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% …

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 …

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 …

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

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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 …

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

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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 …

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

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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 …

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 …

Ten Tips On How To Optimize Nutrition In Children With Chronic Kidney Disease Or On Dialysis., Nonnie Polderman, Vanessa Shaw, Caroline E. Anderson, An Desloovere, Louise Mcalister, Christina L. Nelms, Fabio Paglialonga, Pearl Pugh, Leila Qizalbash, Evelien Snauwaert, José Renken-Terhaerdt, Jetta Tuokkola, Larry A. Greenbaum, Rukshana Shroff

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Optimal nutrition is central to growth and development in all children, and is particularly relevant in those with chronic illnesses. This document provides 10 practical, evidence-informed tips on the assessment and management of the nutritional prescription in children with chronic kidney disease (CKD) and those receiving dialysis. The 10 tips synthesize work of the Paediatric Renal Nutrition Taskforce, an international team of pediatric renal dietitians and pediatric nephrologists who develop clinical practice recommendations for the nutritional management of children with kidney diseases. We describe a comprehensive, three-dimensional nutritional assessment (anthropometry, dietary intake, biochemical markers) and regular monitoring tailored to age, …

Glomerular Transcriptome Analysis Reveals Endothelial Disturbances In Patients With Idiopathic Nephrotic Syndrome., Sarah K. Nelson-Taylor, Jonathan Troost, Courtney Giannini, Colin Bauer, Tarak Srivastava, Jarcy Zee, Markus Bitzer, Laura Barisoni, Llse Daehn, Julie A. Dougherty, William E. Smoyer, Bryce A. Kerlin, Audrey Fetsko, Imtiazul Islam, Xin Wang, Christine Sethna, Richard J. Johnson, Carmen De Lucas Collantes, Kazunari Kaneko, Gabriel Cara-Fuentes

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE & OBJECTIVE: Idiopathic nephrotic syndrome (INS) is viewed as a podocyte-specific disease. Recent reports indicate endothelial involvement, but its significance is unclear. Here, we investigated the relationship between the glomerular expression of selected genes relevant to endothelial health and clinical markers of disease severity.

STUDY DESIGN: A cross-sectional study.

SETTING & PARTICIPANTS: Patients with INS (n = 70 minimal change disease and n = 83 focal segmental glomerulosclerosis) from the Nephrotic Syndrome Study Network cohort study and 53 control participants. Validation studies, including animal and cell culture experiments, were performed.

EXPOSURE: Gene expression analysis from micro-dissected human glomeruli. The …

Association Of N-Terminal Pro-Brain Natriuretic Peptide With Ckd Progression In The Ckd In Children Study., Alexander Kula, David K. Prince, Joseph T. Flynn, Susan L. Furth, Bradley A. Warady, Tamara Isakova, Derek Ng, Jeese Seegmiller, Rob Christenson, Nisha Bansal

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Optimizing Kidney Allograft Years: Underutilization Of Pediatric Donors, Nicholas Herrera, Ana Cortez, Diane Cibrik, Bradley A. Warady

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Background. 

The Kidney Donor Profile Index (KDPI) score drives kidney allocation in the United States for both adult and pediatric donors, despite the calculation being primarily derived from adult donor data. A KDPI of ≤20% is considered to have the longest graft survival potential and is typically prioritized for recipients who have a low Estimated Posttransplant Survival (EPTS) score. Using KDPI to assess pediatric donor quality may underestimate graft survival and result in underutilization of these kidneys for recipients with a low EPTS score.

Methods. 

Using Scientific Registry of Transplant Recipients data, we analyzed 5-y posttransplant death-censored graft survival of …

Metabolic Acidosis And Cardiovascular Disease Risk In Children With Ckd., Denver D. Brown, Jennifer Roem, Kimberly Reidy, Susan Furth, Bradley A. Warady, Michal Melamed, Tammy Brady, Ckid Study Investigators

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KEY POINTS: Metabolic acidosis may be associated with adverse cardiovascular disease risk. Prospective studies of the benefit of alkali therapy on cardiovascular disease risk are needed.

BACKGROUND: The link between metabolic acidosis (MA) and cardiovascular disease (CVD) outcomes has been studied in adults but not in children with CKD where MA is a common and early finding. Using longitudinal BP and cardiac measurement data, we evaluated whether low serum bicarbonate (a surrogate for MA) is associated with adverse CVD risk.

METHODS: This study used data from children age 1 year and older with eGFRs between 30 and 90 ml/min per …

Assessment And Management Of Magnesium And Trace Element Status In Children With Ckd Stages 2-5, On Dialysis And Post-Transplantation: Clinical Practice Points From The Pediatric Renal Nutrition Taskforce., Jetta Tuokkola, Caroline E. Anderson, Sheridan Collins, Pearl Pugh, Molly R Wong Vega, Matthew Harmer, Lyndsay A. Harshman, Christina L. Nelms, Barry Toole, An Desloovere, Fabio Paglialonga, Nonnie Polderman, José Renken-Terhaerdt, Rukshana Shroff, Evelien Snauwaert, Stella Stabouli, Johan Vande Walle, Bradley A. Warady, Vanessa Shaw, Larry A. Greenbaum

Manuscripts, Articles, Book Chapters and Other Papers

Children and young people with chronic kidney disease (CKD) are at risk for deficiency or excess of magnesium and trace elements. Kidney function, dialysis, medication, and dietary and supplemental intake can affect their biochemical status. There is much uncertainty about the requirements of magnesium and trace elements in CKD, which leads to variation in practice. The Pediatric Renal Nutrition Taskforce is an international team of pediatric kidney dietitians and pediatric nephrologists, formed to develop evidence-based clinical practice points to improve the nutritional care of children with CKD. PICO (patient, intervention, comparator, and outcomes) questions led the literature searches, which were …

Serum Syndecan-1 Is Associated With Kidney And Cardiovascular Outcomes In Idiopathic Nephrotic Syndrome., Colin Bauer, Arshia Anand, Christine Sethna, Jonathan Troost, Tarak Srivastava, Imtiazul Islam, Audrey Fetsko, Julie A. Dougherty, William E. Smoyer, Richard J. Johnson, Gabriel Cara-Fuentes

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Idiopathic nephrotic syndrome (INS) is associated with important kidney and cardiovascular morbidities. We tested the hypothesis that serum syndecan-1, a marker of endothelial glycocalyx injury, can help identify patients at risk for unfavorable kidney and cardiovascular outcomes.

METHODS: We included 348 children and adults with INS (n = 35 unbiopsied, n = 141 minimal change disease [MCD] and n = 172 focal segmental glomerulosclerosis [FSGS]) from the Nephrotic Syndrome Study Network (NEPTUNE) cohort and 34 healthy participants (n = 22 adults, n = 12 children). We measured baseline serum syndecan-1 levels using an enzyme-linked immunosorbent assay and …

Effectiveness Of Allopurinol On Uric Acid And Pediatric Chronic Kidney Disease Severity In The Chronic Kidney Disease In Children Study., Derek K. Ng, Matthew B. Matheson, George J. Schwartz, Katherine E. Kurgansky, Bradley A. Warady, Susan L. Furth

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE & OBJECTIVE: Clinical trials have shown that serum uric acid reduction does not slow chronic kidney disease (CKD) progression in adults, but it is uncertain whether these findings apply to children.

STUDY DESIGN: An observational cohort study.

SETTING & POPULATION: The Chronic Kidney Disease in Children cohort with participants who initiated allopurinol with a comparison group matched on age, sex, uric acid, CKD diagnosis, estimated glomerular filtration rate (eGFR), and proteinuria.

EXPOSURE: Allopurinol initiation.

OUTCOMES: Uric acid, eGFR, and proteinuria before and after initiation, and longitudinal changes over time.

ANALYTICAL APPROACH: Allopurinol initiators were matched to noninitiators at a …

Cardiovascular And Thromboembolic Events In Children And Adults With Glomerular Disease: Findings From The Cure Glomerulonephropathy (Curegn) Network., Shikha Wadhwani, Sarah A. Mansfield, Abigail R. Smith, Bruce M. Robinson, Eman Abdelghani, Amira Al-Uzri, Isa F. Ashoor, Sharon M. Bartosh, Aftab S. Chishti, Salim S. Hayek, Michelle A. Hladunewich, Bryce A. Kerlin, Siddharth S. Madapoosi, Laura H. Mariani, Amy K. Mottl, Michelle N. Rheault, Michelle M. O'Shaughnessy, Christopher John Sperati, Tarak Srivastava, David T. Selewski, Chia-Shi Wang, Craig S. Wong, Donald J. Weaver, Myda Khalid, Glomerulonephropathy (Curegn) Study Consortium

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE & OBJECTIVE: Cardiovascular (CV) and thromboembolic (TE) events are known complications of glomerular disease (GD), but their incidence and risk factors are poorly characterized. This analysis describes CV and TE outcomes in the Cure GlomeruloNephropathy (CureGN) Network.

STUDY DESIGN: Prospective cohort study.

SETTING & PARTICIPANTS: CureGN is a prospective cohort study of children and adults with biopsy-proven minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), or IgA nephropathy (IgAN)/vasculitis (IgAV). Data from 2,545 children and adults (23% MCD, 23% MN, 25% FSGS, 29% IgAN/IgAV) was analyzed.

EXPOSURE: Estimated glomerular filtration rate (eGFR), proteinuria, serum albumin, tobacco …

Clinical Decision-Making About Immunosuppressive Treatment In Focal Segmental Glomerulosclerosis., Brooke Blazius, Jonathan P. Troost, Jeffrey B. Kopp, Rulan S. Parekh, Brenda Gillespie, Isabelle Ayoub, Mahmoud Kallash, Rasheed Gbadegesin, Pietro A. Canetta, Tarak Srivastava, Tracy E. Hunley, Katherine E. Twombley, Yonatan A. Peleg, Larry A. Greenbaum, Aftab S. Chishti, Carla M. Nester, Amy K. Mottl, Susan L. Hogan, Virginie Royal, Vivette D. D'Agati, Kimberly J. Reidy, Krzysztof Mucha, Julia Steinke, Simone Sanna-Cherchi, David T. Selewski, Dhruti P. Chen, C John Sperati, Ronald J. Falk, Howard Trachtman, Jason M. Kidd, Cure Glomerulonephropathy (Curegn) Study Consortium

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE & OBJECTIVE: Focal segmental glomerulosclerosis (FSGS) is a heterogeneous disorder with a high risk of progression to kidney failure. There are no approved therapies for FSGS, and futility of treatment is poorly defined. The Cure Glomerulonephropathy (CureGN) study offers the opportunity to describe the characteristics of participants who started immunosuppressive therapy (IST), never received IST, or in whom this treatment was discontinued.

STUDY DESIGN: An observational cohort.

SETTINGS & PARTICIPANTS: Participants enrolled in CureGN with FSGS and surveyed nephrologists.

INTERVENTIONS: The clinical and laboratory data from participants with FSGS who were enrolled in the CureGN observational cohort were reviewed …

Developing And Implementing Pediatric Nephrology Milestones 2.0 As An Efficient Tool For Trainee Evaluation And Just-In-Time Feedback., Caroline V. Jackson, Rebecca Hjorten, Laura Edgar, Beatrice Goilav, Roshan P. George, Darcy K. Weidemann

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In 2013, the Accreditation Council for Graduate Medical Education (ACGME) implemented Milestones 1.0 as a tool to assess trainee progress towards readiness for independent practice. Critiques of Milestones 1.0 suggested its complexity made the tool difficult to quickly understand and implement in a standardized fashion. This was particularly challenging among subspecialties due to inherent differences in clinical practice settings and make-up of procedural and patient care needs. In response, ACGME launched Milestones 2.0 in 2016 to harmonize competencies applicable to all subspecialties and develop new subspecialty specific competencies to facilitate precise feedback on subspecialty specific content domains. We describe how …

Cinacalcet For Infants And Young Children On Maintenance Dialysis: Determining The Right Time, The Right Dose And The Right Patients., Kyle Ying-Kit Lin, Fiona Fung-Yee Lai, Eugene Yu-Hin Chan, Bradley A. Warady

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Chronic Kidney Disease - Mineral Bone Disorder (CKD-MBD) is a recognized complication of kidney failure, which can lead to short stature, bone deformity, slipped capital femoral epiphysis, and bone fracture in children. Despite the use of conventional therapies, a subgroup of patients receiving dialysis continues to experience secondary or even tertiary hyperparathyroidism. Cinacalcet, a calcimimetic agent, has been shown to be a promising therapeutic option to control hyperparathyroidism with reasonable safety profiles in adults and older children. Nevertheless, there is a paucity of data and guidance pertaining to its use among the younger children on dialysis, who are often the …

Covid-19 Associated Worry And Emotional Distress In Youth And Young Adults With Chronic Kidney Disease., Joann Carlson, Erin M. Jones, Sharon Manne, Matthew B. Matheson, Lyndsay A. Harshman, Bradley A. Warady, Susan Furth, Stephen R. Hooper, Rebecca J. Johnson

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: The COVID-19 (C19) pandemic impacted youth mental/behavioral health with a 25% increase in prevalence of anxiety and depression worldwide. Little is known about the emotional impact of C19 for children with Chronic Kidney Disease (CKD). The purpose of the current study is to describe C19-related distress and worry among youth with CKD.

METHODS: C19 questionnaire adapted from the SARS-CoV-19 worry scale was completed for participants. Covariate data were collected at or prior to C19 questionnaire administration.Analyses examined rates of C19 emotional distress and worry and included logistic regression to identify associations with socioeconomic and disease-related covariates.

RESULTS: The sample …

The Management Of Dietary Fiber Intake In Children With Chronic Kidney Disease - Clinical Practice Recommendations From The Pediatric Renal Nutrition Taskforce., An Desloovere, Nonnie Polderman, José Renken-Terhaerdt, Vanessa Shaw, Caroline Anderson, Larry A. Greenbaum, Christina L. Nelms, Leila Qizalbash, Stella Stabouli, Jetta Tuokkola, Bradley A. Warady, Johan Vande Walle, Fabio Paglialonga, Rukshana Shroff, Evelien Snauwaert

Manuscripts, Articles, Book Chapters and Other Papers

The benefits of dietary fiber are widely accepted. Nevertheless, a substantial proportion of children fail to meet the recommended intake of dietary fiber. Achieving adequate fiber intake is especially challenging in children with chronic kidney disease (CKD). An international team of pediatric renal dietitians and pediatric nephrologists from the Pediatric Renal Nutrition Taskforce (PRNT) has developed clinical practice recommendations (CPRs) for the dietary intake of fiber in children and adolescents with CKD. In this CPR paper, we propose a definition of fiber, provide advice on the requirements and assessment of fiber intake, and offer practical guidance on optimizing dietary fiber …

Nutritional Management Of The Child With Chronic Kidney Disease And On Dialysis., Vanessa Shaw, Caroline Anderson, An Desloovere, Larry A. Greenbaum, Lyndsay Harshman, Christina L. Nelms, Pearl Pugh, Nonnie Polderman, José Renken-Terhaerdt, Evelien Snauwaert, Stella Stabouli, Jetta Tuokkola, Johan Vande Walle, Bradley A. Warady, Fabio Paglialonga, Rukshana Shroff

Manuscripts, Articles, Book Chapters and Other Papers

While it is widely accepted that the nutritional management of the infant with chronic kidney disease (CKD) is paramount to achieve normal growth and development, nutritional management is also of importance beyond 1 year of age, particularly in toddlers, to support the delayed infantile stage of growth that may extend to 2-3 years of age. Puberty is also a vulnerable period when nutritional needs are higher to support the expected growth spurt. Inadequate nutritional intake throughout childhood can result in failure to achieve full adult height potential, and there is an increased risk for abnormal neurodevelopment. Conversely, the rising prevalence …

Emerging Role Of Sh3bp2 As Regulator Of Immune And Nonimmune Cells In Nephrotic Syndrome., Tarak Srivastava, Mukut Sharma

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BACKGROUND: Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are major forms of nephrotic syndrome that remain difficult to treat. MCD and FSGS have distinct but also overlapping clinical, histological, metabolic, and molecular features. Effective use of immunosuppressive drugs, activated immune cells, altered cytokine profiles, and upregulated signaling pathways suggest a link between immune dysfunction and nephrotic syndrome, but the exact mechanism of immunopathogenesis is unclear. Immune dysfunction is an area of ongoing research for identifying novel molecular targets for treating nephrotic syndrome. However, the available animal models do not directly address the role of immune dysfunction in nephrotic …

Responding To The Workforce Crisis: Consensus Recommendations From The Second Workforce Summit Of The American Society Of Pediatric Nephrology., Danielle E. Soranno, Sandra Amaral, Isa Ashoor, Meredith A. Atkinson, Gina-Marie Barletta, Michael C. Braun, Joann Carlson, Caitlin Carter, Annabelle Chua, Vikas R. Dharnidharka, Keri Drake, Elif Erkan, Dan Feig, Stuart L. Goldstein, David Hains, Lyndsay A. Harshman, Elizabeth Ingulli, Alexander J. Kula, Mary Leonard, Sudha Mannemuddhu, Shina Menon, Zubin J. Modi, Marva Moxey-Mims, Arwa Nada, Victoria Norwood, Michelle C. Starr, Priya S. Verghese, Darcy K. Weidemann, Adam Weinstein, Jodi Smith

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IMPORTANCE: Pediatric patients with complex medical problems benefit from pediatric sub-specialty care; however, a significant proportion of children live greater than 80 mi. away from pediatric sub-specialty care.

OBJECTIVE: To identify current knowledge gaps and outline concrete next steps to make progress on issues that have persistently challenged the pediatric nephrology workforce.

EVIDENCE REVIEW: Workforce Summit 2.0 employed the round table format and methodology for consensus building using adapted Delphi principles. Content domains were identified via input from the ASPN Workforce Committee, the ASPN's 2023 Strategic Plan survey, the ASPN's Pediatric Nephrology Division Directors survey, and ongoing feedback from ASPN …

Assessment And Management Of Vitamin Status In Children With Ckd Stages 2-5, On Dialysis And Post-Transplantation: Clinical Practice Points From The Pediatric Renal Nutrition Taskforce., Caroline E. Anderson, Jetta Tuokkola, Leila Qizalbash, Matthew Harmer, Christina L. Nelms, Stella Stabouli, Barry Toole, Nonnie Polderman, An Desloovere, Jose Renken-Terhaerdt, Molly R Wong Vega, Evelien Snauwaert, Johan Vande Walle, Dieter Haffner, Fabio Paglialonga, Rukshana Shroff, Vanessa Shaw, Larry A. Greenbaum, Bradley A. Warady

Manuscripts, Articles, Book Chapters and Other Papers

Children with chronic kidney disease (CKD) are at risk for vitamin deficiency or excess. Vitamin status can be affected by diet, supplements, kidney function, medications, and dialysis. Little is known about vitamin requirements in CKD, leading to practice variation.The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric kidney dietitians and pediatric nephrologists, was established to develop evidence-based clinical practice points (CPPs) to address challenges and to serve as a resource for nutritional care. Questions were formulated using PICO (Patient, Intervention, Comparator, Outcomes), and literature searches undertaken to explore clinical practice from assessment to management of vitamin status in …

Vitamin D And Metabolic Bone Disease In Prolonged Continuous Kidney Replacement Therapy: A Prospective Observational Study., Peace Dorothy Imani, Molly Vega, Naile Tufan Pekkucuksen, Poyyapakkam Srivaths, Ayse Akcan Arikan

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BACKGROUND: Complications of prolonged continuous kidney replacement therapy (CKRT) have not been well described. Our objective was to describe mineral metabolism and bone findings in children who required prolonged CKRT.

METHODS: In this single center prospective observational study, we enrolled 37 patients who required CKRT for ≥ 28 days with regional citrate anticoagulation. Exposure was duration on CKRT and outcomes were 25-hydroxy vitamin D and osteopenia and/or fractures.

RESULTS: The prevalence of vitamin D deficiency and insufficiency was 17.2% and 69.0%, respectively. 29.7% of patients had radiographic findings of osteopenia and/or fractures. There was no association between vitamin D deficiency …

Use Of Clearguard Hd Caps In Pediatric Hemodialysis Patients., Amy E. Nau, Troy Richardson, Diana Cardwell, Jennifer Ehrlich, Jyothsna Gattineni, Melisha Hanna, Mahima Keswani, Emily Neibauer, Kelly Nitz, Raymond Quigley, Michelle Rheault, Rebekah Sims, Mayna Woo, Bradley A. Warady

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BACKGROUND: Bloodstream infections (BSIs) are a leading cause of hospitalizations and mortality among patients receiving hemodialysis (HD) therapy, especially those with a central venous catheter (CVC) for dialysis access. The use of chlorhexidine impregnated catheter caps (ClearGuard) has been associated with a decrease in the rate of HD catheter-related BSIs (CA-BSIs) in adults; similar data have not been published for children.

METHODS: We compared CA-BSI data from participating centers within the Standardizing Care to Improve Outcomes in Pediatric Endstage Kidney Disease (SCOPE) collaborative based on the center's use of ClearGuard caps for patients with HD catheter access. Centers were characterized …

Psychotropic Medication Usage In Pediatric Ckd: Reporting From The Ckd In Children Cohort., Ryan C. Ward, Amy J. Kogon, Matthew B. Matheson, Anne Dawson, Stephen R. Hooper, Stephen Molitor, Cynthia Wong, Susan L. Furth, Bradley A. Warady, Lyndsay A. Harshman

Manuscripts, Articles, Book Chapters and Other Papers

Background: Mental health disorders within the pediatric CKD population are prevalent. The frequency is unknown with which psychotropic medications that commonly treat these conditions are used in this population.

Methods: Data from the CKD in Children (CKiD) cohort study were used to describe the use of psychotropic medications and patient-related characteristics of use. Medications were classified into three groups: antidepressants, central nervous system (CNS) stimulants, and antipsychotic/mood stabilizing medications. Participant age, sex, CKD severity, and duration of medication use were ascertained. Medication use was evaluated in parallel with CKD disease type, presence of urological comorbidity, and hypertension. Chi-square tests compared …

Prospective Study Of Modifiable Risk Factors Of Arterial Hypertension And Left Ventricular Hypertrophy In Pediatric Patients On Hemodialysis., Dagmara Borzych-Dużałka, Rukshana Shroff, Bruno Ranchin, Yihui Zhai, Fabio Paglialonga, Jameela A. Kari, Yo H. Ahn, Hazem S. Awad, Reyner Loza, Nakysa Hooman, Robin Ericson, Dorota Drożdz, Amrit Kaur, Sevcan A. Bakkaloglu, Charlotte Samaille, Marsha Lee, Stephanie Tellier, Julia Thumfart, Marc Fila, Bradley A. Warady, Franz Schaefer, Claus P. Schmitt

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Fluid and salt overload in patients on dialysis result in high blood pressure (BP), left ventricular hypertrophy (LVH) and hemodynamic instability, resulting in cardiovascular morbidity.

METHODS: Analysis of 910 pediatric patients on maintenance hemodialysis/hemodiafiltration (HD/HDF), prospectively followed-up with 2758 observations recorded every 6-months in the International Pediatric Hemodialysis Network (IPHN).

RESULTS: Uncontrolled hypertension was present in 55% of observations, with 27% of patients exhibiting persistently elevated predialysis BP. Systolic and diastolic age- and height-standardized BP (BP-SDS) were independently associated with the number of antihypertensive medications (odds ratio [OR] = 1.47, 95% confidence interval 1.39-1.56, 1.36 [1.23-1.36]) and interdialytic weight …

Consistency Of Metabolite Associations With Measured Glomerular Filtration Rate In Children And Adults., Taibo Li, Morgan E. Grams, Lesley A. Inker, Jingsha Chen, Eugene P. Rhee, Bradley A. Warady, Andrew S. Levey, Michelle R. Denburg, Susan L. Furth, Vasan S. Ramachandran, Paul L. Kimmel, Josef Coresh

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: There is interest in identifying novel filtration markers that lead to more accurate GFR estimates than current markers (creatinine and cystatin C) and are more consistent across demographic groups. We hypothesize that large-scale metabolomics can identify serum metabolites that are strongly influenced by glomerular filtration rate (GFR) and are more consistent across demographic variables than creatinine, which would be promising filtration markers for future investigation.

METHODS: We evaluated the consistency of associations between measured GFR (mGFR) and 887 common, known metabolites quantified by an untargeted chromatography- and spectroscopy-based metabolomics platform (Metabolon) performed on frozen blood samples from 580 participants …