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1. Developing an Edema Clinician-Reported Outcome Measure for Nephrotic Syndrome

Author

Debbie S. Gipson, Maisha Pal, Hailey Desmond, Charles Anderson, Liron Walsh, Howard Trachtman, Susan F. Massengill, Patrick Gipson, Panduranga S. Rao, Joshua Thurman, Jeffrey Kopp, Elaine Kamil, Jennifer Lamothe, Laura H Mariani, Paula LaFleur, Suzanne Vento, Michelle O’Shaughnessy, Youssef M.K. Farag, Christine Simon, and Noelle E. Carlozzi

Subjects
Diseases of the endocrine glands. Clinical endocrinology, RC648-665
Abstract

Introduction: Edema is a common manifestation of proteinuric kidney diseases but there is no consensus approach for reliably evaluating edema. The objective of this study was to develop an edema clinician-reported outcome measure for use in patients with nephrotic syndrome. Methods: A literature review was conducted to assess existing clinician-rated measures of edema. Clinical experts were recruited from internal medicine nephrology and pediatric nephrology practices to participate in concept elicitation using semi-structured interviews and cognitive debriefing. Qualitative analysis methods were used to collate expert input and inform measurement development. In addition, training and assessment modules were developed using an iterative process that also utilized expert input and cognitive debriefing to ensure interrater reliability. Results: While several clinician-rated measures of edema have been proposed, our literature review did not identify any studies to support the reliability or validity of these measures. Fourteen clinician experts participated in the concept elicitation interviews and twelve participated in cognitive debriefing. A clinician-reported outcome measure for edema was developed. The measure assesses edema severity in multiple individual body parts. An online training module and assessment tool was generated and refined using additional clinician input and investigative team expertise. Conclusion: The Edema ClinRO (V1) measure is developed specifically to measure edema in nephrotic syndrome. The tool assesses edema across multiple body parts, and it includes a training module to ensure standardized administration across raters. Future examination of this measure is ongoing to establish its reliability and validity.

Published
2023
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2. Longitudinal Changes in Health-Related Quality of Life in Primary Glomerular Disease: Results From the CureGN Study

Author

Shannon L. Murphy, John D. Mahan, Jonathan P. Troost, Tarak Srivastava, Amy J. Kogon, Yi Cai, T. Keefe Davis, Hilda Fernandez, Alessia Fornoni, Rasheed A. Gbadegesin, Emily Herreshoff, Pietro A. Canetta, Patrick H. Nachman, Bryce B. Reeve, David T. Selewski, Christine B. Sethna, Chia-shi Wang, Sharon M. Bartosh, Debbie S. Gipson, Katherine R. Tuttle, Ali Gharavi, Wooin Ahn, Gerald B. Appel, Rupali S. Avasare, Revekka Babayev, Ibrahim Batal, Andrew S. Bomback, Eric Brown, Eric S. Campenot, Pietro Canetta, Brenda Chan, Vivette D. D’Agati, Bartosz Foroncewicz, Gian Marco Ghiggeri, William H. Hines, Namrata G. Jain, Krzysztof Kiryluk, Fangming Lin, Francesca Lugani, Maddalena Marasa, Glen Markowitz, Sumit Mohan, Krzysztof Mucha, Thomas L. Nickolas, Jai Radhakrishnan, Maya K. Rao, Renu Regunathan-Shenk, 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, Michael C. Braun, Aftab Chishti, Donna Claes, Carl Cramer, Keefe Davis, Elif Erkan, Daniel Feig, Michael Freundlich, Melisha Hanna, Guillermo Hidalgo, Amrish Jain, Myda Khalid, Mahmoud Kallash, MD, Jerome C. Lane, John Mahan, Nisha Mathews, Carla Nester, Cynthia Pan, Hiren Patel, Adelaide Revell, Rajasree Sreedharan, Julia Steinke, Scott E. Wenderfer, Craig S. Wong, Ronald Falk, William Cook, Vimal Derebail, Agnes Fogo, Adil Gasim, Todd Gehr, Raymond Harris, Jason Kidd, Louis-Philippe Laurin, Will Pendergraft, Vincent Pichette, Thomas Brian Powell, Matthew B. Renfrow, Virginie Royal, Lawrence B. Holzman, Sharon Adler, Charles Alpers, Raed Bou Matar, Elizabeth Brown, Michael Choi, Katherine M. Dell, Ram Dukkipati, Fernando C. Fervenza, Crystal Gadegbeku, Patrick Gipson, Leah Hasely, Sangeeta Hingorani, Michelle A. Hladunewich, Jonathan Hogan, J. Ashley Jefferson, Kenar Jhaveri, Duncan B. Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, 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, Jeffrey Schelling, John C. Sperati, Agnes Swiatecka-Urban, Howard Trachtman, Joseph Weisstuch, Olga Zhdanova, Brenda Gillespie, Matthias Kretzler, Bruce M. Robinson, Laura Mariani, Matthew Wladkowski, and Lisa M. Guay-Woodford

Subjects
edema, health-related quality of life, patient-reported outcomes, primary glomerular disease, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Introduction: Prior cross-sectional studies suggest that health-related quality of life (HRQOL) worsens with more severe glomerular disease. This longitudinal analysis was conducted to assess changes in HRQOL with changing disease status. Methods: Cure Glomerulonephropathy (CureGN) is a cohort of patients with minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, IgA vasculitis, or IgA nephropathy. HRQOL was assessed at enrollment and follow-up visits 1 to 3 times annually for up to 5 years with the Patient-Reported Outcomes Measurement Information System (PROMIS). Global health, anxiety, and fatigue domains were measured in all; mobility was measured in children; and sleep-related impairment was measured in adults. Linear mixed effects models were used to evaluate HRQOL responsiveness to changes in disease status. Results: A total of 469 children and 1146 adults with PROMIS scores were included in the analysis. HRQOL improved over time in nearly all domains, though group-level changes were modest. Edema was most consistently associated with worse HRQOL across domains among children and adults. A greater number of symptoms also predicted worse HRQOL in all domains. Sex, age, obesity, and serum albumin were associated with some HRQOL domains. The estimated glomerular filtration rate (eGFR) was only associated with fatigue and adult physical health; proteinuria was not associated with any HRQOL domain in adjusted models. Conclusion: HRQOL measures were responsive to changes in disease activity, as indicated by edema. HRQOL over time was not predicted by laboratory-based markers of disease. Patient-reported edema and number of symptoms were the strongest predictors of HRQOL, highlighting the importance of the patient experience in glomerular disease. HRQOL outcomes inform understanding of the patient experience for children and adults with glomerular diseases.

Published
2020
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3. Time to Initiation of Antihypertensive Therapy After Onset of Elevated Blood Pressure in Patients With Primary Proteinuric Kidney Disease

Author

Donald J. Weaver, Jr., Anne Waldo, Gia J. Oh, Elaine S. Kamil, Matthew Elliott, Sharon Adler, Anne Pesenson, Meg M. Modes, Patrick Gipson, Richard A. Lafayette, David T. Selewski, Samara E. Attalla, Richard Eikstadt, Jonathan P. Troost, Debbie S. Gipson, and Susan F. Massengill

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

Rationale & Objective: The objective of the study was to estimate the prevalence of hypertension in patients with proteinuric kidney disease and evaluate blood pressure (BP) control. Study Design: Retrospective cohort study. Setting & Participants: Data from adults and children with proteinuric kidney disease enrolled in the multicenter Kidney Research Network Registry were used for this study. Exposure: Proteinuric kidney disease. Outcomes: Hypertension and BP control. Analytical Approach: Patients with white-coat hypertension were excluded. Patients were censored at end-stage kidney disease onset. Patients were defined as hypertensive either by hypertension diagnosis code, having 2 or more encounters with elevated BPs, or treatment with antihypertensive therapy excluding renin-angiotensin-aldosterone system blockade. Elevated BP was defined as greater than 95th percentile for children and >140/90 mm Hg in adults. Sustained BP control was defined as 2 or more consecutive encounters with BPs lower than 95th percentile for children and

Published
2020
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4. 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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5. 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

6. 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

7. 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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8. 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

9. Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

Author

David T. Selewski, Josephine M. Ambruzs, Gerald B. Appel, Andrew S. Bomback, Raed Bou Matar, Yi Cai, Daniel C. Cattran, Aftab S. Chishti, Vivette D. D'Agati, Cynthia J. D'Alessandri-Silva, Rasheed A. Gbadegesin, Jonathan J. Hogan, Sandra Iragorri, J. Charles Jennette, Bruce A. Julian, Myda Khalid, Richard A. Lafayette, Helen Liapis, Francesca Lugani, Sarah A. Mansfield, Sherene Mason, Patrick H. Nachman, Cynthia C. Nast, Carla M. Nester, Damien G. Noone, Jan Novak, Michelle M. O'Shaughnessy, Heather N. Reich, Michelle N. Rheault, Dana V. Rizk, Manish K. Saha, Neil S. Sanghani, C. John Sperati, Rajasree Sreedharan, Tarak Srivastava, Agnieszka Swiatecka-Urban, Katherine Twombley, Tetyana L. Vasylyeva, Donald J. Weaver, Hong Yin, Jarcy Zee, Ronald J. Falk, Ali G. Gharavi, Brenda W. Gillespie, Debbie S. Gipson, Larry A. Greenbaum, Lawrence B. Holzman, Matthias Kretzler, Bruce M. Robinson, William E. Smoyer, Michael Flessner, Lisa M. Guay-Woodford, Krzysztof Kiryluk, Ali Gharavi, Wooin Ahn, Rupali S. Avasare, 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, Fangming Lin, Maddalena Marasa, Glen Markowitz, Sumit Mohan, Krzysztof Mucha, Thomas L. Nickolas, Jai Radhakrishnan, Maya K. Rao, Renu Regunathan-Shenk, Simone Sanna-Cherchi, Dominick Santoriello, Michael B. Stokes, Natalie Yu, Anthony M. Valeri, Ronald Zviti, Amira Al-Uzri, Isa Ashoor, Diego Aviles, Rossana Baracco, John Barcia, Sharon Bartosh, Craig Belsha, Michael C. Braun, Aftab Chishti, Donna Claes, Carl Cramer, Keefe Davis, Elif Erkan, Daniel Feig, Michael Freundlich, Melisha Hanna, Guillermo Hidalgo, Amrish Jain, Mahmoud Kallash, Jerome C. Lane, John Mahan, Nisha Mathews, Carla Nester, Cynthia Pan, Hiren Patel, Adelaide Revell, Julia Steinke, Scott E. Wenderfer, Craig S. Wong, Ronald Falk, William Cook, Vimal Derebail, Agnes Fogo, Adil Gasim, Todd Gehr, Raymond Harris, Jason Kidd, Louis-Philippe Laurin, Will Pendergraft, Vincent Pichette, Thomas Brian Powell, Matthew B. Renfrow, Virginie Royal, Sharon Adler, Charles Alpers, 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 A. Hladunewich, Jonathan Hogan, J. Ashley Jefferson, Kenar Jhaveri, Duncan B. Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, 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, Christine B. Sethna, Jeffrey Schelling, Agnes Swiatecka-Urban, Howard Trachtman, Katherine R. Tuttle, Joseph Weisstuch, Olga Zhdanova, Brenda Gillespie, Laura Barisoni, Sarah Mansfield, Laura Mariani, and Matthew Wladkowski

Subjects
medicine.medical_specialty, 030232 urology & nephrology, Disease, 030204 cardiovascular system & hematology, urologic and male genital diseases, Nephropathy, 03 medical and health sciences, 0302 clinical medicine, Focal segmental glomerulosclerosis, Membranous nephropathy, Clinical Research, Internal medicine, IgA nephropathy (IgAN), Biopsy, medicine, Minimal change disease, medicine.diagnostic_test, business.industry, IgA vasculitis (IgAV), medicine.disease, 3. Good health, IgA vasculitis, Nephrology, Cohort, business, glomerulonephritis, Henoch-Schönlein purpura (HSP)
Abstract

Introduction The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients. Methods Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment. Results A total of 667 patients (506 IgAN, 161 IgAV) constitute the IgAN/IgAV cohort (382 adults, 285 children). At biopsy, those with IgAV were younger (13.0 years vs. 29.6 years, P < 0.001), more frequently white (89.7% vs. 78.9%, P = 0.003), had a higher estimated glomerular filtration rate (103.5 vs. 70.6 ml/min per 1.73 m2, P < 0.001), and lower serum albumin (3.4 vs. 3.8 g/dl, P < 0.001) than those with IgAN. Adult and pediatric individuals with IgAV were more likely than those with IgAN to have been treated with immunosuppressive therapy at or prior to enrollment (79.5% vs. 54.0%, P < 0.001). Conclusion This report highlights clinical differences between IgAV and IgAN and between children and adults with these diagnoses. We identified differences in treatment with immunosuppressive therapies by disease type. This description of baseline characteristics will serve as a foundation for future CureGN studies.

Published
2018

10. 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

11. Predictors of post-hospitalization recovery of renal function among patients with acute kidney injury requiring dialysis

Author

Russell, Pajewski, Patrick, Gipson, and Michael, Heung

Subjects
Hospitalization, Male, Renal Dialysis, Humans, Kidney Failure, Chronic, Female, Recovery of Function, Acute Kidney Injury, Middle Aged, Retrospective Studies
Abstract

Acute kidney injury (AKI) requiring dialysis complicates 1% of all hospital admissions, and up to 30% of survivors will still require dialysis at hospital discharge. There is a paucity of data to describe the postdischarge outcomes or to guide evidence-based dialysis management of this vulnerable population.Single-center, retrospective analysis of 100 consecutive patients with AKI who survived to hospital discharge and required outpatient dialysis. Data collection included baseline characteristics, hospitalization characteristics, and outpatient dialysis treatment variables. Primary outcome was dialysis independence 90 days after discharge.Overall, 43% of patients recovered adequate renal function to discontinue dialysis, with the majority recovering within 30 days post discharge. Worse baseline renal function was associated with lower likelihood of renal recovery. In the first week postdischarge, patients with subsequent nonrecovery of renal function had greater net fluid removal (5.3 vs. 4.1 L, P = 0.037), higher ultrafiltration rates (6.0 vs. 4.7 mL/kg/h, P = 0.041) and more frequent intradialytic hypotension (24.6% vs. 9.3% with 3 or more episodes, P = 0.049) compared to patients that later recovered.A significant proportion of AKI survivors will recover renal function following discharge. Outpatient intradialytic factors may influence subsequent renal function recovery.

Published
2017

12. The Phenotypic Spectrum of Nephropathies Associated with Mutations in Diacylglycerol Kinase epsilon

Author

Karolis, Azukaitis, Eva, Simkova, Mohammad Abdul, Majid, Matthias, Galiano, Kerstin, Benz, Kerstin, Amann, Clemens, Bockmeyer, Radha, Gajjar, Kevin E, Meyers, Hae Il, Cheong, Bärbel, Lange-Sperandio, Therese, Jungraithmayr, Véronique, Frémeaux-Bacchi, Carsten, Bergmann, Csaba, Bereczki, Monika, Miklaszewska, Dorottya, Csuka, Zoltán, Prohászka, Paul, Killen, Patrick, Gipson, Matthew G, Sampson, Mathieu, Lemaire, and Franz, Schaefer

Subjects
Male, 0301 basic medicine, Diacylglycerol Kinase, medicine.medical_specialty, Thrombotic microangiopathy, Glomerulonephritis, Membranoproliferative, DNA Mutational Analysis, urologic and male genital diseases, Gastroenterology, Nephropathy, 03 medical and health sciences, Clinical Research, Internal medicine, hemic and lymphatic diseases, Atypical hemolytic uremic syndrome, Membranoproliferative glomerulonephritis, medicine, Humans, Atypical Hemolytic Uremic Syndrome, Proteinuria, business.industry, Genetic heterogeneity, Incidence, Infant, Lithuania, General Medicine, medicine.disease, Pathophysiology, female genital diseases and pregnancy complications, Complement system, Phenotype, 030104 developmental biology, Nephrology, Child, Preschool, Female, Erratum, medicine.symptom, business
Abstract

The recent discovery of mutations in the gene encoding diacylglycerol kinase epsilon (DGKE) identified a novel pathophysiologic mechanism leading to HUS and/or MPGN. We report ten new patients from eight unrelated kindreds with DGKE nephropathy. We combined these cases with all previously published cases to characterize the phenotypic spectrum and outcomes of this new disease entity. Most patients presented with HUS accompanied by proteinuria, whereas a subset of patients exhibited clinical and histologic patterns of MPGN without TMA. We also report the first two patients with clinical and histologic HUS/MPGN overlap. DGKE-HUS typically manifested in the first year of life but was not exclusively limited to infancy, and viral triggers frequently preceded HUS episodes. We observed signs of complement activation in some patients with DGKE-HUS, but the role of complement activation remains unclear. Most patients developed a slowly progressive proteinuric nephropathy: 80% of patients did not have ESRD within 10 years of diagnosis. Many patients experienced HUS remission without specific treatment, and a few patients experienced HUS recurrence despite complete suppression of the complement pathway. Five patients received renal allografts, with no post-transplant recurrence reported. In conclusion, we did not observe a clear genotype-phenotype correlation in patients with DGKE nephropathy, suggesting additional factors mediating phenotypic heterogeneity. Furthermore, the benefits of anti-complement therapy are questionable but renal transplant may be a feasible option in the treatment of patients with this condition.

Published
2017

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