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1. Tobacco exposure in adults and children with proteinuric glomerulopathies: a NEPTUNE cohort study

Author

Linda Wang, Bayle Smith-Salzberg, Kevin EC Meyers, Dorey A. Glenn, Katherine R. Tuttle, Vimal K. Derebail, Tammy M. Brady, Keisha Gibson, Abigail R. Smith, Michelle M. O’Shaughnessy, Tarak Srivastava, Gentzon Hall, Jarcy Zee, Markus Bitzer, and Christine B. Sethna

Subjects
Cigarette smoking, Second-hand smoking, Nephrotic syndrome, Cotinine, Cardiovascular risk factors, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Abstract Background Tobacco exposure has been recognized as a risk factor for cardiovascular disease (CVD) and progression of kidney disease. Patients with proteinuric glomerulopathies are at increased risk for cardiovascular morbidity and mortality. Multiple studies have linked tobacco exposure to CVD and chronic kidney disease, but the relationships between smoking and proteinuric glomerulopathies in adults and children have not been previously explored. Methods Data from the Nephrotic Syndrome Study Network (NEPTUNE), a multi-center prospective observational study of participants with proteinuric glomerulopathies, was analyzed. 371 adults and 192 children enrolled in NEPTUNE were included in the analysis. Self-reported tobacco exposure was classified as non-smoker, active smoker, former smoker, or exclusive passive smoker. Baseline serum cotinine levels were measured in a sub-cohort of 178 participants. Results The prevalence of active smokers, former smokers and exclusive passive smoking among adults at baseline was 14.6%, 29.1% and 4.9%, respectively. Passive smoke exposure was 16.7% among children. Active smoking (reference non-smoking) was significantly associated with greater total cholesterol among adults (β 17.91 95% CI 0.06, 35.76, p = 0.049) while passive smoking (reference non-smoking) was significantly associated with greater proteinuria over time among children (β 1.23 95% CI 0.13, 2.33, p = 0.03). Higher cotinine levels were associated with higher baseline eGFR (r = 0.17, p = 0.03). Conclusion Tobacco exposure is associated with greater risk for CVD and worse kidney disease outcomes in adults and children with proteinuric glomerulopathies. Preventive strategies to reduce tobacco exposure may help protect against future cardiovascular and kidney morbidity and mortality in patients with proteinuric glomerulopathies.

Published
2023
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2. High risk of acute kidney injury in Malawian trauma patients: a prospective observational cohort study

Author

Erica C. Bjornstad, Zachary H. Smith, William Muronya, Charles K. Munthali, Amy K. Mottl, Stephen W. Marshall, Yvonne M. Golightly, Keisha Gibson, Anthony Charles, and Emily W. Gower

Subjects
Trauma, Acute kidney injury, Africa, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Abstract Background Trauma is a common cause of acute kidney injury (AKI). Yet little data exist regarding trauma-related-AKI in low-resourced settings, where the majority of deaths from AKI and trauma occur. We prospectively evaluated epidemiology of AKI in hospitalized Malawian trauma patients. Methods AKI was defined by creatinine-only Kidney Disease Improving Global Outcomes (KDIGO) criteria. Those with AKI were followed up 3–6 months later to determine persistent kidney abnormalities. We calculated univariate statistics with Wilcoxon rank sum tests, Fisher’s exact, and chi-square tests to compare those with and without AKI. Multivariate log-risk regression modelling was used to determine risk ratios (RR) and 95% confidence intervals (CI) for AKI development. Results Of 223 participants, 14.4% (n = 32) developed AKI. Most patients were young (median age 32) males (n = 193, 86.5%) involved in road traffic injuries (n = 120, 53.8%). After adjusting for confounders, those with severe anemia during their admission were 1.4 times (RR 1.4, 95% CI 1.1–1.8) more likely to develop AKI than those without. Overall mortality was 7.6% (n = 17), and those who developed AKI were more likely to die than those who did not (18.8% vs 5.6%, p-value = 0.02). Almost half of those with AKI (n = 32) either died (n = 6) or had persistent kidney dysfunction at follow-up (n = 8). Conclusion In one of the few African studies on trauma-related AKI, we found a high incidence of AKI (14.4%) in Malawian trauma patients with associated poor outcomes. Given AKI’s association with increased mortality and potential ramifications on long-term morbidity, urgent attention is needed to improve AKI-related outcomes.

Published
2021
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3. Immunosuppression Exposure and Risk of Infection-Related Acute Care Events in Patients With Glomerular Disease: An Observational Cohort StudyPlain-Language Summary

Author

Dorey A. Glenn, Jarcy Zee, Sarah Mansfield, Michelle M. O’Shaughnessy, Andrew S. Bomback, Keisha Gibson, Larry A. Greenbaum, Laura Mariani, Ronald Falk, Susan Hogan, Amy Mottl, and Michelle R. Denburg

Subjects
Corticosteroids, glomerular disease, glomerulonephritis, hospitalization, infection, immunosuppression, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Rationale & Objective: Infections cause morbidity and mortality in patients with glomerular disease. The relative contributions from immunosuppression exposure and glomerular disease activity to infection risk are not well characterized. To address this unmet need, we characterized the relationship between time-varying combinations of immunosuppressant exposure and infection-related acute care events while controlling for disease activity, among individuals with glomerular disease. Study Design: Prospective, multicenter, observational cohort study. Setting & Participants: Adults and children with biopsy-proven minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or immunoglobulin A nephropathy/vasculitis were enrolled at 71 clinical sites in North America and Europe. A total of 2,388 Cure Glomerulonephropathy Network participants (36% aged

Published
2022
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4. Validity of Urine NGALds Dipstick for Acute Kidney Injury in a Malawian Trauma Cohort

Author

Erica C. Bjornstad, William Muronya, Manly Kamija, Zachary Smith, Charles K. Munthali, Keisha Gibson, Amy K. Mottl, Anthony Charles, Stephen W. Marshall, Yvonne M. Golightly, and Emily W. Gower

Subjects
acute kidney injury, Africa, NGAL, point-of-care, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Introduction: Acute kidney injury (AKI) is a major cause of mortality worldwide, particularly in low-resource settings with limited diagnostic testing. Neutrophil gelatinase-associated lipocalin (NGAL) has shown promise in predicting AKI. Nested within a larger, prospective cohort study evaluating AKI incidence in admitted trauma patients, our objective was to evaluate a novel dipstick, NGALds, for the prediction of AKI in Malawi, Africa. Methods: Participants were >6 months of age. Spearman rank correlation coefficients (R) assessed NGAL categories (negative [≤50 ng/ml], low risk [51−149 ng/ml], moderate risk [150−299 ng/ml], and high risk [≥300 ng/ml]) for the urine NGALds dipstick and laboratory-based NGAL Test. Results: We enrolled 285 participants (one-third children). Thirteen percent developed AKI. The dipstick captured 45 of 52 participants (86.5%) with moderate- or high-risk NGAL values on laboratory-based testing (R = 0.74). The dipstick had sensitivity of 44.4%, specificity of 73.5%, positive predictive value of 19.5%, and negative predictive value of 90.2% for predicting AKI. Acute kidney injury was associated with an increased risk of mortality (relative risk [RR] = 3.9, 95% confidence interval [CI] = 1.9−8.2), but mortality risk greatly increased among children who first had a positive (≥150 ng/ml) NGALds result (RR = 12.0, 95% CI = 1.8−78.4). Conclusions: The NGALds dipstick performed similarly to the NGAL Test in this low-resource setting and may be a useful tool to rule out AKI. It may be even more important in predicting high mortality risk among children.

Published
2020
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5. Incidence and epidemiology of acute kidney injury in a pediatric Malawian trauma cohort: a prospective observational study

Author

Erica C. Bjornstad, William Muronya, Zachary H. Smith, Keisha Gibson, Amy K. Mottl, Anthony Charles, Stephen W. Marshall, Yvonne M. Golightly, Charles K. Munthali, and Emily W. Gower

Subjects
Acute kidney injury, Trauma, Africa, Pediatrics, Epidemiology, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Abstract Background Acute kidney injury (AKI) is highly associated with mortality risk in children worldwide. Trauma can lead to AKI and is a leading cause of pediatric death in Africa. However, there is no information regarding the epidemiology of pediatric, trauma-associated AKI in Africa. Methods Prospective cohort study of pediatric trauma patients admitted to a tertiary referral hospital in Malawi. Participants enrolled at admission were followed prospectively throughout their hospitalization. AKI was defined by creatinine-only Kidney Disease Improving Global Outcomes criteria. We calculated descriptive statistics and univariate relative risks (RR) for hypothesis-generation of potential risk factors associated with AKI. Results We analyzed data from 114 participants. Depending on baseline creatinine definition, AKI incidence ranged from 4 to 10%. The new Schwartz equation estimated baseline creatinine values best and yielded an AKI incidence of 9.7%. Almost one in ten children died during hospitalization, but those with AKI (n = 4) were at significantly higher risk of death compared to those without AKI (40.0% vs 6.2%; RR 6.5, 95% CI 2.2–19.1). Burn injuries were most commonly associated with AKI (63.6%). Other potential AKI risk factors included multiple injuries, trunk or facial injuries, and recent consumption of herbal remedies. Conclusions AKI occurs in up to 10% of admitted pediatric trauma patients in Malawi and increases the risk of death 7-fold compared to those without AKI. This large unrecognized burden in trauma requires further investment by researchers, clinicians and policymakers to develop evidenced-based triage, recognition, and management approaches to prevent the associated sequelae and potential mortality from AKI.

Published
2020
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6. Text Messaging for Disease Monitoring in Childhood Nephrotic Syndrome

Author

Chia-shi Wang, Jonathan P. Troost, Larry A. Greenbaum, Tarak Srivastava, Kimberly Reidy, Keisha Gibson, Howard Trachtman, John D. Piette, Christine B. Sethna, Kevin Meyers, Katherine M. Dell, Cheryl L. Tran, Suzanne Vento, Krishna Kallem, Emily Herreshoff, Sangeeta Hingorani, Kevin Lemley, Gia Oh, Elizabeth Brown, Jen-Jar Lin, Frederick Kaskel, and Debbie S. Gipson

Subjects
Diseases of the genitourinary system. Urology, RC870-923
Abstract

Introduction: There is limited information on effective disease monitoring for prompt interventions in childhood nephrotic syndrome. We examined the feasibility and effectiveness of a novel text messaging system (SMS) for disease monitoring in a multicenter, prospective study. Methods: A total of 127 patients

Published
2019
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7. Update on the efficacy and safety profile of voclosporin

Author

Cristina Arriens, Y. K. Onno Teng, Ellen M. Ginzler, Samir V. Parikh, Anca D. Askanase, Amit Saxena, Keisha Gibson, Dawn J. Caster, Tatsuya Atsumi, Laura Lisk, Simrat Randhawa, Rashieda Gluck, Neil Solomons, and Robert B. Huizinga

Subjects
Rheumatology
Abstract

Objective: This integrated analysis evaluates the efficacy and safety of voclosporin, a novel calcineurin inhibitor, at 23.7 mg twice daily in combination with mycophenolate mofetil (MMF) and oral glucocorticoids in lupus nephritis (LN) using pooled data from two large phase II and phase III clinical trials. The purpose was to expand the pool of patients for safety analyses and to increase power for efficacy analyses in patient subpopulations. Methods: Aurinia Urinary Protein Reduction in Active Lupus with Voclosporin (AURA-LV) (phase II) and Aurinia Renal Response in Active Lupus With Voclosporin (AURORA 1) (phase III) were randomized, placebo-controlled, double-blind trials with similar designs and end points comparing voclosporin to control in combination with MMF and oral glucocorticoids for the treatment of LN. The primary efficacy outcome of the integrated analysis was complete renal response (CRR) at approximately one year (Week 48 data from AURA-LV and Week 52 from AURORA 1). Safety was assessed throughout the trials. Results: Overall, 534 patients (268 voclosporin; 266 control) were included in the integrated analysis. Significantly more patients achieved a CRR at one year in the voclosporin group than in the control group (43.7% vs. 23.3%; OR 2.76; 95% CI 1.88, 4.05 P < 0.0001). The incidence of adverse events (AEs) was similar (91.4% voclosporin; 87.2% control). Most AEs were mild to moderate in severity; the most commonly reported AEs were classified as infections and infestations (62.2% voclosporin; 54.9% control) and gastrointestinal disorders (45.3% voclosporin; 35.3% placebo). No new or unexpected safety signals were detected. Conclusions: This integrated analysis demonstrates the efficacy and safety of voclosporin in the treatment of LN across the diverse racial and ethnic groups studied.

Published
2023

9. Saliva urea nitrogen dipsticks to predict acute kidney injury in Malawian trauma patients

Author

Erica Bjornstad, William Muronya, Zachary H Smith, Manly Kamija, Rhys Evans, Amy K Mottl, Yvonne M Golightly, Keisha Gibson, Anthony Charles, and Emily W Gower

Subjects
Psychiatry and Mental health, Neuropsychology and Physiological Psychology
Abstract

Background: Many low-resource settings have limited access to serum creatinine tests necessary for kidney disease identification. Among Malawian patients who are hospitalized after trauma, we evaluated the use of point-of-care saliva urea nitrogen (SUN) dipsticks to predict acute kidney injury (AKI). Methods: In a nested prospective cohort study, we enrolled hospitalized acute trauma patients aged ≥6 months to evaluate AKI (defined by KDIGO criteria) and the test characteristics of SUN to predict AKI.

Published
2023
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10. Seeking Racial Equity in Hematology and Oncology: a Fellow-Led Educational Series to Promote Reflection and Action

Author

Jacob N. Stein, Jacquelyne J. Gaddy, Jonathan D. Sorah, Melissa Wooten, Keisha Gibson, and Frances A. Collichio

Subjects
Cancer Research, Oncology, Public Health, Environmental and Occupational Health
Abstract

11004 Background: With the murder of George Floyd and health disparities laid bare by the COVID pandemic, the US is reckoning with racial injustice. Across medicine and oncology, institutions are grappling with how to address systemic racism and improve care for patients of color. At the University of North Carolina (UNC), trainees developed an educational curriculum to raise awareness of implicit bias and introduce methods to address racial inequities. We present our findings on feasibility and acceptability of a fellow-led course on racism in medicine at a major academic medical center. Methods: UNC oncology fellows adapted a curriculum on implicit bias and racism in medicine in spring 2021. Our aims were 1) to improve knowledge and awareness about implicit bias and systemic racism and 2) introduce methods to address racial inequities. We used lived experiences and collated materials from scientific literature and lay media to illustrate key points. Sessions were: 1) Introduction and Implicit Bias, 2) Implicit Bias in Action: A Case Study, 3) Race-Based Metrics: Journal Club, 4) Career Perspective on Equity in Oncology. Videos, journal articles, and group discussion were employed to appeal to many learning styles. Results: Four sessions were held virtually for the Divisions of Oncology and Hematology. Attendance ranged from 28 to 35 per session. A post curriculum survey assessed perception of racial inequality in medicine and the series’ effects using a Likert scale. Twenty-nine participants completed the survey, 12 of whom were fellows. Of all participants, 71% reported that the course improved knowledge or awareness of racial inequities "some" or "a great deal" and 61% reported that it improved their comfort level addressing racial inequities "some" or "a great deal." All participants endorsed at least “some” racial inequity in medicine. Notably, over 75% of participants indicated interest in further sessions. Conclusions: Formulation of an educational curriculum by fellows and delivered in a division wide setting was feasible and well received by participants with robust discussion and interest in further work. Fundamental to this series’ effectiveness was creating a space for discussion and reflection among colleagues. The goals of improving knowledge and introducing methods to address racial inequities were met. Importantly, our course was integrated alongside institutional efforts on DEI. We were limited by a lack of pre-course survey results due to a technical error. Given the current groundswell of interest and focus in improving racial equity in our society, we encourage other institutions to take similar steps to highlight issues of systemic racism and continue to move our field in the right direction.

Published
2022

11. Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulopathy Network

Author

Dhruti P. Chen, Margaret E. Helmuth, Abigail R. Smith, Pietro A. Canetta, Isabelle Ayoub, Krzysztof Mucha, Mahmoud Kallash, Jeffrey B. Kopp, Rasheed Gbadegesin, Brenda W. Gillespie, Larry A. Greenbaum, Rulan S. Parekh, Tracy E. Hunley, C. John Sperati, David T. Selewski, Jason Kidd, Aftab Chishti, Kimberly Reidy, Amy K. Mottl, Debbie S. Gipson, Tarak Srivastava, Katherine E. Twombley, Wooin Ahn, Gerald Appel, Paul Appelbaum, Revekka Babayev, Andrew Bomback, Brenda Chan, Vivette Denise D’Agati, Samitri Dogra, Hilda Fernandez, Ali Gharavi, William Hines, Syed Ali Husain, Namrata Jain, Krzysztof Kiryluk, Fangming Lin, Maddalena Marasa, Glen Markowitz, Hila Milo Rasouly, Sumit Mohan, Nicola Mongera, Jordan Nestor, Thomas Nickolas, Jai Radhakrishnan, Maya Rao, Simone Sanna-Cherchi, Shayan Shirazian, Michael Barry Stokes, Natalie Uy, Anthony Valeri, Natalie Vena, Bartosz Foroncewicz, Barbara Moszczuk, Agnieszka Perkowska-Ptasińska, Gian Marco Ghiggeri, Francesca Lugani, Josephine Ambruzs, Helen Liapis, Rossana Baracco, Amrish Jain, Isa Ashoor, Diego Aviles, Sun-Young Ahn, Prasad Devarajan, Elif Erkan, Donna Claes, Hillarey Stone, Sherene Mason, Liliana Gomez-Mendez, Chia-shi Wang, Hong Yin, Yi Cai, Goebel Jens, Julia Steinke, Donald Weaver, Jerome Lane, Carl Cramer, Cindy Pan, Neil Paloian, Rajasree Sreedharan, Corinna Bowers, Mary Dreher, John Mahan, Samantha Sharpe, William Smoyer, Amira Al-Uzri, Sandra Iragorri, Myda Khalid, Craig Belsha, Joseph Alge, Michael Braun, A.C. Gomez, Scott Wenderfer, Tetyana Vasylyeva, Daniel Feig, Gabriel Cara Fuentes, Melisha Hannah, Carla Nester, Jon Klein, Chryso Katsoufis, Wacharee Seeherunvong, Michelle Rheault, Craig Wong, Nisha Mathews, John Barcia, Agnes Swiatecka-Urban, Sharon Bartosh, Vikas Dharnidharka, Joseph Gaut, Louis-Philippe Laurin, Virginie Royal, Anand Achanti, Milos Budisavljevic, Sally Self, Cybele Ghossein, Yonatan Peleg, Shikha Wadhwani, Salem Almaani, Tibor Nadasdy, null Samir, null Parikh, Brad Rovin, Anthony Chang, Huma Fatima, Bruce Julian, Jan Novak, Matthew Renfrow, Dana Rizk, Vimal Derebail, Ronald Falk, Keisha Gibson, Dorey Glenn, Susan Hogan, Koyal Jain, J. Charles Jennette, Caroline Poulton, Manish Kanti Saha, Agnes Fogo, Neil Sanghani, Selvaraj Muthusamy, Jeffrey Schelling, Jean Hou, Kevin Lemley, Warren Mika, Pierre Russo, Michelle Denburg, Amy Kogon, Kevin Meyers, Madhura Pradhan, Raed Bou Matar, John O’Toole, John Sedor, Christine Sethna, Suzanne Vento, Mohamed Atta, Serena Bagnasco, Alicia Neu, Sharon Adler, Tiane Dai, Ram Dukkipati, Fernando Fervenza, Sanjeev Sethi, Frederick Kaskel, Kaye Brathwaite, Joseph Weisstuch, Ming Wu, Olga Zhdanova, Jurgen Heymann, Meryl Waldman, Cheryl Winkler, Katherine Tuttle, Jill Krissberg, Richard Lafayette, Kamal Fahmeedah, Elizabeth Talley, Michelle Hladunewich, Carmen Avila-Casado, Daniel Cattran, Reich Heather, Philip Boll, Yelena Drexler, Alessia Fornoni, Patrick Gipson, Jeffrey Hodgin, Andrea Oliverio, Jon Hogan, Lawrence Holzman, Matthew Palmer, Gaia Coppock, Blaise Abromovitz, Michael Mortiz, Charles Alpers, J. Ashley Jefferson, Elizabeth Brown, Kamal Sambandam, Bethany Roehm, Bruce Robinson, Cynthia Nast, Laura Barisoni, Matthias Kretzler, Laura Mariani, and Lisa M. Guay-Woodford

Subjects
Nephrology
Abstract

Adolescent- and adult-onset disease minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulopathy Network (CureGN) and assessed predictors of rituximab response.Prospective, multicenter, observational study.CureGN participants with proven MCD on biopsy.Age at disease onset. Initiation of RAAS blockade and immunosuppression including rituximab during the study period.Relapse and remission, change in eGFR and kidney failure.Remission and relapse probabilities are estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in estimated glomerular filtration rate (eGFR). Cox proportional hazards models were used to estimate the association between rituximab administration and remission.304 childhood (≤12 years old), 49 adolescent (13-17 years old), and 201 adult onset (≥18 years) participants were included with 2.7-3.2 years of follow-up after enrollment. Children had longer time to biopsy (238 days vs. 23 in adolescents, and 36 in adults, p0.001) and were more likely to have received therapy prior to biopsy. Children were more likely to be treated with immunosuppression but not RAAS blockade. The rate of relapse was higher in childhood-onset versus adult-onset participants (hazard ratio (HR) 1.69 (95% confidence interval (CI) 1.29-2.21)). The probability of remission was also higher in childhood-onset disease (HR 1.33 (95%CI 1.02-1.72)). eGFR loss in all groups was minimal. Children were more likely to remit after rituximab than adolescents and adults (adjusted HR 2.1, p=0.003). Across all groups, glucocorticoid-sensitivity was associated with a greater likelihood of achieving complete remission after rituximab (adjusted HR 2.62, p=0.002).CureGN was limited to biopsy-proven disease. Comparisons of childhood to non-childhood cases of MCD may be subject to selection bias, given that childhood cases who are biopsied may be limited to those patients who are least responsive to initial therapy.Among patients with MCD who underwent kidney biopsy, there were differences in the course (relapse and remission) of childhood-onset compared to adolescent and adult-onset disease, as well as rituximab response.

Published
2022

12. Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Author

Elisa Delbarba, Maddalena Marasa, Pietro A. Canetta, Stacy E. Piva, Debanjana Chatterjee, Byum Hee Kil, Xueru Mu, Keisha L. Gibson, Michelle A. Hladunewich, Jonathan J. Hogan, Bruce A. Julian, Jason M. Kidd, Louis-Philippe Laurin, Patrick H. Nachman, Michelle N. Rheault, Dana V. Rizk, Neil S. Sanghani, Howard Trachtman, Scott E. Wenderfer, Ali G. Gharavi, Andrew S. Bomback, Wooin Ahn, Gerald B. Appel, Revekka Babayev, Ibrahim Batal, Eric Brown, Eric S. Campenot, Pietro Canetta, Brenda Chan, Vivette D. D’Agati, Hilda Fernandez, Bartosz Foroncewicz, Gian Marco Ghiggeri, William H. Hines, Namrata G. Jain, Krzysztof Kiryluk, Wai L. Lau, Fangming Lin, Francesca Lugani, Glen Markowitz, Sumit Mohan, Krzysztof Mucha, Thomas L. Nickolas, Stacy Piva, Jai Radhakrishnan, Maya K. Rao, Simone Sanna-Cherchi, Dominick Santoriello, Michael B. Stokes, Natalie Yu, Anthony M. Valeri, Ronald Zviti, Larry A. Greenbaum, William E. Smoyer, Amira Al-Uzri, Isa Ashoor, Diego Aviles, Rossana Baracco, John Barcia, Sharon Bartosh, Craig Belsha, Corinna Bowers, Michael C. Braun, Aftab Chishti, Donna Claes, Carl Cramer, Keefe Davis, Elif Erkan, Daniel Feig, Michael Freundlich, Rasheed Gbadegesin, Melisha Hanna, Guillermo Hidalgo, Tracy E. Hunley, Amrish Jain, Mahmoud Kallash, Myda Khalid, Jon B. Klein, Jerome C. Lane, John Mahan, Nisha Mathews, Carla Nester, Cynthia Pan, Larry Patterson, Hiren Patel, Adelaide Revell, Cynthia Silva, Rajasree Sreedharan, Tarak Srivastava, Julia Steinke, Katherine Twombley, Tetyana L. Vasylyeva, Donald J. Weaver, Craig S. Wong, Salem Almaani, Isabelle Ayoub, Milos Budisavljevic, Vimal Derebail, Huma Fatima, Ronald Falk, Agnes Fogo, Todd Gehr, Keisha Gibson, Dorey Glenn, Raymond Harris, Susan Hogan, Koyal Jain, J. Charles Jennette, Bruce Julian, Jason Kidd, H. Davis Massey, Amy Mottl, Patrick Nachman, Tibor Nadasdy, Jan Novak, Samir Parikh, Vincent Pichette, Caroline Poulton, Thomas Brian Powell, Matthew Renfrow, Dana Rizk, Brad Rovin, Virginie Royal, Manish Saha, Neil Sanghani, Sally Self, Sharon Adler, Charles Alpers, Raed Bou Matar, Elizabeth Brown, Daniel Cattran, Michael Choi, Katherine M. Dell, Ram Dukkipati, Fernando C. Fervenza, Alessia Fornoni, Crystal Gadegbeku, Patrick Gipson, Leah Hasely, Sangeeta Hingorani, Michelle Hladunewich, Jonathan Hogan, Lawrence B. Holzman, J. Ashley Jefferson, Kenar Jhaveri, Duncan B. Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, Richard Lafayette, Kevin V. Lemley, Laura Malaga-Dieguez, Kevin Meyers, Alicia Neu, Michelle Marie O’Shaughnessy, John F. O’Toole, Rulan Parekh, Heather Reich, Kimberly Reidy, Helbert Rondon, Kamalanathan K. Sambandam, John R. Sedor, David T. Selewski, Christine B. Sethna, Jeffrey Schelling, John C. Sperati, Agnes Swiatecka-Urban, Katherine R. Tuttle, Joseph Weisstuch, Suzanne Vento, Olga Zhdanova, Brenda Gillespie, Debbie S. Gipson, Peg Hill-Callahan, Margaret Helmuth, Emily Herreshoff, Matthias Kretzler, Chrysta Lienczewski, Sarah Mansfield, Laura Mariani, Cynthia C. Nast, Bruce M. Robinson, Jonathan Troost, Matthew Wladkowski, Jarcy Zee, Dawn Zinsser, and Lisa M. Guay-Woodford

Subjects
medicine.medical_specialty, glomerulonephropathy, glomerular disease, 030232 urology & nephrology, Disease, 030204 cardiovascular system & hematology, Nephropathy, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Focal segmental glomerulosclerosis, Membranous nephropathy, Clinical Research, Internal medicine, Biopsy, medicine, Minimal change disease, focal segmental glomerulosclerosis, Creatinine, medicine.diagnostic_test, business.industry, membranous nephropathy, IgA nephropathy, medicine.disease, minimal change disease, chemistry, Nephrology, Cohort, business
Abstract

Introduction Glomerular diseases are characterized by variable disease activity over many years. We aimed to analyze the relationship between clinical disease activity and duration of glomerular disease. Methods Disease activity in adults with chronic minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy (IgAN; first diagnostic biopsy >5 years before enrollment; Of Longstanding Disease [OLD] cohort, n = 256) followed at Columbia University Medical Center (CUMC), was compared with disease activity of an internal and external cohort of patients with first diagnostic biopsy, Graphical abstract

Published
2020
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13. Racial and Ethnic Disparities in Acute Care Utilization Among Patients With Glomerular Disease

Author

Jill R. Krissberg, Michelle M. O’Shaughnessy, Abigail R. Smith, Margaret E. Helmuth, Salem Almaani, Diego H. Aviles, Kaye E. Brathwaite, Yi Cai, Daniel Cattran, Rasheed Gbadegesin, Dorey A. Glenn, Larry A. Greenbaum, Sandra Iragorri, Koyal Jain, Myda Khalid, Jason Kidd, Jeffrey Kopp, Richard Lafayette, Jerome C. Lane, Francesca Lugani, Jordan G. Nestor, Rulan S. Parekh, Kimberly Reidy, David T. Selewski, Christine B. Sethna, C. John Sperati, Katherine Tuttle, Katherine Twombley, Tetyana L. Vasylyeva, Donald J. Weaver, Scott E. Wenderfer, Keisha Gibson, Wooin Ahn, Gerald Appel, Paul Appelbaum, Revekka Babayev, Andrew Bomback, Eric Brown, Pietro Canetta, Lucrezia Carlassara, Brenda Chan, Vivette Denise D’Agati, Samitri Dogra, Hilda Fernandez, Ali Gharavi, William Hines, Syed Ali Husain, Krzysztof Kiryluk, Fangming Lin, Maddalena Marasa, Glen Markowitz, Hila Milo Rasouly, Sumit Mohan, Nicola Mongera, Thomas Nickolas, Jai Radhakrishnan, Maya Rao, Simone Sanna-Cherchi, Shayan Shirazian, Michael Barry Stokes, Natalie Uy, Anthony Valeri, Natalie Vena, Bartosz Foroncewicz, Barbara Moszczuk, Krzysztof Mucha, Agnieszka Perkowska-Ptasińska, Gian Marco Ghiggeri, Josephine Ambruzs, Helen Liapis, Rossana Baracco, Amrish Jain, Isa Ashoor, Tarak Srivastava, Sun-Young Ahn, Prasad Devarajan, Elif Erkan, Donna Claes, Hillarey Stone, Sherene Mason, Cynthia Silva, Liliana Gomez-Mendez, Chia-shi Wang, Hong (Julie) Yin, Goebel Jens, Julia Steinke, Carl Cramer, Cindy Pan, Rajasree Sreedharan, Corinna Bowers, Mary Dreher, Mahmoud Kallash, John Mahan, Samantha Sharpe, William Smoyer, Amira Al-Uzri, Craig Belsha, Michael Braun, A.C. Gomez, Daniel Feig, Gabriel Cara Fuentes, Melisha Hannah, Carla Nester, Aftab Chishti, Jon Klein, Chryso Katsoufis, Wacharee Seeherunvong, Michelle Rheault, Craig Wong, Nisha Mathews, John Barcia, Agnes Swiatecka-Urban, Sharon Bartosh, Tracy Hunley, Vikas Dharnidharka, Joseph Gaut, Louis-Philippe Laurin, Virginie Royal, Anand Achanti, Milos Budisavljevic, Sally Self, Cybele Ghossein, Shikha Wadhwani, Isabelle Ayoub, Tibor Nadasdy, Samir Parikh, Brad Rovin, Anthony Chang, Huma Fatima, Jan Novak, Matthew Renfrow, Dana Rizk, Dhruti Chen, Vimal Derebail, Ronald Falk, Susan Hogan, J. Charles Jennette, Amy Mottl, Caroline Poulton, Manish Kanti Saha, Agnes Fogo, Neil Sanghani, Hugh Massey, Selvaraj Muthusamy, Santhi Ganesan, Agustin Gonzalez-Vicente, Jeffrey Schelling, Jean Hou, Kevin Lemley, Warren Mika, Pierre Russo, Michelle Denburg, Amy Kogon, Kevin Meyers, Madhura Pradhan, Raed Bou Matar, John O’Toole, John Sedor, Serena Bagnasco, Alicia Neu, Sharon Adler, Tiane Dai, Ram Dukkipati, Fernando Fervenza, Sanjeev Sethi, Frederick Kaskel, Suzanne Vento, Joseph Weisstuch, Ming Wu, Olga Zhdanova, Jurgen Heymann, Meryl Waldman, Cheryl Winkler, Michelle Hladunewich, Carmen Avila-Casado, Reich Heather, Philip Boll, Yelena Drexler, Alessia Fornoni, Patrick Gipson, Jeffrey Hodgin, Andrew Oliverio, Jon Hogan, Lawrence Holzman, Matthew Palmer, Blaise Abromovitz, Michael Mortiz, Charles Alpers, J. Ashley Jefferson, Elizabeth Brown, Kamal Sambandam, Bruce Robinson, Cynthia Nast, Laura Barisoni, Brenda Gillespie, Deb Gipson, Maggie Hicken, Matthias Kretzler, Laura Mariani, and Lisa M. Guay-Woodford

Subjects
Nephrology
Abstract

The effects of race, ethnicity, socioeconomic status (SES), and disease severity on acute care utilization in patients with glomerular disease are unknown.Prospective cohort study.1,456 adults and 768 children with biopsy-proven glomerular disease enrolled in the Cure Glomerulonephropathy (CureGN) cohort.Race and ethnicity as a participant-reported social factor.Acute care utilization defined as hospitalizations or emergency department visits.Multivariable recurrent event proportional rate models were used to estimate associations between race and ethnicity and acute care utilization.Black or Hispanic participants had lower SES and more severe glomerular disease than White or Asian participants. Acute care utilization rates were 45.6, 29.5, 25.8, and 19.2 per 100 person-years in Black, Hispanic, White, and Asian adults, respectively, and 55.8, 42.5, 40.8, and 13.0, respectively, for children. Compared with the White race (reference group), Black race was significantly associated with acute care utilization in adults (rate ratio [RR], 1.76 [95% CI, 1.37-2.27]), although this finding was attenuated after multivariable adjustment (RR, 1.31 [95% CI, 1.03-1.68]). Black race was not significantly associated with acute care utilization in children; Asian race was significantly associated with lower acute care utilization in children (RR, 0.32 [95% CI 0.14-0.70]); no significant associations between Hispanic ethnicity and acute care utilization were identified.We used proxies for SES and lacked direct information on income, household unemployment, or disability.Significant differences in acute care utilization rates were observed across racial and ethnic groups in persons with prevalent glomerular disease, although many of these difference were explained by differences in SES and disease severity. Measures to combat socioeconomic disadvantage in Black patients and to more effectively prevent and treat glomerular disease are needed to reduce disparities in acute care utilization, improve patient wellbeing, and reduce health care costs.

Published
2022

14. Comparing Kidney Health Outcomes in Children, Adolescents, and Adults With Focal Segmental Glomerulosclerosis

Author

Debbie S, Gipson, Jonathan P, Troost, Cathie, Spino, Samara, Attalla, Joshua, Tarnoff, Susan, Massengill, Richard, Lafayette, Virginia, Vega-Warner, Sharon, Adler, Patrick, Gipson, Matthew, Elliott, Frederick, Kaskel, Damian, Fermin, Marva, Moxey-Mims, Richard N, Fine, Elizabeth J, Brown, Kimberly, Reidy, Katherine, Tuttle, Keisha, Gibson, Kevin V, Lemley, Larry A, Greenbaum, Meredith A, Atkinson, Sangeeta, Hingorani, Tarak, Srivastava, Christine B, Sethna, Kevin, Meyers, Cheryl, Tran, Katherine M, Dell, Chia-Shi, Wang, Jennifer Lai, Yee, Matthew G, Sampson, Rasheed, Gbadegesin, J J, Lin, Tammy, Brady, Michelle, Rheault, and Howard, Trachtman

Subjects
Adult, Male, Nephrotic Syndrome, Adolescent, Glomerulosclerosis, Focal Segmental, General Medicine, Apolipoprotein L1, Kidney, Cohort Studies, Outcome Assessment, Health Care, Humans, Kidney Failure, Chronic, Female, Child
Abstract

Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage kidney disease (ESKD) across the lifespan. While 10% to 15% of children and 3% of adults who develop ESKD have FSGS, it remains uncertain whether the natural history differs in pediatric vs adult patients, and this uncertainty contributes to the exclusion of children and adolescents in clinical trials.To examine whether there are differences in the kidney health outcomes among children, adolescents, and adults with FSGS.This cohort study used pooled and parallel analyses, completed July 5, 2022, from 3 complimentary data sources: (1) Nephrotic Syndrome Rare Disease Clinical Research Network (NEPTUNE); (2) FSGS clinical trial (FSGS-CT); and (3) Kidney Research Network (KRN). NEPTUNE is a multicenter US/Canada cohort study; FSGS-CT is a multicenter US/Canada clinical trial; and KRN is a multicenter US electronic health record-based registry from academic and community nephrology practices. NEPTUNE included 166 patients with incident FSGS enrolled at first kidney biopsy; FSGS-CT included 132 patients with steroid-resistant FSGS randomized to cyclosporine vs dexamethasone with mycophenolate; and KRN included 184 patients with prevalent FSGS. Data were collected from November 2004 to October 2019 and analyzed from October 2020 to July 2022.Age: children (age13 years) vs adolescents (13-17 years) vs adults (≥18 years). Covariates of interest included sex, disease duration, APOL1 genotype, urine protein-to-creatinine ratio, estimated glomerular filtration rate (eGFR), edema, serum albumin, and immunosuppressive therapy.ESKD, composite outcome of ESKD or 40% decline in eGFR, and complete and/or partial remission of proteinuria.The study included 127 (26%) children, 102 (21%) adolescents, and 253 (52%) adults, including 215 (45%) female participants and 138 (29%) who identified as Black, 98 (20%) who identified as Hispanic, and 275 (57%) who identified as White. Overall, the median time to ESKD was 11.9 years (IQR, 5.2-19.1 years). There was no difference in ESKD risk among children vs adults (hazard ratio [HR], 0.67; 95% CI, 0.43-1.03) or adolescents vs adults (HR, 0.85; 95% CI, 0.52-1.36). The median time to the composite end point was 5.7 years (IQR 1.6-15.2 years), with hazard ratio estimates for children vs adults of 1.12 (95% CI, 0.83-1.52) and adolescents vs adults of 1.06 (95% CI, 0.75-1.50).In this study, the association of FSGS with kidney survival and functional outcomes was comparable at all ages.

Published
2022
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16. The american pediatric society and society for pediatric research joint statement against racism and social injustice

Author

Larry W Markham, Lina F. Chalak, Susan S. Baker, Joel N. Hirschhorn, Leslie R Walker-Harding, Beth Tarini, Candice D. Fike, Lauren C. Frazer, Monika Goyal, Michael R. DeBaun, Clifford W. Bogue, Keisha Gibson, Nathalie L. Maitre, Michelle A. Gill, Linda McAllister-Lucas, Sarah C. Armstrong, Jordan S. Orange, David A. Hunstad, Hannah C. Glass, Stephanie D. Davis, Catherine M. Gordon, Stephen R. Daniels, Waldemar A. Carlo, Robin H. Steinhorn, Tamara Simon, Prachi E. Shah, Steven H. Abman, Mary B. Leonard, and Lori R. Holtz

Subjects
medicine.medical_specialty, Statement (logic), media_common.quotation_subject, Pediatric research, Published Erratum, MEDLINE, Pediatrics, Racism, United States, Special Article, Social injustice, Social Justice, Family medicine, Pediatrics, Perinatology and Child Health, Pediatric surgery, medicine, Joint (building), Psychology, Societies, Medical, media_common
Abstract

Although the coronavirus disease 2019 pandemic has served as a flashlight, illuminating and unmasking deep socio-economic and health care divides in our country, the terrible events surrounding the horrific murder of Mr. George Floyd in Minneapolis has spawned even greater outrage. As we all know, Mr. Floyd’s death is not an isolated incident, as there have been a tragic string of such deaths in recent years that further reflect deep issues regarding racism and systemic underlying causes of injustice. Unfortunately, the country’s inability to fully address these systemic foundations of injustice persists.

Published
2020
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17. An eQTL Landscape of Kidney Tissue in Human Nephrotic Syndrome

Author

Christopher E. Gillies, Rosemary Putler, Rajasree Menon, Edgar Otto, Kalyn Yasutake, Viji Nair, Paul Hoover, David Lieb, Shuqiang Li, Sean Eddy, Damian Fermin, Michelle T. McNulty, Nir Hacohen, Krzysztof Kiryluk, Matthias Kretzler, Xiaoquan Wen, Matthew G. Sampson, John Sedor, Katherine Dell, Marleen Schachere, Kevin Lemley, Lauren Whitted, Tarak Srivastava, Connie Haney, Christine Sethna, Kalliopi Grammatikopoulos, Gerald Appel, Michael Toledo, Laurence Greenbaum, Chia-shi Wang, Brian Lee, Sharon Adler, Cynthia Nast, Janine LaPage, Ambarish Athavale, Alicia Neu, Sara Boynton, Fernando Fervenza, Marie Hogan, John C. Lieske, Vladimir Chernitskiy, Frederick Kaskel, Neelja Kumar, Patricia Flynn, Jeffrey Kopp, Eveleyn Castro-Rubio, Jodi Blake, Howard Trachtman, Olga Zhdanova, Frank Modersitzki, Suzanne Vento, Richard Lafayette, Kshama Mehta, Crystal Gadegbeku, Duncan Johnstone, Daniel Cattran, Michelle Hladunewich, Heather Reich, Paul Ling, Martin Romano, Alessia Fornoni, Laura Barisoni, Carlos Bidot, Debbie Gipson, Amanda Williams, Renee Pitter, Patrick Nachman, Keisha Gibson, Sandra Grubbs, Anne Froment, Lawrence Holzman, Kevin Meyers, Krishna Kallem, Fumei Cerecino, Kamal Sambandam, Elizabeth Brown, Natalie Johnson, Ashley Jefferson, Sangeeta Hingorani, Kathleen Tuttle, Laura Curtin, S. Dismuke, Ann Cooper, Barry Freedman, Jen Jar Lin, Stefanie Gray, Larua Barisoni, Brenda Gillespie, Laura Mariani, Peter Song, Johnathan Troost, Jarcy Zee, Emily Herreshoff, Colleen Kincaid, Chrysta Lienczewski, Tina Mainieri, Kevin Abbott, Cindy Roy, Tiina Urv, and John Brooks

Subjects
Adult, Male, 0301 basic medicine, Nephrotic Syndrome, Adolescent, Quantitative Trait Loci, 030232 urology & nephrology, Genomics, Genome-wide association study, Computational biology, Human leukocyte antigen, Biology, Kidney, Polymorphism, Single Nucleotide, Article, Transcriptome, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Focal segmental glomerulosclerosis, Genetics, medicine, Humans, Minimal change disease, Allele, Alleles, Genetics (clinical), Gene Expression Profiling, Bayes Theorem, Middle Aged, medicine.disease, 030104 developmental biology, Expression quantitative trait loci, Female, Genome-Wide Association Study
Abstract

Expression quantitative trait loci (eQTL) studies illuminate the genetics of gene expression and, in disease research, can be particularly illuminating when using the tissues directly impacted by the condition. In nephrology, there is a paucity of eQTL studies of human kidney. Here, we used whole-genome sequencing (WGS) and microdissected glomerular (GLOM) and tubulointerstitial (TI) transcriptomes from 187 individuals with nephrotic syndrome (NS) to describe the eQTL landscape in these functionally distinct kidney structures. Using MatrixEQTL, we performed cis-eQTL analysis on GLOM (n = 136) and TI (n = 166). We used the Bayesian “Deterministic Approximation of Posteriors” (DAP) to fine-map these signals, eQTLBMA to discover GLOM- or TI-specific eQTLs, and single-cell RNA-seq data of control kidney tissue to identify the cell type specificity of significant eQTLs. We integrated eQTL data with an IgA Nephropathy (IgAN) GWAS to perform a transcriptome-wide association study (TWAS). We discovered 894 GLOM eQTLs and 1,767 TI eQTLs at FDR < 0.05. 14% and 19% of GLOM and TI eQTLs, respectively, had >1 independent signal associated with its expression. 12% and 26% of eQTLs were GLOM specific and TI specific, respectively. GLOM eQTLs were most significantly enriched in podocyte transcripts and TI eQTLs in proximal tubules. The IgAN TWAS identified significant GLOM and TI genes, primarily at the HLA region. In this study, we discovered GLOM and TI eQTLs, identified those that were tissue specific, deconvoluted them into cell-specific signals, and used them to characterize known GWAS alleles. These data are available for browsing and download via our eQTL browser, “nephQTL.”

Published
2018
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18. Correction: The american pediatric society and society for pediatric research joint statement against racism and social injustice

Author

Steven H. Abman, Sarah Armstrong, Susan Baker, Clifford W. Bogue, Waldemar Carlo, Lina Chalak, Stephen R. Daniels, Stephanie Davis, Michael R. Debaun, Candice Fike, Lauren Frazer, Keisha Gibson, Michelle Gill, Hannah Glass, Catherine M. Gordon, Monika Goyal, Joel Hirschhorn, Lori Holtz, David A. Hunstad, Mary B. Leonard, Nathalie Maitre, Larry Markham, Linda McAllister-Lucas, Jordan Orange, Prachi Shah, Tamara Simon, Robin H. Steinhorn, Beth Tarini, and Leslie R. Walker-Harding

Subjects
Pediatrics, Perinatology and Child Health
Published
2021
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20. CureGN Study Rationale, Design, and Methods: Establishing a Large Prospective Observational Study of Glomerular Disease

Author

Laura H. Mariani, Andrew S. Bomback, Pietro A. Canetta, Michael F. Flessner, Margaret Helmuth, Michelle A. Hladunewich, Jonathan J. Hogan, Krzysztof Kiryluk, Patrick H. Nachman, Cynthia C. Nast, Michelle N. Rheault, Dana V. Rizk, Howard Trachtman, Scott E. Wenderfer, Corinna Bowers, Peg Hill-Callahan, Maddalena Marasa, Caroline J. Poulton, Adelaide Revell, Suzanne Vento, Laura Barisoni, Dan Cattran, Vivette D’Agati, J. Charles Jennette, Jon B. Klein, Louis-Philippe Laurin, Katherine Twombley, Ronald J. Falk, Ali G. Gharavi, Brenda W. Gillespie, Debbie S. Gipson, Larry A. Greenbaum, Lawrence B. Holzman, Matthias Kretzler, Bruce Robinson, William E. Smoyer, Lisa M. Guay-Woodford, Wooin Ahn, Gerald B. Appel, Revekka Babayev, Ibrahim Batal, Eric Brown, Eric S. Campenot, Pietro Canetta, Lucrezia Carlassara, Brenda Chan, Debanjana Chatterjee, Vivette D. D’Agati, Elisa Delbarba, Samriti Dogra, Hilda Fernandez, Bartosz Foroncewicz, Gian Marco Ghiggeri, William H. Hines, S. Ali Husain, Namrata G. Jain, Pascale Khairallah, Byum Hee Kil, Anushya Jeyabalan, Wai L. Lau, Fangming Lin, Francesca Lugani, Glen Markowitz, Sumit Mohan, Xueru Mu, Krzysztof Mucha, Thomas L. Nickolas, Stacy Piva, Jai Radhakrishnan, Maya K. Rao, Renu Regunathan-Shenk, Simone Sanna-Cherchi, Dominick Santoriello, Shayan Shirazian, Michael B. Stokes, Natalie Yu, Anthony M. Valeri, Ronald Zviti, Amira Al-Uzri, Josephine Ambruzs, Isa Ashoor, Diego Aviles, Rossana Baracco, John Barcia, Sharon Bartosh, Craig Belsha, Michael C. Braun, Yi Cai, Vladimir Chernitskiy, Aftab Chishti, Donna Claes, Kira Clark, Carl Cramer, Keefe Davis, Amy Dutcher, Elif Erkan, Daniel Feig, Michael Freundlich, Joseph Gaut, Rasheed Gbadegesin, Melisha Hanna, Guillermo Hidalgo, David Hooper, Tracy E. Hunley, Amrish Jain, Mahmoud Kallash, Margo Kamel, Myda Khalid, Theresa Kump, Jerome C. Lane, Helen Liapis, John Mahan, Nisha Mathews, Carla Nester, Cynthia Pan, Larry Patterson, Hiren Patel, Alice Raad, Cynthia Silva, Rajasree Sreedharan, Tarak Srivastava, Julia Steinke, Susan Sumner, Tetyana L. Vasylyeva, Chia-shi Wang, Donald J. Weaver, Craig S. Wong, Hong Yin, Anand Achanti, Salem Almaani, Isabelle Ayoub, Milos Budisavljevic, Maggie D'Angelo, Vimal Derebail, Huma Fatima, Ronald Falk, Agnes Fogo, Keisha Gibson, Dorey Glenn, Susan Hogan, Koyal Jain, Bruce Julian, Jason Kidd, H. Davis Massey, Amy Mottl, Shannon Murphy, Tibor Nadasdy, Jan Novak, Samir Parikh, Caroline Poulton, Thomas Brian Powell, Bryce Reeve, Matthew Renfrow, Monica Reynolds, Dana Rizk, Brad Rovin, Virginie Royal, Manish Saha, Neil Sanghani, Sally Self, Sharon Adler, Nada Alachkar, Charles Alpers, Raed Bou Matar, Carmen Avila-Casado, Serena Bagnasco, Emily Brede, Elizabeth Brown, Daniel Cattran, Michael Choi, Gabriel Contreras, Katherine M. Dell, Darren Dewalt, Michelle Denburg, Ram Dukkipati, Fernando C. Fervenza, Alessia Fornoni, Crystal Gadegbeku, Patrick Gipson, Anny Gonzalez-Zea, Leah Hasely, Elizabeth Hendren, Sangeeta Hingorani, Michelle Hladunewich, Jonathan Hogan, Jean Hou, J. Ashley Jefferson, Kenar Jhaveri, Duncan B. Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, Richard Lafayette, Kevin V. Lemley, Laura Malaga-Dieguez, Kevin Meyers, Alicia Neu, Michelle Marie O'Shaughnessy, John F. O’Toole, Andrea Oliverio, Matthew Palmer, Rulan Parekh, Renee Pitter, Heather Reich, Kimberly Reidy, Helbert Rondon, Kamalanathan K. Sambandam, Matthew Sampson, John R. Sedor, David T. Selewski, Christine B. Sethna, Jeffrey Schelling, John C. Sperati, Agnes Swiatecka-Urban, Katherine R. Tuttle, Meryl Waldman, Joseph Weisstuch, Roger Wiggins, David Williams, Cheryl Winkler, Eric Young, Olga Zhdanova, Charlotte Beil, Richard Eikstadt, Brenda Gillespie, John Graff, Stephen Hewitt, Emily Herreshoff, Chrysta Lienczewski, Sarah Mansfield, Laura Mariani, Keith McCullough, Nicholas Moore, Bruce M. Robinson, Melissa Sexton, Jonathan Troost, Matthew Wladkowski, Jarcy Zee, and Dawn Zinsser

Subjects
Adult, Male, medicine.medical_specialty, Henoch-Schonlein purpura, Adolescent, 030232 urology & nephrology, Disease, Glomerulonephritis, Membranous, Risk Assessment, Severity of Illness Index, Article, Nephropathy, Diagnosis, Differential, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Focal segmental glomerulosclerosis, Glomerulonephritis, Sex Factors, Membranous nephropathy, Internal medicine, medicine, Humans, Minimal change disease, 030212 general & internal medicine, Prospective Studies, Prospective cohort study, Child, Academic Medical Centers, business.industry, Glomerulosclerosis, Focal Segmental, Nephrosis, Lipoid, Biopsy, Needle, Age Factors, Glomerulonephritis, IGA, Middle Aged, medicine.disease, Prognosis, Immunohistochemistry, Survival Analysis, Nephrology, Multivariate Analysis, Disease Progression, Linear Models, Kidney Failure, Chronic, Female, business, Kidney disease
Abstract

Rationale & Objectives Glomerular diseases, including minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and immunoglobulin A (IgA) nephropathy, share clinical presentations, yet result from multiple biological mechanisms. Challenges to identifying underlying mechanisms, biomarkers, and new therapies include the rarity of each diagnosis and slow progression, often requiring decades to measure the effectiveness of interventions to prevent end-stage kidney disease (ESKD) or death. Study Design Multicenter prospective cohort study. Setting & Participants Cure Glomerulonephropathy (CureGN) will enroll 2,400 children and adults with minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (including IgA vasculitis) and a first diagnostic kidney biopsy within 5 years. Patients with ESKD and those with secondary causes of glomerular disease are excluded. Exposures Clinical data, including medical history, medications, family history, and patient-reported outcomes, are obtained, along with a digital archive of kidney biopsy images and blood and urine specimens at study visits aligned with clinical care 1 to 4 times per year. Outcomes Patients are followed up for changes in estimated glomerular filtration rate, disease activity, ESKD, and death and for nonrenal complications of disease and treatment, including infection, malignancy, cardiovascular, and thromboembolic events. Analytical Approach The study design supports multiple longitudinal analyses leveraging the diverse data domains of CureGN and its ancillary program. At 2,400 patients and an average of 2 years' initial follow-up, CureGN has 80% power to detect an HR of 1.4 to 1.9 for proteinuria remission and a mean difference of 2.1 to 3.0mL/min/1.73m2 in estimated glomerular filtration rate per year. Limitations Current follow-up can only detect large differences in ESKD and death outcomes. Conclusions Study infrastructure will support a broad range of scientific approaches to identify mechanistically distinct subgroups, identify accurate biomarkers of disease activity and progression, delineate disease-specific treatment targets, and inform future therapeutic trials. CureGN is expected to be among the largest prospective studies of children and adults with glomerular disease, with a broad goal to lessen disease burden and improve outcomes.

Published
2018

21. Health-related quality of life in glomerular disease

Author

Pietro A. Canetta, Jonathan P. Troost, Shannon Mahoney, Amy J. Kogon, Noelle Carlozzi, Sharon M. Bartosh, Yi Cai, T. Keefe Davis, Hilda Fernandez, Alessia Fornoni, Rasheed A. Gbadegesin, Emily Herreshoff, John D. Mahan, Patrick H. Nachman, David T. Selewski, Christine B. Sethna, Tarak Srivastava, Katherine R. Tuttle, Chia-shi Wang, Ronald J. Falk, Ali G. Gharavi, Brenda W. Gillespie, Larry A. Greenbaum, Lawrence B. Holzman, Matthias Kretzler, Bruce M. Robinson, William E. Smoyer, Lisa M. Guay-Woodford, Bryce Reeve, Debbie S. Gipson, Wooin Ahn, Gerald B. Appel, Revekka Babayev, Ibrahim Batal, Andrew S. Bomback, Eric Brown, Eric S. Campenot, Pietro Canetta, Lucrezia Carlassara, Brenda Chan, Debanjana Chatterjee, Vivette D. D’Agati, Elisa Delbarba, Samriti Dogra, Bartosz Foroncewicz, Gian Marco Ghiggeri, William H. Hines, S. Ali Husain, Namrata G. Jain, Pascale Khairallah, Byum Hee Kil, Krzysztof Kiryluk, Anushya Jeyabalan, Wai L. Lau, Fangming Lin, Francesca Lugani, Maddalena Marasa, Glen Markowitz, Sumit Mohan, Xueru Mu, Krzysztof Mucha, Thomas L. Nickolas, Stacy Piva, Jai Radhakrishnan, Maya K. Rao, Regunathan-Shenk Renu, Simone Sanna-Cherchi, Dominick Santoriello, Shayan Shirazian, Michael B. Stokes, Natalie Uy, Anthony M. Valeri, Amira Al-Uzri, Josephine Ambruzs, Isa Ashoor, Diego Aviles, Rossana Baracco, John Barcia, Sharon Bartosh, Craig Belsha, Corinna Bowers, Michael C. Braun, Vladimir Chernitskiy, Aftab Chishti, Donna Claes, Kira Clark, Carl Cramer, Keefe Davis, Elif Erkan, Daniel Feig, Michael Freundlich, Joseph Gaut, Rasheed Gbadegesin, Melisha Hanna, Guillermo Hidalgo, David Hooper, Tracy E. Hunley, Amrish Jain, Mahmoud Kallash, Margo Kamel, Myda Khalid, Jon B. Klein, Theresa Kump, Jerome C. Lane, Helen Liapis, John Mahan, Carla Nester, Cynthia Pan, Larry Patterson, Hiren Patel, Alice Raad, Adelaide Revell, Michelle N. Rheault, Cynthia Silva, Rajasree Sreedharan, Julia Steinke, Susan Sumner, Katherine Twombley, Scott E. Wenderfer, Tetyana L. Vasylyeva, Donald J. Weaver, Craig S. Wong, Hong Yin, Anand Achanti, Salem Almaani, Isabelle Ayoub, Milos Budisavljevic, Maggie D’Angelo, Huma Fatima, Ronald Falk, Agnes Fogo, Keisha Gibson, Dorey Glenn, Susan Hogan, J. Charles Jennette, Bruce Julian, Jason Kidd, Louis-Philippe Laurin, H. Davis Massey, Amy Mottl, Shannon Murphy, Patrick Nachman, Tibor Nadasdy, Jan Novak, Samir Parikh, Caroline Poulton, Thomas Brian Powell, Matthew Renfrow, Monica Reynolds, Dana Rizk, Brad Rovin, Virginie Royal, Neil Sanghani, Sally Self, Sharon Adler, Nada Alachkar, Charles Alpers, Raed Bou Matar, Carmen Avila-Casado, Serena Bagnasco, Emily Brede, Elizabeth Brown, Daniel Cattran, Michael Choi, Katherine M. Dell, Darren Dewalt, Michelle Denburg, Ram Dukkipati, Fernando C. Fervenza, Crystal Gadegbeku, Patrick Gipson, Anny Gonzalez-Zea, Leah Hasely, Elizabeth Hendren, Sangeeta Hingorani, Michelle Hladunewich, Jonathan Hogan, Jean Hou, J. Ashley Jefferson, Kenar Jhaveri, Duncan B. Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, Richard Lafayette, Kevin V. Lemley, Laura Malaga-Dieguez, Kevin Meyers, Alicia Neu, Michelle Marie O'Shaughnessy, John F. O’Toole, Andrea Oliverio, Matthew Palmer, Rulan Parekh, Renee Pitter, Heather Reich, Kimberly Reidy, Helbert Rondon, Kamalanathan K. Sambandam, Matthew Sampson, John R. Sedor, Jeffrey Schelling, John C. Sperati, Agnes Swiatecka-Urban, Howard Trachtman, Meryl Waldman, Joseph Weisstuch, Roger Wiggins, David Williams, Cheryl Winkler, Suzanne Vento, Eric Young, Olga Zhdanova, Laura Barisoni, Charlotte Beil, Richard Eikstadt, Brenda Gillespie, John Graff, Stephen Hewitt, Peg Hill-Callahan, Margaret Helmuth, Chrysta Lienczewski, Sarah Mansfield, Laura Mariani, Keith McCullough, Nicholas Moore, Cynthia C. Nast, Melissa Sexton, Jonathan Troost, Matthew Wladkowski, Jarcy Zee, and Dawn Zinsser

Subjects
0301 basic medicine, Adult, Male, medicine.medical_specialty, Adolescent, 030232 urology & nephrology, Renal function, Article, Nephropathy, 03 medical and health sciences, 0302 clinical medicine, Focal segmental glomerulosclerosis, Glomerulonephritis, Membranous nephropathy, Quality of life, Internal medicine, medicine, Edema, Humans, Minimal change disease, Longitudinal Studies, Child, Aged, business.industry, Middle Aged, medicine.disease, Obesity, humanities, 030104 developmental biology, Nephrology, Quality of Life, Anxiety, Female, Self Report, medicine.symptom, business
Abstract

There is scant literature describing the effect of glomerular disease on health-related quality of life (HRQOL). The Cure Glomerulonephropathy study (CureGN) is an international longitudinal cohort study of children and adults with four primary glomerular diseases (minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy). HRQOL is systematically assessed using items from the Patient-Reported Outcomes Measurement Informative System (PROMIS). We assessed the relationship between HRQOL and demographic and clinical variables in 478 children and 1115 adults at the time of enrollment into CureGN. Domains measured by PROMIS items included global assessments of health, mobility, anxiety, fatigue, and sleep impairment, as well as a derived composite measure incorporating all measured domains. Multivariable models were created that explained 7 to 32% of variance in HRQOL. Patient-reported edema consistently had the strongest and most robust association with each measured domain of HRQOL in multivariable analysis (adjusted β [95% CI] for composite PROMIS score in children, -5.2 [-7.1 to -3.4]; for composite PROMIS score in adults, -6.1 [-7.4 to -4.9]). Female sex, weight (particularly obesity), and estimated glomerular filtration rate were also associated with some, but not all, domains of HRQOL. Primary diagnosis, disease duration, and exposure to immunosuppression were not associated with HRQOL after adjustment. Sensitivity analyses and interaction testing demonstrated no significant association between disease duration or immunosuppression and any measured domain of HRQOL. Thus, patient-reported edema has a consistent negative association with HRQOL in patients with primary glomerular diseases, with substantially greater impact than other demographic and clinical variables.

Published
2018

22. Annual Congress of the Chinese Blood Purification Center Administration Committee. September 25-28, 2014, Guangzhou: Abstracts

Author

Giovanni Grazi, Jicheng Zhang, Sampson Antwi, Jonathan M. Rubin, Leonardo C Ribeiro, Hanjie Zhang, Jing Gao, Gioacchino Li Cavoli, Susana Roca, Rainer Himmele, M. Ted Ferris, Paolo Armignacco, Alberto Rosati, Anna Meyring-Wösten, William J. Federspiel, Nefroint investigators, Fansan Zhu, Corinne E. Zeller-Knuth, Jose A. Diaz-Buxo, Gianluca Villa, April Bowman, Laura Bucalo, Grant H. Kruger, Marianne Wilhelmi, Daniel Muñoz-Aguayo, Sarah Cohen, Alberto A.E. Bertelli, Kristi Bickford, Aldo Casani, Mar Orzáez, Ekou Niamien, Emma Arcos, Jeremy D. Kimmel, Rafael de la Torre, Carmela Zagarrigo, Florence Desrosiers, Beatriz Santamaría, A. Mary Vilay, Michael J. Germain, María Ángeles García, Michael Heung, Jwa-Kyung Kim, Rinaldo Bellomo, Giovanni Manca Rizza, Karina Javalkar, Isaac Teitelbaum, Onofrio Schillaci, J. Michael Yardman-Frank, Juan Jesus Carrero, Laurence Adonis-Koffy, Gérard Abel, Len A. Usvyat, M. Valles, Marina Abramyan, Renhua Lu, Jordi Calabia, William F. Weitzel, Massimo Antonelli, Elizabeth A. Hughson, A. Phillips, John A. Kellum, Kyu Hun Choi, Khaled Abdel-Kader, Oleh Akchurin, Aileen Grassman, Pessa Albert Coulibaly, Jose J. Zaragoza, Alexandra Ternier, Jessica Cuttance, Krotenko Np, Michael J. Somers, Matteo Ruggeri, Alberto Ortiz, Claudio Ronco, Craig S. Wong, Ludimila Guedim de Campos, Maggie Han, María J. Vicent, Manuel Molina Nuñez, Frederick J. Kaskel, Angel Celdrán, Francesco Garzotto, Panduranga Rao, Roberto Bigazzi, Paul Zabetakis, Corrado Bellini, Druckerei Stückle, Mattia Palmeri, Alvaro C. Ucero, Cristina Jimeno, Aashish Sharma, Audie Métayer, Isabel Villegas, Sabrina Paoletti, Sarbjit V. Jassal, Donald Maberry, Stanley Fan, Massimiliano Migliori, Xiaoqi Xu, Maribel Covas, Jean Baptiste Yaokreh, Jacob Volpe, Peter Kotanko, Morton Satin, Maria E. Ferris, Popov Da, Marina Plyushch, Morgan Lam, Mathias Schaller, Keisha L. Gibson, Alberto Benito-Martin, Melissa Mendoza, Michail Yaroustovsky, Dalia Yousif, Anna Lorenzin, Keisha Gibson, Vincenzo Panichi, Sung Kyu Ha, Adriana Di Giorgio, Jordi Comas, Marco Sartori, Tancredi Vincenzo Li Cavoli, Hyeong Cheon Park, Karin True, Judith Exantus, Jean-Hénold Buteau, Sung Jin Moon, Gnenefoly Diarrassouba, Dorey A. Glenn, Sanah Parvez, Mark Unruh, Rasha Hussein, Mikhail Artemyev, Viviane Calice-Silva, Jung Eun Lee, Zhiyong Peng, Ugo Rotolo, Isa F. Ashoor, Montserrat Fitó, Alan Vollmer, María Soledad Ros, Hans Oberleithner, Alessandro Sgambato, Renee-Claude Mercier, James A. Hamilton, Nathan W. Levin, Alessia Scatena, Mauro Neri, Paul Balter, Gero von Gersdorff, Kenneth R. Wilund, Ekaterina Rogalskaya, Soo Young Yoon, Elizabeth Ley Oei, Joseph L. Bull, Roberto Pecoits-Filho, Danielle Marcelli, Paul K. Whelton, Franca Servillo, Michael Etter, Rosa de Alarcón, Xianglong Wang, Shin Wook Kang, Rafael Selgas, Gracia Álvarez, Mi Kyung Song, Kerry Anne Rambaran, and Marta Ruiz-Ortega

Subjects
medicine.medical_specialty, Nephrology, business.industry, Emergency medicine, medicine, Blood purification, Center (algebra and category theory), Hematology, General Medicine, business, Administration (government)
Published
2015
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