Pathophysiology of Glomerular Diseases and Nephrotic Syndromes

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Floege, J., Gibson, K. L., Vivarelli, M., Liew, A., Radhakrishnan, J., Balk, E. M., Gordon, C. E., Adam, G., Tonelli, M., Earley, A., & Rovin, B. H. (2025). Executive summary of the KDIGO 2025 Clinical Practice Guideline for the Management of Nephrotic Syndrome in Children. Kidney International, 107(5), 806–808. https://doi.org/10.1016/j.kint.2024.11.006
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Tarragón, B., Han, H., Navarro-Torres, M., Canetta, P., Wooden, B., Derebail, V. K., Glenn, D., Mottl, A., Massicotte-Azarniouch, D., Kerlin, B., Hladunewich, M., Coppock, G., Rheault, M., Mariani, L. H., & Bomback, A. (2025). Prophylactic anticoagulation in patients with nephrotic syndrome in the Cure Glomerulonephropathy (CureGN) cohort. Clinical Kidney Journal. https://doi.org/10.1093/ckj/sfaf104
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Shao, D., Jimenez, A. L., Guerrero, M. S., Wang, S., & Broder, A. (2025). Factors associated with worsening interstitial fibrosis/tubular atrophy in lupus nephritis patients undergoing clinically indicated repeat kidney biopsy. BMC Nephrology, 26(1). https://doi.org/10.1186/s12882-025-04108-0
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Bu, L., Valeri, A. M., Said, S. M., Zhu, Y., Hernandez, L. H., Gladish, R., Prasad, B., Sethi, S., Leung, N., & Nasr, S. H. (2025). IgM Variant of Proliferative Glomerulonephritis With Monoclonal Immunoglobulin Deposits: A Case Series. American Journal of Kidney Diseases. https://doi.org/10.1053/j.ajkd.2025.01.009
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Rebello, S., Hsu, K., Nativi-Nicolau, J., Karam, C., Grogan, M., Lousada, I., & Maurer, M. S. (2025). Factors associated with financial toxicity in patients with transthyretin amyloidosis: results from Amyloidosis Research Consortium’s treatment affordability patient and caregiver survey. Amyloid, 1–10. https://doi.org/10.1080/13506129.2025.2462541
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Blazius, B., Troost, J. P., Kopp, J. B., Parekh, R. S., Gillespie, B., Ayoub, I., Kallash, M., Gbadegesin, R., Canetta, P. A., Srivastava, T., Hunley, T. E., Twombley, K. E., Peleg, Y. A., Greenbaum, L. A., Chishti, A. S., Nester, C. M., Mottl, A. K., Hogan, S. L., Royal, V., … Kidd, J. M. (2025). Clinical Decision-Making About Immunosuppressive Treatment in Focal Segmental Glomerulosclerosis. Kidney Medicine, 7(4), 100975. https://doi.org/10.1016/j.xkme.2025.100975
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Simeone, C. A., McNulty, M. T., Gupta, Y., Genovese, G., Sampson, M. G., Sanna-Cherchi, S., Friedman, D. J., & Pollak, M. R. (2024). The APOL1 p.N264K variant is co-inherited with the G2 kidney disease risk variant through a proximity recombination event. G3: Genes, Genomes, Genetics. https://doi.org/10.1093/g3journal/jkae290
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Haruhara, K., Okabayashi, Y., Sasaki, T., Kubo, E., D’Agati, V. D., Bertram, J. F., Tsuboi, N., & Yokoo, T. (2024). Podocyte density as a predictor of long-term kidney outcome in obesity-related glomerulopathy. Kidney International. https://doi.org/10.1016/j.kint.2024.05.025
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Zanoni, F., Naesens, M., Leal, R., Amoroso, A., Caliskan, Y., Keating, B., Gharavi, A., Castellano, G., Batal, I., & Kiryluk, K. (2024). #1450 Polygenic risk score of native IgA nephropathy predicts risk of post-transplant disease recurrence. Nephrology Dialysis Transplantation, 39(Supplement_1). https://doi.org/10.1093/ndt/gfae069.184
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Fakhouri, F., Bomback, A., Kavanagh, D., Remuzzi, G., Sunder-Plassmann, G., Kanellis, J., Daina, E., Walker, P., Wang, Z., & Ahmad, Z. (2024). #1467 Pegcetacoplan for post-transplant recurrent C3 glomerulopathy or immune complex membranoproliferative glomerulonephritis in NOBLE: 12-week evolution. Nephrology Dialysis Transplantation, 39(Supplement_1). https://doi.org/10.1093/ndt/gfae069.442
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Escudero-Saiz, V. J., Gonzalez, Á., García-Herrera, A., Larque, A. B., Bomback, A. S., Morantes, L., Martínez-Chillarón, M., Ollé, J., Guillén, E., Xipell, M., Molina-Andújar, A., Rodríguez, D., Cuadrado, E., Cacho, J., Arana, C., Esforzado, N., Bastida, C., Poch, E., Diekman, F., … Blasco, M. (2024). Factor B Inhibition with Iptacopan in Recurrent C3 Glomerulopathy Following Kidney Transplant: A Report of Two Cases. Kidney Medicine, 6(6), 100823. https://doi.org/10.1016/j.xkme.2024.100823
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Ndife, B., Aldworth, C., Murphy, K. P., Nguyen, J., Pivneva, I., Edwards, M. L., Anderson, A., Signorovitch, J., & Canetta, P. (2024). WCN24-551 CKD PROGRESSION IN PATIENTS WITH COMPLEMENT 3 GLOMERULOPATHY (C3G) IN A US MULTI-CENTER ASSESSMENT. Kidney International Reports, 9(4), S244. https://doi.org/10.1016/j.ekir.2024.02.501
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Ndife, B., Aldworth, C., Murphy, K. P., Nguyen, J., Pivneva, I., Edwards, M. L., Anderson, A., Signorovitch, J., & Canetta, P. (2024). WCN24-479 CHARACTERISTICS OF PATIENTS WITH COMPLEMENT 3 GLOMERULOPATHY (C3G) IN A US MULTI-CENTER ASSESSMENT. Kidney International Reports, 9(4), S243. https://doi.org/10.1016/j.ekir.2024.02.499
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DiFranza, L. T., Daniel, E., Serban, G., Thomas, S. M., Santoriello, D., Ratner, L. E., D’Agati, V. D., Vasilescu, E.-R., Husain, S. A., & Batal, I. (2024). Collapsing glomerulopathy is likely a major contributing factor for worse allograft survival in patients receiving kidney transplants from black donors. Frontiers in Medicine, 11. https://doi.org/10.3389/fmed.2024.1369225
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Raina, R., Jothi, S., Haffner, D., Somers, M., Filler, G., Vasistha, P., Chakraborty, R., Shapiro, R., Randhawa, P. S., Parekh, R., Licht, C., Bunchman, T., Sethi, S., Mangat, G., Zaritsky, J., Schaefer, F., Warady, B., Bartosh, S., McCulloch, M., … Radhakrishnan, J. (2024). Post-transplant recurrence of focal segmental glomerular sclerosis: consensus statements. Kidney International, 105(3), 450–463. https://doi.org/10.1016/j.kint.2023.10.017
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Gbadegesin, R., Martinelli, E., Gupta, Y., Friedman, D. J., Sampson, M. G., Pollak, M. R., & Sanna-Cherchi, S. (2024). APOL1 genotyping is incomplete without testing for the protective M1 modifier p.N264K variant. Glomerular Diseases. Portico. https://doi.org/10.1159/000537948
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Duret, L. C., Hamidouche, T., Steers, N. J., Pons, C., Soubeiran, N., Buret, D., Gilson, E., Gharavi, A. G., D’Agati, V. D., & Shkreli, M. (2024). Targeting WIP1 phosphatase promotes partial remission in experimental collapsing glomerulopathy. Kidney International, 105(5), 980–996. https://doi.org/10.1016/j.kint.2024.02.009
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Avasare, R. S., Clark, S., Spain, R. I., Wusirika, R., Rope, R., Gurley, S., Stanaway, M., Sekulic, M., Santoriello, D., Bomback, A. S., Canetta, P., Iyer, S. J., Kung, V., Charu, V., Troxell, M. L., Kudose, S., & Andeen, N. K. (2024). Characteristics and Outcomes of NELL1 Membranous Nephropathy in Lipoic Acid Users and Nonusers. Kidney International Reports, 9(5), 1379–1386. https://doi.org/10.1016/j.ekir.2024.02.1401
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Andeen, N. K., DiFranza, L., Kung, V. L., Henriksen, K., Gupta, R., Dinesh, K., Akilesh, S., Kudose, S., Smith, K. D., & Troxell, M. L. (2024). AA amyloidosis With Ig-Dominant Staining and Diagnostically Unusual Features. Kidney International Reports, 9(1), 162–170. https://doi.org/10.1016/j.ekir.2023.10.005
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Gupta, Y., Friedman, D. J., McNulty, M. T., Khan, A., Lane, B., Wang, C., Ke, J., Jin, G., Wooden, B., Knob, A. L., Lim, T. Y., Appel, G. B., Huggins, K., Liu, L., Mitrotti, A., Stangl, M. C., Bomback, A., Westland, R., Bodria, M., … Sanna-Cherchi, S. (2023). Strong protective effect of the APOL1 p.N264K variant against G2-associated focal segmental glomerulosclerosis and kidney disease. Nature Communications, 14(1). https://doi.org/10.1038/s41467-023-43020-9
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Rajasekaran, A., Larkina, M., Julian, B. A., Canetta, P. A., Roehm, B. A., Khalid, M., Mariani, L. H., & Rizk, D. V. (2023). Optimal Conservative Therapy Use among Adult Cure Glomerulonephropathy Participants with IgA Nephropathy. Kidney360, 4(12), 1763–1769. https://doi.org/10.34067/kid.0000000000000306
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Peters, D. T., Amburn, J., Tuchman, S., Lichtman, E. I., Jensen, C. E., Jain, K., Lentzsch, S., Chakraborty, R., Bhutani, D., & Rubinstein, S. M. (2023). Outcomes Associated with Clone-Directed Therapies for Monoclonal Gammopathy of Renal Significance. Blood, 142(Supplement 1), 2027–2027. https://doi.org/10.1182/blood-2023-189341
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Sethi, S., Beck, L. H., Glassock, R. J., Haas, M., De Vriese, A. S., Caza, T. N., Hoxha, E., Lambeau, G., Tomas, N. M., Madden, B., Debiec, H., D’Agati, V. D., Alexander, M. P., Amer, H., Appel, G. B., Barbour, S. J., Caravaca-Fontan, F., Cattran, D. C., Casal Moura, M., … Fervenza, F. C. (2023). Mayo Clinic Consensus Report on Membranous Nephropathy: Proposal for a Novel Classification. Mayo Clinic Proceedings, 98(11), 1671–1684. https://doi.org/10.1016/j.mayocp.2023.08.006
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Sethi, S., Beck, L. H., Glassock, R. J., Haas, M., De Vriese, A. S., Caza, T. N., Hoxha, E., Lambeau, G., Tomas, N. M., Madden, B., Debiec, H., D’Agati, V. D., Alexander, M. P., Amer, H., Appel, G. B., Barbour, S. J., Caravaca-Fontan, F., Cattran, D. C., Casal Moura, M., … Fervenza, F. C. (2023). Mayo Clinic consensus report on membranous nephropathy: proposal for a novel classification. Kidney International, 104(6), 1092–1102. https://doi.org/10.1016/j.kint.2023.06.032
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Oliva-Damaso, N., & Bomback, A. S. (2023). Intravenous Methylprednisolone in Induction Therapy for ANCA-Associated Vasculitis: How Low Can We Go? Kidney360, 4(9), e1199–e1200. https://doi.org/10.34067/kid.0000000000000257
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Cortazar, F. B., Cerda, J., Dhanani, R., Roglieri, J., & Santoriello, D. (2023). Avacopan in Patients With Rapidly Progressive Glomerulonephritis Requiring Dialysis. Kidney International Reports, 8(8), 1687–1691. https://doi.org/10.1016/j.ekir.2023.05.017
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Lamanna, N., Tam, C., Jurczak, W., Kontos, A., Konig, H., Ruppert, A. S., & Tsai, D. E. (2023). P1088: COMPARISON OF BLEEDING-RELATED EVENTS IN PATIENTS WHO RECEIVED PIRTOBRUTINIB WITH AND WITHOUT ANTITHROMBOTIC AGENTS. HemaSphere, 7(S3), e724362f. https://doi.org/10.1097/01.hs9.0000971248.72436.2f
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Marasa, M., Ahram, D. F., Rehman, A. U., Mitrotti, A., Abhyankar, A., Jain, N. G., Weng, P. L., Piva, S. E., Fernandez, H. E., Uy, N. S., Chatterjee, D., Kil, B. H., Nestor, J. G., Felice, V., Robinson, D., Whyte, D., Gharavi, A. G., Appel, G. B., Radhakrishnan, J., … Sanna-Cherchi, S. (2023). Implementation and Feasibility of Clinical Genome Sequencing Embedded Into the Outpatient Nephrology Care for Patients With Proteinuric Kidney Disease. Kidney International Reports, 8(8), 1638–1647. https://doi.org/10.1016/j.ekir.2023.05.021
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Kudose, S., Cossey, L. N., Canetta, P. A., Sekulic, M., Vanbeek, C. A., Huls, F. B., Gupta, I., Bu, L., Alexander, M. P., Cornell, L. D., Fidler, M. E., Markowitz, G. S., Larsen, C. P., D’Agati, V. D., Nasr, S. H., & Santoriello, D. (2023). Clinicopathologic Spectrum of Lysozyme-Associated Nephropathy. Kidney International Reports, 8(8), 1585–1595. https://doi.org/10.1016/j.ekir.2023.05.007
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See, S. B., Yang, X., Burger, C., Lamarthée, B., Snanoudj, R., Shihab, R., Tsapepas, D. S., Roy, P., Larivière-Beaudoin, S., Hamelin, K., Mendoza Rojas, A., van Besouw, N. M., Bartosic, A., Daniel, N., Rodica, V. E., Mohan, S., Cohen, D., Ratner, L., Baan, C. C., … Zorn, E. (2023). Natural Antibodies Are Associated With Rejection and Long-term Renal Allograft Loss in a Multicenter International Cohort. Transplantation, 107(7), 1580–1592. https://doi.org/10.1097/tp.0000000000004472
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Mottl, A. K., Bomback, A. S., Mariani, L. H., Coppock, G., Jennette, J. C., Almaani, S., Gipson, D. S., Kelley, S., Kidd, J., Laurin, L.-P., Mucha, K., Oliverio, A., Palmer, M., Rizk, D., Sanghani, N., Stokes, M. B., Susztak, K., Wadhwani, S., & Nast, C. C. (2023). CureGN-Diabetes Study: Rationale, Design, and Methods of a Prospective Observational Study of Glomerular Disease Patients with Diabetes. Glomerular Diseases, 155–164. Portico. https://doi.org/10.1159/000531679
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Perkovic, V., Barratt, J., Radhakrishnan, J., Rizk, D., Trimarchi, H., Khawaja, Z., Leiske, J., Sorensen, B., King, A., Jones-Burton, C., & Campbell, K. (2023). #4333 A PHASE 3, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED STUDY OF BION-1301 IN ADULTS WITH IGA NEPHROPATHY. Nephrology Dialysis Transplantation, 38(Supplement_1). https://doi.org/10.1093/ndt/gfad063c_4333
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Jurczak, W., Lamanna, N., Tam, C. S., Kontos, A., Konig, H., Ruppert, A. S., & Tsai, D. E. (2023). COMPARISON OF BLEEDING‐RELATED EVENTS IN PATIENTS WHO RECEIVED PIRTOBRUTINIB WITH AND WITHOUT ANTITHROMBOTIC AGENTS. Hematological Oncology, 41(S2), 758–759. Portico. https://doi.org/10.1002/hon.3165_605
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Okabayashi, Y., Tsuboi, N., Marumoto, H., Sasaki, T., Haruhara, K., Kanzaki, G., Koike, K., Ueda, H., Shimizu, A., Puelles, V. G., D’Agati, V., & Yokoo, T. (2023). Single-Nephron GFR in Different Glomerular Basement Membrane Stages of Membranous Nephropathy. Kidney360, 4(6), e777–e786. https://doi.org/10.34067/kid.0000000000000142
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Bomback, A. (2023). Plain language summary of the design of the APPEAR-C3G Study: A Study to Learn How Well Iptacopan Works in People with Complement 3 Glomerulopathy. Future Rare Diseases, 3(2). https://doi.org/10.2217/frd-2022-0020
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Zuo, Y., Navaz, S., Liang, W., Li, C., Ayers, C. R., Rysenga, C. E., Harbaugh, A., Norman, G. L., Solow, E. B., Bermas, B., Akinmolayemi, O., Rohatgi, A., Karp, D. R., Knight, J. S., & de Lemos, J. A. (2023). Prevalence of Antiphospholipid Antibodies and Association With Incident Cardiovascular Events. JAMA Network Open, 6(4), e236530. https://doi.org/10.1001/jamanetworkopen.2023.6530
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Reynolds, M. L., Oliverio, A. L., Zee, J., Hendren, E. M., O’Shaughnessy, M. M., Ayoub, I., Almaani, S., Vasylyeva, T. L., Twombley, K. E., Wadhwani, S., Steinke, J. M., Rizk, D. V., Waldman, M., Helmuth, M. E., Avila-Casado, C., Alachkar, N., Nester, C. M., Derebail, V. K., Hladunewich, M. A., & Mariani, L. H. (2023). Pregnancy History and Kidney Disease Progression Among Women Enrolled in Cure Glomerulonephropathy. Kidney International Reports, 8(4), 805–817. https://doi.org/10.1016/j.ekir.2023.01.036
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Mariani, L. H., Eddy, S., AlAkwaa, F. M., McCown, P. J., Harder, J. L., Nair, V., Eichinger, F., Martini, S., Ademola, A. D., Boima, V., Reich, H. N., El Saghir, J., Godfrey, B., Ju, W., Tanner, E. C., Vega-Warner, V., Wys, N. L., Adler, S. G., Appel, G. B., … Kretzler, M. (2023). Precision nephrology identified tumor necrosis factor activation variability in minimal change disease and focal segmental glomerulosclerosis. Kidney International, 103(3), 565–579. https://doi.org/10.1016/j.kint.2022.10.023
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Kallash, M., Wang, Y., Smith, A., Trachtman, H., Gbadegesin, R., Nester, C., Canetta, P., Wang, C., Hunley, T. E., Sperati, C. J., Selewski, D., Ayoub, I., Srivastava, T., Mottl, A. K., Kopp, J., Gillespie, B., Robinson, B., Chen, D., … Steinke, J. (2023). Rapid Progression of Focal Segmental Glomerulosclerosis in Patients with High-Risk APOL1 Genotypes. Clinical Journal of the American Society of Nephrology, 18(3), 344–355. https://doi.org/10.2215/cjn.0000000000000069
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Batal, I., Khairallah, P., Weins, A., Andeen, N. K., & Stokes, M. B. (2023). The role of HLA antigens in recurrent primary focal segmental glomerulosclerosis. Frontiers in Immunology, 14. https://doi.org/10.3389/fimmu.2023.1124249
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Nester, C., Appel, G. B., Bomback, A. S., Bouman, K. P., Cook, H. T., Daina, E., Dixon, B. P., Rice, K., Najafian, N., Hui, J., Podos, S. D., Langman, C. B., Lightstone, L., Parikh, S. V., Pickering, M. C., Sperati, C. J., Trachtman, H., Tumlin, J., de Vries, A. P., … Remuzzi, G. (2022). Clinical Outcomes of Patients with C3G or IC-MPGN Treated with the Factor D Inhibitor Danicopan: Final Results from Two Phase 2 Studies. American Journal of Nephrology, 53(10), 687–700. Portico. https://doi.org/10.1159/000527167
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Kudose, S., Batal, I., Lucia, J., Patel, P., Soni, R. K., Markowitz, G. S., D’Agati, V. D., & Stokes, M. B. (2023). Proliferative Glomerulonephritis With Hidden Monotypic IgG3κ Deposits: A Case Report. American Journal of Kidney Diseases, 81(1), 114–117. https://doi.org/10.1053/j.ajkd.2022.05.018
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Podos, S. D., Trachtman, H., Appel, G. B., Bomback, A. S., Dixon, B. P., Wetzels, J. F. M., Cook, H. T., Parikh, S. V., Pickering, M. C., Tumlin, J., Langman, C. B., Lightstone, L., Sperati, C. J., Daina, E., Bouman, K. P., Rice, K., Thanassi, J. A., Huang, M., Nester, C., & Remuzzi, G. (2022). Baseline Clinical Characteristics and Complement Biomarkers of Patients with C3 Glomerulopathy Enrolled in Two Phase 2 Studies Investigating the Factor D Inhibitor Danicopan. American Journal of Nephrology, 53(10), 675–686. Portico. https://doi.org/10.1159/000527166
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DiFranza, L. T., Chafouleas, E., Katipally, S., Stokes, M. B., Kudose, S., & Sekulic, M. (2022). Crescentic Fibrillary Glomerulonephritis in the Setting of Immune Checkpoint Inhibitor Therapy: A Report of Two Cases. Glomerular Diseases, 3(1), 69–74. Portico. https://doi.org/10.1159/000528881
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Ravipati, P., Freese, R. L., Royal, V., Bu, L., Canetta, P., Gipson, D., Kallash, M., Kiryluk, K., Nast, C., Reich, H. N., Rheault, M. N., Saha, M., & Nachman, P. H. (2022). Clinicopathologic Significance of Predominant Lambda Light Chain Deposition in IgA Nephropathy. Kidney International Reports, 7(11), 2462–2473. https://doi.org/10.1016/j.ekir.2022.08.003
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Bomback, A. S., Kavanagh, D., Vivarelli, M., Meier, M., Wang, Y., Webb, N. J. A., Trapani, A. J., & Smith, R. J. H. (2022). Alternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial. Kidney International Reports, 7(10), 2150–2159. https://doi.org/10.1016/j.ekir.2022.07.004
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Jones, A. V., Curtiss, D., Harris, C., Southerington, T., Hautalahti, M., Wihuri, P., Mäkelä, J., Kallionpää, R. E., Makkonen, E., Knopp, T., Mannermaa, A., Mäkinen, E., Moilanen, A.-M., Tezel, T. H., & Waheed, N. K. (2022). An assessment of prevalence of Type 1 CFI rare variants in European AMD, and why lack of broader genetic data hinders development of new treatments and healthcare access. PLOS ONE, 17(9), e0272260. https://doi.org/10.1371/journal.pone.0272260
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Floege, J., Gibson, K. L., Praga, M., Radhakrishnan, J., Reich, H. N., Schreuder, M. F., Wetzels, J. F., Tesař, V., Vivarelli, M., Biechele, S., & Tonelli, M. (2022). Diagnosis and Treatment of Patients With Focal Segmental Glomerulosclerosis/Steroid-Resistant Nephrotic Syndrome: A Delphi Survey. Kidney International Reports, 7(9), 2081–2085. https://doi.org/10.1016/j.ekir.2022.06.010
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