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1. Co-Occurrence of Nephronophthisis Type 1 and Alström Syndrome: A Case Report

Author

Rossoni, Lisa, Lugani, Francesca, Orsi, Silvia Maria, Verrina, Enrico Eugenio, Ghiggeri, Gian Marco, Angeletti, Andrea, Caridi, Gianluca, and La Porta, Edoardo

Abstract

We describe the unique case of a patient in whom two ciliopathies with autosomal recessive transmission were clinically and molecularly diagnosed: nephronophthisis type 1 (NPHP1) and Alström syndrome (AS). NPHP1is one of the main genetic causes of terminal kidney failure in childhood. AS is an ultra-rare multi-systemic disease, characterized by progressive kidney disease, hepatic failure, dystrophy of the rods and cones to blindness, slowly progressive neuro-sensory deafness, dilated cardiomyopathy, obesity, insulin resistance/type 2 diabetes mellitus. The coexistence in the same patient of two rare syndromes with overlapping clinical manifestations but genetically different is an eventuality to be considered. This case report would describe the onset and progression of the multi-organ manifestations of both syndromes to highlight that ciliopathies present a strong phenotype overlap but also specific peculiarities. Therefore, to make a correct diagnosis that is essential to achieve the best clinical management could be challenging.

Published
2024
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2. Post-transplant recurrence of steroid resistant nephrotic syndrome in children: the Italian experience

Author

Morello, William, Puvinathan, Sairaj, Puccio, Giuseppe, Ghiggeri, Gian Marco, Dello Strologo, Luca, Peruzzi, Licia, Murer, Luisa, Cioni, Michela, Guzzo, Isabella, Cocchi, Enrico, Benetti, Elisa, Testa, Sara, Ghio, Luciana, Caridi, Gianluca, Cardillo, Massimo, Torelli, Rosanna, and Montini, Giovanni

Abstract

Background: Steroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss. Methods: We identified all children with SRNS who underwent renal transplantation in Italy, between 2005 and 2017. Data were retrospectively collected for the presence of a causative gene mutation, sex, histology, duration of pre-transplant dialysis, age at onset and transplant, HLA matching, recurrence, therapy for recurrence, and graft survival. Results: 101 patients underwent a first and 22 a second renal transplant. After a median follow-up of 58.5 months, the disease recurred on the first renal transplant in 53.3% of patients with a non-genetic and none with a genetic SRNS. Age at transplant >?9 years and the presence of at least one HLA-AB match were independent risk factors for recurrence. Duration of dialysis was longer in children with relapse, but did not reach statistical significance. Overall, 24% of patients lost the first graft, with recurrence representing the commonest cause. Among 22 patients who underwent a second transplant, 5 suffered of SRNS recurrence. SRNS relapsed in 5/9 (55%) patients with disease recurrence in their first transplant and 2 of them lost the second graft. Conclusions: Absence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. A prolonged time spent on dialysis before transplantation has no protective effect on the risk of relapse and should not be encouraged. Retransplantation represents a second chance after graft loss for recurrence.

Published
2024
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4. Rare Single Nucleotide and Copy Number Variants and the Etiology of Congenital Obstructive Uropathy: Implications for Genetic Diagnosis

Author

Ahram, Dina F., Lim, Tze Y., Ke, Juntao, Jin, Gina, Verbitsky, Miguel, Bodria, Monica, Kil, Byum Hee, Chatterjee, Debanjana, Piva, Stacy E., Marasa, Maddalena, Zhang, Jun Y., Cocchi, Enrico, Caridi, Gianluca, Gucev, Zoran, Lozanovski, Vladimir J., Pisani, Isabella, Izzi, Claudia, Savoldi, Gianfranco, Gnutti, Barbara, Capone, Valentina P., Morello, William, Guarino, Stefano, Esposito, Pasquale, Lambert, Sarah, Radhakrishnan, Jai, Appel, Gerald B., Uy, Natalie S., Rao, Maya K., Canetta, Pietro A., Bomback, Andrew S., Nestor, Jordan G., Hays, Thomas, Cohen, David J., Finale, Carolina, Wijk, Joanna A.E. van, La Scola, Claudio, Baraldi, Olga, Tondolo, Francesco, Di Renzo, Dacia, Jamry-Dziurla, Anna, Pezzutto, Alessandro, Manca, Valeria, Mitrotti, Adele, Santoro, Domenico, Conti, Giovanni, Martino, Marida, Giordano, Mario, Gesualdo, Loreto, Zibar, Lada, Masnata, Giuseppe, Bonomini, Mario, Alberti, Daniele, La Manna, Gaetano, Caliskan, Yasar, Ranghino, Andrea, Marzuillo, Pierluigi, Kiryluk, Krzysztof, Krzemień, Grażyna, Miklaszewska, Monika, Lin, Fangming, Montini, Giovanni, Scolari, Francesco, Fiaccadori, Enrico, Arapović, Adela, Saraga, Marijan, McKiernan, James, Alam, Shumyle, Zaniew, Marcin, Szczepańska, Maria, Szmigielska, Agnieszka, Sikora, Przemysław, Drożdż, Dorota, Mizerska-Wasiak, Malgorzata, Mane, Shrikant, Lifton, Richard P., Tasic, Velibor, Latos-Bielenska, Anna, Gharavi, Ali G., Ghiggeri, Gian Marco, Materna-Kiryluk, Anna, Westland, Rik, and Sanna-Cherchi, Simone

Published
2023
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8. Long-Term Efficacy and Safety of Repeated Rituximab to Maintain Remission in Idiopathic Childhood Nephrotic Syndrome: An International Study

Author

Chan, Eugene Yu-hin, Yu, Ellen L.M., Angeletti, Andrea, Arslan, Zainab, Basu, Biswanath, Boyer, Olivia, Chan, Chang-Yien, Colucci, Manuela, Dorval, Guillaume, Dossier, Claire, Drovandi, Stefania, Ghiggeri, Gian Marco, Gipson, Debbie S., Hamada, Riku, Hogan, Julien, Ishikura, Kenji, Kamei, Koichi, Kemper, Markus J., Ma, Alison Lap-tak, Parekh, Rulan S., Radhakrishnan, Seetha, Saini, Priya, Shen, Qian, Sinha, Rajiv, Subun, Chantida, Teo, Sharon, Vivarelli, Marina, Webb, Hazel, Xu, Hong, Yap, Hui Kim, and Tullus, Kjell

Abstract

Children with frequently relapsing, steroid-dependent nephrotic syndrome (FRSDNS) often require multiple courses of rituximab. However, long-term effects from repeated treatments remain unknown. In this international, multicenter study of 346 children receiving 1149 courses of rituximab, the risk of relapse decreased and relapse-free survival significantly improved with repeated treatments. Important side effects, including hypogammaglobulinemia, neutropenia, and infections, were mostly mild, but significant adverse events could occur. The incidence of side effects did not increase with more treatment courses nor a higher cumulative dose of rituximab. These findings suggest that repeating rituximab therapy is an effective and reasonably safe approach for most children with FRSDNS.

Published
2022
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9. Second Wave Antibodies in Autoimmune Renal Diseases: The Case of Lupus Nephritis

Author

Angeletti, Andrea, Bruschi, Maurizio, Moroni, Gabriella, Sinico, Renato Alberto, Franceschini, Franco, Fredi, Micaela, Vaglio, Augusto, Cavagna, Lorenzo, Petretto, Andrea, Pratesi, Federico, Migliorini, Paola, Locatelli, Francesco, Pazzola, Giulia, Pesce, Giampaola, Bagnasco, Marcello, Manfredi, Angelo, Ramirez, Giuseppe A., Esposito, Pasquale, Murdaca, Giuseppe, Negrini, Simone, Cipriani, Leda, Trezzi, Barbara, Emmi, Giacomo, Cavazzana, Ilaria, Binda, Valentina, d’Alessandro, Matteo, Fenaroli, Paride, Pisani, Isabella, Garibotto, Giacomo, Montecucco, Carlomaurizio, Santoro, Domenico, Scolari, Francesco, Volpi, Stefano, Mosca, Marta, Tincani, Angela, Candiano, Giovanni, Prunotto, Marco, Verrina, Enrico, Ravelli, Angelo, and Ghiggeri, Gian Marco

Abstract

Clinical Trial registry name and registration number:Zeus study, NCT02403115

Published
2021
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13. Protective Role of the M-Sec–Tunneling Nanotube System in Podocytes

Author

Barutta, Federica, Kimura, Shunsuke, Hase, Koji, Bellini, Stefania, Corbetta, Beatrice, Corbelli, Alessandro, Fiordaliso, Fabio, Barreca, Antonella, Papotti, Mauro Giulio, Ghiggeri, Gian Marco, Salvidio, Gennaro, Roccatello, Dario, Audrito, Valentina, Deaglio, Silvia, Gambino, Roberto, Bruno, Stefania, Camussi, Giovanni, Martini, Miriam, Hirsch, Emilio, Durazzo, Marilena, Ohno, Hiroshi, and Gruden, Gabriella

Published
2021
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16. Both the rituximab dose and maintenance immunosuppression in steroid-dependent/frequently-relapsing nephrotic syndrome have important effects on outcomes

Author

Chan, Eugene Yu-hin, Webb, Hazel, Yu, Ellen, Ghiggeri, Gian Marco, Kemper, Markus J., Ma, Alison Lap-tak, Yamamura, Tomohiko, Sinha, Aditi, Bagga, Arvind, Hogan, Julien, Dossier, Claire, Vivarelli, Marina, Liu, Isaac Desheng, Kamei, Koichi, Ishikura, Kenji, Saini, Priya, and Tullus, Kjell

Abstract

Rituximab is an effective treatment for steroid-dependent/ frequently-relapsing nephrotic syndrome (SDFRNS) in children. However, the optimal rituximab regimen remains unknown. To help determine this we conducted an international, multicenter retrospective study at 11 tertiary pediatric nephrology centers in Asia, Europe and North America of children 1-18 years of age with complicated SDFRNS receiving rituximab between 2005-2016 for 18 or more months follow-up. The effect of rituximab prescribed at three dosing levels: low (375mg/m2), medium (750mg/m2) and high (1125-1500mg/m2), with or without maintenance immunosuppression (defined as concurrent use of corticosteroids, mycophenolate motile or calcineurin inhibition at first relapse or for at least six months following the rituximab treatment) was examined. Among the 511 children (median age 11.5 year, 67% boys), 191, 208 and 112 received low, medium and high dose rituximab, respectively. Within this total cohort of 511 children, 283 (55%) received maintenance immunosuppression. Renal biopsies were performed in 317 children indicating the predominant histology was minimal change disease (74%). Without maintenance immunosuppression, low-dose rituximab had a shorter relapse-free period and a higher relapse risk (8.5 months) than medium (12.7 months; adjusted hazard ratio, 0.62) and high dose (14.3 months; adjusted hazard ratio, 0.50; all significant). With maintenance immunosuppression, the relapse-free survival in low-dose rituximab (14 months) was similar to medium (10.9 months; adjusted hazard ratio, 1.23) and high dose (12.0 months; adjusted hazard ratio, 0.92; all non-significant). Most adverse events were mild. Thus, children receiving low-dose rituximab without maintenance immunosuppression had the shortest relapse-free survival. Hence, both rituximab dose and maintenance immunosuppression have important effects on the treatment outcomes.

Published
2020
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17. Neutrophil Extracellular Traps Profiles in Patients with Incident Systemic Lupus Erythematosus and Lupus Nephritis

Author

Bruschi, Maurizio, Bonanni, Alice, Petretto, Andrea, Vaglio, Augusto, Pratesi, Federico, Santucci, Laura, Migliorini, Paola, Bertelli, Roberta, Galetti, Maricla, Belletti, Silvana, Cavagna, Lorenzo, Moroni, Gabriella, Franceschini, Franco, Fredi, Micaela, Pazzola, Giulia, Allegri, Landino, Sinico, Renato Alberto, Pesce, Giampaola, Bagnasco, Marcello, Manfredi, Angelo, Ramirez, Giuseppe A., Ramoino, Paola, Bianchini, Paolo, Puppo, Francesco, Pupo, Francesca, Negrini, Simone, Mattana, Federico, Emmi, Giacomo, Garibotto, Giacomo, Santoro, Domenico, Scolari, Francesco, Ravelli, Angelo, Tincani, Angela, Cravedi, Paolo, Volpi, Stefano, Candiano, Giovanni, and Ghiggeri, Gian Marco

Abstract

Objective.Neutrophil extracellular traps (NET) expose modified antigens for autoantibodies in vasculitis. Little is known about levels and removal pathways of NET in systemic lupus erythematosus (SLE), especially in lupus nephritis (LN). We determined circulating levels and defined NET removal in large subsets of patients with incident SLE (iSLE), some of whom had new-onset nephritis.Methods.Serum levels of NET (ELISA), DNase1/DNase1L3 (ELISA), and DNase activity (functional assay) were determined in 216 patients with iSLE [103 had incident LN (iLN)], in 50 patients with other primary glomerulonephritis, and in healthy controls. Ex vivoNET production by neutrophils purified from a random selection of patients was quantified as elastase/DNA release and by immunofluorescence techniques.Results.Serum NET levels were very high in iSLE/iLN compared to all groups of controls and correlated with anti-dsDNA, C3–C4, and proteinuria; iLN had the highest levels. DNase activity was decreased in iLN compared to SLE (20% had one-half DNase activity) despite similar serum levels of DNase1/DNase1L3. In these cases, pretreatment of serum with protein A restored DNase efficiency; 1 patient was homozygous for a c.289_290delAC variant of DNASE1L3. Ex vivoNET production by neutrophils purified from LN, SLE, and normal controls was similar in all cases.Conclusion.Patients with iLN have increased circulating NET and reduced DNase activity, the latter being explained by the presence of inhibitory substances in circulation and/or by rare DNase1L3mutations. Accumulation of NET derives from a multifactorial mechanism, and is associated and may contribute to disease severity in SLE, in particular to renal lesions. (Clinical trial registration: The Zeus study was registered at ClinicalTrials.gov, study number NCT02403115).

Published
2020
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18. Atypical IgM on T cells predict relapse and steroid dependence in idiopathic nephrotic syndrome

Author

Colucci, Manuela, Carsetti, Rita, Rosado, Maria Manuela, Cascioli, Simona, Bruschi, Maurizio, Candiano, Giovanni, Corpetti, Giorgia, Giardino, Laura, Serafinelli, Jessica, Giannone, Chiara, Ghiggeri, Gian Marco, Rastaldi, Maria Pia, Sitia, Roberto, Emma, Francesco, and Vivarelli, Marina

Abstract

The clinical heterogeneity of idiopathic nephrotic syndrome in childhood may reflect different mechanisms of disease that are as yet unclear. Here, we evaluated the association between an atypical presence of IgM on the surface of T cells (T-cell IgM) and the response to steroid therapy in a total of 153 pediatric patients with idiopathic nephrotic syndrome in different phases of disease. At disease onset, T-cell IgM median levels were significantly elevated and predictive of risk of relapse in 47 patients. They were also significantly increased comparing 58 steroid-dependent to 8 infrequently relapsing and 14 frequently relapsing patients, especially during relapse, whereas they were within the normal range in 7 genetic steroid-resistant patients. T-cell IgM in vivowas not affected by the amount of total circulating IgM, nor by concomitant acute infections or oral immunosuppression. However, it was affected by rituximab treatment in 21 steroid-dependent patients. By in vitroexperiments, elevated T-cell IgM was not influenced by total circulating IgM levels or by the presence of other circulating factors, and there was no distinctive antigen-specificity or atypical IgM polymerization. Rather, we found that increased T-cell IgM correlates with reduced IgM sialylation, which influences T-cell response to steroid inhibition and T-cell production of podocyte-damaging factors. Thus, the atypical presence of IgM on the surface of T cells may predispose a subset of steroid-sensitive pediatric patients with idiopathic nephrotic syndrome to a poor response to steroid therapy since disease onset.

Published
2019
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19. Health-related quality of life in glomerular disease

Author

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

Abstract

There is scant literature describing the effect of glomerular disease on health-related quality of life (HRQOL). The Cure Glomerulonephropathy study (CureGN) is an international longitudinal cohort study of children and adults with four primary glomerular diseases (minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy). HRQOL is systematically assessed using items from the Patient-Reported Outcomes Measurement Informative System (PROMIS). We assessed the relationship between HRQOL and demographic and clinical variables in 478 children and 1115 adults at the time of enrollment into CureGN. Domains measured by PROMIS items included global assessments of health, mobility, anxiety, fatigue, and sleep impairment, as well as a derived composite measure incorporating all measured domains. Multivariable models were created that explained 7 to 32% of variance in HRQOL. Patient-reported edema consistently had the strongest and most robust association with each measured domain of HRQOL in multivariable analysis (adjusted β [95% CI] for composite PROMIS score in children, -5.2 [-7.1 to -3.4]; for composite PROMIS score in adults, -6.1 [-7.4 to -4.9]). Female sex, weight (particularly obesity), and estimated glomerular filtration rate were also associated with some, but not all, domains of HRQOL. Primary diagnosis, disease duration, and exposure to immunosuppression were not associated with HRQOL after adjustment. Sensitivity analyses and interaction testing demonstrated no significant association between disease duration or immunosuppression and any measured domain of HRQOL. Thus, patient-reported edema has a consistent negative association with HRQOL in patients with primary glomerular diseases, with substantially greater impact than other demographic and clinical variables.

Published
2019
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20. An international, multi-center study evaluated rituximab therapy in childhood steroid-resistant nephrotic syndrome

Author

Chan, Eugene Yu-hin, Sinha, Aditi, Yu, Ellen L.M., Akhtar, Naureen, Angeletti, Andrea, Bagga, Arvind, Banerjee, Sushmita, Boyer, Olivia, Chan, Chang-Yien, Francis, Anna, Ghiggeri, Gian Marco, Hamada, Riku, Hari, Pankaj, Hooman, Nakysa, Hopf, Luke Sydney, I, Mohamad Ikram, Ijaz, Iftikhar, Ivanov, Dmytro D., Kalra, Suprita, Kang, Hee Gyung, Lucchetti, Laura, Lugani, Francesca, Ma, Alison Lap-tak, Morello, William, Camargo Muñiz, María Dolores, Pradhan, Subal Kumar, Prikhodina, Larisa, Raafat, Reem H., Sinha, Rajiv, Teo, Sharon, Tomari, Kouki, Vivarelli, Marina, Webb, Hazel, Yap, Hui Kim, Yap, Desmond Yat-hin, and Tullus, Kjell

Abstract

The efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS) remains unclear. Therefore, we conducted a retrospective cohort study at 28 pediatric nephrology centers from 19 countries in Asia, Europe, North America and Oceania to evaluate this. Children with SRNS treated with rituximab were analyzed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [6 months or more (CNI-resistant) and under 6 months]. Primary outcome was complete/partial remission (CR/PR) as defined by IPNA/KDIGO guidelines. Secondary outcomes included kidney failure and adverse events. Two-hundred-forty-six children (mean age, 6.9 years; 136 boys; 57% focal segmental glomerulosclerosis, FSGS) were followed a median of 32.4 months after rituximab. All patients were in non-remission before rituximab. (146 and 100 children received CNIs for 6 month or more or under 6 months before rituximab, respectively). In patients with CNI-resistant SRNS, the remission rates (CR/PR) at 3-, 6-, 12- and 24-months were 26% (95% confidence interval 19.3-34.1), 35.6% (28.0-44.0), 35.1% (27.2-43.8) and 39.1% (29.2-49.9), respectively. Twenty-five patients were in PR at 12-months, of which 22 had over 50% reduction in proteinuria from baseline. The remission rates among children treated with CNIs under 6 months before rituximab were 42% (32.3-52.3), 52% (41.8-62.0), 54% (44.3-64.5) and 60% (47.6-71.3) at 3-, 6-, 12-, and 24-months. Upon Kaplan-Meier analysis, non-remission and PR at 12-months after rituximab, compared to CR, were associated with significantly worse kidney survival. Adverse events occurred in 30.5% and most were mild. Thus, rituximab enhances remission in a subset of children with SRNS, is generally safe and CR following rituximab is associated with favorable kidney outcome.

Published
2024
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21. Anti-slit diaphragm antibodies on kidney biopsy identify pediatric patients with steroid-resistant nephrotic syndrome responsive to second-line immunosuppressants

Author

Raglianti, Valentina, Angelotti, Maria Lucia, Cirillo, Luigi, Ravaglia, Fiammetta, Landini, Samuela, Palazzo, Viviana, Melica, Maria Elena, Antonelli, Giulia, Conte, Carolina, Buti, Elisa, Errichiello, Carmela, De Chiara, Letizia, Peired, Anna Julie, Lasagni, Laura, Buccoliero, Anna Maria, Allinovi, Marco, Montero, Anna Manonelles, Cruzado, Josep Maria, Bruschi, Maurizio, Ghiggeri, Gian Marco, Angeletti, Andrea, Anders, Hans-Joachim, Lazzeri, Elena, Mazzinghi, Benedetta, Becherucci, Francesca, and Romagnani, Paola

Abstract

Podocytopathies represent a group of glomerular disorders associated with minimal changes (MC) or focal segmental glomerulosclerosis (FSGS) lesion patterns at biopsy and heterogeneous responses to steroids. Anti-nephrin antibodies were previously found in such patients, suggesting an autoimmune form of podocytopathy. High resolution confocal microscopy on kidney biopsies of a cohort of 128 pediatric patients revealed localization of IgG along the slit diaphragm in 30% of patients with MC and 25% of those with FSGS, but not in other lesion patterns. Anti-nephrin IgG ELISA assay in the serum and stimulated emission depletion microscopy of kidney biopsies showed IgG-nephrin co-localization only in 77.8% of cases. Similar observations were obtained in a cohort of 48 adult patients with MC or FSGS at kidney biopsy, where IgG-nephrin colocalization was only 44.4%, suggesting the existence of autoantibodies binding to other slit proteins. Patients with anti-slit antibodies showed nephrotic syndrome at onset in 94.4% of cases. Patients with primary steroid-resistance had anti-slit antibodies in 27%, while those with secondary steroid-resistance in 87.5% of cases, irrespective of the histopathological lesion pattern. Steroid-resistant patients with anti-slit antibodies responded to second-line immunosuppressants in 92.3% vs. only 20% of patients that were anti-slit negative. No patient with anti-slit antibodies developed kidney failure vs. 51.7% of those negative for antibodies (66.7% with a genetic cause and 41.2% with a non-genetic cause). Thus, the detection of anti-slit antibodies can identify patients with an autoimmune podocytopathy responsive to treatment with second-line immunosuppressants, irrespective of the histopathological lesion pattern at biopsy.

Published
2024
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22. Mouse and human studies support DSTYKloss of function as a low-penetrance and variable expressivity risk factor for congenital urinary tract anomalies

Author

Martino, Jeremiah, Liu, Qingxue, Vukojevic, Katarina, Ke, Juntao, Lim, Tze Y., Khan, Atlas, Gupta, Yask, Perez, Alejandra, Yan, Zonghai, Milo Rasouly, Hila, Vena, Natalie, Lippa, Natalie, Giordano, Jessica L., Saraga, Marijan, Saraga-Babic, Mirna, Westland, Rik, Bodria, Monica, Piaggio, Giorgio, Bendapudi, Pavan K., Iglesias, Alejandro D., Wapner, Ronald J., Tasic, Velibor, Wang, Fan, Ionita-Laza, Iuliana, Ghiggeri, Gian Marco, Kiryluk, Krzysztof, Sampogna, Rosemary V., Mendelsohn, Cathy L., D’Agati, Vivette D., Gharavi, Ali G., and Sanna-Cherchi, Simone

Abstract

Previous work identified rare variants in DSTYKassociated with human congenital anomalies of the kidney and urinary tract (CAKUT). Here, we present a series of mouse and human studies to clarify the association, penetrance, and expressivity of DSTYKvariants.

Published
2023
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23. CureGN Study Rationale, Design, and Methods: Establishing a Large Prospective Observational Study of Glomerular Disease

Author

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

Abstract

Glomerular diseases, including minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and immunoglobulin A (IgA) nephropathy, share clinical presentations, yet result from multiple biological mechanisms. Challenges to identifying underlying mechanisms, biomarkers, and new therapies include the rarity of each diagnosis and slow progression, often requiring decades to measure the effectiveness of interventions to prevent end-stage kidney disease (ESKD) or death.

Published
2019
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24. The copy number variation landscape of congenital anomalies of the kidney and urinary tract

Author

Verbitsky, Miguel, Westland, Rik, Perez, Alejandra, Kiryluk, Krzysztof, Liu, Qingxue, Krithivasan, Priya, Mitrotti, Adele, Fasel, David A., Batourina, Ekaterina, Sampson, Matthew G., Bodria, Monica, Werth, Max, Kao, Charlly, Martino, Jeremiah, Capone, Valentina P., Vivante, Asaf, Shril, Shirlee, Kil, Byum Hee, Marasà, Maddalena, Zhang, Jun Y., Na, Young-Ji, Lim, Tze Y., Ahram, Dina, Weng, Patricia L., Heinzen, Erin L., Carrea, Alba, Piaggio, Giorgio, Gesualdo, Loreto, Manca, Valeria, Masnata, Giuseppe, Gigante, Maddalena, Cusi, Daniele, Izzi, Claudia, Scolari, Francesco, van Wijk, Joanna A. E., Saraga, Marijan, Santoro, Domenico, Conti, Giovanni, Zamboli, Pasquale, White, Hope, Drozdz, Dorota, Zachwieja, Katarzyna, Miklaszewska, Monika, Tkaczyk, Marcin, Tomczyk, Daria, Krakowska, Anna, Sikora, Przemyslaw, Jarmoliński, Tomasz, Borszewska-Kornacka, Maria K., Pawluch, Robert, Szczepanska, Maria, Adamczyk, Piotr, Mizerska-Wasiak, Malgorzata, Krzemien, Grazyna, Szmigielska, Agnieszka, Zaniew, Marcin, Dobson, Mark G., Darlow, John M., Puri, Prem, Barton, David E., Furth, Susan L., Warady, Bradley A., Gucev, Zoran, Lozanovski, Vladimir J., Tasic, Velibor, Pisani, Isabella, Allegri, Landino, Rodas, Lida M., Campistol, Josep M., Jeanpierre, Cécile, Alam, Shumyle, Casale, Pasquale, Wong, Craig S., Lin, Fangming, Miranda, Débora M., Oliveira, Eduardo A., Simões-e-Silva, Ana Cristina, Barasch, Jonathan M., Levy, Brynn, Wu, Nan, Hildebrandt, Friedhelm, Ghiggeri, Gian Marco, Latos-Bielenska, Anna, Materna-Kiryluk, Anna, Zhang, Feng, Hakonarson, Hakon, Papaioannou, Virginia E., Mendelsohn, Cathy L., Gharavi, Ali G., and Sanna-Cherchi, Simone

Abstract

Congenital anomalies of the kidney and urinary tract (CAKUT) are a major cause of pediatric kidney failure. We performed a genome-wide analysis of copy number variants (CNVs) in 2,824 cases and 21,498 controls. Affected individuals carried a significant burden of rare exonic (that is, affecting coding regions) CNVs and were enriched for known genomic disorders (GD). Kidney anomaly (KA) cases were most enriched for exonic CNVs, encompassing GD-CNVs and novel deletions; obstructive uropathy (OU) had a lower CNV burden and an intermediate prevalence of GD-CNVs; and vesicoureteral reflux (VUR) had the fewest GD-CNVs but was enriched for novel exonic CNVs, particularly duplications. Six loci (1q21, 4p16.1-p16.3, 16p11.2, 16p13.11, 17q12 and 22q11.2) accounted for 65% of patients with GD-CNVs. Deletions at 17q12, 4p16.1-p16.3 and 22q11.2 were specific for KA; the 16p11.2 locus showed extensive pleiotropy. Using a multidisciplinary approach, we identified TBX6as a driver for the CAKUT subphenotypes in the 16p11.2 microdeletion syndrome.

Published
2019
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25. The Genetics of Idiopathic Nephrotic Syndrome at the Population Level

Author

Wooden, Benjamin, Jin, Gina, Mitrotti, Adele, Gupta, Yask, Elliott, Mark, Lim, Tze Yin, Canetta, Pietro A., Marasa, Maddalena, Khan, Atlas, Montini, Giovanni, Tasic, Velibor, Giordano, Mario, Scolari, Francesco, Conti, Giovanni, Magistroni, Riccardo, Bomback, Andrew S., Kiryluk, Krzysztof, Fiaccadori, Enrico, Gesualdo, Loreto, Caliskan, Yasar, D'Agati, Vivette D., Ghiggeri, Gian Marco, Gharavi, Ali G., and Sanna-Cherchi, Simone

Published
2023
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30. Microplastics: First Proteomic Analysis on Kidney Tubular Cells

Author

La Porta, Edoardo, Bruschi, Maurizio, Candiano, Giovanni, Artini, Cristina, Verzola, Daniela, Massardo, Sara, Cravedi, Paolo, Esposito, Pasquale, Angeletti, Andrea, Lugani, Francesca, Bigatti, Carolina, Verrina, Enrico E., Ghiggeri, Gian Marco, Alberti, Stefano, and Gentile, Micaela

Published
2023
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32. Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network

Author

Chen, Dhruti P., Helmuth, Margaret E., Smith, Abigail R., Canetta, Pietro A., Ayoub, Isabelle, Mucha, Krzysztof, Kallash, Mahmoud, Kopp, Jeffrey B., Gbadegesin, Rasheed, Gillespie, Brenda W., Greenbaum, Larry A., Parekh, Rulan S., Hunley, Tracy E., Sperati, C. John, Selewski, David T., Kidd, Jason, Chishti, Aftab, Reidy, Kimberly, Mottl, Amy K., Gipson, Debbie S., Srivastava, Tarak, Twombley, Katherine E., Ahn, Wooin, Appel, Gerald, Appelbaum, Paul, Babayev, Revekka, Bomback, Andrew, Chan, Brenda, D’Agati, Vivette Denise, Dogra, Samitri, Fernandez, Hilda, Gharavi, Ali, Hines, William, Husain, Syed Ali, Jain, Namrata, Kiryluk, Krzysztof, Lin, Fangming, Marasa, Maddalena, Markowitz, Glen, Rasouly, Hila Milo, Mohan, Sumit, Mongera, Nicola, Nestor, Jordan, Nickolas, Thomas, Radhakrishnan, Jai, Rao, Maya, Sanna-Cherchi, Simone, Shirazian, Shayan, Stokes, Michael Barry, Uy, Natalie, Valeri, Anthony, Vena, Natalie, Foroncewicz, Bartosz, Moszczuk, Barbara, Perkowska-Ptasińska, Agnieszka, Ghiggeri, Gian Marco, Lugani, Francesca, Ambruzs, Josephine, Liapis, Helen, Baracco, Rossana, Jain, Amrish, Ashoor, Isa, Aviles, Diego, Ahn, Sun-Young, Devarajan, Prasad, Erkan, Elif, Claes, Donna, Stone, Hillarey, Mason, Sherene, Gomez-Mendez, Liliana, Wang, Chia-shi, Yin, Hong, Cai, Yi, Jens, Goebel, Steinke, Julia, Weaver, Donald, Lane, Jerome, Cramer, Carl, Pan, Cindy, Paloian, Neil, Sreedharan, Rajasree, Bowers, Corinna, Dreher, Mary, Mahan, John, Sharpe, Samantha, Smoyer, William, Al-Uzri, Amira, Iragorri, Sandra, Khalid, Myda, Belsha, Craig, Alge, Joseph, Braun, Michael, Gomez, A.C., Wenderfer, Scott, Vasylyeva, Tetyana, Feig, Daniel, Fuentes, Gabriel Cara, Hannah, Melisha, Nester, Carla, Klein, Jon, Katsoufis, Chryso, Seeherunvong, Wacharee, Rheault, Michelle, Wong, Craig, Mathews, Nisha, Barcia, John, Swiatecka-Urban, Agnes, Bartosh, Sharon, Dharnidharka, Vikas, Gaut, Joseph, Laurin, Louis-Philippe, Royal, Virginie, Achanti, Anand, Budisavljevic, Milos, Self, Sally, Ghossein, Cybele, Peleg, Yonatan, Wadhwani, Shikha, Almaani, Salem, Nadasdy, Tibor, Samir, Parikh, Rovin, Brad, Chang, Anthony, Fatima, Huma, Julian, Bruce, Novak, Jan, Renfrow, Matthew, Rizk, Dana, Derebail, Vimal, Falk, Ronald, Gibson, Keisha, Glenn, Dorey, Hogan, Susan, Jain, Koyal, Jennette, J. Charles, Poulton, Caroline, Saha, Manish Kanti, Fogo, Agnes, Sanghani, Neil, Muthusamy, Selvaraj, Schelling, Jeffrey, Hou, Jean, Lemley, Kevin, Mika, Warren, Russo, Pierre, Denburg, Michelle, Kogon, Amy, Meyers, Kevin, Pradhan, Madhura, Matar, Raed Bou, O’Toole, John, Sedor, John, Sethna, Christine, Vento, Suzanne, Atta, Mohamed, Bagnasco, Serena, Neu, Alicia, Adler, Sharon, Dai, Tiane, Dukkipati, Ram, Fervenza, Fernando, Sethi, Sanjeev, Kaskel, Frederick, Brathwaite, Kaye, Weisstuch, Joseph, Wu, Ming, Zhdanova, Olga, Heymann, Jurgen, Waldman, Meryl, Winkler, Cheryl, Tuttle, Katherine, Krissberg, Jill, Lafayette, Richard, Fahmeedah, Kamal, Talley, Elizabeth, Hladunewich, Michelle, Avila-Casado, Carmen, Cattran, Daniel, Heather, Reich, Boll, Philip, Drexler, Yelena, Fornoni, Alessia, Gipson, Patrick, Hodgin, Jeffrey, Oliverio, Andrea, Hogan, Jon, Holzman, Lawrence, Palmer, Matthew, Coppock, Gaia, Abromovitz, Blaise, Mortiz, Michael, Alpers, Charles, Jefferson, J. Ashley, Brown, Elizabeth, Sambandam, Kamal, Roehm, Bethany, Robinson, Bruce, Nast, Cynthia, Barisoni, Laura, Kretzler, Matthias, Mariani, Laura, and Guay-Woodford, Lisa M.

Abstract

Adolescent- and adult-onset minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulonephropathy Network (CureGN) and assessed predictors of rituximab response.

Published
2023
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35. Racial and Ethnic Disparities in Acute Care Utilization Among Patients With Glomerular Disease

Author

Krissberg, Jill R., O’Shaughnessy, Michelle M., Smith, Abigail R., Helmuth, Margaret E., Almaani, Salem, Aviles, Diego H., Brathwaite, Kaye E., Cai, Yi, Cattran, Daniel, Gbadegesin, Rasheed, Glenn, Dorey A., Greenbaum, Larry A., Iragorri, Sandra, Jain, Koyal, Khalid, Myda, Kidd, Jason, Kopp, Jeffrey, Lafayette, Richard, Lane, Jerome C., Lugani, Francesca, Nestor, Jordan G., Parekh, Rulan S., Reidy, Kimberly, Selewski, David T., Sethna, Christine B., Sperati, C. John, Tuttle, Katherine, Twombley, Katherine, Vasylyeva, Tetyana L., Weaver, Donald J., Wenderfer, Scott E., Gibson, Keisha, Ahn, Wooin, Appel, Gerald, Appelbaum, Paul, Babayev, Revekka, Bomback, Andrew, Brown, Eric, Canetta, Pietro, Carlassara, Lucrezia, Chan, Brenda, D’Agati, Vivette Denise, Dogra, Samitri, Fernandez, Hilda, Gharavi, Ali, Hines, William, Husain, Syed Ali, Kiryluk, Krzysztof, Lin, Fangming, Marasa, Maddalena, Markowitz, Glen, Rasouly, Hila Milo, Mohan, Sumit, Mongera, Nicola, Nickolas, Thomas, Radhakrishnan, Jai, Rao, Maya, Sanna-Cherchi, Simone, Shirazian, Shayan, Stokes, Michael Barry, Uy, Natalie, Valeri, Anthony, Vena, Natalie, Foroncewicz, Bartosz, Moszczuk, Barbara, Mucha, Krzysztof, Perkowska-Ptasińska, Agnieszka, Ghiggeri, Gian Marco, Ambruzs, Josephine, Liapis, Helen, Baracco, Rossana, Jain, Amrish, Ashoor, Isa, Srivastava, Tarak, Ahn, Sun-Young, Devarajan, Prasad, Erkan, Elif, Claes, Donna, Stone, Hillarey, Mason, Sherene, Silva, Cynthia, Gomez-Mendez, Liliana, Wang, Chia-shi, Yin, Hong (Julie), Jens, Goebel, Steinke, Julia, Cramer, Carl, Pan, Cindy, Sreedharan, Rajasree, Bowers, Corinna, Dreher, Mary, Kallash, Mahmoud, Mahan, John, Sharpe, Samantha, Smoyer, William, Al-Uzri, Amira, Belsha, Craig, Braun, Michael, Gomez, A.C., Feig, Daniel, Fuentes, Gabriel Cara, Hannah, Melisha, Nester, Carla, Chishti, Aftab, Klein, Jon, Katsoufis, Chryso, Seeherunvong, Wacharee, Rheault, Michelle, Wong, Craig, Mathews, Nisha, Barcia, John, Swiatecka-Urban, Agnes, Bartosh, Sharon, Hunley, Tracy, Dharnidharka, Vikas, Gaut, Joseph, Laurin, Louis-Philippe, Royal, Virginie, Achanti, Anand, Budisavljevic, Milos, Self, Sally, Ghossein, Cybele, Wadhwani, Shikha, Ayoub, Isabelle, Nadasdy, Tibor, Parikh, Samir, Rovin, Brad, Chang, Anthony, Fatima, Huma, Novak, Jan, Renfrow, Matthew, Rizk, Dana, Chen, Dhruti, Derebail, Vimal, Falk, Ronald, Gibson, Keisha, Hogan, Susan, Jain, Koyal, Jennette, J. Charles, Mottl, Amy, Poulton, Caroline, Saha, Manish Kanti, Fogo, Agnes, Sanghani, Neil, Kidd, Jason, Massey, Hugh, Muthusamy, Selvaraj, Ganesan, Santhi, Gonzalez-Vicente, Agustin, Schelling, Jeffrey, Hou, Jean, Lemley, Kevin, Mika, Warren, Russo, Pierre, Denburg, Michelle, Kogon, Amy, Meyers, Kevin, Pradhan, Madhura, Matar, Raed Bou, O’Toole, John, Sedor, John, Bagnasco, Serena, Neu, Alicia, Adler, Sharon, Dai, Tiane, Dukkipati, Ram, Fervenza, Fernando, Sethi, Sanjeev, Kaskel, Frederick, Vento, Suzanne, Weisstuch, Joseph, Wu, Ming, Zhdanova, Olga, Heymann, Jurgen, Waldman, Meryl, Winkler, Cheryl, Hladunewich, Michelle, Avila-Casado, Carmen, Cattran, Daniel, Heather, Reich, Boll, Philip, Drexler, Yelena, Fornoni, Alessia, Gipson, Patrick, Hodgin, Jeffrey, Oliverio, Andrew, Hogan, Jon, Holzman, Lawrence, Palmer, Matthew, Abromovitz, Blaise, Mortiz, Michael, Alpers, Charles, Jefferson, J. Ashley, Brown, Elizabeth, Sambandam, Kamal, Robinson, Bruce, Nast, Cynthia, Barisoni, Laura, Gillespie, Brenda, Gipson, Deb, Hicken, Maggie, Kretzler, Matthias, Mariani, Laura, and Guay-Woodford, Lisa M.

Abstract

The effects of race, ethnicity, socioeconomic status (SES), and disease severity on acute care utilization in patients with glomerular disease are unknown.

Published
2023
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36. Proteomic-based research strategy identified laminin subunit alpha 2 as a potential urinary-specific biomarker for the medullary sponge kidney disease

Author

Fabris, Antonia, Bruschi, Maurizio, Santucci, Laura, Candiano, Giovanni, Granata, Simona, Dalla Gassa, Alessandra, Antonucci, Nadia, Petretto, Andrea, Ghiggeri, Gian Marco, Gambaro, Giovanni, Lupo, Antonio, and Zaza, Gianluigi

Abstract

Medullary sponge kidney (MSK) disease, a rare kidney malformation featuring recurrent renal stones and nephrocalcinosis, continues to be diagnosed using expensive and time-consuming clinical/instrumental tests (mainly urography). Currently, no molecular diagnostic biomarkers are available. To identify such we employed a proteomic-based research strategy utilizing urine from 22 patients with MSK and 22 patients affected by idiopathic calcium nephrolithiasis (ICN) as controls. Notably, two patients with ICN presented cysts. In the discovery phase, the urine of 11 MSK and 10 controls, were randomly selected, processed, and analyzed by mass spectrometry. Subsequently, several statistical algorithms were undertaken to select the most discriminative proteins between the two study groups. ELISA, performed on the entire patients’ cohort, was used to validate the proteomic results. After an initial statistical analysis, 249 and 396 proteins were identified exclusive for ICN and MSK, respectively. A Volcano plot and ROC analysis, performed to restrict the number of MSK-associated proteins, indicated that 328 and 44 proteins, respectively, were specific for MSK. Interestingly, 119 proteins were found to differentiate patients with cysts (all patients with MSK and the two ICN with renal cysts) from ICN without cysts. Eventually, 16 proteins were found to be common to three statistical methods with laminin subunit alpha 2 (LAMA-2) reaching the higher rank by a Support Vector Machine, a binary classification/prediction scheme. ELISA for LAMA-2 validated proteomic results. Thus, using high-throughput technology, our study identified a candidate MSK biomarker possibly employable in future for the early diagnosis of this disease.

Published
2017
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37. Mutational Spectrum of CYP24A1Gene in a Cohort of Italian Patients with Idiopathic Infantile Hypercalcemia

Author

Gigante, Maddalena, Santangelo, Luisa, Diella, Sterpeta, Caridi, Gianluca, Argentiero, Lucia, D''Alessandro, Maria Michela, Martino, Marida, Stea, Emma Diletta, Ardissino, Gianluigi, Carbone, Vincenza, Pepe, Silvana, Scrutinio, Domenico, Maringhini, Silvio, Ghiggeri, Gian Marco, Grandaliano, Giuseppe, Giordano, Mario, and Gesualdo, Loreto

Abstract

Background/Aims:Loss-of-function mutations in the CYP24A1gene, which encodes the vitamin D-24 hydroxylase, have been recognized as a cause of elevated 1,25-dihydroxyvitamin D concentrations, hypercalcemia, hypercalciuria, nephrocalcinosis and nephrolithiasis in infants and adults. As only a case report describing 2 adult patients has been reported in Italian population, we report here the mutation analysis of CYP24A1gene in an Italian cohort of 12 pediatric and adult patients with idiopathic infantile hypercalcemia (IIH). Methods:We performed mutational screening of CYP24A1 gene in a cohort of 12 Italian patients: 8 children with nephrocalcinosis, hypercalcemia and PTH levels <10 pg/ml and 4 adult patients with nephrolithiasis, mild hypercalcemia and PTH levels <10 pg/ml from 11 unrelated Italian families. Clinical and biochemical data were collected. Genomic DNA was extracted from peripheral blood leucocytes using standard methods, and whole coding sequence of CYP24A1 gene was analysed in all patients and family members by polymerase chain reaction and direct sequencing. The potential pathogenicity of the newly identified missense mutations was evaluated by 3 different in silico approaches (Sorting Intolerant from Tolerant, Polyphen and Mutation Taster) and by comparative analysis in 14 different species using ClustalW software. Results:CYP24A1 bi-allelic mutations were found in 8 individuals from 7 Italian families (7/11; 64%). Overall, 6 different CYP24A1 mutations, including one small deletion (p.Glu143del), 4 missense mutations (p.Leu148Pro; p.Arg396Trp; p.Pro503Leu; p.Glu383Gln) and one nonsense mutation (p.Tyr220*) were identified. Two out of 6 mutations (p.Tyr220* and p.Pro503Leu) were not previously described. Moreover, a new CYP24A1variant was identified by genetic screening of asymptomatic controls. Conclusion:To the best of our knowledge, this is the first report of a CYP24A1molecular analysis performed in an Italian cohort of adult and pediatric Italian patients. This study (1) confirms that CYP24A1 plays a causal role in some but not all cases of IIH (64%); (2) expands the spectrum of known CYP24A1pathogenic mutations; (3) describes 2 hotspots detected in 50% of all Italian cases; and (4) emphasizes the importance of recognition and genetic diagnosis of CYP24A1defects in infantile as well as adult hypercalcemia.

Published
2016
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39. Efficacy of Combined Rituximab and Daratumumab Treatment in Post-Transplant Recurrent FSGS

Author

Angeletti, Andrea, Bin, Sofia, Magnasco, Alberto, Bruschi, Maurizio, Cravedi, Paolo, and Ghiggeri, Gian Marco

Abstract

Focal Segmental Glomerulosclerosis (FSGS) is one of the leading causes of kidney failure and it is characterized by an high rate o recurrence after kidney transplant. Moreover, FSGS recurrence is worsened by an increased risk of graft failure. Common therapies of FSGS recurrence mostly consist of plasma exchange treatments, also for prolonged time, and rituximab, with variable efficacy. We report five cases of early FSGS recurrence after kidney transplant, resistant to plasma exchange and rituximab treatment, that subsequently resolved after combined therapy with rituximab and daratumumab. All cases were negative for genetic FSGS. The combined treatment induced a complete response in all the cases and resulted well tolerated. We also performed a comprehensive flow cytometry analysis in two subjects, that may suggest a mechanistic link between plasma cells and disease activity. In conclusion, given the lack of viable treatments for recurrent FSGS, our reports support the rationale for a pilot trial testing the safety/efficacy profile of combined rituximab and daratumumab in post-transplant FSGS recurrence.

Published
2023
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40. Association of COVID-19 Versus COVID-19 Vaccination With Kidney Function and Disease Activity in Primary Glomerular Disease: A Report of the Cure Glomerulonephropathy Study

Author

Wang, Chia-shi, Glenn, Dorey A., Helmuth, Margaret, Smith, Abigail R., Bomback, Andrew S., Canetta, Pietro A., Coppock, Gaia M., Khalid, Myda, Tuttle, Katherine R., Bou-Matar, Raed, Greenbaum, Larry A., Robinson, Bruce M., Holzman, Lawrence B., Smoyer, William E., Rheault, Michelle N., Gipson, Debbie, Mariani, Laura H., Ahn, Wooin, Appel, Gerald, Appelbaum, Paul, Babayev, Revekka, Chan, Brenda, D’Agati, Vivette Denise, Dogra, Samitri, Fernandez, Hilda, Gharavi, Ali, Hines, William, Husain, Syed Ali, Jain, Namrata, Kiryluk, Krzysztof, Lin, Fangming, Marasa, Maddalena, Markowitz, Glen, Rasouly, Hila Milo, Mohan, Sumit, Mongera, Nicola, Nestor, Jordan, Nickolas, Thomas, Radhakrishnan, Jai, Rao, Maya, Sanna-Cherchi, Simone, Shirazian, Shayan, Stokes, Michael Barry, Uy, Natalie, Valeri, Anthony, Vena, Natalie, Foroncewicz, Bartosz, Moszczuk, Barbara, Mucha, Krzysztof, Perkowska-Ptasińska, Agnieszka, Ghiggeri, Gian Marco, Lugani, Francesca, Ambruzs, Josephine, Liapis, Helen, Baracco, Rossana, Jain, Amrish, Ashoor, Isa, Aviles, Diego, Srivastava, Tarak, Ahn, Sun-Young, Devarajan, Prasad, Erkan, Elif, Claes, Donna, Stone, Hillarey, Mason, Sherene, Gbadegesin, Rasheed, Gomez-Mendez, Liliana, Yin, Hong (Julie), Cai, Yi, Jens, Goebel, Steinke, Julia, Weaver, Donald, Lane, Jerome, Cramer, Carl, Pan, Cindy, Paloian, Neil, Sreedharan, Rajasree, Selewski, David, Twombley, Katherine, Bowers, Corinna, Dreher, Mary, Kallash, Mahmoud, Mahan, John, Sharpe, Samantha, Al-Uzri, Amira, Iragorri, Sandra, Belsha, Craig, Alge, Joseph, Braun, Michael, Gomez, A.C., Wenderfer, Scott, Vasylyeva, Tetyana, Feig, Daniel, Fuentes, Gabriel Cara, Hannah, Melisha, Nester, Carla, Chishti, Aftab, Klein, Jon, Katsoufis, Chryso, Seeherunvong, Wacharee, Wong, Craig, Mathews, Nisha, Barcia, John, Swiatecka-Urban, Agnes, Bartosh, Sharon, Hunley, Tracy, Dharnidharka, Vikas, Gaut, Joseph, Laurin, Louis-Philippe, Royal, Virginie, Achanti, Anand, Budisavljevic, Milos, Self, Sally, Ghossein, Cybele, Peleg, Yonatan, Wadhwani, Shikha, Almaani, Salem, Ayoub, Isabelle, Nadasdy, Tibor, Parikh, Samir, Rovin, Brad, Chang, Anthony, Fatima, Huma, Julian, Bruce, Novak, Jan, Renfrow, Matthew, Rizk, Dana, Chen, Dhruti, Derebail, Vimal, Falk, Ronald, Gibson, Keisha, Hogan, Susan, Jain, Koyal, Jennette, J. Charles, Mottl, Amy, Poulton, Caroline, Saha, Manish Kanti, Fogo, Agnes, Sanghani, Neil, Kidd, Jason, Muthusamy, Selvaraj, Hou, Jean, Lemley, Kevin, Mika, Warren, Russo, Pierre, Denburg, Michelle, Kogon, Amy, Meyers, Kevin, Pradhan, Madhura, O’Toole, John, Sedor, John, Sethna, Christine, Vento, Suzanne, Atta, Mohamed, Bagnasco, Serena, Neu, Alicia, Sperati, John, Adler, Sharon, Dai, Tiane, Dukkipati, Ram, Fervenza, Fernando, Sethi, Sanjeev, Kaskel, Frederick, Brathwaite, Kaye, Reidy, Kimberly, Weisstuch, Joseph, Wu, Ming, Zhdanova, Olga, Heymann, Jurgen, Kopp, Jeffrey, Waldman, Meryl, Winkler, Cheryl, Krissberg, Jill, Lafayette, Richard, Fahmeedah, Kamal, Talley, Elizabeth, Hladunewich, Michelle, Parekh, Rulan, Avila-Casado, Carmen, Cattran, Daniel, Heather, Reich, Boll, Philip, Drexler, Yelena, Fornoni, Alessia, Blazius, Brooke, Hodgin, Jeffrey, Oliverio, Andrea, Hogan, Jon, Palmer, Matthew, Abromovitz, Blaise, Mortiz, Michael, Alpers, Charles, Jefferson, J. Ashley, Brown, Elizabeth, Sambandam, Kamal, Roehm, Bethany, Graff, John, Gillespie, Brenda, Kretzler, Matthias, Nast, Cynthia, Barisoni, Laura, and Guay-Woodford, Lisa M.

Abstract

Patients with glomerular disease (GN) may be at increased risk of severe COVID-19, yet concerns over vaccines causing disease relapse may lead to vaccine hesitancy. We examined the associations of COVID-19 with longitudinal kidney function and proteinuria and compared these with similar associations with COVID-19 vaccination.

Published
2023
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41. Age and memory B cells at baseline are associated with risk of relapse and memory B-cell reappearance following anti-CD20 treatment in pediatric frequently-relapsing/steroid-dependent nephrotic syndrome

Author

Colucci, Manuela, Angeletti, Andrea, Zotta, Federica, Carsetti, Rita, Lugani, Francesca, Ravà, Lucilla, Ravani, Pietro, Emma, Francesco, Ghiggeri, Gian Marco, and Vivarelli, Marina

Abstract

B-cell depleting anti-CD20 monoclonal antibodies, mainly rituximab, have proven efficacy in children with frequently-relapsing/steroid-dependent nephrotic syndrome (FR/SDNS). However, drug-free remission is variable and specific baseline markers predictive of relapse after anti-CD20 treatment are still being defined. To clarify these, we performed a bicentric observational study in a large cohort of 102 children and young adults with FR/SDNS treated with anti-CD20 monoclonal antibodies (rituximab and ofatumumab). Sixty-two patients (60.8%) relapsed during a 24-month period (median [interquartile range] relapse-free survival, 14.4 months [7.9-24.0]). A higher risk of relapse was significantly associated with a younger age (under 9.8 years, hazard ratio, 0.44; 95% confidence interval, 0.26-0.74) and with significantly higher circulating levels of memory B cells (1.14; 1.09-1.32) at time of anti-CD20 infusion, independent of time elapsed from onset, previous anti-CD20 treatment, type of administered anti-CD20 monoclonal antibodies, and previous or maintenance oral immunosuppression. Patients younger than 9.8 years at anti-CD20 infusion had a subsequent higher recovery of total, transitional, mature-naïve and memory B-cell subsets independent of previous anti-CD20 treatment and maintenance immunosuppression. Significantly, younger age and higher circulating levels of memory B cells at time of anti-CD20 infusion were also independently associated with the recovery of memory B cells by linear mixed-effects modelling. Thus, both younger age and higher circulating levels of memory B cells at time of infusion are independently associated with a higher risk of relapse and an earlier recovery of memory B cells following anti-CD20 treatment in children with FR/SDNS.

Published
2023
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42. Copy number variation analysis identifies novel CAKUT candidate genes in children with a solitary functioning kidney

Author

Westland, Rik, Verbitsky, Miguel, Vukojevic, Katarina, Perry, Brittany J, Fasel, David A, Zwijnenburg, Petra J G, Bökenkamp, Arend, Gille, Johan J P, Saraga-Babic, Mirna, Ghiggeri, Gian Marco, D'Agati, Vivette D, Schreuder, Michiel F, Gharavi, Ali G, van Wijk, Joanna A E, and Sanna-Cherchi, Simone

Abstract

Copy number variations associate with different developmental phenotypes and represent a major cause of congenital anomalies of the kidney and urinary tract (CAKUT). Because rare pathogenic copy number variations are often large and contain multiple genes, identification of the underlying genetic drivers has proven to be difficult. Here we studied the role of rare copy number variations in 80 patients from the KIMONO study cohort for which pathogenic mutations in three genes commonly implicated in CAKUT were excluded. In total, 13 known or novel genomic imbalances in 11 of 80 patients were absent or extremely rare in 23,362 population controls. To identify the most likely genetic drivers for the CAKUT phenotype underlying these rare copy number variations, we used a systematic in silico approach based on frequency in a large data set of controls, annotation with publicly available databases for developmental diseases, tolerance and haploinsufficiency scores, and gene expression profile in the developing kidney and urinary tract. Five novel candidate genes for CAKUT were identified that showed specific expression in the human and mouse developing urinary tract. Among these genes, DLG1 and KIF12 are likely novel susceptibility genes for CAKUT in humans. Thus, there is a significant role of genomic imbalance in the determination of kidney developmental phenotypes. Additionally, we defined a systematic strategy to identify genetic drivers underlying rare copy number variations.

Published
2015
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43. Copy number variation analysis identifies novel CAKUT candidate genes in children with a solitary functioning kidney

Author

Westland, Rik, Verbitsky, Miguel, Vukojevic, Katarina, Perry, Brittany J., Fasel, David A., Zwijnenburg, Petra J.G., Bökenkamp, Arend, Gille, Johan J.P., Saraga-Babic, Mirna, Ghiggeri, Gian Marco, D'Agati, Vivette D., Schreuder, Michiel F., Gharavi, Ali G., van Wijk, Joanna A.E., and Sanna-Cherchi, Simone

Abstract

Copy number variations associate with different developmental phenotypes and represent a major cause of congenital anomalies of the kidney and urinary tract (CAKUT). Because rare pathogenic copy number variations are often large and contain multiple genes, identification of the underlying genetic drivers has proven to be difficult. Here we studied the role of rare copy number variations in 80 patients from the KIMONO study cohort for which pathogenic mutations in three genes commonly implicated in CAKUT were excluded. In total, 13 known or novel genomic imbalances in 11 of 80 patients were absent or extremely rare in 23,362 population controls. To identify the most likely genetic drivers for the CAKUT phenotype underlying these rare copy number variations, we used a systematic in silicoapproach based on frequency in a large data set of controls, annotation with publicly available databases for developmental diseases, tolerance and haploinsufficiency scores, and gene expression profile in the developing kidney and urinary tract. Five novel candidate genes for CAKUT were identified that showed specific expression in the human and mouse developing urinary tract. Among these genes, DLG1and KIF12are likely novel susceptibility genes for CAKUT in humans. Thus, there is a significant role of genomic imbalance in the determination of kidney developmental phenotypes. Additionally, we defined a systematic strategy to identify genetic drivers underlying rare copy number variations.

Published
2015
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46. Immature renal structures associated with a novel UMOD sequence variant.

Author

Benetti E, Caridi G, Vella MD, Rampoldi L, Ghiggeri GM, Artifoni L, Murer L, Benetti, Elisa, Caridi, Gianluca, Vella, Manuela Della, Rampoldi, Luca, Ghiggeri, Gian Marco, Artifoni, Lina, and Murer, Luisa

Abstract

Mutations of the UMOD gene, encoding uromodulin, have been associated with medullary cystic kidney disease 2, familial juvenile hyperuricemic nephropathy, and glomerulocystic kidney disease. We report on a 13-year-old boy presenting with chronic reduced kidney function, hyperuricemia, and impairment in urine-concentrating ability. His father was affected by an undefined nephropathy that required transplantation. The boy's renal ultrasonography showed reduced bilateral kidney volumes and cortical hyperechogenicity, with 2 tiny cysts in the left kidney. Renal biopsy showed up to 60% of glomeruli featuring an enlargement of Bowman space (glomerular cysts), with mild interstitial fibrosis (alpha-smooth muscle actin [alphaSMA] positive), inflammatory infiltrate, and focal tubular atrophy at the cortical level. At the corticomedullary junction, immature tubules (some dilated) with cytokeratin- and paired box gene 2 (PAX2)-positive immunostaining were seen, surrounded by vimentin-positive mesenchymal tissue. Unlike previously reported cases, no uromodulin-positive globular aggregates within the cytoplasm of tubular cells were observed. Uromodulin urinary excretion was absent. Genetic analysis showed a novel heterozygous sequence change in the UMOD gene (NM_003361.2:c.149G-->C; p.Cys50Ser) involving the first epidermal growth factor-like domain of the protein in both the boy and his father. This novel UMOD sequence variant, which is associated with an immunohistochemical pattern different from previous reports and a histological picture characterized by immature renal structures, suggests a possible role for UMOD in renal development. [ABSTRACT FROM AUTHOR]

Published
2009
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47. Posttransplant Soluble B-Cell Activating Factor Kinetics in Pediatric Recipients of First Kidney Allograft

Author

Comoli, Patrizia, Quartuccio, Giuseppe, Cioni, Michela, Parodi, Angelica, Nocera, Arcangelo, Basso, Sabrina, Fontana, Iris, Magnasco, Alberto, Sioli, Viviana, Guido, Ilaria, Klersy, Catherine, Zecca, Marco, Cardillo, Massimo, Ghiggeri, Gian Marco, and Ginevri, Fabrizio

Abstract

Development of de novo donor-specific antibodies (dnDSA) is associated with late or chronic antibody-mediated rejection (CAMR) and poor graft outcome in low-risk kidney transplant recipients. High-level soluble B-cell activating factor (sBAFF) was observed in kidney recipients at higher risk of developing dnDSA.

Published
2015
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