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1. Expert consensus guidelines for the genetic diagnosis of Alport syndrome

Author

Savige, J, Ariani, F, Mari, F, Bruttini, M, Renieri, A, Gross, O, Deltas, C, Flinter, F, Ding, J, Gale, DP, Nagel, M, Yau, M, Shagam, L, Torra, R, Ars, E, Hoefele, J, Garosi, G, and Storey, H

Subjects
Next generation sequencing, Collagen IV genes, Pathogenic variants, urologic and male genital diseases, Alport syndrome
Abstract

Recent expert guidelines recommend genetic testing for the diagnosis of Alport syndrome. Here, we describe current best practice and likely future developments. In individuals with suspected Alport syndrome, all three COL4A5, COL4A3 and COL4A4 genes should be examined for pathogenic variants, probably by high throughput-targeted next generation sequencing (NGS) technologies, with a customised panel for simultaneous testing of the three Alport genes. These techniques identify up to 95% of pathogenic COL4A variants. Where causative pathogenic variants cannot be demonstrated, the DNA should be examined for deletions or insertions by re-examining the NGS sequencing data or with multiplex ligation-dependent probe amplification (MLPA). These techniques identify a further 5% of variants, and the remaining few changes include deep intronic splicing variants or cases of somatic mosaicism. Where no pathogenic variants are found, the basis for the clinical diagnosis should be reviewed. Genes in which mutations produce similar clinical features to Alport syndrome (resulting in focal and segmental glomerulosclerosis, complement pathway disorders, MYH9-related disorders, etc.) should be examined. NGS approaches have identified novel combinations of pathogenic variants in Alport syndrome. Two variants, with one in COL4A3 and another in COL4A4, produce a more severe phenotype than an uncomplicated heterozygous change. NGS may also identify further coincidental pathogenic variants in genes for podocyte-expressed proteins that also modify the phenotype. Our understanding of the genetics of Alport syndrome is evolving rapidly, and both genetic and non-genetic factors are likely to contribute to the observed phenotypic variability.

Published
2019

2. Liquid-liquid transition revealed by quasi-static cooling of an ultra-viscous metallic liquid

Author

Hechler, S., Ruta, B., Stolpe, M., Pineda, E., Evenson, Z., Gross, O., Hembree, W., Bernasconi, A., Busch, R., and Gallino, I.

Subjects
Condensed Matter::Soft Condensed Matter, FOS: Physical sciences, Disordered Systems and Neural Networks (cond-mat.dis-nn), Condensed Matter - Disordered Systems and Neural Networks, Condensed Matter::Disordered Systems and Neural Networks
Abstract

Temperature-driven polyamorphism has been reported in various supercooled liquids and glasses. The dynamical and structural routes followed by the system during such crossovers are however not universal and appear to be related to intrinsic kinetic properties of the liquid. By combining x-ray photon correlation spectroscopy and high energy x-ray diffraction, we have followed the collective atomic motion and structural changes during quasi-static cooling of the Au49Cu26.9Si16.3Ag5.5Pd2.3 metallic glass-former. Due to this ultra-slow thermal protocol, the glass transition temperature is lowered far enough to reveal a liquid-liquid crossover in the ultra-viscous supercooled phase. This transition is usually hidden by vitrification and leads to a strong liquid characterized by increasing correlation at the medium range.

Published
2017
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3. Advances and unmet needs in genetic, basic and clinical science in Alport syndrome: Report from the 2015 International Workshop on Alport Syndrome

Author

Gross, O., Kashtan, C. E., Rheault, M. N., Flinter, F., Savige, J., Miner, J. H., Torra, R., Ars, E., Constantinou-Deltas, Constantinos D., Savva, Isavella, Perin, L., Renieri, A., Ariani, F., Mari, F., Baigent, C., Judge, P., Knebelman, B., Heidet, L., Lagas, S., Blatt, D., Ding, J., Zhang, Y., Gale, D. P., Prunotto, M., Xue, Y., Schachter, A. D., Morton, L. C. G., Blem, J., Huang, M., Liu, S., Vallee, S., Renault, D., Schifter, J., Skelding, J., Gear, S., Friede, T., Turner, A. N., Lennon, R., and Constantinou-Deltas, Constantinos D. [0000-0001-5549-9169]

Subjects
Chronic kidney disease, Hereditary kidney disease, Guidelines, Nephroprotection, Alport syndrome
Abstract

Alport syndrome (AS) is a genetic disease characterized by haematuric glomerulopathy variably associated with hearing loss and anterior lenticonus. It is caused by mutations in the COL4A3, COL4A4 or COL4A5 genes encoding the a3a4a5(IV) collagen heterotrimer. AS is rare, but it accounts for >1% of patients receiving renal replacement therapy. Angiotensin-converting enzyme inhibition slows, but does not stop, the progression to renal failure therefore, there is an urgent requirement to expand and intensify research towards discovering new therapeutic targets and new therapies. The 2015 International Workshop on Alport Syndrome targeted unmet needs in basic science, genetics and diagnosis, clinical research and current clinical care. In three intensive days, more than 100 international experts including physicians, geneticists, researchers from academia and industry, and patient representatives from all over the world participated in panel discussions and breakout groups. This report summarizes the most important priority areas including (i) understanding the crucial role of podocyte protection and regeneration, (ii) targeting mutations by new molecular techniques for new animal models and potential gene therapy, (iii) creating optimal interaction between nephrologists and geneticists for early diagnosis, (iv) establishing standards for mutation screening and databases, (v) improving widespread accessibility to current standards of clinical care, (vi) improving collaboration with the pharmaceutical/biotech industry to investigate new therapies, (vii) research in hearing loss as a huge unmet need in Alport patients and (viii) the need to evaluate the risk and benefit of novel (including 'repurposing') therapies on an international basis. © The Author 2016. 32 916 924 Cited By :1

Published
2017

4. The inflammasome: an integrated view

Author

Gross O Thomas CJ Guarda G Tschopp J

Subjects
integumentary system
Abstract

An inflammasome is a multiprotein complex that serves as a platform for caspase 1 activation and caspase 1 dependent proteolytic maturation and secretion of interleukin 1beta (IL 1beta). Though a number of inflammasomes have been described the NLRP3 inflammasome is the most extensively studied but also the most elusive. It is unique in that it responds to numerous physically and chemically diverse stimuli. The potent proinflammatory and pyrogenic activities of IL 1beta necessitate that inflammasome activity is tightly controlled. To this end a priming step is first required to induce the expression of both NLRP3 and proIL 1beta. This event renders the cell competent for NLRP3 inflammasome activation and IL 1beta secretion and it is highly regulated by negative feedback loops. Despite the wide array of NLRP3 activators the actual triggering of NLRP3 is controlled by integration a comparatively small number of signals that are common to nearly all activators. Minimally these include potassium efflux elevated levels of reactive oxygen species (ROS) and for certain activators lysosomal destabilization. Further investigation of how these and potentially other as yet uncharacterized signals are integrated by the NLRP3 inflammasome and the relevance of these biochemical events in vivo should provide new insight into the mechanisms of host defense and autoinflammatory conditions.

Published
2011
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6. Alport syndrome from bench to bedside: The potential of current treatment beyond RAAS blockade and the horizon of future therapies

Author

Gross, O., Perin, L., Constantinou-Deltas, Constantinos D., and Constantinou-Deltas, Constantinos D. [0000-0001-5549-9169]

Subjects
Paricalcitol, induced pluripotent stem cell, podocyte, MicroRNA-21, genotype phenotype correlation, Nephritis, Hereditary, Disease, Review, hydroxymethylglutaryl coenzyme A reductase inhibitor, hyperparathyroidism, Renin-Angiotensin System, Type IV collagen, angiotensin 1 receptor antagonist, cardiovascular disease, nephritis, renin angiotensin aldosterone system, dipeptidyl carboxypeptidase inhibitor, genetics, cerivastatin, kidney development, renin inhibitor, Proteinuria, hypercholesterolemia, adult, pathogenesis, drug effect, Discoidin domain receptor 1, unfolded protein response, unclassified drug, priority journal, Nephrology, Chaperon therapy, paricalcitol, recurrent infection, medicine.symptom, medicine.drug, Adult, Collagen Type IV, medicine.medical_specialty, microRNA 21, stem cell transplantation, Angiotensin Receptor Antagonists, Kidney fibrosis, collagen type 4, medicine, Humans, human, Alport syndrome, Intensive care medicine, collagen receptor, Transplantation, Hyperparathyroidism, nonhuman, business.industry, medicine.disease, embryonic stem cell, Blockade, very late activation antigen 1, angiotensin receptor antagonist, very late activation antigen 2, Immunology, Tubulointerstitial fibrosis, glomerulus basement membrane, prognosis, proteinuria, business, aldosterone antagonist
Abstract

The hereditary type IV collagen disease Alport syndrome (AS) always leads to end-stage renal failure. Yesterday, for the past 90 years, this course was described as 'inevitable'. Today, RAAS blockade has changed the 'inevitable' course to a treatable disease. Tomorrow, researchers hope to erase the 'always' from 'always leads to renal failure' in the textbooks. This review elucidates therapeutic targets that evolve from research: (i) kidney embryogenesis and pathogenesis (ii) phenotypegenotype correlation and the role of collagen receptors and podocytes (iii) the malfunctioning Alport-GBM (iv) tubulointerstitial fibrosis (v) the role of proteinuria in pathogenesis and prognosis and (vi) secondary events such as infections, hyperparathyroidism and hypercholesterolaemia. Therefore, moderate lifestyle, therapy of bacterial infections, Paricalcitol in adult patients with hyperparathyroidism and HMG-CoAreductase inhibitors in adult patients with dyslipoproteinemia might contribute to a slower progression of AS and less cardiovascular events. In the future, upcoming treatments including stem cells, chaperon therapy, collagen receptor blockade and anti-microRNA therapy will expand our perspective in protecting the kidneys of Alport patients from further damage. This perspective on current and future therapies is naturally limited by our personal focus in research, but aims to motivate young scientists and clinicians to find a multimodal cure for AS. © The Author 2014. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. 29 iv124 iv130 Cited By :14

Published
2014

14. The death domain-containing protein Unc5CL is a novel MyD88-independent activator of the pro-inflammatory IRAK signaling cascade

Author

Heinz LX Rebsamen M Rossi DC Staehli F Schroder K Quadroni M Gross O Schneider P Tschopp J

Abstract

The family of death domain (DD) containing proteins are involved in many cellular processes including apoptosis inflammation and development. One of these molecules the adapter protein MyD88 is a key factor in innate and adaptive immunity that integrates signals from the Toll like receptor/interleukin (IL) 1 receptor (TLR/IL 1R) superfamily by providing an activation platform for IL 1R associated kinases (IRAKs). Here we show that the DD containing protein Unc5CL (also known as ZUD) is involved in a novel MyD88 independent mode of IRAK signaling that culminates in the activation of the transcription factor nuclear factor kappa B (NF kappaB) and c Jun N terminal kinase. Unc5CL required IRAK1 IRAK4 and TNF receptor associated factor 6 but not MyD88 for its ability to activate these pathways. Interestingly the protein is constitutively autoproteolytically processed and is anchored by its N terminus specifically to the apical face of mucosal epithelial cells. Transcriptional profiling identified mainly chemokines including IL 8 CXCL1 and CCL20 as Unc5CL target genes. Its prominent expression in mucosal tissues as well as its ability to induce a pro inflammatory program in cells suggests that Unc5CL is a factor in epithelial inflammation and immunity as well as a candidate gene involved in mucosal diseases such as inflammatory bowel disease

15. Stem cell therapy for Alport syndrome: the hope beyond the hype

Author

Gross, O, Borza, D B, Andres, H J, Licht, C, Weber, M, Segerer, S, Torra, R, Gubler, M C, Heidet, L, Harvey, S, Cosgrove, D, Lees, G, Kashtan, C, Gregory, M, Savige, J, Ding, J, Thorner, P, Abrahamson, D R, Antignac, C, Tryggvason, K, Hudson, B, and Miner, J H

Subjects
3. Good health

18. The DESCARTES-Nantes survey of kidney transplant recipients displaying clinical operational tolerance identifies 35 new tolerant patients and 34 almost tolerant patients

Author

Michaela Prokopova, Friedrich Thaiss, Andries J. Hoitsma, Bruno Hurault de Ligny, Anja Mühlfeld, Séverine Martin, Oliver Gross, Władysław Sułowicz, Annick Massart, Judith Racapé, Miguel Angel Gentil Govantes, A. Yussim, Frieder Keller, Umberto Maggiore, Matthew Howse, Gian Benedetto Piredda, Ricardo Lauzurica, Magali Giral, Luis Antonio Jiménez del Cerro, Marie-Christine Moal, Tomas Reischig, François Glowacki, Jean-François Subra, Bénédicte Janbon, Consuelo De Biase, María José Pérez-Sáez, Marian Klinger, Goce Spasovski, Philippe Gatault, Gaetano La Manna, David Berglund, Cem Tugmen, Giovanni M. Frascà, Uyen Huynh-Do, Christophe Legendre, Annaïck Pallier, Christopher Dudley, Mélanie Chesneau, Laura Braun, Daniel Abramowicz, Karine Hadaya, Christian Noel, Evangeline Pillebout, Carmen Díaz-Corte, Julio Pascual, Ondrej Viklicky, Florence Villemain, Luigi Biancone, Ana Ramírez Puga, Marije C. Baas, Alain Le Moine, Marc Abramowicz, Frederike J. Bemelman, Rainer Oberbauer, Jean-Paul Soulillou, Nurhan Seyahi, Jadranka Buturović Ponikvar, Johan W. de Fijter, Maarten Naesens, Vania Cuna, Klemens Budde, Serhan Tuglular, Pierrick Guerif, Angel Alonso Hernandez, Piero Stratta, Arnaud Garnier, Hulya Colak, K. Clemente, Sophie Brouard, Marc Hazzan, Søren Schwartz Sørensen, Giuseppe Orlando, Daniel Serón, Luboslav Beňa, Quirino Lai, Francesco Pisani, Aisling E. Courtney, Alexandre Dufay, Mehmet Sukru Sever, Thomas Wekerle, Hervé Le Monies De Sagazan, Hakim Mazouz, Aljoša Kandus, Maria Carmen Cantarell, André Gaasbeek, Massart, A, Pallier, A, Pascual, J, Viklicky, O, Budde, K, Spasovski, G, Klinger ,M, Sever, MS, Sørensen, SS, Hadaya, K, Oberbauer, R, Dudley, C, De Fijter, JW, Yussim, A, Hazzan, M, Wekerle, T, Berglund, D, De Biase, C, Pérez-Sáez, MJ, Mühlfeld, A, Orlando, G, Clemente, K, Lai, Q, Pisani, F, Kandus, A, Baas, M, Bemelman, F, Ponikvar, JB, Mazouz ,H, Stratta, P, Subra, JF, Villemain, F, Hoitsma, A, Braun, L, Cantarell, MC, Colak, H, Courtney, A, Frasca, GM, Howse, M, Naesens, M, Reischig, T, Serón, D, Seyahi, N, Tugmen, C, Alonso Hernandez, A, Beňa, L, Biancone, L, Cuna, V, Díaz-Corte, C, Dufay, A, Gaasbeek, A, Garnier, A, Gatault, P, Gentil Govantes, MA, Glowacki, F, Gross, O, Hurault de Ligny, B, Huynh-Do, U, Janbon, B, Jiménez Del Cerro, LA, Keller, F, La Manna, Gaetano, Lauzurica, R, Le Monies De Sagazan, H, Thaiss, F, Legendre, C, Martin, S, Moal, MC, Noël, C, Pillebout, E, Piredda, GB, Puga, AR, Sulowicz, W, Tuglular, S, Prokopova, M, Chesneau, M, Le Moine, A, Guérif, P, Soulillou, JP, Abramowicz, M, Giral, M, Racapé, J, Maggiore, U, Brouard, S, Abramowicz, D, AII - Amsterdam institute for Infection and Immunity, Nephrology, Renal Unit [Brussels, Belgium] (ULB), Université libre de Bruxelles (ULB)-CUB Hôpital Erasme [Bruxelles, Belgium], Medical Genetics Department [Brussels, Belgium], Université libre de Bruxelles (ULB), Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Nephrology [Barcelona, Spain] (Hospital del Mar), Hospital del Mar [Barcelona, Spain], Department of Nephrology [Prague, Czech Republic] (Transplant Center), Institute for Clinical and Experimental Medicine (IKEM), Department of Nephrology [Berlin, Germany], Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], Department of Nephrology [Skopje, Macedonia], Ss. Cyril and Methodius University in Skopje, Nephrology and Transplantation Medicine [Wrocław, Poland], University of Wrocław [Poland] (UWr), Internal Medicine, Nephrology [Istanbul, Turkey], Istanbul School of Medicine [Istanbul, Turkey], Nephrology P [Copenhagen, Denmark], Rigshospitalet [Copenhagen], Copenhagen University Hospital-Copenhagen University Hospital, Nephrology and Transplantation [Geneva, Switzerland], Geneva University Hospitals - HUG [Switzerland], Department of Medicine III–Nephrology, Hypertension and Renal Transplantation [Linz, Austria], Krankenhaus Elisabethinen Linz [Linz, Austria], Richard Bright Renal Centre [Bristol, UK], Southmead Hospital [Bristol, UK]-North Bristol NHS Trust [Bristol, UK], Department of Nephrology [Leiden, The Netherlands], Leiden University Medical Center (LUMC), Department of Transplantation [Tel Aviv, Israël] (Rabin Medical Center), Rabin Medical Center [Tel Aviv, Israël]-Tel Aviv University Sackler School of Medicine [Tel Aviv, Israël], Département de Néphrologie [CHRU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Department of Surgery [Vienna, Austria] (Section of Transplantation Immunology), Medizinische Universität Wien = Medical University of Vienna, Section of Clinical Immunology [Uppsala, Sweden] (Department of Immunology, Genetics and Pathology), Uppsala University, UOS Trapianti Rene Pancreas [Parma, Italy] (Centro Trapianti di Parma), Azienda Ospedaliero-Universitaria di Parma, Department of Nephrology [Aachen, Germany], University Hospital Aachen, Section of Transplantation [Winston-Salem, NC, USA] (Department of Surgery), Wake Forest School of Medicine [Winston-Salem], Wake Forest Baptist Medical Center-Wake Forest Baptist Medical Center, U.O.C. Trapianti D’Organo [L’Aquila, Italy], Department of Nephrology [Ljubljana, Slovenia] (Renal Transplantation Centre Ljubljana), University Medical Centre Ljubljana [Ljubljana, Slovenia] (UMCL), Kidney Diseases [Nijmegen, The Netherlands], Radboudumc Nijmegen [The Netherlands], Renal Transplant Unit [Amsterdam, The Netherlands] (Department of Nephrology), Academic Medical Center [Amsterdam, Netherlands], Unité de Transplantation Rénale et Pancréatique [CHU Sud, Amiens] (Service de Néphrologie), CHU Amiens-Picardie, Department of Translational Medicine [Novara, Italy], Amedeo Avogadro University of Eastern Piedmont, Service de Néphrologie-Dialyse-Transplantation [Angers], Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), service de Néphrologie et Transplantation Rénale [CHU Strasbourg] (Hôpital de jour de Néphrologie), Centre Hospitalier Universitaire de Strasbourg (CHU de Strasbourg )-Nouvel Hôpital Civil - NHC [Strasbourg], Pediatric Nephrology [Barcelona, Spain] (Vall d’Hebron Hospital), Universitat Autònoma de Barcelona (UAB)-Vall d'Hebron University Hospital [Barcelona], Department of Nephrology [Izmir, Turkey], Tepecik Training and Research Hospital [Izmir, Turkey], Regional Nephrology Unit [Belfast, UK], Belfast City Hospital [Belfast, UK], Nefrologia, Dialisi e Trapianto di rene [Ancona, Italy], AO Torrette Umberto I [Ancona, Italy], Nephrology/Transplantation [Liverpool, UK], Royal Liverpool University Hospital [Liverpool, UK], Department of Nephrology and Renal Transplantation [Leuven, Belgium], University Hospitals Leuven [Leuven]-Catholic University Leuven, Nephrology Ward [Pilsen, Czech Republic] (Department of Internal Medicine), University Hospital Pilsen [Pilsen, Czech Republic], Istanbul University, Servicio de Nefrología, Hospital Universitario, A Coruña, Transplant Centre, University Hospital Louis Pasteur Kosice, University of Turin, St. Orsola University Hospital, University of Bologna, Hospital Universitario Central de Asturias (HUCA), Service Néphrologie [Roubaix], Hôpital Victor Provo, Leids Universitair Medisch Centrum [Leiden, The Netherlands], Néphrologie - Médecine Interne - Hypertension Pédiatrique, Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Service Néphrologie - Immunoclinique [CHRU Tours], Hôpital Bretonneau, Hospital Universitario Virgen del Rocío [Sevilla], Service de Néphrologie et Transplantation rénale [CHRU-lille], University Medicine Göttingen, Service de Néphrologie-Dialyse-Transplantation rénale [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), University of Bern [Bern, Switzerland] (University Hospital Bern ), Transplantation rénale [CHU Grenoble], Centre Hospitalier Universitaire [Grenoble] (CHU), Hospital General Universitario de Alicante, Universitätsklinikum Ulm - University Hospital of Ulm, Hospital Universitario Germans Trias I Pujol, University Hospital Hamburg-Eppendorf, Universitaetsklinikum Hamburg-Eppendorf = University Medical Center Hamburg-Eppendorf [Hamburg] (UKE), Université Paris Descartes - Paris 5 (UPD5), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'hémodialyse et de Néphrologie [Libourne], Hôpital Robert Boulin, CHRU - Service de néphrologie, dialyse et transplantation rénale, Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Département de Néphrologie et transplantation [Hôpital Saint Louis - APHP], Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Kidney Transplant Az. Osp. G. Brotzu, Hospital Universitario Insular de Gran Canaria, University Hospital in Krakow, Marmara School of Medicine Hastanesi, Institute of Transplantation Urology and Nephrology [CHU Nantes], Centre hospitalier universitaire de Nantes (CHU Nantes), Institut de Recherche Interdisciplinaire en Biologie Humaine et Moleculaire, Research Center of Epidemiology, Biostatistics and Clinical Research, Nephrology-Renal Transplantation Department, Université libre de Bruxelles (ULB)-Universitair Ziekenhuis Antwerp, ERA-EDTA-DESCARTES working group, the Fonds Erasme (research grant), the Fonds Carine Vyghen, the Fonds Horlait-Dapsens, the RTRS Fondation de Coopération Scientifique CENTAURE and the IHUCesti project., ANR-10-IBHU-0005,CESTI (TSI-IHU),Centre Européen des Sciences de la Transplantation et de l'Immunothérapie (TSI-IHU)(2010), Le Bihan, Sylvie, Instituts Hospitalo-Universitaires B - Centre Européen des Sciences de la Transplantation et de l'Immunothérapie (TSI-IHU) - - CESTI (TSI-IHU)2010 - ANR-10-IBHU-0005 - IBHU - VALID, Ss. Cyril and Methodius University in Skopje (UKIM), Tel Aviv University (TAU)-Rabin Medical Center [Tel Aviv, Israël], Università degli studi di Torino = University of Turin (UNITO), University of Bologna/Università di Bologna, Service Néphrologie, médecine interne et hypertension pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), CUB Hôpital Erasme [Bruxelles, Belgium]-Université libre de Bruxelles (ULB), and Hadaya, Karine

Subjects
0301 basic medicine, Nephrology, Graft Rejection, Male, medicine.medical_treatment, 030230 surgery, Kidney transplant, 0302 clinical medicine, Surveys and Questionnaires, Allograft survival, Kidney transplantation, ddc:616, Graft Survival/immunology, Survival Rate/trends, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Incidence, Graft Survival, Immunosuppression, operational tolerance, Transplantation, 3. Good health, Europe, Survival Rate, frequency, minimally immunosuppressed patients, Female, Hemodialysis, Graft Rejection/epidemiology/immunology/prevention & control, Homologous, Adult, medicine.medical_specialty, Ronyons -- Trasplantació -- Aspectes immunològics, Immunosuppression/methods, kidney transplantation, Europe/epidemiology, 03 medical and health sciences, Immune Tolerance/immunology, Internal medicine, medicine, Immune Tolerance, Humans, Transplantation, Homologous, Survival rate, Immunosuppression Therapy, graft survival, business.industry, medicine.disease, Kidney Transplantation, Transplant Recipients, Surgery, 030104 developmental biology, Operational tolerance, Human medicine, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

Background Kidney recipients maintaining a prolonged allograft survival in the absence of immunosuppressive drugs and without evidence of rejection are supposed to be exceptional. The ERA-EDTA-DESCARTES working group together with Nantes University launched a European-wide survey to identify new patients, describe them and estimate their frequency for the first time. Methods Seventeen coordinators distributed a questionnaire in 256 transplant centres and 28 countries in order to report as many operationally tolerant patients (TOL; defined as having a serum creatinine

Published
2015
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