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2. Contactin 1, a Potential New Antigen Target in Membranous Nephropathy: A Case Report

Author

Domenico Santoro, Pierre Ronco, Hanna Debiec, Elisa Longhitano, Antonella Barreca, Giorgina Barbara Piccoli, Massimo Torreggiani, Elisa Vegezzi, Massimo Russo, Antonio Toscano, and Anna Mazzeo

Subjects
Pathology, medicine.medical_specialty, Proteinuria, medicine.diagnostic_test, biology, business.industry, Autoantibody, medicine.disease, Western blot, Membranous nephropathy, Antigen, Nephrology, Biopsy, medicine, biology.protein, medicine.symptom, Antibody, business, Nephrotic syndrome
Abstract

Several novel antigens have recently been characterized in membranous nephropathy (MN), but those involved in the rare cases of MN associated with inflammatory neuropathies (IN) remain elusive. Although several antibody specificities were identified in the serum, there is no evidence so far for their deposition in glomeruli. We report the case of a 73 y/o woman who was referred because of subacute onset of proximal asymmetric lower limb weakness together with ataxic gait. She was diagnosed with IN. Blood tests showed an eGFR of 73 ml/min/1.73m2, hypoalbuminemia (2.89 g/dl) and proteinuria (3.6 g/24h). Autoantibodies (ANA, ENA, DNA, LAC, anti-cardiolipin, ANCA) were undetectable. Serum Ig and complement levels were normal. A kidney biopsy with electron microscopy examination showed a classical picture of MN. Anti-PLA2R antibody was negative in the serum and PLA2R and THSD7A antigens were not detected in kidney tissue. Anti-contactin1 (CNTN1) antibody was found by ELISA at 1:100 serum dilution and shown to be mostly of IgG4 subclass by Western blot. CNTN1 antigen was co-localized with IgG4 within immune deposits by confocal microscopy. This observation suggests a pathophysiological link between IN and MN. CNTN1 should be considered as a potential candidate antigen involved in MN and tested in PLA2R negative forms associated with IN.

Published
2022

5. Incremental and Personalized Hemodialysis Start: A New Standard of Care

Author

Massimo Torreggiani, Antioco Fois, Antoine Chatrenet, Louise Nielsen, Lurlynis Gendrot, Elisa Longhitano, Léna Lecointre, Claudine Garcia, Conrad Breuer, Béatrice Mazé, Assia Hami, Guillaume Seret, Patrick Saulniers, Pierre Ronco, Frederic Lavainne, and Giorgina Barbara Piccoli

Subjects
Nephrology
Abstract

Incremental hemodialysis (iHD) may attenuate "dialysis shock" and reduce costs, preserving quality of life. It is considered difficult to reconcile with HD wards' routine; fear of underdialysis and increasing mortality are additional concerns. The aim of this study was to evaluate mortality, morbidity, and costs in a large HD ward where iHD is the standard of HD start.This observational study included all incident HD patients in 2017 to 2021, stratified according to HD start: iHD (1-2 sessions/wk), decremental HD (dHD, 3 sessions/wk at start, later reduced), or standard (3 sessions/wk). Results were compared with data recorded in the same unit before the incremental program (2015-2017) and with a propensity score-matched cohort from the French Renal Epidemiology and Information Network (REIN) registry.A total of 158 patients started HD in 2017 to 2021, 57.6% on iHD, 8.9% dHD, and 33.5% standard HD schedule. Patients on the standard schedule had lower initial estimated glomerular filtration rate (eGFR) (5 vs. 7 ml/min per 1.72 mOur study reveals that iHD can be a new standard of care, as it is safe and feasible in up to two-thirds of patients on incident HD.

Published
2022

6. Improving Clinical Trials for Anticomplement Therapies in Complement-Mediated Glomerulopathies: Report of a Scientific Workshop Sponsored by the National Kidney Foundation

Author

Minnie M. Sarwal, Nicole Arceneaux, Jonathan Barratt, Mark D. Stegall, Christine B. Sethna, Debbie S. Gipson, Shadab Ali, Richard J.H. Smith, Matthew C. Pickering, Lindsey Fuller, Brad H. Rovin, Ashley Frazer-Abel, Carla M. Nester, Rasheed Gbadegesin, Fernando C. Fervenza, Gerald B. Appel, Samir V. Parikh, Daniel C. Cattran, Véronique Frémeaux-Bacchi, Cathie Spino, Sanjay Ram, Pierre Ronco, Laura Bailey-Wickins, Paolo Cravedi, Jonathan J. Hogan, Joshua M. Thurman, Laurence H. Beck, John D. Mahan, Marina Vivarelli, Isa Ashoor, Michelle N. Rheault, Elif Erkan, David L. Feldman, Richard J. Quigg, Richard A. Lafayette, Peter S. Heeger, Andrew S. Bomback, Moglie le Quintrec-Donnette, Michelle A. Hladunewich, Christoph Licht, Krzysztof Kiryluk, and Howard Trachtman

Subjects
medicine.medical_specialty, business.industry, Clinical study design, MEDLINE, Foundation (evidence), Complement (complexity), Clinical trial, Nephrology, medicine, Patient representatives, Intensive care medicine, Risk assessment, business, Therapeutic strategy
Abstract

Blocking the complement system as a therapeutic strategy has been proposed for numerous glomerular diseases but presents a myriad of questions and challenges, not the least of which is demonstrating efficacy and safety. In light of these potential issues and because there are an increasing number of anti-complement therapy trials either planned or underway, the National Kidney Foundation (NKF) facilitated an all-virtual format scientific workshop entitled, "Improving Clinical Trials for Anti-complement Therapies in Complement-mediated Glomerulopathies." Attended by patient representatives and experts in glomerular diseases, complement physiology, and clinical trial design, the aim of this workshop was to develop standards applicable for designing and conducting clinical trials for anti-complement therapies across a wide spectrum of complement-mediated glomerulopathies. Discussions focused on study design, subject risk assessment and mitigation, laboratory measurements and biomarkers to support these studies, and identification of optimal outcome measures to detect benefit, specifically for trials in complement-mediated diseases. This report summarizes the discussions from this workshop and outlines consensus recommendations.

Published
2022

7. International Society of Nephrology first consensus guidance for preclinical animal studies in translational nephrology

Author

Masaomi Nangaku, A. Richard Kitching, Peter Boor, Alessia Fornoni, Jürgen Floege, P. Toby Coates, Jonathan Himmelfarb, Rachel Lennon, Hans-Joachim Anders, Benjamin D. Humphreys, Fergus J. Caskey, Agnes B. Fogo, Andrea Angeletti, Patricia W. Bedard, Ariela Benigni, Anna Björnson Granqvist, Vera Certikova Chabova, Christos Chatziantoniou, Jennifer Cross, Sandrine Damster, Jo-Ann Donner, Frank Eitner, Stanislas Faguer, Antonio Fontanella, Yuri Fujimoto, Joseph Gaut, Leslie Gewin, Pernille B.L. Hansen, John Cijiang He, Jeremy Hughes, Reiko Inagi, Celia Jenkinson, Vivekanand Jha, Mikio Kato, Darren Kelly, Jeffrey Kopp, Ron Korstanje, Romaldas Mačiulaitis, Patrick B. Mark, Hans-Peter Marti, Stephen P. McAdoo, Jeffrey H. Miner, Alberto Ortiz, Samir M. Parikh, Ambra Pozzi, Paola Romagnani, Pierre Ronco, Brad H. Rovin, Julio Saez-Rodriguez, Moin A. Saleem, John A. Sayer, Stuart Shankland, Andrey S. Shaw, Yusuke Suzuki, Tomoko Takano, Sydney Tang, Rene Tolba, John Viel, Yoshihisa Yamada, Motoko Yanagita, Takashi Yokoo, Nobuya Yoshida, Darren Yuen, Roy Zent, and Aihua Zhang

Subjects
Nephrology
Published
2023

9. The role of PLA2R antibody monitoring: what we know and what we do not know

Author

Pierre Ronco, Emmanuelle Plaisier, and Hanna Debiec

Subjects
Transplantation, Nephrology
Abstract

For a long time, kidney biopsy was the only diagnostic means for membranous nephropathy (MN) and proteinuria and serum creatinine were the only markers of disease activity. The discovery of the phospholipase A2 receptor (PLA2R) antibody in 2009 has induced a paradigm shift in both the diagnosis and monitoring of patients. Two serological tests are routinely used: the enzyme-linked immunosorbent assay (ELISA), which is quantitative, and the immunofluorescence assay (IFA), which is more sensitive. In centres where the two assays are available, the recommendation is to use IFA for screening and diagnosis of immunological remission and ELISA for monitoring the effectiveness of therapy. In patients with positive PLA2R antibody serology, normal kidney function and no evidence of an underlying disease, a kidney biopsy is not mandatory given the almost 100% specificity of the assays. Because MN has different phases, one cannot base a clinical or therapeutic decision on a single measurement of PLA2R antibody at baseline. Risk evaluation of disease progression is a dynamic process that should be performed repeatedly to capture the trajectory of the disease based on both the traditional biomarkers (proteinuria and serum creatinine) and PLA2R antibody levels. The effectiveness of therapy is also evaluated on the PLA2R antibody trajectory, particularly during the first 6 months. Finally, PLA2R antibody monitoring has transformed the management of patients with kidney allografts. Future studies are needed to develop more subtle immunological tests, including monitoring of antigen-specific memory B cells.

Published
2021

10. Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases

Author

Frank Bridoux, David Jayne, Yusuke Suzuki, Amy Earley, Kelly A. Burdge, Marina Vivarelli, Zhihong Liu, Jai Radhakrishnan, Adrian Liew, Keisha L. Gibson, Marcello Tonelli, Richard J. Glassock, Sydney C.W. Tang, Sharon G. Adler, Jan-Stephan F. Sanders, H. Terence Cook, Jonathan C. Craig, Vladimir Tesar, Sanjeev Sethi, Jonathan Barratt, Pierre Ronco, Elizabeth M. Rave, Michael Cheung, Brad H. Rovin, David J. Tunnicliffe, Carla M. Nester, Juan M. Mejia-Vilet, Vivekanand Jha, Martin Howell, Fernando C. Fervenza, Tak Mao Chan, Lyubov Lytvyn, Jürgen Floege, Jack F.M. Wetzels, Heather N. Reich, Groningen Kidney Center (GKC), Groningen Institute for Organ Transplantation (GIOT), Jayne, David [0000-0002-1712-0637], and Apollo - University of Cambridge Repository

Subjects
Adult, medicine.medical_specialty, Evidence-based practice, Kidney, Glomerulonephritis, Membranous, anti-GBM, Nephropathy, IgA vasculitis, Glomerulonephritis, Focal segmental glomerulosclerosis, systematic review, Membranous nephropathy, evidence-based, medicine, Humans, complement, Minimal change disease, C3, Child, Intensive care medicine, glomerular diseases, infection-related glomerulonephritis, KDIGO, lupus nephritis, ANCA, nephrotic syndrome, business.industry, MPGN, Nephrosis, Lipoid, membranous nephropathy, AAV, Glomerulonephritis, IGA, IgA nephropathy, Guideline, medicine.disease, FSGS, minimal change disease, Systematic review, Nephrology, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business, guideline
Abstract

Kidney international 100(4), 753-779 (2021). doi:10.1016/j.kint.2021.05.015, Published by Elsevier, New York, NY

Published
2021

12. A Fresh Perspective on Monoclonal Gammopathies of Renal Significance

Author

Pierre Ronco, Pierre Aucouturier, Vivette D. D'Agati, Centre de Recherche Saint-Antoine (CR Saint-Antoine), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service d'immunologie et hématologies biologiques [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Columbia University Medical Center (CUMC), Columbia University [New York], Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Aucouturier, Pierre, Centre de Recherche Saint-Antoine (CRSA), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)

Subjects
[SDV.IMM] Life Sciences [q-bio]/Immunology, Immunoglobulin Isotypes, 030232 urology & nephrology, Review, 030204 cardiovascular system & hematology, Bioinformatics, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, glomerulopathy, 03 medical and health sciences, 0302 clinical medicine, Medicine, immunoglobulin isotypes, immunoglobulin variable regions, business.industry, monoclonal gammopathy, monoclonalgammopathy, IgG3, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, monotypic deposits, 3. Good health, Monoclonal gammopathy, PGNMID, Nephrology, Monoclonal, [SDV.IMM]Life Sciences [q-bio]/Immunology, Treatment strategy, medicine.symptom, business, Clone (B-cell biology)
Abstract

International audience; Monoclonal gammopathies of renal significance (MGRS) encompass a remarkable variety of kidney diseases that result from intrinsic nephrotoxic properties of certain monoclonal Igs or their subunits. Effective disease-modifying treatments rely on the targeting of a malignant B-cell clone that may be demonstrable but often is quite hypothetical. Hence, convincing arguments for the genuine monoclonal character of the causative mono-isotypic Ig tissue deposits is needed for design of appropriate treatment strategies. The purpose of this article was to critically analyze distinct situations of suspected MGRS that occur in the practice of pathologists, nephrologists, hematologists, and immunologists. A particular focus of interest is the group of conditions known as proliferative glomerulonephritis with mono-isotypic immunoglobulin deposits (PGNMIDs), which illustrates the difficulties and ambiguities surrounding a definitive assignment of MGRS status.

Published
2021

13. The Exostosin Immunohistochemical Status Differentiates Lupus Membranous Nephropathy Subsets With Different Outcomes

Author

Xavier Belenfant, Philippe Rouvier, Pierre Ronco, Isabelle Brocheriou, Emmanuel Esteve, Michèle Saïdi, David Buob, Tim Ulinski, Sophie Tuffet, Hanna Debiec, Zahir Amoura, Makoto Miyara, Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Service d'Anatomie et cytologie pathologiques [CHU Pitié-Salpêtrière] (ACP), CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie et Dialyses [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Unité de Recherche Clinique de l’Est Parisien [CHU Saint-Antoine] (URC-EST), Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts (CHNO)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de médecine interne et d'immunologie clinique [CHU Pitié-Salpêtrière], Centre Hospitalier Intercommunal André Grégoire [Montreuil] (CHI André Gregoire), Service de néphrologie et pédiatrie générale [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Service d'Anatomie et cytologie pathologiques = Service de Pathologie [CHU Pitié-Salpêtrière] (ACP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Département de Néphrologie = Service de Néphrologie et Dialyses [CHU Tenon], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Direction de la Recherche Clinique et de l'Innovation [AP-HP] (DRCI), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière] (DMIIC), CHU Trousseau [APHP], HAL-SU, Gestionnaire, and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP]

Subjects
Pathology, medicine.medical_specialty, 030232 urology & nephrology, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 03 medical and health sciences, 0302 clinical medicine, renal biopsy, systemic lupus erythematosus, Membranous nephropathy, Research Letter, medicine, skin and connective tissue diseases, 030304 developmental biology, 0303 health sciences, Systemic lupus erythematosus, medicine.diagnostic_test, business.industry, membranous nephropathy, medicine.disease, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 3. Good health, [SDV.IMM.IA]Life Sciences [q-bio]/Immunology/Adaptive immunology, Nephrology, [SDV.IMM.IA] Life Sciences [q-bio]/Immunology/Adaptive immunology, exostosin, immunohistochemistry, Immunohistochemistry, Renal biopsy, business
Abstract

International audience; Exostosin (EXT) 1 and EXT2 proteins are recently discovered major components of the subepithelial deposits in a subset of patients with membranous nephropathy (MN).1,2 Although the detection of EXT1/2 seems to be a distinctive feature of MN related to systemic lupus erythematosus (SLE),1 the exact frequency of EXT-positive, SLE-associated MN (SLE-MN) and its potential clinical significance are not well determined.Here, we retrospectively evaluated 86 consecutive patients with biopsy-proven pure class 5 SLE-MN diagnosed between January 2010 and December 2018 in 2 nephropathology centers (La Pitié Salpêtrière and Tenon hospitals) in Paris, France; mixed classes (3 + 5 and 4 + 5 of the 2003 ISN/Renal Pathology Society classification)3 were excluded.

Published
2021

14. Immune response to SARS-CoV-2 infection and vaccination in patients receiving kidney replacement therapy

Author

T. Alp Ikizler, Pierre Ronco, Brad H. Rovin, and P. Toby Coates

Subjects
Male, 0301 basic medicine, COVID-19 Vaccines, medicine.medical_treatment, 030232 urology & nephrology, 03 medical and health sciences, 0302 clinical medicine, Immune system, Renal Dialysis, Immunity, medicine, Humans, Renal replacement therapy, Pandemics, Letter to the Editor, Kidney transplantation, Dialysis, Aged, SARS-CoV-2, business.industry, Immunogenicity, Vaccination, Antibody titer, COVID-19, Middle Aged, medicine.disease, Kidney Transplantation, Renal Replacement Therapy, Outcome and Process Assessment, Health Care, 030104 developmental biology, Nephrology, Immunology, Commentary, Female, business
Abstract

In this issue of Kidney International, the initial experience regarding the immunogenicity of prior coronavirus disease 2019 (COVID-19) infection and the response to the COVID-19 vaccines among patients on maintenance dialysis and kidney transplant recipients is summarized. Preliminary data suggest that there is durability of immune response after COVID-19 infection. Although immune response to the first dose of vaccine is less in infection-naïve patients than healthy individuals in both groups, after the second vaccine dose a significant portion of patients receiving maintenance dialysis develop robust antibody titers, whereas kidney transplant recipients show a less-strong immune response.

Published
2021

16. Time to Abandon Kidney Biopsy to Diagnose Membranous Nephropathy?

Author

Emmanuelle Plaisier and Pierre Ronco

Subjects
Nephrology, Transplantation, Kidney, medicine.medical_specialty, Pathology, biology, medicine.diagnostic_test, Epidemiology, business.industry, Editorials, Glomerulosclerosis, Disease, Critical Care and Intensive Care Medicine, medicine.disease, Serology, medicine.anatomical_structure, Membranous nephropathy, Internal medicine, Biopsy, medicine, biology.protein, Antibody, business
Abstract

The discovery of phospholipase A2 receptor (PLA2R) antibody by Beck and associates ([1][1]) has been transformative for the diagnosis of membranous nephropathy and for the monitoring of patients affected with the disease. Less than 2 years after this discovery, serologic tests were developed: first

Published
2021

17. When contactin antibodies hit the podocyte: a new neurorenal syndrome

Author

Hanna Debiec and Pierre Ronco

Subjects
Pathology, medicine.medical_specialty, Kidney, biology, Podocytes, business.industry, Cell adhesion molecule, Disease, medicine.disease, Glomerulonephritis, Membranous, Antibodies, Podocyte, medicine.anatomical_structure, Membranous nephropathy, Antigen, Contactins, Nephrology, biology.protein, Humans, Medicine, In patient, Antibody, business
Abstract

Membranous nephropathy can be associated with various etiologies and antigens. In this issue of Kidney International, Le Quintrec et al. described contactin-1 cell adhesion molecule as a novel target antigen shared by the peripheral nerve and podocyte in patients with neurological disease and membranous nephropathy.

Published
2021

18. HLA-D and PLA2R1 risk alleles associate with recurrent primary membranous nephropathy in kidney transplant recipients

Author

Ann M. Moyer, Petra Mrázová, Guillaume Canaud, Jack F.M. Wetzels, Nadhir Yousfi, Maryvonne Hourmant, Christophe Legendre, Manish J. Gandhi, Vladimir Tesar, Mariam P. Alexander, Ondrej Viklický, Christiane Mousson, Hanna Debiec, Pierre Ronco, Philippe Rieu, Valérie Dubois, Anne-Els van de Logt, Paul Brenchley, Sylvain Guibert, Vincent Vuiblet, Charlène Levi, Patrick Hamilton, Armando Torres, Bruno Moulin, Eric Letouzé, Josep M. Cruzado, José Aurelio Ballarín Castan, Lena Berchtold, Gabriel Choukroun, Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Geneva University Hospital (HUG), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université de Paris (UP), Mayo Clinic [Rochester], Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), Service Néphrologie et transplantation rénale Adultes [CHU Necker], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Radboud University Medical Center [Nijmegen], University of Manchester [Manchester], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Biospectroscopie Translationnelle - EA 7506 (BIOSPECT), Université de Reims Champagne-Ardenne (URCA), Centre Hospitalier Universitaire de Reims (CHU Reims), Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), Etablissement français du sang - Auvergne-Rhône-Alpes (EFS), Institut d'Investigació Biomèdica de Bellvitge [Barcelone] (IDIBELL), University of Barcelona, Universidad de La Laguna [Tenerife - SP] (ULL), Charles University [Prague] (CU), Institute for Clinical and Experimental Medicine (IKEM), Centre hospitalier universitaire de Nantes (CHU Nantes), Immuno-Rhumatologie Moléculaire, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Strasbourg (UNISTRA), Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg (UNISTRA), Matrice extracellulaire et dynamique cellulaire - UMR 7369 (MEDyC), Université de Reims Champagne-Ardenne (URCA)-SFR CAP Santé (Champagne-Ardenne Picardie Santé), Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Centre National de la Recherche Scientifique (CNRS), CHU Amiens-Picardie, Département de Néphrologie [CHU Necker], Service de néphrologie et de transplantation rénale [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Gestionnaire, Hal Sorbonne Université, Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Mécanismes physiopathologiques et conséquences des calcifications vasculaires - UR UPJV 7517 (MP3CV), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'Urgences néphrologiques et transplantation rénale [CHU Tenon], CHU Tenon [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)

Subjects
0301 basic medicine, Oncology, medicine.medical_specialty, Linkage disequilibrium, recurrence, HLA-D, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Locus (genetics), Single-nucleotide polymorphism, Glomerulonephritis, Membranous, Polymorphism, Single Nucleotide, genetic risk score, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Internal medicine, medicine, Humans, genetics, PLA2R1, Alleles, Retrospective Studies, Genetic testing, next generation sequencing, medicine.diagnostic_test, business.industry, Donor selection, Receptors, Phospholipase A2, membranous nephropathy, Retrospective cohort study, medicine.disease, Kidney Transplantation, 3. Good health, [SDV] Life Sciences [q-bio], Transplantation, 030104 developmental biology, Nephrology, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business, transplantation
Abstract

Contains fulltext : 234032.pdf (Publisher’s version ) (Closed access) Recurrence of primary membranous nephropathy after transplantation occurs in up to 44% of patients and is driven by PLA2R antibody. Here, we asked whether genetic determinants could improve risk prediction. First, we sequenced PLA2R1 and HLA-D loci in 248 patients with primary membranous nephropathy and identified two independent single nucleotide polymorphisms (SNPs) at risk for primary membranous nephropathy at each locus. These were rs9271188 (intergenic between HLA-DRB1 and HLA-DQA1,) and rs9275086 (intergenic between HLA-DQB1 and HLA-DQA2) at the HLA-D locus along with rs6726925 and rs13018963 at the PLA2R1 locus. Then we investigated whether primary membranous nephropathy at-risk variants were associated with recurrence in a retrospective cohort of 105 donor-recipient pairs and a replication cohort of 40 pairs. Seven SNPs located between HLA-DRB1 and HLA-DQA1 in linkage disequilibrium with rs9271188, and three SNPs in the PLA2R1 region predicted recurrence when presented by the donor, but not when presented by the recipient. The two SNPs in the HLA-D region most strongly associated with recurrence (rs9271705 and rs9271550) were confirmed in the replication cohort. A genetic risk score based on the two best predictors at each locus (rs9271705, rs9271550, rs17830558, and rs3828323) identified a group of patients with high risk of recurrence. Thus, our results suggest that the graft contributes to recurrence of primary membranous nephropathy through the disease susceptibility HLA-D and PLA2R1 SNPs in an autoimmune milieu. Further studies are needed before implementation of genetic testing for these in donor selection.

Published
2021

19. The clinicopathologic spectrum of segmental membranous glomerulopathy

Author

Pietro A. Canetta, M. Barry Stokes, Glen S. Markowitz, Satoru Kudose, Dominick Santoriello, Vivette D. D'Agati, Hanna Debiec, Andrew S. Bomback, Pierre Ronco, and Ibrahim Batal

Subjects
0301 basic medicine, medicine.medical_specialty, Nephrotic Syndrome, Biopsy, medicine.medical_treatment, 030232 urology & nephrology, Renal function, Immunofluorescence, Glomerulonephritis, Membranous, Stain, Gastroenterology, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Internal medicine, medicine, Humans, Stage (cooking), Retrospective Studies, Creatinine, medicine.diagnostic_test, business.industry, Immunosuppression, medicine.disease, Staining, 030104 developmental biology, chemistry, Nephrology, business, Nephrotic syndrome, Immunosuppressive Agents
Abstract

Membranous glomerulopathy (MGN) is characterized by global subepithelial immune deposits that stain most intensely by immunofluorescence for IgG. Here we describe the clinical and pathologic findings in a cohort of patients with MGN in which, by definition, only segmental immune deposits are present. This rare variant, termed segmental MGN (sMGN), is poorly characterized. We retrospectively identified all patients with sMGN diagnosed at Columbia University from January 2010 to October 2018, excluding those with systemic lupus erythematosus. Data on presenting features, pathologic findings, and outcomes were collected. Fifty cases of sMGN were identified, representing 2.5% of MGN. In 21 of 50 biopsies, there was an alternative, predominant disease process. The remaining 29 patients with isolated sMGN had a median creatinine of 0.97 mg/dl, median 24-hour urine protein 3.1 g/day, and 32% had nephrotic syndrome. Staining for NELL-1 (a protein kinase C binding protein) was positive in five of 17 cases. Staining for PLA2R, THSD7A, and exostosin 1 (autoantigens in primary MGN) was negative in all biopsies evaluated. Ultrastructural evaluation revealed predominantly early stage sMGN (stage 1 or 1-2 in 14/29). Follow-up was available for 21 of the 29 patients with isolated sMGN (median 12 months), including seven who received immunosuppression (primarily glucocorticoids). During follow-up, 86% had stable/improved kidney function and 45% achieved complete while 15% achieved partial remission. Among the 15 patients with isolated sMGN without full nephrotic syndrome, only two received immunosuppression; nonetheless, 50% achieved complete while 21% achieved partial remission. Thus, sMGN is a rare PLA2R-negative variant of MGN with 29% NELL-1 positivity and favorable prognosis, even in the absence of immunosuppressive treatment.

Published
2021

20. Semaphorin 3B–associated membranous nephropathy is a distinct type of disease predominantly present in pediatric patients

Author

David Buob, Hanna Debiec, Francesco Emma, Cheryl L. Tran, Fernando C. Fervenza, M. Cristine Charlesworth, Aishwarya Ravindran, Tim Ulinski, Benjamin J. Madden, Francesca Diomedi-Camassei, Sanjeev Sethi, Marina Vivarelli, Pierre Ronco, and Lou Ann Gross

Subjects
0301 basic medicine, Immunoglobulin A, Pathology, medicine.medical_specialty, 030232 urology & nephrology, Semaphorins, Glomerulonephritis, Membranous, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Semaphorin, Glomerular Basement Membrane, Biopsy, Humans, Medicine, Child, Frozen section procedure, Membrane Glycoproteins, Microscopy, Confocal, biology, medicine.diagnostic_test, business.industry, Glomerular basement membrane, medicine.disease, Immunohistochemistry, 3. Good health, 030104 developmental biology, medicine.anatomical_structure, Nephrology, Immunoglobulin M, biology.protein, business
Abstract

Membranous nephropathy results from subepithelial antigen-antibody complex deposition along the glomerular basement membrane. Although PLA2R, THSD7A, and NELL-1 account for a majority (about 80%) of the target antigens, the target antigen in the remaining cases is not known. Using laser microdissection of PLA2R-negative glomeruli of patients with membranous nephropathy followed by mass spectrometry we identified a unique protein, Semaphorin 3B, in three cases. Mass spectrometry failed to detect Semaphorin-3B in 23 PLA2R-associated cases of membranous nephropathy and 88 controls. Semaphorin 3B in all three cases was localized to granular deposits along the glomerular basement membrane by immunohistochemistry. Next, an additional eight cases of Semaphorin 3B-associated membranous nephropathy were identified in three validation cohorts by immunofluorescence microscopy. In four of 11 cases, kidney biopsy also showed tubular basement membrane deposits of IgG on frozen sections. Confocal microscopy showed that both IgG and Semaphorin 3B co-localized to the glomerular basement membrane. Western blot analysis of five available sera showed reactivity to reduced Semaphorin 3B in four of four patients with active disease and no reactivity in one patient in clinical remission; there was also no reactivity in control sera. Eight of the 11 cases of Semaphorin 3B-associated membranous nephropathy were pediatric cases. Furthermore, in five cases, the disease started at or below the age of two. Thus, Semaphorin 3B-associated membranous nephropathy appears to be a distinct type of disease; more likely to be present in pediatric patients.

Published
2020

21. A challenge to the kidney community by a man-made crisis

Author

P. Toby Coates, Germaine Wong, Brad H. Rovin, Pierre Ronco, Brad Rovin, Olivier Devuyst, Tilman B. Drueke, Iain Drummond, Jurgen Floege, Agnes B. Fogo, T. Alp Ikizler, Krzysztof Kiryluk, Masaomi Nangaku, Jai Radhakrishnan, Christina Wyatt, Jun Xia, Pat Morrissey, Susan Small, and Christine Burgos-Clavel

Subjects
Male, Nephrology, Renal Dialysis, Humans, Kidney Failure, Chronic, Female, Kidney
Published
2022

22. Nomenclature for kidney function and disease: report of a Kidney Disease

Author

Caroline Vinck, Andrew S. Levey, Mark Lambie, Josephine P. Briggs, Andrew Davenport, Susan J. Allison, Stacy L. Christiansen, Shari E. Leventhal, Stephen B. Walsh, Joseph Laycock, Jonathan S. Bromberg, Paul M. Palevsky, Denis Fouque, Brenda R. Hemmelgarn, Jennifer St. Clair Russell, Lesley A. Inker, Pascale H. Lane, Michael Cheung, Rajnish Mehrotra, Detlef Bockenhauer, Adeera Levin, Matthias Kretzler, Mark A. Perazella, Ron T. Gansevoort, Kai-Uwe Eckardt, Lesley Rees, Daniel E. Weiner, John S. Gill, Harold I. Feldman, Julie R. Ingelfinger, Allison Tong, Michael Mittelman, Michel Jadoul, Pierre Ronco, Nijsje M. Dorman, Marlies Ostermann, Patricia Morrissey, Wolfgang C. Winkelmayer, Franz Schaefer, Eddie L. Greene, Ewout J. Hoorn, School of Metallurgy and Materials, University of Birmingham [Birmingham], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), California Institute of Technology (CALTECH), Service de Néphrologie et Dialyses [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU), UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service de néphrologie, Cardiovascular Centre (CVC), Groningen Kidney Center (GKC), and Internal Medicine

Subjects
0301 basic medicine, medicine.medical_specialty, Glossary, kidney disease, precision medicine, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Renal function, Disease, Q1, 03 medical and health sciences, 0302 clinical medicine, kidney measures, medicine, patient-centeredness, Intensive care medicine, kidney function, Kidney, acute kidney diseases and disorders, business.industry, urogenital system, Acute kidney injury, Precision medicine, medicine.disease, R1, 3. Good health, kidney failure, 030104 developmental biology, medicine.anatomical_structure, acute kidney injury, Nephrology, Albuminuria, nomenclature, medicine.symptom, business, chronic kidney disease, Kidney disease
Abstract

The worldwide burden of kidney disease is rising, but public awareness remains limited, underscoring the need for more effective communication by stakeholders in the kidney health community. Despite this need for clarity, the nomenclature for describing kidney function and disease lacks uniformity. In June 2019, Kidney Disease: Improving Global Outcomes (KDIGO) convened a Consensus Conference with the goal of standardizing and refining the nomenclature used in the English language to describe kidney function and disease, and of developing a glossary that could be used in scientific publications. Guiding principles of the conference were that the revised nomenclature should be patient-centered, precise, and consistent with nomenclature used in the KDIGO guidelines. Conference attendees reached general consensus on the following recommendations: (i) to use "kidney" rather than "renal" or "nephro-" when referring to kidney disease and kidney function; (ii) to use "kidney failure" with appropriate descriptions of presence or absence of symptoms, signs, and treatment, rather than "end-stage kidney disease"; (iii) to use the KDIGO definition and classification of acute kidney diseases and disorders (AKD) and acute kidney injury (AKI), rather than alternative descriptions, to define and classify severity of AKD and AKI; (iv) to use the KDIGO definition and classification of chronic kidney disease (CKD) rather than alternative descriptions to define and classify severity of CKD; and (v) to use specific kidney measures, such as albuminuria or decreased glomerular filtration rate (GFR), rather than "abnormal" or "reduced" kidney function to describe alterations in kidney structure and function. A proposed 5-part glossary contains specific items for which there was general agreement. Conference attendees acknowledged limitations of the recommendations and glossary, but they considered standardization of scientific nomenclature to be essential for improving communication.

Published
2020

23. Proteomic Analysis of Complement Proteins in Membranous Nephropathy

Author

Hanna Debiec, Daniel C. Cattran, Richard J.H. Smith, Benjamin J. Madden, Aishwarya Ravindran, Sanjeev Sethi, Amit Sethi, Rishi Sharma, M. Cristine Charlesworth, Fernando C. Fervenza, and Pierre Ronco

Subjects
Clusterin, biology, business.industry, 030232 urology & nephrology, CD59, 030204 cardiovascular system & hematology, medicine.disease, Proteomics, lcsh:Diseases of the genitourinary system. Urology, lcsh:RC870-923, Molecular biology, Complement system, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Nephrology, medicine, biology.protein, Alternative complement pathway, Properdin, Vitronectin, business
Abstract

Introduction: Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in Caucasian adults. Phospholipase A2 receptor (PLA2R)– and exostosin 1 (EXT1)/exostosin 2 (EXT2)–associated MN represent the most common primary and secondary forms of MN. The complement profile using a proteomics approach has not been studied in these 2 common forms of MN. Methods: We used laser microdissection and mass spectrometry (MS/MS) to dissect glomeruli and identify glomerular complement proteins in PLA2R-associated (n = 7), EXT1/EXT2-associated MN (n = 21), and 11 control cases (time 0 transplant biopsies). Results: MS/MS identified high total spectral counts for PLA2R and EXT1/EXT2 in corresponding cases of PLA2R- and EXT1/EXT2-positive MN. Both PLA2R- and EXT1/EXT2-associated MN had high spectral counts of complement proteins C3, C4, C5, C6, C7, C8, and C9. Complement protein C1 was present in low spectral counts in EXT1/EXT2-associated MN. Regulators of complement activation that were detected in MN included higher spectral counts of FH, FHR-1, FHR-5, clusterin, vitronectin and lower spectral counts of FHR-3, FHR-4, and CD59. Low spectral counts of FB and properdin, key components of the alternative pathway, also were detected. IgG4 and IgG1 were the most abundant IgG subclasses in PLA2R- and EXT1/EXT2-associated MN. Lower spectral counts for C3, C4, and C5 were detected in control cases when compared with MN. Conclusion: Significant complement activation is present in MN as evidenced by large spectral counts of complement proteins from C3- and C4-based pathways, including regulatory proteins of complement pathways. These data suggest that anticomplement drugs may be effective in treatment for MN. Keywords: complement, laser microdissection, mass spectrometry, membranous nephropathy

Published
2020

24. Development of a Standardized Chemiluminescence Immunoassay for the Detection of Autoantibodies Against Human M-Type Phospholipase A2 Receptor in Primary Membranous Nephropathy

Author

Iva Gunnarsson, Hanna Debiec, Cornelia Dähnrich, Sandra Saschenbrecker, Wolfgang Schlumberger, Pierre Ronco, Karolinska Institutet [Stockholm], Karolinska University Hospital [Stockholm], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, and Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)

Subjects
anti-PLA2R, Chemiluminescence immunoassay, 030232 urology & nephrology, 030204 cardiovascular system & hematology, lcsh:RC870-923, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, law.invention, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Clinical Research, law, parasitic diseases, medicine, Indirect immunofluorescence, phospholipase A2 receptor, business.industry, Antibody titer, Autoantibody, membranous nephropathy, medicine.disease, lcsh:Diseases of the genitourinary system. Urology, Molecular biology, 3. Good health, chemiluminescence immunoassay, Fully automated, Nephrology, Recombinant DNA, [SDV.IMM]Life Sciences [q-bio]/Immunology, business, Phospholipase A2 receptor, autoantibody
Abstract

Introduction Autoantibodies against the M-type phospholipase A2 receptor (PLA2R) are important markers in the diagnosis and monitoring of primary membranous nephropathy (pMN). For the detection of anti-PLA2R autoantibodies, a standardized recombinant cell-based indirect immunofluorescence assay (RC-IFA) and enzyme-linked immunosorbent assay (ELISA) are widely used, the former providing higher sensitivity but lacking a finely graduated quantification of antibody titers. In this study, we evaluated the diagnostic performance characteristics of a novel standardized chemiluminescence immunoassay (ChLIA) by comparison with the established anti-PLA2R test systems. Methods Sera from 155 patients with biopsy-proven pMN and 154 disease controls were analyzed for autoantibodies against PLA2R by the novel ChLIA as well as by ELISA and RC-IFA. Results The clinical sensitivity of the ChLIA (83.9%) was higher compared with ELISA (73.5%) and equaled that of RC-IFA (83.2%), at similar specificities (≥99.4%). Among ELISA-negative pMN samples, ChLIA and RC-IFA yielded positive results in 39.0% and 36.6%, respectively. The qualitative agreement amounted to 94.5% (ChLIA vs. ELISA) and 99.4% (ChLIA vs. RC-IFA). Conclusion The novel anti-PLA2R ChLIA outperforms the ELISA in detecting patients with pMN and demonstrates almost perfect agreement with RC-IFA. It thus presents a promising alternative tool for accurate anti-PLA2R testing, with the advantage of rapid turnaround times and fully automated random-access processing., Graphical abstract

Published
2020

25. Mapping the T cell epitopes of the M-type transmembrane phospholipase A2 receptor in primary membranous nephropathy

Author

Xiao-dan Zhang, Cai-xia Lin, Zhao Cui, Qiu-hua Gu, Bing-jia Yan, Lei Liu, Wen-chao Song, Yi Shi, Hanna Debiec, Pierre Ronco, and Ming-hui Zhao

Subjects
Nephrology
Abstract

The M-type phospholipase A2 receptor (PLA2R) is the major autoantigen of primary membranous nephropathy (MN). Despite many studies on B-cell epitopes recognized by antibodies, little is known about T-cell epitopes. Herein, we synthesized 123 linear peptides, each consisting of 15-22 amino acids with 8-12 amino acid overlaps, across ten domains of PLA2R. Their binding capacity to risk (DRB1*1501, DRB1*0301) and protective (DRB1*0901, DRB1*0701) HLA molecules was then assessed by flow cytometry. Proliferation of CD4+ T cells from patients with anti-PLA2R positive MN was analyzed after peptide stimulation. Cytokines produced by activated peripheral blood mononuclear cells were measured by cytometric bead arrays. We identified 17 PLA2R peptides that bound to both DRB1*1501 and DRB1*0301 molecules with high capacity. Some of these peptides showed decreased binding to heterozygous DRB1*1501/0901 and DRB1*0301/0701. Ten of the 17 peptides (CysR1, CysR10, CysR12, FnII-3, CTLD3-9, CTLD3-10, CTLD3-11, CTLD5-2-1, CTLD7-1 and CTLD7-2) induced significant proliferation of CD4+ T cells from patients with MN than cells from healthy individuals. Upon activation by these peptides, peripheral blood mononuclear cells from patients with MN produced higher levels of pro-inflammatory cytokines, predominantly IL-6, TNF-α, IL-10, IL-9 and IL-17. Thus, we mapped and identified ten peptides in the CysR, FnII, CTLD3, CTLD5, CTLD7 domains of PLA2R as potential T-cell epitopes of MN. These findings are a first step towards developing peptide-specific immunotherapies.

Published
2021

26. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases

Author

Carla M. Nester, Adrian Liew, Jürgen Floege, Elizabeth M. Rave, Sharon G. Adler, Kelly A. Burdge, Richard J. Glassock, Sydney C.W. Tang, Vivekanand Jha, Brad H. Rovin, Pierre Ronco, Jai Radhakrishnan, Jan-Stephan F. Sanders, Jonathan Barratt, Yusuke Suzuki, David Jayne, Sanjeev Sethi, Zhihong Liu, Juan M. Mejia-Vilet, Keisha L. Gibson, Heather N. Reich, Fernando C. Fervenza, Tak Mao Chan, Marina Vivarelli, H. Terence Cook, Vladimir Tesar, Jack F.M. Wetzels, Frank Bridoux, Apollo - University of Cambridge Repository, Rovin, Brad H., Adler, Sharon G., Jayne, David R. W., Jha, Vivekanand, Liew, Adrian, Liu, Zhi-Hong, Mejía-Vilet, Juan Manuel, Nester, Carla M., Radhakrishnan, Jai, Rave, Elizabeth M., Reich, Heather N., Ronco, Pierre, Barratt, Jonathan, Sanders, Jan-Stephan F., Sethi, Sanjeev, Suzuki, Yusuke, Tang, Sydney C. W., Tesar, Vladimir, Vivarelli, Marina, Wetzels, Jack F. M., Floege, Jürgen, Bridoux, Frank, Burdge, Kelly A., Chan, Tak Mao, Cook, H. Terence, Fervenza, Fernando C., Gibson, Keisha L., and Glassock, Richard J.

Subjects
medicine.medical_specialty, business.industry, 1103 Clinical Sciences, Guideline, Urology & Nephrology, Clinical Practice, Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group, Nephrology, Medicine, Humans, Kidney Diseases, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], Renal Insufficiency, Chronic, business, Intensive care medicine, Glomerular diseases, Glomerular Filtration Rate
Abstract

Kidney international 100(4, Supplement) S1-S276 (2021). doi:10.1016/j.kint.2021.05.021 special issue: "KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases", Published by Elsevier, New York, NY

Published
2021
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27. Recurrence of Anti-Semaphorin 3B-Mediated Membranous Nephropathy after Kidney Transplantation

Author

Marc Fila, Hanna Debiec, Hélène Perrochia, Nabila Djouadi, Marie-Christine Verpont, David Buob, and Pierre Ronco

Subjects
Adult, Male, Receptors, Phospholipase A2, General Medicine, Semaphorins, Glomerulonephritis, Membranous, Kidney Transplantation, Autoimmune Diseases, Nephrology, Recurrence, Rapid Communications, Humans, Female, Child, Rituximab
Abstract

BACKGROUND: Membranous nephropathy (MN) is rare in pediatric patients, although its diagnosis may be underestimated in children who are responsive to corticosteroid therapy prescribed for a suspicion of minimal change disease. It is most often associated with an autoimmune disease, predominantly lupus. We previously reported the occurrence of early-onset MN associated with semaphorin 3B in nine children and two adults. METHODS: Biopsies were performed on native kidney and at 1 and 5 months after transplantation. Semaphorin 3B antigen was detected in immune deposits by immunohistochemistry and confocal microscopy on paraffin-embedded biopsies. Anti-semaphorin antibodies were detected by Western blot and analyzed sequentially. RESULTS: We report the first case of early recurrence after transplantation in a 7-year-old boy who presented with severe nephrotic syndrome and advanced kidney failure. There was no evidence of hereditary or associated autoimmune disease. Abundant, almost coalescent deposits were seen by electron microscopy and bright granular, subepithelial staining was observed for semaphorin 3B antigen. Western blot analysis of serum revealed anti-semaphorin 3B antibodies. Recurrence of MN occurred 25 days after transplantation and manifested as nephrotic range proteinuria despite conventional immunosuppressive therapy. Kidney biopsies confirmed histologic MN recurrence with colocalization of semaphorin 3B antigen and IgG. The patient was treated with rituximab. Anti-semaphorin 3B antibodies, which were detected at transplantation, were not detected 40 days after rituximab. CONCLUSION: This case provides evidence that anti-semaphorin 3B antibodies are pathogenic and should be monitored in patients with MN.

Published
2021

28. Antenatal Membranous Nephropathy and Type 2 (Axonal) Charcot-Marie-Tooth With Mutations in the Metallo-Membrane Endopeptidase Gene: A Call for Family Screening and Pharmacovigilance

Author

Hanna Debiec, Gauthier Remiche, Marina Vivarelli, Nicolette C. Notermans, Jeroen Nauta, Guilhem Solé, Paul Delrée, Pierre Ronco, Joëlle Nortier, Daria Diodato, Pediatrics, Centre Hospitalier Universitaire Brugmann [Bruxelles] (CHU), Hôpital Erasme [Bruxelles] (ULB), Faculté de Médecine [Bruxelles] (ULB), Université libre de Bruxelles (ULB)-Université libre de Bruxelles (ULB), Institut de Pathologie et Génétique [Gosselies] (I.P.G.), Eramus MC-Sophia Children’s Hospital, Partenaires INRAE, University Medical Center [Utrecht], Bambino Gesù Children’s Hospital [Rome, Italy], Hôpital Pellegrin, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), and Gestionnaire, HAL Sorbonne Université 5

Subjects
peripheral neuropathy, Pharmacology, neprilysin, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Pharmacovigilance, Research Letter, medicine, 030212 general & internal medicine, Neprilysin, Gene, ComputingMilieux_MISCELLANEOUS, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, business.industry, maternofetal allo-immunization, MME gene, membranous nephropathy, Généralités, medicine.disease, congenital nephropathy, neutral endopeptidase, Endopeptidase, 3. Good health, Peripheral neuropathy, Nephrology, business, 030217 neurology & neurosurgery, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

SCOPUS: ar.j, info:eu-repo/semantics/published

Published
2021

29. High-Dose Rituximab and Early Remission in PLA2R1-Related Membranous Nephropathy

Author

Hanna Debiec, Christelle Zaghrini, Alessandra Rosenthal, Michel Ticchioni, Karine Dahan, Gérard Lambeau, Alexandra Rousseau, Barbara Seitz-Polski, Vincent L.M. Esnault, Marine Andreani, Ghislaine Bernard, Pierre Ronco, Sylvia Benzaken, Departement d'immunologie, hôpital de l'Archet, CHU de Nice, Département de Néphrologie - Hôpital Pasteur [Nice], Hôpital Pasteur [Nice] (CHU), Institut de pharmacologie moléculaire et cellulaire (IPMC), Centre National de la Recherche Scientifique (CNRS)-Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Université Côte d'Azur (UCA), Service de Néphrologie et Dialyses [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Sorbonne Université (SU), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Département d'Immunologie, Centre Hospitalier Universitaire de Nice (CHU de Nice)-Groupe hospitalier l'Archet (Nice), Laboratoire Immunologie [Nice], Hôpital de l'Archet-Université Côte d'Azur (UCA)-Université Nice Sophia Antipolis (... - 2019) (UNS), and COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)

Subjects
Male, medicine.medical_specialty, Epidemiology, 030232 urology & nephrology, Nice, Early remission, Critical Care and Intensive Care Medicine, Glomerulonephritis, Membranous, Gastroenterology, Cohort Studies, Epitopes, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Interquartile range, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Internal medicine, medicine, Immunologic Factors, Humans, Prospective Studies, ComputingMilieux_MISCELLANEOUS, Aged, 030304 developmental biology, computer.programming_language, Epitope spreading, 0303 health sciences, Transplantation, business.industry, Receptors, Phospholipase A2, Remission Induction, Editorials, Original Articles, Middle Aged, 16. Peace & justice, medicine.disease, 3. Good health, Nephrology, Cohort, Female, Rituximab, business, computer, medicine.drug, Cohort study
Abstract

BACKGROUND AND OBJECTIVES: Different rituximab protocols are used to treat membranous nephropathy. We compared two rituximab protocols in patients with membranous nephropathy. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Twenty-eight participants from the NICE cohort received two infusions of 1-g rituximab at 2-week intervals, whereas 27 participants from the Prospective Randomized Multicentric Open Label Study to Evaluate Rituximab Treatment for Membranous Nephropathy (GEMRITUX) cohort received two infusions of 375 mg/m(2) at 1-week interval. We measured serum rituximab levels and compared remission at month 6 and before any treatment modification and analyzed factors associated with remission and relapses. RESULTS: Remissions occurred in 18 (64%) versus eight (30%) from the NICE and GEMRITUX cohort (P=0.02) at month 6, respectively, and in 24 (86%) versus 18 (67%) participants (P=0.12) before treatment modification, respectively. Median time to remission was 3 [interquartile range (IQR), 3–9] and 9 [IQR, 6–12] months for NICE and GEMRITUX cohorts respectively (P=0.01). Participants from the NICE cohort had higher circulating level of rituximab and lower CD19 counts (3.3 µg/L [IQR, 0.0–10.8] versus 0.0 [IQR, 0.0–0.0] P

Published
2019

30. Prevalence of tubulopathy and association with renal function loss in HIV-infected patients

Author

Emmanuelle Plaisier, Marie-Gisèle Lebrette, Pierre Ronco, Emmanuel Esteve, Jacqueline Capeau, J.-B. Guiard-Schmid, Soraya Fellahi, Anne-Line Eme, Gilles Pialoux, François-Xavier Lescure, Dominique Costagliola, Jean-Philippe Bastard, Service des Maladies Infectieuses et Tropicales [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Services de Maladies Infectieuses et Tropicales [CHU Bichat], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Infection, Anti-microbiens, Modélisation, Evolution (IAME (UMR_S_1137 / U1137)), Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Sorbonne Paris Nord, Université Paris Diderot - Paris 7 (UPD7), Université Sorbonne Paris Cité (USPC), Service de biochimie et hormonologie [CHU Tenon], Centre de Recherche Saint-Antoine (CR Saint-Antoine), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP]-Sorbonne Université (SU), Sorbonne Université (SU), Service des maladies infectieuses et tropicales [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP]-Sorbonne Université (SU), Service de Néphrologie et Dialyses [CHU Tenon], Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris 13 (UP13)-Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, microalbuminuria, Anti-HIV Agents, Population, proximal tubule dysfunction, 030232 urology & nephrology, Renal function, HIV Infections, urologic and male genital diseases, GFR, 03 medical and health sciences, 0302 clinical medicine, Tubulopathy, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Internal medicine, Prevalence, medicine, Humans, Prospective Studies, Renal Insufficiency, Chronic, Tenofovir, 10. No inequality, education, Transplantation, education.field_of_study, Proteinuria, business.industry, Incidence (epidemiology), Editorials, HIV, Odds ratio, Middle Aged, medicine.disease, 030112 virology, 3. Good health, Kidney Tubules, Nephrology, ethnicity, Female, Microalbuminuria, France, medicine.symptom, business, Biomarkers, Glomerular Filtration Rate, Kidney disease
Abstract

Background The incidence of chronic kidney disease (CKD) is 10 times higher in human immunodeficiency virus (HIV)-infected patients than in the general population. We explored the prevalence and determinants of proximal tubular dysfunction (PTD) in HIV-infected individuals, and assessed the impact of the tubulopathy on the estimated glomerular filtration rate (eGFR) outcome. Methods A cohort study was performed on 694 outpatients followed in a French centre to analyse the prevalence of PTD, the diagnosis performance of screening tools and the associated factors. eGFR was prospectively evaluated to analyse the predictive value of the tubulopathy on eGFR decrease. Results At inclusion, 14% of the patients presented with PTD and 5% with CKD. No individual tubular marker, including non-glomerular proteinuria, glycosuria dipstick or hypophosphataemia, registered sufficient performance to identify PTD. We found a significant interaction between tenofovir disoproxil fumarate exposure and ethnicity (P = 0.03) for tubulopathy risk. Tenofovir disoproxil fumarate exposure was associated with PTD in non-Africans [adjusted odds ratio (aOR) = 4.71, P Conclusion PTD is not rare in HIV-infected individuals but is less frequent in sub-Saharan African patients and is associated with tenofovir disoproxil fumarate exposure only in non-Africans. Its diagnosis requires multiple biochemical testing and it is not associated with an accelerated eGFR decline.

Published
2019

31. Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference

Author

Brad H. Rovin, Dawn J. Caster, Daniel C. Cattran, Keisha L. Gibson, Jonathan J. Hogan, Marcus J. Moeller, Dario Roccatello, Michael Cheung, David C. Wheeler, Wolfgang C. Winkelmayer, Jürgen Floege, Sharon G. Adler, Charles E. Alpers, Isabelle Ayoub, Arvind Bagga, Sean J. Barbour, Jonathan Barratt, Daniel T.M. Chan, Anthony Chang, Jason Chon Jun Choo, H. Terence Cook, Rosanna Coppo, Fernando C. Fervenza, Agnes B. Fogo, Jonathan G. Fox, Richard J. Glassock, David Harris, Elisabeth M. Hodson, Elion Hoxha, Kunitoshi Iseki, J. Charles Jennette, Vivekanand Jha, David W. Johnson, Shinya Kaname, Ritsuko Katafuchi, A. Richard Kitching, Richard A. Lafayette, Philip K.T. Li, Adrian Liew, Jicheng Lv, Ana Malvar, Shoichi Maruyama, Juan Manuel Mejía-Vilet, Chi Chiu Mok, Patrick H. Nachman, Carla M. Nester, Eisei Noiri, Michelle M. O'Shaughnessy, Seza Özen, Samir M. Parikh, Hyeong-Cheon Park, Chen Au Peh, William F. Pendergraft, Matthew C. Pickering, Evangéline Pillebout, Jai Radhakrishnan, Manish Rathi, Pierre Ronco, William E. Smoyer, Sydney C.W. Tang, Vladimír Tesař, Joshua M. Thurman, Hernán Trimarchi, Marina Vivarelli, Giles D. Walters, Angela Yee-Moon Wang, Scott E. Wenderfer, Jack F.M. Wetzels, Baylor College of Medicine (BCM), Baylor University, Division of Nephrology, Rheinisch-Westfälische Technische Hochschule Aachen (RWTH), Department of Pediatrics, Division of Pediatric Nephrology and Genetics, All India Institute of Medical Sciences, University of British Columbia (UBC), Science of Turin Health Agency [Turin, Italy] (City of the Health), Regina Margherita University Children's Hospital [Turin, Italy], Mayo Clinic [Rochester], University College of London [London] (UCL), Molecular Otolaryngology and Renal Research Laboratories [Iowa City, IA, USA] (Carver College of Medicine), University of Iowa [Iowa City]-Carver College of Medicine, University of Iowa, Centre for Complement and Inflammation Research [London, UK] (Department of Medicine), Imperial College London, Service de rhumatologie, CHU Bordeaux [Bordeaux], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), University of Colorado [Denver], Department of Medicine, The University of Hong Kong (HKU), Department of Nephrology [Nijmegen, The Netherlands], and Radboud University Medical Centre [Nijmegen, The Netherlands]

Subjects
0301 basic medicine, Nephrology, medicine.medical_specialty, membranoproliferative glomerulonephritis, Consensus Development Conferences as Topic, 030232 urology & nephrology, Lupus nephritis, Paraproteinemias, Context (language use), Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, anti-neutrophil cytoplasmic antibody–associated vasculitis, 03 medical and health sciences, 0302 clinical medicine, Glomerulonephritis, Risk Factors, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Internal medicine, Membranoproliferative glomerulonephritis, medicine, Humans, Minimal change disease, C3 glomerulopathy, Genetic Testing, Intensive care medicine, ComputingMilieux_MISCELLANEOUS, KDIGO, lupus nephritis, business.industry, Podocytes, Nephrosis, Lipoid, monoclonal gammopathies of renal significance, Guideline, medicine.disease, focal and segmental glomerulosclerosis, 3. Good health, minimal change disease, 030104 developmental biology, Treatment Outcome, Practice Guidelines as Topic, Disease Progression, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business, Biomarkers, Kidney disease, Glomerular Filtration Rate
Abstract

Contains fulltext : 203024.pdf (Publisher’s version ) (Open Access) In November 2017, the Kidney Disease: Improving Global Outcomes (KDIGO) initiative brought a diverse panel of experts in glomerular diseases together to discuss the 2012 KDIGO glomerulonephritis guideline in the context of new developments and insights that had occurred over the years since its publication. During this KDIGO Controversies Conference on Glomerular Diseases, the group examined data on disease pathogenesis, biomarkers, and treatments to identify areas of consensus and areas of controversy. This report summarizes the discussions on primary podocytopathies, lupus nephritis, anti-neutrophil cytoplasmic antibody-associated nephritis, complement-mediated kidney diseases, and monoclonal gammopathies of renal significance.

Published
2019

32. HLA class II alleles differing by a single amino acid associate with clinical phenotype and outcome in patients with primary membranous nephropathy

Author

Hanna Debiec, Pierre Ronco, Li-qiang Meng, Ming-Hui Zhao, Fang Wang, Hong Zhang, Zhao Cui, Xu-yang Cheng, Li-jie Zhang, Yi-miao Zhang, Gang Liu, Zhen Qu, Zhi-yong Pei, Huai-yu Wang, Xu-jie Zhou, Jing Huang, Fang-jin Chen, Xin Wang, Li-jun Xie, Laboratoire Traitement et Communication de l'Information (LTCI), Télécom ParisTech-Institut Mines-Télécom [Paris] (IMT)-Centre National de la Recherche Scientifique (CNRS), China Agriculture University [Beijing], Centre de Géosciences (GEOSCIENCES), MINES ParisTech - École nationale supérieure des mines de Paris, Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL), China Agricultural University (CAU), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Nanjing University of Science and Technology (NJUST), RONCO, Pierre, Peking University [Beijing], Renal Division [Beijing, China], Beijing Computing Center [Beijing, China], Beijing National Laboratory for Molecular Sciences (BNLMS), Beihang University (BUAA), Université Pierre et Marie Curie - Paris 6 (UPMC), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Tsinghua-Peking Joint Center for Life Sciences, and Tsinghua University [Beijing] (THU)

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, 030232 urology & nephrology, Renal function, Human leukocyte antigen, Glomerulonephritis, Membranous, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Internal medicine, Genotype, medicine, Humans, Risk factor, ComputingMilieux_MISCELLANEOUS, Alleles, Aged, Autoantibodies, Proportional Hazards Models, Kidney, business.industry, Receptors, Phospholipase A2, Hazard ratio, Odds ratio, Middle Aged, medicine.disease, 3. Good health, Molecular Docking Simulation, Phenotype, 030104 developmental biology, medicine.anatomical_structure, Nephrology, [SDV.BBM.GTP] Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Female, business, HLA-DRB1 Chains
Abstract

Genome-wide associations and HLA genotyping have revealed associations between HLA alleles and susceptibility to primary membranous nephropathy. However, associations with clinical phenotypes and kidney outcome are poorly defined. We previously identified DRB1*1501 and DRB1*0301 as independent risk alleles for primary membranous nephropathy. Here, we investigated HLA associations with demographic characteristics, anti-phospholipase A2 receptor (PLA2R) antibody, treatment response and kidney outcome after a median follow-up of 52 months in 258 patients. DRB1*0301, but not DRB1*1501, was associated with a significantly higher level of PLA2R antibody (odds ratio 1.58, 95% confidence interval 1.13-2.22). Although DRB1*1502, which differs from DRB1*1501 by a single amino acid, was not a risk allele for primary membranous nephropathy (odds ratio 1.01), it was associated with significantly lower estimated glomerular filtration rates both at baseline (1.79, 1.18-2.72) and at last follow-up (1.72, 1.17-2.53), a significantly worse renal outcome by Kaplan-Meier analysis and a significantly higher risk of end-stage renal disease by Cox regression analysis (hazard ratio 4.52, 1.22-16.74). Nevertheless, the absence of remission remained the only independent risk factor for end-stage renal disease by multivariate analysis. DRB1*1502 was also associated with a significantly higher median PLA2R antibody level [161.4 vs. 36.3 U/mL] and showed interaction with DRB1*0301 for this variable. Thus, HLA genes control PLA2R antibody production and primary membranous nephropathy severity and outcome. Additionally, DRB1*1502 behaves like a modifier gene with a strong predictor value when associated with HLA risk alleles. Other modifier genes need further investigations in larger cohorts.

Published
2018

33. Protocadherin 7-Associated Membranous Nephropathy

Author

Vivian Negron, Sanjeev Sethi, Hanna Debiec, Michel Jadoul, Lou Ann Gross, Benjamin J. Madden, David Buob, M. Cristine Charlesworth, Pierre Ronco, Sidharth Chaudhry, Johann Morelle, Fernando C. Fervenza, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, and UCL - (SLuc) Service de néphrologie

Subjects
Adult, Male, Pathology, medicine.medical_specialty, glomerular disease, Laser Capture Microdissection, Immunofluorescence, Glomerulonephritis, Membranous, Mass Spectrometry, Cohort Studies, Membranous nephropathy, Western blot, renal biopsy, Clinical Research, renal pathology, Biopsy, medicine, Humans, Aged, Microscopy, Confocal, medicine.diagnostic_test, Chemistry, nephrotic syndrome, Glomerular basement membrane, membranous nephropathy, General Medicine, Middle Aged, medicine.disease, Cadherins, Protocadherins, Staining, medicine.anatomical_structure, Nephrology, immunology and pathology, Case-Control Studies, Immunohistochemistry, Female, Renal biopsy
Abstract

Background Membranous nephropathy (MN) results from deposition of antigen-antibody complexes along the glomerular basement membrane (GBM). PLA2R, THSD7A, NELL1, and SEMA3B account for 80%-90% of target antigens in MN. Methods We performed laser microdissection and mass spectrometry (MS/MS) in kidney biopsies from 135 individuals with PLA2R-negative MN, and used immunohistochemistry/immunofluorescence and confocal microscopy to confirm the MS/MS finding, detect additional cases, and localize the novel protein. We also performed MS/MS and immunohistochemistry on 116 controls and used immunofluorescence microscopy to screen biopsy samples from two validation cohorts. Western blot and elution studies were performed to detect antibodies in serum and biopsy tissue. Results MS/MS studies detected a unique protein, protocadherin 7 (PCDH7), in glomeruli of ten (5.7%) PLA2R-negative MN cases, which also were negative for PLA2R, THSD7A, EXT1/EXT2, NELL1, and SEMA3B. Spectral counts ranged from six to 24 (average 13.2 [SD 6.6]). MS/MS did not detect PCDH7 in controls (which included 28 PLA2R-positive cases). In all ten PCDH7-positive cases, immunohistochemistry showed bright granular staining along the GBM, which was absent in the remaining cases of PLA2R-negative MN and control cases. Four of 69 (5.8%) cases in the validation cohorts (all of which were negative for PLA2R, THSD7A, EXT1, NELL1, and SEMA3B) were PCDH7-positive MN. Kidney biopsy showed minimal complement deposition in 12 of the 14 PCDH7-associated cases. Confocal microscopy showed colocalization of PCDH7 and IgG along the GBM. Western blot analysis using sera from six patients showed antibodies to nonreduced PCDH7. Elution of IgG from frozen tissue of PCDH7-associated MN showed reactivity against PCDH7. Conclusions MN associated with the protocadherin PCDH7 appears to be a distinct, previously unidentified type of MN.

Published
2021

34. Looking back and moving forward

Author

Jai Radhakrishnan, T. Alp Ikizler, Tilman B. Drüeke, Agnes B. Fogo, P. Toby Coates, Brad H. Rovin, Pierre Ronco, Christina M. Wyatt, Jürgen Floege, Germaine Wong, Olivier Devuyst, Masaomi Nangaku, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, and UCL - (SLuc) Service de néphrologie

Subjects
Biomedical Research, Coronavirus disease 2019 (COVID-19), Information Dissemination, Nephrology, Media studies, COVID-19, Humans, Social media, Sociology, Journal Impact Factor, Periodicals as Topic, Dialysis (biochemistry), Editorial Policies
Abstract

2020 has truly been an annus horribilis. The viral tsunami took away our best-established certitudes, caused the death of more 1 million individuals worldwide, and led to the suffering that each of us endured either directly or through its wake of economic, political, and social upheaval. The huge challenges we faced daily in our professional and private lives still stay with us. [...]

Published
2021

35. Diagnostic performance of glomerular PLA2R and THSD7A antibodies in biopsy confirmed primary membranous nephropathy in South Africans

Author

Ugochi S. Okpechi-Samuel, Pierre Ronco, Nicola Wearne, Ikechi G. Okpechi, Aminu K. Bello, Francois Cj Botha, Oluwatoyin I. Ameh, Bingileki F Lwezaula, and Udeme E. Ekrikpo

Subjects
Nephrology, Adult, Male, medicine.medical_specialty, PLA2R, Kidney Glomerulus, 030232 urology & nephrology, Lupus nephritis, 030204 cardiovascular system & hematology, lcsh:RC870-923, Gastroenterology, Glomerulonephritis, Membranous, Primary membranous nephropathy, Diabetic nephropathy, 03 medical and health sciences, South Africa, 0302 clinical medicine, Membranous nephropathy, Internal medicine, Biopsy, medicine, Humans, Autoantibodies, biology, medicine.diagnostic_test, business.industry, Receptors, Phospholipase A2, Histology, Middle Aged, medicine.disease, lcsh:Diseases of the genitourinary system. Urology, Cross-Sectional Studies, THSD7A, biology.protein, Immunohistochemistry, Female, Antibody, business, Thrombospondins, Research Article, Africans
Abstract

Background: Serum and tissue-based tests using phospholipase A2 receptor 1 (PLA2R) and thrombospondin type-1 domain containing 7A (THSD7A) are established immune biomarkers for the diagnosis of primary membranous nephropathy (PMN). This study assessed the diagnostic performance of these biomarkers in the diagnosis of PMN in South Africans. Methods This was a cross-sectional analysis from a single centre in Cape Town, South Africa. Relevant biodata was collected from all patients. Histology, including slides for PLA2R and THSD7A were processed and assessed by typical microscopic and immunohistochemical features. Biopsy tissues of patients with membranous lupus nephritis (LN-V) and diabetic nephropathy (DN) were used as controls. The diagnostic accuracy for diagnosis of PMN using positive PLA2R and THSD7A were evaluated. Results Of the 88 patients included, 41 had PMN with a mean age of 44.5 ± 17.5 years and 61.0% were female. Histologically, PLA2R and THSD7A were only positive in the PMN group (51.2% and 4.9%, respectively) but negative in both control groups. The sensitivity of PLA2R and THSD7A for identifying PMN was 51.2% and 4.9%, respectively. The sensitivity of both tests together was 53.7% while the specificity and positive predictive values (PPV) for any of the tests (alone or in combination) was 100%. There was no difference in the sensitivity and specificity when using PLA2R alone compared to combining the two tests (p=0.32). Conclusion Glomerular staining of PLA2R and THSD7A could have potential diagnostic values in South Africans. This has implications on how immunotherapies can be initiated and used in these settings.

Published
2021

36. Transcription factor 21 expression in injured podocytes of glomerular diseases

Author

Mayumi Takahashi-Kobayashi, Satoshi Yamazaki, Joichi Usui, Kunihiro Yamagata, Pierre Ronco, Surya V. Seshan, Shuzo Kaneko, Tetsuya Kawamura, Misa Yaguchi, Université de Tsukuba = University of Tsukuba, Weill Medical College of Cornell University [New York], Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), and Gestionnaire, Hal Sorbonne Université

Subjects
0301 basic medicine, Adult, Male, medicine.medical_specialty, Nephrotic Syndrome, Urinary system, [SDV]Life Sciences [q-bio], Kidney Glomerulus, 030232 urology & nephrology, lcsh:Medicine, Kidney development, Biology, Severity of Illness Index, Article, Podocyte, 03 medical and health sciences, Mice, 0302 clinical medicine, Internal medicine, medicine, Basic Helix-Loop-Helix Transcription Factors, Animals, Humans, lcsh:Science, Urinary Tract, Transcription factor, Multidisciplinary, Kidney diseases, Podocytes, lcsh:R, Middle Aged, medicine.disease, Actin cytoskeleton, Rats, [SDV] Life Sciences [q-bio], Actin Cytoskeleton, Disease Models, Animal, Proteinuria, 030104 developmental biology, medicine.anatomical_structure, Endocrinology, Cell culture, Apoptosis, Nephrology, lcsh:Q, Female, Nephrotic syndrome
Abstract

Transcription factor 21 (TCF21) is one of the essential transcription factors in kidney development. To elucidate its influence on glomerular disease, we have investigated TCF21 expression in human and rat kidney tissue, and its urinary concentration. Immunohistological analysis suggested the highest TCF21 expression in nephrotic syndrome along with the urinary protein level. Urinary TCF21 concentration in human showed a positive correlation with its podocyte expression level. In nephrotic rat models, TCF21 expression in podocytes increased along with the severity of nephrotic syndrome. Next, in vitro experiments using Tcf21-expressing murine podocyte cell line, we could observe some Tcf21-dependent effects, related with actin cytoskeleton dysregulation and apoptosis. Our study illustrated TCF21 expression changes in vivo and its in vitro-functional significance injured podocytes.

Published
2020

37. Fasting Urinary Osmolality, CKD Progression, and Mortality: A Prospective Observational Study

Author

Jean-Philippe Haymann, Emmanuel Letavernier, Emmanuelle Vidal-Petiot, Martin Flamant, H. Fessi, Christian Jacquot, Jean-Jacques Boffa, Caroline du Halgouet, Pascal Houillier, Marion Vallet, Sandra Wagner, Gérard Maruani, Renaud de la Faille, François Vrtovsnik, Pierre Ronco, Eric Thervet, Eric Daugas, Marine Livrozet, Laurence Nicolet-Barousse, Marie Metzger, Bénédicte Stengel, Nahid Tabibzadeh, Alexandre Karras, Service d'Explorations fonctionnelles multidisciplinaires [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), French-Clinical Research Infrastructure Network [Lyon] (FCRIN INI-CRCT - Coordination Nationale Réseau FORCE), Centre de Recherche en Nutrition Humaine Rhône-Alpes (CRNH-RH), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Recherche Agronomique (INRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-CHU Saint-Etienne-Hospices Civils de Lyon (HCL)-CHU Grenoble-Université Joseph Fourier - Grenoble 1 (UJF)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Recherche Agronomique (INRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-CHU Saint-Etienne-Hospices Civils de Lyon (HCL)-CHU Grenoble-Université Joseph Fourier - Grenoble 1 (UJF)-Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Service de Physiologie [Bichat-Claude Bernard], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Service de Physiologie [Georges-Pompidou], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), Remodelage et Reparation du Tissu Renal, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Immunopathologie rénale, récepteurs et inflammation, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, CHU Tenon [APHP], Service de Néphrologie et Dialyses [CHU Tenon], Paris-Centre de Recherche Cardiovasculaire (PARCC - UMR-S U970), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Service de Néphrologie et Hémodialyse [CHU HEGP], Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Université Paris Descartes - Paris 5 (UPD5)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, F-CRIN, INICRCT network, Paris, Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris Diderot - Paris 7 (UPD7), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université de Paris (UP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), and NephroTest Study Group: Marine Livrozet, Emmanuel Letavernier, Pierre Ronco, Hafedh Fessi, Emmanuelle Vidal-Petiot, Eric Daugas, Caroline du Halgouet, Renaud de La Faille, Gerard Maruani, Marion Vallet, Laurence Nicolet-Barousse, Alexandre Karras, Christian Jacquot

Subjects
Male, urine concentration ability, 030232 urology & nephrology, Urine osmolality, urologic and male genital diseases, tubular damage, 0302 clinical medicine, Risk Factors, 030212 general & internal medicine, Prospective Studies, prognostic factor, ComputingMilieux_MISCELLANEOUS, Fasting, Middle Aged, Prognosis, female genital diseases and pregnancy complications, 3. Good health, Survival Rate, Nephrology, Disease Progression, Biomarker (medicine), biomarker, Female, France, medicine.symptom, glomerular filtration rate (GFR), Cohort study, Glomerular Filtration Rate, medicine.medical_specialty, Urinary system, CKD progression, Urology, chronic kidney disease (CKD), 03 medical and health sciences, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], medicine, Humans, Risk factor, Renal Insufficiency, Chronic, business.industry, Osmolar Concentration, medicine.disease, measured GFR (mGFR), Decreased glomerular filtration rate, end-stage renal disease (ESRD), Albuminuria, GFR decline, business, Biomarkers, Kidney disease
Abstract

International audience; Rationale & objective: Chronic kidney disease (CKD) characterized by decreased glomerular filtration rate (GFR) is often accompanied by various degrees of impaired tubular function in the cortex and medulla. Assessment of tubular function may therefore be useful in establishing the severity of kidney disease and identifying those at greater risk for CKD progression. We explored reductions in urinary concentrating ability, a well-known feature of CKD, as a risk factor for GFR decline and end-stage renal disease (ESRD).Study design: Prospective longitudinal cohort study.Setting & participants: 2,084 adult patients with CKD stages 1 to 4 from the French NephroTest Cohort Study.Predictor: Fasting urinary osmolality measured using delta cryoscopy.Outcomes: ESRD, mortality before ESRD, and measured GFR (mGFR) assessed using 51Cr-EDTA renal clearance.Analytical approach: Cause-specific hazards models were fit to estimate crude and adjusted associations of urinary osmolality with ESRD and death before ESRD. Linear mixed models with random intercepts were fit to evaluate the association of urinary osmolality with slope of decline in mGFR.Results: At baseline, mean age was 58.7±15.2 (SD) years with a median mGFR of 40.2 (IQR, 29.1-54.5) mL/min/1.73m2 and a median fasting urinary osmolality of 502.7±151.7mOsm/kg H2O. Baseline fasting urinary osmolality was strongly associated with mGFR (R=0.54; P < 0.001). 380 ESRD events and 225 deaths before ESRD occurred during a median follow-up of 5.9 (IQR, 3.8-8.2) years. Patients with lower baseline fasting urinary osmolality had higher adjusted risk for ESRD but not for mortality (HRs of 1.97 [95% CI, 1.26-3.08] and 0.99 [95% CI, 0.68-1.44], respectively, for the lowest vs highest tertile). Based on a mixed linear model adjusted for baseline mGFR and clinical characteristics, patients in the lowest tertile of baseline urinary osmolality had a steeper decline in kidney function (-4.9% ± 0.9% per year; P < 0.001) compared with patients in the highest tertile.Limitations: Fasting was self-reported.Conclusions: Fasting urinary osmolality may be a useful tool, in addition to GFR and albuminuria, for assessing nonglomerular damage in patients with CKD who are at higher risk for CKD progression.

Published
2019

38. Milestones in nephrology and welcoming the future: the 61st anniversary of the International Society of Nephrology

Author

Pierre Ronco, Brad Rovin, Masaomi Nangaku, Christina Wyatt, Vivek Jha, P. Toby Coates, Olivier Devuyst, Tilman B. Drueke, Jürgen Floege, Agnes B. Fogo, T. Alp Ikizler, Jai Radhakrishnan, and Germaine Wong

Subjects
Nephrology, medicine.medical_specialty, Anniversaries and Special Events, business.industry, Family medicine, Internal medicine, medicine, Humans, Glomerulonephritis, IGA, business
Published
2020

39. Management of acute kidney injury in symptomatic multiple myeloma

Author

Jean Paul Fermand, Frank Bridoux, Mohamed Belmouaz, Samih H. Nasr, Virginie Royal, Nelson Leung, and Pierre Ronco

Subjects
0301 basic medicine, medicine.medical_specialty, medicine.medical_treatment, 030232 urology & nephrology, Urology, Nephropathy, Bortezomib, 03 medical and health sciences, 0302 clinical medicine, Renal Dialysis, medicine, Humans, Dialysis, Multiple myeloma, Kidney, business.industry, Acute kidney injury, Acute Kidney Injury, medicine.disease, Transplantation, 030104 developmental biology, medicine.anatomical_structure, Nephrology, Quality of Life, Immunoglobulin Light Chains, Hemodialysis, business, Multiple Myeloma, medicine.drug
Abstract

Symptomatic multiple myeloma is commonly complicated by acute kidney injury through various mechanisms. The most frequent is the precipitation of monoclonal free light chains with uromodulin in the distal tubules, defining light chain cast nephropathy. Early diagnosis and identification of the cause of acute kidney injury are required for optimizing management and avoiding chronic kidney injury that strongly affects quality of life and patient survival. In light chain cast nephropathy, often manifesting with severe acute kidney injury, renal recovery requires urgent intervention based on vigorous rehydration, correction of precipitating factors, and efficient anti–plasma cell chemotherapy to rapidly reduce the secretion of nephrotoxic free light chains. Currently, the association of the proteasome inhibitor bortezomib with high-dose dexamethasone is the standard regimen in newly diagnosed patients. The addition of another drug such as cyclophosphamide or an immunodulatory agent may improve free light chain response but raises tolerance concerns in frail patients. Further studies are warranted to confirm the role of anti-CD38 monoclonal antibodies, whose efficacy and tolerance have been documented in patients without renal impairment. Despite controversial results from randomized studies, recent data suggest that in patients with light chain cast nephropathy and acute kidney injury requiring dialysis, the combination of chemotherapy with free light chain removal through high-cutoff hemodialysis may increase renal response recovery rates. Kidney biopsy may be helpful in guiding management and assessing renal prognosis that appears to depend on the extent of cast formation and interstitial fibrosis/tubular atrophy. Because of continuous improvement in life expectancy of patients with multiple myeloma, renal transplantation is likely to be increasingly considered in selected candidates.

Published
2020

40. Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome

Author

Xiaoyuan Jia, Tomohiko Yamamura, Rasheed Gbadegesin, Michelle T. McNulty, Kyuyong Song, China Nagano, Yuki Hitomi, Dongwon Lee, Yoshihiro Aiba, Seik-Soon Khor, Kazuko Ueno, Yosuke Kawai, Masao Nagasaki, Eisei Noiri, Tomoko Horinouchi, Hiroshi Kaito, Riku Hamada, Takayuki Okamoto, Koichi Kamei, Yoshitsugu Kaku, Rika Fujimaru, Ryojiro Tanaka, Yuko Shima, Jiwon Baek, Hee Gyung Kang, Il-Soo Ha, Kyoung Hee Han, Eun Mi Yang, Asiri Abeyagunawardena, Brandon Lane, Megan Chryst-Stangl, Christopher Esezobor, Adaobi Solarin, Claire Dossier, Georges Deschênes, Marina Vivarelli, Hanna Debiec, Kenji Ishikura, Masafumi Matsuo, Kandai Nozu, Pierre Ronco, Hae Il Cheong, Matthew G. Sampson, Katsushi Tokunaga, Kazumoto Iijima, Yoshinori Araki, Yoshinobu Nagaoka, Yasuyuki Sato, Asako Hayashi, Toshiyuki Takahashi, Hayato Aoyagi, Michihiko Ueno, Masanori Nakanishi, Nariaki Toita, Kimiaki Uetake, Norio Kobayashi, Shoji Fujita, Kazushi Tsuruga, Naonori Kumagai, Hiroki Kudo, Eriko Tanaka, Tae Omori, Mari Okada, Yoshiho Hatai, Tomohiro Udagawa, Yaeko Motoyoshi, Masao Ogura, Mai Sato, Yuji Kano, Motoshi Hattori, Kenichiro Miura, Yutaka Harita, Shoichiro Kanda, Emi Sawanobori, Anna Kobayashi, Manabu Kojika, Yoko Ohwada, Kunimasa Yan, Hiroshi Hataya, Chikako Terano, Ryoko Harada, Yuko Hamasaki, Junya Hashimoto, Shuichi Ito, Hiroyuki Machida, Aya Inaba, Takeshi Matsuyama, Miwa Goto, Masaki Shimizu, Kazuhide Ohta, Yohei Ikezumi, Takeshi Yamada, Toshiaki Suzuki, Soichi Tamamura, Yukiko Mori, Yoshihiko Hidaka, Daisuke Matsuoka, Tatsuya Kinoshita, Shunsuke Noda, Masashi Kitahara, Naoya Fujita, Satoshi Hibino, Shogo Minamikawa, Keita Nakanishi, Junya Fujimura, Nana Sakakibara, Yuya Aoto, Shinya Ishiko, Kyoko Kanda, Yosuke Inaguma, Yuya Hashimura, Shingo Ishimori, Naohiro Kamiyoshi, Takayuki Shibano, Yasuhiro Takeshima, Hiroaki Ueda, Akira Ashida, Hideki Matsumura, Takuo Kubota, Taichi Kitaoka, Yusuke Okuda, Toshihiro Sawai, Tomoyuki Sakai, Taketsugu Hama, Mikiya Fujieda, Masayuki Ishihara, Shigeru Itoh, Takuma Iwaki, Maki Shimizu, Koji Nagatani, Shoji Kagami, Maki Urushihara, Manao Nishimura, Miwa Yoshino, Ken Hatae, Maiko Hinokiyama, Rie Kuroki, Yasufumi Ohtsuka, Masafumi Oka, Shinji Nishimura, Tadashi Sato, Seiji Tanaka, Ayuko Zaitsu, Hitoshi Nakazato, Hiroshi Tamura, Koichi Nakanishi, Min Hyun Cho, Tae-Sun Ha, Ji Hyun Kim, Peong Gang Park, Myung Hyun Cho, Alejandro Quiroga, Asha Moudgil, Blanche Chavers, Charles Kwon, Corinna Bowers, Deb Gipson, Deepa Chand, Donald Jack Weaver, Elizabeth Abraham, Halima Janjua, Jen-Jar Lin, Larry Greenbaum, Mahmoud Kallash, Michelle Rheault, Nilka De Jeus Gonzalez, Patrick Brophy, Shashi Nagaraj, Susan Massengill, Tarak Srivastava, Tray Hunley, Yi Cai, Abiodun Omoloja, Cynthia Silva, Adebowale Adeyemo, Shenal Thalgahagoda, Jameela A. Kari, Sherif El Desoky, Mohammed Abdelhadi, Rachida Akil, Sonia Azib, Romain Basmaci, Gregoire Benoist, Philippe Bensaid, Philippe Blanc, Olivia Boyer, Julie Bucher, Anne Chace, Arnaud Chalvon, Marion Cheminee, Sandrine Chendjou, Patrick Daoud, Ossam Elias, Chantal Gagliadone, Vincent Gajdos, Aurélien Galerne, Evelyne Jacqz Aigrain, Lydie Joly Sanchez, Mohamed Khaled, Fatima Khelfaoui, Yacine Laoudi, Anis Larakeb, Tarek Limani, Fouad Mahdi, Alexis Mandelcwaijg, Stephanie Muller, Kacem Nacer, Sylvie Nathanson, Béatrice Pellegrino, Isabelle Pharaon, Véronica Roudault, Sébastien Rouget, Marc Saf, Tabassom Simon, Cedric Tahiri, Tim Ulinski, Férielle Zenkhri, CHU Tenon [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
Tumor Necrosis Factor Ligand Superfamily Member 15, 0301 basic medicine, podocyte, Steroid-sensitive nephrotic syndrome, 030232 urology & nephrology, Polygenic disease, glomerulus, Biology, Monogenic disease, Article, 03 medical and health sciences, 0302 clinical medicine, pediatric nephrology, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], medicine, Humans, Child, Gene, Allele frequency, Congenital nephrotic syndrome, Alleles, ComputingMilieux_MISCELLANEOUS, Genetics, nephrotic syndrome, Membrane Proteins, Odds ratio, medicine.disease, 3. Good health, 030104 developmental biology, Haplotypes, Nephrology, Mutation, Steroids, Nephrotic syndrome, Genome-Wide Association Study
Abstract

To understand the genetics of steroid-sensitive nephrotic syndrome (SSNS), we conducted a genome-wide association study in 987 childhood SSNS patients and 3,206 healthy controls with Japanese ancestry. Beyond known associations in the HLA-DR/DQ region, common variants in NPHS1-KIRREL2 (rs56117924, P=4.94E-20, odds ratio (OR) =1.90) and TNFSF15 (rs6478109, P=2.54E-8, OR=0.72) regions achieved genome-wide significance and were replicated in Korean, South Asian and African populations. Trans-ethnic meta-analyses including Japanese, Korean, South Asian, African, European, Hispanic and Maghrebian populations confirmed the significant associations of variants in NPHS1-KIRREL2 (P(meta)=6.71E-28, OR=1.88) and TNFSF15 (P(meta)=5.40E-11, OR=1.33) loci. Analysis of the NPHS1 risk alleles with glomerular NPHS1 mRNA expression from the same person revealed allele specific expression with significantly lower expression of the transcript derived from the risk haplotype (Wilcox test p=9.3E-4). Because rare pathogenic variants in NPHS1 cause congenital nephrotic syndrome of the Finnish type (CNSF), the present study provides further evidence that variation along the allele frequency spectrum in the same gene can cause or contribute to both a rare monogenic disease (CNSF) and a more complex, polygenic disease (SSNS).

Published
2020

41. Kidney International and the COVID-19 infection

Author

Tilman B. Drüeke, T. Alp Ikizler, Brad H. Rovin, Jürgen Floege, Jai Radhakrishnan, Germaine Wong, Pierre Ronco, Christina M. Wyatt, Masaomi Nangaku, P. Toby Coates, Agnes B. Fogo, Olivier Devuyst, University of Zurich, Rovin, Brad H, Ohio State University [Columbus] (OSU), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Associate Editors, Entire Editorial Team, Bodescot, Myriam, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), UCL - SSS/IREC/NEFR - Pôle de Néphrologie, and UCL - (SLuc) Service de néphrologie

Subjects
Nephrology, medicine.medical_specialty, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), short review process, 610 Medicine & health, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 10052 Institute of Physiology, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, ComputingMilieux_MISCELLANEOUS, [SDV.MP.VIR] Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.MHEP.ME] Life Sciences [q-bio]/Human health and pathology/Emerging diseases, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Kidney, 2727 Nephrology, business.industry, COVID-19, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Virology, medicine.anatomical_structure, 030228 respiratory system, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, 570 Life sciences, biology, business
Abstract

International audience

Published
2020

42. Screening for Cancer in Patients with Glomerular Diseases

Author

Emmanuelle Plaisier, Pierre Ronco, Service de Néphrologie et Dialyses [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Sorbonne Université (SU), Service de Département de Néphrologie = Service de Néphrologie et Dialyses [CHU Tenon], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Gestionnaire, Hal Sorbonne Université

Subjects
Nephrology, Oncology, Epidemiology, Paraneoplastic Syndromes, membranoproliferative glomerulonephritis, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, neoplasms, human PLA2R1 protein, 030204 cardiovascular system & hematology, Critical Care and Intensive Care Medicine, Glomerulonephritis, Membranous, nephrologists, 0302 clinical medicine, cytosolic phospholipases A2, phospholipase A2 receptors, Membranoproliferative glomerulonephritis, risk factors, humans, ComputingMilieux_MISCELLANEOUS, Cancer, Kidney, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, nephrotic syndrome, phospholipases A2, follow-up studies, 3. Good health, [SDV] Life Sciences [q-bio], medicine.anatomical_structure, Perspectives, medicine.medical_specialty, kidney, thrombospondins, recurrence, nephrology, Malignancy, immunosuppressive agents, 03 medical and health sciences, Membranous nephropathy, Internal medicine, medicine, In patient, Autoantibodies, treatment failure, Transplantation, urogenital system, business.industry, Receptors, Phospholipase A2, cost-benefit analysis, membranous nephropathy, medicine.disease, early detection of cancer, business, Nephrotic syndrome, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

The diagnosis of malignancy in the setting of nephrotic syndrome is associated with a poor overall and kidney survival regardless of whether the glomerular disease represents a paraneoplastic manifestation or the cancer is a coincidental finding. The diagnosis of the neoplasia leads to consideration

Published
2020

43. KDIGO Controversies Conference on onco-nephrology: kidney disease in hematological malignancies and the burden of cancer after kidney transplantation

Author

Farhad R. Danesh, Kunitoshi Iseki, Mitchell H. Rosner, Laura Cosmai, Kenar D. Jhaveri, Michael Cheung, Hatem Amer, Manuel Macía, Germaine Wong, Artur Jurczyszyn, Alicja Dębska-Ślizień, Rumeyza Kazancioglu, Elena Zakharova, Romano Danesi, Jeremy R. Chapman, Pierre Ronco, Aristotelis Bamias, Yang Li, Amit Lahoti, Anitha Vijayan, Petra Tesarova, Maria Jose Soler Romeo, Abhijat Kitchlu, Verônica Torres da Costa e Silva, Morie A. Gertz, Eisei Noiri, Walter M. Stadler, Wolfgang C. Winkelmayer, Maurizio Gallieni, Camillo Porta, Filippo De Stefano, Dionysios Mitropoulos, Christian Kollmannsberger, Paul E. Stevens, Edgar A. Jaimes, Vladimír Tesař, Gernot Beutel, Xiaohong Chen, Ali K. Abu-Alfa, Ben Sprangers, Biruh Workeneh, David H. Vesole, Jolanta Malyszko, Jan T. Kielstein, Ondřej Viklický, David C. Wheeler, Jerzy Chudek, Mark A. Perazella, Motoko Yanagita, and Takeshi Matsubara

Subjects
0301 basic medicine, Nephrology, medicine.medical_specialty, medicine.medical_treatment, 030232 urology & nephrology, kidney transplantation, chemotherapy, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Intensive care medicine, Kidney transplantation, Multiple myeloma, Dialysis, business.industry, Acute kidney injury, Cancer, medicine.disease, Tumor lysis syndrome, 030104 developmental biology, chronic kidney disease, oncology, tumor lysis syndrome, business, Kidney disease
Abstract

The bidirectional relationship between cancer and chronic kidney disease (CKD) is complex. Patients with cancer, particularly those with hematological malignancies such as multiple myeloma and lymphoma, are at increased risk of developing acute kidney injury and CKD. On the other hand, emerging evidence from large observational registry analyses have consistently shown that cancer risk is increased by at least 2- to 3-fold in kidney transplant recipients, and the observed increased risk occurs not only in those who have received kidney transplants but also in those on dialysis and with mild- to moderate-stage CKD. The interactions between cancer and CKD have raised major therapeutic and clinical challenges in the management of these patients. Given the magnitude of the problem and uncertainties, and current controversies within the existing evidence, Kidney Disease: Improving Global Outcomes (KDIGO) assembled a global panel of multidisciplinary clinical and scientific expertise for a controversies conference on onco-nephrology to identify key management issues in nephrology relevant to patients with malignancy. This report covers the discussed controversies in kidney disease in hematological malignancies, as well as cancer after kidney transplantation. An overview of future research priorities is also discussed.

Published
2020

44. Early experience with COVID-19 in kidney transplantation

Author

Jürgen Floege, Tilman B. Drüeke, T. Alp Ikizler, Agnes B. Fogo, Masaomi Nangaku, Pierre Ronco, P. Toby Coates, Jai Radhakrishnan, Olivier Devuyst, Germaine Wong, Brad H. Rovin, Christina M. Wyatt, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service de néphrologie, University of Zurich, and Coates, P Toby

Subjects
Nephrology, medicine.medical_specialty, 2727 Nephrology, biology, Coronavirus disease 2019 (COVID-19), business.industry, 610 Medicine & health, biology.organism_classification, medicine.disease, medicine.disease_cause, Virology, 10052 Institute of Physiology, Transplantation, Pneumonia, Internal medicine, Pandemic, 570 Life sciences, Medicine, business, Betacoronavirus, Kidney transplantation, Coronavirus
Abstract

In this edition of Kidney International, we publish 2 series of patients who had undergone kidney transplantation during the coronavirus disease 2019 (COVID-19) pandemic that have taken different approaches to transplant immunosuppressive therapy and antiviral treatment in the early days of the developing pandemic in the United Kingdom and Italy. The first series of 7 cases of COVID-19 infection in patients who had undergone kidney transplantation comes from St. George's, St. Helier’s, and King’s College Hospitals in south London, United Kingdom,1 and the second series of 20 cases originates from Spedali Civili University Hospital in Brescia, Italy. [...]

Published
2020

45. The STARMEN trial indicates that alternating treatment with corticosteroids and cyclophosphamide is superior to sequential treatment with tacrolimus and rituximab in primary membranous nephropathy

Author

Gema Fernández-Juárez, Jorge Rojas-Rivera, Anne-Els van de Logt, Joana Justino, Angel Sevillano, Fernando Caravaca-Fontán, Ana Ávila, Cristina Rabasco, Virginia Cabello, Alfonso Varela, Montserrat Díez, Guillermo Martín-Reyes, Marian Goicoechea Diezhandino, Luis F. Quintana, Irene Agraz, Juan Ramón Gómez-Martino, Mercedes Cao, Antolina Rodríguez-Moreno, Begoña Rivas, Cristina Galeano, Jose Bonet, Ana Romera, Amir Shabaka, Emmanuelle Plaisier, Mario Espinosa, Jesus Egido, Alfonso Segarra, Gérard Lambeau, Pierre Ronco, Jack Wetzels, Manuel Praga, Fernando Caravaca-Fontan, Hernando Trujillo, Eduardo Gutiérrez, Gema Fernandez Juarez, Alberto Ortiz, Marian Goicoechea, Úrsula Verdalles, Alfons Segarra, Lara Perea, Ildefonso Valera, Mónica Martín, Miguel Angel Pérez Valdivia, Miquel Blasco, Andrés López Muñiz, Ana Avila, Tamara Malek, Montserrat Diaz, Iara DaSilva, Jordi Bonet, Maruja Navarro, Ana Huerta, Ezequiel Rodríguez-Paternina, Ana Vigil, Roberto Alcázar, Vicente Paraíso, Vicente Barrio, Julia Hofstra, Institut Català de la Salut, [Fernández-Juárez G] Nephrology Division, Hospital Universitario Fundación Alcorcón, Madrid, Spain. [Rojas-Rivera J] Nephrology Division, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain. [Logt AV] Nephrology Division, Radboud University Medical Center, Nijmegen, The Netherlands. [Justino J] Institut de Pharmacologie Moléculaire et Cellulaire (IPMC), Université Côte d’Azur, Centre National de la Recherche Scientifique (CNRS), Valbonne Sophia Antipolis, France. [Sevillano A, Caravaca-Fontán F] Nephrology Division, Instituto de Investigación Hospital Universitario 12 Octubre, Madrid, Spain. [Agraz I] Divisió de Nefrologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus, Nephrology Division, Hospital Universitario Fundación Alcorcón, Madrid, Spain, Nephrology Division, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain, Radboud Institute for Health Sciences, Radboud University Medical Center [Nijmegen], Institut de pharmacologie moléculaire et cellulaire (IPMC), Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), Hospital Universitario 12 de Octubre [Madrid], Dr Peset University Hospital, Hospital Reina Sofia, Cordoba, Hospital Universitario Virgen del Rocío [Sevilla], Hospital of Virgen de la Victoria de Malaga, Institut Investigacions Biomèdiques (IBB) Sant Pau [Barcelona, Spain], Hospital Regional Universitario de Málaga = Regional University Hospital of Malaga [Spain], Hospital General Universitario 'Gregorio Marañón' [Madrid], Department of Nephrology, Hospital Clinic, Barcelona, Spain., Vall d'Hebron University Hospital [Barcelona], San Pedro de Alcantara Hospital [Cáceres, Espagne], Instituto de Investigación Biomédica de A Coruña (INIBIC), Complexo Hospitalario Universitario de A Coruña (CHUAC), Universidade da Coruña (UDC), Spain, Hospital Clinico San Carlos, Hospital Clínico San Carlos, Hospital Universitario La Paz [Madrid, Espagne], Hospital Universitario Ramón y Cajal [Madrid], Universidad de Alcalá - University of Alcalá (UAH), Germans Trias i Pujol Hospital, Universitat Autònoma de Barcelona (UAB), Hospital General Universitario de Ciudad Real [Ciudad Real, Spain], Maladies rénales fréquentes et rares : des mécanismes moléculaires à la médecine personnalisée (CoRaKID), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Radboud Institute for Health Sciences [Nijmegen, the Netherlands], Hospital Universitario Fundación Alcorcón, Hospital Universitario Fundación Jiménez Díaz [Madrid, Spain], Barcelona Centre for International Health Research, Hospital Clinic (CRESIB), Universitat de Barcelona (UB), Hospital Universitario, A Coruña, Fundació Puigvert [Barcelona, Spain], Germans Trias i Pujol University Hospital [Badalona, Barcelona, Spain] (GTPUH), Hospital Universitario Puerta de Hierro-Majadahonda [Madrid, Spain], Getafe University Hospital, Madrid, Severo Ochoa Hospital, Hospital Universitario Infanta Leonor [Madrid], Del Henares Hospital, Hospital Universitario Infanta Sofía, Radboud University Medical Center, PO Box 9101, 6500 HB Nijmegen, The Netherlan ds, Lambeau, Gerard, Centre National de la Recherche Scientifique (CNRS)-Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Université Côte d'Azur (UCA), Hospital Regional Universitario de Málaga [Spain], Barcelona Autonomous University, Common and Rare Kidney Diseases = Maladies Rénales Fréquentes et Rares: des Mécanismes Moléculaires à la Médecine Personnalisée (CORAKID), and Germans Trias i Pujol University Hospital

Subjects
0301 basic medicine, medicine.medical_specialty, Cyclophosphamide, medicine.drug_class, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Otros calificadores::Otros calificadores::/farmacoterapia [Otros calificadores], Other subheadings::Other subheadings::/drug therapy [Other subheadings], Gastroenterology, Tacrolimus, Primary membranous nephropathy, law.invention, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Randomized controlled trial, law, Internal medicine, medicine, Corticosteroids, ComputingMilieux_MISCELLANEOUS, business.industry, Male Urogenital Diseases::Urologic Diseases::Kidney Diseases [DISEASES], 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], 3. Good health, [SDV] Life Sciences [q-bio], Regimen, 030104 developmental biology, Nephrology, Avaluació de resultats (Assistència sanitària), Corticosteroid, Rituximab, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business, Nephrotic syndrome, enfermedades urogenitales masculinas::enfermedades urológicas::enfermedades renales [ENFERMEDADES], Ronyons - Malalties - Tractament, medicine.drug
Abstract

Altres ajuts: Fondo Europeo de Desarrollo Regional (FEDER); Red de Investigación Renal (RedInRen); European Renal Association-European Dialysis and Transplant Association (ERA-EDTA); Fundación Renal Iñigo Álvarez de Toledo (FRIAT); Fundación para la Investigación Biomédica Hospital 12 de Octubre (i+12); Centre National de la Recherche Scientifique; Fondation Maladies Rares (LAM-RD_20170304); National Research Agency (ANR, grants MNaims ANR-17-CE17-0012-01); "Investments for the Future" Laboratory of Excellence SIGNALIFE, a network for innovation on signal transduction pathways in life sciences (ANR-11-LABX-0028-01); Initiative of Excellence (IDEX; UCAJedi ANR-15-IDEX-01); Fondation pour la Recherche Médicale (FRM, ING20140129210, DEQ20180339193, FDT201805005509). A cyclical corticosteroid-cyclophosphamide regimen is recommended for patients with primary membranous nephropathy at high risk of progression. We hypothesized that sequential therapy with tacrolimus and rituximab is superior to cyclical alternating treatment with corticosteroids and cyclophosphamide in inducing persistent remission in these patients. This was tested in a randomized, open-label controlled trial of 86 patients with primary membranous nephropathy and persistent nephrotic syndrome after six-months observation and assigned 43 each to receive six-month cyclical treatment with corticosteroid and cyclophosphamide or sequential treatment with tacrolimus (full-dose for six months and tapering for another three months) and rituximab (one gram at month six). The primary outcome was complete or partial remission of nephrotic syndrome at 24 months. This composite outcome occurred in 36 patients (83.7%) in the corticosteroid-cyclophosphamide group and in 25 patients (58.1%) in the tacrolimus-rituximab group (relative risk 1.44; 95% confidence interval 1.08 to 1.92). Complete remission at 24 months occurred in 26 patients (60%) in the corticosteroid-cyclophosphamide group and in 11 patients (26%) in the tacrolimus-rituximab group (2.36; 1.34 to 4.16). Anti-PLA2R titers showed a significant decrease in both groups but the proportion of anti-PLA2R-positive patients who achieved immunological response (depletion of anti-PLA2R antibodies) was significantly higher at three and six months in the corticosteroid-cyclophosphamide group (77% and 92%, respectively), as compared to the tacrolimus-rituximab group (45% and 70%, respectively). Relapses occurred in one patient in the corticosteroid-cyclophosphamide group, and three patients in the tacrolimus-rituximab group. Serious adverse events were similar in both groups. Thus, treatment with corticosteroid-cyclophosphamide induced remission in a significantly greater number of patients with primary membranous nephropathy than tacrolimus-rituximab.

Published
2020

46. How to assess kidney function in oncology patients

Author

Jolanta Malyszko, Giovambattista Capasso, Anna Capasso, Paweł Kulicki, Davide Viggiano, Pierre Ronco, Petra Tesarova, Paul E. Stevens, Michael W. Lee, Joanna Matuszkiewicz-Rowińska, Malyszko, J., Lee, M. W., Capasso, G., Kulicki, P., Matuszkiewicz-Rowinska, J., Ronco, P., Stevens, P., Tesarova, P., Viggiano, D., and Capasso, A.

Subjects
0301 basic medicine, medicine.medical_specialty, Urinalysis, biomarkers, glomerular filtration rate, kidney function, oncology, medicine.medical_treatment, Population, 030232 urology & nephrology, Renal function, Kidney, Kidney Function Tests, Nephrotoxicity, 03 medical and health sciences, 0302 clinical medicine, Neoplasms, medicine, Humans, Intensive care medicine, education, education.field_of_study, Chemotherapy, medicine.diagnostic_test, business.industry, urogenital system, Clinical trial, 030104 developmental biology, Nephrology, Creatinine, Toxicity, biomarker, Oncology patients, business
Abstract

Assessment of kidney function in oncology patients is a fundamental factor in profiling the survival risk, determining the appropriate dose of chemotherapeutic drugs, and defining a patient eligibility for clinical trials with novel agents. Both overestimation and underestimation of kidney function may affect the treatment efficacy and outcomes. Overestimation may lead to overdosing or inappropriate agent selection and the corresponding toxicity, whereas underestimation may be responsible for underdosing or inappropriate agent exclusion and subsequent treatment failure. This is of utmost importance in patients with cancer. Evaluation of kidney function is not only limited to the estimation of glomerular filtration rate or creatinine clearance. An accurate assessment of kidney function is advisable to reduce variability in decision making and ultimately the therapeutic outcomes of toxicity and clinical benefit. Therefore, additional studies are needed to investigate the validity of currently used formulas estimating kidney function in this population as well as their applicability to traditional chemotherapy, novel targeted therapies, and immunotherapies. Because of rapid discovery and development of new cancer agents, a reliable and comprehensive manner to screen for potential nephrotoxicity is critically important. As kidney function not only is limited to glomerular filtration rate changes but also involves tubular and even vascular dysfunction, urinalysis and kidney imaging studies should also be considered before therapeutic decisions are taken. However, several questions remain regarding these new technologies such as kidney-on-a-chip systems for the assessment of kidney function and injury, particularly in oncology, and it has yet to be implemented in clinical practice.

Published
2020

47. Podocyte Antigen Staining to Identify Distinct Phenotypes and Outcomes in Membranous Nephropathy: A Retrospective Multicenter Cohort Study

Author

Hanna Debiec, Ashley Broughton, Michel Wauthier, Albert Hermant, Benoit Georges, Abdelhamid Lalaoui, Diego Castanares-Zapatero, Matthieu Lemaire, Fabienne Mestrez, Yves Pirson, Nicolas Hanset, Nada Kanaan, Gregory Van Ingelgem, Roxana Sava, Benoit Guillaume, Jean-Michel Pochet, Frédéric Debelle, Lionel Mazzoleni, Valentine Gillion, Michel Tintillier, Nicolas Cecere, Philippe Leroy, Jean-Claude Stolear, Gabriela Migali, Benjamin Seront, Pierre Ronco, Corinne Langen, Michele Muller, Georges Cornet, Arnaud Devresse, Guy Fomegne, Nathalie Demoulin, Yvan Philips, Charlotte Van Ende, Assma Ballout, Delphine Halleux, Nadejda Ranguelov, Pierre-Yves Decleire, Ahmed Goubella, Christine Hurtgen, Ralph Crott, Pauline Biller, Johann Morelle, An Van Audenhove, Philippe Durieux, Caroline Clerckx, Nathalie Godefroid, Dominique Becker, Charles Cuvelier, Selda Aydin, Hélène Munyentwali, Francois Reginster, Jean-Louis Christophe, Jean-Philippe Lengelé, René Cuvelier, Benoit Buysschaert, Joëlle Ghysen, Jean-Jacques Lafontaine, Jean-François Cambier, Eric Goffin, Fabrice Gankam, Agnès Dejardin, Olivier Mat, Laura Labriola, Jean Jamez, Jean-Pierre Cosyns, Gaetan Clerbaux, Pierre Bernis, Michel Jadoul, Miguel-Ange Guillen-Anaya, Gaëlle Gillerot, Marie Rommelaere, Bénédicte Vanderperren, Joseph Mbaba Mena, Alina Tirdea, Liesbeth Smets, Frédéric Houssiau, Zuzana Rihova, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - SSS/IREC/MORF - Pôle de Morphologie, UCL - SSS/IREC/RUMA - Pôle de Pathologies rhumatismales, UCL - (SLuc) Service de néphrologie, UCL - (SLuc) Service d'anatomie pathologique, UCL - (SLuc) Service de soins intensifs, UCL - (SLuc) Service de rhumatologie, CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Cliniques Universitaires Saint-Luc [Bruxelles], Lille économie management - UMR 9221 (LEM), Université d'Artois (UA)-Université catholique de Lille (UCL)-Université de Lille-Centre National de la Recherche Scientifique (CNRS), Université Catholique de Louvain = Catholic University of Louvain (UCL), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de Recherche Expérimentale et Clinique (IREC), ANR-17-CE17-0012,MNaims,Dissection moléculaire de la glomérulonéphrite extramembraneuse liée à PLA2R1: vers l'identification de nouveaux biomarqueurs cliniques(2017), and UCL - SSS/IREC - Institut de recherche expérimentale et clinique

Subjects
Male, Biopsy, 030232 urology & nephrology, glomerular disease, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Gastroenterology, Glomerulonephritis, Membranous, phospholipase A(2) receptor (PLA(2)R), 0302 clinical medicine, Medicine, thrombospondin type 1 domain-containing 7A (THSD7A), 030212 general & internal medicine, Kidney, immunostaining, medicine.diagnostic_test, Podocytes, nephrotic syndrome, Middle Aged, 3. Good health, medicine.anatomical_structure, Phenotype, Nephrology, Disease Progression, outcome, Female, Adult, medicine.medical_specialty, kidney biopsy, thrombospondin type 1 domain-containing 7A, 03 medical and health sciences, Membranous nephropathy, Internal medicine, Humans, cancer, Aged, Autoantibodies, Retrospective Studies, phospholipase A2 receptor, Lupus erythematosus, Staining and Labeling, business.industry, membranous nephropathy, Cancer, Retrospective cohort study, medicine.disease, podocyte antigen, business, Nephrotic syndrome, Kidney disease, Follow-Up Studies, Membranous nephropathy (MN), malignancy
Abstract

Rationale & Objective Membranous nephropathy (MN) is characterized by the deposition of immune complexes along glomerular basement membranes. M-Type phospholipase A2 receptor (PLA2R), thrombospondin type 1 domain-containing 7A (THSD7A), exostosin 1 and 2 (EXT1/2), and neural epidermal growth factor-like 1 protein (NELL-1) have been identified as established or potential podocyte antigens in MN. We investigated the association of podocyte antigen staining with MN clinical phenotype and outcomes. Study Design Multicenter retrospective cohort study. Setting & Participants 177 consecutive patients with MN unrelated to lupus erythematosus, identified after screening of 3,875 native kidney biopsies performed in the Belgian UCLouvain Kidney Disease Network from 2000 through 2018. Predictor Positive immunostaining for podocyte antigens on archived kidney biopsy samples. Outcomes Association with different phenotypes (baseline characteristics of patients and pathologic findings on kidney biopsy), time to cancer and to kidney failure. Analytical Approach Kaplan-Meier estimates and Cox regression analyses to assess time to cancer and kidney failure. Results 177 patients were followed up for a median of 4.0 (IQR, 1.3-8.0) years. Diagnosis of PLA2R-positive (PLA2R+), THSD7A+, and double-negative (PLA2R−/THSD7A−) MN was made in 117 (66.1%), 6 (3.4%), and 54 (30.5%) patients, respectively. Progression to kidney failure was similar in all groups. Although the number of patients with THSD7A+ MN was small, they showed a higher incidence (50%) and increased risk for developing cancer during follow-up (adjusted HR, 5.0 [95% CI, 1.4-17.9]; P = 0.01). 8% and 5% of patients with double-negative MN stained positively for EXT1/2 and NELL-1, respectively. Most patients with EXT1/2+ MN were women, had features of systemic autoimmunity, and showed glomerular C1q deposits. Limitations Retrospective design; small number of patients in the THSD7A group; lack of evaluation of immunoglobulin G subclasses deposition. Conclusions Our real-world data describe the relative prevalence of subgroups of MN and support the hypothesis that a novel classification of MN based on podocyte antigen staining may be clinically relevant.

Published
2020

48. Standardized reporting of monoclonal immunoglobulin-associated renal diseases: recommendations from a Mayo Clinic/Renal Pathology Society Working Group

Author

Shaji Kumar, Fernando G. Cosio, David L. Murray, Mariam P. Alexander, Gerald B. Appel, Mark Haas, Dario Roccatello, Ladan Zand, Virginie Royal, Hatem Amer, Loren P. Herrera Hernandez, Cynthia C. Nast, Richard J. Glassock, Anthony Chang, Antonello Pani, An S. De Vriese, Pingchuan Zhang, Kelly D. Smith, Nelson Leung, Vivette D. D'Agati, Ashutosh D. Wechalekar, Sanjeev Sethi, Glen S. Markowitz, Michael B. Stokes, Aishwarya Ravindran, Samih H. Nasr, Maria M. Picken, Pierre Ronco, Fernando C. Fervenza, Jack F.M. Wetzels, Mayo Clinic [Rochester], David Geffen School of Medicine [Los Angeles], University of California [Los Angeles] (UCLA), University of California (UC)-University of California (UC), New York University Langone Medical Center (NYU Langone Medical Center), NYU System (NYU), Melting the frontiers between Light, Shape and Matter (MANAO), Laboratoire Bordelais de Recherche en Informatique (LaBRI), Université de Bordeaux (UB)-École Nationale Supérieure d'Électronique, Informatique et Radiocommunications de Bordeaux (ENSEIRB)-Centre National de la Recherche Scientifique (CNRS)-Université de Bordeaux (UB)-École Nationale Supérieure d'Électronique, Informatique et Radiocommunications de Bordeaux (ENSEIRB)-Centre National de la Recherche Scientifique (CNRS)-Inria Bordeaux - Sud-Ouest, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Laboratoire Photonique, Numérique et Nanosciences (LP2N), and Université de Bordeaux (UB)-Institut d'Optique Graduate School (IOGS)-Centre National de la Recherche Scientifique (CNRS)-Institut d'Optique Graduate School (IOGS)-Centre National de la Recherche Scientifique (CNRS)

Subjects
medicine.medical_specialty, Kidney, business.industry, Paraproteinemias, Monoclonal immunoglobulin, monoclonal gammopathy of renal significance, kidney biopsy glomerulonephritis, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, monoclonal Ig, medicine.anatomical_structure, Renal pathology, Nephrology, Internal medicine, medicine, Humans, Kidney Diseases, Paraproteins, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business
Abstract

Contains fulltext : 225148.pdf (Publisher’s version ) (Closed access)

Published
2020

49. Rituximab in Patients With Phospholipase A2 Receptor-Associated Membranous Nephropathy and Severe CKD

Author

Nicolas Hanset, Karine Dahan, Patrick Fievet, Pierre Ronco, Catherine Johanet, P.A. Michel, Emmanuelle Plaisier, Laurent Mesnard, Johann Morelle, Emmanuel Esteve, Jean-Jacques Boffa, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service de néphrologie, UCL - (SLuc) Service de médecine interne générale, Service de Néphrologie et Dialyses [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Cliniques Universitaires Saint-Luc [Bruxelles], Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), CHU Tenon [APHP], Service d'immunologie et hématologies biologiques [Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Sorbonne Université (SU)-CHU Saint-Antoine [APHP], Urgences néphrologiques et transplantation rénale [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'immunologie et hématologies biologiques [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)

Subjects
medicine.medical_specialty, Urinary system, PLA2R, 030232 urology & nephrology, Renal function, 030204 cardiovascular system & hematology, lcsh:RC870-923, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, rituximab, Membranous nephropathy, Clinical Research, Interquartile range, Internal medicine, medicine, CKD, ESKD, business.industry, membranous nephropathy, immunosuppressive treatment, [SDV.IMM.IMM]Life Sciences [q-bio]/Immunology/Immunotherapy, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, 3. Good health, Regimen, Nephrology, Rituximab, business, Nephrotic syndrome, medicine.drug, Kidney disease
Abstract

Introduction Patients with phospholipase A2 receptor (PLA2R)–associated membranous nephropathy and stage 4 or 5 chronic kidney disease are at high risk of end-stage kidney disease. In recent years, rituximab (RTX) emerged as a safe and efficient treatment for patients with PLA2R-associated membranous nephropathy. Whether its use is also appropriate in patients with an estimated glomerular filtration rate, Graphical abstract

Published
2020

50. KDIGO Controversies Conference on onco-nephrology: understanding kidney impairment and solid-organ malignancies, and managing kidney cancer

Author

Romano Danesi, Anitha Vijayan, Verônica Torres da Costa e Silva, Jolanta Malyszko, Maria Jose Soler Romeo, Maurizio Gallieni, Edgar A. Jaimes, David H. Vesole, Takeshi Matsubara, Christian Kollmannsberger, Camillo Porta, Motoko Yanagita, Walter M. Stadler, Dionysios Mitropoulos, Morie A. Gertz, Gernot Beutel, Ben Sprangers, Biruh Workeneh, Elena Zakharova, Aristotelis Bamias, Jerzy Chudek, Xiaohong Chen, Kenar D. Jhaveri, Kunitoshi Iseki, Amit Lahoti, Ali K. Abu-Alfa, Paul E. Stevens, Laura Cosmai, Michael Cheung, Mark A. Perazella, Eisei Noiri, Filippo De Stefano, Wolfgang C. Winkelmayer, Jan T. Kielstein, Abhijat Kitchlu, Manuel Macía, Mitchell H. Rosner, Farhad R. Danesh, Rumeyza Kazancioglu, David C. Wheeler, ladimír Tesař, Ondřej Viklický, Artur Jurczyszyn, Alicja Dębska-Ślizień, Jeremy R. Chapman, Pierre Ronco, Hatem Amer, Germaine Wong, Yang Li, Petra Tesarova, and KAZANCIOĞLU, RÜMEYZA

Subjects
0301 basic medicine, Nephrology, medicine.medical_specialty, renal cell carcinoma, Palliative care, glomerular filtration rate, nephrotoxicity, oncology, 030232 urology & nephrology, Pharmacy, Malignancy, law.invention, 03 medical and health sciences, 0302 clinical medicine, law, Internal medicine, medicine, Intensive care medicine, Clinical pharmacology, business.industry, Cancer, medicine.disease, 030104 developmental biology, business, Kidney cancer, Kidney disease
Abstract

© 2020 Kidney Disease:Improving Global Outcomes (KDIGO)The association between kidney disease and cancer is multifaceted and complex. Persons with chronic kidney disease (CKD) have an increased incidence of cancer, and both cancer and cancer treatments can cause impaired kidney function. Renal issues in the setting of malignancy can worsen patient outcomes and diminish the adequacy of anticancer treatments. In addition, the oncology treatment landscape is changing rapidly, and data on tolerability of novel therapies in patients with CKD are often lacking. Caring for oncology patients has become more specialized and interdisciplinary, currently requiring collaboration among specialists in nephrology, medical oncology, critical care, clinical pharmacology/pharmacy, and palliative care, in addition to surgeons and urologists. To identify key management issues in nephrology relevant to patients with malignancy, KDIGO (Kidney Disease: Improving Global Outcomes) assembled a global panel of multidisciplinary clinical and scientific expertise for a controversies conference on onco-nephrology in December 2018. This report covers issues related to kidney impairment and solid organ malignancies as well as management and treatment of kidney cancer. Knowledge gaps, areas of controversy, and research priorities are described.

Published
2020

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