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1,663 results on '"A. Lemelle"'

51. The Mucosally-Adherent Rectal Microbiota Contains Features Unique to Alcohol-Related Cirrhosis

Author

Ting-Chin David Shen, Scott G. Daniel, Shivali Patel, Emily Kaplan, Lillian Phung, Kaylin Lemelle-Thomas, Lillian Chau, Lindsay Herman, Calvin Trisolini, Aimee Stonelake, Emily Toal, Vandana Khungar, Kyle Bittinger, K. Rajender Reddy, and Gary D. Wu

Subjects
alcohol-related cirrhosis, gut microbiota, mucosal microbiome, rectal swab, Diseases of the digestive system. Gastroenterology, RC799-869
Abstract

Most studies examining correlations between the gut microbiota and disease states focus on fecal samples due to ease of collection, yet there are distinct differences when compared to samples collected from the colonic mucosa. Although fecal microbiota has been reported to be altered in cirrhosis, correlation with mucosal microbiota characterized via rectal swab has not been previously described in this patient population. We conducted a cross-sectional analysis using 39 stool and 39 rectal swabs from adult patients with cirrhosis of different etiologies and performed shotgun metagenomic sequencing. Bacterial growth studies were performed with Escherichia coli. Two asaccharolytic bacterial taxa, Finegoldia magna and Porphyromonas asaccharolytica, were increased in rectal swabs relative to stool (FDR < 0.01). Genomic analysis of the microbiome revealed 58 genes and 16 pathways that differed between stool and rectal swabs (FDR < 0.05), where rectal swabs were enriched for pathways associated with protein synthesis and cellular proliferation but decreased in carbohydrate metabolism. Although no features in the fecal microbiome differentiated cirrhosis etiologies, the mucosal microbiome revealed decreased abundances of E. coli and Enterobacteriaceae in alcohol-related cirrhosis relative to non-alcohol related cirrhosis (FDR < 0.05). In vitro bacterial culture studies showed that physiological concentrations of ethanol and its oxidative metabolites inhibited E. coli growth in a pH- and concentration-dependent manner. Characterization of the mucosally associated gut microbiome via rectal swab revealed findings consistent with amino acid/nitrogen abundance versus carbohydrate limitation in the mucosal microenvironment as well as unique features of alcohol-related cirrhosis possibly consistent with the influence of host-derived metabolites on the composition of mucosally adherent microbiota.

Published
2021
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52. Development of new fluorescent silica and multifunctional nanoparticles for bio-imaging and diagnostics

Author

Lemelle, Arnaud, Morgan, Sarah, and Tothill, I. E.

Subjects
615.84, Nanomedicine, imaging, sol-gel, nanoparticles, silica, iron oxide,, gold
Abstract

Silica nanoparticles are effective fluorophore carriers with high potential in imaging, diagnostics, and therapy. The particles are resistant to drastic change of environmental conditions (pH, temperature etc.) and insulate the dyes so as to protect them from photobleaching. Silica chemistry is also versatile and affords an easy modification of the particle composition and surface to integrate targeting ligands or to integrate other nanoparticles. Regardless of their advantages, there exists a lack of dye diversity in the literature that is connected to a low affinity for potential tools for biology and medicineThis thesis describes the development of an alternative method for the synthesis of fluorescent silica nanoparticles and their modification to incorporate iron oxide and gold. cont/d.

Published
2011

53. Surface contamination rates at different spatial scales in the Columbus module (ISS) during the MATISS campaigns

Author

Lemelle, Laurence, primary, Mottin, Eléonore, additional, Le Tourneau, Denis, additional, Rouquette, Sébastien, additional, Campagnolo, Lucie, additional, Thévenot, Cécile, additional, Maillet, Alain, additional, Barde, Sébastien, additional, Garre, Emmanuel, additional, Teisseire, Jérémie, additional, Fontelaye, Caroline, additional, Jousseaume, Vincent, additional, Pudda, Catherine, additional, Constantin, Olivier, additional, Marcoux, Pierre, additional, Nonglaton, Guillaume, additional, and Place, Christophe, additional

Published
2023
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55. A 10‐year experience in testicular tissue cryopreservation for boys under 18 years of age: What can be learned from 350 cases?

Author

Barraud‐Lange, Virginie, primary, Boissel, Nicolas, additional, Gille, Anne‐Sophie, additional, Jean, Camille, additional, Sitbon, Leslie, additional, Schubert, Benoit, additional, Yakouben, Karima, additional, Fahd, Mony, additional, Peycelon, Matthieu, additional, Paye‐Jaouen, Annabel, additional, Chalas, Céline, additional, Vanhaesebrouck, Alexis, additional, Doz, François, additional, Surun, Aurore, additional, Lemelle, Lauriane, additional, Sarnacki, Sabine, additional, Neven, Bénédicte, additional, Philippe‐Chomette, Pascale, additional, Dufour, Christelle, additional, Rigaud, Charlotte, additional, Leverger, Guy, additional, Tabone, Marie‐Dominique, additional, Irtan, Sabine, additional, Pondarée, Corinne, additional, Lezeau, Harry, additional, Lenaour, Gilles, additional, Sibony, Mathilde, additional, Comperat, Eva, additional, Brocheriou, Isabelle, additional, Wolf, Jean Philippe, additional, Dalle, Jean‐Hugue, additional, and Poirot, Catherine, additional

Published
2023
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56. Neurotrophic tropomyosin receptor kinase (NTRK) fusion positive tumors: a historical cohort analysis

Author

Lemelle, Lauriane, primary, Guillemot, Delphine, additional, Hermann, Anne-Laure, additional, Gauthier, Arnaud, additional, Carton, Matthieu, additional, Corradini, Nadège, additional, Rome, Angélique, additional, Berlanga, Pablo, additional, Jourdain, Anne, additional, Marie Cardine, Aude, additional, Jannier, Sarah, additional, Boutroux, Hélène, additional, Defachelles, Anne Sophie, additional, Aerts, Isabelle, additional, Geoerger, Birgit, additional, Karanian, Marie, additional, Doz, François, additional, Brisse, Hervé J, additional, Schleiermacher, Gudrun, additional, Delattre, Olivier, additional, Pierron, Gaëlle, additional, and Orbach, Daniel, additional

Published
2023
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57. Familial and syndromic lupus share the same phenotype as other early-onset forms of lupus

Author

Weill, Olivia, Decramer, Stéphane, Malcus, Christophe, Kassai, Behrouz, Rouvet, Isabelle, Ginhoux, Tiphanie, Crow, Yanick J., Rieux-Laucat, Fredéric, Soulas-Sprauel, Pauline, Pagnier, Anne, Koné-Paut, Isabelle, Piram, Maryam, Galeotti, Caroline, Samaille, Charlotte, Reumaux, Héloïse, Lanteri, Aurélia, Dubois, Sandrine Morell, Lefebvre, Hélène, Burtey, Stéphane, Maurier, François, Carbasse, Aurélia, Lemelle, Irène, Meinzer, Ulrich, Despert, Véronique, Flodrops, Hugues, Fabien, Nicole, Ranchin, Bruno, Hachulla, Eric, Bader-Meunier, Brigitte, and Belot, Alexandre

Published
2017
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58. Le phénotype des lupus familiaux ou syndromiques n’est pas différent des autres formes de lupus juvéniles

Author

Weill, Olivia, Decramer, Stéphane, Malcus, Christophe, Kassai, Behrouz, Rouvet, Isabelle, Ginhoux, Tiphanie, Crow, Yanick, Rieux-Laucat, Fredéric, Soulas-Sprauel, Pauline, Pagnier, Anne, Koné-Paut, Isabelle, Piram, Maryam, Galeotti, Caroline, Samaille, Charlotte, Reumaux, Héloïse, Lanteri, Aurélia, Dubois, Sandrine Morell, Lefebvre, Hélène, Burtey, Stéphane, Maurier, François, Carbasse, Aurélia, Lemelle, Irène, Meinzer, Ulrich, Despert, Véronique, Flodrops, Hugues, Fabien, Nicole, Ranchin, Bruno, Hachulla, Eric, Bader-Meunier, Brigitte, and Belot, Alexandre

Published
2017
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63. What Should Students and Trainees Learn About Patient-Centered Documentation?

Author

Chong, Nubia, Bodic, Maria Mirabela, Steen, Peter, Salamanca, Ludwing, and LeMelle, Stephanie

Subjects
DOCUMENTATION, WOUNDS & injuries, LANGUAGE & languages, MEDICAL education, RESPECT, TEACHING methods, PATIENT-centered care, MEDICAL students, HOSPITAL medical staff, ELECTRONIC health records, PHYSICIAN-patient relations, CURRICULUM planning, ATTITUDES of medical personnel
Abstract

What clinicians document about patients can have important consequences for those patients. Paternalistic language in patients' health records is of specific ethical concern because it emphasizes clinicians' power and patients' vulnerabilities and can be demeaning and traumatizing. This article considers the importance of person-centered, trauma-informed language in clinical documentation and suggests strategies for teaching students and trainees documentation practices that express clinical neutrality and respect. [ABSTRACT FROM AUTHOR]

Published
2024
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64. A 10‐year experience in testicular tissue cryopreservation for boys under 18 years of age: What can be learned from 350 cases?

Author

Barraud‐Lange, Virginie, Boissel, Nicolas, Gille, Anne‐Sophie, Jean, Camille, Sitbon, Leslie, Schubert, Benoit, Yakouben, Karima, Fahd, Mony, Peycelon, Matthieu, Paye‐Jaouen, Annabel, Chalas, Céline, Vanhaesebrouck, Alexis, Doz, François, Surun, Aurore, Lemelle, Lauriane, Sarnacki, Sabine, Neven, Bénédicte, Philippe‐Chomette, Pascale, Dufour, Christelle, and Rigaud, Charlotte

Subjects
ALKYLATING agents, FERTILITY preservation, CLINICAL medicine, MULTIVARIATE analysis, UNIVARIATE analysis
Abstract

Background: A growing number of centers worldwide are preserving testicular tissue (TT) of young boys at risk of fertility loss to preserve their fertility. Data in this regard are scarce and experience sharing is essential to the optimization of the process. Objectives: This report of our 10‐year activity of pediatric fertility preservation (FP) has the objective to (1) improve knowledge regarding the feasibility, acceptability, safety, and potential usefulness of the procedure; (2) analyze the impact of chemotherapy on spermatogonia in the cryopreserved TT. Materials and methods: For this retrospective study of data prospectively recorded, we included all boys under 18 years of age referred to the FP consultation of our academic network between October 2009 and December 2019. Characteristics of patients and cryopreservation of testicular tissue (CTT) were extracted from the clinical database. Univariate and multivariate analyses were used to assess factors associated with the risk of absence of spermatogonia in the TT. Results: Three hundred and sixty‐nine patients (7.2 years; 0.5–17.0) were referred to the FP consultation for malignant (70%) or non‐malignant (30%) disease, of whom 88% were candidates for CTT, after a previous chemotherapy exposure (78%). The rate of recorded immediate adverse events was 3.5%, with painful episodes dominating. Spermatogonia were detected in the majority of TTs: 91.1% of those exposed to chemotherapy and 92.3% of those not exposed (p = 0.962). In multivariate analysis, the risk of absence of spermatogonia was almost three‐fold higher in boys > 10 years of age ([OR] 2.74, 95% CI 1.09–7.26, p = 0.035) and four‐fold higher in boys exposed to alkylating agents prior to CTT ([OR] 4.09, 95% CI 1.32–17.94, p = 0.028). Discussion/conclusion: This large series of pediatric FP shows that this procedure is well accepted, feasible, and safe in the short term, strengthening its place in the clinical care pathway of young patients requiring a highly gonadotoxic treatment. Our results demonstrate that CTT post‐chemotherapy does not impair the chance to preserve spermatogonia in the TT except when the treatment includes alkylating agents. More data on post‐CTT follow‐up are still required to ensure the long‐term safety and usefulness of the procedure. [ABSTRACT FROM AUTHOR]

Published
2024
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65. Targeted therapies in children with renal cell carcinoma (RCC): An International Society of Pediatric Oncology— Renal Tumor Study Group (SIOP-RTSG)-related retrospective descriptive study.

Author

Sprokkerieft, Julia, van der Beek, Justine N., Spreafico, Filippo, Selle, Barbara, Thebaud, Estelle, Chowdhury, Tanzina, Brok, Jesper, Ottóffy, Gábor, Xiaofei Sun, Ramírez Villar, Gema L., Sagoyan, Garik, Segers, Heidi, Doganis, Dimitrios, Serra, Annalisa, Lemelle, Lauriane, Graf, Norbert, Verschuur, Arnauld C., Tytgat, Godelieve A. M., and van den Heuvel-Eibrink, Marry M.

Subjects
RENAL cell carcinoma, PEDIATRIC oncology, KIDNEY tumors, CHILD patients, PROTEIN-tyrosine kinase inhibitors
Abstract

Introduction: Introduction: Renal cell carcinoma (RCC) is a very rare pediatric renal tumor. Robust evidence to guide treatment is lacking and knowledge on targeted therapies and immunotherapy is mainly based on adult studies. Currently, the International Society of Pediatric Oncology–Renal Tumor Study Group (SIOP-RTSG) 2016 UMBRELLA protocol recommends sunitinib for metastatic or unresectable RCC. Methods: This retrospective study describes the effects of tyrosine kinase inhibitors (TKI), anti-programmed cell death 1 (PD-(L)1) monoclonal antibodies, and immunotherapeutic regimens in advanced-stage and relapsed pediatric RCC. Results: Of the 31 identified patients (0–18 years) with histologically proven RCC, 3/31 presented with TNM stage I/II, 8/31 with TNM stage III, and 20/31 with TNM stage IV at diagnosis. The majority were diagnosed with translocation type RCC (MiT-RCC) (21/31) and the remaining patients mainly presented with papillary or clear-cell RCC. Treatment in a neoadjuvant or adjuvant setting, or upon relapse or progression, included mono- or combination therapy with a large variety of drugs, illustrating center specific choices in most patients. Sunitinib was often administered as first choice and predominantly resulted in stable disease (53%). Other frequently used drugs included axitinib, cabozantinib, sorafenib, and nivolumab; however, no treatment seemed more promising than sunitinib. Overall, 15/31 patients died of disease, 12/31 are alive with active disease, and only four patients had a complete response. The sample size and heterogeneity of this cohort only allowed descriptive statistical analysis. Conclusion: This study provides an overview of a unique series of clinical and treatment characteristics of pediatric patients with RCC treated with targeted therapies. [ABSTRACT FROM AUTHOR]

Published
2024
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67. Induction Therapy in Elderly Kidney Transplant Recipients With Low Immunological Risk

Author

Masset, Christophe, Boucquemont, Julie, Garandeau, Claire, Buron, Fanny, Morelon, Emmanuel, Girerd, Sophie, Ladrière, Marc, Mourad, Georges, Garrigue, Valérie, Cassuto, Elisabeth, Albano, Laetitia, Foucher, Yohann, Dantal, Jacques, Badet, Lionel, Brunet, Maria, Buron, Fanny, Cahen, Rémi, Daoud, Sameh, Fournie, Coralie, Grégoire, Arnaud, Koenig, Alice, Lévi, Charlène, Morelon, Emmanuel, Pouteil-Noble, Claire, Rimmelé, Thomas, Thaunat, Olivier, Delmas, Sylvie, Garrigue, Valérie, Le Quintrec, Moglie, Pernin, Vincent, Serre, Jean-Emmanuel, Eschwege, Pascal, Frimat, Luc, Girerd, Sophie, Hubert, Jacques, Ladriere, Marc, Laurain, Emmanuelle, Leblanc, Louis, Lecoanet, Pierre, Lemelle, Jean-Louis, Blancho, Gilles, Branchereau, Julien, Cantarovich, Diego, Chapelet, Agnès, Dantal, Jacques, Deltombe, Clément, Figueres, Lucile, Garandeau, Claire, Giral, Magali, Gourraud-Vercel, Caroline, Hourmant, Maryvonne, Karam, Georges, Kerleau, Clarisse, Meurette, Aurélie, Ville, Simon, Kandell, Christine, Moreau, Anne, Renaudin, Karine, Cesbron, Anne, Delbos, Florent, Walencik, Alexandre, Devis, Anne, Albano, Laeticia, and Cassuto, Elisabeth

Published
2020
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68. Conventional radiography in juvenile idiopathic arthritis: Joint recommendations from the French societies for rheumatology, radiology and paediatric rheumatology

Author

Marteau, Pauline, Adamsbaum, Catherine, Rossi-Semerano, Linda, De Bandt, Michel, Lemelle, Irène, Deslandre, Chantal, Tran, Tu Anh, Lohse, Anne, Solau-Gervais, Elisabeth, Sordet, Christelle, Pillet, Pascal, Bader-Meunier, Brigitte, Wipff, Julien, Gaujoux-Viala, Cécile, Breton, Sylvain, and Devauchelle-Pensec, Valérie

Published
2018
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69. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one

Author

F. De Benedetti, J. Anton, M. Gattorno, H. Lachmann, I. Kone-Paut, S. Ozen, J. Frenkel, A. Simon, A. Zeft, E. Ben-Chetrit, H. M. Hoffman, Y. Joubert, K. Lheritier, A. Speziale, J. Guido, Roberta Caorsi, Federica Penco, Alice Grossi, Antonella Insalaco, Maria Alessio, Giovanni Conti, Federico Marchetti, Alberto Tommasini, Silvana Martino, Romina Gallizzi, Annalisa Salis, Francesca Schena, Francesco Caroli, Alberto Martini, Gianluca Damonte, Isabella Ceccherini, Marco Gattorno, Marie-Louise Frémond, Carolina Uggenti, Lien Van Eyck, Isabelle Melki, Darragh Duffy, Vincent Bondet, Yoann Rose, Bénédicte Neven, Yanick Crow, Mathieu P. Rodero, Yvonne Kusche, Johannes Roth, Katarzyna Barczyk-Kahlert, Giovanna Ferrara, Annalisa Chiocchetti, Silvio Polizzi, Josef Vuch, Diego Vozzi, Anna Mondino, Erica Valencic, Serena Pastore, Andrea Taddio, Flavio Faletra, Umberto Dianzani, Ugo Ramenghi, Qing Zhou, Xiaomin Yu, Erkan Demirkaya, Natalie Deuitch, Deborah Stone, Wanxia Tsai, Amanda Ombrello, Tina Romeo, Elaine F. Remmers, JaeJin Chae, Massimo Gadina, Steven Welch, Seza Ozen, Rezan Topaloglu, Mario Abinun, Daniel L. Kastner, Ivona Aksentijevich, Donatella Vairo, Rosalba Monica Ferraro, Giulia Zani, Jessica Galli, Micaela De Simone, Marco Cattalini, Elisa Fazzi, Silvia Giliani, Ebun Omoyinmi, Ariane Standing, Dorota Rowczenio, Annette Keylock, Sonia Melo Gomes, Fiona Price-Kuehne, Sira Nanthapisal, Claire Murphy, Thomas Cullup, Lucy Jenkins, Kimberly Gilmour, Despina Eleftheriou, Helen Lachmann, Philip Hawkins, Nigel Klein, Paul Brogan, Anita Dhanrajani, Mercedes Chan, Stephanie Pau, Janet Ellsworth, Jaime Guzman, Florence A. Aeschlimann, Marinka Twilt, Simon W. Eng, Shehla Sheikh, Ronald M. Laxer, Diane Hebert, Damien Noone, Christian Pagnoux, Susanne M. Benseler, Rae S. Yeung, Christoph Kessel, Katrin Lippitz, Toni Weinhage, Claas Hinze, Helmut Wittkowski, Dirk Holzinger, Niklas Grün, Dirk Föll, Pieter Van Dijkhuizen, Federica Del Chierico, Clara Malattia, Alessandra Russo, Denise Pires Marafon, Nienke M. ter Haar, Silvia Magni-Manzoni, Sebastiaan J. Vastert, Bruno Dallapiccola, Berent Prakken, Fabrizio De Benedetti, Lorenza Putignani, Berna Eren Fidanci, Kenan Barut, Serap Arıcı, Dogan Simsek, Mustafa Cakan, Ezgi D. Batu, Sezgin Şahin, Ayşenur Kısaarslan, Ebru Yilmaz, Özge Basaran, Ferhat Demir, Kubra Ozturk, Zübeyde Gunduz, Betül Sozeri, Balahan Makay, Nuray Ayaz, Onder Yavascan, Ozlem Aydog, Yelda Bilginer, Zelal Ekinci, Dilek Yıldız, Faysal Gök, Muferret Erguven, Erbil Unsal, Ozgur Kasapcopur, For the FMF Arthritis Vasculitis and Orphan Disease Research in Paediatric Rheumatology (FAVOR), Hafize E. Sönmez, Betül Sözeri, Yonatan Butbul, Seza Özen, Claudia Bracaglia, Giusi Prencipe, Manuela Pardeo, Geneviève Lapeyre, Emiliano Marasco, Walter Ferlin, Robert Nelson, Cristina de Min, N. Ruperto, H. I. Brunner, P. Quartier, T. Constantin, E. Alexeeva, K. Marzan, N. Wulffraat, R. Schneider, S. Padeh, V. Chasnyk, C. Wouters, J. B. Kuemmerle-Deschner, T. Kallinich, B. Lauwerys, E. Haddad, E. Nasonov, M. Trachana, O. Vougiouka, K. Leon, E. Vritzali, A. Martini, D. Lovell, PRINTO/PRCSG, Stefano Volpi, Claudia Pastorino, Francesca Kalli, Alessia Omenetti, Sabrina Chiesa, Arinna Bertoni, Paolo Picco, Gilberto Filaci, Elisabetta Traggiai, Marie-Louise Fremond, Naoki Kitabayashi, Olivero Sacco, Isabelle Meyts, Marie-Anne Morren, Carine Wouters, Eric Legius, Isabelle Callebaut, Christine Bodemer, Frederic Rieux-Laucat, Mathieu Rodero, Nadia Jeremiah, Alexandre Belot, Eric Jeziorski, Didier Bessis, Guilhem Cros, Gillian I. Rice, Bruno Charbit, Anne Hulin, Nihel Khoudour, Consuelo Modesto Caballero, Monique Fabre, Laureline Berteloot, Muriel Le Bourgeois, Philippe Reix, Thierry Walzer, Despina Moshous, Stéphane Blanche, Alain Fischer, Brigitte Bader-Meunier, Frédéric Rieux-Laucat, K. Annink, N. ter Haar, S. Al-Mayouf, G. Amaryan, K. Barron, S. Benseler, P. Brogan, L. Cantarini, M. Cattalini, A. Cochino, F. Dedeoglu, A. De Jesus, O. Dellacasa, E. Demirkaya, P. Dolezalova, K. Durrant, G. Fabio, R. Gallizzi, R. Goldbach-Mansky, E. Hachulla, V. Hentgen, T. Herlin, M. Hofer, H. Hoffman, A. Insalaco, A. Jansson, I. Koné-Paut, A. Kozlova, J. Kuemmerle-Deschner, R. Laxer, S. Nielsen, I. Nikishina, A. Ombrello, E. Papadopoulou-Alataki, A. Ravelli, D. Rigante, R. Russo, Y. Uziel, Nienke ter Haar, Jerold Jeyaratnam, Anna Simon, Matteo Doglio, Jordi Anton, Consuelo Modesto, Pierre Quartier, Esther Hoppenreijs, Luca Cantarini, Loredana Lepore, Inmaculada Calvo Penades, Christina Boros, Rita Consolini, Donato Rigante, Ricardo Russo, Jana Pachlopnik Schmid, Thirusha Lane, Nicolino Ruperto, Joost Frenkel, Chiara Passarelli, Elisa Pisaneschi, Virginia Messia, Antonio Novelli, Fabrizio Debenedetti, P. A. Brogan, X. Wei, Martina Finetti, Francesca Orlando, Elisabetta Cortis, Angela Miniaci, Nicola Ruperto, Charlotte Eijkelboom, Pavla Dolezalova, Isabelle Koné-Paut, Marija Jelusic-Drazic, Liliana Bezrodnik, Mari Carmen Pinedo, Valda Stanevicha, Marielle van Gijn, Silvia Federici, Hermann Girschick, Gerd Ganser, Susan Nielsen, Troels Herlin, Sulaiman Mohammed Al-Mayouf, Michael Hofer, Jasmin Kuemmerle-Deschner, Susanne Schalm, Annette Jansson, on behalf of PRINTO and Eurofever registry, Marta Marchi, Chiara Marini, Angelo Ravelli, Alberto Garaventa, Sonia Carta, Enrica Balza, Patrizia Castellani, Caterina Pellecchia, Silvia Borghini, Maria Libera Trotta, Anna Rubartelli, Andrew Henrey, Thomas Loughin, Roberta Berard, Natalie Shiff, Roman Jurencak, Susanne Benseler, Lori Tucker, on behalf of ReACCh-Out Investigators, Charalampia Papadopoulou, Ying Hong, Petra Krol, Yiannis Ioannou, Clarissa Pilkington, Hema Chaplin, Stephania Simou, Marietta Charakida, Lucy Wedderburn, Lynn R. Spiegel, Sara Ahola Kohut, Jennifer Stinson, Paula Forgeron, Miriam Kaufman, Nadia Luca, Khush Amaria, Mary Bell, J Swart, F. Boris, E. Castagnola, A. Groll, G. Giancane, G. Horneff, H. I. Huppertz, T. Wolfs, E. Alekseeva, V. Panaviene, F. Uettwiller, V. Stanevicha, L. M. Ailioaie, E. Tsitami, S. Kamphuis, G. Susic, F. Sztajnbok, B. Flato, A. Pistorio, Stephanie J. W. Shoop, Suzanne M. M. Verstappen, Janet E. McDonagh, Wendy Thomson, Kimme L. Hyrich, CAPS, Maarit Tarkiainen, Pirjo Tynjala, Pekka Lahdenne, Janne Martikainen, Acute-JIA Study Group, Meredyth Wilkinson, Christopher Piper, Georg Otto, Claire T. Deakin, Stefanie Dowle, Stefania Simou, Daniel Kelberman, Claudia Mauri, Elizabeth Jury, David Isenberg, Lucy R. Wedderburn, Kiran Nistala, I. Foeldvari, D. J. Lovell, G. Simonini, M. Bereswill, J. Kalabic, Kiem Oen, Brian M. Feldman, Brenden Dufault, Jennifer Lee, Karen Watanabe Duffy, Ciaran Duffy, ReACCh-Out Investigators, N. Tzaribachev, G. Vega-Cornejo, I. Louw, A. Berman, I. Calvo, R. Cuttica, F. Avila-Zapata, R. Cimaz, E. Solau-Gervais, R. Joos, G. Espada, X. Li, M. Nys, R. Wong, S. Banerjee, For Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology Collaborative Study Group (PRCSG), Rebecca Nicolai, Margherita Verardo, Adele D’Amico, Luisa Bracci-Laudiero, Gian Marco Moneta, Gillian Rice, Anne-Laure Mathieu, Sulliman O. Omarjee, Tracy A. Briggs, James O’Sullivan, Simon Williams, Rolando Cimaz, Eve Smith, Michael W. Beresford, Yanick J. Crow, GENIAL Investigators, UK JSLE Study Group, Madeleine Rooney, Nick Bishop, joyce davidson, Clarissa pilkington, Michael Beresford, Jacqui Clinch, Rangaraj Satyapal, Helen Foster, Janet Gardner Medwin, Janet McDonagh, Sue Wyatt, On Behalf of the British Society for Paediatric and Adolescent Rheumatology, Valentina Litta Modignani, Francesco Baldo, Stefano Lanni, Alessandro Consolaro, Giovanni Filocamo, Helen J. Lachmann, on behalf of Eurofever Registry, Gianmarco Moneta, Camilla Celani, Bilade Cherqaoui, Linda Rossi-Semerano, Perrine Dusser, Véronique Hentgen, Claire Grimwood, Linda Rossi, Isabelle Kone Paut, Veronique Hentgen, Denise Lasigliè, Denise Ferrera, Giulia Amico, Marco Di Duca, Laura Obici, Roberto Ravazzolo, Ryuta Nishikomori, Juan Arostegui, Andrea Petretto, Chiara Lavarello, Elvira Inglese, Federica Vanoni, Michaël Hofer, on behalf of EUROFEVER PROJECT, P. N. Hawkins, T. van der Poll, U. A. Walker, H. H. Tilson, Pascal N. Tyrrell, Raphaela Goldbach-Mansky, Norbert Blank, Hal M. Hoffman, Elisabeth Weissbarth-Riedel, Boris Huegle, Tilmann Kallinich, Ahmet Gul, Marlen Oswald, Fatma Dedeoglu, Aki Hanaya, Takako Miyamae, Manabu Kawamoto, Yumi Tani, Takuma Hara, Yasushi Kawaguchi, Satoru Nagata, Hisashi Yamanaka, Almira Ćosićkić, Fahrija Skokić, Belkisa Čolić, Sanimir Suljendić, Anna Kozlova, Irina Mersiyanova, Mariya Panina, Lily Hachtryan, Vasiliy Burlakov, Elena Raikina, Alexey Maschan, Anna Shcherbina, Banu Acar, Meryem Albayrak, Betul Sozeri, Sezgin Sahin, Amra Adrovic, Nese Inan, Serhan Sevgi, Caroline M. Andreasen, Anne Grethe Jurik, Mia B. Glerup, Christian Høst, Birgitte T. Mahler, Ellen-Margrethe Hauge, Cecilia Lazea, Laura Damian, Calin Lazar, Rodica Manasia, Chloe M. Stephenson, Vimal Prajapati, Paivi M. Miettunen, Dilek Yılmaz, Yavuz Tokgöz, Yasin Bulut, Harun Çakmak, Ferah Sönmez, Elif Comak, Gülşah Kaya Aksoy, Mustafa Koyun, Sema Akman, Yunus Arıkan, Ender Terzioğlu, Osman Nidai Özdeş, İbrahim Keser, Hüseyin Koçak, Ayşen Bingöl, Aygen Yılmaz, Reha Artan, X. Xu, Fatemeh F. Mehregan, Vahid Ziaee, Mohammad H. Moradinejad, Francesco La Torre, Clotilde Alizzi, Pio D’Adamo, G. Junge, J. Gregson, Hasmik Sargsyan, Hulya Zengin, Berna E. Fidanci, Cagla Kaymakamgil, Dilek Konukbay, Dilek Yildiz, Faysal Gok, Iris Stoler, Judith Freytag, Banu Orak, Christine Seib, Lars Esmann, Eva Seipelt, Faekah Gohar, Dirk Foell, Ismail Dursun, Sebahat Tulpar, Sibel Yel, Demet Kartal, Murat Borlu, Funda Bastug, Hakan Poyrazoglu, Zubeyde Gunduz, Kader Kose, Mehmet E. Yuksel, Abdullah Calıskan, Ahmet B. Cekgeloglu, Ruhan Dusunsel, Katerina Bouchalova, Jana Franova, Marcel Schuller, Marie Macku, Katerina Theodoropoulou, Raffaella Carlomagno, Annette von Scheven-Gête, Claudia Poloni, Laura O. Damian, Dan Cosma, Amanda Radulescu, Dan Vasilescu, Liliana Rogojan, Simona Rednic, Mihaela Lupse, Lien De Somer, Pierre Moens, Rocio Galindo Zavala, Laura Martín Pedraz, Esmeralda Núñez Cuadros, Gisela Díaz-Cordovés Rego, Antonio L. Urda Cardona, Ilaria Dal Forno, Sara Pieropan, Ombretta Viapiana, Davide Gatti, Gloria Dallagiacoma, Paola Caramaschi, Domenico Biasi, Daniel Windschall, Ralf Trauzeddel, Hartwig Lehmann, Rainer Berendes, Maria Haller, Manuela Krumrey-Langkammerer, Antje Nimtz-Talaska, Philipp Schoof, Ralf Felix Trauzeddel, Christine Nirschl, Estefania Quesada-Masachs, Carla Aguilar Blancafort, Sara Marsal Barril, Francisca Aguiar, Rita Fonseca, Duarte Alves, Ana Vieira, Alberto Vieira, Jorge A. Dias, Iva Brito, Gordana Susic, Vera Milic, Goran Radunovic, Ivan Boricic, Pauline Marteau, Catherine Adamsbaum, Michel De Bandt, Irène Lemelle, Chantal Deslandre, Tu Anh Tran, Anne Lohse, Elisabeth Solau-Gervais, Pascal Pillet, Julien Wipff, Cécile Gaujoux-Viala, Sylvain Breton, Valérie Devauchelle-Pensec, Sandra Gran, Olesja Fehler, Stefanie Zenker, Michael Schäfers, Thomas Vogl, Severine Guillaume Czitrom, EH Pieter Van Dijkhuizen, Silvia Magni Manzoni, Francesca Magnaguagno, Laura Tanturri de Horatio, Nienke M. Ter Haar, Annemieke S. Littooij, Vitor A. Teixeira, Raquel Campanilho-Marques, Ana F. Mourão, Filipa O. Ramos, Manuela Costa, Wafa A. Madan, Orla G. Killeen, Adriana Rodriguez Vidal, Diana Sueiro Delgado, Maria Isabel Gonzalez Fernandez, Berta Lopez Montesinos, Aleksey Kozhevnikov, Nina Pozdeeva, Mikhail Konev, Evgeniy Melchenko, Vladimir Kenis, Gennadiy Novik, Aysenur Pac Kısaarslan, Butsabong Lerkvaleekul, Suphaneewan Jaovisidha, Witaya Sungkarat, Niyata Chitrapazt, Praman Fuangfa, Thumanoon Ruangchaijatuporn, Soamarat Vilaiyuk, Dan Ø. Pradsgaard, Arne Hørlyck, Anne H. Spannow, Carsten W. Heuck, Talia Diaz, Fernando Garcia, Lorenia De La Cruz, Nadina Rubio, Joanna Świdrowska-Jaros, Elzbieta Smolewska, Mirta Lamot, Lovro Lamot, Mandica Vidovic, Edi Paleka Bosak, Ivana Rados, Miroslav Harjacek, Nikolay Tzaribachev, Polymnia Louka, Romiesa Hagoug, Chiara Trentin, Olga Kubassova, Mark Hinton, Mikael Boesen, Olena A. Oshlianska, Illya A. Chaikovsky, G. Mjasnikov, A. Kazmirchyk, Umberto Garagiola, Irene Borzani, Paolo Cressoni, Fabrizia Corona, Eszter Dzsida, Giampietro Farronato, Antonella Petaccia, Alenka Gagro, Agneza Marija Pasini, Goran Roic, Ozren Vrdoljak, Lucija Lujic, Matija Zutelija-Fattorini, Monika M. Esser, Deepthi R. Abraham, Craig Kinnear, Glenda Durrheim, Mike Urban, Eileen Hoal, Victoria B. Nikolayenko, Kubilay Şahin, Yasar Karaaslan, Adele Civino, Giovanni Alighieri, Sergio Davì, Roberto Rondelli, Andrea Magnolato, Francesca Ricci, Alma Olivieri, Valeria Gerloni, Bianca Lattanzi, Francesca Soscia, Alessandro De Fanti, Stefania Citiso, Lorenzo Quartulli, Maria Cristina Maggio, Manuela Marsili, Maria Antonietta Pelagatti, Valentino Conter, Franca Fagioli, Andrea Pession, Marco Garrone, Mariangela Rinaldi, Jaime De Inocencio, Stella Garay, Daniel J. Lovell, Berit Flato, EPOCA Study Group, Angela Aquilani, Simona Cascioli, Ivan Caiello, Denise Pires-Marafón, Rita Carsetti, Emily Robinson, Salvatore Albani, Wilco de Jager, Sytze de Roock, Trang Duong, Justine Ellis, Kimme Hyrich, Laetitia Jervis, Daniel Lovell, Lucy Marshall, Elizabeth D. Mellins, Kirsten Minden, Jane Munro, Peter A. Nigrovic, Jason Palman, Sunil Sampath, Laura E. Schanberg, Susan D. Thompson, Richard Vesely, Chris Wallace, Chris Williams, Qiong Wu, Nico Wulffraat, Rae S. M. Yeung, M. B. Seyger, D. Arikan, J. K. Anderson, A. Lazar, D. A. Williams, C. Wang, R. Tarzynski-Potempa, J. S. Hymans, Gabriele Simonini, Erika Scoccimarro, Irene Pontikaki, Teresa Giani, Alessandro Ventura, Pier Luigi Meroni, Gaetana Minnone, Marzia Soligo, Luigi Manni, Luisa Bracci Laudiero, Noortje Groot, I. Grein, N. M. Wulffraat, R. Schepp, G. Berbers, C. C. Barbosa Sandoval de Souza, V. Paes Leme Ferriani, G. Pileggi, S. de Roock, Ingrid H. R. Grein, Silvia Scala, Elisa Patrone, Casper Schoemaker, on behalf of Dutch JIA patient organization, Wendy Costello, on behalf of ENCA, Suzanne Parsons, Jean-David Cohen, Damien Bentayou, Marc-Antoine Bernard Brunel, Sonia Trope, Jens Klotsche, Miriam Listing, Martina Niewerth, Gerd Horneff, Angelika Thon, Hans-Iko Huppertz, Kirsten Mönkemöller, Ivan Foeldvari, ICON study group, Achille Marino, Stefano Stagi, Niccolò Carli, Federico Bertini, Adriana S. Díaz-Maldonado, Sally Pino, Pilar Guarnizo, Alfonso Ragnar Torres-Jimenez, Berenice Sanchez-Jara, Eunice Solis-Vallejo, Adriana Ivonne Cespedes-Cruz, Maritza Zeferino-Cruz, Julia Veronica Ramirez-Miramontes, Ankur Kumar, Anju Gupta, Deepti Suri, Amit Rawat, Nandita Kakkar, Surjit Singh, Özge A. Gücenmez, Erbil Ünsal, Bo Magnusson, Karina Mördrup, Anna Vermé, Christina Peterson, Board of the Swedish Pediatric Rheumatology Registry, Caroline Freychet, Jean Louis Stephan, Cathryn E. Harkness, Leanne Foster, Emma Henry, Pauline Taggart, Coskun F. Ozkececi, Esra Kurt, Gokalp Basbozkurt, Daiva Gorczyca, Jacek Postępski, Aleksandra Czajkowska, Bogumiła Szponar, Mariola Paściak, Anna Gruenpeter, Iwona Lachór-Motyka, Daria Augustyniak, Edyta Olesińska, Emediong S. Asuka, Tatyana Golovko, Samuel U. Aliejim, Emilio Inarejos Clemente, Estibaliz Iglesias Jimenez, Joan Calzada Hernandez, Sergi Borlan Fernandez, Clara Gimenez Roca, David Moreno Romo, Natalia Rodriguez Nieva, Juan Manuel Mosquera Angarita, Jordi Anton Lopez, Esmeralda Nuñez-Cuadros, Gisela Diaz-Cordovés, Rocío Galindo-Zavala, Antonio Urda-Cardona, Antonio Fernández-Nebro, Daniel Álvarez de la Sierra, Marina Garcia Prat, Mónica Martínez Gallo, Ricardo Pujol Borrell, Ana M. Marín Sánchez, Etienne Merlin, Sylvie Fraitag, Jean-Louis Stephan, Federico Annoni, Giancarla Di Landro, Sofia Torreggiani, Marta Torcoletti, Georgina Tiller, Jo Buckle, Angela Cox, Peter Gowdie, Roger C. Allen, Jonathan D. Akikusa, Hayde G. Hernández-Huirache, Edel R. Rodea-Montero, William Fahy, Christelle Sordet, Karin B. Berggren, Johanna T. Kembe, Joyce Bos, Wineke Armbrust, Marco van Brussel, Jeanette Cappon, Pieter Dijkstra, Jan Geertzen, Elizabeth Legger, Marion van Rossum, Pieter Sauer, Otto Lelieveld, Levent Buluc, Gur Akansel, Bahar Muezzinoglu, Ljubov Rychkova, Tatyana Knyazeva, Anna Pogodina, Tatyana Belova, Tamara Mandzyak, Ekaterina Kulesh, Alessandro Cafarotti, Cosimo Giannini, Roberta Salvatore, Giuseppe Lapergola, Caterina Di Battista, Maria Loredana Marcovecchio, Raffaella Basilico, Piernicola Pelliccia, Francesco Chiarelli, Luciana Breda, Beverley Almeida, Sarah Tansley, Harsha Gunawardena, Neil McHugh, Juvenile Dermatomyositis Research Group (JDRG), Jessie Aouizerate, Marie De Antonio, Christine Barnerias, Guillaume Bassez, Isabelle Desguerre, Romain Gherardi, Jean-Luc Charuel, François-Jérôme Authier, Cyril Gitiaux, C. H. Spencer, Rabheh Abdul Aziz, Chack-Yung Yu, Brent Adler, Sharon Bout-Tabaku, Katherine Lintner, Melissa Moore-Clingenpeel, Liza McCann, Nicola Ambrose, Mario Cortina-Borja, Juvenile Dermatomyositis Cohort and Biomarker Study (JCDBS), Prasad T. Oommen, Fabian Speth, Johannes-Peter Haas, Working Group “Juvenile Dermatomyositis” of the German Society for Paediatric and Adolescent Rheumatology (GKJR), Claudio Lavarello, Gabriella Giancane, Angela Pistorio, Lisa Rider, Rohit Aggarwal, Sheila K. Oliveira, Ruben Cuttica, Michel Fischbach, Gary Sterba, Karine Brochard, Frank Dressler, Patrizia Barone, Ruben Burgos-Vargas, Elizabeth Candell Chalom, Marine Desjonqueres, Graciela Espada, Anders Fasth, Stella Maris Garay, Rose-Marie Herbigneaux, Claire Hoyoux, Chantal Job Deslandre, Frederick W. Miller, Jiri Vencovsky, Erdal Sag, Gulsev Kale, Haluk Topaloglu, Beril Talim, Francesco Zulian, Tadej Avcin, Roberto Marini, Anne Pagnier, Michel Rodiere, Christine Soler, Rebecca Ten Cate, Yosef Uziel, Jelena Vojinovic, Ana V. Villarreal, Nydia Acevedo, Yuridiana Ramirez, Enrique Faugier, Rocio Maldonado, Bita Arabshahi, John H. Lee, Ian Leibowitz, Lawrence O. Okong’o, Jo Wilmshurst, Monika Esser, Christiaan Scott, Ezgi Deniz Batu, Nagehan Emiroglu, Hafize Emine Sonmez, Gokcen Dilsa Tugcu, Zehra Serap Arici, Ebru Yalcin, Deniz Dogru, Ugur Ozcelik, Mithat Haliloglu, Nural Kiper, Masato Yashiro, Mutsuko Yamada, Toshihiko Yabuuchi, Tomonobu Kikkawa, Nobuyuki Nosaka, Yosuke Fujii, Yukie Saito, Hirokazu Tsukahara, Sulaiman M. Al-Mayouf, Nora AlMutiari, Mohammed Muzaffer, Rawiah shehata, Adel Al-Wahadneh, Reem Abdwani, Safia Al-Abrawi, Mohammed Abu-shukair, Zeyad El-Habahbeh, Abdullah Alsonbul, Aleksandra Szabat, Monika Chęć, Violetta Opoka-Winiarska, Biman Saikia, Ranjana W. Minz, Christine Arango, Clara Malagon, Maria D. P. Gomez, Angela C. Mosquera, Ricardo Yepez, Tatiana Gonzalez, Camilo Vargas, GRIP study group, Marta Balzarin, Biagio Castaldi, Elena Reffo, Francesca Sperotto, Giorgia Martini, Alessandra Meneghel, Ornella Milanesi, Ozgur Kasapçopur, Maria Teresa Terreri, Ekaterina Alexeeva, Maria Katsicas, Mikhail Kostik, Thomas Lehman, W.-Alberto Sifuentes-Giraldo, Vanessa Smith, Flavio Sztajnbok, Tadey Avcin, Maria Jose Santos, Dana Nemcova, Cristina Battagliotti, Liora Harel, Mahesh Janarthanan, Kathryn Torok, Nicola Helmus, Eileen Baildem, Michael Blakley, Kim Fligelstone, Antonia Kienast, Clare Pain, Amanda Saracino, Gabriele Simoni, Lisa Weibel, Maria K. Osminina, Nathalia A. Geppe, Olga V. Niconorova, Olesya V. Karashtina, Oksana V. Abbyasova, Olga V. Shpitonkova, Sinem Durmus, Hafize Uzun, Angela Mauro, Eleonora Fanti, Fabio Voller, Franca Rusconi, Fernando Garcia-Rodriguez, Ana V. Villarreal-Treviño, Angel J. Flores-Pineda, Paola B. Lara-Herrea, Diego R. Salinas-Encinas, Talia Diaz-Prieto, Maria R. Maldonado-Velazquez, Sarbelio Moreno-Espinosa, Enrique Faugier-Fuentes, Mirella Crapanzano, Ilaria Parissenti, Man S. Parihar, Pandiarajan Vignesh, ManojKumar Rohit, Kavitha Gopalan, Savita V. Attri, Alan Salama, David Jayne, Mark Little, Yulia Kostina, Galina Lyskina, Olga Shpitonkova, Alena Torbyak, Olga Shirinsky, Maria Francesca Gicchino, Maria Cristina Smaldone, Mario Diplomatico, Alma Nunzia Olivieri, C H. Spencer, Richard McClead, Hiren Patel, Chung-Yung Yu, Dita Cebecauerová, Tomáš Dallos, Edita Kabíčková, Martin Kynčl, Daniela Chroustová, Jozef Hoza, Dana Němcová, Vladimír Tesař, Pavla Doležalová, Tuncay Hazirolan, Fatih Ozaltin, Fabiola Almeida, Isabela H. Faria de Paula, Maíra M. Sampaio, Fernando N. Arita, Andressa G. Alves, Maria Carolina Santos, Eunice M. Okuda, Silvana B. Sacchetti, Fernanda Falcini, Marini Francesca, Gemma Lepri, Marco Matucci-Cerinic, Maria Luisa Brandi, Hakan Kisaoglu, Sema Misir, Selim Demir, Yuksel Aliyazicioglu, Mukaddes Kalyoncu, Carlos Eduardo Ramalho, Fabiola D. Almeida, Joan Calzada-Hernández, Rosa Bou, Estíbaliz Iglesias, Judith Sánchez-Manubens, Fredy Hermógenes Prada Martínez, Clara Giménez Roca, Sergi Borlan Fernández, Marek Bohm, Kamran Mahmood, Valentina Leone, Mark Wood, Ken-Ichi Yamaguchi, Satoshi Fujikawa, Working Group of Behçet’s Disease, Pediatric Rheumatology Association of Japan (PRAJ), Kyu Yeun Kim, Do Young Kim, Dong Soo Kim, Maka Ioseliani, Ivane Chkhaidze, Maia Lekishvili, Nana Tskhakaia, Shorena Tvalabeishvili, Aleksandre Kajrishvili, Maiko Takakura, Masaki Shimizu, Natsumi Inoue, Mao Mizuta, Akihiro Yachie, Giovanni Corsello, Maryam Piram, Carla Maldini, Sandra Biscardi, Nathalie Desuremain, Catherine Orzechowski, Emilie Georget, Delphine Regnard, Isabelle Kone-Paut, Alfred Mahr, Mihaela Sparchez, Zeno Sparchez, Nydia Acevedo Silva, Ana V. Villarreal Treviño, Yuridiana Ramirez Loyola, Talia Diaz Prieto, Enrique Faugier Fuentes, Maria D. R. Maldonado Velazquez, Pilar Perez, Sagar Bhattad, Ranjana Minz, Jitendra Shandilya, Pediatric Allergy and Immunology Unit, PGIMER, Chandigarh, Ana Villarreal, Yuridiana Ramírez, Zeynep Birsin Özçakar, Suat Fitoz, Fatos Yalcinkaya, Annacarin Horne, Francesca Minoia, Francesca Bovis, Sergio Davi, Priyankar Pal, Kimo Stein, Sandra Enciso, Michael Jeng, Despoina Maritsi, Randy C. Cron, Anne Thorwarth, Sae Lim von Stuckrad, Angela Rösen-Wolff, Hella Luksch, Patrick Hundsdoerfer, Peter Krawitz, Nuray Aktay Ayaz, Doğan Simsek, Şebnem Sara Kılıc, Emine Sonmez, Aysenur Pac Kisaarslan, Ozge Altug Gucenmez, Z. Serap Arıcı, Fatih Kelesoglu, Zelal Ekinci Ekinci, Maria Miranda-Garcia, Carolin Pretzer, Michael Frosch, F. Gohar, Angela McArdle, Niamh Callan, Belinda Hernandez, Miha Lavric, Oliver FitzGerald, Stephen R. Pennington, Joachim Peitz, Joern Kekow, Ariane Klein, Anna C. Schulz, Frank Weller-Heinemann, Anton Hospach, J-Peter Haas, BIKER collaborative group, Karen Put, Jessica Vandenhaute, Anneleen Avau, Annemarie van Nieuwenhuijze, Ellen Brisse, Tim Dierckx, Omer Rutgeerts, Josselyn E. Garcia-Perez, Jaan Toelen, Mark Waer, Georges Leclercq, An Goris, Johan Van Weyenbergh, Adrian Liston, Patrick Matthys, Carine H. Wouters, Yasuo Nakagishi, Michael J. Ombrello, Victoria Arthur, Anne Hinks, Patricia Woo, International Childhood Arthritis Genetics (INCHARGE) Consortium, Barbara Stanimirovic, Biljana Djurdjevic-Banjac, Olivera Ljuboja, Boris Hugle, MArgarita Onoufriou, Olga Vougiouka, Kenza Bouayed, Sanae El Hani, Imane Hafid, Nabiha Mikou, Nunu Shelia, Mari Laan, Jaanika Ilisson, and Chris Pruunsild

Subjects
Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Published
2017
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70. Journal of Clinical Pharmacy and Therapeutics

Author

Elham Rahmani, Tricia Lemelle, Hunter Sharp, Ehsan Smarbafzadeh, and Anita Kablinger

Subjects
Pharmacology, agitation, traumatic brain injury, Pharmacology (medical)
Abstract

What Is Known and Objective Traumatic brain injury (TBI) is a major cause of disability, and it has been associated with agitation and aggression. In a previous study, we reviewed the literature to identify evidence-based pharmacological agents for treatment of agitation in TBI. Based on the results of our previous study that summarizes the findings of several systematic reviews, the use of haloperidol and benzodiazepines is not supported by the available evidence while the use of amantadine, beta blockers, antiepileptics and methylphenidate is supported by the limited available evidence. In this study, we describe the psycho-pharmacological agents that were administered to patients with agitation and/or aggression in the context of TBI in inpatient facilities of a private, non-profit health care system in southwest Virginia. We will also compare the psycho-pharmacological agents ordered before and after psychiatric consultation. Methods Adult patients who were admitted to Carilion Clinic's inpatient facilities from March 30, 2013, to March 30, 2018, had a diagnosis of TBI, and received psychiatric consultation for agitation and/or aggression were enrolled in this study. A retrospective review of electronic medical records was conducted by researchers and data were collected on the following measures: ordered psycho-pharmacological agents, frequency, dosing and duration of orders, whether each administered psycho-pharmacological agent was started before or after psychiatric consultation, and psycho-pharmacological agents prescribed upon discharge. Results and Discussion About 68% of patients were started on benzodiazepines and/or typical antipsychotics and 23% of patients were subsequently discharged on these medication categories. Only 23% of patients were ordered to receive medications supported by the evidence such as amantadine, beta blockers or antiepileptics. The percentage of patient-days with an order to receive typical antipsychotics significantly decreased following psychiatric consultation (p = 0.0056), but the percentage of patient-days with an order to receive benzodiazepines significantly increased following psychiatric consultation (p = 0.0001). This finding remained statistically significant after excluding patients with active or unclear alcohol/benzodiazepine withdrawal (p < 0.0001). What Is New and Conclusion This study demonstrates the widespread use of typical antipsychotics and benzodiazepines in the management of agitation in TBI and the importance of multidisciplinary collaboration, research and education of providers to improve patient care. Published version

Published
2022
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71. French recommendations for the management of non-infectious chronic uveitis

Author

Quartier, P., primary, Saadoun, D., additional, Belot, A., additional, Errera, M.-H., additional, Kaplanski, G., additional, Kodjikian, L., additional, Kone-Paut, I., additional, Miceli-Richard, C., additional, Monnet, D., additional, Audouin-Pajot, C., additional, Seve, P., additional, Uettwiller, F., additional, Weber, M., additional, Bodaghi, B., additional, Abad, S., additional, Bayen, M., additional, Bielefeld, P., additional, Chalumeau, M., additional, Chiquet, C., additional, Cohen, J.-D., additional, Despert, V., additional, Devilliers, H., additional, Fardeau, C., additional, Georgin-Lavialle, S., additional, Guex-Crosier, Y., additional, Guillaume Czitrom, S., additional, Heron, E., additional, Hofer, M., additional, Agbo Kpati, K.P., additional, Labalette, P., additional, Lemelle, I., additional, Nouar, D., additional, Pugnet, G., additional, Sellam, J., additional, Sene, D., additional, Terrier, B., additional, and Trad, G.S., additional

Published
2023
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74. Seat belt syndrome with caecal perforation and Chance fracture in an adolescent.

Author

Destinval, Christelle, Larmure, Olivier, Journeau, Pierre, and Lemelle, Jean-Louis

Abstract

Seat belt syndrome describes a combination of injuries a person restrained by a seat belt may have after a high-velocity motor vehicle crash. A seat belt-shaped bruise may be present across the torso. A Chance fracture is a horizontal vertebral fracture, resulting from spinal hyperflexion, and in disruption of the three columns of the vertebral bodies. Its association with intra-abdominal injury is rare in children. A male, in early adolescence, was involved in a high-energy car crash. A total-body CT scan highlighted lacerations of the anterolateral abdominal wall and an acute L4 Chance fracture. Another CT scan performed because of clinical deterioration, showed cecal perforation, caused by a mesenteric tear and non-displaced fracture of the left sacral ala. He underwent a right hemicolectomy with ileocolic anastomosis and protective ileostomy and was immobilised in a body jacket orthosis for 3 months. The ileostomy was closed 3 months later. At 2-year follow-up, he is well. [ABSTRACT FROM AUTHOR]

Published
2023
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76. Cancer-associated Mutations in Congenital Pulmonary Malformations: A Prospective Cohort

Author

Garinet, Simon, primary, Rahshenas, Makan, additional, Galmiche-Rolland, Louise, additional, Abbo, Olivier, additional, Bonnard, Arnaud, additional, Hameury, Frédéric, additional, Khen-Dunlop, Naziha, additional, Khoshnood, Babak, additional, Blons, Hélène, additional, Delacourt, Christophe, additional, Boudjemaa, Sabah, additional, Choupeaux, Laure, additional, Collardeau-Frachon, Sophie, additional, Fouquet, Virginie, additional, Habonimana, Edouard, additional, Hervieux, Erik, additional, Lelong, Nathalie, additional, Levard, Guillaume, additional, Molina, Thierry Jo, additional, Picard, Cecile, additional, Piolat, Christophe, additional, Sfeir, Rony, additional, Biquard, Florence, additional, Bory, Jean-Paul, additional, Goffinet, François, additional, As, Valat-Rigot, additional, Winer, Norbert, additional, Mousty, Eve, additional, Muszynski, Charles, additional, Benachi, Alexandra, additional, Salomon, Laurent J., additional, Goua, Valérie, additional, Jouannic, Jean-Marie, additional, Bouar, Gwenaelle Le, additional, Massardier, Jérôme, additional, Rosenblatt, Jonathan, additional, Sartor, Agnès, additional, Thong-Vanh, Catherine, additional, Prieur, Fabienne, additional, Quibel, Thibaud, additional, Touboul, Claudine, additional, Gondry, Jean, additional, Sentilhes, Loic, additional, Bonfiglioli, Valérie, additional, Carbillon, Lionel, additional, Saliou, Anne-Helene, additional, Saada, Julien, additional, Letourneau, Alexandra, additional, Laurichesse, Helene, additional, Egron, Carole, additional, Deslandes, Loren, additional, Castaigne, Vanina, additional, Dazel-Salonne, Claire, additional, Stirnemann, Julien, additional, Roth, Philippe, additional, Bultez, Thierry, additional, Ville, Yves, additional, Tsatsaris, Vassilis, additional, Guilbaud, Lucie, additional, Darras, Anne-Marie, additional, Oury, Jean-Francois, additional, Fanget, Cecile, additional, Favre, Romain, additional, Paris, Anne, additional, Perrotin, Franck, additional, Banaszkiewicz, Nathalie, additional, Rakza, Thameur, additional, Morin, Mathieu, additional, Trastour, Cynthia, additional, Mottet, Nicolas, additional, Coatleven, Frederic, additional, Alanio, Elisabeth, additional, Morel, Olivier, additional, Perdriolle, Estelle, additional, Eszto-Cambon, Marie-Laure, additional, Berrazaga, Insaf, additional, Podevin, Guillaume, additional, Feghali, Hala, additional, Thumerelle, Caroline, additional, Mangione, Rafaelle, additional, Lebras, Marie-Noelle, additional, Ducoin, Héloise, additional, Romeo, Bernard, additional, Buisson, Philippe, additional, Cros, Pierrick, additional, Roditis, Léa, additional, Devries, Hiline, additional, Brouard, Jacques, additional, Petit, Thierry, additional, Petit, Isabelle, additional, Simo, Adam Kandem, additional, Pons, Maguelonne, additional, Jaby, Olivier, additional, Llerena, Catherine, additional, Sabatier, Edith, additional, Thouvenin, Guillaune, additional, Larroquet, Michele, additional, Irtan, Sabine, additional, Grapin, Christine, additional, Parico, Caroline, additional, Varlet, François, additional, Matis, Jacqueline, additional, Becmeur, François, additional, Gibertini, Isabelle, additional, Lardy, Hubert, additional, Denapolicocci, Stephan, additional, Deschildre, Antoine, additional, Breaud, Jean, additional, Lecompte, Jean-François, additional, Kamdem, Arnaud Fotso, additional, Elbaz, Frédéric, additional, Lavrand, Frédéric, additional, Pomedio, Mickael, additional, Lefebvre, Francis, additional, Lemelle, Jean Louis, additional, Gomola, Vladimir, additional, Vigier, Clementine, additional, and Monier, Isabelle, additional

Published
2023
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77. Assessment of Puberty and Hypothalamic–Pituitary–Gonadal Axis Function After Childhood Brain Tumor Treatment

Author

Rosimont, Manon, primary, Kariyawasam, Dulanjalee, additional, Samara-Boustani, Dinane, additional, Giani, Elisa, additional, Beltrand, Jacques, additional, Bolle, Stephanie, additional, Fresneau, Brice, additional, Puget, Stephanie, additional, Sainte-Rose, Christian, additional, Alapetite, Claire, additional, Pinto, Graziella, additional, Touraine, Philippe, additional, Piketty, Marie-Liesse, additional, Brabant, Séverine, additional, Abbou, Samuel, additional, Aerts, Isabelle, additional, Beccaria, Kevin, additional, Bourgeois, Marie, additional, Roujeau, Thomas, additional, Blauwblomme, Thomas, additional, Rocco, Federico Di, additional, Thalassinos, Caroline, additional, Rigaud, Charlotte, additional, James, Syril, additional, Busiah, Kanetee, additional, Simon, Albane, additional, Bourdeaut, Franck, additional, Lemelle, Lauriane, additional, Guerrini-Rousseau, Léa, additional, Orbach, Daniel, additional, Doz, François, additional, Dufour, Christelle, additional, Grill, Jacques, additional, Polak, Michel, additional, and Briceño, Laura González, additional

Published
2023
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79. Osteonecrosis in patients with juvenile dermatomyositis: is it associated with anti-MDA5 autoantibody?

Author

Bader-Meunier, Brigitte, primary, Breton, Sylvain, additional, Duffy, Darragh, additional, Gitiaux, Cyril, additional, Quartier, Pierre, additional, Lemelle, Irène, additional, Meyer, Alain, additional, Welfringer-Morin, Anne, additional, Frémond, Marie-Louise, additional, Charuel, Jean-Luc, additional, Rodero, Mathieu P, additional, and Melki, Isabelle, additional

Published
2023
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80. Evaluation of continuous wound infusion with local analgesics in postoperative renal transplantation patients: A retrospective study

Author

Alshuaibi, MuaathKhaled, primary, Khogeer, Abdulghani, additional, Ambusaidi, Hamed, additional, Mazeaud, Charles, additional, Larose, Clement, additional, Lecoanet, Pierre, additional, Urmès, Isabelle, additional, Lagrange, Francois, additional, Lemelle, Jean-Louis, additional, Manuguerra, Anthony, additional, Fuchs-Buder, Thomas, additional, Hubert, Jacques, additional, and Eschwège, Pascal, additional

Published
2023
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81. L’alliance thérapeutique est associée à l’adhésion au traitement chez les enfants atteints d’arthrite juvénile idiopathique

Author

Valérie Devauchelle-Pensec, Anaïs Arbault, Elisabeth Solau-Gervais, Irène Lemelle, L. Sparsa, Agnès Duquesne, Tu-Anh Tran, Anne Lohse, Camille Alleyrat, Francis Guillemin, Pascal Pillet, Linda Rossi, Laurence Goumy, Claire Ballot, Héloïse Reumaux, Centre Hospitalier de Belfort (Service de Rhumatologie), CH Belfort-Montbéliard, Service d'Onco-Hématologie Pédiatrique, Centre Hospitalier Universitaire de Nancy, hôpital des Enfants [CHU Bordeaux], Pédiatrie hospices civils de Lyon, hôpital Femme-Mère-Enfant, Service de pédiatrie [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Service d'Hématologie Pédiatrique, CHU Nîmes, France, Centre Hospitalier de Mulhouse (Service de Rhumatologie), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA)-Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Service de Pédiatrie Générale [CHU Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Service de Rhumatologie Pédiatrique, Centre Universitaire de Lille, Pediatric Rheumatology [Le Kremlin-Bicêtre], Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses [Le Kremlin-Bicêtre] (CeRéMAIA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Service de rhumatologie [Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de Rhumatologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre d'investigation clinique - Epidémiologie clinique [Nancy] (CIC-EC), Centre d'investigation clinique [Nancy] (CIC), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)-Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
030203 arthritis & rheumatology, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, [SDV.IMM]Life Sciences [q-bio]/Immunology, 030212 general & internal medicine, ComputingMilieux_MISCELLANEOUS
Abstract

Objectifs L’alliance therapeutique (AT) se definit comme l’engagement mutuel entre le soignant et le patient au cours du processus therapeutique. L’adhesion therapeutique est un enjeu majeur dans la prise en charge de l’arthrite juvenile idiopathique (AJI) et repose sur la forte capacite de l’enfant a suivre les traitements. Cette etude avait pour objectif d’evaluer l’association entre l’AT et l’adhesion therapeutique chez des patients atteints d’AJI. Methodes Etude observationnelle, transversale, multicentrique. Des enfants atteints d’AJI, âges de 8 a 16 ans ont ete inclus. Les enfants, leurs parents et les medecins ont complete le Helping Alliance Questionnaire (HAq-CP) pour mesurer l’AT. L’observance aux traitements a ete mesuree a l’aide du Child/Parent Adherence Report Questionnaire (CARQ & PARQ). Les donnees demographiques, les caracteristiques de la maladie, les traitements en cours et l’environnement social ont ete recueillis. La relation univariee entre l’AT et l’adhesion a ete mesuree a l’aide du coefficient de correlation de Pearson. L’analyse multivariee a utilise un modele de regression lineaire multiple. Resultats 119 patients ont ete inclus : 68,9 % de filles, l’âge moyen (ET) etait de 12,4 (2,9) ans, avec une duree de la maladie de 73,1 (48,2) mois. L’AJI etait en remission (52 %), de faible activite (32 %) et active (16 %). Les scores de l’AT etaient eleves (≥ 80/100) chez les enfants, les parents et les medecins. Le HAQ-CP a montre une forte correlation avec le CARQ (r = 0,31 ; p Conclusion L’AT exerce une forte influence sur l’adhesion therapeutique et pourrait jouer un role majeur dans l’efficacite du traitement.

Published
2022
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82. Assessment of Puberty and Hypothalamic–Pituitary–Gonadal Axis Function After Childhood Brain Tumor Treatment

Author

Manon Rosimont, Dulanjalee Kariyawasam, Dinane Samara-Boustani, Elisa Giani, Jacques Beltrand, Stephanie Bolle, Brice Fresneau, Stephanie Puget, Christian Sainte-Rose, Claire Alapetite, Graziella Pinto, Philippe Touraine, Marie-Liesse Piketty, Séverine Brabant, Samuel Abbou, Isabelle Aerts, Kevin Beccaria, Marie Bourgeois, Thomas Roujeau, Thomas Blauwblomme, Federico Di Rocco, Caroline Thalassinos, Charlotte Rigaud, Syril James, Kanetee Busiah, Albane Simon, Franck Bourdeaut, Lauriane Lemelle, Léa Guerrini-Rousseau, Daniel Orbach, François Doz, Christelle Dufour, Jacques Grill, Michel Polak, and Laura González Briceño

Subjects
Endocrinology, Endocrinology, Diabetes and Metabolism, Biochemistry (medical), Clinical Biochemistry, Biochemistry
Abstract

ContextEndocrine complications are common in pediatric brain tumor patients.ObjectiveTo describe hypothalamic–pituitary–gonadal axis (HPGA) function in patients treated in childhood for a primary brain tumor more than 5 years earlier, in order to identify risk factors for HPGA impairment.MethodsWe retrospectively included 204 patients diagnosed with a primary brain tumor before 18 years of age and monitored at the pediatric endocrinology unit of the Necker Enfants-Malades University Hospital (Paris, France) between January 2010 and December 2015. Patients with pituitary adenoma or untreated glioma were excluded.ResultsAmong patients with suprasellar glioma not treated by radiotherapy, the prevalence of advanced puberty was 65% overall and 70% when the diagnosis occurred before 5 years of age. Medulloblastoma chemotherapy caused gonadal toxicity in 70% of all patients and in 87.5% of those younger than 5 years at diagnosis. In the group with craniopharyngioma, 70% of patients had hypogonadotropic hypogonadism, which was consistently accompanied by growth hormone deficiency.ConclusionTumor type, location, and treatment were the risk main factors for HPGA impairment. Awareness that onset can be delayed is essential to guide information of parents and patients, patient monitoring, and timely hormone replacement therapy.

Published
2023
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83. Osteonecrosis in patients with juvenile dermatomyositis: is it associated with anti-MDA5 autoantibody?

Author

Brigitte Bader-Meunier, Sylvain Breton, Darragh Duffy, Cyril Gitiaux, Pierre Quartier, Irène Lemelle, Alain Meyer, Anne Welfringer-Morin, Marie-Louise Frémond, Jean-Luc Charuel, Mathieu P Rodero, Isabelle Melki, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Référence pour les Maladies Rhumatologiques Auto-Immunes et Systémiques [CHU Necker] (RAISE), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Immunogenetics of pediatric autoimmune diseases (Equipe Inserm U1163), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Immunologie Translationnelle - Translational Immunology lab, Institut Pasteur [Paris] (IP)-Université Paris Cité (UPCité), Hôpital Brabois, Hôpital de Hautepierre [Strasbourg], Laboratory of neurogenetics and neuroinflammation (Equipe Inserm U1163), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Laboratoire de Chimie et de Biochimie Pharmacologiques et Toxicologiques (LCBPT - UMR 8601), Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Hôpital Robert Debré, This work was supported by a research grant from Agence Nationale de la Recherche (ANR) via project JDMINF2 (ANR-21-CE17-0025)., and ANR-21-CE17-0025,JDMINF2,Dérégulation de la voie de l'interféron de type I dans la dermatomyosite juvénile: mieux comprendre la physiopathologie pour mieux identifier de nouveaux biomarqueurs pronostiques(2021)

Subjects
Rheumatology, [SDV.IMM]Life Sciences [q-bio]/Immunology, Pharmacology (medical)
Abstract

International audience

Published
2023
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84. NUT Carcinoma in Children and Adolescents : The Expert European Standard Clinical Practice Harmonized Recommendations

Author

Lauriane Lemelle, Tim Flaadt, Brice Fresneau, Antoine Moya-Plana, Beate Timmermann, Jelena Roganovic, Andrea Ferrari, Giulia Fichera, Ulrich M. Lauer, Tal Ben-Ami, Dominik T. Schneider, Christian Vokuhl, Stephanie Bolle, Elisabeth Fox, Steven G. DuBois, Carlos Rodriguez-Galindo, Gianni Bisogno, Aurore Surun, Ines B. Brecht, and Daniel Orbach

Subjects
Oncology, Pediatrics, Perinatology and Child Health, Medizin, Hematology
Abstract

Nuclear protein of the testis (NUT) carcinoma (NC) is a rare and highly aggressive tumor mainly occurring in adolescents and young adults, defined by the presence of a somatic NUTM1 rearrangement. The aim is to establish internationally harmonized consensus recommendations for the diagnosis and treatment of adolescents and young adults with NC in the framework of the European Reference Network for Paediatric Oncology.The European Cooperative Study Group for Pediatric Rare Tumors developed recommendations according to the Consensus Conference Standard Operating procedure methodology and reviewed by external "experts." No evidence of level I to II exists. Recommendations were developed based on published prospective (level III), but more frequently retrospective series (level IV), case reports (level V), and personal expertise (level V). In addition, "strength" of recommendations were categorized by grading (grade A to E).Histology is mandatory for the diagnosis of NC, including immunolabeling with anti-NUT antibodies and molecular biology (NUTM1 rearrangement) (level V; grade A). Treatment of NC usually combines aggressive approaches in multimodal regimens. Chemotherapy should be considered as first-line treatment (neoadjuvant vincristine-adriamycin-ifosfamide/cisplatin-adriamycin-ifsofamide or vincristine-doxorubicin-cyclophosphamide/ifosfamide-etoposide) for unresectable or metastatic tumor (ie, 3 courses), rapidly followed by local treatment (level IV; grade B). Referral to a specialized surgical oncology center is highly recommended (level V; grade A). In localized NC, a complete microscopic surgical resection should be attempted whenever and as soon as possible, followed by primary irradiation (60 to 70 Gy) and involved lymph nodes area (level IV; grade B). For head and neck tumors, a systematic neck dissection might be considered, even if N0 (level V; grade C). Adjuvant postirradiation chemotherapy is recommended, for a total of 9 to 12 courses (level IV; grade B). For first-line resected tumors, concomitant adjuvant chemotherapy to radiotherapy may be discussed (level IV; grade B). Targeted therapies and immunotherapeutic regimens should be delivered in the setting of prospective trials (level V; grade B).This project leads to a consensus strategy based on international experience with this very rare disease.

Published
2023

86. High Prevalence of Early Endocrine Disorders After Childhood Brain Tumors in a Large Cohort

Author

Laura Gabriela González Briceño, Dulanjalee Kariyawasam, Dinane Samara-Boustani, Elisa Giani, Jacques Beltrand, Stéphanie Bolle, Brice Fresneau, Stéphanie Puget, Christian Sainte-Rose, Claire Alapetite, Graziella Pinto, Marie-Liesse Piketty, Séverine Brabant, Samuel Abbou, Isabelle Aerts, Kevin Beccaria, Marie Bourgeois, Thomas Roujeau, Thomas Blauwblomme, Federico Di Rocco, Caroline Thalassinos, Christian Pauwels, Charlotte Rigaud, Syril James, Kanetee Busiah, Albane Simon, Franck Bourdeaut, Lauriane Lemelle, Léa Guerrini-Rousseau, Daniel Orbach, Philippe Touraine, François Doz, Christelle Dufour, Jacques Grill, and Michel Polak

Subjects
Adult, Male, Brain Neoplasms, Endocrinology, Diabetes and Metabolism, Biochemistry (medical), Clinical Biochemistry, Endocrine System Diseases, Biochemistry, Endocrinology, Prevalence, Humans, Female, Pituitary Neoplasms, Cerebellar Neoplasms, Child, Retrospective Studies
Abstract

Context Endocrine complications are common in pediatric brain tumor patients. Objective We aimed to describe the endocrine follow-up of patients with primary brain tumors. Methods This is a noninterventional observational study based on data collection from medical records of 221 patients followed at a Pediatric Endocrinology Department. Results Median age at diagnosis was 6.7 years (range, 0-15.9), median follow-up 6.7 years (0.3-26.6), 48.9% female. Main tumor types were medulloblastoma (37.6%), craniopharyngioma (29.0%), and glioma (20.4%). By anatomic location, 48% were suprasellar (SS) and 52% non-suprasellar (NSS). Growth hormone deficiency (GHD) prevalence was similar in both groups (SS: 83.0%, NSS: 76.5%; P = 0.338), appearing at median 1.8 years (−0.8 to 12.4) after diagnosis; postradiotherapy GHD appeared median 1.6 years after radiotherapy (0.2-10.7). Hypothyroidism was more prevalent in SS (76.4%), than NSS (33.9%) (P < 0.001), as well as ACTH deficiency (SS: 69.8%, NSS: 6.1%; P < 0.001). Early puberty was similar in SS (16%) and NSS (12.2%). Hypogonadotropic hypogonadism was predominant in SS (63.1%) vs NSS (1.3%), P < 0.001, and postchemotherapy gonadal toxicity in NSS (29.6%) vs SS (2.8%), P < 0.001. Adult height was lower for NSS compared to target height (−1.0 SD, P < 0.0001) and to SS patients (P < 0.0001). Thyroid nodules were found in 13/45 patients (28.8%), including 4 cancers (4.8-11.5 years after radiotherapy). Last follow-up visit BMI was higher in both groups (P = 0.0001), and obesity incidence was higher for SS (46.2%) than NSS (17.4%). Conclusion We found a high incidence of early-onset endocrine disorders. An endocrine consultation and nutritional evaluation should be mandatory for all patients with a brain tumor, especially when the tumor is suprasellar or after hypothalamus/pituitary irradiation.

Published
2021
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88. Mutations in the B30.2 and the Central Helical scaffold domains of pyrin differentially affect inflammasome activation

Author

Daria Chirita, Pauline Bronnec, Flora Magnotti, Sarah Dalmon, Amandine Martin, Michel Popoff, Mathieu Gerfaud-Valentin, Pascal Sève, Alexandre Belot, Anne Contis, Agnes Duquesne, Gaetane Nocturne, Irene Lemelle, Sophie Georgin-Lavialle, Guilaine Boursier, Isabelle Touitou, Yvan Jamilloux, and Thomas Henry

Subjects
Cancer Research, Cellular and Molecular Neuroscience, Immunology, Cell Biology
Abstract

Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disorder. FMF is caused by mutations in the MEFV gene, encoding pyrin, an inflammasome sensor. The best characterized pathogenic mutations associated with FMF cluster in exon 10. Yet, mutations have been described along the whole MEFV coding sequence. Exon 10 encodes the B30.2 domain of the pyrin protein, but the function of this human-specific domain remains unclear. Pyrin is an inflammasome sensor detecting RhoA GTPase inhibition following exposure to bacterial toxins such as TcdA. Here, we demonstrate that the B30.2 domain is dispensable for pyrin inflammasome activation in response to this toxin. Deletion of the B30.2 domain mimics the most typical FMF-associated mutation and confers spontaneous inflammasome activation in response to pyrin dephosphorylation. Our results indicate that the B30.2 domain is a negative regulator of the pyrin inflammasome that acts independently from and downstream of pyrin dephosphorylation. Additionally, we identify the central helical scaffold (CHS) domain of pyrin, which lies immediately upstream of the B30.2 domain as a second regulatory domain. Mutations affecting the CHS domain mimic pathogenic mutations in the B30.2 domain and render the pyrin inflammasome activation under the sole control of the dephosphorylation. In addition, specific mutations in the CHS domain strongly increase the cell susceptibility to steroid catabolites, recently described to activate pyrin, in both a cell line model and in monocytes from genotype-selected FMF patients. Taken together, our work reveals the existence of two distinct regulatory regions at the C-terminus of the pyrin protein, that act in a distinct manner to regulate positively or negatively inflammasome activation. Furthermore, our results indicate that different mutations in pyrin regulatory domains have different functional impacts on the pyrin inflammasome which could contribute to the diversity of pyrin-associated autoinflammatory diseases.

Published
2022
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89. Cancer-associated Mutations in Congenital Pulmonary Malformations: A Prospective Cohort

Author

Simon Garinet, Makan Rahshenas, Louise Galmiche-Rolland, Olivier Abbo, Arnaud Bonnard, Frédéric Hameury, Naziha Khen-Dunlop, Babak Khoshnood, Hélène Blons, Christophe Delacourt, Sabah Boudjemaa, Laure Choupeaux, Sophie Collardeau-Frachon, Virginie Fouquet, Edouard Habonimana, Erik Hervieux, Nathalie Lelong, Guillaume Levard, Thierry Jo Molina, Cecile Picard, Christophe Piolat, Rony Sfeir, Florence Biquard, Jean-Paul Bory, François Goffinet, Valat-Rigot As, Norbert Winer, Eve Mousty, Charles Muszynski, Alexandra Benachi, Laurent J. Salomon, Valérie Goua, Jean-Marie Jouannic, Gwenaelle Le Bouar, Jérôme Massardier, Jonathan Rosenblatt, Agnès Sartor, Catherine Thong-Vanh, Fabienne Prieur, Thibaud Quibel, Claudine Touboul, Jean Gondry, Loic Sentilhes, Valérie Bonfiglioli, Lionel Carbillon, Anne-Helene Saliou, Julien Saada, Alexandra Letourneau, Helene Laurichesse, Carole Egron, Loren Deslandes, Vanina Castaigne, Claire Dazel-Salonne, Julien Stirnemann, Philippe Roth, Thierry Bultez, Yves Ville, Vassilis Tsatsaris, Lucie Guilbaud, Anne-Marie Darras, Jean-Francois Oury, Cecile Fanget, Romain Favre, Anne Paris, Franck Perrotin, Nathalie Banaszkiewicz, Thameur Rakza, Mathieu Morin, Cynthia Trastour, Nicolas Mottet, Frederic Coatleven, Elisabeth Alanio, Olivier Morel, Estelle Perdriolle, Marie-Laure Eszto-Cambon, Insaf Berrazaga, Guillaume Podevin, Hala Feghali, Caroline Thumerelle, Rafaelle Mangione, Marie-Noelle Lebras, Héloise Ducoin, Bernard Romeo, Philippe Buisson, Pierrick Cros, Léa Roditis, Hiline Devries, Jacques Brouard, Thierry Petit, Isabelle Petit, Adam Kandem Simo, Maguelonne Pons, Olivier Jaby, Catherine Llerena, Edith Sabatier, Guillaune Thouvenin, Michele Larroquet, Sabine Irtan, Christine Grapin, Caroline Parico, François Varlet, Jacqueline Matis, François Becmeur, Isabelle Gibertini, Hubert Lardy, Stephan Denapolicocci, Antoine Deschildre, Jean Breaud, Jean-François Lecompte, Arnaud Fotso Kamdem, Frédéric Elbaz, Frédéric Lavrand, Mickael Pomedio, Francis Lefebvre, Jean Louis Lemelle, Vladimir Gomola, Clementine Vigier, and Isabelle Monier

Subjects
Pulmonary and Respiratory Medicine, Critical Care and Intensive Care Medicine
Published
2022

90. Mutations in the B30.2 and the Central Helical scaffold domains of pyrin differentially affect inflammasome activation

Author

chirita, Daria, primary, Magnotti, Flora, additional, Bronnec, Pauiine, additional, Dalmon, Sarah, additional, Martin, Amandine, additional, Popoff, michel, additional, Gerfaud-Valentin, Mathieu, additional, Seve, Pascal, additional, Belot, Alexandre, additional, Contis, Anne, additional, Duquesne, Agnes, additional, nocturne, gaetane, additional, lemelle, Irene, additional, Georgin-Lavialle, Sophie, additional, Boursier, Guilaine, additional, Touitou, Isabelle, additional, Jamilloux, Yvan, additional, and Henry, Thomas, additional

Published
2022
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91. NUT Carcinoma in Children and Adolescents: The Expert European Standard Clinical Practice Harmonized Recommendations

Author

Lemelle, Lauriane, primary, Flaadt, Tim, additional, Fresneau, Brice, additional, Moya-Plana, Antoine, additional, Timmermann, Beate, additional, Roganovic, Jelena, additional, Ferrari, Andrea, additional, Fichera, Giulia, additional, Lauer, Ulrich M., additional, Ben-Ami, Tal, additional, Schneider, Dominik T., additional, Vokuhl, Christian, additional, Bolle, Stephanie, additional, Fox, Elisabeth, additional, DuBois, Steven G., additional, Rodriguez-Galindo, Carlos, additional, Bisogno, Gianni, additional, Surun, Aurore, additional, Brecht, Ines B., additional, and Orbach, Daniel, additional

Published
2022
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95. Evidence for interstellar origin of seven dust particles collected by the Stardust spacecraft

Author

Westphal, Andrew J., Stroud, Rhonda M., Bechtel, Hans A., Brenker, Frank E., Butterworth, Anna L., Flynn, George J., Frank, David R., Gainsforth, Zack, Hillier, Jon K., Postberg, Frank, Simionovici, Alexandre S., Sterken, Veerle J., Nittler, Larry R., Allen, Carlton, Anderson, David, Ansari, Asna, Bajt, Saša, Bastien, Ron K., Bassim, Nabil, Bridges, John, Brownlee, Donald E., Burchell, Mark, Burghammer, Manfred, Changela, Hitesh, Cloetens, Peter, Davis, Andrew M., Doll, Ryan, Floss, Christine, Grün, Eberhard, Heck, Philipp R., Hoppe, Peter, Hudson, Bruce, Huth, Joachim, Kearsley, Anton, King, Ashley J., Lai, Barry, Leitner, Jan, Lemelle, Laurence, Leonard, Ariel, Leroux, Hugues, Lettieri, Robert, Marchant, William, Ogliore, Ryan, Ong, Wei Jia, Price, Mark C., Sandford, Scott A., Tresseras, Juan-Angel Sans, Schmitz, Sylvia, Schoonjans, Tom, Schreiber, Kate, Silversmit, Geert, Solé, Vicente A., Srama, Ralf, Stadermann, Frank, Stephan, Thomas, Stodolna, Julien, Sutton, Stephen, Trieloff, Mario, Tsou, Peter, Tyliszczak, Tolek, Vekemans, Bart, Vincze, Laszlo, Von Korff, Joshua, Wordsworth, Naomi, Zevin, Daniel, and Zolensky, Michael E.

Published
2014

98. Free at last?

Author

Battle, Juan, primary, Bennett, Michael, additional, and Lemelle, Anthony, additional

Published
2017
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