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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 more...

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 more...

Published
2022

21. P0372RITUXIMAB IN IDIOPATHIC FOCAL SEGMENTAL GLOMERULOSCLEROSIS OF THE ADULT: A MULTICENTRE RETROSPECTIVE SURVEY OF 31 PATIENTS

Author

Alberici, F, Vaglio, A, Cirami, C, Scolari, F, Gallieni, M, Cozzolino, M, Sinico, R, Ferrantelli, A, Delbarba, E, Lazzarin, R, Santoro, D, Esposito, P, Esposito, C, Ferrario, F, Santostefano, M, Del Vecchio, L, Casazza, G, Allinovi, M, Pisani, I, Tedesco, M, Alberici, Federico, Vaglio, Augusto, Cirami, Calogero, Scolari, Francesco, Gallieni, Maurizio, Cozzolino, Mario Gennaro, Sinico, Renato A, Ferrantelli, Angelo, Delbarba, Elisa, Lazzarin, Roberta, Santoro, Domenico, Esposito, Pasquale, Esposito, Ciro, Ferrario, Francesca, Santostefano, Marisa, Del Vecchio, Lucia, Casazza, Giovanni, Allinovi, Marco, Pisani, Isabella, Tedesco, Martina, Alberici, F, Vaglio, A, Cirami, C, Scolari, F, Gallieni, M, Cozzolino, M, Sinico, R, Ferrantelli, A, Delbarba, E, Lazzarin, R, Santoro, D, Esposito, P, Esposito, C, Ferrario, F, Santostefano, M, Del Vecchio, L, Casazza, G, Allinovi, M, Pisani, I, Tedesco, M, Alberici, Federico, Vaglio, Augusto, Cirami, Calogero, Scolari, Francesco, Gallieni, Maurizio, Cozzolino, Mario Gennaro, Sinico, Renato A, Ferrantelli, Angelo, Delbarba, Elisa, Lazzarin, Roberta, Santoro, Domenico, Esposito, Pasquale, Esposito, Ciro, Ferrario, Francesca, Santostefano, Marisa, Del Vecchio, Lucia, Casazza, Giovanni, Allinovi, Marco, Pisani, Isabella, and Tedesco, Martina more...

Abstract

Idiopathic Focal Segmental Glomerulosclerosis (FSGS) is a rare glomerulonephritis often complicated by a chronic relapsing course frequently characterized by dependency or resistance to immunosuppressive treatment. Moreover, about half of the patients with active disease would develop end-stage renal disease within 10 years from the diagnosis, highlighting the need of novel therapeutic approaches. Rituximab (RTX), a chimeric monoclonal antibody against CD20, showed promising results in pediatric steroid-dependent/frequently relapsing FSGS and in post-transplantation recurrence. However, evidence about its role in the FSGS of the adult is still lacking with small case series suggesting conflicting results. In this study we assess the efficacy of RTX in the largest cohort of adults with FSGS currently available in literature. more...

Published
2020

25. Porous media for advanced hybrid thermal energy storages

Author

Lazzarin, R., Mancin, S., Noro, M., and Righetti, G.

Subjects
Metal foam, Hybrid thermal energy storage, Control and Systems Engineering, Paraffin waxes, PCM, Mechanical Engineering, 3-D period structure, Electrical and Electronic Engineering, Condensed Matter Physics
Published
2018

28. Alport syndrome: impact of digenic inheritance in patients management

Author

Fallerini, C, Baldassarri, M, Trevisson, Eva, Morbidoni, Valeria, La Manna, A, Lazzarin, R, Pasini, A, Barbano, G, Pinciaroli, Ar, Garosi, G, Frullanti, E, Pinto, Am, Mencarelli, Ma, Mari, F, Renieri, A, and Ariani, F. . more...

Subjects
Adult, Collagen Type IV, Male, Multifactorial Inheritance, Alport syndrome, digenic inheritance, hypomorphic allele, massive parallel sequencing, hypomorphic allele, massive parallel sequencing, High-Throughput Nucleotide Sequencing, Genetic Counseling, Nephritis, Hereditary, Middle Aged, digenic inheritance, Autoantigens, Risk Assessment, Pedigree, Genes, X-Linked, Mutation, Humans, Female, Alport syndrome, Aged
Abstract

Alport syndrome (ATS) is a genetically heterogeneous nephropathy with considerable phenotypic variability and different transmission patterns, including monogenic (X-linked/autosomal) and digenic inheritance (DI). Here we present a new series of families with DI and we discuss the consequences for genetic counseling and risk assessment. Out of five families harboring variants in more than one COL4 gene detected by next generation sequencing (NGS), minigene-splicing assay allowed us to identify four as true digenic. Two families showed COL4A3/A4 mutations in cis, mimicking an autosomal dominant inheritance with a more severe phenotype and one showed COL4A3/A4 mutations in trans, mimicking an autosomal recessive inheritance with a less severe phenotype. In a fourth family, a de novo mutation (COL4A5) combined with an inherited mutation (COL4A3) triggered a more severe phenotype. A fifth family, predicted digenic on the basis of silico tools, rather showed monogenic X-linked inheritance due to a hypomorphic mutation, in accordance with a milder phenotype. In conclusion, this study highlights the impact of DI in ATS and explains the associated atypical presentations. More complex inheritance should be therefore considered when reviewing prognosis and recurrence risks. On the other side, these findings emphasize the importance to accompany NGS with splicing assays in order to avoid erroneous identification of at risk members. more...

Published
2016

29. Alport syndrome: impact of digenic inheritance in patients management

Author

Fallerini, C., primary, Baldassarri, M., additional, Trevisson, E., additional, Morbidoni, V., additional, La Manna, A., additional, Lazzarin, R., additional, Pasini, A., additional, Barbano, G., additional, Pinciaroli, A.R., additional, Garosi, G., additional, Frullanti, E., additional, Pinto, A.M., additional, Mencarelli, M.A., additional, Mari, F., additional, Renieri, A., additional, and Ariani, F., additional more...

Published
2017
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30. Effect of ozonated water, modified atmosphere packaging and different storage temperature on shelf life of cut lettuce (Lactuca sativa acephala) and arugala (Diplotaxis spp.)

Author

Lazzarin R., Tosini F., Mayr A., Bacci A., Ferasin M., Tonutti P., Galetto A., PROSDOCIMI GIANQUINTO, GIORGIO, Sambo P., Pimpini F., BERTOLINI, PAOLO, Lazzarin R., Tosini F., Mayr A., Bacci A., Bertolini P., Ferasin M., Tonutti P., Galetto A., Gianquinto G., Sambo P., and Pimpini F. more...

Subjects
SHELF LIFE, CUT LETTUCE
Published
2004

43. Double urine circulation:importance of pores

Author

Antonello, A, D'Angelo, Angela, Gambaro, G, Nalesso, F, Capezzi, M, Malagoli, A, Pastori, G, Lazzarin, R, Calo', Lorenzo, and Bonfante, Luciana

Published
2003

50. Extracorporeal dialysis: techniques and adequacy

Author

Passalacqua, S., primary, Staffolani, E., additional, Brescia, P., additional, Loschiavo, C., additional, Mancini, E., additional, Monaci, G., additional, Russo, G. E., additional, Ramunni, A., additional, Granger Vallee, A., additional, Chenine, L., additional, Leray-Moragues, H., additional, Gontier-Picard, A., additional, Rodriguez, A., additional, Chalabi, L., additional, Canaud, B., additional, Lantz, B., additional, Kapke, A., additional, Pearson, J., additional, Vanholder, R., additional, Tomo, T., additional, Robinson, B., additional, Port, F., additional, Daugirdas, J., additional, Ramirez, S., additional, Akonur, A., additional, Agar, B. U., additional, Culleton, B. F., additional, Gellens, M. E., additional, Leypoldt, J. K., additional, Troidle, L., additional, Finkelstein, F. O., additional, Kohn, O. F., additional, Basile, C., additional, Libutti, P., additional, Di Turo, A. L., additional, Casucci, F., additional, Losurdo, N., additional, Teutonico, A., additional, Vernaglione, L., additional, Lomonte, C., additional, Umimoto, K., additional, Nata, Y., additional, Shimamoto, Y., additional, Miyata, M., additional, Krisp, C., additional, Gmerek, A., additional, Wagner, J., additional, Wolters, D., additional, Pedrini, L. A., additional, Kopec, J., additional, Sulowicz, W., additional, Falkenhagen, D., additional, Thijssen, S., additional, Brandl, M., additional, Hartmann, J., additional, Strobl, K., additional, Wallner, M., additional, Mahieu, E., additional, Verhamme, P., additional, Op De Beeck, K., additional, Kuypers, D., additional, Claes, K., additional, Vitale, C., additional, Bagnis, C., additional, Berutti, S., additional, Soragna, G., additional, Gabella, P., additional, Fruttero, C., additional, Marangella, M., additional, Khadzhynov, D., additional, Baumann, C., additional, Lieker, I., additional, Slowinski, T., additional, Neumayer, H.-H., additional, Peters, H., additional, Bibiano, L., additional, Freddi, P., additional, Ricciatti, A., additional, Sagripanti, S., additional, Manarini, G., additional, Frasca, G. M., additional, Hwang, K. S., additional, Park, J.-S., additional, Lee, C. H., additional, Kang, C. M., additional, Kim, G.-H., additional, Urabe, S., additional, Kokubo, K., additional, Tsukao, H., additional, Shimbo, T., additional, Hirose, M., additional, Kobayashi, H., additional, Itoh, Y., additional, Kikuchi, K., additional, Murakami, K., additional, Tsuruta, Y., additional, Niwa, T., additional, Masakane, I., additional, Esashi, S., additional, Igarashi, H., additional, Djogan, M., additional, Boltina, I., additional, Dudar, I., additional, Pastori, G., additional, Favaro, E., additional, Ferraro, A., additional, Marcon, R., additional, Guizzo, M., additional, Lazzarin, R., additional, Conte, F., additional, Nichelatti, M., additional, Limido, A., additional, Zhu, F., additional, Liu, L., additional, Kaysen, G. A., additional, Abbas, S. R., additional, Kotanko, P., additional, Levin, N. W., additional, Debska-Slizien, A., additional, Malgorzewicz, S., additional, Dudziak, M., additional, Rutkowski, B., additional, Svojanovsky, J., additional, Dob ak, P., additional, Nedbalkova, M., additional, Reichertova, A., additional, Soucek, M., additional, Kirmizis, D., additional, Kougioumtzidou, O., additional, Vakianis, P., additional, Papagianni, A., additional, Sestigiani, E., additional, Gissara, Z., additional, Palladino, G., additional, Santoro, A., additional, Schneditz, D., additional, Stockinger, J., additional, Ribitsch, W., additional, Branco, P., additional, Figueiredo, S., additional, Santana, S., additional, Rocha, C., additional, Carvalho, L., additional, Borges, S., additional, Marques, D., additional, Barata, D., additional, Matsuyama, M., additional, Matsuyama, K., additional, Matsuyama, I., additional, Minakuchi, J., additional, Schiffl, H., additional, Fischer, R., additional, Lang, S., additional, de los Santos, C. A., additional, Antonello, I. C., additional, Poli-de-Figueiredo, C. E., additional, d'Avila, D., additional, Rosales, L., additional, Ulloa, D., additional, Carter, M., additional, Umehara, S., additional, Sakai, K., additional, Krieter, D. H., additional, Seidel, S., additional, Merget, K., additional, Lemke, H.-D., additional, Morgenroth, A., additional, Wanner, C., additional, Onogi, T., additional, Nishida, Y., additional, Ueno, J., additional, Taoka, M., additional, Sato, T., additional, Sakurai, K., additional, Saito, T., additional, Yamauchi, F., additional, Asahi, D., additional, Hosoya, H., additional, Maruyama, N., additional, Suzuki, A., additional, Alain, R., additional, Christian, D., additional, Romano, J.-M., additional, Printz, J., additional, Philippe, B., additional, Micha, T., additional, Hadjiyannakos, D., additional, Pani, I., additional, Sonikian, M., additional, Karatzas, I., additional, Vlassopoulos, D., additional, Kanaki, A., additional, Caprioli, R., additional, Lippi, A., additional, Donadio, C., additional, Malliekal, S., additional, Kubey, W., additional, Bernardo, A. A., additional, Katzarski, K., additional, Galach, M., additional, Waniewski, J., additional, Sambale, S., additional, Reising, A., additional, Donnerstag, F., additional, Hafer, C., additional, Schmidt, B., additional, Kielstein, J. T., additional, Ervo, R., additional, Angeletti, S., additional, Turrini Dertenois, L., additional, Cavatorta, F., additional, Gondouin, B., additional, Bevins, A., additional, Cockwell, P., additional, Hutchison, C. A., additional, Doria, M., additional, Genovesi, S., additional, Biagi, F., additional, Grandi, F., additional, Frontini, A., additional, Stella, A., additional, Cases, A., additional, Fort, J., additional, Maduell, F., additional, Comas, J., additional, Arcos, E., additional, Deulofeu, R., additional, Rroji (Molla), M., additional, Seferi, S., additional, Barbullushi, M., additional, Spahia, N., additional, Likaj, E., additional, Thereska, N., additional, Morena, M., additional, Jaussent, I., additional, Bargnoux, A.-S., additional, Dupuy, A.-M., additional, Cristol, J.-P., additional, Hammer, F., additional, Scherberich, J. E., additional, Pizzarelli, F., additional, Ferro, G., additional, Amidone, M., additional, Dattolo, P., additional, Gauly, A., additional, Golla, P., additional, Clajus, C., additional, Beutel, G., additional, Haller, H., additional, Schmidt, B. M. W., additional, Kielstein, J., additional, Nakazawa, R., additional, Shimizu, Y., additional, Uemura, Y., additional, Kashiwabara, H., additional, Watanabe, D., additional, Kato, T., additional, Fuse, M., additional, Azuma, N., additional, Nakanishi, N., additional, Kabayama, S., additional, Alquist Hegbrant, M., additional, Bosch, J. P., additional, Righetti, M., additional, Ferrario, G., additional, Serbelloni, P., additional, Milani, S., additional, Lisi, L., additional, Tommasi, A., additional, Mambelli, E., additional, Bolasco, P. G., additional, Scotto, P., additional, Savoldi, S., additional, Serra, A., additional, Corazza, L., additional, Tomisawa, N., additional, Jinbo, Y., additional, Kobayashi, Y., additional, Kawakubo, Y., additional, and Sakurasawa, T., additional more...

Published
2011
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