstrated the potential to enhance ultrasound interpretations, refine kidney biopsy assessments, and streamline pathology evaluations. Coupled with robust decision support systems, these innovations also optimize medication dosing and dialysis regimens, ultimately improving patient outcomes. AI-powered chatbots hold promise for improving patient engagement and adherence, while …