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4. Implementation and Feasibility of Clinical Genome Sequencing Embedded Into the Outpatient Nephrology Care for Patients With Proteinuric Kidney Disease

Author

Marasa, Maddalena, Ahram, Dina F., Rehman, Atteeq U., Mitrotti, Adele, Abhyankar, Avinash, Jain, Namrata G., Weng, Patricia L., Piva, Stacy E., Fernandez, Hilda E., Uy, Natalie S., Chatterjee, Debanjana, Kil, Byum H., Nestor, Jordan G., Felice, Vanessa, Robinson, Dino, Whyte, Dilys, Gharavi, Ali G., Appel, Gerald B., Radhakrishnan, Jai, Santoriello, Dominick, Bomback, Andrew, Lin, Fangming, D’Agati, Vivette D., Jobanputra, Vaidehi, and Sanna-Cherchi, Simone

Published
2023
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5. Clinicopathologic Spectrum of Lysozyme-Associated Nephropathy

Author

Kudose, Satoru, Cossey, L. Nicholas, Canetta, Pietro A., Sekulic, Miroslav, Vanbeek, Christine A., Huls, Forest B., Gupta, Isha, Bu, Lihong, Alexander, Mariam P., Cornell, Lynn D., Fidler, Mary E., Markowitz, Glen S., Larsen, Christopher P., D’Agati, Vivette D., Nasr, Samih H., and Santoriello, Dominick

Published
2023
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7. Diagnostic Approach to Glomerulonephritis With Fibrillar IgG Deposits and Light Chain Restriction

Author

Satoru Kudose, Pietro Canetta, Nicole K. Andeen, M. Barry Stokes, Ibrahim Batal, Glen S. Markowitz, Vivette D. D’Agati, and Dominick Santoriello

Subjects
fibrillary, fibrillary glomerulonephritis, immunotactoid glomerulonephritis, monotypic, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Introduction: The pathologic approach to glomerulonephritis (GN) with fibrillar IgG deposits and light chain restriction remains a diagnostic challenge. Method: All GN with fibrillar deposits of IgG and apparent light chain restriction on standard immunofluorescence on frozen tissue (IF-F) accessioned at the Columbia Renal Pathology Laboratory from 2012 to 2019 were identified. Additional studies including staining for Congo red, DNAJB9, IgG subtypes, and immunofluorescence on pronase-digested paraffin sections (IF-P) were performed. Result: Based on the results, biopsy samples were reclassified as polytypic DNAJB9-positive fibrillary glomerulonephritis (pFGN, n = 14), monotypic DNAJB9-positive FGN (mFGN, n = 7), GN with polytypic DNAJB9-negative fibrillar IgG deposits (n = 2), and GN with monotypic DNAJB9-negative fibrillar IgG deposits (n = 6). Among DNAJB9-positive FGN samples, IgG subtype staining was able to exclude monotypic deposits by demonstrating reactivity for ≥2 IgG subtypes (usually IgG1 and IgG4) in 67% (14 of 21), including 9 that would have been misclassified as monotypic by IF-F and IF-P alone. Monotypic DNAJB9-positive fibrillary glomerulonephritis (FGN) was not associated with monoclonal gammopathy in 5 of 6 patients. GN with monotypic DNAJB9-negative fibrillar IgG deposits exhibited focal parallel fibril alignment and frequent association with chronic lymphocytic leukemia, but lacked the diagnostic microtubules of immunotactoid GN. Conclusion: A systematic diagnostic approach with ancillary techniques is essential for proper classification and assignment of monoclonal gammopathy of renal significance status in cases of GN with fibrillary IgG deposits and light chain restriction by IF-F.

Published
2021
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8. Impact of Deceased Donor Kidney Procurement Biopsy Technique on Histologic Accuracy

Author

Husain, S. Ali, Shah, Vaqar, Alvarado Verduzco, Hector, King, Kristen L., Brennan, Corey, Batal, Ibrahim, Coley, Shana M., Hall, Isaac E., Stokes, M. Barry, Dube, Geoffrey K., Crew, R. John, Perotte, Adler, Natarajan, Karthik, Carpenter, Dustin, Sandoval, P. Rodrigo, Santoriello, Dominick, D’Agati, Vivette, Cohen, David J., Ratner, Lloyd, Markowitz, Glen, and Mohan, Sumit

Published
2020
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9. Longitudinal Changes in Health-Related Quality of Life in Primary Glomerular Disease: Results From the CureGN Study

Author

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

Published
2020
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10. Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Author

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

Published
2020
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11. 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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13. Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

Author

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

Published
2018
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14. Lysozyme-Induced Nephropathy

Author

Dominick Santoriello, Laurie May Andal, Robert Cox, Vivette D. D’Agati, and Glen S. Markowitz

Subjects
Diseases of the genitourinary system. Urology, RC870-923
Published
2017
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17. Clinicopathologic Spectrum of Lysozyme-Associated Nephropathy

Author

Satoru Kudose, L. Nicholas Cossey, Pietro A. Canetta, Miroslav Sekulic, Christine A. Vanbeek, Forest B. Huls, Isha Gupta, Lihong Bu, Mariam P. Alexander, Lynn D. Cornell, Mary E. Fidler, Glen S. Markowitz, Christopher P. Larsen, Vivette D. D’Agati, Samih H. Nasr, and Dominick Santoriello

Subjects
Nephrology
Published
2023
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19. Implementation and feasibility of clinical genome sequencing embedded into the outpatient nephrology care for patients with proteinuric kidney disease

Author

Maddalena Marasa, Dina F. Ahram, Atteeq U. Rehman, Adele Mitrotti, Avinash Abhyankar, Namrata G. Jain, Patricia L. Weng, Stacy E. Piva, Hilda E. Fernandez, Natalie S. Uy, Debanjana Chatterjee, Byum H. Kil, Jordan G. Nestor, Vanessa Felice, Dino Robinson, Dilys Whyte, Ali G. Gharavi, Gerald B. Appel, Jai Radhakrishnan, Dominick Santoriello, Andrew Bomback, Fangming Lin, Vivette D. D’Agati, Vaidehi Jobanputra, and Simone Sanna-Cherchi

Subjects
Nephrology
Published
2023
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20. Diagnostic Approach to Glomerulonephritis With Fibrillar IgG Deposits and Light Chain Restriction

Author

Pietro A. Canetta, Glen S. Markowitz, Satoru Kudose, Vivette D. D'Agati, Ibrahim Batal, Nicole K. Andeen, M. Barry Stokes, and Dominick Santoriello

Subjects
Pathology, medicine.medical_specialty, Chronic lymphocytic leukemia, 030232 urology & nephrology, 030204 cardiovascular system & hematology, lcsh:RC870-923, Immunoglobulin light chain, Immunofluorescence, 03 medical and health sciences, 0302 clinical medicine, monotypic, Clinical Research, Biopsy, medicine, fibrillary, medicine.diagnostic_test, business.industry, Fibrillary Glomerulonephritis, Glomerulonephritis, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, Staining, Renal pathology, Nephrology, business, fibrillary glomerulonephritis, immunotactoid glomerulonephritis
Abstract

Introduction The pathologic approach to glomerulonephritis (GN) with fibrillar IgG deposits and light chain restriction remains a diagnostic challenge. Method All GN with fibrillar deposits of IgG and apparent light chain restriction on standard immunofluorescence on frozen tissue (IF-F) accessioned at the Columbia Renal Pathology Laboratory from 2012 to 2019 were identified. Additional studies including staining for Congo red, DNAJB9, IgG subtypes, and immunofluorescence on pronase-digested paraffin sections (IF-P) were performed. Result Based on the results, biopsy samples were reclassified as polytypic DNAJB9-positive fibrillary glomerulonephritis (pFGN, n = 14), monotypic DNAJB9-positive FGN (mFGN, n = 7), GN with polytypic DNAJB9-negative fibrillar IgG deposits (n = 2), and GN with monotypic DNAJB9-negative fibrillar IgG deposits (n = 6). Among DNAJB9-positive FGN samples, IgG subtype staining was able to exclude monotypic deposits by demonstrating reactivity for ≥2 IgG subtypes (usually IgG1 and IgG4) in 67% (14 of 21), including 9 that would have been misclassified as monotypic by IF-F and IF-P alone. Monotypic DNAJB9-positive fibrillary glomerulonephritis (FGN) was not associated with monoclonal gammopathy in 5 of 6 patients. GN with monotypic DNAJB9-negative fibrillar IgG deposits exhibited focal parallel fibril alignment and frequent association with chronic lymphocytic leukemia, but lacked the diagnostic microtubules of immunotactoid GN. Conclusion A systematic diagnostic approach with ancillary techniques is essential for proper classification and assignment of monoclonal gammopathy of renal significance status in cases of GN with fibrillary IgG deposits and light chain restriction by IF-F., Graphical abstract

Published
2021

23. Impact of Deceased Donor Kidney Procurement Biopsy Technique on Histologic Accuracy

Author

R. John Crew, David J. Cohen, Shana M. Coley, Dustin Carpenter, Glen S. Markowitz, Isaac E. Hall, Vivette D. D'Agati, Lloyd E. Ratner, S. Ali Husain, Geoffrey K. Dube, Adler J. Perotte, Vaqar H. Shah, Dominick Santoriello, Hector Alvarado Verduzco, Corey Brennan, Sumit Mohan, Ibrahim Batal, P. Rodrigo Sandoval, Kristen L. King, Karthik Natarajan, and M. Barry Stokes

Subjects
medicine.medical_specialty, deceased donor kidney transplant, Concordance, 030232 urology & nephrology, 030204 cardiovascular system & hematology, organ utilization, 03 medical and health sciences, 0302 clinical medicine, Procurement, Clinical Research, Biopsy, organ quality, Medicine, Deceased donor kidney, medicine.diagnostic_test, business.industry, Vascular disease, Glomerulosclerosis, Retrospective cohort study, medicine.disease, kidney procurement biopsy, kidney transplant outcomes, Nephrology, Relative risk, epidemiology, Radiology, business
Abstract

Introduction The factors that influence deceased donor kidney procurement biopsy reliability are not well established. We examined the impact of biopsy technique and pathologist training on procurement biopsy accuracy. Methods We retrospectively identified all deceased donor kidney-only transplants at our center from 2006 to 2016 with both procurement and reperfusion biopsies performed and information available on procurement biopsy technique and pathologist (n = 392). Biopsies were scored using a previously validated system, classifying “suboptimal” histology as the presence of at least 1 of the following: glomerulosclerosis ≥11%, moderate/severe interstitial fibrosis/tubular atrophy, or moderate/severe vascular disease. We calculated relative risk ratios (RRR) to determine the influence of technique (core vs. wedge) and pathologist (renal vs. nonrenal) on concordance between procurement and reperfusion biopsy histologic classification. Results A total of 171 (44%) procurement biopsies used wedge technique, and 221 (56%) used core technique. Results of only 36 biopsies (9%) were interpreted by renal pathologists. Correlation between procurement and reperfusion glomerulosclerosis was poor for both wedge (r2 = 0.11) and core (r2 = 0.14) biopsies. Overall, 34% of kidneys had discordant classification on procurement versus reperfusion biopsy. Neither biopsy technique nor pathologist training was associated with concordance between procurement and reperfusion histology, but a larger number of sampled glomeruli was associated with a higher likelihood of concordance (adjusted RRR = 1.12 per 10 glomeruli, 95% confidence interval = 1.04−1.22). Conclusions Biopsy technique and pathologist training were not associated with procurement biopsy histologic accuracy in this retrospective study. Prospective trials are needed to determine how to optimize procurement biopsy practices., Graphical abstract

Published
2020
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24. 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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27. Collapsing Glomerulopathy Associated With Hydrophilic Polymer Emboli

Author

Satoru Kudose, Dominick Santoriello, Vivette D. D'Agati, and Emmanuel A. Adomako

Subjects
Gastrointestinal tract, Pathology, medicine.medical_specialty, Endothelium, business.industry, Collapsing glomerulopathy, Vasospasm, medicine.disease, medicine.anatomical_structure, Hydrophilic polymers, Biodegradable coating, Nephrology, medicine, business, Nephrology Round, Renal embolization
Abstract

Hydrophilic polymer is a synthetic, biodegradable coating applied to various intravascular devices and vascular grafts to reduce friction between instruments and endothelium, in an effort to prevent vasospasm and thrombosis.1, 2 However, the hydrophilic polymer itself can dislodge from these devices resulting in downstream ischemic complications involving brain, heart, lungs, kidneys, gastrointestinal tract, or skin.1, 2, 3, 4, 5, 6, 7, 8, 9,S1–S9 To date, 3 cases of hydrophilic polymer emboli have been reported to involve the kidneys, but none has been associated with collapsing glomerulopathy.4,S1,S6 Here, we report the first case of collapsing glomerulopathy temporally related to renal embolization by hydrophilic polymer.

Published
2018

28. Persistent Hepatitis C Virus−Associated Cryoglobulinemic Glomerulonephritis in Patients Successfully Treated With Direct-Acting Antiviral Therapy

Author

Jai Radhakrishnan, Shawn Dhupar, Dominick Santoriello, Nanda K Pullela, Kalpana Uday, Glen S. Markowitz, and Vivette D. D'Agati

Subjects
0301 basic medicine, business.industry, Hepatitis C virus, Antiviral therapy, medicine.disease_cause, Virology, 03 medical and health sciences, Cryoglobulinemic Glomerulonephritis, 030104 developmental biology, 0302 clinical medicine, Nephrology, Medicine, 030211 gastroenterology & hepatology, In patient, business, Direct acting, Nephrology Round
Published
2018

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

Author

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

Published
2020
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31. Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Author

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

Published
2020
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32. 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

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

Author

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

Published
2018
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36. Lysozyme-Induced Nephropathy

Author

Vivette D. D'Agati, Dominick Santoriello, Glen S. Markowitz, Robert Cox, and Laurie May Andal

Subjects
Pathology, medicine.medical_specialty, urogenital system, business.industry, 030232 urology & nephrology, Acute kidney injury, Chronic myelomonocytic leukemia, lcsh:Diseases of the genitourinary system. Urology, lcsh:RC870-923, urologic and male genital diseases, medicine.disease, Nephropathy, Tumor lysis syndrome, 03 medical and health sciences, Leukemia, 0302 clinical medicine, Nephrology, 030220 oncology & carcinogenesis, medicine, Monocytic leukemia, business, Obstructive uropathy, Nephrology Round, Kidney disease
Abstract

Leukemia is among the most common malignancies associated with hospital admissions for acute kidney injury (AKI).1, 2 AKI develops in up to one-third of patients affected by hematologic malignancy, particularly in older patients with prior chronic kidney disease (CKD),3 and has been shown to have an adverse effect on long-term prognosis in this patient population.4 Intravascular volume depletion, tumor lysis syndrome, and drug-induced acute tubular injury are among the most common causes of leukemia-associated AKI.4, 5 Radiologic studies may be useful to exclude obstructive uropathy secondary to retroperitoneal lymphadenopathy or tumorous masses. Direct infiltration of the renal parenchyma by leukemic cells, although a common finding at autopsy,5 is only rarely associated with the development of symptomatic AKI.4 Rare cases of AKI secondary to intravascular leukostasis have also been described, particularly in patients with white blood cell counts greater than 100,000/mm3.6 Lysozyme-induced nephropathy (LyN) is a rare and underrecognized complication of chronic myelomonocytic leukemia (CMML) and other forms of monocytic leukemia in which lysozyme, a small cationic protein, is released into the circulation, filtered by the glomerulus and reabsorbed by the proximal tubule, causing toxic tubular injury.7

Published
2016
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37. Lysozyme-Induced Nephropathy

Author

Santoriello, Dominick, primary, Andal, Laurie May, additional, Cox, Robert, additional, D’Agati, Vivette D., additional, and Markowitz, Glen S., additional

Published
2017
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