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1. Hyperphosphatemia and its relationship with blood pressure, vasoconstriction, and endothelial cell dysfunction in hypertensive hemodialysis patients

Author

Jinwoo Jung, Haekyung Jeon-Slaughter, Hang Nguyen, Jiten Patel, Kamalanathan K. Sambandam, Shani Shastri, and Peter Noel Van Buren

Subjects
Phosphate, Hemodialysis, Endothelial cell dysfunction, Mineral bone disease, Vasoconstriction, Diseases of the genitourinary system. Urology, RC870-923
Abstract

Abstract Background Hyperphosphatemia occurs frequently in end-stage renal disease patients on hemodialysis and is associated with increased mortality. Hyperphosphatemia contributes to vascular calcification in these patients, but there is emerging evidence that it is also associated with endothelial cell dysfunction. Methods We conducted a cross-sectional study in hypertensive hemodialysis patients. We obtained pre-hemodialysis measurements of total peripheral resistance index (TPRI, non-invasive cardiac output monitor) and plasma levels of endothelin-1 (ET-1) and asymmetric dimethylarginine (ADMA). We ascertained the routine peridialytic blood pressure (BP) measurements from that treatment and the most recent pre-hemodialysis serum phosphate levels. We used generalized linear regression analyses to determine independent associations between serum phosphate with BP, TPRI, ET-1, and ADMA while controlling for demographic variables, parathyroid hormone (PTH), and interdialytic weight gain. Results There were 54 patients analyzed. Mean pre-HD supine and seated systolic and diastolic BP were 164 (27), 158 (21), 91.5 (17), and 86.1 (16) mmHg. Mean serum phosphate was 5.89 (1.8) mg/dL. There were significant correlations between phosphate with all pre-hemodialysis BP measurements (r = 0.3, p = .04; r = 0.4, p = .002; r = 0.5, p

Published
2022
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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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4. Precision nephrology identified tumor necrosis factor activation variability in minimal change disease and focal segmental glomerulosclerosis

Author

Mariani, Laura H., Eddy, Sean, AlAkwaa, Fadhl M., McCown, Phillip J., Harder, Jennifer L., Nair, Viji, Eichinger, Felix, Martini, Sebastian, Ademola, Adebowale D., Boima, Vincent, Reich, Heather N., El Saghir, Jamal, Godfrey, Bradley, Ju, Wenjun, Tanner, Emily C., Vega-Warner, Virginia, Wys, Noel L., Adler, Sharon G., Appel, Gerald B., Athavale, Ambarish, Atkinson, Meredith A., Bagnasco, Serena M., Barisoni, Laura, Brown, Elizabeth, Cattran, Daniel C., Coppock, Gaia M., Dell, Katherine M., Derebail, Vimal K., Fervenza, Fernando C., Fornoni, Alessia, Gadegbeku, Crystal A., Gibson, Keisha L., Greenbaum, Laurence A., Hingorani, Sangeeta R., Hladunewich, Michelle A., Hodgin, Jeffrey B., Hogan, Marie C., Holzman, Lawrence B., Jefferson, J. Ashley, Kaskel, Frederick J., Kopp, Jeffrey B., Lafayette, Richard A., Lemley, Kevin V., Lieske, John C., Lin, Jen-Jar, Menon, Rajarasee, Meyers, Kevin E., Nachman, Patrick H., Nast, Cynthia C., O’Shaughnessy, Michelle M., Otto, Edgar A., Reidy, Kimberly J., Sambandam, Kamalanathan K., Sedor, John R., Sethna, Christine B., Singer, Pamela, Srivastava, Tarak, Tran, Cheryl L., Tuttle, Katherine R., Vento, Suzanne M., Wang, Chia-shi, Ojo, Akinlolu O., Adu, Dwomoa, Gipson, Debbie S., Trachtman, Howard, and Kretzler, Matthias

Published
2023
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6. APOL1 genotype-associated morphologic changes among patients with focal segmental glomerulosclerosis

Author

Zee, Jarcy, McNulty, Michelle T., Hodgin, Jeffrey B., Zhdanova, Olga, Hingorani, Sangeeta, Jefferson, Jonathan Ashley, Gibson, Keisha L., Trachtman, Howard, Fornoni, Alessia, Dell, Katherine M., Reich, Heather N., Bagnasco, Serena, Greenbaum, Larry A., Lafayette, Richard A., Gipson, Debbie S., Brown, Elizabeth, Kretzler, Matthias, Appel, Gerald, Sambandam, Kamalanathan K., Tuttle, Katherine R., Chen, Dhruti, Atkinson, Meredith A., Hogan, Marie C., Kaskel, Frederick J., Meyers, Kevin E., O’Toole, John, Srivastava, Tarak, Sethna, Christine B., Hladunewich, Michelle A., Lin, JJ, Nast, Cynthia C., Derebail, Vimal K., Patel, Jiten, Vento, Suzanne, Holzman, Lawrence B., Athavale, Ambarish M., Adler, Sharon G., Lemley, Kevin V., Lieske, John C., Hogan, Jonathan J., Gadegbeku, Crystal A., Fervenza, Fernando C., Wang, Chia-Shi, Matar, Raed Bou, Singer, Pamela, Kopp, Jeffrey B., Barisoni, Laura, and Sampson, Matthew G.

Published
2021
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7. 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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8. Health-related quality of life in glomerular disease

Author

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

Published
2019
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9. CureGN Study Rationale, Design, and Methods: Establishing a Large Prospective Observational Study of Glomerular Disease

Author

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

Published
2019
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10. 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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11. Secondary osteoarthritis due to alkaptonuria: a case report

Author

Sabina Lois, Aruna Veeruswamy, Kamalanathan Kulandaivelu, Mohan Prasad Muthusamy, and Yee Tun Oo

Subjects
alkaptonuria, ammoniacal silver nitrate test, homogentisic acid, ochronotic arthropathy, Medicine
Abstract

Background & Aims: Alkaptonuria is a rare autosomal recessive genetic disorder caused by a deficiency of homogentisate 1,2-dioxygenase, leading to the accumulation of homogentisic acid. It presents with a characteristic triad of homogentisic aciduria, ochronosis, and progressive arthritis, often resulting in secondary osteoarthritis, particularly affecting the hip and knee joints. Case Description: A 58-year-old male presented with severe pain in the right hip and left shoulder. Since childhood, his urine had turned black upon exposure to sunlight. His medical history included multiple fractures and orthopedic interventions. On clinical examination, bluish-black discoloration of the ear lobes and sclera was noted. Biochemical testing, including Benedict’s test, ammoniacal silver nitrate test, and a filter paper spot test using 10% sodium hydroxide, confirmed the diagnosis of alkaptonuria. The patient was diagnosed with osteoarthritis secondary to alkaptonuria and underwent a successful total hip replacement. Conclusion: Early diagnosis and intervention in alkaptonuria are crucial to preventing the debilitating effects of secondary osteoarthritis. A multidisciplinary approach in the management of patients with alkaptonuria can help reduce morbidity and improve quality of life by addressing both symptoms and the underlying disease.

Published
2025

14. Cadherin-11, Sparc-related modular calcium binding protein-2, and Pigment epithelium-derived factor are promising non-invasive biomarkers of kidney fibrosis

Author

Mark E. Williams, Katherine R. Tuttle, Jing Liu, Jinghui Luo, Yougqun He, Laura Pyle, Blue B. Lake, Brad H. Rovin, Lynda Hayashi, Yuguang Xiong, Dennis G. Moledina, Andreas Bueckle, Steven Menez, Glenda V. Roberts, Anand Srivastava, Paul Appelbaum, Heather Ascani, Catherine Campbell, Stephanie M. Grewenow, Mark Aulisio, Jennifer Sun, Christopher R. Anderton, Jamie L. Marshall, Sharon Bledso, John P. Shapiro, Theodore Alexandrov, Richard M. Caprioli, Michele Elder, Leslie Cooperman, Shweta Bansal, Lakeshia Bush, Krzysztof Kiryluk, Mitchell Tublin, Olga G. Troyanskaya, Emilio D. Poggio, Kristina N. Blank, Andrew Janowczyk, Paul Hoover, Sabine M. Diettman, R. Tyler Miller, Katy Borner, Leonidas G. Alexopoulos, James Winters, Anant Madabhushi, Haojia Wu, Chirag R. Parikh, Yumeng Wen, Avi Z. Rosenberg, Agustin Gonzalez-Vicente, Leal Herlitz, Keith Brown, Matthew Gilliam, Joseph P. Gaut, Vidya S. Viswanathan, Karla Mehl, Stewart H. Lecker, Pierre C. Dagher, Dana C. Crawford, Camille Johansen, Anna Greka, Tiffany Shi, Ari Pollack, Renee Frey, Kavya Sharman, Isaac E. Stillman, Stuart J. Shankland, Ricardo Melo Ferreira, Jack Bebiak, Jing Su, Matthias Kretzler, Ellen Palmer, Yury Goltsev, Aaron K. Wong, Matthew R. Rosengart, Taneisha Campbell, Tina Vita, Helmut G. Rennke, Nir Hacohen, Satoru Kudose, Christine Limonte, Kun Zhang, Robyn L. McClelland, Ulysses J. Balis, Katherine J. Kelly, Simon Lee, Ninive C. Conser, Adele Rike, Frederick Dowd, Timothy A. Sutton, Steve Bogen, Petter M. Bjornstad, Zoltan Laszik, Dianbo Zhang, Benjamin D. Humphreys, Pinaki Sarder, Jeffrey M. Spraggins, Ravi Iyengar, Marcelino Rivera, Roy Pinkeney, James C. Williams, Tarek M. El-Achkar, Laura H. Mariani, Richard J. Knight, Manjeri A. Venkatachalam, Pietro A. Canetta, Lloyd G. Cantley, Kayleen Williams, Catherine P. Jayapandian, Edgar A. Otto, Jessica Lukowski, Kassandra Spates-Harden, Ashish Verma, John Saul, Tariq Mukatash, Mia R. Colona, Shana Maikhor, Laurence H. Beck, Titlayo Ilori, Charles E. Alpers, Ellen M. Quardokus, Mujeeb Basit, Dušan Veličković, Raf Van de Plas, Jonathan Himmelfarb, Michael T. Eadon, Chrysta Lienczewski, Christopher Y. Lu, Yijiang M. Chen, Kasra Rezaei, Richard Montellano, Pottumarthi V. Prasad, Francis P. Wilson, Christy Stutzke, Jane Nguyen, Kamalanathan K. Sambandam, Miguel A. Vazquez, Vishal S. Vaidya, Vivette D. D'Agati, Patrick Boada, Adam Wilcox, Astrid Weins, Jennifer A. Schaub, Harold Park, Kumar Sharma, M. Todd Valerius, Stephen Daniel, Sean Eddy, Bruce W. Herr, Kenneth W. Dunn, Jamie Snyder, E. Steve Woodle, Dianna Sendrey, Ljiljana Paša-Tolić, Raghavan Murugan, Brandon Ginley, Bryan Kestenbaum, Celia P. Corona-Villalobos, Olivia Balderes, Sushrut Waikar, Carissa Vinovskis, Brooke Berry, Parmjeet Randhawa, Seth Winfree, Jose R. Torrealba, Ning Shang, Rachel Sealfon, Michael J. Ferkowicz, William S. Bush, Jonas Carson, Robert Koewler, Guanshi Zhang, Robert D. Toto, Ian H. de Boer, Gearoid M. McMahon, Andrew N. Hoofnagle, Vijaykumar R. Kakade, Brendon Lutnick, Melissa M. Shaw, Rita R. Alloway, Rajasree Menon, Afolarin Amodu, Jeanine Basta, Paul J. Lee, Ingrid Onul, Sylvia E. Rosas, Cijang (John) He, Andrew S. Bomback, Yinghua Cheng, Jeffrey B. Hodgin, Samir M. Parikh, Garry Nolan, John A. Kellum, Anil Pillai, Annapurna Pamreddy, Orson W. Moe, Jiten Patel, Jonathan J. Taliercio, S. Susan Hedayati, Anitha Vijayan, Tanima Arora, Evren U. Azeloglu, Paul M. Palevsky, Nathan Heath Patterson, Asra Kermani, Becky Steck, Kavya Anjani, Ashley Berglund, Yashvardhan Jain, Stacey E. Jolly, John R. Sedor, George (Holt) Oliver, Natasha Wen, Nancy Wang, Ruikang Wang, Joseph Ardayfio, Michael Rauchman, Ashley R. Burg, Victoria Blanc, Minnie M. Sarwal, Daniel Hall, Sethu M. Madhavan, Sean D. Mooney, Sushrut S. Waikar, Daria Barwinska, Christopher Y. Park, Tara K. Sigdel, Ugochukwu Ugwuowo, John F. O'Toole, Ragnar Palsson, Insa M. Schmidt, Joel M. Henderson, Hongping Ye, Jens Hansen, Jonathan Barasch, Neil Roy, Nicholas Lucarelli, Anna Shpigel, Ashveena Dighe, Elizabeth Record, Sanjay Jain, and Nichole Jefferson

Subjects
0301 basic medicine, Pathology, medicine.medical_specialty, Urinary system, 030232 urology & nephrology, Kidney, Article, 03 medical and health sciences, 0302 clinical medicine, PEDF, Fibrosis, Biopsy, Humans, Medicine, Osteonectin, Nerve Growth Factors, Prospective Studies, Renal Insufficiency, Chronic, Eye Proteins, Serpins, medicine.diagnostic_test, urogenital system, business.industry, Calcium-Binding Proteins, Cadherins, medicine.disease, 030104 developmental biology, medicine.anatomical_structure, Nephrology, Cohort, Disease Progression, Biomarker (medicine), business, Biomarkers, Kidney disease
Abstract

Kidney fibrosis constitutes the shared final pathway of nearly all chronic nephropathies, but biomarkers for the non-invasive assessment of kidney fibrosis are currently not available. To address this, we characterize five candidate biomarkers of kidney fibrosis: Cadherin-11 (CDH11), Sparc-related modular calcium binding protein-2 (SMOC2), Pigment epithelium-derived factor (PEDF), Matrix-Gla protein, and Thrombospondin-2. Gene expression profiles in single-cell and single-nucleus RNA-sequencing (sc/snRNA-seq) datasets from rodent models of fibrosis and human chronic kidney disease (CKD) were explored, and Luminex-based assays for each biomarker were developed. Plasma and urine biomarker levels were measured using independent prospective cohorts of CKD: the Boston Kidney Biopsy Cohort, a cohort of individuals with biopsy-confirmed semiquantitative assessment of kidney fibrosis, and the Seattle Kidney Study, a cohort of patients with common forms of CKD. Ordinal logistic regression and Cox proportional hazards regression models were used to test associations of biomarkers with interstitial fibrosis and tubular atrophy and progression to end-stage kidney disease and death, respectively. Sc/snRNA-seq data confirmed cell-specific expression of biomarker genes in fibroblasts. After multivariable adjustment, higher levels of plasma CDH11, SMOC2, and PEDF and urinary CDH11 and PEDF were significantly associated with increasing severity of interstitial fibrosis and tubular atrophy in the Boston Kidney Biopsy Cohort. In both cohorts, higher levels of plasma and urinary SMOC2 and urinary CDH11 were independently associated with progression to end-stage kidney disease. Higher levels of urinary PEDF associated with end-stage kidney disease in the Seattle Kidney Study, with a similar signal in the Boston Kidney Biopsy Cohort, although the latter narrowly missed statistical significance. Thus, we identified CDH11, SMOC2, and PEDF as promising non-invasive biomarkers of kidney fibrosis.

Published
2021
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16. Hyperphosphatemia and its Relationship with Blood Pressure, Vasoconstriction, and Endothelial Cell Dysfunction in Hypertensive Hemodialysis Patients

Author

Jinwoo, Jung, Haekyung, Jeon-Slaughter, Hang, Nguyen, Jiten, Patel, Kamalanathan K, Sambandam, Shani, Shastri, and Peter Noel, Van Buren

Subjects
Endothelial Cells, Blood Pressure, Weight Gain, Phosphates, Hyperphosphatemia, Cross-Sectional Studies, Parathyroid Hormone, Renal Dialysis, Vasoconstriction, Nephrology, Hypertension, Humans, Kidney Failure, Chronic, Vascular Calcification
Abstract

Background Hyperphosphatemia occurs frequently in end-stage renal disease patients on hemodialysis and is associated with increased mortality. Hyperphosphatemia contributes to vascular calcification in these patients, but there is emerging evidence that it is also associated with endothelial cell dysfunction. Methods We conducted a cross-sectional study in hypertensive hemodialysis patients. We obtained pre-hemodialysis measurements of total peripheral resistance index (TPRI, non-invasive cardiac output monitor) and plasma levels of endothelin-1 (ET-1) and asymmetric dimethylarginine (ADMA). We ascertained the routine peridialytic blood pressure (BP) measurements from that treatment and the most recent pre-hemodialysis serum phosphate levels. We used generalized linear regression analyses to determine independent associations between serum phosphate with BP, TPRI, ET-1, and ADMA while controlling for demographic variables, parathyroid hormone (PTH), and interdialytic weight gain. Results There were 54 patients analyzed. Mean pre-HD supine and seated systolic and diastolic BP were 164 (27), 158 (21), 91.5 (17), and 86.1 (16) mmHg. Mean serum phosphate was 5.89 (1.8) mg/dL. There were significant correlations between phosphate with all pre-hemodialysis BP measurements (r = 0.3, p = .04; r = 0.4, p = .002; r = 0.5, p r = 0.5, p = .0003.) The correlations with phosphate and TPRI, ET-1, and ADMA were 0.3 (p = .01), 0.4 (p = .007), and 0.3 (p = .04). In our final linear regression analyses controlling for baseline characteristics, PTH, and interdialytic weight gain, independent associations between phosphate with pre-hemodialysis diastolic BP, TPRI, and ET-1 were retained (β = 4.33, p = .0002; log transformed β = 0.05, p = .005; reciprocal transformed β = -0.03, p = .047). Conclusions Serum phosphate concentration is independently associated with higher pre-HD BP, vasoconstriction, and markers of endothelial cell dysfunction. These findings demonstrate an additional negative impact of hyperphosphatemia on cardiovascular health beyond vascular calcification. Trial registration The study was part of a registered clinical trial, NCT01862497 (May 24, 2013).

Published
2022
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17. Precision nephrology identified tumor necrosis factor activation variability in minimal change disease and focal segmental glomerulosclerosis

Author

Laura H. Mariani, Sean Eddy, Fadhl M. AlAkwaa, Phillip J. McCown, Jennifer L. Harder, Viji Nair, Felix Eichinger, Sebastian Martini, Adebowale D. Ademola, Vincent Boima, Heather N. Reich, Jamal El Saghir, Bradley Godfrey, Wenjun Ju, Emily C. Tanner, Virginia Vega-Warner, Noel L. Wys, Sharon G. Adler, Gerald B. Appel, Ambarish Athavale, Meredith A. Atkinson, Serena M. Bagnasco, Laura Barisoni, Elizabeth Brown, Daniel C. Cattran, Gaia M. Coppock, Katherine M. Dell, Vimal K. Derebail, Fernando C. Fervenza, Alessia Fornoni, Crystal A. Gadegbeku, Keisha L. Gibson, Laurence A. Greenbaum, Sangeeta R. Hingorani, Michelle A. Hladunewich, Jeffrey B. Hodgin, Marie C. Hogan, Lawrence B. Holzman, J. Ashley Jefferson, Frederick J. Kaskel, Jeffrey B. Kopp, Richard A. Lafayette, Kevin V. Lemley, John C. Lieske, Jen-Jar Lin, Rajarasee Menon, Kevin E. Meyers, Patrick H. Nachman, Cynthia C. Nast, Michelle M. O’Shaughnessy, Edgar A. Otto, Kimberly J. Reidy, Kamalanathan K. Sambandam, John R. Sedor, Christine B. Sethna, Pamela Singer, Tarak Srivastava, Cheryl L. Tran, Katherine R. Tuttle, Suzanne M. Vento, Chia-shi Wang, Akinlolu O. Ojo, Dwomoa Adu, Debbie S. Gipson, Howard Trachtman, and Matthias Kretzler

Subjects
Nephrology
Abstract

The diagnosis of nephrotic syndrome relies on clinical presentation and descriptive patterns of injury on kidney biopsies, but not specific to underlying pathobiology. Consequently, there are variable rates of progression and response to therapy within diagnoses. Here, an unbiased transcriptomic-driven approach was used to identify molecular pathways which are shared by subgroups of patients with either minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). Kidney tissue transcriptomic profile-based clustering identified three patient subgroups with shared molecular signatures across independent, North American, European, and African cohorts. One subgroup had significantly greater disease progression (Hazard Ratio 5.2) which persisted after adjusting for diagnosis and clinical measures (Hazard Ratio 3.8). Inclusion in this subgroup was retained even when clustering was limited to those with less than 25% interstitial fibrosis. The molecular profile of this subgroup was largely consistent with tumor necrosis factor (TNF) pathway activation. Two TNF pathway urine markers were identified, tissue inhibitor of metalloproteinases-1 (TIMP-1) and monocyte chemoattractant protein-1 (MCP-1), that could be used to predict an individual's TNF pathway activation score. Kidney organoids and single nucleus RNA-sequencing of participant kidney biopsies, validated TNF-dependent increases in pathway activation score, transcript and protein levels of TIMP-1 and MCP-1, in resident kidney cells. Thus, molecular profiling identified a subgroup of patients with either MCD or FSGS who shared kidney TNF pathway activation and poor outcomes. A clinical trial testing targeted therapies in patients selected using urinary markers of TNF pathway activation is ongoing.

Published
2022

18. Multidimensional Data Integration Identifies Tumor Necrosis Factor Activation in Nephrotic Syndrome: A Model for Precision Nephrology

Author

Gerald B. Appel, Fernando C. Fervenza, Sharon G. Adler, Vincent Boima, Michelle Hladunewich, Richard A. Lafayette, Katherine R. Tuttle, Jennifer L. Harder, Suzanne Vento, Kimberly J. Reidy, Marie C. Hogan, Virginia Vega-Warner, Daniel C. Cattran, Akinlolu Ojo, Elizabeth J. Brown, Laura Barisoni, Dwomoa Adu, Larry A. Greenbaum, Noel L. Wys, Vimal K. Derebail, Lawrence B. Holzman, Sean Eddy, Katherine MacRae Dell, Sangeeta Hingorani, Fadhl M. Al-Akwaa, Felix Eichinger, Crystal A. Gadegbeku, Adebowale D. Ademola, Rajarasee Menon, Alessia Fornoni, Keisha L. Gibson, Jeffrey B. Hodgin, Debbie S. Gipson, Chia-shi Wang, John C. Lieske, Pamela Singer, Alicia M. Neu, Christine B. Sethna, Cheryl L. Tran, Meredith A. Atkinson, Kevin V. Lemley, Phillip J. McCown, Jeffrey B. Kopp, Ambarish M. Athavale, John R. Sedor, Jonathan J. Hogan, Jen-Jar Lin, Sebastian Martini, Patrick H. Nachman, Matthias Kretzler, Kamalanathan K. Sambandam, Jamal El Saghir, Serena M. Bagnasco, Cynthia C. Nast, Laura H. Mariani, Bradley Godfrey, Viji Nair, Tarak Srivastava, Kevin E.C. Meyers, Wenjun Ju, Heather N. Reich, J. Ashley Jefferson, Edgar A. Otto, Michelle M. O’Shaughnessy, Emily Tanner, Frederick J. Kaskel, and Howard Trachtman

Subjects
Oncology, Nephrology, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Disease, medicine.disease, Clinical trial, Focal segmental glomerulosclerosis, Internal medicine, Biopsy, medicine, Biomarker (medicine), Minimal change disease, business, Nephrotic syndrome
Abstract

BackgroundClassification of nephrotic syndrome relies on clinical presentation and descriptive patterns of injury on kidney biopsies. This approach does not reflect underlying disease biology, limiting the ability to predict progression or treatment response.MethodsSystems biology approaches were used to categorize patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) based on kidney biopsy tissue transcriptomics across three cohorts and assessed association with clinical outcomes. Patient-level tissue pathway activation scores were generated using differential gene expression. Then, functional enrichment and non-invasive urine biomarker candidates were identified. Biomarkers were validated in kidney organoid models and single nucleus RNA-seq (snRNAseq) from kidney biopsies.ResultsTranscriptome-based categorization identified three subgroups of patients with shared molecular signatures across independent North American, European and African cohorts. One subgroup demonstrated worse longterm outcomes (HR 5.2, p = 0.001) which persisted after adjusting for diagnosis and clinical measures (HR 3.8, p = 0.035) at time of biopsy. This subgroup’s molecular profile was largely (48%) driven by tissue necrosis factor (TNF) activation and could be predicted based on levels of TNF pathway urinary biomarkers TIMP-1 and MCP-1 and clinical features (correlation 0.63, p ConclusionsMolecular profiling identified a patient subgroup within nephrotic syndrome with poor outcome and kidney TNF pathway activation. Clinical trials using non-invasive biomarkers of pathway activation to target therapies are currently being evaluated.Significance StatementMechanistic, targeted therapies are urgently needed for patients with nephrotic syndrome. The inability to target an individual’s specific disease mechanism using currently used diagnostic parameters leads to potential treatment failure and toxicity risk. Patients with focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) were grouped by kidney tissue transcriptional profiles and a subgroup associated with poor outcomes defined. The segregation of the poor outcome group was driven by tumor necrosis factor (TNF) pathway activation and could be identified by urine biomarkers, MCP1 and TIMP1. Based on these findings, clinical trials utilizing non-invasive biomarkers of pathway activation to target therapies, improve response rates and facilitate personalized treatment in nephrotic syndrome have been initiated.

Published
2021
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19. Idiopathic Hypokalemia in Lupus Nephritis: A Newly Recognized Entity

Author

Saira Bilal, Peter N. Van Buren, Ayesha Malik, Kamalanathan K. Sambandam, Shani Shastri, Yu-lun Liu, and Emmanuel A. Adomako

Subjects
medicine.medical_specialty, Population, Lupus nephritis, Hypokalemia, medicine.disease_cause, Gastroenterology, Autoimmunity, Distal renal tubular acidosis, Renal potassium wasting, Internal medicine, medicine, Humans, education, Original Investigation, education.field_of_study, business.industry, Autoantibody, General Medicine, Acidosis, Renal Tubular, medicine.disease, Lupus Nephritis, Bicarbonates, Cohort, Potassium, medicine.symptom, business
Abstract

BACKGROUND: Various causes of hypokalemia (HK) from renal potassium wasting, including distal renal tubular acidosis (RTA), have been described in lupus nephritis (LN). We report a phenomenon of otherwise unexplained HK among a population with LN. METHODS: From our population of 403 patients with LN, we identified a cohort of 20 patients with idiopathic HK, defined by serum potassium

Published
2021

21. Multidimensional Data Integration Identifies Tumor Necrosis Factor Activation in Nephrotic Syndrome: A Model for Precision Nephrology

Author

Mariani, Laura H., primary, Eddy, Sean, additional, AlAkwaa, Fadhl M., additional, McCown, Phillip J., additional, Harder, Jennifer L., additional, Martini, Sebastian, additional, Ademola, Adebowale D., additional, Boima, Vincent, additional, Reich, Heather N., additional, Eichinger, Felix, additional, Saghir, Jamal El, additional, Godfrey, Bradley, additional, Ju, Wenjun, additional, Nair, Viji, additional, Tanner, Emily, additional, Vega-Warner, Virginia, additional, Wys, Noel L., additional, Adler, Sharon G., additional, Appel, Gerald B., additional, Athavale, Ambarish, additional, Atkinson, Meredith A., additional, Bagnasco, Serena M., additional, Barisoni, Laura, additional, Brown, Elizabeth, additional, Cattran, Daniel C., additional, Dell, Katherine M., additional, Derebail, Vimal K., additional, Fervenza, Fernando C., additional, Fornoni, Alessia, additional, Gadegbeku, Crystal A., additional, Gibson, Keisha L., additional, Greenbaum, Larry A., additional, Hingorani, Sangeeta R., additional, Hladunewich, Michelle A., additional, Hodgin, Jeffrey B., additional, Hogan, Jonathan J., additional, Hogan, Marie, additional, Holzman, Lawrence B., additional, Jefferson, J. Ashley, additional, Kaskel, Frederick J., additional, Kopp, Jeffrey B., additional, Lafayette, Richard A., additional, Lemley, Kevin V., additional, Lieske, John C., additional, Lin, Jen-Jar, additional, Menon, Rajarasee, additional, Meyers, Kevin E., additional, Nachman, Patrick H., additional, Nast, Cynthia C., additional, Neu, Alicia M., additional, O’Shaughnessy, Michelle M., additional, Otto, Edgar A., additional, Reidy, Kimberly J., additional, Sambandam, Kamalanathan K., additional, Sedor, John R., additional, Sethna, Christine B., additional, Singer, Pamela, additional, Srivastava, Tarak, additional, Tran, Cheryl L., additional, Tuttle, Katherine R., additional, Vento, Suzanne, additional, Wang, Chia-shi, additional, Ojo, Akinlolu O., additional, Adu, Dwomoa, additional, Gipson, Debbie S., additional, Trachtman, Howard, additional, and Kretzler, Matthias, additional

Published
2021
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23. Perturbations in serum chloride homeostasis in heart failure with preserved ejection fraction: insights from TOPCAT

Author

Kamalanathan K. Sambandam, W.H. Wilson Tang, Mark H. Drazner, Ambarish Pandey, Justin L. Grodin, James C. Fang, and Jeffrey M. Testani

Subjects
medicine.medical_specialty, Aldosterone, medicine.drug_class, Proportional hazards model, business.industry, Hazard ratio, 030204 cardiovascular system & hematology, Loop diuretic, medicine.disease, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, chemistry, Heart failure, Internal medicine, medicine, Cardiology, Spironolactone, Serum chloride, 030212 general & internal medicine, Cardiology and Cardiovascular Medicine, business, Heart failure with preserved ejection fraction
Abstract

AIMS Prior cohorts demonstrating the importance of serum chloride levels in heart failure either excluded or had partial representation of patients with heart failure with preserved ejection fraction (HFpEF). We aimed to examine the relationship between serum chloride concentration and outcomes in HFpEF. METHODS AND RESULTS We included participants from the Treatment of Preserved Cardiac Function Heart Failure with an Aldosterone Antagonist Trial (TOPCAT) who met the following criteria: met inclusion by the natriuretic peptide stratum, had recorded serum chloride levels, and were from the Americas (n = 942). Multivariable Cox proportional hazards models tested the association of serum chloride with clinical outcomes, and mixed effects modelling tested the association of spironolactone or loop diuretic on serial serum chloride levels. The median serum chloride level was 102 [25th-75th percentile 100-105 mmol/L (range 84-114 mmol/L)]. After multivariable adjustment, every standard deviation decrease in serum chloride (4.05 mmol/L) was associated with ∼50% increased risk for cardiovascular death [hazard ratio (HR) 1.51, 95% confidence interval (CI) 1.11-2.06, P = 0.008] and ∼30% increased risk for all-cause death (HR 1.29, 95% CI 1.02-1.62, P = 0.04), but not with the primary composite endpoint or heart failure hospitalization (P > 0.3 for both). There were no significant interactions between spironolactone use and the serum chloride-risk relationship (P > 0.1) for each endpoint. Spironolactone was not (P = 0.33) but loop diuretic use was associated with lower serial serum chloride levels (P

Published
2018
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24. 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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25. 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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26. The longitudinal relationship between patient-reported outcomes and clinical characteristics among patients with focal segmental glomerulosclerosis in the nephrotic syndrome study network

Author

Daniel C. Cattran, Ambarish M. Athavale, Noelle E. Carlozzi, Kevin V. Lemley, Elizabeth J. Brown, David T. Selewski, Meredith A. Atkinson, Christine B. Sethna, Pamela Singer, Katherine R. Tuttle, Fernando C. Fervenza, Shannon Murphy, Crystal A. Gadegbeku, Debbie S. Gipson, John C. Lieske, Frederick J. Kaskel, Jonathan Ashley Jefferson, Larry A. Greenbaum, Sangeeta Hingorani, Jen Jar Lin, Alessia Fornoni, Sharon G. Adler, Vimal K. Derebail, Patrick H. Nachman, Lawrence B. Holzman, Matthias Kretzler, Jeffrey B. Kopp, Keisha L. Gibson, Gerald B. Appel, Kimberly J. Reidy, Michelle A. Hladunewich, Tarak Srivastava, Kamalanathan K. Sambandam, Chia-shi Wang, Emily Herreshoff, John R. Sedor, Richard A. Lafayette, Marie C. Hogan, Kevin E.C. Meyers, Frank Modersitzki, Heather N. Reich, Laura Barisoni, Anne Waldo, Howard Trachtman, Katherine MacRae Dell, and Jonathan P. Troost

Subjects
medicine.medical_specialty, 030232 urology & nephrology, PROMIS, 03 medical and health sciences, 0302 clinical medicine, Focal segmental glomerulosclerosis, remission, Quality of life, Internal medicine, Medicine, 030212 general & internal medicine, Prospective cohort study, AcademicSubjects/MED00340, Depression (differential diagnoses), focal segmental glomerulosclerosis, Transplantation, prospective cohort study, business.industry, nephrotic syndrome, Clinical study design, Original Articles, medicine.disease, Clinical trial, Nephrology, patient-reported outcomes, Anxiety, medicine.symptom, proteinuria, business, Nephrotic syndrome
Abstract

Background Understanding the relationship between clinical and patient-reported outcomes (PROs) will help support clinical care and future clinical trial design of novel therapies for focal segmental glomerulosclerosis (FSGS). Methods FSGS patients ≥8 years of age enrolled in the Nephrotic Syndrome Study Network completed Patient-Reported Outcomes Measurement Information System PRO measures of health-related quality of life (HRQoL) (children: global health, mobility, fatigue, pain interference, depression, anxiety, stress and peer relationships; adults: physical functioning, fatigue, pain interference, sleep impairment, mental health, depression, anxiety and social satisfaction) at baseline and during longitudinal follow-up for a maximum of 5 years. Linear mixed-effects models were used to determine which demographic, clinical and laboratory features were associated with PROs for each of the eight children and eight adults studied. Results There were 45 children and 114 adult FSGS patients enrolled that had at least one PRO assessment and 519 patient visits. Multivariable analyses among children found that edema was associated with global health (−7.6 points, P = 0.02) and mobility (−4.2, P = 0.02), the number of reported symptoms was associated with worse depression (−2.7 per symptom, P = 0.009) and anxiety (−2.3, P = 0.02) and the number of emergency room (ER) visits in the prior 6 months was associated with worse mobility (−2.8 per visit, P Conclusions PROs provide important information about HRQoL for persons with FSGS that is not captured solely by the examination of laboratory-based markers of disease. However, it is critical that instruments capture the patient experience and FSGS clinical trials may benefit from a disease-specific instrument more sensitive to within-patient changes.

Published
2020
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27. 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

28. 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

29. Health-related quality of life in glomerular disease

Author

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

Published
2019
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30. Complete Remission in the Nephrotic Syndrome Study Network

Author

Daniel C. Cattran, Alicia M. Neu, Patrick H. Nachman, Kevin V. Lemley, Matthew G. Sampson, Matthew Palmer, Laura H. Mariani, Michael J. Choi, Crystal A. Gadegbeku, Titilayo O. Ilori, Katherine R. Tuttle, Keisha L. Gibson, Sangeeta Hingorani, Frederick J. Kaskel, Christoph Licht, Cynthia C. Nast, Sharon G. Adler, Stephen M. Hewitt, Olga Zhdanova, Gerald B. Appel, Gaston Zilleruelo, J. Charles Jennette, Kevin E.C. Meyers, John R. Sedor, Jonathan P. Troost, Fernando C. Fervenza, Tammy M. Brady, Heather N. Reich, Jeffrey B. Kopp, Marie C. Hogan, Michelle Hladunewich, Duncan B. Johnstone, Kalyani Perumal, Susan L. Hogan, Marva Moxey-Mims, Richard A. Lafayette, Jeffrey B. Hodgin, Avi Z. Rosenberg, Howard Trachtman, Christine B. Sethna, Peter X.-K. Song, Peter J. Nelson, Michele H. Mokrzycki, Kamalanathan K. Sambandam, Serena M. Bagnasco, Debbie S. Gipson, John C. Lieske, Carmen Avila-Casado, Laura Barisoni-Thomas, Katherine MacRae Dell, Lawrence B. Holzman, Alessia Fornoni, Larry A. Greenbaum, and Matthias Kretzler

Subjects
Adult, Male, medicine.medical_specialty, Nephrotic Syndrome, Adolescent, Epidemiology, Biopsy, 030232 urology & nephrology, 030204 cardiovascular system & hematology, Kidney, Critical Care and Intensive Care Medicine, Gastroenterology, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Focal segmental glomerulosclerosis, Membranous nephropathy, Interquartile range, Internal medicine, Outcome Assessment, Health Care, medicine, Humans, Minimal change disease, Prospective Studies, Proportional Hazards Models, Transplantation, Proteinuria, medicine.diagnostic_test, business.industry, Remission Induction, Hazard ratio, Original Articles, Middle Aged, medicine.disease, Surgery, Nephrology, Kidney Failure, Chronic, Female, Renal biopsy, medicine.symptom, business, Nephrotic syndrome, Glomerular Filtration Rate
Abstract

Background and objectives This analysis from the Nephrotic Syndrome Study Network (NEPTUNE) assessed the phenotypic and pathology characteristics of proteinuric patients undergoing kidney biopsy and defined the frequency and factors associated with complete proteinuria remission (CRever). Design, setting, participants, & measurements We enrolled adults and children with proteinuria ≥0.5 g/d at the time of first clinically indicated renal biopsy at 21 sites in North America from April 2010 to June 2014 into a prospective cohort study. NEPTUNE central pathologists assigned participants to minimal-change disease (MCD), FSGS, membranous nephropathy, or other glomerulopathy cohorts. Outcome measures for this analysis were (1) CRever with urine protein-to-creatinine ratio (UPC)

Published
2016
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31. 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

32. Collapsing Focal Segmental Glomerulosclerosis Resulting from a Single Dose of Zoledronate

Author

Javier A. Neyra, Kamalanathan K. Sambandam, Allen R. Hendricks, and Omkar U. Vaidya

Subjects
African american female, medicine.medical_specialty, Proteinuria, business.industry, Osteoporosis, Urology, Acute kidney injury, Renal function, Case Report, Focal segmental glomerulosclerosis, Bisphosphonates, medicine.disease, lcsh:Diseases of the genitourinary system. Urology, lcsh:RC870-923, urologic and male genital diseases, Metastatic breast cancer, Surgery, Nephrology, medicine, medicine.symptom, business, Multiple myeloma, Zoledronate
Abstract

Bisphosphonates are commonly used for the treatment of osteoporosis, Paget's disease, multiple myeloma and hypercalcemia. Collapsing focal segmental glomerulosclerosis (FSGS) is known to occur uncommonly with exposure to bisphosphonates, specifically pamidronate and alendronate; it has rarely and equivocally been reported with zoledronate therapy. We describe the case of a 36-year-old African American female with metastatic breast cancer who presented with nephrotic-range proteinuria and acute kidney injury within 2 weeks of exposure to a single dose of zoledronate. The patient had a partial recovery of her renal function and showed improved proteinuria to a subnephrotic level after discontinuing zoledronate. In contrast to 2 prior reports of zoledronate-induced collapsing FSGS, the causative role of the exposure described here is certain. Our case necessitates the addition of zoledronate to the list of known causes of collapsing FSGS. Furthermore, it highlights the importance of periodically monitoring renal function and urine protein excretion with the use of zoledronate, which allows prompt diagnosis and withdrawal of the drug to increase the probability of renal recovery.

Published
2014

33. CureGN Study Rationale, Design, and Methods: Establishing a Large Prospective Observational Study of Glomerular Disease

Author

Mariani, Laura H., primary, Bomback, Andrew S., additional, Canetta, Pietro A., additional, Flessner, Michael F., additional, Helmuth, Margaret, additional, Hladunewich, Michelle A., additional, Hogan, Jonathan J., additional, Kiryluk, Krzysztof, additional, Nachman, Patrick H., additional, Nast, Cynthia C., additional, Rheault, Michelle N., additional, Rizk, Dana V., additional, Trachtman, Howard, additional, Wenderfer, Scott E., additional, Bowers, Corinna, additional, Hill-Callahan, Peg, additional, Marasa, Maddalena, additional, Poulton, Caroline J., additional, Revell, Adelaide, additional, Vento, Suzanne, additional, Barisoni, Laura, additional, Cattran, Dan, additional, D’Agati, Vivette, additional, Jennette, J. Charles, additional, Klein, Jon B., additional, Laurin, Louis-Philippe, additional, Twombley, Katherine, 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, additional, Smoyer, William E., additional, Guay-Woodford, Lisa M., additional, Ahn, Wooin, additional, Appel, Gerald B., additional, Babayev, Revekka, additional, Batal, Ibrahim, additional, Brown, Eric, additional, Campenot, Eric S., additional, Canetta, Pietro, additional, Carlassara, Lucrezia, additional, Chan, Brenda, additional, Chatterjee, Debanjana, additional, D’Agati, Vivette D., additional, Delbarba, Elisa, additional, Dogra, Samriti, additional, Fernandez, Hilda, additional, Foroncewicz, Bartosz, additional, Ghiggeri, Gian Marco, additional, Hines, William H., additional, Ali Husain, S., additional, Jain, Namrata G., additional, Khairallah, Pascale, additional, Kil, Byum Hee, additional, Jeyabalan, Anushya, additional, Lau, Wai L., additional, Lin, Fangming, additional, Lugani, Francesca, additional, Markowitz, Glen, additional, Mohan, Sumit, additional, Mu, Xueru, additional, Mucha, Krzysztof, additional, Nickolas, Thomas L., additional, Piva, Stacy, additional, Radhakrishnan, Jai, additional, Rao, Maya K., additional, Regunathan-Shenk, Renu, additional, Sanna-Cherchi, Simone, additional, Santoriello, Dominick, additional, Shirazian, Shayan, additional, Stokes, Michael B., additional, Yu, Natalie, additional, Valeri, Anthony M., additional, Zviti, Ronald, additional, Al-Uzri, Amira, additional, Ambruzs, Josephine, additional, Ashoor, Isa, additional, Aviles, Diego, additional, Baracco, Rossana, additional, Barcia, John, additional, Bartosh, Sharon, additional, Belsha, Craig, additional, Braun, Michael C., additional, Cai, Yi, additional, Chernitskiy, Vladimir, additional, Chishti, Aftab, additional, Claes, Donna, additional, Clark, Kira, additional, Cramer, Carl, additional, Davis, Keefe, additional, Dutcher, Amy, additional, Erkan, Elif, additional, Feig, Daniel, additional, Freundlich, Michael, additional, Gaut, Joseph, additional, Gbadegesin, Rasheed, additional, Hanna, Melisha, additional, Hidalgo, Guillermo, additional, Hooper, David, additional, Hunley, Tracy E., additional, Jain, Amrish, additional, Kallash, Mahmoud, additional, Kamel, Margo, additional, Khalid, Myda, additional, Kump, Theresa, additional, Lane, Jerome C., additional, Liapis, Helen, additional, Mahan, John, additional, Mathews, Nisha, additional, Nester, Carla, additional, Pan, Cynthia, additional, Patterson, Larry, additional, Patel, Hiren, additional, Raad, Alice, additional, Silva, Cynthia, additional, Sreedharan, Rajasree, additional, Srivastava, Tarak, additional, Steinke, Julia, additional, Sumner, Susan, additional, Vasylyeva, Tetyana L., additional, Wang, Chia-shi, additional, Weaver, Donald J., additional, Wong, Craig S., additional, Yin, Hong, additional, Achanti, Anand, additional, Almaani, Salem, additional, Ayoub, Isabelle, additional, Budisavljevic, Milos, additional, D'Angelo, Maggie, additional, Derebail, Vimal, additional, Fatima, Huma, additional, Falk, Ronald, additional, Fogo, Agnes, additional, Gibson, Keisha, additional, Glenn, Dorey, additional, Hogan, Susan, additional, Jain, Koyal, additional, Julian, Bruce, additional, Kidd, Jason, additional, Massey, H. Davis, additional, Mottl, Amy, additional, Murphy, Shannon, additional, Nadasdy, Tibor, additional, Novak, Jan, additional, Parikh, Samir, additional, Poulton, Caroline, additional, Powell, Thomas Brian, additional, Reeve, Bryce, additional, Renfrow, Matthew, additional, Reynolds, Monica, additional, Rizk, Dana, additional, Rovin, Brad, additional, Royal, Virginie, additional, Saha, Manish, additional, Sanghani, Neil, additional, Self, Sally, additional, Adler, Sharon, additional, Alachkar, Nada, additional, Alpers, Charles, additional, Matar, Raed Bou, additional, Avila-Casado, Carmen, additional, Bagnasco, Serena, additional, Brede, Emily, additional, Brown, Elizabeth, additional, Cattran, Daniel, additional, Choi, Michael, additional, Contreras, Gabriel, additional, Dell, Katherine M., additional, Dewalt, Darren, additional, Denburg, Michelle, additional, Dukkipati, Ram, additional, Fervenza, Fernando C., additional, Fornoni, Alessia, additional, Gadegbeku, Crystal, additional, Gipson, Patrick, additional, Gonzalez-Zea, Anny, additional, Hasely, Leah, additional, Hendren, Elizabeth, additional, Hingorani, Sangeeta, additional, Hladunewich, Michelle, additional, Hogan, Jonathan, additional, Hou, Jean, 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, Oliverio, Andrea, additional, Palmer, Matthew, additional, Parekh, Rulan, additional, Pitter, Renee, additional, Reich, Heather, additional, Reidy, Kimberly, additional, Rondon, Helbert, additional, Sambandam, Kamalanathan K., additional, Sampson, Matthew, 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, Waldman, Meryl, additional, Weisstuch, Joseph, additional, Wiggins, Roger, additional, Williams, David, additional, Winkler, Cheryl, additional, Young, Eric, additional, Zhdanova, Olga, additional, Beil, Charlotte, additional, Eikstadt, Richard, additional, Gillespie, Brenda, additional, Graff, John, additional, Hewitt, Stephen, additional, Herreshoff, Emily, additional, Lienczewski, Chrysta, additional, Mansfield, Sarah, additional, Mariani, Laura, additional, McCullough, Keith, additional, Moore, Nicholas, additional, Robinson, Bruce M., additional, Sexton, Melissa, additional, Troost, Jonathan, additional, Wladkowski, Matthew, additional, Zee, Jarcy, additional, and Zinsser, Dawn, additional

Published
2019
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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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35. Prognostic significance of repeat biopsy in lupus nephritis: Histopathologic worsening and a short time between biopsies is associated with significantly increased risk for end stage renal disease and death

Author

David R. Karp, Ramesh Saxena, Kamalanathan K. Sambandam, Cristina Arriens, Sixia Chen, Judith A. James, Eliza F. Chakravarty, and Joan T. Merrill

Subjects
Adult, Male, medicine.medical_specialty, Pathology, Adolescent, Cost effectiveness, Biopsy, Immunology, 030232 urology & nephrology, Lupus nephritis, Kaplan-Meier Estimate, Kidney, Article, End stage renal disease, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Risk Factors, Internal medicine, medicine, Immunology and Allergy, Humans, Young adult, 030203 arthritis & rheumatology, medicine.diagnostic_test, business.industry, Hazard ratio, Middle Aged, medicine.disease, Prognosis, Lupus Nephritis, Kidney Failure, Chronic, Histopathology, Female, Renal biopsy, business
Abstract

Approximately half of patients with systemic lupus erythematosus (SLE) develop lupus nephritis (LN), a major cause of morbidity and early mortality in that disease. Prolonged renal inflammation is associated with irreversible kidney damage which confers a 30% risk of end stage renal disease (ESRD), making early, aggressive treatment mandatory. Failure to achieve therapeutic response or recurrence of renal flare often prompts repeat biopsy. However, the role of repeat biopsy in determining long-term renal prognosis remains controversial. For this reason repeat biopsies are usually not utilized unless clinical evidence of refractory or recurrent disease is already present, despite known mismatches between clinical and biopsy findings. The current study quantifies the degree to which histopathologic worsening between first and second biopsies and duration between them predicts ESRD and death.Medical records of 141 LN patients with more than one biopsy were obtained from a single large urban medical center. Cases were attained using billing codes for diagnosis and procedures from 1/1999-1/2015. Biopsy worsening was defined as unfavorable histopathologic classification transitions and/or increased chronicity; if neither were present, the patient was defined as non-worsening. We used Cox proportional hazard models to study the relationship between ESRD and survival adjusting for covariates which included age at first biopsy, gender, race, initial biopsy class, and initial induction therapy.Of 630 patients screened, 141 had more than one biopsy. Advancing chronicity was detected in 48 (34.0%) and a renal class switch to worse grade of pathology was found in 54 (38.3%). At least one of these adverse second biopsy features was reported in 79 (56.0%) patients. Five years following initial biopsy, 28 (35.4%) of those with worsening histopathology on second biopsy developed ESRD, compared to 6 (9.7%) of non-worsening patients and 10 (12.7%) of patients with worsening histopathology had died compared to 2 (3.2%) of non-worsening patients. Biopsy worsening was associated with a significantly greater 15-year risk of ESRD (Hazard Ratio 4.2, p=0.0001) and death (Hazard Ratio 4.3, p=0.022), adjusting for age, gender, race, biopsy class, and treatment. Time between first and second biopsies was1year in 32 patients, 1-5years in 81, and5years in 28. Over a 15-year period, those with1year between first and second biopsies (presumably enriched for patients with early clinical signs of progression) had a significantly greater risk of ESRD (Hazard Ratio 13.7, p0.0001) and death (Hazard Ratio 16.9, p=0.0022) after adjusting for age, gender, race, biopsy class, and treatment.A repeat renal biopsy demonstrating worsening pathology increases the risk of ESRD and death more than four-fold compared to non-worsening patients. Given known potential mismatch between biopsy and clinical data, repeat biopsies may add important information and justify changes in treatment not considered on clinical grounds. Earlier detection of poor prognostic signs in those without early clinical deterioration might improve outcomes in enough patients to reconsider cost effectiveness of routine repeat biopsy.

Published
2016

36. Overexpression of Evi-1 oncoprotein represses TGF-β signaling in colorectal cancer

Author

Mark R. Hellmich, Courtney M. Townsend, Kamalanathan K. Sambandam, Alan P. Fields, Archibald S. Perkins, E. Aubrey Thompson, Tien C. Ko, Xiyun Deng, Yan Liu, Fazhi Li, Srinivasan Rajaraman, and Yanna Cao

Subjects
Cancer Research, Colon, Colorectal cancer, Article, Mice, Rapid amplification of cDNA ends, Transforming Growth Factor beta, Cell Line, Tumor, Proto-Oncogenes, medicine, Animals, Humans, Promoter Regions, Genetic, neoplasms, Molecular Biology, Transcription factor, Regulation of gene expression, Base Sequence, biology, Rectum, Exons, Transforming growth factor beta, Transfection, medicine.disease, Molecular biology, MDS1 and EVI1 Complex Locus Protein, digestive system diseases, Up-Regulation, DNA-Binding Proteins, Gene Expression Regulation, Neoplastic, Cancer cell, biology.protein, Colorectal Neoplasms, Signal Transduction, Transcription Factors, Transforming growth factor
Abstract

Human colorectal cancer (CRC) cells are resistant to the anti-proliferative effect of transforming growth factor-β (TGF-β), suggesting that disruption of TGF-β signaling plays an important role in colorectal carcinogenesis. Ecotropic virus integration site-1 (Evi-1) oncoprotein represses TGF-β signaling by interacting with Smads, but its role in CRC has not been established. The purpose of this study is to determine whether Evi-1 plays role(s) in CRCs and to characterize Evi-1 transcript(s) in CRCs. Evi-1 was overexpressed in 53% of human CRC samples, 100% of colon adenoma samples, and 100% of human colon cancer cell lines tested. Using 5' RACE, we cloned a novel Evi-1 transcript (Evi-1e) from a human CRC tissue and found that this novel transcript was expressed at a higher level in CRC tissues than in normal tissues and was the major Evi-1 transcript in CRCs. Transient Evi-1 transfection inhibited TGF-β-induced transcriptional activity and reversed the growth inhibitory effect of TGF-β in MC-26 mouse colon cancer cells. In conclusion, we have identified overexpression of Evi-1 oncoprotein as a novel mechanism by which a subset of human CRCs may escape TGF-β regulation. We have also identified a novel Evi-1 transcript, Evi-1e, as the major Evi-1 transcript expressed in human CRCs.

Published
2011
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38. Digital Healthcare - Cyberattacks in Asian Organizations: An Analysis of Vulnerabilities, Risks, NIST Perspectives, and Recommendations

Author

Kamalanathan Kandasamy, Sethuraman Srinivas, Krishnashree Achuthan, and Venkat P. Rangan

Subjects
Cyberattacks, healthcare, vulnerability, risk, vulnerability management, risk management, Electrical engineering. Electronics. Nuclear engineering, TK1-9971
Abstract

Cyberattacks on healthcare institutions are on an upsurge all over the world. Recently, Asian hospitals have become targets of numerous cyberattacks. While Western countries like the United States have implemented security-related laws, policies, standards, and other protective measures to deal with the healthcare cyberattacks, Asian countries are lagging. The Healthcare insurance portability and accountability act (HIPAA), enacted by the United States federal government, is a classic example of a law that has been in existence for a quarter-century now. Awareness about electronic health records (EHR) and their importance is increasing in Asia. Many hospitals and healthcare systems successfully implement solutions to protect healthcare data, including sensitive patient data. However, protecting healthcare data involves a sophisticated technology and compliance-driven approach due to the high value associated with the data. In this research, an earnest attempt is made to investigate the recent cyberattacks in Asian healthcare institutions. Based on the investigation, five types of cyberattacks are found to dominate Asian healthcare institutions. A detailed analysis of these attacks, their vulnerabilities, and associated risks are performed as a part of this study. In many countries with higher cybersecurity maturity, risk frameworks are successfully employed to manage the risks associated with healthcare data. In this study, the cyberattacks on Asian healthcare institutions are also analyzed through the lens of the National Institute of standards and technology (NIST) risk framework. Based on the literature review, a few unique recommendations are included in this research study to be used as risk mitigation measures by Asian healthcare organizations and researchers to manage and improve the growing situation of cyberattacks.

Published
2022
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41. Effective use of loop diuretics in heart failure exacerbation: a nephrologist's view

Author

Kamalanathan K. Sambandam and Biff F. Palmer

Subjects
Nephrology, Heart Failure, medicine.medical_specialty, Acute decompensated heart failure, Exacerbation, Dose-Response Relationship, Drug, business.industry, medicine.drug_class, Renal function, Diuresis, Angiotensin-Converting Enzyme Inhibitors, General Medicine, Loop diuretic, medicine.disease, Sodium Potassium Chloride Symporter Inhibitors, Internal medicine, Heart failure, medicine, Cardiology, Humans, Renal Insufficiency, Intensive care medicine, business, Tubuloglomerular feedback, Glomerular Filtration Rate
Abstract

Unfortunately, patients with congestive heart failure suffer frequent admissions for the management of fluid overload. Loop diuretics are pivotal in the management of this common clinical problem. Although loop diuretics have been in clinical use since the 1960s, we still do not understand how to optimally administer these drugs. It is unknown why some decompensated heart failure patients exhibit improvements in renal function with diuresis, whereas others display renal function deterioration, limiting attainment of euvolemia. Here the physiologic interactions between the failing heart and kidneys are reviewed. A conceptual framework is presented that emphasizes the balance between tubuloglomerular feedback and venous congestion in determining renal function during loop diuretic use in heart failure. within this framework, guidelines are derived that seek to maximize the chance for achieving adequate volume removal while maintaining stable or improved renal function during the treatment of acute decompensated heart failure.

Published
2013

42. Energy comparison of balanced and progressive sensor networks

Author

Maneesha Vinodini Ramesh, Sreedevi A. G, Kamalanathan K, and P. Venkat Rangan

Subjects
Key distribution in wireless sensor networks, business.industry, Topology control, Computer science, Distributed computing, Small number, Mobile wireless sensor network, Energy minimization, business, Wireless sensor network, Energy (signal processing), Computer network, Power (physics)
Abstract

Wireless Sensor Networks are deployed in almost all areas as they are one of the efficient solutions for monitoring, detecting and providing early warnings. Energy minimization being one of the main challenges in WSNs, we present four different topology control algorithms which will compute the aggregated data in a distributed way, addressing this issue. Each algorithm optimizes power to different levels, by reducing the number of computations and transmissions. The discussed methods include two level and multi levels of balanced and progressive network and its analysis of energy savings. Among the four networks, multi-level progressive is found to be better for small number of sensor nodes (N

Published
2012
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43. Complete Remission in the Nephrotic Syndrome Study Network

Author

Gipson, Debbie S., primary, Troost, Jonathan P., additional, Lafayette, Richard A., additional, Hladunewich, Michelle A., additional, Trachtman, Howard, additional, Gadegbeku, Crystal A., additional, Sedor, John R., additional, Holzman, Lawrence B., additional, Moxey-Mims, Marva M., additional, Perumal, Kalyani, additional, Kaskel, Frederick J., additional, Nelson, Peter J., additional, Tuttle, Katherine R., additional, Bagnasco, Serena M., additional, Hogan, Marie C., additional, Dell, Katherine M., additional, Appel, Gerald B., additional, Lieske, John C., additional, Ilori, Titilayo O., additional, Sethna, Christine B., additional, Fervenza, Fernando C., additional, Hogan, Susan L., additional, Nachman, Patrick H., additional, Rosenberg, Avi Z., additional, Greenbaum, Larry A., additional, Meyers, Kevin E.C., additional, Hewitt, Stephen M., additional, Choi, Michael J., additional, Kopp, Jeffrey B., additional, Zhdanova, Olga, additional, Hodgin, Jeffrey B., additional, Johnstone, Duncan B., additional, Adler, Sharon G., additional, Avila-Casado, Carmen, additional, Neu, Alicia M., additional, Hingorani, Sangeeta R., additional, Lemley, Kevin V., additional, Nast, Cynthia C., additional, Brady, Tammy M., additional, Barisoni-Thomas, Laura, additional, Fornoni, Alessia, additional, Jennette, J. Charles, additional, Cattran, Daniel C., additional, Palmer, Matthew B., additional, Gibson, Keisha L., additional, Reich, Heather N., additional, Mokrzycki, Michele H., additional, Sambandam, Kamalanathan K., additional, Zilleruelo, Gaston E., additional, Licht, Christoph, additional, Sampson, Matthew G., additional, Song, Peter, additional, Mariani, Laura H., additional, and Kretzler, Matthias, additional

Published
2015
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44. Miscellaneous Etiologies of Acute Kidney Injury

Author

Anitha Vijayan and Kamalanathan K. Sambandam

Subjects
medicine.medical_specialty, Acute interstitial nephritis, business.industry, Acute kidney injury, Etiology, Medicine, business, medicine.disease, Intensive care medicine
Published
2009
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47. IoT cyber risk: a holistic analysis of cyber risk assessment frameworks, risk vectors, and risk ranking process

Author

Kamalanathan Kandasamy, Sethuraman Srinivas, Krishnashree Achuthan, and Venkat P. Rangan

Subjects
Risk assessment, Internet of Medical Things, Risk vectors, Cybersecurity risk assessment frameworks, Risk rank, Computer engineering. Computer hardware, TK7885-7895, Electronic computers. Computer science, QA75.5-76.95
Abstract

Abstract Security vulnerabilities of the modern Internet of Things (IoT) systems are unique, mainly due to the complexity and heterogeneity of the technology and data. The risks born out of these IoT systems cannot easily fit into an existing risk framework. There are many cybersecurity risk assessment approaches and frameworks that are under deployment in many governmental and commercial organizations. Extending these existing frameworks to IoT systems alone will not address the new risks that have arisen in the IoT ecosystem. This study has included a review of existing popular cyber risk assessment methodologies and their suitability to IoT systems. National Institute of Standards and Technology, Operationally Critical Threat, Asset, and Vulnerability Evaluation, Threat Assessment & Remediation Analysis, and International Standards Organization are the four main frameworks critically analyzed in this research study. IoT risks are presented and reviewed in terms of the IoT risk category and impacted industries. IoT systems in financial technology and healthcare are dealt with in detail, given their high-risk exposure. Risk vectors for IoT and the Internet of Medical Things (IoMT) are discussed in this study. A unique risk ranking method to rank and quantify IoT risk is introduced in this study. This ranking method initiates a risk assessment approach exclusively for IoT systems by quantifying IoT risk vectors, leading to effective risk mitigation strategies and techniques. A unique computational approach to calculate the cyber risk for IoT systems with IoT-specific impact factors has been designed and explained in the context of IoMT systems.

Published
2020
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