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2. The genetic landscape and clinical spectrum of nephronophthisis and related ciliopathies

Author

Friederike Petzold, Katy Billot, Xiaoyi Chen, Charline Henry, Emilie Filhol, Yoann Martin, Marina Avramescu, Maxime Douillet, Vincent Morinière, Pauline Krug, Cécile Jeanpierre, Kalman Tory, Olivia Boyer, Anita Burgun, Aude Servais, Remi Salomon, Alexandre Benmerah, Laurence Heidet, Nicolas Garcelon, Corinne Antignac, Mohamad Zaidan, Sophie Saunier, Tania Attié-Bitach, Valerie Comier-Daire, Jean-Michel Rozet, Yaacov Frishberg, Brigitte Llanas, Michel Broyer, Nabil Mohsin, Marie-Alice Macher, Nicole Philip, Véronique Baudouin, Damian Brackman, Chantal Loirat, Marina Charbit, Maud Dehennault, Claude Guyot, Pierre Bataille, Mariet Elting, Georges Deschenes, Andrea Gropman, Geneviève Guest, Marie-France Gagnadoux, Philippe Nicoud, Pierre Cochat, Bruno Ranchin, Albert Bensman, Anne-Marie Guerrot, Bertrand Knebelmann, Ilmay Bilge, Danièle Bruno, Stéphane Burtey, Caroline Rousset Rouvière, Valérie Caudwell, Denis Morin, Hélène Dollfus, Anne Maisin, Christian Hamel, Eric Bieth, Sophie Gie, Judith Goodship, Gwenaelle Roussey, Hermine La Selve, Hubert Nivet, Lucie Bessenay, Mathilde Caillez, Jean Bernard Palcoux, Stéphane Benoît, Philippe Dubot, Marc Fila, Fabienne Giuliano, Daouya Iftene, Michele Kessler, Theresa Kwon, Anine Lahoche, Audrey Laurent, Anne-Laure Leclerc, David Milford, Thomas Neuhaus, Sylvie Odent, Philippe Eckart, Dominique Chauveau, Patrick Niaudet, Horacio Repetto, Sophie Taque, Alexandra Bruel, Alexandra Noel-Botte, Emma Allain Launay, Lisa Allard, Dany Anlicheau, Anne-Laure Adra, Arnaud Garnier, Arvind Nagra, Remy Baatard, Justine Bacchetta, Banu Sadikoglu, Christine Barnerias, Anne Barthelemy, Lina Basel, Nader Bassilios, Hedi Ben Maiz, Fatma Ben Moussa, Faïza Benmati, Romain Berthaud, Aurélia Bertholet, Dominique Blanchier, Jean Jacques Boffa, Karim Bouchireb, Ihab Bouhabel, Zakaria Boukerroucha, Guylhène Bourdat-Michel, Odile Boute, Karine Brochard, Roseline Caumes, Siham Chafai Elalaoui, Bernard Chamontin, Marie Caroline Chastang, Christine Pietrement, Christine Richer, Christophe Legendre, Karin Dahan, Fabienne Dalla-Vale, Damien Thibaudin, Maxime Dauvergne, Salandre Davourie, Martin Debeukelaer, Jean Daniel Delbet, Constantinos Deltas, Denis Graber, Nadège Devillars, Boucar Diouf, Martine Doco Fenzy, Jean-Luc André, Dominique Joly, Alan Fryer, Laetitia Albano, Elisabeth Cassuto, Aline Pincon, Ana Medeira, Annabelle Chaussenot, Anne Mensire-Marinier, Francois Bouissou, Stephane Decramer, Armand Bottani, Aurélie Hummel, Alexandre Karras, Avi Katz, Christine Azema, Bénédicte Janbon, Bernard Roussel, Claude Bonniol, Christiophe Mariat, Gérard Champion, Deborah Chantreuil, Nicolas Chassaing, Christiane Mousson, Christine Baudeau, Delphine Hafdar Cuntz, Cyril Mignot, Laurene Dehoux, Didier Lacombe, Thierry Hannedouche, Elodie Mérieau, Emmanuelle Charlin, Eric Gauthier, Florent Plasse, Stanislas Faguer, Fanny Lebas, Florence Demurger, Francesco Emma, François Cartault, Geneviève Dumont, Nathalie Godefroid, Vincent Guigonis, Sophie Hillaire, Jaap Groothoff, Jan Dudley, Noémie Jourde-Chiche, Khalil El Karoui, Saoussen Krid, Krier Coudert, Larbi Bencheick, Laurent Yver, Marie-Pierre Lavocat, Le Monies De Sagazan, Valerie Leroy, Lise Thibaudin, Liz Ingulli, Lorraine Gwanmesia, Lydie Burglen, Marie-Hélène Saïd-Menthon, Marta Carrera, Mathilde Nizon, Catherine Melander, Michel Foulard, Monique Blayo, Jacques Prinseau, Nadine Jay, Nathalie Brun, Nicolas Camille, François Nobili, Olivier Devuyst, Ouafa Ben Brahim, Paloma Parvex, Laurence Perrin Sabourin, Philippe Blanc, Philippe Vanhille, Pierre Galichon, Sophie Pierrepont, Vincent Planquois, Gwenaelle Poussard, Claire Pouteil Noble, Radia Allal, Raphaelle Bernard, Raynaud Mounet, Rémi Cahen, Renaud Touraine, Claire Rigothier, Amélie Ryckewaert, Mathieu Sacquepee, Salima El Chehadeh, Charlotte Samaille, Shuman Haq, Ari Simckes, Stéphanie Lanoiselée, Stephanie Tellier, Jean-François Subra, Sylvie Cloarec, Julie Tenenbam, Thomas Lamy, Valérie Drouin Garraud, Huguette Valette, Vanina Meyssonnier, Rosa Vargas-Poussou, Yves Snajer, Sandrine Durault, Emmanuelle Plaisier, Etienne Berard, Fadi Fakhouri, Ferielle Louillet, Paul Finielz, Michel Fischbach, Bernard Foliguet, Hélène Francois-Pradier, Florentine Garaix, Marion Gerard, Gianfranco Rizzoni, Brigitte Gilbert, Denis Glotz, Astrid Godron Dubrasquet, Jean-Pierre Grünfeld, Guillaume Bollee, Michelle Hall, Sverker Hansson, Damien Haye, Hélène Taffin, Friedhelm Hildebrandt, Maryvonne Hourmand, Hümya Kayserili, Ivan Tack, Marie Line Jacquemont, Jennifer Fabre-Teste, Cliff Kashtan, Kkoen Van Hoeck, Alexandre Klein, Yannick Knefati, Nine Knoers, Martin Konrad, Alain Lachaux, Isabelle Landru, Gilbert Landthaler, Philippe Lang, Patrick Le Pogamp, Tristan Legris, Catherine Didailler, Thierry Lobbedez, Loïc de Parscau, Lucile Pinson, Hervé Maheut, Marc Duval-Arnould, Marlène Rio, Marie-Claire Gubler, Pierre Merville, Guillaume Mestrallet, Maite Meunier, Karine Moreau, Jérôme Harambat, Graeme Morgan, Georges Mourad, Niksic Stuber, Odile Boespflug-Tanguy, Olivier Dunand, Olivier Niel, Nacera Ouali, Paolo Malvezzi, Pauline Abou Jaoude, Solenne Pelletier, Julie Peltier, M.B. Petersen, Philippe Michel, Philippe Rémy, Jean-Baptiste Philit, Valérie Pichault, Thierry Billette de Villemeur, Bernard Boudailliez, Bruno Leheup, Claire Dossier, Djamal-Dine Djeddi, Yves Berland, Bruno Hurault de Ligny, Susan Rigden, Christophe Robino, Annick Rossi, Sabine Sarnacki, Messaoud Saidani, Albane Brodin Sartorius, Elise Schäfer, Sztriha Laszlo, Marie-Christine Thouret, Angélique Thuillier-Lecouf, Howard Trachtman, Claire Trivin, Michel Tsimaratos, Rita Van Damme-Lombaerts, Marjolaine Willems, Michel Youssef, Ariane Zaloszyc, Alexis Zawodnik, and Marie-Julia Ziliotis

Subjects
Nephrology
Abstract

Nephronophthisis (NPH) is an autosomal-recessive ciliopathy representing one of the most frequent causes of kidney failure in childhood characterized by a broad clinical and genetic heterogeneity. Applied to one of the worldwide largest cohorts of patients with NPH, genetic analysis encompassing targeted and whole exome sequencing identified disease-causing variants in 600 patients from 496 families with a detection rate of 71%. Of 788 pathogenic variants, 40 known ciliopathy genes were identified. However, the majority of patients (53%) bore biallelic pathogenic variants in NPHP1. NPH-causing gene alterations affected all ciliary modules defined by structural and/or functional subdomains. Seventy six percent of these patients had progressed to kidney failure, of which 18% had an infantile form (under five years) and harbored variants affecting the Inversin compartment or intraflagellar transport complex A. Forty eight percent of patients showed a juvenile (5-15 years) and 34% a late-onset disease (over 15 years), the latter mostly carrying variants belonging to the Transition Zone module. Furthermore, while more than 85% of patients with an infantile form presented with extra-kidney manifestations, it only concerned half of juvenile and late onset cases. Eye involvement represented a predominant feature, followed by cerebellar hypoplasia and other brain abnormalities, liver and skeletal defects. The phenotypic variability was in a large part associated with mutation types, genes and corresponding ciliary modules with hypomorphic variants in ciliary genes playing a role in early steps of ciliogenesis associated with juvenile-to-late onset NPH forms. Thus, our data confirm a considerable proportion of late-onset NPH suggesting an underdiagnosis in adult chronic kidney disease.

Published
2023
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3. The Natural History of Clinical Operational Tolerance After Kidney Transplantation Through Twenty-Seven Cases

Author

Brouard, S., Pallier, A., Renaudin, K., Foucher, Y., Danger, R., Devys, A., Cesbron, A., Guillot-Guegen, C., Ashton-Chess, J., Le Roux, S., Harb, J., Roussey, G., Subra, J.-F., Villemain, F., Legendre, C., Bemelman, F.J., Orlando, G., Garnier, A., Jambon, H., Le Monies De Sagazan, H., Braun, L., Noël, C., Pillebout, E., Moal, M.-C., Cantarell, C., Hoitsma, A., Ranbant, M., Testa, A., Soulillou, J.-P., and Giral, M.

Published
2012
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4. The DESCARTES-Nantes survey of kidney transplant recipients displaying clinical operational tolerance identifies 35 new tolerant patients and 34 almost tolerant patients

Author

Massart, Annick, Pallier, Annaïck, Pascual, Julio, Viklicky, Ondrej, Budde, Klemens, Spasovski, Goce, Klinger, Marian, Sever, Mehmet Sukru, Sørensen, Søren Schwartz, Hadaya, Karine, Oberbauer, Rainer, Dudley, Christopher, De Fijter, Johan W., Yussim, Alexander, Hazzan, Marc, Wekerle, Thomas, Berglund, David, De Biase, Consuelo, Pérez-Sáez, María José, Mühlfeld, Anja, Orlando, Giuseppe, Clemente, Katia, Lai, Quirino, Pisani, Francesco, Kandus, Aljosa, Baas, Marije, Bemelman, Frederike, Ponikvar, Jadranka Buturovic, Mazouz, Hakim, Stratta, Piero, Subra, Jean-François, Villemain, Florence, Hoitsma, Andries, Braun, Laura, Cantarell, Maria Carmen, Colak, Hulya, Courtney, Aisling, Frasca, Giovanni Maria, Howse, Matthew, Naesens, Maarten, Reischig, Tomas, Serón, Daniel, Seyahi, Nurhan, Tugmen, Cem, Alonso Hernandez, Angel, Beňa, Luboslav, Biancone, Luigi, Cuna, Vania, Díaz-Corte, Carmen, Dufay, Alexandre, Gaasbeek, André, Garnier, Arnaud, Gatault, Philippe, Gentil Govantes, Miguel Angel, Glowacki, François, Gross, Oliver, Hurault de Ligny, Bruno, Huynh-Do, Uyen, Janbon, Bénédicte, Jiménez del Cerro, Luis Antonio, Keller, Frieder, La Manna, Gaetano, Lauzurica, Ricardo, Le Monies De Sagazan, Hervé, Thaiss, Friedrich, Legendre, Christophe, Martin, Séverine, Moal, Marie-Christine, Noël, Christian, Pillebout, Evangeline, Piredda, Gian Benedetto, Puga, Ana Ramírez, Sulowicz, Wladyslaw, Tuglular, Serhan, Prokopova, Michaela, Chesneau, Mélanie, Le Moine, Alain, Guérif, Pierrick, Soulillou, Jean-Paul, Abramowicz, Marc, Giral, Magali, Racapé, Judith, Maggiore, Umberto, Brouard, Sophie, and Abramowicz, Daniel

Published
2016
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5. Résistances à base de nanofils de silicium pour la détection de bactéries

Author

Salaün, Anne-Claire, Le Borgne, Brice, Pichon, Laurent, Jolivet-Gougeon, Anne, Martin, Sophie, Rogel, Regis, Le Monies de Sagazan, Olivier, Institut d'Électronique et des Technologies du numéRique (IETR), Nantes Université (NU)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS), Microbiologie : Risques Infectieux, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-CHU Pontchaillou [Rennes]-Faculté de Chirurgie Dentaire de Rennes-Faculté d'Odontologie-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Pichon, Laurent, Université de Nantes (UN)-Université de Rennes 1 (UR1), Université de Nantes (UN)-Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS), Université de Rennes (UR)-CHU Pontchaillou [Rennes]-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Université de Rennes - UFR d'Odontologie (UR Odontologie), and Université de Rennes (UR)-Université de Rennes (UR)

Subjects
[SPI.NANO] Engineering Sciences [physics]/Micro and nanotechnologies/Microelectronics, [SPI.NANO]Engineering Sciences [physics]/Micro and nanotechnologies/Microelectronics, ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2017

6. Low temperature SINWS based devices fabrication for flexible electronic applications

Author

Yang, Kai, Salaün, Anne-Claire, Coulon, Nathalie, Le Monies de Sagazan, Olivier, Pichon, Laurent, Institut d'Électronique et des Technologies du numéRique (IETR), Université de Nantes (UN)-Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS), Université de Nantes (UN)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS), and Nantes Université (NU)-Université de Rennes 1 (UR1)

Subjects
[SPI.NANO]Engineering Sciences [physics]/Micro and nanotechnologies/Microelectronics, ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2017

7. 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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8. Effectiveness of Cetuximab as First-Line Therapy for Patients With Wild-Type KRAS and Unresectable Metastatic Colorectal Cancer in Real-Life Practice: Results of the EREBUS Cohort

Author

Denis Smith, Antonio Sa Cunha, Jérémy Jové, Alise Le Monies de Sagazan, Régis Lassalle, Magali Rouyer, Eric Francois, Pernelle Noize, Cécile Droz-Perroteau, Alain Monnereau, Annie Fourrier-Réglat, Nicholas Moore, Philip Robinson, Bordeaux population health (BPH), and Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Oncology, Adult, Male, medicine.medical_specialty, Colorectal cancer, Cetuximab, Kaplan-Meier Estimate, medicine.disease_cause, Cohort Studies, Proto-Oncogene Proteins p21(ras), 03 medical and health sciences, 0302 clinical medicine, Antineoplastic Agents, Immunological, Internal medicine, medicine, Humans, 030212 general & internal medicine, Neoplasm Metastasis, Aged, PharmacoEpi-Drugs, Proportional hazards model, business.industry, Hazard ratio, Liver Neoplasms, Gastroenterology, EPICENE, Middle Aged, medicine.disease, Confidence interval, Progression-Free Survival, 3. Good health, Treatment Outcome, CIC1401, 030220 oncology & carcinogenesis, Cohort, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Female, KRAS, business, Colorectal Neoplasms, medicine.drug, Cohort study
Abstract

Introduction Few real-life data are available on cetuximab benefit. The EREBUS cohort was performed to assess metastases resection rate, use, safety, and survival outcomes in wild-type KRAS (Kirsten rat sarcoma viral oncogene) patients with initially unresectable metastatic colorectal cancer (mCRC) treated by cetuximab in real practice. Patients and Methods The study cohort comprised patients initiating cetuximab between January 2009 and December 2010 in 65 French centers, with initially unresectable mCRC and wild-type KRAS . Kaplan-Meier analysis estimated 24-month probability of metastases resection and progression-free survival, and 36-month overall survival (OS). Cox proportional hazards models investigated factors associated with survival outcomes. Results Among the 389 patients included, median age was 64 years, 67.4% were male, 77.9% had Eastern Cooperative Oncology Group performance status ≤ 1, and hepatic metastases were most frequent at baseline (n = 146 exclusively, n = 149 not exclusively, n = 94 nonliver only). Median duration of cetuximab use was 4.8 months. Metastases resection was performed in 106 patients (27.2%) (n = 60 liver exclusively, n = 33 not exclusively, n = 13 nonliver only). The 24-month probability (95% confidence interval) of metastases resection occurrence was 33.6% (28.5-39.3). Median progression-free survival was 9.2 (8.5-9.8) months for the total cohort and 13.0 (11.6-15.1) for those resected; median OS was 23.0 (20.6-26.3) months for the total cohort and was not reached after 36 months for those who were resected. The strongest factor associated with higher OS was metastases resection with complete remission (hazard ratio, 0.41; 95% confidence interval, 0.19-0.88). Conclusion This cohort study highlights in French real-life practice the benefit of cetuximab in first-line mCRC therapy, notably in case of metastases resection with complete remission.

Published
2017
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9. Les addictions dans les portfolios des internes de médecine générale de l’Université de Bordeaux : à propos des 131 portfolios de la session de septembre 2013

Author

Le Monies de Sagazan, Mathilde and UB -, BU Carreire

Subjects
[SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Médecine générale, Addiction, Interne, Portfolio
Abstract

Background: Practitioners are frequently confronted with addictive behaviors of their patients. The portfolio is the current means of validating the general medical status of specialty diploma in Bordeaux 2. It is composed of 12 long clinical cases (scripts) experienced by residents, complex and suitable to primary care and 10 short clinical cases (specific cases). Objective: To describe the management of addictions by general medicine residents and their perceptions through their portfolio. Method: Retrospective descriptive study from portfolios collected in the Department of General Medicine of the University of Bordeaux. The scripts and specific cases considered as addiction situations were analyzed on the NVIVO software. Results: the portfolios of 99% of the 132 residents (including 67% of women) were analyzed, 1571 scripts and 1310 specific cases. 92% have described at least 2 addiction scripts (total of 518 scripts (33%). The status of the 3 main substance use in all of the scripts is often stated but unsystematic: 27% for tobacco, 14% for alcohol and 4% for cannabis. 64% of 520 patients in selected scripts, are dependent or abusers. Residents use the terms dependence and abuse in 13% and 14% of the stories involved. They take into account at least partially 59% of addictive behaviors. For patients with dependence or abuse without withdrawal request, the resident has an educational approach in 22% of 132 patients without somatic complication and in 34% of the 119 patients with a complication. In 76% of the 80 patients in withdrawal, it offers medical treatment. Felt them more expressed by residents on management of addictive behaviors, are a lack of personal resources (43%), empathy (27%) and called into question (24%). Conclusion: The residents are a priori sensitized to addictive behaviors but seem to miss training. An action plan should be implemented., Contexte : Les médecins généralistes sont fréquemment confrontés aux conduites addictives de leurs patients. Le portfolio est le moyen actuel de validation du DES de médecine générale à Bordeaux 2. Il est composé de 22 cas cliniques, 12 longs (les scripts), vécus par les internes, complexes et adaptés aux soins primaires et 10 courts (les cas ponctuels). Objectif : Décrire la prise en charge des addictions par les internes de médecine générale et leurs perceptions via leur portfolio. Méthode : Etude descriptive rétrospective à partir des portfolios collectés au département de médecine générale de l’Université de Bordeaux. Les scripts et cas ponctuels considérés comme addictologiques ont été analysés sur le logiciel NVIVO. Résultats : Les portfolios de 99% des 132 internes (dont 67% de femmes) ont été analysés, soit 1571 scripts et 1310 cas ponctuels. 92% ont décrit au moins 2 scripts addictologiques (soit 518 scripts retenus (33%). Le statut de consommation des 3 principales substances psychoactives des patients de l’ensemble des scripts est souvent tracé mais est non systématique : 27% pour le tabac, 14% pour l’alcool et 4% pour le cannabis. 64% des 520 patients des scripts retenus, sont dépendants et/ou abuseurs. Les internes emploient les termes dépendance et abus dans 13% et 14% des récits concernés. Ils prennent en compte au moins partiellement 59% des conduites addictives. Pour les patients dépendants et/ou abuseurs sans demande de sevrage, l’interne a une démarche éducative dans 22% des 132 patients sans complication somatique et dans 34% des 119 patients avec une complication. Dans 76% des 80 patients en sevrage, il propose un traitement médical. Les ressentis les plus exprimés par les internes sur la prise en charge addictologique, sont un manque de ressources personnelles (43%), de l’empathie (27%) et une remise en question (24%). Conclusion : Les internes sont a priori sensibilisés aux situations addictologiques mais semblent manquer de formation. Un plan d’action devrait être mis en place.

Published
2016

10. Efficacité en vie réelle du traitement par injection intravitréenne de ranibizumab pour une baisse de l’acuité visuelle due à un œdème maculaire secondaire à une occlusion veineuse rétinienne : les cohortes BOREAL

Author

Blin, P., primary, Delcourt, C., additional, Glacet-Bernard, A., additional, Fajnkuchen, F., additional, Girmens, J.-F., additional, Lassalle, R., additional, Chartier, A., additional, Bernard, M.-A., additional, Diez, P., additional, Douina, N., additional, Le Monies de Sagazan, A., additional, Finzi, L., additional, Droz-Perroteau, C., additional, Grolleau, A., additional, Grelaud, A., additional, and Moore, N., additional

Published
2017
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11. Difficultés diagnostiques d’un thrombus de la crosse aortique

Author

H Le Monies de Sagazan, M Hoffmann, A. Talaszka, A Liesse, and P Samaille

Subjects
Gynecology, Aorta, medicine.medical_specialty, business.industry, Polyarteritis nodosa, Arterial disease, Vascular disease, Gastroenterology, medicine.disease, Aortic disease, Thrombosis, Embolism, medicine.artery, Internal Medicine, medicine, business, Vasculitis
Abstract

Resume Introduction. – La thrombose de la crosse aortique est une cause rare et souvent meconnue d’evenements emboliques peripheriques. Exegese. – Nous rapportons une observation de presentation tres atypique, puisque le tableau clinique initial etait evocateur d’une periarterite noueuse. C’est l’apparition secondaire de manifestations emboliques disseminees qui nous a permis d’evoquer le diagnostic de thrombus flottant de la crosse aortique. L’echocardiographie par voie transthoracique n’a pas ete contributive a deux reprises. Seule l’echocardiographie par voie transœsophagienne a permis la mise en evidence du thrombus, confirmee par la tomodensitometrie et l’imagerie par resonance magnetique de l’aorte thoracique. Sous traitement anticoagulant, la disparition complete du thrombus est obtenue au bout de six mois. Conclusion. – Entite rare, ce diagnostic est a ne pas meconnaitre dans le bilan d’evenements ischemiques peripheriques recidivants, meme pauci-symptomatiques, sous peine de lourdes sequelles fonctionnelles causees par un retard diagnostique important.

Published
2002
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12. Efficacité en vie réelle du traitement par injection intravitréenne de ranibizumab pour une baisse de l’acuité visuelle due à un œdème maculaire secondaire à une occlusion veineuse rétinienne : les cohortes BOREAL

Author

F. Fajnkuchen, A. Le Monies de Sagazan, M.-A. Bernard, P. Diez, Angela Grelaud, A. Glacet-Bernard, Cécile Delcourt, Cécile Droz-Perroteau, Régis Lassalle, A. Chartier, Nicholas Moore, N. Douina, Patrick Blin, A. Grolleau, J.-F. Girmens, and Laetitia Finzi

Subjects
Epidemiology, Public Health, Environmental and Occupational Health
Abstract

Objectif Evaluer les modalites d’utilisation et l’efficacite en vie reelle du traitement par injection intravitreenne (IVT) de ranibizumab chez des patients presentant une baisse de l’acuite visuelle (BAV) due a un œdeme maculaire secondaire a une occlusion veineuse retinienne (OM-OVR) qu’elle soit de branche (B) ou centrale (C), a la demande de la Haute Autorite de sante (HAS). Methode Etude de cohorte observationnelle nationale de patients initiant un traitement par IVT de ranibizumab pour une BAV due a un OM-OVR (320 patients attendus par pathologie, pour avoir une precision de ± 2 lettres), inclus et suivis pendant 24 mois par des ophtalmologistes specialistes de la retine (retinologues). Le critere principal d’evaluation est la variation de la meilleure acuite visuelle corrigee (MAVC) a six mois par rapport a l’inclusion. Les criteres secondaires sont la variation de la MAVC, celle de l’epaisseur de la zone centrale de la retine (ECR), la description des modalites du suivi ophtalmologique ainsi que les evenements indesirables au cours du suivi. Resultats Entre decembre 2013 et avril 2015, pour les cohortes B/C, 223/196 patients ont ete inclus dans l’etude et 207/180 (92,8/91,8 %) presentaient des donnees de suivi analysables a six mois. Les caracteristiques des patients etaient un âge moyen (ET) de 70,3 (11,1)/70,5 (14,4) ans avec 47,8/50,6 % d’hommes, des premiers symptomes apparus en moyenne depuis 4,4 (12,6)/3,0 (6,0) mois, 9,2/8,3 % des patients deja traites pour leur OM-OVR (4,8/0,0 % par laser maculaire, 6,3/7,8 % par corticotherapie intravitreenne, 1,0/1,1 % par un autre anti-VEGF), et 3,9/2,8 % avec une atteinte bilaterale. A l’inclusion, la MAVC moyenne etait de 54,7 (18,9)/40,5 (25,7) lettres d’ETDRS avec 13,5/8,3 % des patients qui presentaient une MAVC superieure a 70 lettres. L’ECR moyenne etait de 558 (178)/649 (216) μm. Au cours des trois premiers mois, 80,7/76,1 % des patients avaient recu les trois IVT recommandees (traitement d’induction). A 3 (± 1,0) mois de suivi, la variation moyenne de la MAVC [IC 95 %] par rapport a l’inclusion etait de 14,7 [12,4 a 17,1]/16,0 [12,1 a 19,8] lettres et l’evolution de l’ECR etait de −240 [−269 a −211]/−323 [−371 a −275] μm. A six mois de suivi, le nombre moyen d’IVT etait de 3,8 (1,2)/3,6 (1,2) par œil etudie et 59,4/56,1 % des patients avaient eu au moins une interruption des IVT de ranibizumab (52,7/38,3 % pour amelioration de la pathologie et 7,2/15,6 % pour manque d’efficacite). A 6 (± 1,5) mois de suivi, la variation moyenne de la MAVC par rapport a l’inclusion etait de 13,9 [11,5 a 16,3]/9,5 [5,5 a 13,5] lettres avec 43,5/30,6 % des patients presentant une MAVC superieure 70 lettres. En parallele, l’evolution de l’ECR etait de −223 [−254 a −192]/−264 [−311 a −217] μm. Conclusion Cette etude en vie reelle chez des patients initiant un traitement par IVT de ranibizumab dans l’OM-OVR montre une amelioration de l’acuite visuelle a trois mois proche de celle des essais cliniques pre-AMM (BRAVO/CRUISE), et un peu plus faible a six mois mais avec une intensite plus faible d’injections en vie reelle (3,8/3,6 dans BOREAL contre 6 dans BRAVO/CRUISE).

Published
2017
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13. Intraoperative decay profile of intact (1-84) parathyroid hormone in surgery for renal hyperparathyroidism—a consecutive series of 80 patients

Author

J. Lokey, Pierre VanHille, Michele Minuto, François Pattou, A. Mondragon-Sanchez, Philippe Foissac-Geroux, Barbara Mullineris, Henri Le Monies de Sagazan, Charles Proye, François Wambergue, and Christian Noel

Subjects
Adult, Male, Parathyroidectomy, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Parathyroid hormone, Resection, Preoperative level, Monitoring, Intraoperative, medicine, Humans, Aged, Hyperparathyroidism, Renal hyperparathyroidism, business.industry, Middle Aged, medicine.disease, Surgery, Dissection, Parathyroid Hormone, Kidney Failure, Chronic, Female, Hyperparathyroidism, Secondary, business, hormones, hormone substitutes, and hormone antagonists, Kidney disease
Abstract

Background. The utility of intraoperative parathyroid hormone (PTH) monitoring is unclear in the surgical management of renal hyperparathyroidism. Our goal was to define the normal pattern of decay during operation for renal hyperparathyroidism by using the rapid intact (1-84) parathyroid hormone (PTH) assay. Methods. Eighty consecutive patients underwent neck exploration for renal hyperparathyroidism. Intact PTH levels were monitored with a rapid immunochemiluminometric assay. Samples were assayed at the induction of anesthesia, after dissection before resection, and 20 and 40 minutes after resection. Follow-up ranged from 3 to 24 months. Results. Twenty minutes after resection, PTH levels remained many-fold supranormal. Seventy-seven patients (96%) were cured. Of these, 75 patients (94%) had PTH decay of more than 50% from the preoperative level; 74 (99%) were cured. Only 1 of 3 patients (33%) in whom the PTH level decreased less than 40% from the preoperative level was cured. Two patients had intermediate values and both were cured. Conclusions. The intraoperative decay of PTH during operation for renal hyperparathyroidism is slower than for patients with normal renal function. However, 20 minutes after resection, a decline to less than 50% of the preoperative level predicts cure, while a level greater than 60% predicts failure. (Surgery 2000;128:1029-34.)

Published
2000
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14. [Acute renal failure and 2,8-dihydroxyadeninuria]

Author

M, Hoffmann, A, Talaszka, J P, Bocquet, H, Le Monies de Sagazan, and M, Daudon

Subjects
Male, Recurrence, Adenine, Adenine Phosphoribosyltransferase, Humans, Urinary Calculi, Acute Kidney Injury, Aged
Abstract

We report the case of a 65 year old man, with a history of recurrent urolithiasis, who was referred for an acute renal failure. Investigations deny obstructive or glomerular involvement. 2,8-Dihydroxyadeninuria was diagnosed with the help of crystalluria. This rare metabolic disease is due to a deficiency of adenine phosphoribosyltransferase, a purine salvage enzyme. Allopurinol, a low purine diet and high fluid intake made possible the nearly entire regression of renal failure.

Published
2004

15. [Difficulties in diagnosing an aortic arch thrombus]

Author

M, Hoffmann, A, Talaszka, A, Liesse, P, Samaille, and H, Le Monies de Sagazan

Subjects
Male, Esophagus, Echocardiography, Aortic Diseases, Humans, Aorta, Thoracic, Thrombosis, Middle Aged, Tomography, X-Ray Computed, Magnetic Resonance Imaging
Abstract

Thrombosis of the aortic arch is a rare and often underdiagnosed source of peripherical arterial embolic events.We report a case with a non-typical initial clinical presentation of polyarteritis nodosa. A mobile thrombus in the aortic arch was secondarily suspected when disseminated arterial embolism appeared. Transthoracic echocardiography failed twice to diagnosticate the source of embolism. The diagnosis was only performed with transesophageal echocardiography and confirmed by computed tomography and magnetic resonance imaging of the thoracic aorta. The thrombus completely disappeared after six months of oral anticoagulant therapy.Although rare, this diagnosis mustn't be disregarded in an etiologic view of recurrent and disseminated peripherical ischemic events (even clinically silent ones) under penalty of detrimental functional consequences due to a delayed diagnosis.

Published
2002

16. EREBUS : conditions d’utilisation et efficacité du cétuximab en traitement de 1re ligne d’un cancer colorectal métastatique en vie réelle

Author

Fourrier-Réglat, A., primary, Noize, P., additional, Rouyer, M., additional, Mitry, E., additional, François, E., additional, Monnerau, A., additional, Sa-Cunha, A., additional, Bignon, E., additional, Le Monies de Sagazan, A., additional, Jové, J., additional, Lassalle, R., additional, Moore, N., additional, and Smith, D., additional

Published
2014
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20. [Silent kidney and carcinoid tumor of the appendix]

Author

G, Lemaire, M, Florin, H, Le Monies De Sagazan, J, Padonou, and J, Leroy

Subjects
Male, Serotonin, Appendiceal Neoplasms, Humans, Retroperitoneal Fibrosis, Carcinoid Tumor, Hydronephrosis, Middle Aged, Ureteral Obstruction
Published
1978

22. [Cutaneous atypical Mycobacterium infection with hematogenic dissemination]

Author

Boulle C, Eric BOULANGER, Talaszka A, and Le Monies de Sagazan H

Subjects
Humans, Mycobacterium Infections, Nontuberculous, Female, Nontuberculous Mycobacteria, Skin Diseases, Bacterial, Aged
Abstract

Non-tuberculous mycobacteria are ubiquitous non-pathogens except for subjects with deficient local or general defence systems. Nearly 80% of the cases observed in France occur in HIV+ patients. Lung, lymph node or skin infections usually simulate tuberculosis and diagnosis must be based on precise identification of the infectious strain. Recommended treatment of M. kansasii is now well established but protocols for other mycobacteria are still under evaluation. We observed a case presenting with persistent fever resistant to large spectrum antibiotics. This case occurred in a 75-year-old non-immunodepressed women who presented typical microcalcifications of the skin subsequent to blood stream dissemination.

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

Author

Maggiore, Umberto, Yussim, Alexander, Garnier, Arnaud, Gross, Oliver, Prokopova, Michaela, Le Monies De Sagazan, Hervé, Pascual, Julio, Gentil Govantes, Miguel Angel, Biancone, Luigi, Lai, Quirino, Abramowicz, Daniel, La Manna, Gaetano, Tuglular, Serhan, Bemelman, Frederike, Naesens, Maarten, Gaasbeek, André, Gatault, Philippe, Martin, Séverine, Frasca, Giovanni Maria, Colak, Hulya, Stratta, Piero, Seyahi, Nurhan, Keller, Frieder, Sulowicz, Wladyslaw, De Biase, Consuelo, Viklicky, Ondrej, Dudley, Christopher, Subra, Jean-François, Thaiss, Friedrich, Huynh-Do, Uyen, Sever, Mehmet Sukru, Hadaya, Karine, Noël, Christian, Racapé, Judith, Villemain, Florence, Guérif, Pierrick, Cantarell, Maria Carmen, Hurault De Ligny, Bruno, Berglund, David, Pérez-Sáez, María José, Kandus, Aljosa, Ponikvar, Jadranka Buturovic, Massart, Annick, Janbon, Bénédicte, De Fijter, Johan W, Howse, Matthew, Abramowicz, Marc, Glowacki, François, Klinger, Marian, Wekerle, Thomas, Mühlfeld, Anja, Braun, Laura, Pisani, Francesco, Díaz-Corte, Carmen, Legendre, Christophe, Chesneau, Mélanie, Jiménez Del Cerro, Luis Antonio, Clemente, Katia, Budde, Klemens, Tugmen, Cem, Le Moine, Alain, Spasovski, Goce, Lauzurica, Ricardo, Serón, Daniel, Reischig, Tomas, Hazzan, Marc, Hoitsma, Andries, Courtney, Aisling, Pillebout, Evangeline, Giral, Magali, Pallier, Annaïck, Cuna, Vania, Mazouz, Hakim, Brouard, Sophie, Baas, Marije, Sørensen, Søren Schwartz, Puga, Ana Ramírez, Dufay, Alexandre, Alonso Hernandez, Angel, Soulillo, Jean-Paul, Beňa, Luboslav, Moal, Marie-Christine, Piredda, Gian Benedetto, Oberbauer, Rainer, and Orlando, Giuseppe

Subjects
610 Medicine & health, 3. Good health
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

24. [Chester-Erdheim's disease. A case]

Author

Eric BOULANGER, Talaszka A, and Le Monies de Sagazan H

Subjects
Brain Diseases, Intertrigo, Exophthalmos, Humans, Female, Bone Diseases, Middle Aged, Tomography, X-Ray Computed, Histiocytosis, Diabetes Insipidus
Abstract

We report the 32nd case of a multivisceral form of Erdheim-Chester disease. This exceptional pathology is a diffuse xanthogranulomatosis which comes within the scope of histiocytosis. The originality of this case is due to cerebral localizations and to the fact that some symptoms have been observed for a long time: diabetes insipidus, exophthalmos and stubborn intertrigo.

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