Search

Your search keyword '"Quartier A"' showing total 10,044 results
Start Over Author "Quartier A"
10,044 results on '"Quartier A"'

153. Type I interferon-mediated autoinflammation due to DNase II deficiency

Author

Mathieu P. Rodero, Alessandra Tesser, Eva Bartok, Gillian I. Rice, Erika Della Mina, Marine Depp, Benoit Beitz, Vincent Bondet, Nicolas Cagnard, Darragh Duffy, Michael Dussiot, Marie-Louise Frémond, Marco Gattorno, Flavia Guillem, Naoki Kitabayashi, Fabrice Porcheray, Frederic Rieux-Laucat, Luis Seabra, Carolina Uggenti, Stefano Volpi, Leo A H. Zeef, Marie-Alexandra Alyanakian, Jacques Beltrand, Anna Monica Bianco, Nathalie Boddaert, Chantal Brouzes, Sophie Candon, Roberta Caorsi, Marina Charbit, Monique Fabre, Flavio Faletra, Muriel Girard, Annie Harroche, Evelyn Hartmann, Dominique Lasne, Annalisa Marcuzzi, Bénédicte Neven, Patrick Nitschke, Tiffany Pascreau, Serena Pastore, Capucine Picard, Paolo Picco, Elisa Piscianz, Michel Polak, Pierre Quartier, Marion Rabant, Gabriele Stocco, Andrea Taddio, Florence Uettwiller, Erica Valencic, Diego Vozzi, Gunther Hartmann, Winfried Barchet, Olivier Hermine, Brigitte Bader-Meunier, Alberto Tommasini, and Yanick J. Crow

Subjects
Science
Abstract

Nucleic acid sensing is important to ensure that an innate immune response is only mounted against microbial nucleic acid. Here, the authors identify loss-of-function mutations in the DNASE2 gene that cause type I interferon-mediated autoinflammation due to enhanced systemic interferon signaling.

Published
2017
Full Text
View/download PDF

154. Sakramentalität der Lebensform. Monastische und Liturgische Theologie in der Praxis des Klosterlebens

Author

Thomas Quartier

Subjects
klosterliturgie, liturgische spiritualität, lebensform, sakramentalität, liturgische erfahrung, Philosophy. Psychology. Religion
Abstract

Was bedeutet Sakramentalität im monastischen Leben? In diesem Beitrag nähern wir uns dieser Frage anhand von Konzepten, die aus der liturgischen und der monastischen Theologie stammen. Sie dienen einer ‘Wiederentdeckung’ von Sakramentalität in der monastischen Lebensform und Liturgie. Um die theologische Konzepte mit der heutigen monastischen Praxis zu verbinden, haben wir Dialoge geführt, in denen Mönche nach ihren Erfahrungen gefragt wurden. Diese fanden im Rahmen eines Seminars mit internationalen Teilnehmern statt, in dem der Begriff Sakrament theoretisch und experientiell vertieft wurde. Der Feldforschung liegen zwölf Interviews (kollegiale Interviews) und vier Gruppengespräche (Collatio) zugrunde. Es zeigte sich, dass die monastische Lebensform durch die Bereiche Arbeiten, Lernen und Wohnen sakramental verstanden werden kann. Sie ist verantwortlich, offen und frei. Weiterhin hat die monastische Liturgie sakramentale Dimensionen durch die Bereiche Kirche, Tradition und Schöpfung. Die theologische Reflexion trägt zu einer existenziellen Wiederentdeckung von Sakramentalität in Lebensform und Liturgie bei.

Published
2017
Full Text
View/download PDF

155. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part two

Author

Olga Lomakina, Ekaterina Alekseeva, Sania Valieva, Tatiana Bzarova, Irina Nikishina, Elena Zholobova, Svetlana Rodionovskaya, Maria Kaleda, Yasuo Nakagishi, Masaki Shimizu, Mao Mizuta, Akihiro Yachie, Yuko Sugita, Nami Okamoto, Kousuke Shabana, Takuji Murata, Hiroshi Tamai, Eve M. Smith, Peng Yin, Andrea L. Jorgensen, Michael W. Beresford, on behalf of On behalf of the UK JSLE Cohort Study, Antonio Eleuteri, Beatrice Goilav, Laura Lewandowski, Angel Phuti, Dawn Wahezi, Tamar Rubinstein, Caroline Jones, Paul Newland, Stephen Marks, Rachel Corkhill, Diana Ekdawy, Clarissa Pilkington, Kjell Tullus, Chaim Putterman, Chris Scott, Antony C. Fisher, Andrea Jorgensen, Ezgi Deniz Batu, Can Kosukcu, Ekim Taskiran, Sema Akman, Kubra Ozturk, Betul Sozeri, Erbil Unsal, Zelal Ekinci, Yelda Bilginer, Mehmet Alikasifoglu, Seza Ozen, Hanna Lythgoe, Hermine I. Brunner, Gaurav Gulati, Jordan T. Jones, Mekibib Altaye, Jamie Eaton, Mark Difrancesco, Joo Guan Yeo, Jingyao Leong, Loshinidevi D/O Thana Bathi, Thaschawee Arkachaisri, Salvatore Albani, Nagla Abdelrahman, Michael W Beresford, Valentina Leone, UK JSLE study group supported by the National Institute of Health Research Clinical Research Network, Noortje Groot, D. Shaikhani, I. E. M. Bultink, M. Bijl, R. J. E. M. Dolhain, Y. K. O. Teng, E. Zirkzee, K. de Leeuw, R. Fritsch-Stork, S. S. M. Kamphuis, Rachael D. Wright, Reem Abdawani, Laila Al Shaqshi, Ibrahim Al Zakwani, Natali W. Gormezano, David Kern, Oriany L. Pereira, Gladys C. C. Esteves, Adriana M. Sallum, Nadia E. Aikawa, Rosa M. Pereira, Clovis A. Silva, Eloisa Bonfa, Jessica Beckmann, Nora Bartholomä, Nils Venhoff, Philipp Henneke, Ulrich Salzer, Ales Janda, Alina Lucica Boteanu, Sandra Garrote Corral, Alberto Sifuentes Giraldo, Mariluz Gámir Gámir, Antonio Zea Mendoza, Amra Adrovic, Reyhan Dedeoglu, Sezgin Sahin, Kenan Barut, Aida Koka, Funda Oztunc, Ozgur Kasapcopur, Ana Luisa Rodriguez-Lozano, Francisco Rivas-Larrauri, Silvestre García de la Puente, Andressa G. F. Alves, Maria F. D. A. Giacomin, Juliana Farhat, Alfésio L. F. Braga, Adriana M. E. Sallum, Lúcia M. D. A. Campos, Luiz A. A. Pereira, Ana J. D. F. C. Lichtenfels, Clóvis A. Silva, Sylvia C. L. Farhat, Banu Acar, Z. Birsin Ozcakar, Nilgün Çakar, Nermin Uncu, Gökçe Gür, Semanur Özdel, Fatoş Yalçınkaya, Christiaan Scott, Nicky Brice, Peter Nourse, Christine Arango, Angela C. Mosquera, Clara Malagon, Ana P. Sakamoto, Marco F. C. D. Silva, Ananadreia S. Lopes, Gleice C. S. Russo, Adriana E. M. Sallum, Katia Kozu, Eloisa Bonfá, Claudia Saad-Magalhães, Rosa M. R. Pereira, Claudio A. Len, Maria T. Terreri, Deepti Suri, Siyaram Didel, Amit Rawat, Surjit Singh, Despoina Maritsi, MArgarita Onoufriou, Olga Vougiouka, Maria Tsolia, Edi Paleka Bosak, Mandica Vidović, Mirta Lamot, Lovro Lamot, Miroslav Harjaček, Erika Van Nieuwenhove, Adrian Liston, Carine Wouters, Fatemeh Tahghighi, Vahid Ziaee, Seid-Reza Raeeskarami, Francisca Aguiar, Sandra Pereira, Mariana Rodrigues, Cláudia Moura, Gustavo Rocha, Hercília Guimarães, Iva Brito, Rita Fonseca, Gerd Horneff, Ariane Klein, Kirsten Minden, Hans-Iko Huppertz, Frank Weller-Heinemann, Jasmin Kuemmerle-Deschner, J-Peter Haas, Anton Hospach, BIKER collaborative group, Ricardo Menendez-Castro, Boris Huegle, Johannes-Peter Haas, Joost Swart, Gabriella Giancane, Francesca Bovis, Elio Castagnola, Andreas Groll, Daniel J. Lovell, Tom Wolfs, Michael Hofer, Violeta Panaviene, Susan Nielsen, Jordi Anton, Florence Uettwiller, Valda Stanevicha, Maria Trachana, Denise Pires Marafon, Constantin Ailioaie, Elena Tsitsami, Sylvia Kamphuis, Troels Herlin, Pavla Doležalová, Gordana Susic, Berit Flatø, Flavio Sztajnbok, Angela Pistorio, Alberto Martini, Nico Wulffraat, Nicolino Ruperto, Marco Gattorno, Antonio Brucato, Martina Finetti, George Lazaros, Silvia Maestroni, Mara Carraro, Davide Cumetti, Alessandra Carobbio, Monia Lorini, Alessandro Rimini, Renzo Marcolongo, Anna Valenti, Gian Luca Erre, Riccardo Belli, Fiorenzo Gaita, Maria Pia Sormani, Massimo Imazio, Mario Abinun, Nicola Smith, Tim Rapley, Flora McErlane, Lianne Kearsley-Fleet, Kimme L. Hyrich, Helen Foster, Nikolay Tzaribachev, Andrew Zeft, Rolando Cimaz, John Bohnsack, Thomas Griffin, Ruy Carrasco, Jason Dare, Ivan Foeldvari, Richard Vehe, Teresa Simon, Hermine Brunner, S. Verazza, S. Davì, A. Consolaro, A. Insalaco, V. Gerloni, R. Cimaz, F. Zulian, S. Pastore, F. Corona, G. Conti, P. Barone, M. Cattalini, E. Cortis, L. Breda, A. N. Olivieri, A. Civino, R. Podda, D. Rigante, F. La Torre, G. D’Angelo, M. Jorini, R. Gallizzi, M. C. Maggio, R. Consolini, A. De Fanti, M. G. Alpigiani, A. Martini, A. Ravelli, on behalf of Italian Pediatric Rheumatology Study Group, Aysenur Pac Kısaarslan, Zubeyde Gunduz, Ruhan Dusunsel, Ismail Dursun, Hakan Poyrazoglu, Ekaterina Kuchinskaya, Farida Abduragimova, Mikhail Kostik, Erik Sundberg, Soley Omarsdottir, Lena Klevenvall, Helena Erlandsson-Harris, Gokalp Basbozkurt, Ozge Erdemli, Dogan Simsek, Fatih Yazici, Yildirim Karsioglu, Aysen Tezcaner, Dilek Keskin, Huseyin Ozkan, Cengizhan Acikel, Erkan Demirkaya, Ilonka Orbán, Krisztina Sevcic, Valentin Brodszky, Emese Kiss, Ismaiel A. Tekko, Madeleine Rooney, James McElnay, Cliff Taggart, Helen McCarthy, Ryan F. Donnelly, Drug Delivery Group, Mary Slatter, Zohreh Nademi, Mark Friswell, Sharmila Jandial, Terence Flood, Sophie Hambleton, Andrew Gennery, Andrew Cant, Phoi-Ngoc Duong, Isabelle Koné-Paut, Giovanni Filocamo, María Luz Gamir, Helga Sanner, Laura Carenini, Mesut Topdemir, Yildirim Karslioglu, Faysal Gok, Nadezhda Tsurikova, Elena Ligostaeva, Navdha R. Ramchurn, O. Kostareva, I. Nikishina, S. Arsenyeva, S. Rodionovskaya, M. Kaleda, D. Alexeev, Ismail Dursun Dursun, Sara Murias, Estefania Barral, Rosa Alcobendas, Eugenia Enriquez, Agustin Remesal, Jaime de Inocencio, Tania M. Castro, Simone A. Lotufo, Tatjana Freye, Raffaella Carlomagno, Thomas Zumbrunn, Jan Bonhoeffer, Elvira Cannizzaro Schneider, Daniela Kaiser, Michaël Hofer, Véronique Hentgen, Andreas Woerner, Juvenile Inflammatory Rheumatism (JIR) Cohort, Tobias Schwarz, Jens Klotsche, Martina Niewerth, Gerd Ganser, ICON study group, Jerold Jeyaratnam, Nienke ter Haar, Donato Rigante, Fatma Dedeoglu, Ezgi Baris, Sebastiaan Vastert, Joost Frenkel, Jonathan S. Hausmann, Kathleen G. Lomax, Ari Shapiro, Karen L. Durrant, P. A. Brogan, M. Hofer, J. B. Kuemmerle-Deschner, B. Lauwerys, A. Speziale, K. Leon, X. Wei, R. M. Laxer, Sara Signa, Marta Rusmini, Elena Campione, Sabrina Chiesa, Alice Grossi, Alessia Omenetti, Roberta Caorsi, Gianmaria Viglizzo, Isabella Ceccherini, Silvia Federici, Helen Lachmann, Nicola Ruperto, on behalf of PRINTO and Eurofever Registry, Federica Vanoni, on behalf of PRINTO and Eurofever Project, Sonia Melo Gomes, Ebun Omoyinmi, Juan I. Arostegui, Eva Gonzalez-Roca, Despina Eleftheriou, Nigel Klein, Paul Brogan, Stefano Volpi, Elettra Santori, Paolo Picco, Claudia Pastorino, Gillian Rice, Alessandra Tesser, Yanick Crow, Fabio Candotti, Ada B. Sinoplu, Gozde Yucel, Gizem Pamuk, Laura O. Damian, Cecilia Lazea, Mihaela Sparchez, Paulina Vele, Laura Muntean, Adriana Albu, Simona Rednic, Calin Lazar, Leonardo O. Mendonça, Alessandra Pontillo, Jorge Kalil, Fabio M. Castro, Myrthes T. Barros, Manuela Pardeo, Virginia Messia, Fabrizio De Benedetti, Antonella Insalaco, Giorgia Malighetti, Chiara Gorio, Francesca Ricci, Ilaria Parissenti, Paola Montesano, Barbara Bonafini, Veronica Medeghini, Marco Cattalini, Lucio Giordano, Giulia Zani, Rosalba Ferraro, Donatella Vairo, Silvia Giliani, Maria Cristina Maggio, Girolamo Luppino, Giovanni Corsello, Maria Isabel Gonzalez Fernandez, Berta Lopez Montesinos, Adriana Rodriguez Vidal, Juan I. Arostegui Gorospe, Inmaculada Calvo Penades, Nadia K. Rafiq, Karen Wynne, Khalid Hussain, Paul A. Brogan, Elizabeth Ang, Nicholas Ng, Ayla Kacar, Ozge Altug Gucenmez, Balahan Makay, Sevket Erbil Unsal, Yasin Sahin, Tufan Kutlu, Fugen Cullu-Cokugras, Hasret Ayyildiz-Civan, Tulay Erkan, Sana Al Zuhbi, Eiman Abdalla, Ricardo A. Russo, María M. Katsicas, Francesca Minoia, Angelo Ravelli, Sagar Bhattad, Anju Gupta, Vignesh Pandiarajan, Ritambhra Nada, Kaara Tiewsoh, Philip Hawkins, Dorota Rowczenio, Sarka Fingerhutova, Jana Franova, Leona Prochazkova, Eva Hlavackova, Pavla Dolezalova, Havva Evrengül, Selçuk Yüksel, Mustafa Doğan, Dolunay Gürses, Harun Evrengül, Silvia De Pauli, Serena Pastore, Anna Monica Bianco, Giovanni Maria Severini, Andrea Taddio, Alberto Tommasini, Svetlana O. Salugina, Evgeny Fedorov, Elena Kamenets, Ekaterina Zaharova, Tatiana Sleptsova, Ekaterina Alexeeva, Kirill Savostyanov, Alexander Pushkov, Tatyana Bzarova, Saniya Valieva, Rina Denisova, Kseniya Isayeva, Evgeniya Chistyakova, Margarita Soloshenko, Elena Kaschenko, Utako Kaneko, Chihaya Imai, Akihiko Saitoh, Vitor A. Teixeira, Filipa O. Ramos, Manuela Costa, Yonatan Butbul Aviel, Shafe Fahoum, Riva Brik, Zeynep Birsin Özçakar, Banu Acar Celikel, Fatos Yalcinkaya, Benedetta Schiappapietra, Sergio Davi’, Federica Mongini, Luisa Giannone, Cecilia Bava, Maria Giannina Alpigiani, Alessandro Consolaro, Dragana S. Lazarevic, Jelena Vojinovic, Jelena Basic, Valentina Muratore, Valentina Marzetti, Neus Quilis, Belen Serrano Benavente, Alessandra Alongi, Adele Civino, Lorenzo Quartulli, Giedre Januskeviciute, Pieter van Dijkhuizen, N. Groot, W. van Dijk, A. Kardolus, Raul Gutiérrez Suárez, Ellen B. Nordal, Veronika G. Rypdal, Lillemor Berntson, Maria Ekelund, Kristiina Aalto, Suvi Peltoniemi, Marek Zak, Mia Glerup, Ellen D. Arnstad, Anders Fasth, Marite Rygg, the Nordic Study Group of Pediatric Rheumatology (NoSPeR), Ana Catarina Duarte, Sandra Sousa, Lídia Teixeira, Ana Cordeiro, Mª José Santos, Ana Filipa Mourão, Maria José Santos, Mónica Eusébio, Ana Lopes, Filipa Oliveira-Ramos, Manuel Salgado, Paula Estanqueiro, José Melo-Gomes, Fernando Martins, José Costa, Carolina Furtado, Ricardo Figueira, Jaime C. Branco, João E. Fonseca, Helena Canhão, Ana F. Mourão, Maria Jose Santos, Andrea Coda, Samuel Cassidy, Kerry West, Gordon Hendry, Debra Grech, Julie Jones, Fiona Hawke, Davinder Singh Grewal, Charlene Foley, Orla Killeen, Emma MacDermott, Douglas Veale, Ursula Fearon, Dilek Konukbay, Ela Tarakci, Nilay Arman, Sezgin Şahin, Jane Munro, Esi Morgan, Meredith Riebschleger, Jennifer Horonjeff, Vibeke Strand, Clifton Bingham, Ma. Theresa M. Collante, Margarita Ganeva, Stefan Stefanov, Albena Telcharova, Dimitrina Mihaylova, Radoslava Saraeva, Reni Tzveova, Radka Kaneva, Adelina Tsakova, Katya Temelkova, GRANT Medical University, Sofia 68/, Maria Mercedes C. Picarelli, Luiz C. Danzmann, Florencia Barbé-Tuana, Lucas K. Grun, Marcus H. Jones, Marijan Frković, Karla Ištuk, Ika Birkić, Saša Sršen, Marija Jelušić, Alan Easton, Rachael Quarmby, Raju Khubchandani, Mercedes Chan, Radoslav Srp, Katerina Kobrova, Dana Nemcova, Jozef Hoza, Michal Uher, Melania Saifridova, Lenka Linkova, Sirirat Charuvanij, Isree Leelayuwattanakul, Thita Pacharapakornpong, Sakda A.-O. Vallipakorn, Butsabong Lerkvaleekul, Soamarat Vilaiyuk, Stefano Lanni, Sergio Davì, Randy Q. Cron, Chiara Passarelli, Elisa Pisaneschi, Antonio Novelli, Claudia Bracaglia, Ivan Caiello, Kathy de Graaf, Florence Guilhot, Walter Ferlin, Grant Schulert, Alexi A. Grom, Robert Nelson, Cristina de Min, Dirk Holzinger, Christoph Kessel, Ndate Fall, Alexei Grom, Wilco de Jager, Raffaele Strippoli, Anna Horne, Stephan Ehl, Sandra Ammann, Kai Lehmberg, Karin Beutel, Dirk Foell, AnnaCarin Horne, Laura Pagani, Graciela Espada, Yi-jin Gao, Susan Shenoi, Sheila Weitzman, Giusi Prencipe, Antonia Pascarella, Walter G. Ferlin, Laurence Chatel, Philippe Jacqmin, Kathy De Graaf, Maria Ballabio, Zoë Johnson, Geneviève Lapeyre, Fabrizio de Benedetti, de Min Cristina, Hiroyuki Wakiguchi, Shunji Hasegawa, Reiji Hirano, Fumiko Okazaki, Tamaki Nakamura, Hidenobu Kaneyasu, Shouichi Ohga, Kazuko Yamazaki, Tomo Nozawa, Taichi Kanetaka, Shuichi Ito, Shumpei Yokota, Kirsty McLellan, Ishbel MacGregor, Neil Martin, Joyce Davidson, Sandra Hansmann, Andreas Eikelberg, Iris Haug, Sabrina Schuller, Susanne M. Benseler, Single Hub and Access point for paediatric Rheumatology in Europe (SHARE), Liliia S. Nazarova, Kseniia V. Danilko, Viktor A. Malievsky, Tatiana V. Viktorova, Angela Mauro, Angela Barnicoat, Jane Hurst, Nathalie Canham, Sandrine Lacassagne, Anastasia Wiener, Boris Hügle, Bernd Denecke, Ivan Costa-Filho, Johannes Peter Haas, Klaus Tenbrock, David Popp, Arjan Boltjes, Frank Rühle, Stefanie Herresthal, Femke van Wijk, Joachim Schultze, Monika Stoll, Luisa Klotz, Thomas Vogl, Johannes Roth, Estefania Quesada-Masachs, Daniel Álvarez de la Sierra, Marina Garcia Prat, Ana M. Marín Sánchez, Ricardo Pujol Borrell, Sara Marsal Barril, Mónica Martínez Gallo, Consuelo Modesto Caballero, Iryna Chyzheuskaya, Lyudmyla M. Byelyaeva, Rostislav M. Filonovich, Helena K. Khrustaleva, Larisa I. Zajtseva, Tamara M. Yuraga, Thomas Giner, Lukas Hackl, Julia Albrecht, Reinhard Würzner, Juergen Brunner, Marta Minute, Fulvio Parentin, Agostino Nocerino, Mette Nørgaard, Mikel Alberdi-Saugstrup, Marek S. Zak, Susan M. Nielsen, Ellen Nordal, Klaus G. Müller, Nordic Study Group of Pediatric Rheumatology (NoSPeR), Mojca Zajc Avramovič, Vita Dolžan, Nataša Toplak, Tadej Avčin, N. Ruperto, D. J. Lovell, C. Wallace, M. Toth, I. Foeldvari, J. Bohnsack, D. Milojevic, C. Rabinovich, D. Kingsbury, K. Marzan, P. Quartier, K. Minden, E. Chalom, G. Horneff, R. M. Kuester, J. Dare, M. Heinrich, H. Kupper, J. Kalabic, H. I. Brunner, on behalf of PRINTO and PRCSG, Ruben Burgos-Vargas, Tamas Constantin, Joke Dehoorne, Valda Stanevica, Katarzyna Kobusinska, Zbigniew Zuber, Richard Mouy, Ingrida Rumba-Rozenfelde, Chantal Job-Deslandre, Ronald Pederson, Jack Bukowski, Tina Hinnershitz, Bonnie Vlahos, Paula Keskitalo, Salla Kangas, Paula Vähäsalo, Raul A. Chavez Valencia, David Martino, Anne-Louise Ponsonby, Rachel Chiaroni-Clarke, Braydon Meyer, Roger C. Allen, Jonathan D. Akikusa, Jeffrey M. Craig, Richard Saffrey, Justine A. Ellis, Carol Wallace, Yosef Uziel, Gary Sterba, Rayfel Schneider, Ricardo Russo, Athimalaipet V. Ramanan, Jana Pachlopnik Schmid, Kim E Nichols, Paivi Miettunen, Toshiyuki Kitoh, Norman T. Ilowite, Jan-Inge Henter, Alexei A Grom, Edward M. Behrens, Tadej Avcin, Maurizio Aricò, Sriharsha Grevich, Peggy Lee, Sarah Ringold, Brian Leroux, Hannah Leahey, Megan Yuasa, Jessica Foster, Jeremy Sokolove, Lauren Lahey, William Robinson, Joshua Newson, Anne Stevens, Stephanie J. W. Shoop, Suzanne M. M. Verstappen, Wendy Thomson, Janet E. McDonagh, CAPS, Timothy Beukelman, Yuki Kimura, Marc Natter, Norm Ilowite, Kelly Mieszkalski, Grendel Burrell, Brian Best, Helen Bristow, Shannon Carr, Anne Dennos, Rachel Kaufmann, Laura Schanberg, for the CARRA Registry Investigators, Gabriele Simonini, Francesca Lancini, Margaux Gerbaux, Phu-Quoc Lê, Laurence Goffin, Valérie Badot, Céline La, Laure Caspers, François Willermain, Alina Ferster, Maria Ceci, Francesco Licciardi, Marco Turco, Francesca Santarelli, Davide Montin, Claudia Toppino, Clotilde Alizzi, Bruno Papia, Beatrice Vergara, Umberto Corpora, Luca Messina, Maria Tsinti, Vasiliko Dermentzoglou, Panagiotis Tziavas, Marija Perica, Lana Tambić Bukovac, Mustafa Çakan, Nuray Aktay Ayaz, Gonca Keskindemirci, Michael Lang, Catherine Laing, Susanne Benseler, Tommy Gerschman, Nadia Luca, Heinrike Schmeling, Anastasia Dropol, Jaymi Taiani, Nicole Johnson, Brian Rusted, Panagiota Nalbanti, Polyxeni Pratsidou, Grigoris Pardalos, Vasiliki Tzimouli, Anna Taparkou, Maria Stavrakidou, Fotios Papachristou, Florence Kanakoudi-Tsakalidou, Peter Bale, Emily Robinson, Jason Palman, Elizabeth Ralph, Kimberly Gilmour, Clare Heard, Lucy R. Wedderburn, Yara Barrense-Dias, Antonarakis Gregory, Dhouib Amira, Scolozzi Paolo, Hanquinet Sylviane, Hofer Michaël, Nataliya Panko, Salah Shokry, Liudmila Rakovska, Sally Pino, Adriana Diaz-Maldonado, Pilar Guarnizo, Sofia Torreggiani, Paolo Cressoni, Umberto Garagiola, Giancarla Di Landro, Giampietro Farronato, Fabrizia Corona, Samantha Bell, Parveen Bhatti, Lee Nelson, Beth A. Mueller, T. A. Simon, A. Baheti, N. Ray, Z. Guo, Anasuya Hazra, Thomas Stock, Ronnie Wang, Charles Mebus, Christine Alvey, Manisha Lamba, Sriram Krishnaswami, Umberto Conte, Min Wang, Daniel Kingsbury, Elena Koskova, Elzbieta Smolewska, Richard K. Vehe, Daniel Lovell, Tomohiro Kubota, Junko Yasumura, Toshitaka Kizawa, Masato Yashiro, Tsuyoshi Yamatou, Yuichi Yamasaki, Syuji Takei, Yoshifumi Kawano, Ulrika Järpemo Nykvist, Bo Magnusson, Rikard Wicksell, Karin Palmblad, Gunnar L. Olsson, Mohammadreza Modaressi, Mohammad-Hassan Moradinejad, Valentina Seraya, Alisa Vitebskaya, Veronica Moshe, Gil Amarilyo, Liora Harel, Phillip J Hashkes, Amir Mendelson, Noa Rabinowicz, Yonit Reis, Zane Dāvidsone, Arina Lazareva, Ruta Šantere, Dace Bērziņa, Valda Staņēviča, Giulia Camilla Varnier, Susan Maillard, Cristina Ferrari, Silvia Zaffarano, Juvenile Dermatomyositis Research Group and European Federation of Immunological Societies, Judith Wienke, Felicitas Bellutti Enders, Lucas L. van den Hoogen, Jorre S. Mertens, Timothy R. Radstake, Henny G. Hotten, Ruth Fritsch, Lucy Wedderburn, Kiran Nistala, Berent Prakken, Annet van Royen-Kerkhof, Mohammad Alhemairi, Mohammed Muzaffer, Pieter Van Dijkhuizen, Claire T. Deakin, Stefania Simou, Maria De Iorio, Qiong Wu, Tania Amin, Lee Dossetter, Juvenile Dermatomyositis Research Group (JDRG), Raquel Campanilho-Marques, Claire Deakin, Clarissa A. Pilkington, on behalf of Juvenile Dermatomyositis Research Group (JDRG), Silvia Rosina, Sirisucha Soponkanaporn, on behalf of the UK Juvenile Dermatomyositis Research Group (JDRG), Zehra S. Arıcı, Gökçen D. Tuğcu, Ezgi D. Batu, Hafize E. Sönmez, Deniz Doğru-Ersöz, Beril Talim, Nural Kiper, Seza Özen, Alexander Solyom, Ezgi Batu, John Mitchell, Ariana Kariminejad, Fatemeh Hadipour, Zahra Hadipour, Marta Torcoletti, Carlo Agostoni, Maja Di Rocco, Pranoot Tanpaiboon, Andrea Superti-Furga, Luisa Bonafé, Nur Arslan, Norberto Guelbert, Karoline Ehlert, Giedre Grigelioniene, Ratna Puri, Edward Schuchman, Pilar Gomez, Tatiana Gonzalez, Ricardo Yepez, Camilo Vargas, GRIP study group, Falcini Fernanda, Gemma Lepri, Alessandra Ferrari, Marco Matucci-Cerinic, Antonella Meini, Gian Marco Moneta, Emiliano Marasco, Rebecca Nicolai, Luisa Bracci-Laudiero, Olga Kopchak, Alexander Mushkin, Alexey Maletin, Catalina Mosquera, Rita A. Amorim, Juliana Molina, Gustavo Moreira, Flávia H. Santos, Melissa Fraga, Livia Keppeke, Vanessa M. Silva, Camila Hirotsu, Sergio Tufik, Maria Teresa Terreri, Vinícius L. Braga, Maria Beatriz Fonseca, Vania Schinzel, Maria Teresa R. Terreri, Liliana Jorge, Liana Guerra, Edson Amaro Junior, Maria Cristina Castiglione, Alessandra Tricarico, Emily Boulter, Andre Schultz, Kevin Murray, Fernanda Falcini, Stefano Stagi, Eleonora Bellucci, Ingrid H. R. Grein, Gecilmara Pileggi, Natália B. F. Pinto, Aline L. de Oliveira, Lyudmila Belyaeva, Rostislav Filonovich, Helena Khrustaleva, Larisa Zajtseva, Jaanika Ilisson, Chris Pruunsild, Olivier Gilliaux, Francis Corazza, Christophe Lelubre, on behalf of PANLAR Pediatric Rheumatology Study Group, Zoilo Morel, Claudia Saad-Magalhães C, Luis Lira, Mabel Ladino, Ruth Eraso, Ivonne Arroyo, Clovis Silva, Carlos Rose, and PANLAR Pediatric Rheumatology Study Group

Subjects
Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Published
2017
Full Text
View/download PDF

156. De Novo <scp>Gain‐Of‐Function</scp> Variations in <scp> LYN </scp> Associated With <scp>an Early‐Onset</scp> Systemic Autoinflammatory Disorder

Author

Camille Louvrier, Elma El Khouri, Martine Grall Lerosey, Pierre Quartier, Anne‐Marie Guerrot, Brigitte Bader Meunier, Julie Chican, Malaïka Mohammad, Eman Assrawi, Aphrodite Daskalopoulou, Angela Arenas Garcia, Bruno Copin, William Piterboth, Florence Dastot Le Moal, Sonia A. Karabina, Serge Amselem, and Irina Giurgea

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

To identify the molecular basis of a severe systemic autoinflammatory disorder (SAID) and define its main phenotypic features, and to functionally assess the sequence variations identified in LYN, a gene encoding a nonreceptor tyrosine kinase.We used targeted next-generation sequencing and in vitro functional studies of Lyn phosphorylation state and Lyn-dependent NF-κB activity after expression of recombinant Lyn isoforms carrying different sequence variations.We identified a de novo LYN variation (p.Tyr508His) in a patient presenting since birth with recurrent fever, chronic urticaria, atopic dermatitis, arthralgia, increased inflammatory biomarkers, and elevated plasma cytokine levels. We studied the consequences on Lyn phosphorylation state of the p.Tyr508His variation and of the 2 LYN variations reported so far (p.Tyr508Phe and p.Tyr508*), and found that all 3 variations prevent phosphorylation of residue 508 and lead to autophosphorylation of Tyr397. Additionally, these 3 LYN variations activate the NF-κB pathway. These results show a gain-of-function effect of the variations involving Tyr508 on Lyn activity.This study demonstrates the pathogenicity of the first 3 LYN variations identified in SAID patients and delineates the phenotypic spectrum of a disease entity characterized by severe, early-onset, systemic inflammatory disease affecting neonates with no family history of SAID. All 3 LYN variations affect the same tyrosine residue located in the C-terminus of Lyn, thereby demonstrating the critical role of this residue in the proper regulation of Lyn activity in humans.

Published
2022
Full Text
View/download PDF

158. Association of atypical skin manifestations at the onset of systemic juvenile idiopathic arthritis with difficult-to-treat disease: A retrospective multicenter study

Author

Laurye-Anne Eveillard, Pierre Quartier, Naim Ouldali, Brigitte Bader-Meunier, Florence Aeschlimann, Claire Abasq, Claire Ballot, Pauline Bouric, Alexandra Desdoits, Cécile Dumaine, Caroline Galeotti, Véronique Hentgen, Alain Lefevre-Utile, Aurélie Chausset, Thomas Hubiche, Ingrid Kupfer-Bessaguet, Stéphanie Leclerq-Mercier, Stéphanie Mallet, Isabelle Melki, Etienne Merlin, Juliette Miquel, Maryam Piram, Deborah Talmud, Nicolas Garcelon, Caroline Vinit, Anne Welfringer, Emmanuelle Bourrat, Ulrich Meinzer, Hôpital Robert Debré Paris, Hôpital Robert Debré, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Centre hospitalier Bretagne Atlantique (Morbihan) (CHBA), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Centre Hospitalier de Versailles André Mignot (CHV), Hôpital Jean Verdier [AP-HP], Centre d'Investigation Clinique [CHU Clermont-Ferrand] (CIC 1405), Institut National de la Santé et de la Recherche Médicale (INSERM)-Direction de la recherche clinique et de l’innovation [CHU Clermont-Ferrand] (DRCI), CHU Clermont-Ferrand-CHU Clermont-Ferrand, Université Côte d'Azur (UCA), Centre Hospitalier Intercommunal de Cornouaille (CHIC), Hôpital de la Timone [CHU - APHM] (TIMONE), Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Centre de recherche du CHU Sainte-Justine / Research Center of the Sainte-Justine University Hospital [Montreal, Canada], Université de Montréal (UdeM)-CHU Sainte Justine [Montréal], Centre Hospitalier Régional d'Orléans (CHRO), 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 Paris Cité (UPCité), Health data- and model- driven Knowledge Acquisition (HeKA), Inria de Paris, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), 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)-École Pratique des Hautes Études (EPHE), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)

Subjects
[SDV]Life Sciences [q-bio], Humans, Dermatology, Arthritis, Juvenile, Retrospective Studies
Abstract

International audience

Published
2022
Full Text
View/download PDF

159. Natur in der Schule

Author

Bosse, Ulrich, Devantié, Rainer, Gebhard, Ulrich, Goudarzi, Yasmin, Hoke, Torsten, Quartier, Ulrike, Rehr, Jess, and Voß, Ian

Subjects
BNE, Gesundheit, Themenschwerpunkt, Lernen, Naturerfahrung, Bildung, Erfahrung
Abstract

Die Idee des Forschungs- und Entwicklungsprojektes „Natur in der Schule“ fußt sowohl auf den langjährigen naturpädagogischen Erfahrungen der Laborschule Bielefeld und des Oberstufen-Kollegs als auch auf den intensiven Auseinandersetzungen mit dieser Thematik von Ulrich Gebhard und seiner Forschungsgruppe. Das Projekt hat das Ziel, die Bedeutung von Naturerfahrungen während der Schulzeit zu erforschen. Dabei sind sowohl die Wirkungen auf fachliche und überfachliche Lern- und Bildungsprozesse von Interesse als auch die Rolle von Natur als wichtiger Entwicklungsraum für Kinder und Jugendliche. Neben diesem zu erforschenden positiven Einfluss von Naturerfahrungen auf Lern-, Bildungs- und Entwicklungsprozesse werden ebenso die wohltuende Wirkung von Natur, der Einfluss auf soziale Beziehungen und Kompetenzen sowie die Bedeutung bzgl. der Ausbildung eines Nachhaltigkeitsbewusstseins in den Blick genommen. Den Ausgangspunkt der schulischen Naturbegegnungen stellen dabei die naturpädagogischen Prinzipien dar, welche auf theoretischen Grundlagen, pädagogischem Erfahrungswissen und empirischen Erkenntnissen basieren. Das Forschungsfeld sind die Bielefelder Versuchsschulen, in denen auch schon vor dem Vorhaben regelmäßige Naturzeiten zum Schulalltag gehörten. Derzeit werden diese hinsichtlich des Erkenntnisinteresses begleitet und beforscht. Die ersten Ergebnisse werden mit Spannung erwartet., Schule – Forschen – Entwickeln. Beiträge zur Forschung und Entwicklung der Laborschule Bielefeld, Bd. 1 (2022): Jahrbuch Laborschulforschung 2022

Published
2022

160. Targeting the chemokine receptor CXCR4 with histamine analog to reduce inflammation in juvenile arthritis

Author

Bekaddour, Nassima, primary, Smith, Nikaïa, additional, Beitz, Benoit, additional, Llibre, Alba, additional, Dott, Tom, additional, Baudry, Anne, additional, Korganow, Anne-Sophie, additional, Nisole, Sébastien, additional, Mouy, Richard, additional, Breton, Sylvain, additional, Bader-Meunier, Brigitte, additional, Duffy, Darragh, additional, Terrier, Benjamin, additional, Schneider, Benoit, additional, Quartier, Pierre, additional, Rodero, Mathieu P., additional, and Herbeuval, Jean-Philippe, additional

Published
2023
Full Text
View/download PDF

162. On the state-of-the-art of CFD simulations for wave energy converters within the open-source numerical framework of DualSPHysics

Author

Crespo, Alejandro, primary, Tagliafierro, Bonaventura, additional, Martínez-Estévez, Iván, additional, Domínguez, José M., additional, DeCastro, Maite, additional, Gómez-Gesteira, Moncho, additional, Altomare, Corrado, additional, Brito, Moises, additional, Bernardo, Francisco, additional, Ferreira, Rui M., additional, Capasso, Salvatore, additional, Viccione, Giacomo, additional, Quartier, Nicolas, additional, Stratigaki, Vasiliki, additional, Troch, Peter, additional, Simonetti, Irene, additional, Cappietti, Lorenzo, additional, Göteman, Malin, additional, Engström, Jens, additional, Clemente, Daniel, additional, Rosa-Santos, Paulo, additional, Taveira-Pinto, Francisco, additional, Bacelli, Giorgio, additional, Coe, Ryan, additional, Fourtakas, Georgios, additional, Rogers, Benedict, additional, and Stasnby, Peter, additional

Published
2023
Full Text
View/download PDF

164. Hyperphosphatemic Familial Tumoral Calcinosis With Galnt3 Mutation: Transient Response to Anti‐Interleukin‐1 Treatments

Author

Astrid Dauchez, Camille Souffir, Pierre Quartier, Geneviève Baujat, Karine Briot, and Christian Roux

Subjects
HYPERPHOSPHATEMIC FAMILIAL TUMORAL CALCINOSIS, ANTI‐INTERLEUKINE‐1 THERAPIES, ANAKINRA, CANAKINUMAB, Orthopedic surgery, RD701-811, Diseases of the musculoskeletal system, RC925-935
Abstract

ABSTRACT Hyperphosphatemic familial tumoral calcinosis (HFTC) is a rare autosomal recessive disease caused by mutations in genes involved in phosphate homeostasis and characterized by high serum phosphate concentration and occurrence of ectopic calcifications. Management of the disease includes lowering of phosphate concentration and, when clinically necessary, debulking surgery of calcifications. In addition, high inflammatory disease flares can occur. Our case is about a patient with GALNT3 mutation and several localizations of refractory calcinosis. Assuming HFTC acts like an auto‐inflammatory syndrome, we report the effect of anti‐interleukine‐1 therapies on the evolution of the disease. Anakinra (100 mg, then 200 mg subcutaneous daily) and canakinumab (300 mg every 4 weeks) were sequentially given to the patient. Anti‐IL‐1 therapy was effective in controlling inflammatory flares; however, it did not prevent extension of calcinosis. © 2019 The Authors. JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.

Published
2019
Full Text
View/download PDF

169. Anakinra in Patients With Systemic Juvenile Idiopathic Arthritis: Long-term Safety From the Pharmachild Registry

Author

Giancane, G, Papa, R, Vastert, S, Bagnasco, F, Swart, J, Quartier, P, Antón, J, Kamphuis, S, Sanner, H, Glerup, M, De Benedetti, F, Tsitsami, E, Remesal, A, Moreno, E, De Inocencio, J, Myrup, C, Pallotti, C, Koné-Paut, I, Franck-Larsson, K, Malmström, H, Cederholm, S, Pistorio, A, Wulffraat, N, Ruperto, N, Giancane G, Papa R, Vastert S, Bagnasco F, Swart JF, Quartier P, Antón J, Kamphuis S, Sanner H, Glerup M, De Benedetti F, Tsitsami E, Remesal A, Moreno E, De Inocencio J, Myrup C, Pallotti C, Koné-Paut I, Franck-Larsson K, Malmström H, Cederholm S, Pistorio A, Wulffraat N, Ruperto N, Giancane, G, Papa, R, Vastert, S, Bagnasco, F, Swart, J, Quartier, P, Antón, J, Kamphuis, S, Sanner, H, Glerup, M, De Benedetti, F, Tsitsami, E, Remesal, A, Moreno, E, De Inocencio, J, Myrup, C, Pallotti, C, Koné-Paut, I, Franck-Larsson, K, Malmström, H, Cederholm, S, Pistorio, A, Wulffraat, N, Ruperto, N, Giancane G, Papa R, Vastert S, Bagnasco F, Swart JF, Quartier P, Antón J, Kamphuis S, Sanner H, Glerup M, De Benedetti F, Tsitsami E, Remesal A, Moreno E, De Inocencio J, Myrup C, Pallotti C, Koné-Paut I, Franck-Larsson K, Malmström H, Cederholm S, Pistorio A, Wulffraat N, and Ruperto N

Abstract

Objective. To evaluate the long-term safety profile of anakinra in patients with systemic juvenile idiopathic arthritis (sJIA). Methods. Data from patients with sJIA enrolled in the Pharmachild registry (ClinicalTrials.gov: NCT03932344) prior to September 30, 2018, and treated with anakinra were analyzed. The study endpoints were the occurrence of non-serious adverse events (SAEs) of at least moderate severity and SAEs, including macrophage activation syndrome (MAS), and the duration of anakinra treatment with reasons for discontinuation. All endpoints were analyzed overall by 6-month time windows, and in different treatment sets represented by those patients treated continuously with anakinra for at least 12, 18, and 24 months (set-12, -18, and -24, respectively). Results. Three hundred six patients were enrolled. Of these patients, 46%, 34%, and 28% had been treated for at least 12, 18, and 24 months, respectively. Two hundred and one AEs, mostly represented by infections, were reported for 509.3 patient-years (PY) with an overall incidence rate (IR) of 39.5 per 100 PY. Among 56 SAEs (IR 11.0/100 PY), 23.2% were infections and 19.6% MAS episodes. The IR of AEs was higher during the first 6 months of anakinra treatment, followed by decreasing IRs in the long-term treatment sets. Treatment discontinuation occurred in 76% of patients, most frequently in the first 6 months, because of inefficacy (43%), remission (31%), or AEs/intolerance (15%). No deaths or malignancies occurred during anakinra treatment. Conclusion. The results of the present study confirm the long-term safety profile of anakinra in patients with sJIA and demonstrate an overall decreasing incidence of AEs over time.

Published
2022

170. Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial

Author

Ruperto, Nicolino, Pistorio, Angela, Oliveira, Sheila, Zulian, Francesco, Cuttica, Ruben, Ravelli, Angelo, Fischbach, Michel, Magnusson, Bo, Sterba, Gary, Avcin, Tadej, Brochard, Karine, Corona, Fabrizia, Dressler, Frank, Gerloni, Valeria, Apaz, Maria T, Bracaglia, Claudia, Cespedes-Cruz, Adriana, Cimaz, Rolando, Couillault, Gerard, Joos, Rik, Quartier, Pierre, Russo, Ricardo, Tardieu, Marc, Wulffraat, Nico, Bica, Blanca, Dolezalova, Pavla, Ferriani, Virginia, Flato, Berit, Bernard-Medina, Ana G, Herlin, Troels, Trachana, Maria, Meini, Antonella, Allain-Launay, Emma, Pilkington, Clarissa, Vargova, Veronika, Wouters, Carine, Angioloni, Simona, and Martini, Alberto

Published
2016
Full Text
View/download PDF

176. Contributors

Author

Bastian, Susan E.P., primary, Beckley, Jacqueline H., additional, Bellisle, France, additional, Bleibaum, Rebecca N., additional, Blumenthal, David, additional, Bodin Danielsson, Christina, additional, Boya, Ünal Ö., additional, Cardello, Armand V., additional, Classen, Edith, additional, Danner, Lukas, additional, Darcel, Nicolas, additional, Delarue, Julien, additional, Dougkas, Anestis, additional, Edwards, John S.A., additional, Garber, Lawrence L., additional, Giacalone, Davide, additional, Giboreau, Agnès, additional, Goisbault, I., additional, Hartmann, Christina, additional, Hartwell, Heather J., additional, Hehn, Patrick, additional, Herbeth, Nathalie, additional, Higgs, Suzanne, additional, Holm, Lotte, additional, Hyatt, Eva M., additional, Jaeger, Sara R., additional, Johnson, Trent E., additional, Kern, Martin J., additional, Kuesten, Carla Lynn, additional, Lageat, Thierry, additional, Lahne, Jacob, additional, Laureati, Monica, additional, Lion, René, additional, Lund, Thomas Bøker, additional, Lutsch, Dariah, additional, Mäkelä, Johanna, additional, Meiselman, Herbert L., additional, Niimi, Jun, additional, Niva, Mari, additional, Pagliarini, Ella, additional, Pessel, Frank, additional, Phan, Uyen Thuy Xuan, additional, Piqueras-Fiszman, Betina, additional, Poelman, Maartje P., additional, Porcherot, C., additional, Price, Sarah, additional, Quartier, Katelijn, additional, Ristic, Renata, additional, Ruddock, Helen, additional, Saulais, Laure, additional, Siegrist, Michael, additional, Spinelli, Sara, additional, Steenhuis, Ingrid H.M., additional, Thomas, Heather, additional, Vanrie, Jan, additional, Vignon-Mares, M.C., additional, Wansink, Brian, additional, and Zandstra, Elizabeth H., additional

Published
2019
Full Text
View/download PDF

180. Strategies for mainstreaming nature-based solutions in urban governance capacities in ten European cities

Author

Hölscher, Katharina, Frantzeskaki, Niki, Collier, Marcus John, Connop, Stuart, Kooijman, Esmee D., Lodder, Marleen, McQuaid, Siobhan, Vandergert, Paula, Xidous, Dimitra, Bešlagić, Lejla, Dick, Gillian, Dumitru, Adina, Dziubała, Agnieszka, Fletcher, Isobel, Adank, Cristian Garcia Espina, Vázquez, María González, Madajczyk, Natalia, Malekkidou, Eleni, Mavroudi, Maria, Loizou, Eleftherios, Osipiuk, Agnieszka, Pasic, Belma, González, Antonio Prieto, Quartier, Mien, Schepers, Selina, Suljević, Nermina, Trendafilov, Ivaylo, Van De Sijpe, Katrien, Velikova, Velichka, Vos, Peter, Hölscher, Katharina, Frantzeskaki, Niki, Collier, Marcus John, Connop, Stuart, Kooijman, Esmee D., Lodder, Marleen, McQuaid, Siobhan, Vandergert, Paula, Xidous, Dimitra, Bešlagić, Lejla, Dick, Gillian, Dumitru, Adina, Dziubała, Agnieszka, Fletcher, Isobel, Adank, Cristian Garcia Espina, Vázquez, María González, Madajczyk, Natalia, Malekkidou, Eleni, Mavroudi, Maria, Loizou, Eleftherios, Osipiuk, Agnieszka, Pasic, Belma, González, Antonio Prieto, Quartier, Mien, Schepers, Selina, Suljević, Nermina, Trendafilov, Ivaylo, Van De Sijpe, Katrien, Velikova, Velichka, and Vos, Peter

Abstract

This paper explores the institutional mainstreaming of nature-based solutions (NBS) to advance a process-based understanding about how to strategically develop the governance capacities needed for systemic, localised and inclusive NBS. To this end, it reports how policy officers in ten European cities have started to mainstream NBS by interacting with and changing incumbent governance arrangements when experimenting with novel governance processes and mechanisms to plan, deliver and steward NBS. Based on these activities of the policy officers, the analysis identifies three strategies, associated stepping stones and changes in governance conditions, to mainstream NBS in governance capacities: institutionalising (a) a systems’ approach to link NBS to policies, regulations, and departments across goals and sectors, (b) inclusive collaborations for localised and inclusive interventions, and (c) reflexivity and learning about how NBS interact with the (institutional, ecological, social, etc.) contexts and create impacts. The strategies illustrate institutional entrepreneurship in interacting with incumbent governance contexts, and how starting from NBS as a type of systemic innovation can promote broader shifts in urban governance arrangements.

Published
2023

181. On the state-of-the-art of CFD simulations for wave energy converters within the open-source numerical framework of DualSPHysics

Author

Crespo, Alejandro J., Tagliafierro, Bonaventura, Martınez-Estevez, Ivan, Domınguez, Jose M., deCastro, Maite, Gómez-Gesteira, Moncho, Altomare, Corrado, Brito, Moises, Bernardo, Francisco, Ferreira, Rui M., Capasso, Salvatore, Viccione, Giacomo, Quartier, Nicolas, Stratigaki, Vasiliki, Troch, Peter, Simonetti, Irene, Cappietti, Lorenzo, Göteman, Malin, Engström, Jens, Clemente, Daniel, Rosa-Santos, Paulo, Taveira-Pinto, Francisco, Bacelli, Giorgio, Coe, Ryan, Fourtakas, Georgios, Rogers, Benedict, Stansby, Peter, Crespo, Alejandro J., Tagliafierro, Bonaventura, Martınez-Estevez, Ivan, Domınguez, Jose M., deCastro, Maite, Gómez-Gesteira, Moncho, Altomare, Corrado, Brito, Moises, Bernardo, Francisco, Ferreira, Rui M., Capasso, Salvatore, Viccione, Giacomo, Quartier, Nicolas, Stratigaki, Vasiliki, Troch, Peter, Simonetti, Irene, Cappietti, Lorenzo, Göteman, Malin, Engström, Jens, Clemente, Daniel, Rosa-Santos, Paulo, Taveira-Pinto, Francisco, Bacelli, Giorgio, Coe, Ryan, Fourtakas, Georgios, Rogers, Benedict, and Stansby, Peter

Abstract

There are currently several types of devices capable of harnessing wave energy, exploiting a broad variety of physical transformation processes. These devices – known as Wave Energy Converters (WECs) – are developed to maximize their power output. However, there are still uncertainties about their response and survivability to loads induced by adverse environmental conditions, with a consequent increase of the Levelized Cost of Energy (LCOE), which prevents in fact their commercial diffusion. As evidenced by a large body of research, marine renewable energy devices need to have more robust design practices. To address this issue, we propose the CFD-based DualSPHysics toolbox as a support in the design stages. DualSPHysics is high-fidelity software inherently suited to numerically address most challenges posed by multiphysics simulations, which are required to reliably predict WEC response in situations well beyond operational conditions. It should be noted that WECs, generally, may be connected to the seabed and comprise mechanical systems named Power Take-Offs (PTO) used to convert the energy from waves into electricity or other usable energies. To reproduce these features, DualSPHysics benefits from coupling with the multiphysics library Project Chrono and the dynamic mooring model Moordyn+. In this work, the augmented DualSPHysics framework is utilised to simulate a range of very different types of WECs with a variety of elements, such as catenary connections, taut mooring lines, or linear and nonlinear PTO actuators. Version 5.2 of the open-source licensed code was recently released, making the numerical framework publicly available as one unit. This work aims to provide a numerical review of past applications, and to demonstrate how the same open-source code is able to simulate very different technologies. Specifically, this paper proposes routine modeling and validation procedures using the SPH-based solver DualSPHysics applied to five different WEC types: i)

Published
2023
Full Text
View/download PDF

182. Strategies for mainstreaming nature-based solutions in urban governance capacities in ten European cities

Author

International Development Studies, Planning Support Science, Hölscher, Katharina, Frantzeskaki, Niki, Collier, Marcus John, Connop, Stuart, Kooijman, Esmee D., Lodder, Marleen, McQuaid, Siobhan, Vandergert, Paula, Xidous, Dimitra, Bešlagić, Lejla, Dick, Gillian, Dumitru, Adina, Dziubała, Agnieszka, Fletcher, Isobel, Adank, Cristian Garcia Espina, Vázquez, María González, Madajczyk, Natalia, Malekkidou, Eleni, Mavroudi, Maria, Loizou, Eleftherios, Osipiuk, Agnieszka, Pasic, Belma, González, Antonio Prieto, Quartier, Mien, Schepers, Selina, Suljević, Nermina, Trendafilov, Ivaylo, Van De Sijpe, Katrien, Velikova, Velichka, Vos, Peter, International Development Studies, Planning Support Science, Hölscher, Katharina, Frantzeskaki, Niki, Collier, Marcus John, Connop, Stuart, Kooijman, Esmee D., Lodder, Marleen, McQuaid, Siobhan, Vandergert, Paula, Xidous, Dimitra, Bešlagić, Lejla, Dick, Gillian, Dumitru, Adina, Dziubała, Agnieszka, Fletcher, Isobel, Adank, Cristian Garcia Espina, Vázquez, María González, Madajczyk, Natalia, Malekkidou, Eleni, Mavroudi, Maria, Loizou, Eleftherios, Osipiuk, Agnieszka, Pasic, Belma, González, Antonio Prieto, Quartier, Mien, Schepers, Selina, Suljević, Nermina, Trendafilov, Ivaylo, Van De Sijpe, Katrien, Velikova, Velichka, and Vos, Peter

Published
2023

183. Reimagining the future for retail and service design theory and practices

Author

Quartier, Katelijn, Alexander, Bethan, Khan, Zakkiya, Murialdo, Francesca, Munster, Mia, Quartier, Katelijn, Alexander, Bethan, Khan, Zakkiya, Murialdo, Francesca, and Munster, Mia

Abstract

These proceedings were created by compiling the papers presented at the first colloquium of the Special Interest Group (SIG) Designing Retail & Service Futures from the Design Research Society (DRS). The SIG was established in 2021 under the guidance of the Design Research Society. The SIG strives to gain a better understanding of the value of design in the commercial sector, including disciplines, such as interior design, architecture, retail and hospitality, branding, marketing, strategic design, design management and consumer psychology. Design and its value have been a subject of study for many years and from many different disciplinary perspectives (ranging from product design to marketing, business economics, service design, management, environmental psychology, (interior)architecture, etc.). However, these perspectives have been developed in a fragmented way with discrete research methods and results that present limitations to practically applying these findings holistically across the inter-related fields of design, retail, and services. Recent developments, that have been accelerated by the pandemic and the current economic crisis, show that in practice, services are becoming integral to retail and vice versa. The consumers’ needs and the dedication of retailers to serve these needs have sparked new approaches that unite both service and retail design. Whether it be online or offline (or both), for a product or a service or an experience, or all together… It is only natural that the research community support the development of this field through furthering insights. This colloquium focuses on bringing together various disciplines to contribute their related knowledge and insights with the objective of calibrating terms and meanings that strive for consensus across disciplines related to retail and service design. This is to work towards knowledge and practice-based contributions that strive for a more holistic and encompassing retail and service design fu

Published
2023

184. Liturgische performance.

Author

Quartier, T.H. and Quartier, T.H.

Subjects
Center for Religion and Contemporary Society (CRCS), Onderzoek vanaf 01-01-2021.
Published
2023

185. Tegen de hoogmoed. Liturgisch leven.

Author

Quartier, T.H. and Quartier, T.H.

Subjects
Center for Religion and Contemporary Society (CRCS), Onderzoek vanaf 01-01-2021.
Published
2023

186. Je stem verheffen met Maria.

Author

Quartier, T.H. and Quartier, T.H.

Subjects
Center for Religion and Contemporary Society (CRCS), Onderzoek vanaf 01-01-2021.
Published
2023

187. Kloosterambachten. Een duurzaam signaal.

Author

Quartier, T.H. and Quartier, T.H.

Subjects
Parcum Pocket., Center for Religion and Contemporary Society (CRCS), Onderzoek vanaf 01-01-2021.
Published
2023

189. Geen feestmaal zonder vasten.

Author

Quartier, T.H. and Quartier, T.H.

Subjects
Center for Religion and Contemporary Society (CRCS), Onderzoek vanaf 01-01-2021.
Published
2023

191. Buffet en Rozenkrans.

Author

Quartier, T.H. and Quartier, T.H.

Subjects
Center for Religion and Contemporary Society (CRCS), Onderzoek vanaf 01-01-2021.
Published
2023

196. Patterns and determinants of response to novel therapies in juvenile and adult-onset polyarthritis.

Author

Triaille, Clément, Quartier, Pierre, Somer, Lien De, Durez, Patrick, Lauwerys, Bernard R, Verschueren, Patrick, Taylor, Peter C, and Wouters, Carine

Subjects
*BIOTHERAPY, *DRUG efficacy, *BIOMARKERS, *JUVENILE idiopathic arthritis, *INDIVIDUALIZED medicine, *PATIENT-centered care, *ANTIRHEUMATIC agents, *TREATMENT effectiveness, *RHEUMATOID arthritis, *AGE factors in disease, *DISEASE management, *ADULTS
Abstract

Biologic and targeted synthetic DMARDs (b/tsDMARDs) have revolutionized the management of multiple rheumatic inflammatory conditions. Among these, polyarticular JIA (pJIA) and RA display similarities in terms of disease pathophysiology and response pattern to b/tsDMARDs. Indeed, the therapeutic efficacy of novel targeted drugs is variable among individual patients, in both RA and pJIA. The mechanisms and determinants of this heterogeneous response are diverse and complex, such that the development of true 'precision'-medicine strategies has proven highly challenging. In this review, we will discuss pathophysiological, patient-specific, drug-specific and environmental factors contributing to individual therapeutic response in pJIA in comparison with what is known in RA. Although some biomarkers have been identified that stratify with respect to the likelihood of either therapeutic response or non-response, few have proved useful in clinical practice so far, likely due to the complexity of treatment–response mechanisms. Consequently, we propose a pragmatic, patient-centred and clinically based approach, i.e. personalized instead of biomarker-based precision medicine in JIA. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

197. Finite sample corrections for average equivalence testing.

Author

Boulaguiem, Younes, Quartier, Julie, Lapteva, Maria, Kalia, Yogeshvar N., Victoria‐Feser, Maria‐Pia, Guerrier, Stéphane, and Couturier, Dominique‐Laurent

Subjects
*ERROR rates, *CONFIDENCE intervals, *NITRATES
Abstract

Average (bio)equivalence tests are used to assess if a parameter, like the mean difference in treatment response between two conditions for example, lies within a given equivalence interval, hence allowing to conclude that the conditions have "equivalent" means. The two one‐sided tests (TOST) procedure, consisting in testing whether the target parameter is respectively significantly greater and lower than some pre‐defined lower and upper equivalence limits, is typically used in this context, usually by checking whether the confidence interval for the target parameter lies within these limits. This intuitive and visual procedure is however known to be conservative, especially in the case of highly variable drugs, where it shows a rapid power loss, often reaching zero, hence making it impossible to conclude for equivalence when it is actually true. Here, we propose a finite sample correction of the TOST procedure, the α$$ \alpha $$‐TOST, which consists in a correction of the significance level of the TOST allowing to guarantee a test size (or type‐I error rate) of α$$ \alpha $$. This new procedure essentially corresponds to a finite sample and variability correction of the TOST procedure. We show that this procedure is uniformly more powerful than the TOST, easy to compute, and that its operating characteristics outperform the ones of its competitors. A case study about econazole nitrate deposition in porcine skin is used to illustrate the benefits of the proposed method and its advantages compared to other available procedures. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

198. Drug upgrade: A complete methodology from old drug to new chemical entities using Nematic Protein Organization Technique.

Author

Eschbach, Judith, Wagner, Alain, Beahr, Corinne, Bekel, Akkiz, Korganow, Anne‐Sophie, Quartier, Angelique, Peter, Jean‐Christophe, and Eftekhari, Pierre

Subjects
MONONUCLEAR leukocytes, LIQUID chromatography-mass spectrometry, SURFACE plasmon resonance, POLYMER liquid crystals, DRUG repositioning
Abstract

Drug repurposing is used to propose new therapeutic perspectives. Here, we introduce "Drug Upgrade", that is, characterizing the mode of action of an old drug to generate new chemical entities and new therapeutics. We proposed a novel methodology covering target identification to pharmacology validation. As an old drug, we chose hydroxychloroquine (HCQ) for its well‐documented clinical efficacy in lupus and its side effect, retinal toxicity. Using the Nematic Protein Organization Technique (NPOT®) followed by liquid chromatography‐tandem mass spectrometry analyses, we identified myeloperoxidase (MPO) and alpha‐crystallin β chain (CRYAB) as primary and secondary targets to HCQ from lupus patients' peripheral blood mononuclear cells (PBMCs) and isolated human retinas. Surface plasmon resonance (SPR) and enzymatic assays confirmed the interaction of HCQ with MPO and CRYAB. We synthesized INS‐072 a novel analog of HCQ that increased affinity for MPO and decreased binding to CRYAB compared to HCQ. INS‐072 delayed cutaneous eruption significantly compared to HCQ in the murine MRL/lpr model of spontaneous lupus and prevents immune complex vasculitis in mice. In addition, long‐term HCQ treatment caused retinal toxicity in mice, unlike INS‐072. Our study illustrates a method of drug development, where new applications or improvements can be explored by fully characterizing the drug's mode of action. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

199. OP0166 LONG-TERM FOLLOW-UP OF PATIENTS ADMINISTERED EMAPALUMAB, AN ANTI–INTERFERON-Γ MONOCLONAL ANTIBODY, TO TREAT MACROPHAGE ACTIVATION SYNDROME (MAS) SECONDARY TO SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS (SJIA)

Author

De Benedetti, F., primary, Grom, A., additional, Brogan, P., additional, Bracaglia, C., additional, Pardeo, M., additional, Marucci, G., additional, Eleftheriou, D., additional, Papadopoulou, C., additional, Schulert, G., additional, Quartier, P., additional, Antón, J., additional, Laveille, C., additional, Frederiksen, R., additional, Asnaghi, V., additional, Ballabio, M., additional, Jacqmin, P., additional, and De Min, C., additional

Published
2023
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results