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1. Multidisciplinary working group: key role for percutaneous endovascular AV fistula program. Point of view

Author: Fanelli, Fabrizio, Falcone, G., Gabbani, G., Annese, A. L., Gianassi, I., Cutruzzulla, R., Dervishi, E., and Cirami, L.
Published: 2024
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2. Evidence from the large VALIGA cohort validates the subclassification of focal segmental glomerulosclerosis in IgA nephropathy

Author: Coppo, R., Feehaly, J., Troyanov, S., Cattran, D.C., Cook, H.T., Roberts, I., Radcliffe, John, Russo, M.L., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Papagianni, K., Stangou, M., Giacchino, F., Goumenos, D., Papasotirious, M., Kalliakmani, P., Gerolymos, M., Galesic, K., Toric, L., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Hryszko, T., Klinger, M., Kamińska, D., Krajewska, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Cambier, A., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Diomedi-Casadei, F., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Royal, V., Bellur, Shubha S., Troyanov, Stéphan, Vorobyeva, Olga, Coppo, Rosanna, and Roberts, Ian S.D.
Published: 2024
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3. Application of the International IgA Nephropathy Prediction Tool one or two years post-biopsy

Author: Russo, M.L., Troyanov, S., Cook, H.T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Papachristou, E., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Smerud, H. Kloster, Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Terroba, J. Arce, Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Ljubanovic, D. Galesic, Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Bavbek, N., Cook, T., Alpers, C., Berthoux, F., Bonsib, S., D’Agati, V., D’Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zeng, C.-H., Shi, S., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Barbour, Sean J., Coppo, Rosanna, Zhang, Hong, Liu, Zhi-Hong, Suzuki, Yusuke, Matsuzaki, Keiichi, Er, Lee, Reich, Heather N., Barratt, Jonathan, and Cattran, Daniel C.
Published: 2022
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4. Development and testing of an artificial intelligence tool for predicting end-stage kidney disease in patients with immunoglobulin A nephropathy

Author: Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Coppo, R., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Angioi, A., Piras, L., Feehally, J., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Papasotiriou, M., Galesic, K., Toric, L., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Smerud, H. Kloster, Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Rauen, T., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Grinyo, J.M. Boria, Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Martina, G., Messuerotti, A., Canton, A. Dal, Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Mazzucco, Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Terroba, J. Arce, Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., GalesicLjubanovic, D., Gakiopoulou, H., Bertoni, E., Ortiz, P. Cannata, Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., FulladosaOliveras, X., Maldyk, J., Ioachim, E., Abbrescia, Daniela, Kouri, Nikoleta, Stangou, Maria, Papagianni, Aikaterini, Scolari, Francesco, Delbarba, Elisa, Bonomini, Mario, Piscitani, Luca, Stallone, Giovanni, Infante, Barbara, Godeas, Giulia, Madio, Desiree, Biancone, Luigi, Campagna, Marco, Zaza, Gianluigi, Squarzoni, Isabella, Cangemi, Concetta, Schena, Francesco Paolo, Anelli, Vito Walter, Trotta, Joseph, Di Noia, Tommaso, Manno, Carlo, Tripepi, Giovanni, D’Arrigo, Graziella, Chesnaye, Nicholas C., Russo, Maria Luisa, Zoccali, Carmine, Tesar, Vladimir, and Coppo, Rosanna
Published: 2021
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5. Improving treatment decisions using personalized risk assessment from the International IgA Nephropathy Prediction Tool

Author: Russo, M.L., Troyanov, S., Cook, H.T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Bavbek, N., Cook, T., Alpers, C., Berthoux, F., Bonsib, S., D’Agati, V., D’Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa N, N., Zeng, C.-H., Shi, S., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Barbour, Sean J., Canney, Mark, Coppo, Rosanna, Zhang, Hong, Liu, Zhi-Hong, Suzuki, Yusuke, Matsuzaki, Keiichi, Katafuchi, Ritsuko, Induruwage, Dilshani, Er, Lee, Reich, Heather N., Feehally, John, Barratt, Jonathan, and Cattran, Daniel C.
Published: 2020
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6. Application of the International IgA Nephropathy Prediction Tool one or two years post-biopsy

Author: Barbour, Sean J., primary, Coppo, Rosanna, additional, Zhang, Hong, additional, Liu, Zhi-Hong, additional, Suzuki, Yusuke, additional, Matsuzaki, Keiichi, additional, Er, Lee, additional, Reich, Heather N., additional, Barratt, Jonathan, additional, Cattran, Daniel C., additional, Russo, M.L., additional, Troyanov, S., additional, Cook, H.T., additional, Roberts, I., additional, Tesar, V., additional, Maixnerova, D., additional, Lundberg, S., additional, Gesualdo, L., additional, Emma, F., additional, Fuiano, L., additional, Beltrame, G., additional, Rollino, C., additional, Amore, A., additional, Camilla, R., additional, Peruzzi, L., additional, Praga, M., additional, Feriozzi, S., additional, Polci, R., additional, Segoloni, G., additional, Colla, L., additional, Pani, A., additional, Piras, D., additional, Angioi, A., additional, Cancarini, G., additional, Ravera, S., additional, Durlik, M., additional, Moggia, E., additional, Ballarin, J., additional, Di Giulio, S., additional, Pugliese, F., additional, Serriello, I., additional, Caliskan, Y., additional, Sever, M., additional, Kilicaslan, I., additional, Locatelli, F., additional, Del Vecchio, L., additional, Wetzels, J.F.M., additional, Peters, H., additional, Berg, U., additional, Carvalho, F., additional, da Costa Ferreira, A.C., additional, Maggio, M., additional, Wiecek, A., additional, Ots-Rosenberg, M., additional, Magistroni, R., additional, Topaloglu, R., additional, Bilginer, Y., additional, D’Amico, M., additional, Stangou, M., additional, Giacchino, F., additional, Goumenos, D., additional, Papachristou, E., additional, Galesic, K., additional, Geddes, C., additional, Siamopoulos, K., additional, Balafa, O., additional, Galliani, M., additional, Stratta, P., additional, Quaglia, M., additional, Bergia, R., additional, Cravero, R., additional, Salvadori, M., additional, Cirami, L., additional, Fellstrom, B., additional, Smerud, H. Kloster, additional, Ferrario, F., additional, Stellato, T., additional, Egido, J., additional, Martin, C., additional, Floege, J., additional, Eitner, F., additional, Lupo, A., additional, Bernich, P., additional, Menè, P., additional, Morosetti, M., additional, van Kooten, C., additional, Rabelink, T., additional, Reinders, M.E.J., additional, Boria Grinyo, J.M., additional, Cusinato, S., additional, Benozzi, L., additional, Savoldi, S., additional, Licata, C., additional, Mizerska-Wasiak, M., additional, Martina, G., additional, Messuerotti, A., additional, Dal Canton, A., additional, Esposito, C., additional, Migotto, C., additional, Triolo, G., additional, Mariano, F., additional, Pozzi, C., additional, Boero, R., additional, Bellur, S., additional, Mazzucco, G., additional, Giannakakis, C., additional, Honsova, E., additional, Sundelin, B., additional, Di Palma, A.M., additional, Gutiérrez, E., additional, Asunis, A.M., additional, Barratt, J., additional, Tardanico, R., additional, Perkowska-Ptasinska, A., additional, Terroba, J. Arce, additional, Fortunato, M., additional, Pantzaki, A., additional, Ozluk, Y., additional, Steenbergen, E., additional, Soderberg, M., additional, Riispere, Z., additional, Furci, L., additional, Orhan, D., additional, Kipgen, D., additional, Casartelli, D., additional, Ljubanovic, D. Galesic, additional, Gakiopoulou, H., additional, Bertoni, E., additional, Cannata Ortiz, P., additional, Karkoszka, H., additional, Groene, H.J., additional, Stoppacciaro, A., additional, Bajema, I., additional, Bruijn, J., additional, Fulladosa Oliveras, X., additional, Maldyk, J., additional, Ioachim, E., additional, Bavbek, N., additional, Cook, T., additional, Alpers, C., additional, Berthoux, F., additional, Bonsib, S., additional, D’Agati, V., additional, D’Amico, G., additional, Emancipator, S., additional, Emmal, F., additional, Fervenza, F., additional, Florquin, S., additional, Fogo, A., additional, Groene, H., additional, Haas, M., additional, Hill, P., additional, Hogg, R., additional, Hsu, S., additional, Hunley, T., additional, Hladunewich, M., additional, Jennette, C., additional, Joh, K., additional, Julian, B., additional, Kawamura, T., additional, Lai, F., additional, Leung, C., additional, Li, L., additional, Li, P., additional, Liu, Z., additional, Massat, A., additional, Mackinnon, B., additional, Mezzano, S., additional, Schena, F., additional, Tomino, Y., additional, Walker, P., additional, Wang, H., additional, Weening, J., additional, Yoshikawa, N., additional, Zeng, C.-H., additional, Shi, S., additional, Nogi, C., additional, Suzuki, H., additional, Koike, K., additional, Hirano, K., additional, Yokoo, T., additional, Hanai, M., additional, Fukami, K., additional, Takahashi, K., additional, Yuzawa, Y., additional, Niwa, M., additional, Yasuda, Y., additional, Maruyama, S., additional, Ichikawa, D., additional, Suzuki, T., additional, Shirai, S., additional, Fukuda, A., additional, Fujimoto, S., additional, and Trimarchi, H., additional
Published: 2022
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7. The Role of Rituximab in Primary Focal Segmental Glomerulosclerosis of the Adult

Author: Tedesco, M, Mescia, F, Pisani, I, Allinovi, M, Casazza, G, Del Vecchio, L, Santostefano, M, Cirillo, L, Ferrario, F, Esposito, C, Esposito, P, Santoro, D, Lazzarin, R, Rossi, G, Fiaccadori, E, Ferrantelli, A, Sinico, R, Cozzolino, M, Gallieni, M, Cirami, L, Scolari, F, Vaglio, A, Alberici, F, Affatato, S, Caroti, L, Mancini, E, Semeraro, L, Siligato, R, Cassia, M, Napodano, P, Calatroni, M, Distratis, C, Campo, A, Tedesco, Martina, Mescia, Federica, Pisani, Isabella, Allinovi, Marco, Casazza, Giovanni, Del Vecchio, Lucia, Santostefano, Marisa, Cirillo, Luigi, Ferrario, Francesca, Esposito, Ciro, Esposito, Pasquale, Santoro, Domenico, Lazzarin, Roberta, Rossi, Giovanni Maria, Fiaccadori, Enrico, Ferrantelli, Angelo, Sinico, Renato Alberto, Cozzolino, Mario, Gallieni, Maurizio, Cirami, Lino, Scolari, Francesco, Vaglio, Augusto, Alberici, Federico, Affatato, Stefania, Caroti, Leonardo, Mancini, Elena, Semeraro, Luca, Siligato, Rossella, Cassia, Matthias Arnaldo, Napodano, Pietro, Calatroni, Marta, Distratis, Cosimo, Campo, Andrea, Tedesco, M, Mescia, F, Pisani, I, Allinovi, M, Casazza, G, Del Vecchio, L, Santostefano, M, Cirillo, L, Ferrario, F, Esposito, C, Esposito, P, Santoro, D, Lazzarin, R, Rossi, G, Fiaccadori, E, Ferrantelli, A, Sinico, R, Cozzolino, M, Gallieni, M, Cirami, L, Scolari, F, Vaglio, A, Alberici, F, Affatato, S, Caroti, L, Mancini, E, Semeraro, L, Siligato, R, Cassia, M, Napodano, P, Calatroni, M, Distratis, C, Campo, A, Tedesco, Martina, Mescia, Federica, Pisani, Isabella, Allinovi, Marco, Casazza, Giovanni, Del Vecchio, Lucia, Santostefano, Marisa, Cirillo, Luigi, Ferrario, Francesca, Esposito, Ciro, Esposito, Pasquale, Santoro, Domenico, Lazzarin, Roberta, Rossi, Giovanni Maria, Fiaccadori, Enrico, Ferrantelli, Angelo, Sinico, Renato Alberto, Cozzolino, Mario, Gallieni, Maurizio, Cirami, Lino, Scolari, Francesco, Vaglio, Augusto, Alberici, Federico, Affatato, Stefania, Caroti, Leonardo, Mancini, Elena, Semeraro, Luca, Siligato, Rossella, Cassia, Matthias Arnaldo, Napodano, Pietro, Calatroni, Marta, Distratis, Cosimo, and Campo, Andrea
Abstract: Introduction: Primary focal segmental glomerular sclerosis (FSGS) is a rare, likely immune-mediated disease. Rituximab (RTX) may play a role in management, although data in adults are scanty. Methods: We collected cases of RTX-treated primary FSGS within the Italian Society of Nephrology Immunopathology Working Group and explored response rate (24-hour proteinuria <3.5 g and <50% compared with baseline, stable estimated glomerular filtration rate). Results: A total of 31 patients were followed for at least 12 months; further follow-up (median 17 months, interquartile range [IQR] 15–33.5) was available for 11. At first RTX administration, median creatinine and 24-hour proteinuria were 1.17 mg/dl (IQR 0.83–1.62) and 5.2 g (IQR 3.3–8.81), respectively. Response rate at 3, 6, and 12 months was 39%, 52%, and 42%, respectively. In the first 12 months, creatinine level remained stable whereas proteinuria and serum albumin level improved, with an increase in the proportion of patients tapering other immunosuppressants. There were 6 patients who were retreated with RTX within 12 months, either for proteinuria increase or refractory disease; only the 2 responders to the first RTX course experienced a further response. At univariate analysis, 6-month response was more frequent in steroid-dependent patients (odds ratio [OR] 7.7 [95% CI 1.16–52.17]) and those with proteinuria <5 g/24 h (OR 8.25 [1.45–46.86]). During long-term follow-up, 4 of 5 responders at 12 months maintained a sustained response, either without further immunosuppression (2 of 4) or with pre-emptive RTX (2 of 4); 1 relapsed and responded to RTX retreatment. Conclusion: RTX may be an option in primary FSGS, especially in steroid-dependent patients, with 24-hour proteinuria <5 g and previously responders to RTX. Optimal long-term management for responders is unclear, with some patients experiencing sustained remission and others requiring RTX retreatment, either preemptive or after rising proteinuria
Published: 2022

8. Is there long-term value of pathology scoring in immunoglobulin A nephropathy? A validation study of the Oxford Classification for IgA Nephropathy (VALIGA) update

Author: Coppo, R., D'Arrigo, G., Tripepi, G., Russo, M. L., Roberts, I. S. D., Bellur, S., Cattran, D., Cook, T. H., Feehally, J., Tesar, V., Maixnerova, D., Peruzzi, L., Amore, A., Lundberg, S., Di Palma, A. M., Gesualdo, L., Emma, F., Rollino, C., Praga, M., Biancone, L., Pani, A., Feriozzi, S., Polci, R., Barratt, J., Del Vecchio, L., Locatelli, F., Pierucci, A., Caliskan, Y., Perkowska-Ptasinska, A., Durlik, M., Moggia, E., Ballarin, J. C., Wetzels, J. F. M., Goumenos, D., Papasotiriou, M., Galesic, K., Toric, L., Papagianni, A., Stangou, M., Benozzi, L., Cusinato, S., Berg, U., Topaloglu, R., Maggio, M., Ots-Rosenberg, M., D'Amico, M., Geddes, C., Balafa, O., Quaglia, M., Cravero, R., Cirami, C. L., Fellstrom, B., Floege, J., Egido, J., Mallamaci, F., Zoccali, C., Fuiano, L., Beltrame, G., Camilla, R., Segoloni, G., Colla, L., Angioi, A., Piras, L., Cancarini, G., Ravera, S., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Sever, M., Kilicaslan, I., Peters, H., Carvalho, F., Da Costa Ferreira, A. C., Wiecek, A., Magistroni, R., Bilginer, Y., Giacchino, F., Papastirou, M., Siamopoulos, K., Galliani, M., Stratta, P., Bergia, R., Salvadori, M., Cirami, L., Kloster Smerud, H., Ferrario, F., Stellato, T., Martin, C., Eitner, F., Rauen, T., Lupo, A., Bernich, P., Mene, P., Morosetti, M., Van Kooten, C., Rabelink, T., Reinders, M. E. J., Boria Grinyo, J. M., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Gutierrez, E., Asunis, A. M., Tardanico, R., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Akiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., and Internal Medicine
Subjects: Adult, Male, Pathology, medicine.medical_specialty, Adolescent, 030232 urology & nephrology, Renal function, 030204 cardiovascular system & hematology, Kidney, Nephropathy, Cohort Studies, 03 medical and health sciences, Glomerulonephritis, 0302 clinical medicine, renal biopsy, Interquartile range, IgA nephropathy, progression, risk factors, Child, Disease Progression, Female, Follow-Up Studies, Glomerular Filtration Rate, Glomerulonephritis, IGA, Humans, Prognosis, Medicine, Endocapillary hypercellularity, IGA, Transplantation, medicine.diagnostic_test, business.industry, medicine.disease, medicine.anatomical_structure, Renal pathology, Nephrology, Cohort, Renal biopsy, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business
Abstract: Background It is unknown whether renal pathology lesions in immunoglobulin A nephropathy (IgAN) correlate with renal outcomes over decades of follow-up. Methods In 1130 patients of the original Validation Study of the Oxford Classification for IgA Nephropathy (VALIGA) cohort, we studied the relationship between the MEST score (mesangial hypercellularity, M; endocapillary hypercellularity, E; segmental glomerulosclerosis, S; tubular atrophy/interstitial fibrosis, T), crescents (C) and other histological lesions with both a combined renal endpoint [50% estimated glomerular filtration rate (eGFR) loss or kidney failure] and the rate of eGFR decline over a follow-up period extending to 35 years [median 7 years (interquartile range 4.1–10.8)]. Results In this extended analysis, M1, S1 and T1–T2 lesions as well as the whole MEST score were independently related with the combined endpoint (P Conclusion Long-term follow-up analyses of the VALIGA cohort showed that the independent relationship between kidney biopsy findings and the risk of progression towards kidney failure in IgAN remains unchanged across all age groups and decades after the renal biopsy.
Published: 2020
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9. Perioperative and functional outcomes after kidney transplantation from uncontrolled donors after circulatory death vs. expanded-criteria donors after brain death vs. standard-criteria donors: Toward an evidence-based refinement of allocation strategies

Author: Campi, R., primary, Pecoraro, A., additional, Sessa, F., additional, Barzaghi, P., additional, Rivetti, A., additional, Nicoletti, R., additional, Gallo, M.L., additional, Corti, F., additional, Morselli, S., additional, Spatafora, P., additional, Gacci, M., additional, Giancane, S., additional, Sebastianelli, A., additional, Cirami, L., additional, Peris, A., additional, Vignolini, G., additional, Serni, S., additional, and Li Marzi, V., additional
Published: 2022
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10. Quantifying duration of proteinuria remission and association with clinical outcome in IgA nephropathy

Author: Canney, M., Barbour, S. J., Zheng, Y., Coppo, R., Zhang, H., Liu, Z. -H., Matsuzaki, K., Suzuki, Y., Katafuchi, R., Reich, H. N., Cattran, D., Russo, M. L., Troyanov, S., Cook, H. T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J. F. M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A. C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Mene, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M. E. J., Boria Grinyo, J. M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A. M., Gutierrez, E., Asunis, A. M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Bavbek, N., Cook, T., Alpers, C., Feehally, J., Berthoux, F., Bonsib, S., D'Agati, V., D'Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zeng, C. -H., Shi, S., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Anesthesiology, Pathology, Graduate School, ACS - Heart failure & arrhythmias, and ACS - Atherosclerosis & ischemic syndromes
Subjects: Male, Time Factors, glomerular disease, 030232 urology & nephrology, 030204 cardiovascular system & hematology, Gastroenterology, 0302 clinical medicine, Outcome Assessment, Health Care, Clinical endpoint, Medicine, Clinical Epidemiology, Proteinuria, medicine.diagnostic_test, Remission Induction, Hazard ratio, IgA nephropathy, General Medicine, Middle Aged, end stage kidney disease, epidemiology and outcomes, proteinuria, renal function decline, renal pathology, 3. Good health, Renal pathology, Nephrology, Disease Progression, Female, medicine.symptom, Glomerular Filtration Rate, Adult, medicine.medical_specialty, Nephropathy, 03 medical and health sciences, Internal medicine, Biopsy, Humans, Proportional Hazards Models, Retrospective Studies, business.industry, Surrogate endpoint, Glomerulonephritis, IGA, medicine.disease, Confidence interval, Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], Kidney Failure, Chronic, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business
Abstract: Contains fulltext : 232930.pdf (Publisher’s version ) (Closed access) BACKGROUND: On the basis of findings of observational studies and a meta-analysis, proteinuria reduction has been proposed as a surrogate outcome in IgA nephropathy. How long a reduction in proteinuria needs to be maintained to mitigate the long-term risk of disease progression is unknown. METHODS: In this retrospective multiethnic cohort of adult patients with IgA nephropathy, we defined proteinuria remission as a ≥25% reduction in proteinuria from the peak value after biopsy, and an absolute reduction in proteinuria to
Published: 2021

11. Quantifying duration of proteinuria remission and association with clinical outcome in IgA nephropathy

Author: Canney, M. Barbour, S.J. Zheng, Y. Coppo, R. Zhang, H. Liu, Z.-H. Matsuzaki, K. Suzuki, Y. Katafuchi, R. Reich, H.N. Cattran, D. Russo, M.L. Troyanov, S. Cook, H.T. Roberts, I. Tesar, V. Maixnerova, D. Lundberg, S. Gesualdo, L. Emma, F. Fuiano, L. Beltrame, G. Rollino, C. Amore, A. Camilla, R. Peruzzi, L. Praga, M. Feriozzi, S. Polci, R. Segoloni, G. Colla, L. Pani, A. Piras, D. Angioi, A. Cancarini, G. Ravera, S. Durlik, M. Moggia, E. Ballarin, J. Di Giulio, S. Pugliese, F. Serriello, I. Caliskan, Y. Sever, M. Kilicaslan, I. Locatelli, F. Del Vecchio, L. Wetzels, J.F.M. Peters, H. Berg, U. Carvalho, F. da Costa Ferreira, A.C. Maggio, M. Wiecek, A. Ots-Rosenberg, M. Magistroni, R. Topaloglu, R. Bilginer, Y. D'Amico, M. Stangou, M. Giacchino, F. Goumenos, D. Kalliakmani, P. Gerolymos, M. Galesic, K. Geddes, C. Siamopoulos, K. Balafa, O. Galliani, M. Stratta, P. Quaglia, M. Bergia, R. Cravero, R. Salvadori, M. Cirami, L. Fellstrom, B. Kloster Smerud, H. Ferrario, F. Stellato, T. Egido, J. Martin, C. Floege, J. Eitner, F. Lupo, A. Bernich, P. Menè, P. Morosetti, M. van Kooten, C. Rabelink, T. Reinders, M.E.J. Boria Grinyo, J.M. Cusinato, S. Benozzi, L. Savoldi, S. Licata, C. Mizerska-Wasiak, M. Martina, G. Messuerotti, A. Dal Canton, A. Esposito, C. Migotto, C. Triolo, G. Mariano, F. Pozzi, C. Boero, R. Bellur, S. Mazzucco, G. Giannakakis, C. Honsova, E. Sundelin, B. Di Palma, A.M. Gutiérrez, E. Asunis, A.M. Barratt, J. Tardanico, R. Perkowska-Ptasinska, A. Arce Terroba, J. Fortunato, M. Pantzaki, A. Ozluk, Y. Steenbergen, E. Soderberg, M. Riispere, Z. Furci, L. Orhan, D. Kipgen, D. Casartelli, D. Galesic Ljubanovic, D. Gakiopoulou, H. Bertoni, E. Cannata Ortiz, P. Karkoszka, H. Groene, H.J. Stoppacciaro, A. Bajema, I. Bruijn, J. Fulladosa Oliveras, X. Maldyk, J. Ioachim, E. Bavbek, N. Cook, T. Alpers, C. Feehally, J. Berthoux, F. Bonsib, S. D'Agati, V. D'Amico, G. Emancipator, S. Emmal, F. Fervenza, F. Florquin, S. Fogo, A. Groene, H. Haas, M. Hill, P. Hogg, R. Hsu, S. Hunley, T. Hladunewich Jennette, C. Joh, K. Julian, B. Kawamura, T. Lai, F. Leung, C. Li, L. Li, P. Liu, Z. Massat, A. Mackinnon, B. Mezzano, S. Schena, F. Tomino, Y. Walker, P. Wang, H. Weening, J. Yoshikawa, N. Zeng, C.-H. Shi, S. Nogi, C. Suzuki, H. Koike, K. Hirano, K. Yokoo, T. Hanai, M. Fukami, K. Takahashi, K. Yuzawa, Y. Niwa, M. Yasuda, Y. Maruyama, S. Ichikawa, D. Suzuki, T. Shirai, S. Fukuda, A. Fujimoto, S. Trimarchi, H. International IgA Nephropathy Network
Abstract: Background On the basis of findings of observational studies and a meta-analysis, proteinuria reduction has been proposed as a surrogate outcome in IgA nephropathy. How long a reduction in proteinuria needs to be maintained to mitigate the long-term risk of disease progression is unknown. Methods In this retrospective multiethnic cohort of adult patients with IgA nephropathy, we defined proteinuria remission as a $25% reduction in proteinuria from the peak value after biopsy, and an absolute reduction in proteinuria to,1 g/d. The exposure of interest was the total duration of first remission, treated as a time-varying covariate using longitudinal proteinuria measurements. We used time-dependent Cox proportional hazards regression models to quantify the association between the duration of remission and the primary outcome (ESKD or a 50% reduction in eGFR). Results During a median follow-up of 3.9 years, 274 of 1864 patients (14.7%) experienced the primary outcome. The relationship between duration of proteinuria remission and outcome was nonlinear. Each 3 months in sustained remission up to approximately 4 years was associated with an additional 9% reduction in the risk of disease progression (hazard ratio [HR], 0.91; 95% confidence interval [95% CI], 0.89 to 0.93). Thereafter, each additional 3 months in remission was associated with a smaller, nonsignificant risk reduction (HR, 0.99; 95% CI, 0.96 to 1.03). These findings were robust to multivariable adjustment and consistent across clinical and histologic subgroups. Conclusions Our findings support the use of proteinuria as a surrogate outcome in IgA nephropathy, but additionally demonstrate the value of quantifying the duration of proteinuria remission when estimating the risk of hard clinical endpoints. Copyright © 2021 by the American Society of Nephrology.
Published: 2021

12. Optical Coherence Tomography Angiography for the Evaluation of Retinal Vasculature in Fabry Disease: Our Experience and Review of Current Knowledge

Author: Bacherini, Daniela, Vicini, G., Nicolosi, C., Tanini, I., Lenzetti, C., Finocchio, L., Cirami, L. C., Dervishi, E., Rizzo, Stanislao, Virgili, Gianni, Giansanti, F., Sodi, A., Bacherini D., Rizzo S. (ORCID:0000-0001-6302-063X), Virgili G., Bacherini, Daniela, Vicini, G., Nicolosi, C., Tanini, I., Lenzetti, C., Finocchio, L., Cirami, L. C., Dervishi, E., Rizzo, Stanislao, Virgili, Gianni, Giansanti, F., Sodi, A., Bacherini D., Rizzo S. (ORCID:0000-0001-6302-063X), and Virgili G.
Abstract: Purpose: Optical coherence tomography angiography (OCTA) is a non-invasive and objective tool for the evaluation of the retinal microvascular changes in Fabry disease (FD). We investigated changes in retinal vasculature in FD patients, and the possible correlation with systemic parameters, by using OCTA, and reviewed the current status of literature. Methods: Thirteen FD patients (eight females, five males, mean age 49.85 ± 14.7 years) were compared with 13 age- and sex-matched healthy controls. OCTA 3 × 3 mm macular scans were performed in all subjects. We evaluated the vessel density and vessel perfusion in distinct macular areas (whole, inner, and outer) of both the superficial capillary plexus (SCP VD and SCP VP) and of the deep capillary plexus (DCP VD and DCP VP). We also evaluated the foveal avascular zone (FAZ) metrics (area, perimeter, and circularity), and correlation between systemic and OCTA parameters. A literature review on the current understanding of OCTA in FD is then presented. Results: FD patients showed significantly lower SCP VD values in the whole area (17.37 ± 2.08 mm−1 vs. 18.54 ± 1.21 mm−1; p-value 0.022), as well as in the outer area (17.46 ± 2.10 mm−1 vs. 19.08 ± 1.14 mm−1; p-value 0.002), but not in the inner. Even the DCP VD was significantly lower in all the imaged areas: whole (17.75 ± 3.93 mm−1 vs. 19.71 ± 1.20 mm−1; p-value 0.024), outer (18.25 ± 4.17 mm−1 vs. 20.33 ± 1.20 mm−1; p-value 0.023), and inner (19.54 ± 4.17 mm−1 vs. 21.96 ± 1.55 mm−1; p-value 0.011). There were no significant differences in vessel perfusion parameters (both SCP VP and DCP VP ones) and FAZ. No significant correlations were found between the OCTA parameters and systemic parameters (maximal left ventricular wall thickness and glomerular filtration rate) in FD patients. Conclusions: OCTA can be considered as a promising non-invasive tool, which enables a quantitative evaluation of retinal vascular involvement in FD, despite the varying data reported in literat
Published: 2021

13. Cluster analysis identifies distinct pathogenetic patterns in c3 glomerulopathies/immune complex–Mediated membranoproliferative GN

Author: Iatropoulos, P, Daina, E, Curreri, M, Piras, R, Valoti, E, Mele, C, Bresin, E, Gamba, S, Alberti, M, Breno, M, Perna, A, Bettoni, S, Sabadini, E, Murer, L, Vivarelli, M, Noris, M, Remuzzi, G, Bottanelli, L, Donadelli, R, Cuccarolo, P, Abbate, M, Carrara, C, Cannata, A, Ferrari, S, Gaspari, F, Stucchi, N, Bassani, C, Lena, M, Omati, G, Taruscia, D, Bellantuono, R, Giordano, M, Messina, G, Caruso, M, Gotti, E, Mescia, F, Perticucci, E, Schieppati, A, Verdoni, L, Berto, M, Baraldi, O, Montini, G, Pasini, A, Passler, W, Degasperi, T, Gaggiotti, M, Gregorini, G, Miglietti, N, Guarnieri, A, Cirami, L, Roperto, R, Di Giorgio, G, Barbano, G, Innocenti, M, Ghiggeri, G, Magnasco, A, Rolla, D, Casartelli, D, Lambertini, D, Maggio, M, Cosci, P, Conti, G, Amar, K, Ardissino, G, Marinosci, A, Sinico, R, Montoli, A, Bonucchi, D, Facchini, F, Furci, L, Ferretti, A, Nuzzi, F, Pecoraro, C, Visciano, B, Canavese, C, Radin, E, Stratta, P, Nordio, M, Benetti, E, Parolin, M, Alberici, F, Manenti, L, Brugnano, R, Manenti, F, Capitanini, A, Emma, F, Massella, L, Rosa, M, Mazzon, M, Basso, E, Besso, L, Lavacca, A, Mella, A, Bertero, M, Coppo, R, Peruzzi, L, Porcellini, M, Piccoli, G, Clari, R, Pasi, A, Gangemi, C, Alfandary, H, Dagan, A, Conceiçao, M, Sameiro, F, Croze, L, Malvezzi, P, Tsygin, A, Zelan, B, Nastasi, N, Iatropoulos, Paraskevas, Daina, Erica, Curreri, Manuela, Piras, Rossella, Valoti, Elisabetta, Mele, Caterina, Bresin, Elena, Gamba, Sara, Alberti, Marta, Breno, Matteo, Perna, Annalisa, Bettoni, Serena, Sabadini, Ettore, Murer, Luisa, Vivarelli, Marina, Noris, Marina, Remuzzi, Giuseppe, Bottanelli, L., Donadelli, R., Cuccarolo, P., Abbate, M., Carrara, C., Cannata, A., Ferrari, S., Gaspari, F., Stucchi, N., Bassani, C., Lena, M., Omati, G., Taruscia, D., Bellantuono, R., Giordano, M., Messina, G., Caruso, M., Gotti, E., Mescia, F., Perticucci, E., Schieppati, A., Verdoni, L., Berto, M., Baraldi, O., Montini, G., Pasini, A., Passler, W., Degasperi, T., Gaggiotti, M., Gregorini, G., Miglietti, N., Guarnieri, A., Cirami, L., Roperto, R. M., Di Giorgio, G., Barbano, G., Innocenti, M. L. D., Ghiggeri, G. M., Magnasco, A., Rolla, D., Casartelli, D., Lambertini, D., Maggio, M., Cosci, P. M., Conti, G., Amar, K., Ardissino, G., Marinosci, A., Sinico, R. A., Montoli, A., Bonucchi, D., Facchini, F., Furci, L., Ferretti, A., Nuzzi, F., Pecoraro, C., Visciano, B., Canavese, C., Radin, E., Stratta, P., Nordio, M., Benetti, E., Parolin, M., Alberici, F., Manenti, L., Brugnano, R., Manenti, F., Capitanini, A., Emma, F., Massella, L., Rosa, M., Mazzon, M., Basso, E., Besso, L., Lavacca, A., Mella, A., Bertero, M., Coppo, R., Peruzzi, L., Porcellini, M. G., Piccoli, G. B., Clari, R., Pasi, A., Gangemi, C., Alfandary, H., Dagan, A., Conceiçao, M., Sameiro, F. M., Croze, L., Malvezzi, P., Tsygin, A., Zelan, B., Nastasi, null, Iatropoulos, P, Daina, E, Curreri, M, Piras, R, Valoti, E, Mele, C, Bresin, E, Gamba, S, Alberti, M, Breno, M, Perna, A, Bettoni, S, Sabadini, E, Murer, L, Vivarelli, M, Noris, M, Remuzzi, G, Bottanelli, L, Donadelli, R, Cuccarolo, P, Abbate, M, Carrara, C, Cannata, A, Ferrari, S, Gaspari, F, Stucchi, N, Bassani, C, Lena, M, Omati, G, Taruscia, D, Bellantuono, R, Giordano, M, Messina, G, Caruso, M, Gotti, E, Mescia, F, Perticucci, E, Schieppati, A, Verdoni, L, Berto, M, Baraldi, O, Montini, G, Pasini, A, Passler, W, Degasperi, T, Gaggiotti, M, Gregorini, G, Miglietti, N, Guarnieri, A, Cirami, L, Roperto, R, Di Giorgio, G, Barbano, G, Innocenti, M, Ghiggeri, G, Magnasco, A, Rolla, D, Casartelli, D, Lambertini, D, Maggio, M, Cosci, P, Conti, G, Amar, K, Ardissino, G, Marinosci, A, Sinico, R, Montoli, A, Bonucchi, D, Facchini, F, Furci, L, Ferretti, A, Nuzzi, F, Pecoraro, C, Visciano, B, Canavese, C, Radin, E, Stratta, P, Nordio, M, Benetti, E, Parolin, M, Alberici, F, Manenti, L, Brugnano, R, Manenti, F, Capitanini, A, Emma, F, Massella, L, Rosa, M, Mazzon, M, Basso, E, Besso, L, Lavacca, A, Mella, A, Bertero, M, Coppo, R, Peruzzi, L, Porcellini, M, Piccoli, G, Clari, R, Pasi, A, Gangemi, C, Alfandary, H, Dagan, A, Conceiçao, M, Sameiro, F, Croze, L, Malvezzi, P, Tsygin, A, Zelan, B, Nastasi, N, Iatropoulos, Paraskevas, Daina, Erica, Curreri, Manuela, Piras, Rossella, Valoti, Elisabetta, Mele, Caterina, Bresin, Elena, Gamba, Sara, Alberti, Marta, Breno, Matteo, Perna, Annalisa, Bettoni, Serena, Sabadini, Ettore, Murer, Luisa, Vivarelli, Marina, Noris, Marina, Remuzzi, Giuseppe, Bottanelli, L., Donadelli, R., Cuccarolo, P., Abbate, M., Carrara, C., Cannata, A., Ferrari, S., Gaspari, F., Stucchi, N., Bassani, C., Lena, M., Omati, G., Taruscia, D., Bellantuono, R., Giordano, M., Messina, G., Caruso, M., Gotti, E., Mescia, F., Perticucci, E., Schieppati, A., Verdoni, L., Berto, M., Baraldi, O., Montini, G., Pasini, A., Passler, W., Degasperi, T., Gaggiotti, M., Gregorini, G., Miglietti, N., Guarnieri, A., Cirami, L., Roperto, R. M., Di Giorgio, G., Barbano, G., Innocenti, M. L. D., Ghiggeri, G. M., Magnasco, A., Rolla, D., Casartelli, D., Lambertini, D., Maggio, M., Cosci, P. M., Conti, G., Amar, K., Ardissino, G., Marinosci, A., Sinico, R. A., Montoli, A., Bonucchi, D., Facchini, F., Furci, L., Ferretti, A., Nuzzi, F., Pecoraro, C., Visciano, B., Canavese, C., Radin, E., Stratta, P., Nordio, M., Benetti, E., Parolin, M., Alberici, F., Manenti, L., Brugnano, R., Manenti, F., Capitanini, A., Emma, F., Massella, L., Rosa, M., Mazzon, M., Basso, E., Besso, L., Lavacca, A., Mella, A., Bertero, M., Coppo, R., Peruzzi, L., Porcellini, M. G., Piccoli, G. B., Clari, R., Pasi, A., Gangemi, C., Alfandary, H., Dagan, A., Conceiçao, M., Sameiro, F. M., Croze, L., Malvezzi, P., Tsygin, A., Zelan, B., and Nastasi, null
Abstract: Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement–mediated C3 glomerulopathy (C3G) and immune complex–mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN. This approach divided patients into four clusters, indicating the existence of four different pathogenetic patterns. Specifically, this analysis separated patients with fluid-phase complement activation (clusters 1–3) who had low serum C3 levels and a high prevalence of genetic and acquired alternative pathway abnormalities from patients with solid-phase complement activation (cluster 4) who had normal or mildly altered serum C3, late disease onset, and poor renal survival. In patients with fluid-phase complement activation, those in clusters 1 and 2 had massive activation of the alternative pathway, including activation of the terminal pathway, and the highest prevalence of subendothelial deposits, but those in cluster 2 had additional activation of the classic pathway and the highest prevalence of nephrotic syndrome at disease onset. Patients in cluster 3 had prevalent activation of C3 convertase and highly electron-dense intramembranous deposits. In addition, we provide a simple algorithm to assign patients with C3G/IC-MPGN to specific clusters. These distinct clusters may facilitate clarification of disease etiology, improve risk assessment for ESRD, and pave the way for personalized treatment.
Published: 2018

14. Improving treatment decisions using personalized risk assessment from the International IgA Nephropathy Prediction Tool

Author: Barbour, Sean J., primary, Canney, Mark, additional, Coppo, Rosanna, additional, Zhang, Hong, additional, Liu, Zhi-Hong, additional, Suzuki, Yusuke, additional, Matsuzaki, Keiichi, additional, Katafuchi, Ritsuko, additional, Induruwage, Dilshani, additional, Er, Lee, additional, Reich, Heather N., additional, Feehally, John, additional, Barratt, Jonathan, additional, Cattran, Daniel C., additional, Russo, M.L., additional, Troyanov, S., additional, Cook, H.T., additional, Roberts, I., additional, Tesar, V., additional, Maixnerova, D., additional, Lundberg, S., additional, Gesualdo, L., additional, Emma, F., additional, Fuiano, L., additional, Beltrame, G., additional, Rollino, C., additional, Amore, A., additional, Camilla, R., additional, Peruzzi, L., additional, Praga, M., additional, Feriozzi, S., additional, Polci, R., additional, Segoloni, G., additional, Colla, L., additional, Pani, A., additional, Piras, D., additional, Angioi, A., additional, Cancarini, G., additional, Ravera, S., additional, Durlik, M., additional, Moggia, E., additional, Ballarin, J., additional, Di Giulio, S., additional, Pugliese, F., additional, Serriello, I., additional, Caliskan, Y., additional, Sever, M., additional, Kilicaslan, I., additional, Locatelli, F., additional, Del Vecchio, L., additional, Wetzels, J.F.M., additional, Peters, H., additional, Berg, U., additional, Carvalho, F., additional, da Costa Ferreira, A.C., additional, Maggio, M., additional, Wiecek, A., additional, Ots-Rosenberg, M., additional, Magistroni, R., additional, Topaloglu, R., additional, Bilginer, Y., additional, D’Amico, M., additional, Stangou, M., additional, Giacchino, F., additional, Goumenos, D., additional, Kalliakmani, P., additional, Gerolymos, M., additional, Galesic, K., additional, Geddes, C., additional, Siamopoulos, K., additional, Balafa, O., additional, Galliani, M., additional, Stratta, P., additional, Quaglia, M., additional, Bergia, R., additional, Cravero, R., additional, Salvadori, M., additional, Cirami, L., additional, Fellstrom, B., additional, Kloster Smerud, H., additional, Ferrario, F., additional, Stellato, T., additional, Egido, J., additional, Martin, C., additional, Floege, J., additional, Eitner, F., additional, Lupo, A., additional, Bernich, P., additional, Menè, P., additional, Morosetti, M., additional, van Kooten, C., additional, Rabelink, T., additional, Reinders, M.E.J., additional, Boria Grinyo, J.M., additional, Cusinato, S., additional, Benozzi, L., additional, Savoldi, S., additional, Licata, C., additional, Mizerska-Wasiak, M., additional, Martina, G., additional, Messuerotti, A., additional, Dal Canton, A., additional, Esposito, C., additional, Migotto, C., additional, Triolo, G., additional, Mariano, F., additional, Pozzi, C., additional, Boero, R., additional, Bellur, S., additional, Mazzucco, G., additional, Giannakakis, C., additional, Honsova, E., additional, Sundelin, B., additional, Di Palma, A.M., additional, Gutiérrez, E., additional, Asunis, A.M., additional, Barratt, J., additional, Tardanico, R., additional, Perkowska-Ptasinska, A., additional, Arce Terroba, J., additional, Fortunato, M., additional, Pantzaki, A., additional, Ozluk, Y., additional, Steenbergen, E., additional, Soderberg, M., additional, Riispere, Z., additional, Furci, L., additional, Orhan, D., additional, Kipgen, D., additional, Casartelli, D., additional, Galesic Ljubanovic, D., additional, Gakiopoulou, H., additional, Bertoni, E., additional, Cannata Ortiz, P., additional, Karkoszka, H., additional, Groene, H.J., additional, Stoppacciaro, A., additional, Bajema, I., additional, Bruijn, J., additional, Fulladosa Oliveras, X., additional, Maldyk, J., additional, Ioachim, E., additional, Bavbek, N., additional, Cook, T., additional, Alpers, C., additional, Berthoux, F., additional, Bonsib, S., additional, D’Agati, V., additional, D’Amico, G., additional, Emancipator, S., additional, Emmal, F., additional, Fervenza, F., additional, Florquin, S., additional, Fogo, A., additional, Groene, H., additional, Haas, M., additional, Hill, P., additional, Hogg, R., additional, Hsu, S., additional, Hunley, T., additional, Hladunewich, M., additional, Jennette, C., additional, Joh, K., additional, Julian, B., additional, Kawamura, T., additional, Lai, F., additional, Leung, C., additional, Li, L., additional, Li, P., additional, Liu, Z., additional, Massat, A., additional, Mackinnon, B., additional, Mezzano, S., additional, Schena, F., additional, Tomino, Y., additional, Walker, P., additional, Wang, H., additional, Weening, J., additional, Yoshikawa N, N., additional, Zeng, C.-H., additional, Shi, S., additional, Nogi, C., additional, Suzuki, H., additional, Koike, K., additional, Hirano, K., additional, Yokoo, T., additional, Hanai, M., additional, Fukami, K., additional, Takahashi, K., additional, Yuzawa, Y., additional, Niwa, M., additional, Yasuda, Y., additional, Maruyama, S., additional, Ichikawa, D., additional, Suzuki, T., additional, Shirai, S., additional, Fukuda, A., additional, Fujimoto, S., additional, and Trimarchi, H., additional
Published: 2020
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15. Reproducibility of the Oxford classification of immunoglobulin A nephropathy, impact of biopsy scoring on treatment allocation and clinical relevance of disagreements: Evidence from the VALidation of IGA study cohort

Author: Bellur, S.S. Roberts, I.S.D. Troyanov, S. Royal, V. Coppo, R. Cook, H.T. Cattran, D. Terroba, Y.A. Asunis, A.M. Bajema, I. Bertoni, E. Bruijn, J.A. Cannata-Ortiz, P. Casartelli, D. Di Palma, A.M. Ferrario, F. Fortunato, M. Furci, L. Gakiopoulou, H. Ljubanovic, D.G. Giannakakis, K. Gomá, M. Gröne, H.-J. Gutiérrez, E. Haider, S.A. Honsova, E. Ioachim, E. Karkoszka, H. Kipgen, D. Maldyk, J. Mazzucco, G. Orhan, D. Ozluk, Y. Pantzaki, A. Perkowska-Ptasinska, A. Riispere, Z. Soderberg, M.P. Steenbergen, E. Stoppacciaro, A. Von Feilitzen, B.S. Tardanico, R. Tesar, V. Maixnerova, D. Lundberg, S. Gesualdo, L. Emma, F. Fuiano, L. Beltrame, G. Rollino, C. Coppo, R. Amore, A. Camilla, R. Peruzzi, L. Praga, M. Feriozzi, S. Polci, R. Segoloni, G. Colla, L. Pani, A. Angioi, A. Piras, L. Feehally, J. Barratt, J. Cancarini, G. Ravera, S. Durlik, M. Moggia, E. Ballarin, J. Di Giulio, S. Pugliese, F. Serriello, I. Caliskan, Y. Sever, M. Locatelli, F. Del Vecchio, L. Wetzels, J.F.M. Peters, H. Berg, U. Carvalho, F. da Costa Ferreira, A.C. Maggio, M. Wiecek, A. Ots-Rosenberg, M. Magistroni, R. Topaloglu, R. Bilginer, Y. D'Amico, M. Stangou, M. Giacchino, F. Goumenos, D. Kalliakmani, P. Gerolymos, M. Galesic, K. Geddes, C. Siamopoulos, K. Balafa, O. Galliani, M. Stratta, P. Quaglia, M. Bergia, R. Cravero, R. Salvadori, M. Cirami, L. Fellstrom, B. Kloster Smerud, H. Ferrario, F. Stellato, T. Egido, J. Martin, C. Floege, J. Eitner, F. Lupo, A. Bernich, P. Menè, P. Morosetti, M. van Kooten, C. Rabelink, T. Reinders, M.E.J. Grinyo, J.M. Fulladosa, X. Cusinato, S. Benozzi, L. Savoldi, S. Licata, C. Mizerska-Wasiak, M. Roszkowska-Blaim, M. Martina, G. Messuerotti, A. Dal Canton, A. Esposito, C. Migotto, C. Triolo, G. Mariano, F. Pozzi, C. Boero, R. Kilicaslan, I.
Abstract: Background: The VALidation of IGA (VALIGA) study investigated the utility of the Oxford Classification of immunoglobulin A nephropathy (IgAN) in 1147 patients from 13 European countries. Methods. Biopsies were scored by local pathologists followed by central review in Oxford. We had two distinct objectives: to assess how closely pathology findings were associated with the decision to give corticosteroid/immunosuppressive (CS/IS) treatments, and to determine the impact of differences in MEST-C scoring between central and local pathologists on the clinical value of the Oxford Classification. We tested for each lesion the associations between the type of agreement (local and central pathologists scoring absent, local present and central absent, local absent and central present, both scoring present) with the initial clinical assessment, as well as long-term outcomes in those patients who did not receive CS/IS. Results: All glomerular lesions (M, E, C and S) assessed by local pathologists were independently associated with the decision to administer CS/IS therapy, while the severity of tubulointerstitial lesions was not. Reproducibility between local and central pathologists was moderate for S (segmental sclerosis) and T (tubular atrophy/interstitial fibrosis), and poor for M (mesangial hypercellularity), E (endocapillary hypercellularity) and C (crescents). Local pathologists found statistically more of each lesion, except for the S lesion, which was more frequent with central review. Disagreements were more likely to occur when the proportion of glomeruli affected was low. The M lesion, assessed by central pathologists, correlated better with the severity of the disease at presentation and discriminated better with outcomes. In contrast, the E lesion, evaluated by local pathologists, correlated better with the clinical presentation and outcomes when compared with central review. Both C and S lesions, when discordant between local and central pathologists, had a clinical phenotype intermediate to double absent lesions (milder disease) and double present (more severe). Conclusion: We conclude that differences in the scoring of MEST-C criteria between local pathologists and a central reviewer have a significant impact on the prognostic value of the Oxford Classification. Since the decision to offer immunosuppressive therapy in this cohort was intimately associated with the MEST-C score, this study indicates a need for a more detailed guidance for pathologists in the scoring of IgAN biopsies. © 2018 The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.
Published: 2019

16. Evaluating a New International Risk-Prediction Tool in IgA Nephropathy

Author: Barbour, S.J., Coppo, R., Zhang, H., Liu, Z.H., Suzuki, Y., Matsuzaki, K., Katafuchi, R., Er, L., Espino-Hernandez, G., Kim, S.J., Reich, H.N., Feehally, J., Cattran, D.C., Russo, M.L., Troyanov, S., Cook, H.T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Giulio, S. di, Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Vecchio, L. del, Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., Ferreira, A.C.D., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrorn, B., Smerud, H.K., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Mene, R., Morosetti, M., Kooten, C. van, Rabelink, T., Reinders, M.E.J., Grinyo, J.M.B., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Canton, A. dal, Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Palma, A.M. di, Gutierrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Terroba, J.A., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Ljubanovic, D.G., Gakiopoulou, H., Bertoni, E., Ortiz, P.C., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Oliveras, X.F., Maldyk, J., Loachim, E., Bavbek, N., Cook, T., Alpers, C., Berthoux, F., Bonsib, S., D'Agati, V., D'Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zeng, C.H., Shi, S.F., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Int IgA Nephropathy Network, and Pathology
Subjects: Adult, Male, medicine.medical_specialty, Risk Assessment, 01 natural sciences, Nephropathy, 03 medical and health sciences, 0302 clinical medicine, Interquartile range, Internal medicine, Internal Medicine, Humans, Medicine, 030212 general & internal medicine, 0101 mathematics, business.industry, Proportional hazards model, Clinical study design, 010102 general mathematics, Glomerulonephritis, IGA, Middle Aged, Models, Theoretical, medicine.disease, Clinical trial, Cohort, Female, business, Risk assessment, Kidney disease
Abstract: ImportanceAlthough IgA nephropathy (IgAN) is the most common glomerulonephritis in the world, there is no validated tool to predict disease progression. This limits patient-specific risk stratification and treatment decisions, clinical trial recruitment, and biomarker validation. ObjectiveTo derive and externally validate a prediction model for disease progression in IgAN that can be applied at the time of kidney biopsy in multiple ethnic groups worldwide. Design, Setting, and ParticipantsWe derived and externally validated a prediction model using clinical and histologic risk factors that are readily available in clinical practice. Large, multi-ethnic cohorts of adults with biopsy-proven IgAN were included from Europe, North America, China, and Japan. Main Outcomes and MeasuresCox proportional hazards models were used to analyze the risk of a 50% decline in estimated glomerular filtration rate (eGFR) or end-stage kidney disease, and were evaluated using the R-D(2) measure, Akaike information criterion (AIC), C statistic, continuous net reclassification improvement (NRI), integrated discrimination improvement (IDI), and calibration plots. ResultsThe study included 3927 patients; mean age, 35.4 (interquartile range, 28.0-45.4) years; and 2173 (55.3%) were men. The following prediction models were created in a derivation cohort of 2781 patients: a clinical model that included eGFR, blood pressure, and proteinuria at biopsy; and 2 full models that also contained the MEST histologic score, age, medication use, and either racial/ethnic characteristics (white, Japanese, or Chinese) or no racial/ethnic characteristics, to allow application in other ethnic groups. Compared with the clinical model, the full models with and without race/ethnicity had better R-D(2) (26.3% and 25.3%, respectively, vs 20.3%) and AIC (6338 and 6379, respectively, vs 6485), significant increases in C statistic from 0.78 to 0.82 and 0.81, respectively (Delta C, 0.04; 95% CI, 0.03-0.04 and Delta C, 0.03; 95% CI, 0.02-0.03, respectively), and significant improvement in reclassification as assessed by the NRI (0.18; 95% CI, 0.07-0.29 and 0.51; 95% CI, 0.39-0.62, respectively) and IDI (0.07; 95% CI, 0.06-0.08 and 0.06; 95% CI, 0.05-0.06, respectively). External validation was performed in a cohort of 1146 patients. For both full models, the C statistics (0.82; 95% CI, 0.81-0.83 with race/ethnicity; 0.81; 95% CI, 0.80-0.82 without race/ethnicity) and R-D(2) (both 35.3%) were similar or better than in the validation cohort, with excellent calibration. Conclusions and RelevanceIn this study, the 2 full prediction models were shown to be accurate and validated methods for predicting disease progression and patient risk stratification in IgAN in multi-ethnic cohorts, with additional applications to clinical trial design and biomarker research.
Published: 2019

17. Evaluating a New International Risk-Prediction Tool in IgA Nephropathy

Author: Barbour, Sean J. Coppo, Rosanna Zhang, Hong Liu, Zhi-Hong and Suzuki, Yusuke Matsuzaki, Keiichi Katafuchi, Ritsuko Er, Lee Espino-Hernandez, Gabriela Kim, S. Joseph Reich, Heather N. Feehally, John Cattran, Daniel C. Russo, M. L. and Troyanov, S. Cook, H. T. Roberts, I. Tesar, V. and Maixnerova, D. Lundberg, S. Gesualdo, L. Emma, F. and Fuiano, L. Beltrame, G. Rollino, C. Amore, A. Camilla, R. Peruzzi, L. Praga, M. Feriozzi, S. Polci, R. and Segoloni, G. Colla, L. Pani, A. Piras, D. Angioi, A. and Cancarini, G. Ravera, S. Durlik, M. Moggia, E. Ballarin, J. Di Giulio, S. Pugliese, F. Serriello, I. Caliskan, Y. and Sever, M. Kilicaslan, I. Locatelli, F. Del Vecchio, L. and Wetzels, J. F. M. Peters, H. Berg, U. Carvalho, F. and da Costa Ferreira, A. C. Maggio, M. Wiecek, A. and Ots-Rosenberg, M. Magistroni, R. Topaloglu, R. Bilginer, Y. and D'Amico, M. Stangou, M. Giacchino, F. Goumenos, D. and Kalliakmani, P. Gerolymos, M. Galesic, K. Geddes, C. and Siamopoulos, K. Balafa, O. Galliani, M. Stratta, P. and Quaglia, M. Bergia, R. Cravero, R. Salvadori, M. Cirami, L. Fellstrorn, B. Smerud, H. Kloster Ferrario, F. and Stellato, T. Egido, J. Martin, C. Floege, J. Eitner, F. and Lupo, A. Bernich, P. Mene, R. Morosetti, M. van Kooten, C. Rabelink, T. Reinders, M. E. J. Boria Grinyo, J. M. Cusinato, S. Benozzi, L. Savoldi, S. Licata, C. and Mizerska-Wasiak, M. Martina, G. Messuerotti, A. Dal Canton, A. Esposito, C. Migotto, C. Triolo, G. Mariano, F. and Pozzi, C. Boero, R. Bellur, S. Mazzucco, G. Giannakakis, C. Honsova, E. Sundelin, B. Di Palma, A. M. Ferrario, F. and Gutierrez, E. Asunis, A. M. Barratt, J. Tardanico, R. and Perkowska-Ptasinska, A. Arce Terroba, J. Fortunato, M. and Pantzaki, A. Ozluk, Y. Steenbergen, E. Soderberg, M. and Riispere, Z. Furci, L. Orhan, D. Kipgen, D. Casartelli, D. Ljubanovic, D. Galesic Gakiopoulou, H. Bertoni, E. and Cannata Ortiz, P. Karkoszka, H. Groene, H. J. Stoppacciaro, A. Bajema, I. Bruijn, J. Fulladosa Oliveras, X. Maldyk, J. Loachim, E. Bavbek, N. Cook, T. Troyanov, S. and Alpers, C. Amore, A. Barratt, J. Berthoux, F. Bonsib, S. and Bruijn, J. D'Agati, V D'Amico, G. Emancipator, S. and Emmal, F. Ferrario, F. Fervenza, F. Florquin, S. Fogo, A. Geddes, C. Groene, H. Haas, M. Hill, P. Hogg, R. and Hsu, S. Hunley, T. Hladunewich Jennette, C. Joh, K. and Julian, B. Kawamura, T. Lai, F. Leung, C. Li, L. and Li, P. Liu, Z. Massat, A. Mackinnon, B. Mezzano, S. and Schena, F. Tomino, Y. Walker, P. Wang, H. Weening, J. and Yoshikawa, N. Zeng, Cai-Hong Shi, Sufang Nogi, C. and Suzuki, H. Koike, K. Hirano, K. Kawamura, T. Yokoo, T. and Hanai, M. Fukami, K. Takahashi, K. Yuzawa, Y. Niwa, M. Yasuda, Y. Maruyama, S. Ichikawa, D. Suzuki, T. and Shirai, S. Fukuda, A. Fujimoto, S. Trimarchi, H. Int IgA Nephropathy Network
Abstract: ImportanceAlthough IgA nephropathy (IgAN) is the most common glomerulonephritis in the world, there is no validated tool to predict disease progression. This limits patient-specific risk stratification and treatment decisions, clinical trial recruitment, and biomarker validation. ObjectiveTo derive and externally validate a prediction model for disease progression in IgAN that can be applied at the time of kidney biopsy in multiple ethnic groups worldwide. Design, Setting, and ParticipantsWe derived and externally validated a prediction model using clinical and histologic risk factors that are readily available in clinical practice. Large, multi-ethnic cohorts of adults with biopsy-proven IgAN were included from Europe, North America, China, and Japan. Main Outcomes and MeasuresCox proportional hazards models were used to analyze the risk of a 50% decline in estimated glomerular filtration rate (eGFR) or end-stage kidney disease, and were evaluated using the R-D(2) measure, Akaike information criterion (AIC), C statistic, continuous net reclassification improvement (NRI), integrated discrimination improvement (IDI), and calibration plots. ResultsThe study included 3927 patients; mean age, 35.4 (interquartile range, 28.0-45.4) years; and 2173 (55.3%) were men. The following prediction models were created in a derivation cohort of 2781 patients: a clinical model that included eGFR, blood pressure, and proteinuria at biopsy; and 2 full models that also contained the MEST histologic score, age, medication use, and either racial/ethnic characteristics (white, Japanese, or Chinese) or no racial/ethnic characteristics, to allow application in other ethnic groups. Compared with the clinical model, the full models with and without race/ethnicity had better R-D(2) (26.3% and 25.3%, respectively, vs 20.3%) and AIC (6338 and 6379, respectively, vs 6485), significant increases in C statistic from 0.78 to 0.82 and 0.81, respectively (Delta C, 0.04; 95% CI, 0.03-0.04 and Delta C, 0.03; 95% CI, 0.02-0.03, respectively), and significant improvement in reclassification as assessed by the NRI (0.18; 95% CI, 0.07-0.29 and 0.51; 95% CI, 0.39-0.62, respectively) and IDI (0.07; 95% CI, 0.06-0.08 and 0.06; 95% CI, 0.05-0.06, respectively). External validation was performed in a cohort of 1146 patients. For both full models, the C statistics (0.82; 95% CI, 0.81-0.83 with race/ethnicity; 0.81; 95% CI, 0.80-0.82 without race/ethnicity) and R-D(2) (both 35.3%) were similar or better than in the validation cohort, with excellent calibration. Conclusions and RelevanceIn this study, the 2 full prediction models were shown to be accurate and validated methods for predicting disease progression and patient risk stratification in IgAN in multi-ethnic cohorts, with additional applications to clinical trial design and biomarker research.
Published: 2019

18. Evaluating a New International Risk-Prediction Tool in IgA Nephropathy

Author: Barbour, Sean J., Coppo, Rosanna, Zhang, Hong, Liu, Zhi-Hong, Suzuki, Yusuke, Matsuzaki, Keiichi, Katafuchi, Ritsuko, Er, Lee, Espino-Hernandez, Gabriela, Kim, S. Joseph, Reich, Heather N., Feehally, John, Cattran, Daniel C., Russo, M. L., Troyanov, S., Cook, H. T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J. F. M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A. C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellström, Bengt, Smerud, Hilde Kloster, Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Mene, R., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M. E. J., Boria Grinyo, J. M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A. M., Gutierrez, E., Asunis, A. M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Ljubanovic, D. Galesic, Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Loachim, E., Bavbek, N., Cook, T., Alpers, C., Berthoux, F., Bonsib, S., D'Agati, V, D'Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zeng, Cai-Hong, Shi, Sufang, Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Barbour, Sean J., Coppo, Rosanna, Zhang, Hong, Liu, Zhi-Hong, Suzuki, Yusuke, Matsuzaki, Keiichi, Katafuchi, Ritsuko, Er, Lee, Espino-Hernandez, Gabriela, Kim, S. Joseph, Reich, Heather N., Feehally, John, Cattran, Daniel C., Russo, M. L., Troyanov, S., Cook, H. T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J. F. M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A. C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellström, Bengt, Smerud, Hilde Kloster, Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Mene, R., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M. E. J., Boria Grinyo, J. M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A. M., Gutierrez, E., Asunis, A. M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Ljubanovic, D. Galesic, Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Loachim, E., Bavbek, N., Cook, T., Alpers, C., Berthoux, F., Bonsib, S., D'Agati, V, D'Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zeng, Cai-Hong, Shi, Sufang, Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., and Trimarchi, H.
Abstract: Importance Although IgA nephropathy (IgAN) is the most common glomerulonephritis in the world, there is no validated tool to predict disease progression. This limits patient-specific risk stratification and treatment decisions, clinical trial recruitment, and biomarker validation. Objective To derive and externally validate a prediction model for disease progression in IgAN that can be applied at the time of kidney biopsy in multiple ethnic groups worldwide. Design, Setting, and Participants We derived and externally validated a prediction model using clinical and histologic risk factors that are readily available in clinical practice. Large, multi-ethnic cohorts of adults with biopsy-proven IgAN were included from Europe, North America, China, and Japan. Main Outcomes and Measures Cox proportional hazards models were used to analyze the risk of a 50% decline in estimated glomerular filtration rate (eGFR) or end-stage kidney disease, and were evaluated using the R2D measure, Akaike information criterion (AIC), C statistic, continuous net reclassification improvement (NRI), integrated discrimination improvement (IDI), and calibration plots. Results The study included 3927 patients; mean age, 35.4 (interquartile range, 28.0-45.4) years; and 2173 (55.3%) were men. The following prediction models were created in a derivation cohort of 2781 patients: a clinical model that included eGFR, blood pressure, and proteinuria at biopsy; and 2 full models that also contained the MEST histologic score, age, medication use, and either racial/ethnic characteristics (white, Japanese, or Chinese) or no racial/ethnic characteristics, to allow application in other ethnic groups. Compared with the clinical model, the full models with and without race/ethnicity had better R2D (26.3% and 25.3%, respectively, vs 20.3%) and AIC (6338 and 6379, respectively, vs 6485), significant increases in C statistic from 0.78 to 0.82 and 0.81, respectively (ΔC, 0.04; 95% CI, 0.03-0.04 and ΔC, 0.03
Published: 2019
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19. Cluster Analysis Identifies Distinct Pathogenetic Patterns in C3 Glomerulopathies/Immune Complex–Mediated Membranoproliferative GN

Author: Iatropoulos, Paraskevas, Daina, Erica, Curreri, Manuela, Piras, Rossella, Valoti, Elisabetta, Mele, Caterina, Bresin, Elena, Gamba, Sara, Alberti, Marta, Breno, Matteo, Perna, Annalisa, Bettoni, Serena, Sabadini, Ettore, Murer, Luisa, Vivarelli, Marina, Noris, Marina, Remuzzi, Giuseppe, Bottanelli, L., Donadelli, R., Cuccarolo, P., Abbate, M., Carrara, C., Cannata, A., Ferrari, S., Gaspari, F., Stucchi, N., Bassani, C., Lena, M., Omati, G., Taruscia, D., Bellantuono, R., Giordano, M., Messina, G., Caruso, M., Gotti, E., Mescia, F., Perticucci, E., Schieppati, A., Verdoni, L., Berto, M., Baraldi, O., Montini, G., Pasini, A., Passler, W., Degasperi, T., Gaggiotti, M., Gregorini, G., Miglietti, N., Guarnieri, A., Cirami, L., Roperto, R. M., Di Giorgio, G., Barbano, G., Innocenti, M. L. D., Ghiggeri, G. M., Magnasco, A., Rolla, D., Casartelli, D., Lambertini, D., Maggio, M., Cosci, P. M., Conti, G., Amar, K., Ardissino, G., Marinosci, A., Sinico, R. A., Montoli, A., Bonucchi, D., Facchini, F., Furci, L., Ferretti, A., Nuzzi, F., Pecoraro, C., Visciano, B., Canavese, C., Radin, E., Stratta, P., Nordio, M., Benetti, E., Parolin, M., Alberici, F., Manenti, L., Brugnano, R., Manenti, F., Capitanini, A., Emma, F., Massella, L., Rosa, M., Mazzon, M., Basso, E., Besso, L., Lavacca, A., Mella, A., Bertero, M., Coppo, R., Peruzzi, L., Porcellini, M. G., Piccoli, G. B., Clari, R., Pasi, A., Gangemi, C., Alfandary, H., Dagan, A., Conceiçao, M., Sameiro, F. M., Croze, L., Malvezzi, P., Tsygin, A., Zelan, B., Nastasi, null, Iatropoulos, P, Daina, E, Curreri, M, Piras, R, Valoti, E, Mele, C, Bresin, E, Gamba, S, Alberti, M, Breno, M, Perna, A, Bettoni, S, Sabadini, E, Murer, L, Vivarelli, M, Noris, M, Remuzzi, G, Bottanelli, L, Donadelli, R, Cuccarolo, P, Abbate, M, Carrara, C, Cannata, A, Ferrari, S, Gaspari, F, Stucchi, N, Bassani, C, Lena, M, Omati, G, Taruscia, D, Bellantuono, R, Giordano, M, Messina, G, Caruso, M, Gotti, E, Mescia, F, Perticucci, E, Schieppati, A, Verdoni, L, Berto, M, Baraldi, O, Montini, G, Pasini, A, Passler, W, Degasperi, T, Gaggiotti, M, Gregorini, G, Miglietti, N, Guarnieri, A, Cirami, L, Roperto, R, Di Giorgio, G, Barbano, G, Innocenti, M, Ghiggeri, G, Magnasco, A, Rolla, D, Casartelli, D, Lambertini, D, Maggio, M, Cosci, P, Conti, G, Amar, K, Ardissino, G, Marinosci, A, Sinico, R, Montoli, A, Bonucchi, D, Facchini, F, Furci, L, Ferretti, A, Nuzzi, F, Pecoraro, C, Visciano, B, Canavese, C, Radin, E, Stratta, P, Nordio, M, Benetti, E, Parolin, M, Alberici, F, Manenti, L, Brugnano, R, Manenti, F, Capitanini, A, Emma, F, Massella, L, Rosa, M, Mazzon, M, Basso, E, Besso, L, Lavacca, A, Mella, A, Bertero, M, Coppo, R, Peruzzi, L, Porcellini, M, Piccoli, G, Clari, R, Pasi, A, Gangemi, C, Alfandary, H, Dagan, A, Conceiçao, M, Sameiro, F, Croze, L, Malvezzi, P, Tsygin, A, Zelan, B, and Nastasi, N
Subjects: 0301 basic medicine, Complement system, Glomerulonephritis, Membranoproliferative, membranoproliferative glomerulonephritis (MPGN), 030232 urology & nephrology, Disease, Antigen-Antibody Complex, Biology, Kidney, 03 medical and health sciences, 0302 clinical medicine, Glomerulopathy, Clinical Research, medicine, Dense Deposit Disease, Humans, C3 glomerulopathy, General Medicine, Complement System Proteins, C3 glomerulonephriti, medicine.disease, C3-convertase, Immune complex, 030104 developmental biology, Nephrology, Immunology, Alternative complement pathway, Nephrotic syndrome, Rare disease
Abstract: Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement–mediated C3 glomerulopathy (C3G) and immune complex–mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN. This approach divided patients into four clusters, indicating the existence of four different pathogenetic patterns. Specifically, this analysis separated patients with fluid-phase complement activation (clusters 1–3) who had low serum C3 levels and a high prevalence of genetic and acquired alternative pathway abnormalities from patients with solid-phase complement activation (cluster 4) who had normal or mildly altered serum C3, late disease onset, and poor renal survival. In patients with fluid-phase complement activation, those in clusters 1 and 2 had massive activation of the alternative pathway, including activation of the terminal pathway, and the highest prevalence of subendothelial deposits, but those in cluster 2 had additional activation of the classic pathway and the highest prevalence of nephrotic syndrome at disease onset. Patients in cluster 3 had prevalent activation of C3 convertase and highly electron-dense intramembranous deposits. In addition, we provide a simple algorithm to assign patients with C3G/IC-MPGN to specific clusters. These distinct clusters may facilitate clarification of disease etiology, improve risk assessment for ESRD, and pave the way for personalized treatment.
Published: 2017

20. A1101 - Perioperative and functional outcomes after kidney transplantation from uncontrolled donors after circulatory death vs. expanded-criteria donors after brain death vs. standard-criteria donors: Toward an evidence-based refinement of allocation strategies

Author: Campi, R., Pecoraro, A., Sessa, F., Barzaghi, P., Rivetti, A., Nicoletti, R., Gallo, M.L., Corti, F., Morselli, S., Spatafora, P., Gacci, M., Giancane, S., Sebastianelli, A., Cirami, L., Peris, A., Vignolini, G., Serni, S., and Li Marzi, V.
Published: 2022
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21. Robotic kidney transplantation from living and deceased donors: Updated results at a median follow-up of 1 year

Author: Campi, R., primary, Vignolini, G., additional, Sessa, F., additional, Greco, I., additional, Olivieri, C., additional, Larti, A., additional, Cirami, L., additional, Morselli, S., additional, Corti, F., additional, Grosso, A., additional, Barzaghi, P., additional, Giancane, S., additional, Sebastianelli, A., additional, Gacci, M., additional, Peris, A., additional, Breda, A., additional, Siena, G., additional, Li Marzi, V., additional, and Serni, S., additional
Published: 2019
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22. Starting a new kidney transplantation program from uncontrolled donors after circulatory death (DCD) in italy: A single-centre preliminary experience

Author: Li Marzi, V., primary, Campi, R., additional, Sessa, F., additional, Giancane, S., additional, Caroassai, S., additional, Cirami, L., additional, Olivieri, C., additional, Barzaghi, P., additional, Corti, F., additional, Sebastianelli, A., additional, Tuccio, A., additional, Siena, G., additional, Vignolini, G., additional, Greco, I., additional, Di Maria, L., additional, Lazzeri, C., additional, Peris, A., additional, and Serni, S., additional
Published: 2019
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23. Small renal lesions found during organ retrieval for transplantation purposes: Our experience in donors after brain death over a 8 years period

Author: Facchiano, D., primary, Grisanti, S. Caroassai, additional, Migliaccio, M., additional, Peris, A., additional, Cirami, L., additional, Morselli, S., additional, Bombardi, M., additional, Campi, R., additional, Novelli, L., additional, Serni, S., additional, and Li Marzi, V., additional
Published: 2019
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24. Use of perfusion parameters in predicting delayed graft function of machine-preserved kidneys retrieved from uncontrolled DCDs

Author: Li Marzi, V., primary, Lazzeri, C., additional, Caroassai, S., additional, Fulceri, G., additional, Tuccio, A., additional, Siena, G., additional, Vignolini, G., additional, Campi, R., additional, Caroti, L., additional, Migliaccio, M., additional, Cirami, L., additional, Peris, A., additional, and Serni, S., additional
Published: 2019
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25. Development and testing of an artificial intelligence tool for predicting end-stage kidney disease in patients with immunoglobulin A nephropathy

Author: Schena, Francesco Paolo, Anelli, Vito Walter, Trotta, Joseph, Di Noia, Tommaso, Manno, Carlo, Tripepi, Giovanni, D’Arrigo, Graziella, Chesnaye, Nicholas C., Russo, Maria Luisa, Stangou, Maria, Papagianni, Aikaterini, Zoccali, Carmine, Tesar, Vladimir, Coppo, Rosanna, Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Coppo, R., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Angioi, A., Piras, L., Feehally, J., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Papasotiriou, M., Galesic, K., Toric, L., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Smerud, H. Kloster, Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Rauen, T., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Grinyo, J.M. Boria, Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Martina, G., Messuerotti, A., Canton, A. Dal, Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Mazzucco, Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Ferrario, F., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Terroba, J. Arce, Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., GalesicLjubanovic, D., Gakiopoulou, H., Bertoni, E., Ortiz, P. Cannata, Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., FulladosaOliveras, X., Maldyk, J., Ioachim, E., Abbrescia, Daniela, Kouri, Nikoleta, Stangou, Maria, Papagianni, Aikaterini, Scolari, Francesco, Delbarba, Elisa, Bonomini, Mario, Piscitani, Luca, Stallone, Giovanni, Infante, Barbara, Godeas, Giulia, Madio, Desiree, Biancone, Luigi, Campagna, Marco, Zaza, Gianluigi, Squarzoni, Isabella, and Cangemi, Concetta
Abstract: We have developed an artificial neural network prediction model for end-stage kidney disease (ESKD) in patients with primary immunoglobulin A nephropathy (IgAN) using a retrospective cohort of 948 patients with IgAN. Our tool is based on a two-step procedure of a classifier model that predicts ESKD, and a regression model that predicts development of ESKD over time. The classifier model showed a performance value of 0.82 (area under the receiver operating characteristic curve) in patients with a follow-up of five years, which improved to 0.89 at the ten-year follow-up. Both models had a higher recall rate, which indicated the practicality of the tool. The regression model showed a mean absolute error of 1.78 years and a root mean square error of 2.15 years. Testing in an independent cohort of 167patients with IgAN found successful results for 91% of the patients. Comparison of our system with other mathematical models showed the highest discriminant Harrell C index at five- and ten-years follow-up (81% and 86%, respectively), paralleling the lowest Akaike information criterion values (355.01 and 269.56, respectively). Moreover, our system was the best calibrated model indicating that the predicted and observed outcome probabilities did not significantly differ. Finally, the dynamic discrimination indexes of our artificial neural network, expressed as the weighted average of time-dependent areas under the curve calculated at one and two years, were 0.80 and 0.79, respectively. Similar results were observed over a 25-year follow-up period. Thus, our tool identified individuals who were at a high risk of developing ESKD due to IgAN and predicted the time-to-event endpoint. Accurate prediction is an important step toward introduction of a therapeutic strategy for improving clinical outcomes.
Published: 2021
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26. Study of the starting point of the Change Laboratory on a team of university teachers for the promotion of an epistemic attitude towards disciplinary knowledge

Author: Colombo, M.E., Mayol, J., Gestal, L., Cirami, L., and López, Yasmín
Subjects: Expansive learning, Docentes universitarios, Laboratorio de cambios, Change laboratory, Promotion of an epistemic attitude towards disciplinary knowledge, Aprendizaje expansivo, University teachers, Promoción de actitud epistémica del conocimiento disciplinar
Abstract: Se presenta el análisis del proceso de cambio de las dos primeras sesiones que corresponden a la etnografía del punto de partida de un diseño de intervención de un equipo de docentes universitarios de la asignatura Psicología del Ciclo Básico Común de la Universidad de Buenos Aires. El trabajo corresponde al cumplimiento del tercer objetivo del proyecto de investigación, “Lectura y escritura argumentativa académica colaborativa para la promoción de una actitud epistémica del conocimiento disciplinar en dispositivo pedagógico-didáctico que articula enseñanza presencial, no presencial y recursos colaborativos virtuales”, que es: concientizar a los docentes respecto de su función como agentes mediadores para la promoción de habilidades de lectura y escritura argumentativa académica. Se utilizó la metodología de Laboratorio de Cambios –diseño de intervención- que opera como un sistema de actividad que se enfrenta a una importante transformación. Las intervenciones formativas fueron introducidas para el avance del ciclo de aprendizaje expansivo aprovechando la energía de las contradicciones históricas utilizando como material espejo los resultados de trabajo anterior sobre la concepción que tienen los docentes respecto de la función que cumple la lectura y escritura en la Universidad. Los integrantes del Laboratorio son 3 profesores, 7 jefes de trabajos prácticos, 19 auxiliares docentes, 1 auxiliar docente alumno y dos investigadores. Se grabaron las sesiones para su análisis. La etnografía del punto de partida mostró que los docentes identificaban los problemas y perturbaciones que afectaban su práctica como también aparecía la potencialidad expresada en las propuestas de cambio que realizaban, en el comienzo ofrecieron algunas resistencias y luego convocaron a la elaboración de nuevos conceptos, objetos de conocimiento, por medio de los cuales ensayarían, como una prueba piloto, las propuestas emergentes. This article presents the analysis of the process of change of the first two sessions that correspond to the ethnography of the starting point referred to an intervention design on a team of university teachers from the subject Psychology of CBC at University of Buenos Aires. The work corresponds to the fulfilment of the third objective of the research project, “Argumentative collaborative academic reading and writing for the promotion of an epistemic attitude of disciplinary knowledge in pedagogical didactic device that articulates classroom teaching, non presential and virtual collaborative resources”, which is: raise awareness of teachers with respect to its role as mediator agents for the promotion of argumentative academic reading and writing skills. The methodology of Laboratory of Changes was used - intervention design - which operates as an activity system that is facing a major transformation. Formative interventions were introduced by the investigators for the advancement of the cycle of expansive learning taking advantage of the energy of the contradictions using mirror surface. The members of the laboratory are 3 teachers, 7 heads of practical work, 19 professors, 1 auxiliary teaching assistant student and two investigators. The sessions were recorded for analysis. The Ethnography of the starting point revealed that teachers identified the problems and disturbances that affected their practice as also appeared the potential expressed in their change proposals, at the beginning they offered some resistance and then called for the development of new concepts, objects of knowledge, by means of which they would rehearse, as a pilot test, the emerging proposals.
Published: 2016

27. Evidence from the large VALIGA cohort validates the subclassification of focal segmental glomerulosclerosis in IgA nephropathy

Author: Bellur, Shubha S., Troyanov, Stéphan, Vorobyeva, Olga, Coppo, Rosanna, Roberts, Ian S.D., Coppo, R., Feehaly, J., Troyanov, S., Cattran, D.C., Cook, H.T., Roberts, I., Radcliffe, John, Russo, M.L., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Papagianni, K., Stangou, M., Giacchino, F., Goumenos, D., Papasotirious, M., Kalliakmani, P., Gerolymos, M., Galesic, K., Toric, L., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Durlik, M., Hryszko, T., Klinger, M., Kamińska, D., Krajewska, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Cambier, A., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Ferrario, F., Diomedi-Casadei, F., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., and Royal, V.
Abstract: Evidence from the Oxford IgA nephropathy (IgAN) cohort supports the clinical value of subclassifying focal segmental glomerulosclerosis lesions (S1). Using the larger Validation in IgA (VALIGA) study cohort, we investigated the association between podocytopathic changes and higher proteinuria, kidney outcome and response to immunosuppressive therapy. All biopsies were evaluated for glomeruli with segmental capillary occlusion by matrix (“not otherwise specified”, NOS lesion), simple capsular adhesion without capillary occlusion (Adh), tip lesions, and podocyte hypertrophy (PH). S1 required a NOS lesion and/or Adh. A Chi-Squared Automatic Interaction Detection method was used to identify subgroups of FSGS lesions associated with distinctive proteinuria at biopsy and assessed survival from a combined event (kidney failure or 50% decline in estimated glomerular filtration rate). Finally, we evaluated within each subgroup if immunosuppression was associated with a favorable outcome using propensity analysis. In 1147 patients, S1 was found in 70% of biopsies. Subclassification found NOS lesions in 44%, Adh in 59%, PH in 13%, and tip lesions in 3%, with much overlap. Four subgroups were identified with progressively higher proteinuria: from lowest, S1 without NOS, S1 with NOS but without Adh/PH, to highest, S1 with NOS and Adh but without PH, and S1 with NOS and PH. These four subgroups showed progressively worse kidney survival. Immunosuppression was associated with a better outcome only in the two highest proteinuria subgroups. Propensity analysis in these two groups, adjusted for clinical and pathological findings, found a significant time-dependent hazard of combined outcome with corticosteroids. Podocyte hypertrophy and glomeruli with simple adhesions appeared to reflect active lesions associated with a response to corticosteroids, while other S1 lesions defined chronicity. Thus, our findings support subclassifying S1 lesions in IgAN.
Published: 2024
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28. Estudio del punto de partida de Laboratorio de Cambios de un equipo de docentes universitarios para la promoción de una actitud epistémica del conocimiento disciplinar

Author: Colombo, M.E., Mayol, J., Gestal, L., Cirami, L., López, Yasmín, Colombo, M.E., Mayol, J., Gestal, L., Cirami, L., and López, Yasmín
Abstract: Se presenta el análisis del proceso de cambio de las dos primeras sesiones que corresponden a la etnografía del punto de partida de un diseño de intervención de un equipo de docentes universitarios de la asignatura Psicología del Ciclo Básico Común de la Universidad de Buenos Aires. El trabajo corresponde al cumplimiento del tercer objetivo del proyecto de investigación, “Lectura y escritura argumentativa académica colaborativa para la promoción de una actitud epistémica del conocimiento disciplinar en dispositivo pedagógico-didáctico que articula enseñanza presencial, no presencial y recursos colaborativos virtuales”, que es: concientizar a los docentes respecto de su función como agentes mediadores para la promoción de habilidades de lectura y escritura argumentativa académica. Se utilizó la metodología de Laboratorio de Cambios –diseño de intervención- que opera como un sistema de actividad que se enfrenta a una importante transformación. Las intervenciones formativas fueron introducidas para el avance del ciclo de aprendizaje expansivo aprovechando la energía de las contradicciones históricas utilizando como material espejo los resultados de trabajo anterior sobre la concepción que tienen los docentes respecto de la función que cumple la lectura y escritura en la Universidad. Los integrantes del Laboratorio son 3 profesores, 7 jefes de trabajos prácticos, 19 auxiliares docentes, 1 auxiliar docente alumno y dos investigadores. Se grabaron las sesiones para su análisis. La etnografía del punto de partida mostró que los docentes identificaban los problemas y perturbaciones que afectaban su práctica como también aparecía la potencialidad expresada en las propuestas de cambio que realizaban, en el comienzo ofrecieron algunas resistencias y luego convocaron a la elaboración de nuevos conceptos, objetos de conocimiento, por medio de los cuales ensayarían, como una prueba piloto, las propuestas emergentes., This article presents the analysis of the process of change of the first two sessions that correspond to the ethnography of the starting point referred to an intervention design on a team of university teachers from the subject Psychology of CBC at University of Buenos Aires. The work corresponds to the fulfilment of the third objective of the research project, “Argumentative collaborative academic reading and writing for the promotion of an epistemic attitude of disciplinary knowledge in pedagogical didactic device that articulates classroom teaching, non presential and virtual collaborative resources”, which is: raise awareness of teachers with respect to its role as mediator agents for the promotion of argumentative academic reading and writing skills. The methodology of Laboratory of Changes was used - intervention design - which operates as an activity system that is facing a major transformation. Formative interventions were introduced by the investigators for the advancement of the cycle of expansive learning taking advantage of the energy of the contradictions using mirror surface. The members of the laboratory are 3 teachers, 7 heads of practical work, 19 professors, 1 auxiliary teaching assistant student and two investigators. The sessions were recorded for analysis. The Ethnography of the starting point revealed that teachers identified the problems and disturbances that affected their practice as also appeared the potential expressed in their change proposals, at the beginning they offered some resistance and then called for the development of new concepts, objects of knowledge, by means of which they would rehearse, as a pilot test, the emerging proposals.
Published: 2016

29. SC164 - Starting a new kidney transplantation program from uncontrolled donors after circulatory death (DCD) in italy: A single-centre preliminary experience

Author: Li Marzi, V., Campi, R., Sessa, F., Giancane, S., Caroassai, S., Cirami, L., Olivieri, C., Barzaghi, P., Corti, F., Sebastianelli, A., Tuccio, A., Siena, G., Vignolini, G., Greco, I., Di Maria, L., Lazzeri, C., Peris, A., and Serni, S.
Published: 2019
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30. SC165 - Robotic kidney transplantation from living and deceased donors: Updated results at a median follow-up of 1 year

Author: Campi, R., Vignolini, G., Sessa, F., Greco, I., Olivieri, C., Larti, A., Cirami, L., Morselli, S., Corti, F., Grosso, A., Barzaghi, P., Giancane, S., Sebastianelli, A., Gacci, M., Peris, A., Breda, A., Siena, G., Li Marzi, V., and Serni, S.
Published: 2019
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31. SC161 - Use of perfusion parameters in predicting delayed graft function of machine-preserved kidneys retrieved from uncontrolled DCDs

Author: Li Marzi, V., Lazzeri, C., Caroassai, S., Fulceri, G., Tuccio, A., Siena, G., Vignolini, G., Campi, R., Caroti, L., Migliaccio, M., Cirami, L., Peris, A., and Serni, S.
Published: 2019
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32. SC159 - Small renal lesions found during organ retrieval for transplantation purposes: Our experience in donors after brain death over a 8 years period

Author: Facchiano, D., Grisanti, S. Caroassai, Migliaccio, M., Peris, A., Cirami, L., Morselli, S., Bombardi, M., Campi, R., Novelli, L., Serni, S., and Li Marzi, V.
Published: 2019
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33. RENAL HISTOPATHOLOGY. IMMUNOTACTOID GLOMEROLUPATHY: A CASE OF MONOCLONAL GAMMAPATHY OF RENAL SIGNIFICANCE

Author: Volgina, G., Gadzhikulieva, M., Uyshuk, N., Kawamura, E., Hisano, S., Nakashima, H., Saito, T., Boor, P., Babi kova, J., Martin, I. V., Bucher, E. B., Eriksson, U., Van Roeyen, C. R. C., Eitner, F., Floege, J., Peutz Kootstra, C. J., Ostendorf, T., Leh, S., Leh, F., Bjanes, T. K., Ohldieck, C., Svarstad, E., Han, B. G., Kim, J. S., Yang, J. W., Choi, S. O., Lollinga, W., Rahbar, A., De Wit, R. H., Riezebos Brilman, A., Soderberg Naucler, C., Van Son, W. J., Sanders, J. S., Smit, M. J., Van Den Born, J., Koike, K., Tsuboi, N., Ikezumi, Y., Go, K., Ogura, M., Saitoh, A., Yokoo, T., Yamaguchi, T., Nokiba, H., Hara, M., Morito, T., Kakihana, K., Ohashi, K., Ando, M., Kimura, T., Yagisawa, T., Nanmoku, K., Kurosawa, A., Sakuma, Y., Miki, A., Nukui, A., Alfieri, C. M., Regalia, A., Simonini, P., Ikehata, M., Chatziantoniou, C., Moroni, G., Rastaldi, M. P., Messa, P., Bockmeyer, C., Sauber lich, K., Zell, S., Zeuschner, P., Agustian, P. A., Wittig, J., Becker, J. U., Peters, B., Andersson, Y., Hadimeri, H., Stegmayr, B., Molne, J., Li, T., He, Y., Chen, H., Chen, J., Kobayashi, A., Mitome, J., Yamamoto, I., Mafune, A., Yamakawa, T., Nakada, Y., Tanno, Y., Ohkido, I., Yamamoto, H., Yokoyama, K., Dervishi, E., Buti, E., Nozzoli, C., Caldini, L. A., Giannakakis, Konstantinos, Minetti, E. E., Cirami, L., Bergesio, F., Ryuge, A., Nomura, A., Shimizu, H., Fujita, Y., Nishi, S., Goto, S., Nakai, K., Ito, J., Fujii, H., Hara, S., Mori, G., Ligabue, G., Cappelli, G., Pinho, A., Moreno, F., Dias, R., Vizcaino, R., Ossareh, S., Asgari, M., Abdi, E., Ataipour, Y., Malakoutian, T., Saddadi, F., and Rayatnia, M.
Published: 2014

34. PREGNANCY AND PROGRESSION OF IGA NEPHROPATHY: RESULTS OF AN ITALIAN MULTICENTER STUDY

Author: Limardo, M, Imbasciati, E, Ravani, P, Surian, M, Torres, D, Gregorini, G, Magistroni, R, Casellato, D, Gammaro, L, Pozzi, C, Gammaro L, Rene e. Gravidanza Collaborative Group of the Italian Society of N. e. p. h. r. o. l. o. g. y., Beati, S, Ambroso, G, Maggio, M, Del Giudice, A, De Cristofaro, V, Gallo, E, Gernone, G, Marega, A, Rustichelli, R, Cabiddu, G, Rollino, C, Fanciulli, E, Feriozzi, S, Pecchini, P, Bizzarri, D, D'Amico, M, Proietti, E, Baratelli, L, Battista Reinero, G, Garibotto, Giacomo, Rocchietti, M, Allegri, L, Dugo, M, Cirami, L, Esposito, C, Amico, L, Mariani, P, Baroli, A, Giannattasio, M, Stratta, P, Montanaro, D, Gesualdo, L, Daidone, G, Manfellotto, D, and Castellino, S.
Published: 2010

35. Validation of the Oxford classification of IgA nephropathy in cohorts with different presentations and treatments

Author: Coppo, Rosanna, Troyanov, Stéphan, Bellur, Shubha, Cattran, Daniel, Cook, H. Terence, Feehally, John, Roberts, Ian S.D., Morando, Laura, Camilla, Roberta, Tesar, Vladimir, Lunberg, Sigrid, Gesualdo, Loreto, Emma, Francesco, Rollino, Cristiana, Amore, Alessandro, Praga, Manuel, Feriozzi, Sandro, Segoloni, Giuseppe, Pani, Antonello, Cancarini, Giovanni, Durlik, Magalena, Moggia, Elisabetta, Mazzucco, Gianna, Giannakakis, Costantinos, Honsova, Eva, Sundelin, B. Brigitta, Palma, Anna Maria Di, Ferrario, Franco, Gutierrez, Eduardo, Asunis, Anna Maria, Barratt, Jonathan, Tardanico, Regina, Perkowska-Ptasinska, Agnieszka, Maixnerova, D., Lundberg, S., Fuiano, L., Beltrame, G., Peruzzi, L., Polci, R., Colla, L., Angioi, A., Piras, D., Ravera, S., Ballarin, J., Di Giulio, S., Pugliese, F., Caliskan, Y., Locatelli, F., Del Vecchio, L., Wetzels, J. F.M., Peters, H., Berg, U., Carvalho, F., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., Van Kooten, C., Rabelink, T., Reinders, M. E.J., Boria Grinyo, J. M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Ferrario, F., Gutiérrez, E., Arce Terroba, J., Fortunato, M., Ozluk, Y., Kilicaslan, I., Steenberger, E., Soderberg, M., Da Costa Ferreira, A. C., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Bertoni, E., Cannata Ortiz, P., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Coppo, Rosanna, Troyanov, Stéphan, Bellur, Shubha, Cattran, Daniel, Cook, H. Terence, Feehally, John, Roberts, Ian S.D., Morando, Laura, Camilla, Roberta, Tesar, Vladimir, Lunberg, Sigrid, Gesualdo, Loreto, Emma, Francesco, Rollino, Cristiana, Amore, Alessandro, Praga, Manuel, Feriozzi, Sandro, Segoloni, Giuseppe, Pani, Antonello, Cancarini, Giovanni, Durlik, Magalena, Moggia, Elisabetta, Mazzucco, Gianna, Giannakakis, Costantinos, Honsova, Eva, Sundelin, B. Brigitta, Palma, Anna Maria Di, Ferrario, Franco, Gutierrez, Eduardo, Asunis, Anna Maria, Barratt, Jonathan, Tardanico, Regina, Perkowska-Ptasinska, Agnieszka, Maixnerova, D., Lundberg, S., Fuiano, L., Beltrame, G., Peruzzi, L., Polci, R., Colla, L., Angioi, A., Piras, D., Ravera, S., Ballarin, J., Di Giulio, S., Pugliese, F., Caliskan, Y., Locatelli, F., Del Vecchio, L., Wetzels, J. F.M., Peters, H., Berg, U., Carvalho, F., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., Van Kooten, C., Rabelink, T., Reinders, M. E.J., Boria Grinyo, J. M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Ferrario, F., Gutiérrez, E., Arce Terroba, J., Fortunato, M., Ozluk, Y., Kilicaslan, I., Steenberger, E., Soderberg, M., Da Costa Ferreira, A. C., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Bertoni, E., Cannata Ortiz, P., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., and Ioachim, E.
Abstract: The Oxford Classification of IgA Nephropathy (IgAN) identified mesangial hypercellularity (M), endocapillary proliferation (E), segmental glomerulosclerosis (S), and tubular atrophy/interstitial fibrosis (T) as independent predictors of outcome. Whether it applies to individuals excluded from the original study and how therapy influences the predictive value of pathology remain uncertain. The VALIGA study examined 1147 patients from 13 European countries that encompassed the whole spectrum of IgAN. Over a median follow-up of 4.7 years, 86% received renin-angiotensin system blockade and 42% glucocorticoid/immunosuppressive drugs. M, S, and T lesions independently predicted the loss of estimated glomerular filtration rate (eGFR) and a lower renal survival. Their value was also assessed in patients not represented in the Oxford cohort. In individuals with eGFR less than 30 ml/min per 1.73 m 2, the M and T lesions independently predicted a poor survival. In those with proteinuria under 0.5 g/day, both M and E lesions were associated with a rise in proteinuria to 1 or 2 g/day or more. The addition of M, S, and T lesions to clinical variables significantly enhanced the ability to predict progression only in those who did not receive immunosuppression (net reclassification index 11.5%). The VALIGA study provides a validation of the Oxford classification in a large European cohort of IgAN patients across the whole spectrum of the disease. The independent predictive value of pathology MEST score is reduced by glucocorticoid/immunosuppressive therapy.
Published: 2014

36. RENAL HISTOPATHOLOGY

Author: Volgina, G., primary, Gadzhikulieva, M., additional, Uyshuk, N., additional, Kawamura, E., additional, Hisano, S., additional, Nakashima, H., additional, Saito, T., additional, Boor, P., additional, Babi kova, J., additional, Martin, I. V., additional, Bucher, E. B., additional, Eriksson, U., additional, Van Roeyen, C. R. C., additional, Eitner, F., additional, Floege, J., additional, Peutz-Kootstra, C. J., additional, Ostendorf, T., additional, Leh, S., additional, Leh, F., additional, Bjanes, T. K., additional, Ohldieck, C., additional, Svarstad, E., additional, Han, B. G., additional, Kim, J. S., additional, Yang, J. W., additional, Choi, S. O., additional, Lollinga, W., additional, Rahbar, A., additional, De Wit, R. H., additional, Riezebos-Brilman, A., additional, Soderberg-Naucler, C., additional, Van Son, W. J., additional, Sanders, J.-S., additional, Smit, M. J., additional, Van Den Born, J., additional, Koike, K., additional, Tsuboi, N., additional, Ikezumi, Y., additional, Go, K., additional, Ogura, M., additional, Saitoh, A., additional, Yokoo, T., additional, Yamaguchi, T., additional, Nokiba, H., additional, Hara, M., additional, Morito, T., additional, Kakihana, K., additional, Ohashi, K., additional, Ando, M., additional, Kimura, T., additional, Yagisawa, T., additional, Nanmoku, K., additional, Kurosawa, A., additional, Sakuma, Y., additional, Miki, A., additional, Nukui, A., additional, Alfieri, C. M., additional, Regalia, A., additional, Simonini, P., additional, Ikehata, M., additional, Chatziantoniou, C., additional, Moroni, G., additional, Rastaldi, M. P., additional, Messa, P., additional, Bockmeyer, C., additional, Sauberlich, K., additional, Zell, S., additional, Zeuschner, P., additional, Agustian, P. A., additional, Wittig, J., additional, Becker, J. U., additional, Peters, B., additional, Andersson, Y., additional, Hadimeri, H., additional, Stegmayr, B., additional, Molne, J., additional, Li, T., additional, He, Y., additional, Chen, H., additional, Chen, J., additional, Kobayashi, A., additional, Mitome, J., additional, Yamamoto, I., additional, Mafune, A., additional, Yamakawa, T., additional, Nakada, Y., additional, Tanno, Y., additional, Ohkido, I., additional, Yamamoto, H., additional, Yokoyama, K., additional, Dervishi, E., additional, Buti, E., additional, Nozzoli, C., additional, Caldini, L. A., additional, Giannakakis, C., additional, Minetti, E. E., additional, Cirami, L., additional, Bergesio, F., additional, Ryuge, A., additional, Nomura, A., additional, Shimizu, H., additional, Fujita, Y., additional, Nishi, S., additional, Goto, S., additional, Nakai, K., additional, Ito, J., additional, Fujii, H., additional, Hara, S., additional, Mori, G., additional, Ligabue, G., additional, Cappelli, G., additional, Pinho, A., additional, Moreno, F., additional, Dias, R., additional, Vizcaino, R., additional, Ossareh, S., additional, Asgari, M., additional, Abdi, E., additional, Ataipour, Y., additional, Malakoutian, T., additional, Saddadi, F., additional, and Rayatnia, M., additional
Published: 2014
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37. L10. Prediction and prevention of preeclampsia in autoimmune disease

Author: Mecacci, F., primary, Simeone, S., additional, Rambaldi, M.P., additional, Serena, C., additional, Ottanelli, S., additional, Marchetti, G., additional, Cirami, L., additional, and Mello, G., additional
Published: 2011
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38. The MEST score provides earlier risk prediction in lgA nephropathy

Author: Barbour, Sean J., Espino-Hernandez, Gabriela, Reich, Heather N., Coppo, Rosanna, Roberts, Ian S.D., Feehally, John, Herzenberg, Andrew M., Cattran, Daniel C., Bavbek, N., Cook, T., Troyanov, S., Alpers, C., Amore, A., Barratt, J., Berthoux, F., Bonsib, S., Bruijn, J., D’Agati, V., D’Amico, G., Emancipator, S., Emmal, F., Ferrario, F., Fervenza, F., Florquin, S., Fogo, A., Geddes, C., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zhang, H., Coppo, R., Troyanov, S., Cattran, D.C., Cook, H.T., Feehally, J., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., RC, Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Angioi, A., Piras, L., JF, Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Ferrario, F., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., and Ioachim, E.
Abstract: The Oxford Classification of IgA nephropathy (IgAN) includes the following four histologic components: mesangial (M) and endocapillary (E) hypercellularity, segmental sclerosis (S) and interstitial fibrosis/tubular atrophy (T). These combine to form the MEST score and are independently associated with renal outcome. Current prediction and risk stratification in IgAN requires clinical data over 2 years of follow-up. Using modern prediction tools, we examined whether combining MEST with cross-sectional clinical data at biopsy provides earlier risk prediction in IgAN than current best methods that use 2 years of follow-up data. We used a cohort of 901 adults with IgAN from the Oxford derivation and North American validation studies and the VALIGA study followed for a median of 5.6 years to analyze the primary outcome (50% decrease in eGFR or ESRD) using Cox regression models. Covariates of clinical data at biopsy (eGFR, proteinuria, MAP) with or without MEST, and then 2-year clinical data alone (2-year average of proteinuria/MAP, eGFR at biopsy) were considered. There was significant improvement in prediction by adding MEST to clinical data at biopsy. The combination predicted the outcome as well as the 2-year clinical data alone, with comparable calibration curves. This effect did not change in subgroups treated or not with RAS blockade or immunosuppression. Thus, combining the MEST score with cross-sectional clinical data at biopsy provides earlier risk prediction in IgAN than our current best methods.
Published: 2016
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39. The Role of Rituximab in Primary Focal Segmental Glomerular Sclerosis of the Adult

Author: Martina Tedesco, Federica Mescia, Isabella Pisani, Marco Allinovi, Giovanni Casazza, Lucia Del Vecchio, Marisa Santostefano, Luigi Cirillo, Francesca Ferrario, Ciro Esposito, Pasquale Esposito, Domenico Santoro, Roberta Lazzarin, Giovanni Maria Rossi, Enrico Fiaccadori, Angelo Ferrantelli, Renato Alberto Sinico, Mario Cozzolino, Maurizio Gallieni, Lino Cirami, Francesco Scolari, Augusto Vaglio, Federico Alberici, Stefania Affatato, Leonardo Caroti, Elena Mancini, Luca Semeraro, Rossella Siligato, Matthias Arnaldo Cassia, Pietro Napodano, Marta Calatroni, Cosimo Distratis, Andrea Campo, Tedesco, M, Mescia, F, Pisani, I, Allinovi, M, Casazza, G, Del Vecchio, L, Santostefano, M, Cirillo, L, Ferrario, F, Esposito, C, Esposito, P, Santoro, D, Lazzarin, R, Rossi, G, Fiaccadori, E, Ferrantelli, A, Sinico, R, Cozzolino, M, Gallieni, M, Cirami, L, Scolari, F, Vaglio, A, Alberici, F, Affatato, S, Caroti, L, Mancini, E, Semeraro, L, Siligato, R, Cassia, M, Napodano, P, Calatroni, M, Distratis, C, and Campo, A
Subjects: rituximab, Nephrology, focal segmental glomerulosclerosi
Abstract: Introduction: Primary focal segmental glomerular sclerosis (FSGS) is a rare, likely immune-mediated disease. Rituximab (RTX) may play a role in management, although data in adults are scanty. Methods: We collected cases of RTX-treated primary FSGS within the Italian Society of Nephrology Immunopathology Working Group and explored response rate (24-hour proteinuria
Published: 2022
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40. Application of the International IgA Nephropathy Prediction Tool one or two years post-biopsy

Author: Sean J. Barbour, Rosanna Coppo, Hong Zhang, Zhi-Hong Liu, Yusuke Suzuki, Keiichi Matsuzaki, Lee Er, Heather N. Reich, Jonathan Barratt, Daniel C. Cattran, M.L. Russo, S. Troyanov, H.T. Cook, I. Roberts, V. Tesar, D. Maixnerova, S. Lundberg, L. Gesualdo, F. Emma, L. Fuiano, G. Beltrame, C. Rollino, A. Amore, R. Camilla, L. Peruzzi, M. Praga, S. Feriozzi, R. Polci, G. Segoloni, L. Colla, A. Pani, D. Piras, A. Angioi, G. Cancarini, S. Ravera, M. Durlik, E. Moggia, J. Ballarin, S. Di Giulio, F. Pugliese, I. Serriello, Y. Caliskan, M. Sever, I. Kilicaslan, F. Locatelli, L. Del Vecchio, J.F.M. Wetzels, H. Peters, U. Berg, F. Carvalho, A.C. da Costa Ferreira, M. Maggio, A. Wiecek, M. Ots-Rosenberg, R. Magistroni, R. Topaloglu, Y. Bilginer, M. D’Amico, M. Stangou, F. Giacchino, D. Goumenos, E. Papachristou, K. Galesic, C. Geddes, K. Siamopoulos, O. Balafa, M. Galliani, P. Stratta, M. Quaglia, R. Bergia, R. Cravero, M. Salvadori, L. Cirami, B. Fellstrom, H. Kloster Smerud, F. Ferrario, T. Stellato, J. Egido, C. Martin, J. Floege, F. Eitner, A. Lupo, P. Bernich, P. Menè, M. Morosetti, C. van Kooten, T. Rabelink, M.E.J. Reinders, J.M. Boria Grinyo, S. Cusinato, L. Benozzi, S. Savoldi, C. Licata, M. Mizerska-Wasiak, G. Martina, A. Messuerotti, A. Dal Canton, C. Esposito, C. Migotto, G. Triolo, F. Mariano, C. Pozzi, R. Boero, S. Bellur, G. Mazzucco, C. Giannakakis, E. Honsova, B. Sundelin, A.M. Di Palma, E. Gutiérrez, A.M. Asunis, J. Barratt, R. Tardanico, A. Perkowska-Ptasinska, J. Arce Terroba, M. Fortunato, A. Pantzaki, Y. Ozluk, E. Steenbergen, M. Soderberg, Z. Riispere, L. Furci, D. Orhan, D. Kipgen, D. Casartelli, D. Galesic Ljubanovic, H. Gakiopoulou, E. Bertoni, P. Cannata Ortiz, H. Karkoszka, H.J. Groene, A. Stoppacciaro, I. Bajema, J. Bruijn, X. Fulladosa Oliveras, J. Maldyk, E. Ioachim, N. Bavbek, T. Cook, C. Alpers, F. Berthoux, S. Bonsib, V. D’Agati, G. D’Amico, S. Emancipator, F. Emmal, F. Fervenza, S. Florquin, A. Fogo, H. Groene, M. Haas, P. Hill, R. Hogg, S. Hsu, T. Hunley, M. Hladunewich, C. Jennette, K. Joh, B. Julian, T. Kawamura, F. Lai, C. Leung, L. Li, P. Li, Z. Liu, A. Massat, B. Mackinnon, S. Mezzano, F. Schena, Y. Tomino, P. Walker, H. Wang, J. Weening, N. Yoshikawa, C.-H. Zeng, S. Shi, C. Nogi, H. Suzuki, K. Koike, K. Hirano, T. Yokoo, M. Hanai, K. Fukami, K. Takahashi, Y. Yuzawa, M. Niwa, Y. Yasuda, S. Maruyama, D. Ichikawa, T. Suzuki, S. Shirai, A. Fukuda, S. Fujimoto, H. Trimarchi, Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A. M., Russo, M. L., Ferrario, F., Gutiérrez, E., Asunis, A. M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Terroba, J. Arce, Fortunato, M., Pantzaki, A., Ozluk, Y., Troyanov, S., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Ljubanovic, D. Galesic, Gakiopoulou, H., Bertoni, E., Cook, H. T., Cannata Ortiz, P., Karkoszka, H., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Bavbek, N., Roberts, I., Cook, T., Alpers, C., Amore, A., Berthoux, F., Bonsib, S., D'Agati, V., D'Amico, G., Tesar, V., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Geddes, C., Groene, H., Haas, M., Hill, P., Maixnerova, D., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Lundberg, S., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Gesualdo, L., Weening, J., Yoshikawa, N., Zeng, C.-H., Shi, S., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Emma, F., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fuiano, L., Fukuda, A., Fujimoto, S., Trimarchi, H., Beltrame, G., Rollino, C., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J. F. M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A. C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Papachristou, E., Galesic, K., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Smerud, H. Kloster, Stellato, T., Egido, J., Martin, C., Flöge, Jürgen, Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M. E. J., Boria Grinyo, J. M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Pathology, Center of Experimental and Molecular Medicine, Graduate School, and ACS - Heart failure & arrhythmias
Subjects: Adult, disease progression, end-stage kidney disease, IgA nephropathy, prediction tool, risk prediction, Biopsy, Glomerulonephritis, IGA, Prognosis, Cohort Studies, Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], Nephrology, Humans, Renal Insufficiency, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], Glomerular Filtration Rate
Abstract: Kidney international 102(1), 160-172 (2022). doi:10.1016/j.kint.2022.02.042, Published by Elsevier, New York, NY
Published: 2022
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41. Long-Term Efficacy and Safety of the Long-Acting Complement C5 Inhibitor Ravulizumab for the Treatment of Atypical Hemolytic Uremic Syndrome in Adults

Author: Thomas Barbour, Marie Scully, Gema Ariceta, Spero Cataland, Katherine Garlo, Nils Heyne, Yosu Luque, Jan Menne, Yoshitaka Miyakawa, Sung-Soo Yoon, David Kavanagh, Sunil Babu, Nilufer Broeders, Nicole Lietar, Fiona Brown, Philip Campbell, Josep M. Campistol, Paramit Chowdhury, Theo Kasimatis, Lino Cirami, Leonardo Caroti, Guilia Antognoli, Yahsou Delmas, Vladimir Dobronravov, Anja Gaeckler, Cyril Garrouste, Gregory Greenwood, Siân Griffin, Chiu-Ching Huang, I-Ru Chen, Susan Huang, Jin Seok Kim, Gaetano La Manna, Moglie Le Quintrec, Guillaume Jeantet, Iino Fumie, Eric Rondeau, Hermann Haller, Johan Morelle, Eric Goffin, Anja Muhlfeld, Shashi Nagaraj, Gowthami Arepally, Doyeun Oh, Masayoshi Okumi, Manuel Praga Terente, Francois Provot, Ulf Schönermarck, Michael Fischereder, Natalia Ramos Terrada, Barbara Seitz-Polski, Guillaume Favre, Sonia Boyer-Suavet, Maria Vinogradova, Tatiana Kirsanova, Edwin K.S. Wong, Barbour T., Scully M., Ariceta G., Cataland S., Garlo K., Heyne N., Luque Y., Menne J., Miyakawa Y., Yoon S.-S., Kavanagh D., Babu S., Broeders N., Lietar N., Brown F., Campbell P., Campistol J.M., Chowdhury P., Kasimatis T., Cirami L., Caroti L., Antognoli G., Delmas Y., Dobronravov V., Gaeckler A., Garrouste C., Greenwood G., Griffin S., Huang C.-C., Chen I.-R., Huang S., Kim J.S., La Manna G., Le Quintrec M., Jeantet G., Fumie I., Rondeau E., Haller H., Morelle J., Goffin E., Muhlfeld A., Nagaraj S., Arepally G., Oh D., Okumi M., Terente M.P., Provot F., Schonermarck U., Fischereder M., Terrada N.R., Seitz-Polski B., Favre G., Boyer-Suavet S., Vinogradova M., Kirsanova T., Wong E.K.S., Institut Català de la Salut, [Barbour T] Kidney Care, The Royal Melbourne Hospital, Melbourne, Australia. [Scully M] Department of Haematology, University College London Hospitals, London, UK. [Ariceta G] Servei de Nefrologia Pediàtrica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Cataland S] Division of Hematology, The Ohio State University Medical Center, Columbus, Ohio, USA. [Garlo K] Clinical Development, Alexion Pharmaceuticals, Inc., Boston, Massachusetts, USA. [Heyne N] Section of Nephrology and Hypertension, Tübingen University Hospital, Tübingen, Germany, Vall d'Hebron Barcelona Hospital Campus, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service de néphrologie, The Royal Melbourne Hospital, University College London Hospitals (UCLH), Vall d'Hebron University Hospital [Barcelona], Ohio State University [Columbus] (OSU), Universitätsklinikum Tübingen - University Hospital of Tübingen, Eberhard Karls Universität Tübingen = Eberhard Karls University of Tuebingen, Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Seoul National University Hospital, and Newcastle University [Newcastle]
Subjects: medicine.medical_specialty, Thrombotic microangiopathy, [SDV]Life Sciences [q-bio], Hemic and Lymphatic Diseases::Hematologic Diseases::Anemia::Anemia, Hemolytic::Hemolytic-Uremic Syndrome::Hemic and Lymphatic Diseases::Hematologic Diseases::Atypical Hemolytic Uremic Syndrome [DISEASES], 030232 urology & nephrology, Renal function, Otros calificadores::Otros calificadores::/farmacoterapia [Otros calificadores], 030204 cardiovascular system & hematology, Other subheadings::Other subheadings::/drug therapy [Other subheadings], 03 medical and health sciences, 0302 clinical medicine, afecciones patológicas, signos y síntomas::procesos patológicos::atributos de la enfermedad::enfermedades raras [ENFERMEDADES], Clinical Research, Internal medicine, Atypical hemolytic uremic syndrome, Medicine, complement, Adverse effect, Complement component 5, business.industry, Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Rare Diseases [DISEASES], atypical hemolytic uremic syndrome, Acute kidney injury, diagnóstico::pronóstico::resultado del tratamiento [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS], medicine.disease, Diagnosis::Prognosis::Treatment Outcome [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT], ravulizumab, Diseases of the genitourinary system. Urology, 3. Good health, enfermedades hematológicas y linfáticas::enfermedades hematológicas::anemia::anemia hemolítica::síndrome hemolítico-urémico::enfermedades hematológicas y linfáticas::enfermedades hematológicas::síndrome hemolítico urémico atípico [ENFERMEDADES], kidney failure, thrombotic microangiopathy, Clinical trial, Nephrology, Avaluació de resultats (Assistència sanitària), hemolytic uremic syndrome, RC870-923, Síndrome hemolíticourèmica - Tractament, Malalties rares, business, Kidney disease
Abstract: Hemolytic uremic syndrome; Kidney failure; Ravulizumab Síndrome hemolítico urémico; Insuficiencia renal; Ravulizumab Síndrome hemolític urèmic; Insuficiència renal; Ravulizumab Introduction Atypical hemolytic uremic syndrome (aHUS) is a rare, complex, multisystem disease of dysregulated complement activity, characterized by progressive thrombotic microangiopathy (TMA), acute kidney injury, and multiorgan dysfunction, which often progresses to chronic kidney disease. Results from the prospective clinical trial of ravulizumab (NCT02949128) reveal rapid resolution of TMA in patients with aHUS, with sustained efficacy and safety in a 26-week initial evaluation period. Methods The aim of this analysis was to characterize the long-term efficacy and the safety profile of ravulizumab in adults with aHUS who had completed the initial evaluation period of the trial. Complete TMA response, hematologic and kidney functions, and safety were evaluated for all patients available for follow-up in the extension period (median follow-up: 76.7 weeks; range: 0.6–118.3). This trial included a total of 58 patients, 49 of whom entered the extension period. Results A total of 4 additional patients achieved complete TMA response during the follow-up period. Normalization of platelet count, serum lactate dehydrogenase (LDH), and hemoglobin observed in the 26-week initial evaluation period was sustained until the last available follow-up, as were the improvements in the estimated glomerular filtration rate (eGFR) and patient quality of life. All efficacy endpoints were correlated with the sustained inhibition of complement C5. Most adverse events (AEs) occurred early during the initial evaluation period and decreased substantially during the extension period. No patient developed a meningococcal infection or died during the extension period. Conclusion This analysis reveals that ravulizumab administered every 8 weeks is efficacious with an acceptable safety profile for the long-term treatment of adults with aHUS and provides additional clinical benefit beyond 6 months of treatment.
Published: 2021
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42. Corrigendum to 'Rondeau E, Scully M, Ariceta G, Barbour T, Cataland S, Heyne N, Miyakawa Y, Ortiz S, Swenson E, Vallee M, Yoon S-S, Kavanagh D and Haller H; on behalf of the 311 Study Group. The long-acting C5 inhibitor, Ravulizumab, is effective and safe in adult patients with atypical hemolytic uremic syndrome naïve to complement inhibitor treatment.' Kidney Int. 2020;97:1287–1296

Author: Eric Rondeau, Marie Scully, Gema Ariceta, Tom Barbour, Spero Cataland, Nils Heyne, Yoshitaka Miyakawa, Stephan Ortiz, Eugene Swenson, Marc Vallee, Sung-Soo Yoon, David Kavanagh, Hermann Haller, Sunil Babu, Nilufer Broeders, Nicole Lietar, Fiona Brown, Philip Campbell, Josep M. Campistol, Miquel Blasco, Paramit Chowdhury, Theo Kasimatis, Lino Cirami, Leonardo Caroti, Guilia Antognoli, Yahsou Delmas, Vladimir Dobronravov, Anja Gaeckler, Cyril Garrouste, Gregory Greenwood, Siân Griffin, Chiu-Ching Huang, I-Ru Chen, Susan Huang, Jin Seok Kim, Gaetano La Manna, Giorgia Comai, Maria Cappuccilli, Moglie Le Quintrec, Guillaume Jeantet, Iino Fumie, Yosu Luque, Jan Menne, Johan Morelle, Eric Goffin, Anja Muhlfeld, Shashi Nagaraj, Gowthami Arepally, Doyeun Oh, Masayoshi Okumi, Manuel Praga Terente, Elena Gutierréz, Paola Rodriguez, Francois Provot, Ulf Schönermarck, Michael Fischereder, Natalia Ramos Terrada, Barbara Seitz-Polski, Guillaume Favre, Sonia Boyer-Suavet, Maria Vinogradova, Tatiana Kirsanova, Edwin K.S. Wong, Rondeau E., Scully M., Ariceta G., Barbour T., Cataland S., Heyne N., Miyakawa Y., Ortiz S., Swenson E., Vallee M., Yoon S.-S., Kavanagh D., Haller H., Babu S., Broeders N., Lietar N., Brown F., Campbell P., Campistol J.M., Blasco M., Chowdhury P., Kasimatis T., Cirami L., Caroti L., Antognoli G., Delmas Y., Dobronravov V., Gaeckler A., Garrouste C., Greenwood G., Griffin S., Huang C.-C., Chen I.-R., Huang S., Kim J.S., La Manna G., Comai G., Cappuccilli M., Le Quintrec M., Jeantet G., Fumie I., Luque Y., Menne J., Morelle J., Goffin E., Muhlfeld A., Nagaraj S., Arepally G., Oh D., Okumi M., Terente M.P., Gutierrez E., Rodriguez P., Provot F., Schonermarck U., Fischereder M., Terrada N.R., Seitz-Polski B., Favre G., Boyer-Suavet S., Vinogradova M., Kirsanova T., and Wong E.K.S.
Subjects: Kidney, medicine.medical_specialty, Adult patients, business.industry, INT, medicine.disease, Gastroenterology, Complement inhibitor, medicine.anatomical_structure, Long acting, Nephrology, Internal medicine, Atypical hemolytic uremic syndrome, Medicine, Ravulizumab, Thrombotic microangiopathy, Pregnancy microangiopathies, Atypical hemolytic uremic syndrome, business
Abstract: The authors regret that 2 investigators were not included in the 311 Study Group. Members of the 311 Study Group are listed in the Appendix. The authors would like to apologize for any inconvenience caused.
Published: 2021
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43. Erratum to 'Rondeau E, Scully M, Ariceta G, Barbour T, Cataland S, Heyne N, Miyakawa Y, Ortiz S, Swenson E, Vallee M, Yoon S-S, Kavanagh D, Haller H; on behalf of the 311 Study Group. The long-acting C5 inhibitor, Ravulizumab, is effective and safe in adult patients with atypical hemolytic uremic syndrome naïve to complement inhibitor treatment' Kidney Int. 2020;97:1287–1296

Author: Eric Rondeau, Marie Scully, Gema Ariceta, Tom Barbour, Spero Cataland, Nils Heyne, Yoshitaka Miyakawa, Stephan Ortiz, Eugene Swenson, Marc Vallee, Sung-Soo Yoon, David Kavanagh, Hermann Haller, Sunil Babu, Nilufer Broeders, Nicole Lietar, Fiona Brown, Philip Campbell, Paramit Chowdhury, Theo Kasimatis, Lino Cirami, Leonardo Caroti, Guilia Antognoli, Yahsou Delmas, Vladimir Dobronravov, Anja Gaeckler, Cyril Garrouste, Gregory Greenwood, Siân Griffin, Chiu-Ching Huang, I-Ru Chen, Susan Huang, Jin Seok Kim, Gaetano La Manna, Giorgia Comai, Maria Cappuccilli, Moglie Le Quintrec, Guillaume Jeantet, Iino Fumie, Yosu Luque, Jan Menne, Johan Morelle, Eric Goffin, Anja Muhlfeld, Shashi Nagaraj, Gowthami Arepally, Doyeun Oh, Masayoshi Okumi, Manuel Praga Terente, Elena Gutierréz, Paola Rodriguez, Francois Provot, Ulf Schönermarck, Michael Fischereder, Natalia Ramos Terrada, Barbara Seitz-Polski, Guillaume Favre, Sonia Boyer-Suavet, Maria Vinogradova, Tatiana Kirsanova, Edwin K.S. Wong, Rondeau E., Scully M., Ariceta G., Barbour T., Cataland S., Heyne N., Miyakawa Y., Ortiz S., Swenson E., Vallee M., Yoon S.-S., Kavanagh D., Haller H., Babu S., Broeders N., Lietar N., Brown F., Campbell P., Chowdhury P., Kasimatis T., Cirami L., Caroti L., Antognoli G., Delmas Y., Dobronravov V., Gaeckler A., Garrouste C., Greenwood G., Griffin S., Huang C.-C., Chen I.-R., Huang S., Kim J.S., La Manna G., Comai G., Cappuccilli M., Le Quintrec M., Jeantet G., Fumie I., Luque Y., Menne J., Morelle J., Goffin E., Muhlfeld A., Nagaraj S., Arepally G., Oh D., Okumi M., Terente M.P., Gutierrez E., Rodriguez P., Provot F., Schonermarck U., Fischereder M., Terrada N.R., Seitz-Polski B., Favre G., Boyer-Suavet S., Vinogradova M., Kirsanova T., and Wong E.K.S.
Subjects: N.A, Nephrology
Abstract: The publisher regrets that the members of the 311 Study Group were not included as collaborative authors. Members of the 311 Study Group are listed in the Appendix. The publisher would like to apologize for any inconvenience caused.
Published: 2020
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44. The Role of Rituximab in Primary Focal Segmental Glomerular Sclerosis of the Adult.

Author: Tedesco M, Mescia F, Pisani I, Allinovi M, Casazza G, Del Vecchio L, Santostefano M, Cirillo L, Ferrario F, Esposito C, Esposito P, Santoro D, Lazzarin R, Rossi GM, Fiaccadori E, Ferrantelli A, Sinico RA, Cozzolino M, Gallieni M, Cirami L, Scolari F, Vaglio A, and Alberici F
Abstract: Introduction: Primary focal segmental glomerular sclerosis (FSGS) is a rare, likely immune-mediated disease. Rituximab (RTX) may play a role in management, although data in adults are scanty., Methods: We collected cases of RTX-treated primary FSGS within the Italian Society of Nephrology Immunopathology Working Group and explored response rate (24-hour proteinuria <3.5 g and <50% compared with baseline, stable estimated glomerular filtration rate)., Results: A total of 31 patients were followed for at least 12 months; further follow-up (median 17 months, interquartile range [IQR] 15-33.5) was available for 11. At first RTX administration, median creatinine and 24-hour proteinuria were 1.17 mg/dl (IQR 0.83-1.62) and 5.2 g (IQR 3.3-8.81), respectively. Response rate at 3, 6, and 12 months was 39%, 52%, and 42%, respectively. In the first 12 months, creatinine level remained stable whereas proteinuria and serum albumin level improved, with an increase in the proportion of patients tapering other immunosuppressants. There were 6 patients who were retreated with RTX within 12 months, either for proteinuria increase or refractory disease; only the 2 responders to the first RTX course experienced a further response. At univariate analysis, 6-month response was more frequent in steroid-dependent patients (odds ratio [OR] 7.7 [95% CI 1.16-52.17]) and those with proteinuria <5 g/24 h (OR 8.25 [1.45-46.86]). During long-term follow-up, 4 of 5 responders at 12 months maintained a sustained response, either without further immunosuppression (2 of 4) or with pre-emptive RTX (2 of 4); 1 relapsed and responded to RTX retreatment., Conclusion: RTX may be an option in primary FSGS, especially in steroid-dependent patients, with 24-hour proteinuria <5 g and previously responders to RTX. Optimal long-term management for responders is unclear, with some patients experiencing sustained remission and others requiring RTX retreatment, either preemptive or after rising proteinuria., (© 2022 International Society of Nephrology. Published by Elsevier Inc.)
Published: 2022
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45. Lung ultrasound may help in the differential diagnosis of suspected oligosymptomatic COVID-19 patients on hemodialysis: A case report.

Author: Allinovi M, Laudicina S, Dallari L, Gianassi I, Dervishi E, Biagini M, and Cirami L
Subjects: COVID-19 Testing, Diagnosis, Differential, Humans, Lung diagnostic imaging, Renal Dialysis, SARS-CoV-2, Tomography, X-Ray Computed, Ultrasonography, COVID-19, Pandemics
Abstract: Introduction: Considering that patients on dialysis showed a poor outcome during COVID-19 pandemic, and that COVID-19 symptoms in dialysis patients are often mild or absent, each dialysis unit should implement local strategies to early recognize patients affected by COVID-19. However, many available SARS-CoV-2 diagnostic tests demonstrated a moderate sensitivity, 70%-80% is probably a reasonable estimate. Consequently, having useful tools for differential diagnosis becomes essential. In this scenario, lung ultrasound (LUS) may have an important role in the evaluation of lung involvement in hemodialysis patients during COVID-19 pandemic., Methods: We present two cases of hemodialysis patients with COVID-19 pneumonia in whom LUS had a central role in the diagnostic process. Ultrasound images of COVID-19 pneumonia show a typical bilateral pattern characterized by multiple or confluent B-lines with spared areas, thickened and irregular pleural line, and rare subpleural consolidations. LUS showed high accuracy in diagnosing COVID-19 pneumonia., Findings: Despite both patients appeared clinically euvolemic and afebrile, they presented with acute diarrhea and oxygen saturation level of 92%-93%. Although clinical manifestations were mild and not specific in both patients, LUS raised suspicion on the possible COVID-19 diagnosis which was confirmed by a positive nasopharyngeal RT-PCR., Discussion: There are many reasons for a patient on dialysis to present shortness of breath, fever, and multiple B-lines at LUS assessment (such as heart failure, fluid overload, vascular access infection, interstitial pneumonia) but the recognition of typical ultrasound patterns of the COVID-19 pneumonia is helpful for differential diagnosis. LUS may have an important role in the screening process of hemodialysis patients during the COVID-19 pandemic, especially in oligosymptomatic patients before the SARS-CoV-2 diagnostic tests, and in those with suspected symptoms and/or known exposure with unexpected negative SARS-CoV-2 diagnostic tests., (© 2021 International Society for Hemodialysis.)
Published: 2021
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46. Delayed graft function and perfusion parameters of kidneys from uncontrolled donors after circulatory death.

Author: Peris A, Fulceri GE, Lazzeri C, Bonizzoli M, Li Marzi V, Serni S, Cirami L, and Migliaccio ML
Subjects: Graft Survival, Humans, Kidney, Organ Preservation, Perfusion, Delayed Graft Function etiology, Kidney Transplantation
Abstract: Better preservation and evaluation of kidneys from donors after circulatory death serve to increase the number of kidneys available for transplantation and hypothermic machine perfusion has been shown to decrease ischemia reperfusion injury and delayed graft function. Data on relation between hemodynamic parameters during hypothermic machine perfusion and delayed graft function in kidneys from donors after circulatory death are so far scarce and not univocal. We aimed at assessing whether hemodynamic parameters measured during hypothermic machine perfusion (flow, mean perfusion pressure, and renal resistance) are associated with delayed graft function in 26 kidneys retrieved from uncontrolled donors after circulatory death. In our series, the incidence of delayed graft function was 57.7% (15/26). Recipients who developed delayed graft function had a longer warm ischemic time (p = 0.04). All hemodynamic parameters measured during hypothermic machine perfusion were comparable between recipients with delayed graft function and those without. According to our data, in kidneys from uncontrolled donors after circulatory death, a longer warm ischemic time (that is the overall time of no flow, as the sum of the no-flow and the no-touch period) is associated with delayed graft function. This finding underscores the pivotal role of ischemic injury in terms of absence of flow in affecting graft function. No association was detectable between hemodynamic parameters during hypothermic machine perfusion and the development of delayed graft function in our series.
Published: 2021
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47. Exercise Prescription in Renal Transplant Recipients: From Sports Medicine Toward Multidisciplinary Aspects: A Pilot Study.

Author: Orlandi G, Sofi F, Moscarelli L, Cirami L, Mancini S, and Stefani L
Abstract: Renal transplantation is the choice treatment for end-stage renal disease. In spite of transplantation, cardiovascular morbidity and mortality remains high, possibly due to a prolonged sedentary lifestyle prior to transplantation. This study aimed to evaluate the impact of unsupervised intervention in a tailored home-based aerobic resistance exercise program, based on the anthropometric and cardiovascular parameters in a group of renal transplant recipients (RTRs) followed for 12 months., Methods: a group of 21 RTRs (mean age: 46.8 ± 12 years) were enrolled in a combined aerobic and step count unsupervised prescription program. Body composition (BMI, waist circumferences, skin-folds); water distribution (TBW: Total body water; ECW: Extra cellular water; and ICW: Intracellular water) and myocardial function were measured every 6 months for 1 year. The MEDI-LITE score was used to estimate adherence to the Mediterranean diet., Results: Significant reductions in waist circumference (Waist Cir: 89.12 ± 12.8 cm T0; 89.1 ± 12.5 cm T6 (95% CI: 6.3, 5.7); 88.6 ± 11.4 cm T12; (95% CI: 6.7, 4.7) p < 0.01), weight:71.8 ± 14.8 kg T0; 70.6 ± 14.7 kg T6(95% CI:-8, 6); 70.6 ± 14.7 kg T12(95% CI: 6.6, 7) p < 0.05), as well as an improvement of myocardial function, as shown by the significant increase of contractility and change in the GLS % value (-18.3 ± 3.8% at T0 (95% CI:-16.57, 20.0.2)-20.4 ± 3.0% at T6(95% CI:-4, 0.2);-22.9 ± 3.1%T12(95% CI:-3, 4, -1, 6) p < 0.02), were observed. Adherence to the Mediterranean diet was in the normal range., Conclusions: Despite unsupervised intervention, combined moderate physical exercise appears to have a positive effect on the main parameters related to cardiovascular risk factors. The long-term efficacy of this program requires further investigation, particularly for evaluating constant adherence to the home-based physical exercise program.
Published: 2020
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48. Addition of azathioprine to corticosteroids does not benefit patients with IgA nephropathy.

Author: Pozzi C, Andrulli S, Pani A, Scaini P, Del Vecchio L, Fogazzi G, Vogt B, De Cristofaro V, Allegri L, Cirami L, Procaccini AD, and Locatelli F
Subjects: Adrenal Cortex Hormones therapeutic use, Adult, Antihypertensive Agents therapeutic use, Azathioprine adverse effects, Blood Pressure drug effects, Drug Therapy, Combination, Female, Humans, Immunosuppressive Agents adverse effects, Male, Middle Aged, Proteinuria drug therapy, Tissue Survival drug effects, Azathioprine administration & dosage, Glomerulonephritis, IGA drug therapy, Immunosuppressive Agents administration & dosage
Abstract: The optimal treatment for IgA nephropathy (IgAN) remains unknown. Some patients respond to corticosteroids, suggesting that more aggressive treatment may provide additional benefit. We performed a randomized, multicenter, controlled trial to determine whether adding azathioprine to steroids improves renal outcome. We randomly assigned 207 IgAN patients with creatinine ≤2.0 mg/dl and proteinuria ≥1.0 g/d to either (1) a 3-day pulse of methylprednisolone in months 1, 3, and 5 in addition to both oral prednisone 0.5 mg/kg every other day and azathioprine 1.5 mg/kg per day for 6 months (n = 101, group 1) or (2) steroids alone on the same schedule (n = 106, group 2). The primary outcome was renal survival (time to 50% increase in plasma creatinine from baseline); secondary outcomes were changes in proteinuria over time and safety. After a median follow-up of 4.9 years, the primary endpoint occurred in 13 patients in group 1 (12.9%, 95% CI 7.5 to 20.9%) and 12 patients in group 2 (11.3%, CI 6.5 to 18.9%) (P = 0.83). Five-year cumulative renal survival was similar between groups (88 versus 89%; P = 0.83). Multivariate Cox regression analysis revealed that female gender, systolic BP, number of antihypertensive drugs, ACE inhibitor use, and proteinuria during follow-up predicted the risk of reaching the primary endpoint. Treatment significantly decreased proteinuria from 2.00 to 1.07 g/d during follow-up (P < 0.001) on average, with no difference between groups. Treatment-related adverse events were more frequent among those receiving azathioprine. In summary, adding low-dose azathioprine to corticosteroids for 6 months does not provide additional benefit to patients with IgAN and may increase the risk for adverse events.
Published: 2010
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49. Multiple immune disorders in unrecognized celiac disease: a case report.

Author: La Villa G, Pantaleo P, Tarquini R, Cirami L, Perfetto F, Mancuso F, and Laffi G
Subjects: Abortion, Habitual etiology, Adult, Celiac Disease diet therapy, Diet, Female, Glomerulonephritis, IGA etiology, Glutens administration & dosage, Humans, Hyperamylasemia etiology, Lipase blood, Thyroiditis, Autoimmune etiology, Celiac Disease complications, Immune System Diseases etiology
Abstract: We reported a female patient with unrecognized celiac disease and multiple extra intestinal manifestations, mainly related to a deranged immune function, including macroamilasemia, macrolipasemia, IgA nephropathy, thyroiditis, and anti-b2-glicoprotein-1 antibodies, that disappeared or improved after the implementation of a gluten-free diet.
Published: 2003
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