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117 results on '"Jean-Christophe Dubus"'

1. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part three

Author

Riccardo Papa, Alessandro Consolaro, Francesca Minoia, Roberta Caorsi, Gianmichele Magnano, Marco Gattorno, Angelo Ravelli, Paolo Picco, Roberto Pillon, Denise Pires Marafon, Lidia Meli, Claudia Bracaglia, Andrea Taddio, Fabrizio De Benedetti, Enes Turan, Sara Sebnem Kilic, Yasuhiko Itoh, Tomoko Shigemori, Shingo Yamanishi, Hidehiko Nagasaki, Ela Tarakci, Nilay Arman, Devrim Tarakci, Yusuf S. Akgul, Ozgur Kasapcopur, Emily Wilson, Hanna Lythgoe, Eve Smith, Jenny Preston, Michael W. Beresford, Lynn R. Spiegel, Jennifer Stinson, Mark Connelly, Adam Huber, Nadia Luca, Argerie Tsimicalis, Stephanie Luca, Naweed Tajuddin, Roberta Berard, Julie Barsalou, Sarah Campillo, Brian Feldman, Shirley Tse, Paul Dancey, Ciaran Duffy, Nicole Johnson, Patrick McGrath, Natalie Shiff, Lori Tucker, Charles Victor, Chitra Lalloo, Lauren Harris, Joseph Cafazzo, Kristin Houghton, Ronald Laxer, Madeleine Rooney, Roisin Campbell, Catherine Wright, Wineke Armbrust, Otto Lelieveld, Jolanda Tuinstra, Nico Wulffraat, Joyce Bos, Jeanette Cappon, Marion van Rossum, Mariët Hagedoorn, Anna Vermé, Ylva Lampela, Ayse Huri Ozdogan, S. Ugurlu, K. Barut, A. Androvic, O. Kasapçopu, Jody Etheridge, Katie Dobson, Sue Kemp, AnnaCarin Horne, Karin Palmblad, Malin Höglund, Natalia Stepanenko, Svetlana Salugina, Evgeny Fedorov, Irina Nikishina, Maria Kaleda, Kenan Barut, Amra Adrovic, Sezgin Sahin, Laurence Toumoulin, Johnny Frossard, Stephanie Archimbaut, Anne Paitier, Rolande Guastalli, Severine Guillaume Czitrom, Sirirat Charuvanij, Chollada Chaiyadech, Takako Miyamae, Hisashi Yamanaka, Cecile Picard, Guillaume Thouvenin, Caroline Kannengiesser, Jean-Christophe Dubus, Nadia Jeremiah, Frédéric Rieux-Laucat, Bruno Crestani, Véronique Secq, Christelle Ménard, Martine Reynaud-Gaubert, Françoise Thivolet-Bejui, Philippe Reix, Alexandre Belot, Ezgi Deniz Batu, Hafize Emine Sonmez, Abdulsamet Erden, Ekim Z. Taskiran, Omer Karadag, Umut Kalyoncu, İbrahim Oncel, Berkan Kaplan, Zehra Serap Arici, Cagri Mesut Temucin, Haluk Topaloglu, Yelda Bilginer, Mehmet Alikasifoglu, Seza Ozen, Lien Van Eyck, Ellen De Langhe, Isabelle Jéru, Erika Van Nieuwenhove, Vasiliki Lagou, Paul J. Baker, Jocelyn Garcia-Perez, James Dooley, Lien De Somer, Raf Sciot, Pierre-Yves Jeandel, Julia Ruuth-Praz, Bruno Copin, Myrna Medley-Hashim, Andre Megarbane, Sinisa Savic, An Goris, Serge Amselem, Adrian Liston, Seth Masters, Carine Wouters, Nami Okamoto, Yuko Sugita, Kousuke Shabana, Takuji Murata, Hiroshi Tamai, Juliana Ferenczová, Erika Banóova, Pavol Mrážik, Veronika Vargova, Dubravko Bajramovic, Ksenija Stekic Novacki, Kristina Potocki, Marijan Frkovic, Marija Jelusic, Olga Kostareva, Svetlana Arsenyeva, Anna Shapovalenko, Lennart Jans, Nele Herregods, Jacob Jaremko, Rik Joos, Joke Dehoorne, Xenofon Baraliakos, Sofia Ramiro, Julio C. Casasola-Vargas, Désirée van der Heijde, Robert Landewé, Ruben Burgos-Vargas, Shirley M. Tse, Gerd Horneff, Kristina Unnebrink, Jaclyn K. Anderson, Aysenur Paç Kisaarslan, Betül Sözeri, Zübeyde Gündüz, Gökmen Zararsız, Hakan Poyrazoğlu, Ruhan Düşünsel, Kazutaka Ouchi, Shinji Akioka, Hiroshi Kubo, Norio Nakagawa, Hajime Hosoi, Lovro Lamot, Fran Borovecki, Sanja Kapitanovic, Kristina Gotovac, Mandica Vidovic, Mirta Lamot, Edi Paleka Bosak, Miroslav Harjacek, Ricardo A. Russo, María M. Katsicas, Ruben Burgos Vargas, Ana L. Ortiz-Peyegahud, Zhang Pingping, Mou Yikun, Qi Jun, Jiang Yutong, Gu Jieruo, Mikhail M. Kostik, Shilova Ekaterina, Ilia Avrusin, Yuriy Korin, Olga Kopchak, Eugenia Isupova, Irina Chikova, Panova Tatyana, Margarita Dubko, Vera Masalova, Ludmila Snegireva, Tatyana Kornishina, Olga Kalashnikova, Vyacheslav Chasnyk, Tatyana Likhacheva, N. Ruperto, H. I. Brunner, P. Quartier, T. Constantin, E. Alexeeva, R. Schneider, I. Kone-Paut, K. Schikler, K. Marzan, N. Wulffraat, S. Padeh, V. Chasnyk, C. Wouters, J. B. Kuemmerle-Deschner, T. Kallinich, B. Lauwerys, E. Haddad, E. Nasonov, M. Trachana, O. Vougiouka, K. Leon, A. Speziale, K. Lheritier, E. Vritzali, A. Martini, D. Lovell, PRINTO/PRCSG, Nienke Ter Haar, Rianne Scholman, Wilco de Jager, Tamar Tak, Pieter Leliefeld, Bas Vastert, Sytze de Roock, Ariane de Ganck, Nadia Ryter, Miha Lavric, Dirk Foell, Renee F. Modica, Kathleen G. Lomax, Pamela Batzel, Armelle Cassanas, Melissa E. Elder, Rina Denisova, Ekaterina Alexeeva, Saniya Valieva, Tatyana Bzarova, Kseniya Isayeva, Tatyana Sleptsova, Olga Lomakina, Alexandra Chomahidze, Margarita Soloshenko, Meyry Shingarova, Elena Kachshenko, Wilco De Jager, Sebastiaan J. Vastert, Gerdien Mijnheer, Berent J. Prakken, Nico M. Wulffraat, Hafize E. Sönmez, Asuman N. Karhan, Ezgi D. Batu, Zehra S. Arıcı, Ersin Gümüş, Hülya Demir, Aysel Yüce, Seza Özen, Jasmina Ahluwalia, Bhavneet Bharti, Sweta Rajpal, Varun Uppal, Alaknanda Walia, Surjit S. Samlok, Narender Kumar, Clarissa C. Valões, Beatriz C. Molinari, Ana Claudia G. Pitta, Natali W. Gormezano, Sylvia C. Farhat, Kátia Kozu, Adriana M. Sallum, Simone Appenzeller, Ana Paula Sakamoto, Maria T. Terreri, Rosa M. Pereira, Claudia S. Magalhães, Cássia Maria Barbosa, Francisco Hugo Gomes, Eloisa Bonfá, Clovis A. Silva, Kubra Ozturk, Zelal Ekinci, Maie Helal, Natalia Cabrera, Jean Christophe Lega, Jocelyne Drai, Rene Ecochard, O. V. Shpitonkova, N. S. Podchernyaeva, Y. O. Kostina, N. G. Dashkova, M. K. Osminina, Gozde Yucel, Ahmet Arvas, Nandini Moorthy, Paraskevi Dimou, Angela Midgley, Matthew Peak, Simon C. Satchell, Rachael D. Wright, Rachel Corkhill, Eve M. Smith, Sagar Bhattad, Amit Rawat, Surjit Singh, Anju Gupta, Deepti Suri, Martin de Boer, Taco Kuijpers, Vignesh Pandiarajan, Sapna Sandal, Sebastian Giraldo, Roy Sanguino, Adriana S. Diaz, Selcuk Uzuner, Gizem Durcan, Ali Guven Kilicoglu, Ayhan Bilgic, Kayhan Bahali, Sinem Durmus, Hafize Uzun, Nur Canpolat, Salim Caliskan, Lale Sever, Tomomi Sato, Fuminori Kimura, Wafaa Suwairi, Reem Abdwani, Abdulaziz Al Rowais, Jubran Al qanatish, Abdulrahman Al Asiri, Ekaterina Gaidar, Mikhail Kostik, Elena Serogodskaya, Tatyana Nikitina, Evgenia Isupova, Elham Sardar, Perrine Dusser, Antoine Rousseau, Marc Labetoulle, Emanuel Barreau, Bahram Bodaghi, Isabelle Kone-Paut, Ivan Foeldvari, Jordi Anton, Rosa Bou, Sheila Angeles-Han, Regitze Bangsgaard, Gabriele Brumm, Tamas Constantin, Clive Edelsten, Jens Klotsche, Kirsten Minden, Elisabetta Miserocchi, Susan Nielsen, Gabriele Simonini, Arnd Heiligenhaus, Juan Manuel Mosquera Angarita, Carmen Garcia de Vicuña, Maria Victoria Hernandez, Alfredo Adan, Victor Llorens, Rosa Alcobendas, Susana Noval, Juan Carlos Lopez Robledillo, Isabel Valls, Mari Carmen Pinedo, Alejandro Fonollosa, Jaime de Inocencio, Pilar Tejada, Beatriz Bravo, Manuel Torribio, María Jesús García de Yebenes, Jordi Antón, Uveitis Working Group of the Spanish Pediatric Rheumatology Society, Lorenza Maria Argolini, Irene Pontikaki, Maria Orietta Borghi, Laura Cesana, Barbara Castiglioni, Maurizio Gattinara, Pierluigi Meroni, Pierre Quartier, Veronique Despert, Sylvaine Poignant, Amandine Baptiste, Caroline Elie, Laurent Kodjikian, Dominique Monnet, Michel Weber, Laura Moal, LuuLy Pham, Emmanuel Barreau, Cherif Titah, Pascal Dureau, Vanessa Cecchin, Maria Elisabetta Zannin, Daniele Ferrari, Francesco Comacchio, Rolando Cimaz, Fernanda Falcini, Antonella Petaccia, Stefania Viola, Luciana Breda, Francesco La Torre, Fabio Vittadello, Giorgia Martini, Francesco Zulian, Caroline Galeotti, Guillaume Sarrabay, Olivier Fogel, Isabelle Touitou, Corinne Miceli-Richard, Isabelle Koné-Paut, Hala Etayari, Hashad Soad, Ihab El Kadry, Habibullah Eatamadi, Kais AlAlgawi, Mustafa Al Maini, Khulood Khawaja, Sophie Van den Berghe, Ilse de Schryver, Ann Raes, Lídia L. C. Teixeira, Ana Duarte, Sandra Sousa, Filipe Vinagre, Maria J. Santos, Nataly S. Shevchenko, Ludmila F. Bogmat, Marina V. Demyanenko, Navdha R. Ramchurn, Mark Friswell, Rebecca A. James, Lucy R. Wedderburn, Reshma Pattani, Clarissa A. Pilkington, Sandrine Compeyrot-Lacassagne, Ana V. Villarreal, Nydia Acevedo, Enrique Faugier, Rocio Maldonado, Dilek Yılmaz, Hilal Bektaş Uysal, Elena Kamenets, Ekaterina Zaharova, Stefka Radenska-Lopovok, Joao Nascimento, Helena Sofia, Carla Zilhão, Rui Almeida, Margarida Guedes, Murat Deveci, Svetlana Rodionovskaya, Vera Vinnikova, Irina Tsymbal, Edyta Olesińska, Jacek Postępski, Agnieszka Mroczkowska-Juchkiewicz, Agnieszka Pawłowska-Kamieniak, Beata Chrapko, Damjana Ključevšek, Nina Emeršič, Nataša Toplak, Tadej Avčin, Faina Rokhlina, Galina Glazyrina, Natalia Kolyadina, Kwangnam Kim, Sinae Eom, Daeyoung Kim, Jungwoo Rhim, Francesca Ricci, Paola Montesano, Barbara Bonafini, Veronica Medeghini, Ilaria Parissenti, Antonella Meini, Marco Cattalini, Paolo Airò, Nataliya Panko, Nataliya Shevchenko, Iryna Lebec, Yevgeniya Zajceva, Sara Rostlund, Marie André, Takuma Hara, Takayuki Kishi, Yumi Tani, Aki Hanaya, Satoru Nagata, Velma Selmanovic, Aida Omercahic-Dizdarevic, Adisa Cengic, Almira Cosickic, Aida Omerčahić Dizdarević, Gemma Lepri, Clara Malattia, Eleonora Bellucci, Marco Matucci-Cerinic, Anton Solovyev, Elena Fedotova, Ana Victoria Villarreal, Talia Diaz, Yuridiana Ramirez, Teresa Giani, Achille Marino, Daniel Hunt, Muthana Al Obaidi, Veli Veli, Charalampia Papadopoulou, Jochen Kammermeier, Anna Poluha, Gangadhara C. Bharmappanavara, Alison Kelly, Lindsay Shaw, Giovanna Ferrara, Michele Luzzati, Mattia Giovannini, Liliana Jurado, Juliana Chamorro, Lorena Sarmiento, Ester Conversano, Maria Francesca Gicchino, Giulia Macchini, Carmela Granato, Assunta Tirelli, Alma N. Olivieri, Marija Perica, Lana Tambić Bukovac, Reza Sinaei, Vadood Javadi Parvaneh, Reza Shiari, Khosro Rahmani, Fatemeh F. Mehregan, Mehrnoush Hassas Yeganeh, Inmaculada Calvo Penadés, Berta López Montesinos, Ma Isabel González Fernández, Adriana Rodríguez Vidal, Anand Prahalad Rao, Ayesha Romana, Jyothi Raghuram, Ankur Kumar, Vishali Gupta, Elif Comak, Gülşah Kaya Aksoy, Aygen Yılmaz, Atike Atalay, Mustafa Koyun, Reha Artan, Sema Akman, Maria I. Kaleda, Irina P. Nikishina, Sergei K. Soloviev, Victor A. Malievsky, Ekaterina V. Nikolaeva, Agnieszka Gazda, Beata Kołodziejczyk, Lidia Rutkowska-Sak, Angela Mauro, Pierluigi Marzuillo, Stefano Guarino, and Angela La Manna

Subjects
Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Published
2017
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2. Pulmonary contusion after bumper car collision: Case report and review of the literature

Author

Mahé Berland, Marjolaine Oger, Emi Cauchois, Karine Retornaz, Valérie Arnoux, and Jean-Christophe Dubus

Subjects
Diseases of the respiratory system, RC705-779
Abstract

Thoracic trauma is a major source of morbi-mortality in injured children. Their pliable chest wall makes pulmonary contusion the most common chest injury. It is most often secondary to blunt trauma caused by traffic accidents. We report a case of severe chest trauma caused by a bumper car collision in an 8-years old girl. She sustained right lung contusion that led to complete atelectasis. After a week of supportive therapy, bronchoscopy removed a mucous plug from the main bronchus, resulting in significant clinical improvement. We aim to raise awareness of the risk of severe chest injuries during bumper car collisions.

Published
2018
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3. Bronchial epithelium in children: a key player in asthma

Author

Ania Carsin, Julie Mazenq, Alexandra Ilstad, Jean-Christophe Dubus, Pascal Chanez, and Delphine Gras

Subjects
Diseases of the respiratory system, RC705-779
Abstract

Bronchial epithelium is a key element of the respiratory airways. It constitutes the interface between the environment and the host. It is a physical barrier with many chemical and immunological properties. The bronchial epithelium is abnormal in asthma, even in children. It represents a key component promoting airway inflammation and remodelling that can lead to chronic symptoms. In this review, we present an overview of bronchial epithelium and how to study it, with a specific focus on children. We report physical, chemical and immunological properties from ex vivo and in vitro studies. The responses to various deleterious agents, such as viruses or allergens, may lead to persistent abnormalities orchestrated by bronchial epithelial cells. As epithelium dysfunctions occur early in asthma, reprogramming the epithelium may represent an ambitious goal to induce asthma remission in children.

Published
2016
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4. Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition

Author

Lucile Fievet, Claudia Natale, Xavier-Benoit D′Journo, Stéphanie Coze, Jean-Christophe Dubus, Jean-Michel Guys, Pascal Thomas, and Pascal De Lagausie

Subjects
Adenomatoid malformation, antenatal, paediatric, sequestration, thoracoscopy, Surgery, RD1-811, Diseases of the digestive system. Gastroenterology, RC799-869
Abstract

In adults, congenital pulmonary malformations are candidates for surgery due to symptoms. A pre-natal diagnosis is simple and effective, and allows an early thoracoscopic surgical treatment. A retrospective study was performed to assess management in two different populations of adults and children to define the best strategy. Subjects and Methods: Pulmonary malformations followed at the University Hospital from 2000 to 2012 were reviewed. Clinical history, malformation site, duration of hospitalisation, complications and pathology examinations were collected. Results: A total of 52 cases (33 children, 19 adults) were identified. In children, 28 asymptomatic cases were diagnosed pre-natally and 5 during the neonatal period due to infections. Surgery was performed on the children between the ages of 2 and 6 months. Nineteen adults underwent surgery, 16 because of symptoms and 3 adults for anomalies mimicking tumours. The mean age within the adult group was 42.5 years. In children, there was one thoracotomy and 32 thoracoscopies, with 7 conversions for difficult exposure, dissection of vascular pedicles, bleeding or bronchial injury. In the adults, there were 15 thoracotomies and 4 thoracoscopies, with one conversion. Post-operative complications in the adults were twice as frequent than in children. The mean time of the children′s hospitalisation was 7.75 days versus 7.16 days for the adults. Pathological examinations showed in the children: 7 sequestrations, 18 congenital cystic pulmonary malformations (CPAM), 8 CPAM associated sequestrations; in adults: 16 sequestrations, 3 intra-pulmonary cysts. Conclusion: Early thoracoscopic surgery allows pulmonary parenchyma conservation with pulmonary development, reduces respiratory and infectious complications, eliminates a false positive cancer diagnosis later in life and decreases risks of thoracic parietal deformation.

Published
2015
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5. Multivariate Analysis As a Support for Diagnostic Flowcharts in Allergic Bronchopulmonary Aspergillosis: A Proof-of-Concept Study

Author

Joana Vitte, Stéphane Ranque, Ania Carsin, Carine Gomez, Thomas Romain, Carole Cassagne, Marion Gouitaa, Mélisande Baravalle-Einaudi, Nathalie Stremler-Le Bel, Martine Reynaud-Gaubert, Jean-Christophe Dubus, Jean-Louis Mège, and Jean Gaudart

Subjects
allergic bronchopulmonary aspergillosis, Aspergillus fumigatus, immunoglobulin, molecular allergens, multivariate analysis, Immunologic diseases. Allergy, RC581-607
Abstract

Molecular-based allergy diagnosis yields multiple biomarker datasets. The classical diagnostic score for allergic bronchopulmonary aspergillosis (ABPA), a severe disease usually occurring in asthmatic patients and people with cystic fibrosis, comprises succinct immunological criteria formulated in 1977: total IgE, anti-Aspergillus fumigatus (Af) IgE, anti-Af “precipitins,” and anti-Af IgG. Progress achieved over the last four decades led to multiple IgE and IgG(4) Af biomarkers available with quantitative, standardized, molecular-level reports. These newly available biomarkers have not been included in the current diagnostic criteria, either individually or in algorithms, despite persistent underdiagnosis of ABPA. Large numbers of individual biomarkers may hinder their use in clinical practice. Conversely, multivariate analysis using new tools may bring about a better chance of less diagnostic mistakes. We report here a proof-of-concept work consisting of a three-step multivariate analysis of Af IgE, IgG, and IgG4 biomarkers through a combination of principal component analysis, hierarchical ascendant classification, and classification and regression tree multivariate analysis. The resulting diagnostic algorithms might show the way for novel criteria and improved diagnostic efficiency in Af-sensitized patients at risk for ABPA.

Published
2017
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6. When Christmas decoration goes hand in hand with bronchial aspiration …

Author

Ania Carsin, Mélisande Baravalle-Einaudi, and Jean-Christophe Dubus

Subjects
Child, Foreign body aspiration, Domestic injury, Diseases of the respiratory system, RC705-779
Abstract

We report the case of a 14-month-old girl suffering from cough and wheeze around Christmas. She was treated with anti-asthmatic drugs with no success, and 3 weeks later a chest X-ray revealed a LED bulb in the left main bronchus. This LED bulb came from a Christmas light garland decorating the Christmas tree. We discuss the different Christmas objects that can be inhaled by young children, the challenge to diagnose bronchial inhalation during this winter period, and the emergence of new foreign bodies, such as LED bulbs, with a particularly aerodynamic shape.

Published
2017
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7. Rapid Improvement after Starting Elexacaftor–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease

Author

Pierre-Régis Burgel, Isabelle Durieu, Raphaël Chiron, Sophie Ramel, Isabelle Danner-Boucher, Anne Prevotat, Dominique Grenet, Christophe Marguet, Martine Reynaud-Gaubert, Julie Macey, Laurent Mely, Annlyse Fanton, Sébastien Quetant, Lydie Lemonnier, Jean-Louis Paillasseur, Jennifer Da Silva, Clémence Martin, Claire Andrejak, Arnaud Becourt, Julie Mounard, Claire Poulet, Cinthia Rames, Marie Talleux, Marie-Chantal Chevalier, Estelle Darviot, Marie Jouvenot, Caroline Marien, Audrey Paris, Cécile Pelatan, Christine Person, Pascaline Priou, Françoise Troussier, Thierry Urban, Marie-Laure Dalphin, Jean-Charles Dalphin, Alice Ladaurade, Didier Pernet, Bénédicte Richaud-Thiriez, Pauline Roux-Claude, Nathalia Blanc, Vincent Boisserie-Lacroix, Stephanie Bui, Cyrielle Collet, Stéphane Debelleix, Emmanuel Bergot, Jacques Brouard, Karine Campbell, Muriel Laurans, Virginie Ribault, Corinne Borderon, Marie-Christine Heraud, Guillaume Labbe, Sylvie Montcouquiol, Isabelle Petit, Marc Ruivard, Céline Delestrain, Benoit Douvry, Ralph Epaud, Bernard Maitre, Natascha Remus, Guillaume Beltramo, Anne Houzel, Frédéric Huet, Stéphanie Perez, Amale Boldron-Ghaddar, Manuela Scalbert, Rabah Bouzioukh, Charles Simon, Boubou Camara, Rébecca Hamidfar, Catherine Llerena, Isabelle Pin, Antoine Deschildre, Alice Gicquello, Olivier Le Rouzic, Clara Leroy, Nicolas Paris, Thierry Perez, Caroline Thumerelle, Dominique Turck, Nathalie Wizla, Magali Dupuy-Grasset, Jane Languepin, Alexandra Masson-Rouchaud, Céline Menetrey, Stéphane Durupt, Sophie L’Excellent, Raphaele Nove-Josserand, Camille Ohlmann, Phillipe Reix, Quitterie Reynaud, Marie-Christine Werck-Gallois, Mélissandre Baravalle, Bérangère Coltey, Nadine Desmazes-Dufeu, Jean-Christophe Dubus, Clarisse Gautier, Jean-Baptiste Rey, Nathalie Stremler, Davide Caimmi, Margot Devrait, Johan Moreau, Yves Billon, Aurore Blondé, Anne Guillaumot, Sébastien Kiefer, Laura Peretti, Aurélie Tatopoulos, Angélica Tiotiu, Myriam Benhamida, Tiphaine Bihouee, Emmanuel Eschapasse, Adrien Tissot, Marie Giannantonio, Sylvie Leroy, Carole Piccini-Bailly, Johana Pradelli, Jonathan Messika, Véronique Boussaud, Nicolas Carlier, Isabelle Honoré, Dominique Hubert, Reem Kanaan, Céline Bailly-Botuha, Frédérique Chedevergne, Jacques De Blic, Christophe Delacourt, David Drummond, Brigitte Fauroux, Chantal Karila, Muriel Le Bourgeois, Isabelle Sermet, Bertrand Delaisi, Michèle Gerardin, Veronique Houdouin, Laurence Leclainche, Guillaume Aubertin, Laura Berdah, Annick Clement, Harriet Corvol, Nadia Nathan, Blandine Prevost, Nicolas Richard, Aline Tamalet, Jessica Taytard, Guillaume Thouvenin, Barbara Tourniaire, Michel Abely, Katia Bessaci-Kabouya, Sandra Dury, Bruno Ravoninjatovo, Alain Dabadie, Michel Dagorne, Eric Deneuville, Marie Jamin, Mélanie Ribault, Clémentine Vigier, Chantal Belleguic, Graziella Brinchault, Benoit Desrues, Audrey Barzic, Anne Dirou-Prigent, Jean Le Bihan, Krista Revert, Thomas Ropars, Laure Couderc, Stéphane Dominique, Hélène Morisse-Pradier, Stéphanie Pramil, Luc Thiberville, Nathalie Allou, Laurent Enaud, Elsa Gachelin, Eric Huchot, Annabelle Payet, Caroline Perisson, Saguiraly Piyaraly, Sophie Valois, Audrey Herzog, Romain Kessler, Michele Porzio, Laurence Weiss, Laurence Beaumont, Olivier Brugiere, Sylvie Colin, de Verdiere, Elise Cuquemelle, Sandra De Miranda, Adbdul Monem Hamid, François Parquin, Clément Picard, Antoine Roux, Charlotte Roy, François Bremont, Alain Didier, Marion Dupuis, Guillaume Faviez, Géraldine Labouret, Marie Mittaine, Marlène Murris-Espin, Léa Roditis, Laure Cosson, Patrice Diot, Thomas Flament, Charlotte Giraut, Julie Mankikian, Baptiste Arnouat, Gaétane Mousset, Véronique Storni, Philippe Vigneron, Emmanuelle Coirier-Duet, Asma Gabsi, Institut Cochin (IC UM3 (UMR 8104 / U1016)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hospices Civils de Lyon (HCL), Université de Lyon, Hôpital Arnaud de Villeneuve [CHRU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Fondation ILDYS (ILDYS), Institut du Thorax [Nantes], Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Foch [Suresnes], CHU Rouen, Normandie Université (NU), INSERM-TRANSFERT [Paris] (IT), Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rouen Normandie (UNIROUEN), Hôpital Nord [CHU - APHM], Aix Marseille Université (AMU), CHU Bordeaux [Bordeaux], Hôpital Renée Sabran [CHU - HCL], CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Universitaire [Grenoble] (CHU), Vaincre la Mucoviscidose, Association de lutte contre la Mucoviscidose, EFFI-STAT, URC/CIC Paris Descartes Necker Cochin, Hôpital Necker, Institut Desbrest de santé publique (IDESP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Supported by Vaincre la Mucoviscidose, Fondation Sauver la Vie, Universite Paris Descartes, Filiere Maladies Rares Muco-CFTR, and Legs PascalBonnet., Participating Investigators of the FrenchCystic Fibrosis Reference Network StudyGroup:Claire Andrejak, Arnaud Becourt, JulieMounard, Claire Poulet, Cinthia Rames, MarieTalleux (Amiens), Marie-Chantal Chevalier,Estelle Darviot, Marie Jouvenot, Caroline Marien,Audrey Paris, C ecile Pelatan, Christine Person,Pascaline Priou, Franc ̧oise Troussier, ThierryUrban (Angers-Le Mans), Marie-Laure Dalphin,Jean-Charles Dalphin, Alice Ladaurade, DidierPernet, B en edicte Richaud-Thiriez, PaulineRoux-Claude (Besanc ̧on), Nathalia Blanc,Vincent Boisserie-Lacroix, Stephanie Bui,Cyrielle Collet, St ephane Debelleix, Julie Macey(Bordeaux), Emmanuel Bergot, JacquesBrouard, Karine Campbell, Muriel Laurans,Virginie Ribault (Caen), Corinne Borderon,Marie-Christine Heraud, Guillaume Labbe,SylvieMontcouquiol, Isabelle Petit, Marc Ruivard(Clermont-Ferrand), C eline Delestrain, BenoitDouvry, Ralph Epaud, Bernard Maitre, NataschaRemus (Cr eteil), Guillaume Beltramo, AnnlyseFanton, Anne Houzel, Fr ed eric Huet, St ephaniePerez (Dijon), AmaleBoldron-Ghaddar, ManuelaScalbert (Dunkerque), Rabah Bouzioukh,Laurent Mely, Charles Simon (Giens), BoubouCamara, R ebecca Hamidfar, Catherine Llerena,Isabelle Pin, S ebastien Quetant (Grenoble), Antoine Deschildre, Alice Gicquello, Olivier LeRouzic, Clara Leroy, Nicolas Paris, Thierry Perez,Anne Prevotat, Caroline Thumerelle, DominiqueTurck, Nathalie Wizla (Lille), Magali Dupuy-Grasset, Jane Languepin, Alexandra Masson-Rouchaud, C eline Menetrey (Limoges), IsabelleDurieu, St ephane Durupt, Sophie L’Excellent,Raphaele Nove-Josserand, Camille Ohlmann,Phillipe Reix, Quitterie Reynaud, Marie-ChristineWerck-Gallois (Lyon), M elissandre Baravalle,B erang ere Coltey, Nadine Desmazes-Dufeu,Jean-Christophe Dubus, Clarisse Gautier, Jean-Baptiste Rey, Martine Reynaud-Gaubert,Nathalie Stremler (Marseille), Davide Caimmi,Rapha€el Chiron, Margot Devrait, Johan Moreau(Montpellier), Yves Billon, Aurore Blond e, AnneGuillaumot, S ebastien Kiefer, Laura Peretti,Aur elie Tatopoulos, Ang elica Tiotiu (Nancy), Myriam Benhamida, Tiphaine Bihouee, IsabelleDanner-Boucher, Emmanuel Eschapasse,Adrien Tissot (Nantes), Marie Giannantonio,Sylvie Leroy, Carole Piccini-Bailly, JohanaPradelli (Nice), Jonathan Messika (Paris, Bichat), V eronique Boussaud, Pierre-R egis Burgel,Nicolas Carlier, Isabelle Honor e, DominiqueHubert, Reem Kanaan, Cl emence Martin (Paris,Cochin), C eline Bailly-Botuha, Fr ed eriqueChedevergne, Jacques De Blic, ChristopheDelacourt, David Drummond, Brigitte Fauroux,Chantal Karila, Muriel Le Bourgeois, IsabelleSermet (Paris, Necker), Bertrand Delaisi,Mich ele Gerardin, Veronique Houdouin,Laurence Leclainche (Paris, Robert Debr e), Guillaume Aubertin, Laura Berdah, AnnickClement, Harriet Corvol, Nadia Nathan, BlandinePrevost, Nicolas Richard, Aline Tamalet, JessicaTaytard, Guillaume Thouvenin, BarbaraTourniaire (Paris, Trousseau), Michel Abely,Katia Bessaci-Kabouya, Sandra Dury, BrunoRavoninjatovo (Reims), Alain Dabadie, MichelDagorne, Eric Deneuville, Marie Jamin, M elanieRibault, Cl ementine Vigier (Rennes – SaintBrieuc), Chantal Belleguic, Graziella Brinchault,Benoit Desrues (Rennes), Audrey Barzic, AnneDirou-Prigent, Jean Le Bihan, Sophie Ramel,Krista Revert, Thomas Ropars (Roscoff), LaureCouderc, St ephane Dominique, ChristopheMarguet, H el ene Morisse-Pradier, St ephaniePramil, Luc Thiberville (Rouen), Nathalie Allou,Laurent Enaud, Elsa Gachelin, Eric Huchot,Annabelle Payet, Caroline Perisson, SaguiralyPiyaraly, Sophie Valois (La R eunion), AudreyHerzog, Romain Kessler, Michele Porzio,Laurence Weiss (Strasbourg), LaurenceBeaumont, Olivier Brugiere, Sylvie Colin deVerdiere, Elise Cuquemelle, Sandra De Miranda,Dominique Grenet, Adbdul Monem Hamid,Franc ̧ois Parquin, Cl ementPicard, Antoine Roux,Charlotte Roy (Suresnes), Franc ̧ois Bremont,Alain Didier, Marion Dupuis, Guillaume Faviez,G eraldine Labouret, Marie Mittaine, Marl eneMurris-Espin, L ea Roditis (Toulouse), LaureCosson, Patrice Diot, Thomas Flament, CharlotteGiraut, JulieMankikian(Tours), Baptiste Arnouat,Ga etane Mousset, V eronique Storni, PhilippeVigneron (Vannes-Lorient), and Emmanuelle Coirier-Duet, and Asma Gabsi (Versailles)

Subjects
Lung Diseases, Male, elexacaftor, Indoles, Cystic Fibrosis, Pyridines, medicine.medical_treatment, Regulator, Aminophenols, Critical Care and Intensive Care Medicine, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Cystic fibrosis, Gastroenterology, Membrane Potentials, Ivacaftor, 0302 clinical medicine, Prospective Studies, 030212 general & internal medicine, Chloride Channel Agonists, Aged, 80 and over, Middle Aged, Transmembrane protein, Drug Combinations, Quinolines, Female, France, medicine.drug, Adult, Pulmonary and Respiratory Medicine, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, cystic fibrosis transmembrane conductance regulator modulators, Adolescent, Pulmonary disease, Young Adult, 03 medical and health sciences, Internal medicine, lung transplantation, medicine, Humans, Lung transplantation, In patient, Aged, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, business.industry, Editorials, medicine.disease, 030228 respiratory system, Tezacaftor, Pyrazoles, business
Abstract

International audience; Rationale: Elexacaftor-tezacaftor-ivacaftor is a CFTR (cystic fibrosis [CF] transmembrane conductance regulator) modulator combination, developed for patients with CF with at least one Phe508del mutation. Objectives: To evaluate the effects of elexacaftor-tezacaftor- ivacaftor in patients with CF and advanced respiratory disease. Methods: A prospective observational study, including all patients aged ⩾12 years and with a percent-predicted FEV1 (ppFEV1)

Published
2021

8. Aide au sevrage tabagique : quelles modalités pratiques en pédiatrie ?

Author

Véronique Houdouin, pour le groupe tabac-cannabis de la Société française de pédiatrie, G. Benoist, Caroline Thumerelle, Jean-Christophe Dubus, and M. Eymery

Subjects
03 medical and health sciences, 0302 clinical medicine, 030212 general & internal medicine, 010501 environmental sciences, 01 natural sciences, 0105 earth and related environmental sciences
Abstract

Resume L’aide au sevrage tabagique est du devoir de tout professionnel de sante. Les pediatres sont eux-memes largement concernes par cette problematique. Se basant sur le modele d’approche comportementale de Prochaska et DiClemente, le modele anglo-saxon des « 5 A » propose une demarche en cinq etapes afin d’accompagner tout fumeur a travers les differents stades du changement. Les trois premiers A (Ask, Advise, Assess) sont accessibles a tout praticien : il s’agit de depister, conseiller l’arret et evaluer la dependance, ainsi que d’apprecier la motivation a l’arret. Plusieurs outils sont a la disposition du pediatre. Les deux A suivants (Assist et Arrange) correspondent au soutien et au suivi de la personne qui passe a l’action et franchit le pas du sevrage. Le soutien psychologique et la prescription de traitements nicotiniques substitutifs quand necessaire s’inscrivent dans ces deux dernieres etapes. Plus specifiques, celles-ci restent neanmoins abordables en pratique pediatrique courante, et permettront au fumeur de retrouver sa liberte face au tabagisme. Trois situations impliqueront tout specialement le pediatre : la grossesse, l’allaitement et l’adolescent fumeur, avec des specificites qu’il faut connaitre, et pour lesquelles des benefices particulierement importants sont a attendre du sevrage.

Published
2020

9. Drug hypersensitivity in children: a retrospective analysis of 101 pharmacovigilance reports

Author

Maria Katsogiannou, Julie Mazenq, Jean-Christophe Dubus, Marie-José Gervoise-Boyer, Ania Carsin, Hôpital de la Timone [CHU - APHM] (TIMONE), Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM], Microbes évolution phylogénie et infections (MEPHI), and Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Hypersensitivity, Immediate, Drug, medicine.medical_specialty, Allergy, medicine.drug_class, [SDV]Life Sciences [q-bio], media_common.quotation_subject, Antibiotics, Provocation test, Drug allergy, Penicillins, Drug Hypersensitivity, Pharmacovigilance, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, Internal medicine, medicine, Humans, 030212 general & internal medicine, Child, Retrospective Studies, Skin Tests, media_common, business.industry, medicine.disease, Anti-Bacterial Agents, 3. Good health, Hypersensitivity reaction, Penicillin, Pediatrics, Perinatology and Child Health, business, medicine.drug
Abstract

International audience; Our objective was to describe and discuss management of recent cases of drug hypersensitivity in children reported in a pharmacovigilance center. Two pediatric allergy units conducted a collaborative retrospective analysis of 101 adverse drug reactions reported to a regional pharmacovigilance center between January 2016 and July 2019. Time lapse between hypersensitivity reaction onset and allergy consultation varied from 1 month to 12 years. Sixty-two patients (61.4%) presented with immediate reactions, 11 (10.9%) with non-immediate reactions, and 28 (27.7%) had reactions impossible to classify through medical interview. Overall, 92 children (91%) were explored for simultaneously administered drugs. All 113 prick tests were negative, and 2 were uncertain. Among 108 intradermal tests, 2 were positive to penicillin and to an iodinated contrast medium, 105 were negative, and 1 was uncertain. Overall, 129 drug provocation tests were proposed. Nine provocation tests among 80 were positive (11.25%): 6 to penicillin, 1 to sulfonamide antibiotics, and 2 to non-steroidal anti-inflammatory drugs; the remaining 71 were negative. No severe reaction was observed during these tests. Finally, drug allergy was only retained in 11 reported cases (10.9%). Conclusion: These pharmacovigilance reports show the difficulty in defining drug allergy in children only by anamnesis, and that explorations, particularly provocation tests, should take place at a reasonable time lapse after drug hypersensitivity reaction onset. What is Known: True drug allergy is rarely observed in children. Absence of full workup leads to falsely labeling children as ``allergic.'' What is New: Short time lapse between hypersensitivity onset and consultation improves classification of pediatric allergy. Timely allergy consultations are essential, and tests are useful to confirm or exclude pediatric allergy.

Published
2020

10. French national cohort of neuroendocrine cell hyperplasia of infancy (FRENCHI) study: diagnosis and initial management

Author

Candice Fabre, Caroline Thumerelle, Morgane Dervaux, Rola Abou-Taam, Tiphaine Bihouee, Jacques Brouard, Annick Clement, Christophe Delacourt, Céline Delestrain, Ralph Epaud, Sofiane Ghdifan, Alice Hadchouel, Véronique Houdouin, Géraldine Labouret, Caroline Perisson, Philippe Reix, Marie-Catherine Renoux, Françoise Troussier, Laurence Weiss, Julie Mazenq, Nadia Nathan, Jean-Christophe Dubus, Hôpital de la Timone [CHU - APHM] (TIMONE), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Aix-Marseille Université - École de médecine (AMU SMPM MED), Aix-Marseille Université - Faculté des sciences médicales et paramédicales (AMU SMPM), Aix Marseille Université (AMU)-Aix Marseille Université (AMU), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre hospitalier universitaire de Nantes (CHU Nantes), Service de Pédiatrie Médicale [Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Intercommunal de Créteil (CHIC), Service de Pneumologie Allergologie [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), CHU Sud Saint Pierre [Ile de la Réunion], Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Microbes évolution phylogénie et infections (MEPHI), and Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Male, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Hyperplasia, Clinical score, Neuroendocrine cell hyperplasia of infancy, Childhood interstitial lung disease, Cohort, Infant, Newborn, Infant, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, Rare Diseases, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Neuroendocrine Cells, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Anti-inflammatory drugs, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Pediatrics, Perinatology and Child Health, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Child, Lung Diseases, Interstitial, Children, Lung, Retrospective Studies
Abstract

Early diagnosis of neuroendocrine cell hyperplasia of infancy (NEHI) is crucial as, conversely to the other causes of intersititial lung disease, corticosteroids are not recommended. Diagnosis is historically based on lung biopsy (NEHI), but in current practice, a clinical and radiological approach is more and more preferred (NEHI syndrome). This national study aimed to address diagnosis and initial management of patients followed up for a NEHI pattern in pediatric centers for rare lung diseases (RespiRare, France). Data on neonatal and familial events, symptoms at diagnosis, explorations performed and results, and therapeutic management were collected by questionnaire. Fifty-four children were included (boys 63%). The mean onset of symptoms was 3.8 ± 2.6 months. The most frequent symptoms at diagnosis were tachypnea (100%), retraction (79.6%), crackles (66.7%), and hypoxemia (59.3%). The mean NEHI clinical score, evocative when ≥ 7/10, was 7.9 ± 1.4 (76% with a score ≥ 7). All chest CT-scans showed ground glass opacities evolving at least the middle lobe and the lingula. Lung biopsy was performed in 38.9% of the cases and was typical of NEHI in only 52.4%, even when the clinical presentation was typical. Initial treatments were oxygen (83.6%) and more curiously intravenous pulses of steroids (83.3%) and azithromycin (70.2%).This national cohort of patients underlines diagnosis difficulties of NEHI. A composite clinical and radiological score should help clinicians for limiting the use of anti-inflammatory drugs.•Neuroendocrine cell hyperplasia of infancy (NEHI) is an interstitial lung disease whose diagnosis is essential to limit corticosteroids therapy.•In this national cohort of 54 patients with a NEHI pattern, diagnosis is mainly based on clinical symptoms and chest CT-scan results. The newly proposed clinical score and, when performed, the lung biopsies are faulted in 25 and 50% of the cases, respectively. •Corticosteroids are widely used. Such results plead for a new composite score to formally diagnose NEHI.

Published
2022

11. Successful Treatment of Interstitial Lung Disease in STAT3 Gain-of-Function Using JAK Inhibitors

Author

Vincent Barlogis, Sarah Marchal, Lisa Giovannini-Chami, Jean-Christophe Dubus, Lisa R. Forbes, Alexandre Fabre, Manuel Silva-Carmona, Tiphanie P Vogel, Sylvie Leroy, and Myriam Guesmi

Subjects
Pulmonary and Respiratory Medicine, STAT3 GOF, Gain of function, biology, business.industry, biology.protein, Interstitial lung disease, Cancer research, Medicine, Critical Care and Intensive Care Medicine, business, STAT3, medicine.disease
Published
2020

12. Les tabagismes passif, expositionnel (environnemental) et actif de l’enfant et de l’adolescent : quelle est la place du pédiatre ?

Author

Jean-Christophe Dubus, M. Eymery, G. Benoist, Véronique Houdouin, groupe tabac-cannabis de la Société Française de Pédiatrie, and Caroline Thumerelle

Subjects
03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, 030212 general & internal medicine
Abstract

Resume Le tabagisme a ete declare « maladie pediatrique » par les instances internationales. Il peut en effet impacter la population pediatrique sous ses trois formes : tabagisme passif (in utero), environnemental (ou expositionnel), et actif a l’adolescence. Ses consequences, avec notamment un risque majore de survenue de pathologies respiratoires et infectieuses sont de mieux en mieux connues, et de nouveaux liens avec differentes pathologies telles que le diabete, la survenue de trouble de l’attention ou encore de certaines tumeurs semblent etablis. Au meme titre que la vaccination ou la prevention des accidents domestiques, le role de prevention du pediatre inclut la lutte anti-tabagique, en informant les parents des risques que represente leur consommation, et en luttant si necessaire contre un certain nombre d’idees recues. Le tabagisme des adolescents est par ailleurs un veritable fleau dont il faut se preoccuper systematiquement. On retrouve chez ces adolescents fumeurs actifs de nombreuses particularites physiologiques, psychologiques et societales quant a la consommation tabagique, au premier rang desquelles un risque de survenue de dependance particulierement rapide. La connaissance de ces particularites permettra de guider les strategies de prevention a leur egard, et ce d’autant plus qu’eux-memes sous-estiment tres largement les risques que leur propre tabagisme peut representer.

Published
2020

13. Real-life requests for Bordetella polymerase chain reaction testing in children presenting to hospital

Author

Aurélie Morand, Julie Mazenq, N. Ghez, Jean-Christophe Dubus, G. Dubourg, E. Bosdure, Department of Pediatric and Prenatal Radiology, Timone's Children-Hospital (APHM), Hôpital de la Timone [CHU - APHM] (TIMONE), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), and Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille)

Subjects
DNA, Bacterial, Male, Pediatrics, medicine.medical_specialty, Pertussis immunization, Whooping Cough, Medical Overuse, Polymerase Chain Reaction, Bordetella pertussis, law.invention, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, law, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, medicine, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Respiratory system, Child, Polymerase chain reaction, ComputingMilieux_MISCELLANEOUS, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Respiratory illness, biology, business.industry, Infant, Emergency department, University hospital, biology.organism_classification, Antibodies, Bacterial, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, Hospitals, Clinical Practice, Bordetella, Cough, Child, Preschool, Pediatrics, Perinatology and Child Health, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Female, France, business
Abstract

From 2015 to 2017, 3197 interpretable Bordetella polymerase chain reaction (PCR) tests were performed for 2760 children presenting to our tertiary university hospital. Requests mainly came from the emergency department (62%) and for children older than 1 year (68%). Only 32 PCR (1%) results were positive, mainly in children younger than 1 year (n = 29/32, 90.6%; p

Published
2022

14. Management of primary spontaneous pneumothorax in teenagers: An 11-year study

Author

Eve Benhaïm, Brimbelle Roth, Fabrice Michel, Thierry Merrot, and Jean‐Christophe Dubus

Subjects
Adult, Male, Adolescent, Thoracic Surgery, Video-Assisted, Pneumothorax, General Medicine, Treatment Outcome, Recurrence, Chest Tubes, Pediatrics, Perinatology and Child Health, Drainage, Humans, Female, Child, Retrospective Studies
Abstract

Management of teenagers with primary spontaneous pneumothorax (PSP) is not consensual. We report our experience over an 11-year period.For each patient under 20 years hospitalised with PSP from 2008 to 2018, demographic data, smoking habits, clinical presentation, hospitalisation unit, radiological management and its results, therapeutic management (observation, needle aspiration, chest tube drainage and surgery), complications, length of stay, given advice at discharge and recurrence were collected.Seventy patients were included in different paediatric or adult surgery or pulmonology wards (82.9% boys; 16.8 ± 1.7 years; one severe presentation; 18/58 smokers). Chest CT-scan (n = 42/70, 60%) revealed blebs/bullae in 18/39 examinations (46.2%). Treatment consisted of observation (14/70, 20%), needle aspiration (2/70, 2.9%), chest tube (53/70, 75.7%) and video-assisted thoracoscopy surgery (27/70, 38.6%). Half patients with interventional procedure presented complications. A median of 10 chest X-rays was noted during a median stay of 8 days. Advice concerning sport practice, flying, smoking, etc., was variably delivered. PSP recurrence concerned 35/70 patients (50%) without identified predictive factors.Compared to recent recommendations of a more conservative approach, chest CT-scan and interventional strategy are overused in our teenagers with PSP. Observation, more or less needle aspiration, should be clearly the first-line treatments.

Published
2021

15. Deep phenotyping of MARS1 (interstitial lung and liver disease) and LARS1 (infantile liver failure syndrome 1) recessive multisystemic disease using Human Phenotype Ontology annotation: Overlap and differences. Case report and review of literature

Author

Charlotte La Fay, Pauline Heux, Anne Spraul, Marine Juzaud, Alice Hadchouel, Alexandre Fabre, Jean-Christophe Dubus, Celia Hoebeke, Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Bicêtre Hospital, Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pédiatrie spécialisée et médecine infantile (neurologie, pneumologie, maladies héréditaires du métabolisme) [Hôpital de la Timone - APHM], Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Service de Pneumologie Allergologie [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Génétique Médicale et Génomique Fonctionnelle (GMGF), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), and COMBE, Isabelle

Subjects
Male, medicine.disease_cause, Bioinformatics, Liver disease, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, MARS1, Hypoalbuminemia, Genetics (clinical), ComputingMilieux_MISCELLANEOUS, [SDV.MP.VIR] Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.MHEP.ME] Life Sciences [q-bio]/Human health and pathology/Emerging diseases, 0303 health sciences, Mutation, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Liver Diseases, 030305 genetics & heredity, LARS1, General Medicine, Syndrome, Phenotype, 3. Good health, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Failure to thrive, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.MHEP.MI] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Female, medicine.symptom, Pulmonary alveolar proteinosis, [SDV.MP.PAR] Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Methionine-tRNA Ligase, 03 medical and health sciences, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Human Phenotype Ontology, Genetics, medicine, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Gene, 030304 developmental biology, business.industry, Infant, Infantile liver failure syndrome type 1, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, Failure to Thrive, tRNA synthetase deficiency, Leucine-tRNA Ligase, [SDV.MP.BAC] Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, business, Lung Diseases, Interstitial
Abstract

Introduction Aminoacyl transfer RNA (tRNA) synthetases are associated with diseases when mutations occur in their encoding genes. Pulmonary alveolar proteinosis can be caused by mutation in the methionyl-tRNA synthetase (MARS) gene while mutations in the leucine-tRNA synthetase (LARS) gene lead to infantile liver failure syndrome type 1. We report the case of a patient with LARS1 pathogenics variants and two patients with MARS1 pathogenics variants. The aim of this study was to analyze the phenotypes of our three patients in detail and classify cases in the literature using Human Phenotype Ontology (HPO) terms. Results The first patient has two previously undescribed heterozygous variants in LARS1 (c.1818dup and c.463A>G). The other two patients' MARS1 variants (c.1177G>A and c.1700C>T) have already been described in the literature. All three patients had anemia, hepatomegaly, feeding difficulties, failure to thrive and hypoalbuminemia. Including ours, 65 patients are described in total, for whom 117 phenotypic abnormalities have been described at least once, 41.9% of which both in patients with LARS1 and MARS1 mutations. Conclusion Patients with LARS1 and MARS1 mutations seem to share a common phenotype but further deep phenotyping studies are required to clarify the details of these complex pathologies.

Published
2021

16. Factors Associated with Asthma Severity in Children: Data from the French COBRAPed Cohort

Author

Muriel Le Bourgeois, Guillaume Lezmi, Raphaël Chiron, Marie-Alexandra Alyanakian, Jacques de Blic, Jacques Brouard, Valérie Jolaine, Isabelle Pin, Sylvain Blanchon, Léa Roditis, Christophe Delacourt, C. Mordacq, Emmanuelle Bosdure, Mathieu Pellan, Véronique Houdoin, Graziella Mingardi, David Drummond, Irina Badiu-Decleyre, Patrick Berger, Rola Abou-Taam, V. Jubin, S. Vrielynck, Caroline Tournegros, Sylvie-Anne André Gomez, Julie Mazenq, Marie-Christine Werck Gallois, Stéphanie Wanin, Philippe Reix, Naïm Bouazza, Stephane Debelleix, Camille Ohlmann, Antoine Deschildre, Caroline Thumerelle, Alice Hadchouel-Duvergé, Guillaume Simon, Marie Noelle Lebras, Laurent Béghin, Lucienne Chatennoud, Stéphanie Lejeune, Isabelle Cabon, Patricia El Boustany, Jean-Christophe Dubus, Cecile Bonnel, Valérie Siao, Michael Fayon, Christophe Marguet, Laure Delbecque, Lisa Giovannini-Chami, Marine Servat, Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), Service de Pneumologie et d'Allergologie Pédiatriques, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), AP-HP Hôpital universitaire Robert-Debré [Paris], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
Pediatrics, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Asthma severity, Disease, Dermatitis, Atopic, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Food allergy, Risk Factors, medicine, Immunology and Allergy, Humans, 030212 general & internal medicine, Child, ComputingMilieux_MISCELLANEOUS, Asthma, Respiratory Sounds, business.industry, Atopic dermatitis, Odds ratio, medicine.disease, 3. Good health, 030228 respiratory system, Child, Preschool, Cohort, Observational study, business, Food Hypersensitivity
Abstract

Background Severe asthma (SA) in children is a complex, heterogeneous disease, associated with a considerable burden. However, factors influencing asthma severity are poorly described and may differ according to age. Objective To determine whether factors associated with asthma severity differ between preschoolers with severe recurrent wheeze (SRW) and school-age children with SA. Methods Data from the French multicenter prospective observational cohort of preschool (3-6 years) children with SRW and nonsevere recurrent wheeze (NSRW) and school-age (7-11 years) children with SA and nonsevere asthma (NSA) (Pediatric Cohort of Bronchial Obstruction and Asthma) were analyzed. Results A total of 131 preschool children (92 SRW and 49 NSRW) and 207 school-age children (92 SA and 115 NSA) were included. In both univariable and multivariable analysis, SRW was associated with second-hand smoke exposure (multivariable analysis: odds ratio [95% CI], 29.8 [3.57-3910]) and exposure to mold/dampness at home (multivariable analysis: odds ratio [95% CI], 4.22 [1.25-18.2]) compared with NSRW. At school-age, history of atopic dermatitis and food allergy was more frequent in children with SA than in those with NSA. Multivariable analysis confirmed that SA was associated with a history of food allergy (odds ratio [95% CI], 5.01 [2.23-11.9]). Conclusions Our data suggest that factors influencing asthma severity may differ according to age. In preschool children with SRW, second-hand smoke and exposure to mold are predominant, whereas associated allergic disorders are mainly involved in SA at school-age.

Published
2021

17. Nebulization and COVID-19: Is the risk of spread actual?

Author

Christophe Thibon, Laurent Vecellio, Jean-Christophe Dubus, Benoît Kabamba, Gregory Reychler, UCL - SSS/IREC/MBLG - Pôle de Microbiologie médicale, UCL - (SLuc) Service de microbiologie, UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, and UCL - (SLuc) Service de pneumologie

Subjects
Pulmonary and Respiratory Medicine, Nebulizers and Vaporizers, Administration, Inhalation, COVID-19, Humans
Abstract

No abstract available

Published
2022

18. Microbiome and the immune system: From a healthy steady-state to allergy associated disruption

Author

Justin Michel, Yannick Chantran, Joana Vitte, Youssouf Sereme, Soraya Mezouar, Jean-Louis Mege, Stéphane Ranque, Jean-Christophe Dubus, Marc Leone, Alexandre Fabre, Pascal Chanez, and Benoit Desnues

Subjects
0301 basic medicine, Microbiology (medical), Allergy, lcsh:QR1-502, Disease, medicine.disease_cause, lcsh:Microbiology, Autoimmunity, Pathogenesis, 03 medical and health sciences, 0302 clinical medicine, Immune system, Hygiene hypothesis, medicine, Microbiome, Asthma, lcsh:R5-920, business.industry, biochemical phenomena, metabolism, and nutrition, medicine.disease, 030104 developmental biology, Infectious Diseases, 030220 oncology & carcinogenesis, Immunology, bacteria, lcsh:Medicine (General), business
Abstract

Microbiome and the immune system are constantly shaping each other, in a mutual aim to thrive, defining the unstable equilibrium of the healthy individual. Microbiome is growingly involved in dysimmune conditions such as allergy, asthma, autoimmunity, and primary or acquired immune deficiencies. The current epidemics of allergic diseases and asthma has long been linked to the microbial environment through the hygiene hypothesis. Progress in the understanding of the microbiome-immune system crosstalk has unraveled a tight connection between microbial communities and the development of allergic diseases and asthma. Disruption of the microbiome affects the immune response of the host and paves the way for disease pathogenesis. Conversely, disease and therapeutic interventions affect microbial communities. We aimed at providing the reader with a view of the state-of-the art of microbiome – immune system crosstalk, with special focus on the loopholes giving potential grip to the pathogenesis of microbiome-related dysimmunity. Keywords: Microbiome, Mycobiome, Immune system, Dysbiosis, Allergy, Asthma, Atopy, Atopic dermatitis, Dendritic cell, Macrophage, Mast cell, Chronic rhinosinusitis

Published
2018

19. Overview of STING-Associated Vasculopathy with Onset in Infancy (SAVI) Among 21 Patients

Author

Olivier Brocq, Pere Soler-Palacín, Jérôme Sirvente, Brigitte Bader-Meunier, Rawane Dagher, Naoki Kitabayashi, Laureline Berteloot, Sophie Willcocks, Nadia Jeremiah, Laura Barnabei, Stefano Volpi, Eline Van Aerde, Sylvain Breton, Damien Chan, Alice Hadchouel, Vincent Bondet, Eric Jeziorski, Despina Moshous, Antonella Insalaco, Jean-Christophe Dubus, Thierry Jo Molina, Isabelle Melki, Nathalie Stremler-Le Bel, Stéphane Blanche, Marie-Anne Morren, Alain Fischer, Mireia Lopez-Corbeto, Gillian I. Rice, Marie-Louise Frémond, Carine Wouters, Yanick J. Crow, Séverine Feuillet-Soummer, Jacques G. Rivière, Françoise Mazingue, Violaine Bresson, Bénédicte Neven, Alexandre Belot, Mathieu Fusaro, Guillaume Thouvenin, Darragh Duffy, Frédéric Rieux-Laucat, Caroline Thumerelle, Luis Seabra, Marco Gattorno, Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Service d'immuno-hématologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Pneumologie Allergologie [CHU Necker], Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), Service de radiologie pédiatrique [CHU Necker], AP-HP Hôpital universitaire Robert-Debré [Paris], Urgences pédiatriques [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Immunité et cancer (U932), Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Curie [Paris], Hospices Civils de Lyon (HCL), Réponse immunitaire innée dans les maladies infectieuses et auto-immunes – Innate immunity in infectious and autoimmune diseases, Centre International de Recherche en Infectiologie - UMR (CIRI), Institut National de la Santé et de la Recherche Médicale (INSERM)-École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Institut Pasteur [Paris], Immunologie Translationnelle - Translational Immunology lab, Hôpital Princesse Grace [Monaco], Women’s and Children’s Hospital [Adelaide], CHU Notre Dame des Secours [Jbeil], Service de pédiatrie spécialisée et médecine infantile (neurologie, pneumologie, maladies héréditaires du métabolisme) [Hôpital de la Timone - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Centre chirurgical Marie Lannelongue, CHU Necker - Enfants Malades [AP-HP], IRCCS Istituto Giannina Gaslini [Genoa, Italy], IRCCS Ospedale Pediatrico Bambino Gesù [Roma], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Pathogénèse et contrôle des infections chroniques (PCCI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre Hospitalier Universitaire de Montpellier (CHU Montpellier ), Vall d'Hebron University Hospital [Barcelona], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), University Hospitals Leuven [Leuven], Lausanne University Hospital, Manchester Academic Health Science Centre (MAHSC), University of Manchester [Manchester], Vall d’Hebron Research Institute (VHIR), Universitat Autònoma de Barcelona (UAB), Service de Pneumologie pédiatrique [CHU Trousseau], CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Queensland Children's Hospital, Partenaires INRAE, Catholic University of Leuven - Katholieke Universiteit Leuven (KU Leuven), Service de pathologie [CHU Necker], Collège de France (CdF (institution)), MRC Institute of Genetics and Molecular Medicine [Edinburgh] (IGMM), University of Edinburgh-Medical Research Council, M.-L. Frémond received a grant from the Institut National de la Santé et de la Recherche Médicale (reference: 000427993). Y. J. Crow acknowledges the European Research Council (GA309449 and 786142-E-T1IFNs) and a state subsidy managed by the National Research Agency (France) under the 'Investments for the Future' program bearing the reference ANR-10-IAHU-01. Y. J. Crow and D. Duffy acknowledge the National Research Agency (France) (grant CE17001002)., The authors wish to thank the patients and their families for their cooperation in this study. The authors are grateful to Elvira Duchesne (NP) and Samira Plassart (PhD) for their very helpful technical assistance. They thank Thomas Blauwblomme (MD, PhD, Neurosurgery Unit, Necker Hospital, Paris, France) for providing CSF from children with idiopathic hydrocephalus., ANR-10-IAHU-0001,Imagine,Institut Hospitalo-Universitaire Imagine(2010), European Project: 309449,EC:FP7:ERC,ERC-2012-StG_20111109,T1-IFN(2013), 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)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Institut Curie [Paris]-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre International de Recherche en Infectiologie (CIRI), École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut Pasteur [Paris] (IP), Centre Chirurgical Marie Lannelongue (CCML), Pathogenesis and Control of Chronic and Emerging Infections (PCCEI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Université des Antilles (UA)-Etablissement français du don du sang [Montpellier], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)

Subjects
Adult, medicine.medical_specialty, Adolescent, Late onset, Interstitial lung disease, Vasculopathy, Systemic inflammation, Gastroenterology, Nephropathy, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Lymphopenia, medicine, Immunology and Allergy, Humans, 030212 general & internal medicine, Vascular Diseases, [SDV.IMM.ALL]Life Sciences [q-bio]/Immunology/Allergology, Child, Type I interferonopathy, Immunodeficiency, Inflammation, STING1, JAK inhibitors, business.industry, Infant, Membrane Proteins, medicine.disease, 3. Good health, Polyarthritis, 030228 respiratory system, Failure to thrive, Mutation, Aicardi–Goutières syndrome, medicine.symptom, Stimulator of interferon genes, business, Lung Diseases, Interstitial
Abstract

BACKGROUND: Gain-of-function mutations in STING1 underlie a type I interferonopathy termed SAVI (STING-associated vasculopathy with onset in infancy). This severe disease is variably characterized by early-onset systemic inflammation, skin vasculopathy, and interstitial lung disease (ILD).OBJECTIVE: To describe a cohort of patients with SAVI.METHODS: Assessment of clinical, radiological and immunological data from 21 patients (17 families) was carried out.RESULTS: Patients carried heterozygous substitutions in STING1 previously described in SAVI, mainly the p.V155M. Most were symptomatic from infancy, but late onset in adulthood occurred in 1 patient. Systemic inflammation, skin vasculopathy, and ILD were observed in 19, 18, and 21 patients, respectively. Extensive tissue loss occurred in 4 patients. Severity of ILD was highly variable with insidious progression up to end-stage respiratory failure reached at teenage in 6 patients. Lung imaging revealed early fibrotic lesions. Failure to thrive was almost constant, with severe growth failure seen in 4 patients. Seven patients presented polyarthritis, and the phenotype in 1 infant mimicked a combined immunodeficiency. Extended features reminiscent of other interferonopathies were also found, including intracranial calcification, glaucoma and glomerular nephropathy. Increased expression of interferon-stimulated genes and interferon α protein was constant. Autoantibodies were frequently found, in particular rheumatoid factor. Most patients presented with a T-cell defect, with low counts of memory CD8+ cells and impaired T-cell proliferation in response to antigens. Long-term follow-up described in 8 children confirmed the clinical benefit of ruxolitinib in SAVI where the treatment was started early in the disease course, underlying the need for early diagnosis. Tolerance was reasonably good.CONCLUSION: The largest worldwide cohort of SAVI patients yet described, illustrates the core features of the disease and extends the clinical and immunological phenotype to include overlap with other monogenic interferonopathies.

Published
2021

20. Bonnes pratiques de l’aérosolthérapie par nébulisation en 2020

Author

Laurent Vecellio, Jean-Christophe Dubus, Gregory Reychler, J.P. Chaumuzeau, Gat, M.H. Becquemin, Microbes évolution phylogénie et infections (MEPHI), Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU), Pédiatrie Multidisciplinaire Timone, CHU Timone?Enfants, Marseille, France, parent, Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Tours, Service Pneumologie Cliniques Universitaires Saint Luc, Université Catholique de Louvain = Catholic University of Louvain (UCL), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, and UCL - (SLuc) Service de pneumologie

Subjects
Pulmonary and Respiratory Medicine, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Recommandations, Recommendations, Aerosol therapy, Nebulization, 03 medical and health sciences, 0302 clinical medicine, Drug Delivery Systems, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Administration, Inhalation, Medicine, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, 030212 general & internal medicine, Medical prescription, Good practice, ComputingMilieux_MISCELLANEOUS, Aerosols, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Nébulisation, business.industry, Nebulizers and Vaporizers, Nebulizer, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, 3. Good health, 030228 respiratory system, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Nébuliseur, Medical emergency, business
Abstract

La nébulisation est un mode d’administration des traitements inhalés dont la prescription et la mise en place restent délicates. Ces bonnes pratiques, réalisées par le GAT (groupe aérosolthérapie de la SPLF), étaient nécessaires, les dernières datant de 2007. Plus encore qu’une actualisation des données sur le sujet, ces recommandations d’experts se veulent une vraie aide pour le prescripteur. [Good practice for aerosol therapy by nebulization in 2020] Nebulization is a drug delivery mode whose prescription and application remain uncertain. A guide to good practice has been proposed by the work group on aerosol therapy of the French Society for Respiratory Diseases, so-called GAT. The previous recommendations date from 2007. In addition to an update of data on nebulization, these expert recommendations aim to be of real help to the prescriber.

Published
2021

21. Key role of pediatricians and disease for influenza vaccination in children with high-risk chronic diseases

Author

Aurélie Morand, Pierre Alauzet, Julie Mazenq, Emmanuelle Bosdure, Jean Gaudart, Jean-Christophe Dubus, Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Sciences Economiques et Sociales de la Santé & Traitement de l'Information Médicale (SESSTIM - U1252 INSERM - Aix Marseille Univ - UMR 259 IRD), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Dupuis, Christine

Subjects
Pediatrics, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Disease, Cystic fibrosis, Chronic disease, 03 medical and health sciences, Influenza A Virus, H1N1 Subtype, 0302 clinical medicine, Vaccination status, 030225 pediatrics, Influenza, Human, medicine, Humans, In patient, Pediatricians, Prospective Studies, 030212 general & internal medicine, Child, Children, Vaccination coverage, business.industry, Vaccination, H1N1 influenza, virus diseases, medicine.disease, Influenza, 3. Good health, [SDV] Life Sciences [q-bio], Europe, Influenza Vaccines, Pediatrics, Perinatology and Child Health, business
Abstract

International audience; Annual influenza vaccination is recommended for children with chronic diseases. Studies on influenza vaccines, following controversies related to the 2009 H1N1 influenza, are scarce in Europe. Our aim was to evaluate the influenza vaccination coverage in such children in a French tertiary hospital. Secondary objectives were the evaluation of the influenza vaccination coverage trend and the identification of factors influencing the vaccination status. A prospective and descriptive study by questionnaire was performed at the end of 2017 in 402 French hospital outpatients with various underlying chronic diseases eligible to the influenza vaccination. The 2016-2017 vaccination coverage was 46.5%. Figures of 75% or greater were only found in patients with cystic fibrosis and sickle cell disease. CART analysis identified vaccination in the previous year, medical recommendation for vaccination, and maternal influenza vaccination as a child's decisive factors for being vaccinated.Conclusion: Influenza vaccination coverage remains insufficient in children receiving hospital follow-up for chronic diseases. Its implementation clearly depends on pediatricians' recommendation to vaccinate and on the type of chronic disease. What is Known: • Despite health policy recommendations, the rate of annual influenza vaccination in children with chronic diseases is low What is New: • Influenza vaccination coverage depends on the type of chronic disease and on the pediatricians' counseling to vaccine.

Published
2021

22. Real-life study of the role of high-flow nasal cannula for bronchiolitis in children younger than 3 months hospitalised in general pediatric departments

Author

J.-M. Bartoli, N. Stremler-Le Bel, Elisabeth Jouve, S. Tardieu, M. Dequin, K. Retornaz, Emmanuelle Bosdure, Mélanie Panciatici, Jean-Christophe Dubus, Emilie Sauvaget, Candice Fabre, Service de médecine infantile et pneumologie pédiatrique, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), and Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)

Subjects
Male, Pediatrics, medicine.medical_specialty, Hospital Departments, medicine.disease_cause, Medical care, Severity of Illness Index, Hypoxemia, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, 030225 pediatrics, medicine, Cannula, Humans, Clinical severity, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, ComputingMilieux_MISCELLANEOUS, Retrospective Studies, Pediatric intensive care unit, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, business.industry, Infant, Newborn, Oxygen Inhalation Therapy, Infant, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, 3. Good health, Hospitalization, Treatment Outcome, Bronchiolitis, Pediatrics, Perinatology and Child Health, Acute Disease, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Female, medicine.symptom, High flow, business, Life study, Nasal cannula
Abstract

We aimed to describe the real-life role of high-flow nasal cannula (HFNC) for bronchiolitis in infants under 3 months of age admitted to three general pediatric departments during the 2017-2018 epidemic period. We retrospectively assessed the clinical severity (Wang score) for every 24-h period of treatment (H0-H24 and H24-H48) according to the initiated medical care (HFNC, oxygen via nasal cannula, or supportive treatments only), the child's discomfort (EDIN score), and transfer to the pediatric intensive care unit (PICU). A total of 138 infants were included: 47±53 days old, 4661±851.9 g, 70 boys (50.7%), 58 with hypoxemia (42%), Wang score of 6.67±2.58, 110 (79.7%) staying for 48 consecutive hours in the same ward. During the H0-H24 period, only patients treated with HFNC had a statistically significant decrease in the severity score (n=21/110; -2 points, P=0.002) and an improvement in the discomfort score (n=15/63; -3.8 points, P0.0001). There was no difference between groups during the H24-H48 period. The rate of admission to the PICU was 2.9% for patients treated for at least 24 h with HFNC (n=34/138, 44% with oxygen) versus 16.3% for the others (P=0.033). Early use of HFNC improves both clinical status and discomfort in infants younger than 3 months admitted for moderately severe bronchiolitis, whatever their oxygen status.

Published
2021

23. Clinical characteristics of paediatric COVID‐19 patients followed for up to 13 months

Author

Philippe Minodier, Brigitte Chabrol, Léa Luciani, Alexandre Fabre, Mohammed Boucekine, Philippe Colson, Tatiana Matteudi, Aurélie Morand, Emmanuelle Bosdure, Jean-Christophe Dubus, Service de pédiatrie spécialisée et médecine infantile (neurologie, pneumologie, maladies héréditaires du métabolisme) [Hôpital de la Timone - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Unité des Virus Emergents (UVE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille), Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Nord [CHU - APHM], Centre d'études et de recherche sur les services de santé et la qualité de vie (CEReSS), Aix Marseille Université (AMU), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), and COMBE, Isabelle

Subjects
2019-20 coronavirus outbreak, Pediatrics, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), MEDLINE, Chest pain, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, 030225 pediatrics, Pandemic, medicine, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, 030212 general & internal medicine, Child, ComputingMilieux_MISCELLANEOUS, Paediatric patients, [SDV.MP.VIR] Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.MHEP.ME] Life Sciences [q-bio]/Human health and pathology/Emerging diseases, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, SARS-CoV-2, business.industry, Brief Report, COVID-19, Muscle weakness, General Medicine, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, 3. Good health, Pediatrics, Perinatology and Child Health, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.MHEP.MI] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Brief Reports, [SDV.MP.BAC] Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, medicine.symptom, business, [SDV.MP.PAR] Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology
Abstract

Children are less affected by COVID-19 than adults1 . But, a small proportion of children develop paediatric inflammatory multisystem syndrome2 , with potentially lethal consequences. Studies have also described persistent long COVID symptoms that last more than four weeks from the onset of illness, including fatigue, muscle weakness, dyspnoea, chest pain, cough and anosmia2-5 . However, information on paediatric long COVID remains scarce and studies often describe hospitalised populations2,3 . We report the first 201 paediatric patients under 16 years old that were tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in Marseille public hospitals during the first wave of the COVID-19 pandemic in France. We described the acute symptoms and long-term consequences of COVID-19 in the children, who were mostly outpatients.

Published
2021

24. Nebulization: A potential source of SARS-CoV-2 transmission

Author

Jean-Christophe Dubus, Laurent Vecellio, Gregory Reychler, Service Pneumologie Cliniques Universitaires Saint Luc, Université Catholique de Louvain = Catholic University of Louvain (UCL), Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Médecine Infantile [Hôpital Nord - APMH], Hôpital Nord [CHU - APHM], Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), COMBE, Isabelle, and Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)

Subjects
Azoles, Antifungal drugs, law.invention, Nebulization, 0302 clinical medicine, Risk Factors, law, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Medicine, Potential source, 030212 general & internal medicine, ComputingMilieux_MISCELLANEOUS, [SDV.MP.VIR] Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.MHEP.ME] Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Cross Infection, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Transmission (mechanics), [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Mucorales, [SDV.MHEP.MI] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, [SDV.MP.PAR] Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Pulmonary and Respiratory Medicine, Respiratory Therapy, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Polyenes, Article, 03 medical and health sciences, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Administration, Inhalation, Disease Transmission, Infectious, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Pandemics, Aerosols, Prophylaxis, SARS-CoV-2, business.industry, Nebulizers and Vaporizers, Oxygen Inhalation Therapy, COVID-19, Virology, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, 030228 respiratory system, Equipment Contamination, [SDV.MP.BAC] Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, business
Abstract

International audience

Published
2020

25. Exploration des immunoglobulines E bronchiques : vers un nouveau monde à explorer ?

Author

Joana Vitte, Moïse Michel, Jean-Christophe Dubus, Benjamin Coiffard, Youssouf Sereme, J. Bermudez, Martine Reynaud-Gaubert, C. Gonzalez, S. Pinchemel, and W. Sahli

Subjects
Immunology and Allergy
Abstract

Introduction La contribution de la reponse IgE aux exacerbations et a la deterioration a long terme de la fonction respiratoire chez les patients atteints de mucoviscidose est acceptee, mais souvent difficile a evaluer. Nous faisons l’hypothese que les nouveaux outils de type biopuce a allergenes peuvent contribuer a mieux caracteriser les reponses IgE pathologiques au niveau pulmonaire. Methodes Nous avons mis au point une methode de detection et de caracterisation des IgE specifiques locales (aspiration bronchique ou crachat) de patients atteints de mucoviscidose. Notre methode consiste en une fluidification puis une lyophilisation des expectorations, avant la realisation d’un biopuce a allergenes (ALEX®, Vienne, Autriche). Resultats Apres la mise au point et optimisation de la methode, nous avons etudie les expectorations de 22 patients mucoviscidosiques, dont 5 transplantes pulmonaires. L’âge etait comparable dans les deux groupes, avec une mediane de 29 ans (extremes 17–72 ans) chez les patients non transplantes et 39 ans (18/53) chez ceux transplantes. Les patients non transplantes presentaient une reponse IgE significativement plus elevee vis-a-vis de plusieurs allergenes environnementaux, notamment les phaneres (p = 0.0004) et les acariens (p = 0,03). La reponse IgE aux moisissures environnementales ne differait pas significativement entre les deux groupes de patients et n’etait pas correlee a l’eventuelle colonisation fongique, cependant les IgE locales vis-a-vis des allergenes fongiques, notamment Cladosporium herbarum, etaient correlees a la presence d’une symptomatologie aigue en cours. Sur le plan methodologique, la richesse en IgE des aspirations bronchiques etait significativement inferieure a celle des crachats. Discussion Nous apportons la preuve de concept d’un outil non invasif pour l’exploration large de l’immunite humorale locale chez des patients mucoviscidosiques. Conclusion L’exploration des prelevements respiratoires par biopuce pourrait apporter des elements importants pour identifier les causes d’exacerbations chez ces patients et contribuer a une prise en charge rapide et adequate.

Published
2021

26. Non accidental repeated lithium poisoning in a child: the role of hair analysis

Author

A.L. Pelissier, K. Retornaz, Jean-Christophe Dubus, C. Pouchoux, E. Bosdure, C. Di Meglio, Jean-Michel Gaulier, Hôpital de la Timone [CHU - APHM] (TIMONE), Impact de l'environnement chimique sur la santé humaine - ULR 4483 (IMPECS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Lille, Institut Pasteur de Lille, Université de Lille, Hôpital de la Timone [CHU - APHM] [TIMONE], and IMPact de l'Environnement Chimique sur la Santé humaine (IMPECS) - ULR 4483

Subjects
Chronic exposure, Child abuse, Pediatrics, medicine.medical_specialty, Lithium (medication), [SDV]Life Sciences [q-bio], Lithium, Accidental intoxication, Lithium poisoning, Toxicological analysis of hair, Seizures, medicine, Humans, Child Abuse, business.industry, Poisoning, Hair analysis, Infant, Child(ren), Non accidental intoxication, Seizure, Infants, Repeated poisoning, Hair Analysis, Accidental, Pediatrics, Perinatology and Child Health, Female, business, medicine.drug, Hair
Abstract

International audience; Non accidental intoxication due to child abuse is rare and its frequency is likely underestimated because it is difficult to diagnose. Here, we report a case of voluntary repeated exposure to lithium in an infant, for whom the clinical manifestations were convulsions. Toxicological analysis was very helpful for documenting lithium exposure during the assumed period of time. Interpreting the results of hair analysis, a simple and minimally invasive examination, is tricky at this age, but it can facilitate the differentiation of acute versus chronic exposure. Although infrequent and underestimated, lithium should be considered as a cause of intoxication in a previously healthy child with acute seizure.

Published
2020

27. Description of two anaphylaxis phenotypes in children: The utility of a clustering analysis approach

Author

Guillaume Pouessel, Jérémy Corriger, Stéphanie Lejeune, Guilhem Noel, Antoine Deschildre, Jean Christophe Dubus, Hélène Behal, and Etienne Beaudouin

Subjects
medicine.medical_specialty, Epinephrine, business.industry, Immunology, medicine.disease, Phenotype, Food allergy, Epidemiology, medicine, Immunology and Allergy, Cluster Analysis, Humans, business, Cluster analysis, Child, Anaphylaxis, Food Hypersensitivity
Published
2020

29. Impact of COVID-19 on Pediatric Asthma: Practice Adjustments and Disease Burden

Author

James E. Gern, Antoine Deschildre, Zhimin Chen, Cindy De Lira, Paraskevi Xepapadaki, Leonard B. Bacharier, Rola Abou Taam, Francine M. Ducharme, Susanne Lau, Yunuen R. Huerta Villalobos, Alessandro Fiocchi, Alan Kaplan, Gunilla Hedlin, Berenice Velasco Benhumea, Peter N. Le Souëf, Laurence Weiss, Jean Christophe Dubus, Monica Medley, Zeinab A El-Sayed, Adnan Custovic, Jose A. Castro-Rodriguez, Major Najaraju, Wanda Phipatanakul, Matteo Bonini, Omer Kalayci, Heather J. Zar, Leyla Namazova-Baranova, Clare S. Murray, Cyril Schweitzer, Robert F. Lemanske, Hugo Azuara, Timothy J. Craig, Graham Roberts, Stanley J. Szefler, Jacques Brouard, Ioana Agache, Mário Morais-Almeida, Elham Hossny, Antonio Nieto Garcia, Pascal Roux, Jon R Konradsen, Tuomas Jartti, Teija Dunder, Nikolaos G. Papadopoulos, Paulo Márcio Pitrez, Wojciech Feleszko, Karthik Nagaraju, Mika J. Mäkelä, Luis Garcia-Marcos, K Efendieva, Rosalaura Villarreal, Piotr Kuna, Osman Yusuf, Alexander G. Mathioudakis, Andrzej Emeryk, Pierrick Cros, Julia Levina, Cesar Fireth Pozo Beltrán, Marja Ruotsalainen, Arunas Valiulis, René Maximiliano Gómez, Nidia Karen, Daniela Rivero Yeverino, Anne Goh, Petr Pohunek, Eckard Hamelmann, Carole Egron, Anna Zawadzka-Krajewska, Gary Wong, HUS Inflammation Center, Department of Dermatology, Allergology and Venereology, Clinicum, and Helsinki University Hospital Area

Subjects
Pediatrics, Time Factors, Global Health, Severity of Illness Index, 0302 clinical medicine, Interquartile range, immune system diseases, Pandemic, Global health, Immunology and Allergy, Medicine, 030212 general & internal medicine, Child, Children, COVID-19, coronavirus disease 2019, education.field_of_study, Incidence (epidemiology), Telemedicine, 3. Good health, Virus, Adherence, Asthma, COVID-19, Children, Control, SARS-CoV2, Virus, Coronavirus Infections, medicine.medical_specialty, Pediatric Asthma in Real Life Collaborators, Population, Pneumonia, Viral, Criança, SARS-CoV-2, severe acute respiratory syndrome coronavirus 2, Article, Medication Adherence, 03 medical and health sciences, Appointments and Schedules, Betacoronavirus, Severity of illness, Control, Humans, education, Pandemics, Disease burden, IQR, interquartile range, Asma, Asthma, business.industry, SARS-CoV-2, COVID-19, medicine.disease, respiratory tract diseases, 030228 respiratory system, Adherence, 3121 General medicine, internal medicine and other clinical medicine, SARS-CoV2, business
Abstract

BACKGROUND: It is unclear whether asthma may affect susceptibility or severity of coronavirus disease 2019 (COVID-19) in children and how pediatric asthma services worldwide have responded to the pandemic. OBJECTIVE: To describe the impact of the COVID-19 pandemic on pediatric asthma services and on disease burden in their patients. METHODS: An online survey was sent to members of the Pediatric Asthma in Real Life think tank and the World Allergy Organization Pediatric Asthma Committee. It included questions on service provision, disease burden, and the clinical course of confirmed cases of COVID-19 infection among children with asthma. RESULTS: Ninety-one respondents, caring for an estimated population of more than 133,000 children with asthma, completed the survey. COVID-19 significantly impacted pediatric asthma services: 39% ceased physical appointments, 47% stopped accepting new patients, and 75% limited patients' visits. Consultations were almost halved to a median of 20 (interquartile range, 10-25) patients per week. Virtual clinics and helplines were launched in most centers. Better than expected disease control was reported in 20% (10%-40%) of patients, whereas control was negatively affected in only 10% (7.5%-12.5%). Adherence also appeared to increase. Only 15 confirmed cases of COVID-19 were reported among the population; the estimated incidence is not apparently different from the reports of general pediatric cohorts. CONCLUSIONS: Children with asthma do not appear to be disproportionately affected by COVID-19. Outcomes may even have improved, possibly through increased adherence and/or reduced exposures. Clinical services have rapidly responded to the pandemic by limiting and replacing physical appointments with virtual encounters. (C) 2020 American Academy of Allergy, Asthma & Immunology.

Published
2020

30. Severe adenovirus pneumonia with hemophagocytic syndrome and respiratory failure

Author

M. Baravalle-Einaudi, N. Stremler-Le Bel, C. La Fay, Jean-Christophe Dubus, J. Mazenq, E. Bosdure, Service de pédiatrie spécialisée et médecine infantile (neurologie, pneumologie, maladies héréditaires du métabolisme) [Hôpital de la Timone - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), and Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille)

Subjects
Serotype, Pediatrics, medicine.medical_specialty, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Pneumonia, Viral, Bronchiolitis obliterans, Antiviral Agents, Severity of Illness Index, Lymphohistiocytosis, Hemophagocytic, Adenovirus Infections, Human, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, 030225 pediatrics, medicine, Humans, Bronchiolitis Obliterans, Chronic respiratory insufficiency, Mechanical ventilation, Bronchiectasis, business.industry, Infant, medicine.disease, Combined Modality Therapy, Respiration, Artificial, 3. Good health, Pneumonia, chemistry, Respiratory failure, Pediatrics, Perinatology and Child Health, Chronic Disease, business, Respiratory Insufficiency, Cidofovir
Abstract

We report the case of an 18-month-old infant with severe serotype 3 adenovirus pneumonia, exceptionally associated with hemophagocytic syndrome. Treatment included cidofovir and mechanical ventilation for 13 days. The child developed chronic respiratory insufficiency due to bronchiectasis and bronchiolitis obliterans. (C) 2020 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.

Published
2020

31. Severe box jellyfish envenomation in 2 children

Author

Audrey Anziani Vente, Charlotte Jaloux, Jean-Jacques Morand, Stéphane LE Bel, Emmanuelle Bosdure, Jean-Christophe Dubus, and Aurélie Morand

Subjects
Infectious Diseases, Antivenins, Cubozoa, Public Health, Environmental and Occupational Health, Animals, Humans, Bites and Stings, Child
Published
2022

32. Use of epinephrine in emergency department depends on anaphylaxis severity in children

Author

Philippe Minodier, Marie-Sophie Lê, Jean-Christophe Dubus, Joana Vitte, Ania Carsin, Aurélie Boutin, Gilles Viudes, Guilhem Noel, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Assistance Publique - Hôpitaux de Marseille (APHM), Service de pédiatrie spécialisée et médecine infantile (neurologie, pneumologie, maladies héréditaires du métabolisme) [Hôpital de la Timone - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Médecine Infantile [Hôpital Nord - APMH], Hôpital Nord [CHU - APHM], and Assistance Publique-Hôpitaux de Marseille (AP-HM)

Subjects
Male, Emergency Medical Services, medicine.medical_specialty, Allergy, Adolescent, Epinephrine, Referral, Injections, Intramuscular, Severity of Illness Index, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Food allergy, 030225 pediatrics, Internal medicine, medicine, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, 030212 general & internal medicine, Practice Patterns, Physicians', Medical prescription, Child, Anaphylaxis, ComputingMilieux_MISCELLANEOUS, Retrospective Studies, Asthma, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, business.industry, Infant, Emergency department, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, humanities, 3. Good health, Hospitalization, Child, Preschool, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Pediatrics, Perinatology and Child Health, Female, France, Emergency Service, Hospital, business, medicine.drug
Abstract

Despite multiple recommendations, intramuscular epinephrine is poorly prescribed in emergency department receiving pediatric anaphylaxis. To evaluate the role of severity symptoms on this use, we included all admissions for a diagnosis linked to possible allergy in the two pediatric emergency departments of our institution between January 2010 and December 2015. Selection and analysis were restricted to children under 18 years fulfilling Sampson's criteria for anaphylaxis. We retrospectively ranked these admissions with the Ring and Messmer anaphylaxis severity score and compared the use of epinephrine according to this classification. Among 422,483 admissions, 204 (0.05%) fulfilled the anaphylaxis criteria (170 (83.3%) grade II anaphylaxis, and 34 (16.7%) grade III; mean age 7.9 years). Previous allergy, anaphylaxis, and asthma were found in respectively 60.8%, 36.8%, and 35.1%. Food allergy was the main suspected causal trigger. Epinephrine was used in 32.7% (n = 65/199), before admission (11.4% (n = 23/201)) or in the emergency department (22.2% (n = 45/202)). Epinephrine was more frequently prescribed in grade III than in grade II anaphylaxis (84.8% vs 22.3%, p 0.001; OR = 19.05 [7.05-54.10]). Upon discharge, epinephrine auto-injectors prescription and allergy referral were rare (31.7% and 44.2%).Conclusion: Pediatricians intuitively adapt their epinephrine use to the severity of the anaphylaxis and contribute to epinephrine underuse in pediatric anaphylaxis. What is known: • Intramuscular epinephrine is the recommended treatment for pediatric anaphylaxis. However, most of the European and North-American studies show a low prescription rate of epinephrine in both prehospital and pediatric emergency department management. • Reasons for such a low prescription rate are unknown. What is new: • This study confirms that intramuscular epinephrine is poorly prescribed in pediatric anaphylaxis (about one case among 10 before admission and one among 5 in pediatric emergency departments). • Despite recommendations, pediatricians intuitively adapt their prescription to the clinical severity of anaphylaxis, with a fourfold increase prescription in grade III compared to grade II anaphylaxis. This medical behavior ascertainment may be in part explained by the delay between the ED admission/management and the anaphylactic episode onset.

Published
2018

33. Development of an ex vivo respiratory pediatric model of bronchopulmonary dysplasia for aerosol deposition studies

Author

Anthony Clotagatide, Sophie Perinel, Yoann Montigaud, Jean-Christophe Dubus, Clémence Goy, Nathalie Prevot, Jérémie Pourchez, Lara Leclerc, Marie Suau, Santé Ingénierie Biologie Saint-Etienne (SAINBIOSE), Centre Ingénierie et Santé (CIS-ENSMSE), École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes (URMITE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48, Institut des sciences biologiques (INSB-CNRS)-Institut des sciences biologiques (INSB-CNRS)-Centre National de la Recherche Scientifique (CNRS), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT), Service d'Histologie Embryologie, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Laboratoire Interdisciplinaire d'Etude des Nanoparticules Aérosolisées (LINA-ENSMSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-CIS, Service de Médecine Nucléaire [Saint-Etienne], INSERM U1059, SAINBIOSE - Santé, Ingénierie, Biologie, Saint-Etienne (SAINBIOSE-ENSMSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), INSB-INSB-Centre National de la Recherche Scientifique (CNRS), CHRU Saint-Etienne, and CHU Saint-Etienne-Hôpital Bellevue

Subjects
0301 basic medicine, Thorax, Models, Anatomic, lcsh:Medicine, Article, 03 medical and health sciences, 0302 clinical medicine, In vivo, medicine, Tidal Volume, Humans, Respiratory system, lcsh:Science, Lung, ComputingMilieux_MISCELLANEOUS, Bronchopulmonary Dysplasia, Aerosols, Multidisciplinary, Inhalation, business.industry, Respiration, lcsh:R, Infant, Newborn, respiratory system, medicine.disease, 030104 developmental biology, medicine.anatomical_structure, Bronchopulmonary dysplasia, Printing, Three-Dimensional, Breathing, lcsh:Q, [SDV.IB]Life Sciences [q-bio]/Bioengineering, business, Nuclear medicine, Pulmonary Ventilation, 030217 neurology & neurosurgery, Ex vivo
Abstract

Ethical restrictions are limitations of in vivo inhalation studies, on humans and animal models. Thus, in vitro or ex vivo anatomical models offer an interesting alternative if limitations are clearly identified and if extrapolation to human is made with caution. This work aimed to develop an ex vivo infant-like respiratory model of bronchopulmonary dysplasia easy to use, reliable and relevant compared to in vivo infant data. This model is composed of a 3D-printed head connected to a sealed enclosure containing a leporine thorax. Physiological data and pleural-mimicking depressions were measured for chosen respiratory rates. Homogeneity of ventilation was assessed by 81mkrypton scintigraphies. Regional radioaerosol deposition was quantified with 99mtechnetium-diethylene triamine pentaacetic acid after jet nebulization. Tidal volumes values are ranged from 33.16 ± 7.37 to 37.44 ± 7.43 mL and compliance values from 1.78 ± 0.65 to 1.85 ± 0.99 mL/cmH2O. Ventilation scintigraphies showed a homogenous ventilation with asymmetric repartition: 56.94% ± 9.4% in right lung and 42.83% ± 9.36 in left lung. Regional aerosol deposition in lungs exerted 2.60% ± 2.24% of initial load of radioactivity. To conclude the anatomical model satisfactorily mimic a 3-months old BPD-suffering bronchopulmonary dysplasia and can be an interesting tool for aerosol regional deposition studies.

Published
2019

34. What is the impact of outdoor pollution on children's asthma?

Author

Véronique Houdouin, Jean-Christophe Dubus, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Assistance Publique - Hôpitaux de Marseille (APHM), and Assistance Publique-Hôpitaux de Marseille (AP-HM)

Subjects
Pollution, medicine.medical_specialty, media_common.quotation_subject, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, 030225 pediatrics, Bronchial inflammation, Environmental health, Air Pollution, Epidemiology, Medicine, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Child, media_common, Asthma, Pollutant, Childhood asthma, Inhalation Exposure, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Asthma exacerbations, business.industry, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, respiratory tract diseases, 3. Good health, 13. Climate action, Pediatrics, Perinatology and Child Health, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, business, Cohort study
Abstract

Outdoor pollution is a complex mix of more than 200 air contaminants. Among these pollutants, ozone, nitrogen dioxide and fine particles may generate bronchial inflammation and hyperreactivity. The hypothesis that pollution contributes to the development of asthma in children is based on epidemiological, clinical and experimental data. Many risk factors during the in utero and postnatal period have been identified in the aetiology of childhood asthma. During pregnancy, outdoor pollution was identified as a causal factor of respiratory disease in neonatal cohort studies. Several epidemiological studies also demonstrate that outdoor pollution is a trigger of asthma exacerbations. This review aims to highlight the current knowledge on outdoor pollution and asthma.

Published
2019

35. La kinésithérapie respiratoire dans la bronchiolite virale aiguë du nourrisson. Arguments pour/contre

Author

G. Postiaux, P. Maffei, Jean-Christophe Dubus, and J.-C. Villiot-Danger

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Acute viral bronchiolitis, Auscultation, medicine.disease, 3. Good health, Medium term, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, Bronchiolitis, 030225 pediatrics, Ambulatory, medicine, Expiration, Respiratory physiotherapy, business, Intensive care medicine, Bronchial obstruction
Abstract

This article reports an exchange of unbiased arguments between Mr Guy Postiaux speaking in favour of respiratory physiotherapy in acute viral bronchiolitis in the newborn and Prof. Jean-Christoph Dubus arguing against. A review of the literature suggests that traditional methods of physiotherapy should be abandoned because they are not validated and because they have harmful side effects. The latest Cochrane revue (2016) suggests the use of slow expiration techniques that have some validated elements and cause no harmful side effects. Large multicentre studies should be undertaken to confirm or refute the results of the five studies in the Cochrane review. Their analysis would allow extraction of objective evidence for the efficacy of slow expiration techniques on the relief of bronchopulmonary obstruction and the reduction of the degree of severity in the short and medium term. Studies of the effect of ambulatory respiratory physiotherapy for bronchiolitis of a moderate degree not requiring hospitalisation are not available. An evaluation is needed which is based on the pathophysiology of multifactorial bronchial obstruction and on the physical signs, of which auscultation is the cornerstone.

Published
2018

36. Chondrite à Pseudomonas aeruginosa après piercing chez l’adolescent : à propos de deux cas

Author

Jean-Christophe Dubus, A.-G. Lelong, S. Mallet, Ania Carsin, C. Le Treut, and E. Bosdure

Subjects
030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Pediatrics, Perinatology and Child Health, 030223 otorhinolaryngology
Abstract

Resume Le piercing se pratique sur des sites corporels divers parmi lesquels les oreilles et notamment le cartilage ont un franc succes. Nous rapportons deux cas d’adolescentes ayant presente une chondrite a Pseudomonas aeruginosa liee a ces piercings.

Published
2017

37. Neighborhood Disadvantage and Early Respiratory Outcomes in Very Preterm Infants with Bronchopulmonary Dysplasia

Author

Steve Nauleau, L. Fayol, Juliette Deschamps, Patricia M. Garcia, Farid Boubred, Jean Christophe Dubus, Mohamed Boucekine, Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), and Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)

Subjects
Male, Pediatrics, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Birth weight, Population, Breastfeeding, Gestational Age, 03 medical and health sciences, 0302 clinical medicine, Residence Characteristics, 030225 pediatrics, Outcome Assessment, Health Care, Humans, Medicine, 030212 general & internal medicine, education, ComputingMilieux_MISCELLANEOUS, Bronchopulmonary Dysplasia, education.field_of_study, business.industry, Age Factors, Infant, Newborn, Infant, Social environment, Gestational age, medicine.disease, Disadvantaged, Hospitalization, Socioeconomic Factors, Bronchopulmonary dysplasia, Bronchiolitis, Infant, Extremely Premature, Pediatrics, Perinatology and Child Health, Female, France, business
Abstract

International audience; Objective To investigate the impact of neighborhood conditions on respiratory-related hospital admissions in the first year after discharge from the neonatal unit in a population of infants born very preterm with bronchopulmonary dysplasia (BPD).Study design Very preterm infants (gestational age

Published
2021

38. Description of two anaphylaxis phenotypes in children: The utility of a clustering analysis

Author

H. Béhal, Antoine Deschildre, Jean-Christophe Dubus, Guillaume Pouessel, S. Lejeune, and J. Corriger

Subjects
medicine.medical_specialty, business.industry, digestive, oral, and skin physiology, Mean age, Atopic dermatitis, Disease, medicine.disease, Adrenaline injection, Food allergy, Internal medicine, medicine, Immunology and Allergy, Insect venom, business, Anaphylaxis, Asthma
Abstract

Background Anaphylaxis appears to be heterogeneous with regards to patients’ characteristics, clinical manifestations, triggers, and severity. Objective We set out to define anaphylaxis phenotypes using a non-supervised cluster analysis in children experiencing anaphylaxis. Methods Characteristics of children ( Results (See Fig. 1 ) Two hundre and twenty two (male: 62%, mean age: 7.9 y; SD: 5.1) children were included. Two anaphylaxis clusters have been described. Cluster 1, “Food (nuts) severe anaphylaxis in highly atopic children” (n = 56, 25%): all children had food-induced anaphylaxis, most often induced by peanut (38%) and nuts (36%), and a previous food allergy to the same trigger (89%). These children had allergic comorbidities (multiple food allergy, 77%; asthma, 63%; atopic dermatitis, 36%) and had more severe anaphylaxis reactions (Ring score 3–4, 45%; adrenaline injection, 46%; hospital admission, 36%). Cluster 2, “Mild anaphylaxis with various triggers and few atopic comorbidities” (n = 168, 75%): these children were younger (p = 0.039), had less frequent allergic disease (asthma, 31%; food allergy, 16%; atopic dermatitis, 17%), with various triggers (food, 68%; drugs, 10%; insect venom, 7%) and less severe reactions (Ring 2, 78%; adrenaline injection, 26%; hospital admission, 20%). Conclusion We confirm the heterogeneity of anaphylaxis in children. Children with allergic comorbidities and food triggers (nuts) are at risk of more severe anaphylaxis reactions as compared to children with less allergic disease and other triggers than food. Larger studies are required to confirm these anaphylaxis phenotypes in children.

Published
2021

39. Aerosolized Antibiotics

Author

Jonathan Dugernier, Gregory Reychler, Jean-Christophe Dubus, and Stephan Ehrmann

Subjects
Pulmonary and Respiratory Medicine, Critical Care and Intensive Care Medicine
Published
2017

40. Aspergillus fumigatus in cystic fibrosis: An update on immune interactions and molecular diagnostics in allergic bronchopulmonary aspergillosis

Author

Joana Vitte, Jean-Christophe Dubus, Thomas Romain, Martine Reynaud-Gaubert, Stéphane Ranque, Ania Carsin, Jean-Louis Mege, COMBE, Isabelle, Assistance Publique - Hôpitaux de Marseille (APHM), Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes (URMITE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48, Institut des sciences biologiques (INSB-CNRS)-Institut des sciences biologiques (INSB-CNRS)-Centre National de la Recherche Scientifique (CNRS), Unité de Recherche sur les Maladies Infectieuses Tropicales Emergentes (URMITE), Institut des sciences biologiques (INSB-CNRS)-Institut des sciences biologiques (INSB-CNRS)-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48, INSB-INSB-Centre National de la Recherche Scientifique (CNRS), and INSB-INSB-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48

Subjects
0301 basic medicine, Pathology, medicine.medical_specialty, Cystic Fibrosis, Immunology, Cystic fibrosis, Aspergillus fumigatus, 03 medical and health sciences, 0302 clinical medicine, Immune system, Fibrosis, medicine, Humans, Immunology and Allergy, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, biology, business.industry, Aspergillosis, Allergic Bronchopulmonary, biology.organism_classification, medicine.disease, Molecular diagnostics, 3. Good health, 030104 developmental biology, 030228 respiratory system, Respiratory failure, Host-Pathogen Interactions, biology.protein, Antibody, Allergic bronchopulmonary aspergillosis, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

International audience; A wide spectrum of pathological conditions may result from the interaction of Aspergillus fumigatus and the immune system of its human host. Allergic bronchopulmonary aspergillosis is one of the most severe A.fumigatus-related diseases due to possible evolution toward pleuropulmonary fibrosis and respiratory failure. Allergic bronchopulmonary aspergillosis occurs almost exclusively in cystic fibrosis or asthmatic patients. An estimated 8%-10% of patients with cystic fibrosis experience this condition. The diagnosis of allergic bronchopulmonary aspergillosis relies on criteria first established in 1977. Progress in the understanding of host-pathogen interactions in A.fumigatus and patients with cystic fibrosis and the ongoing validation of novel laboratory tools concur to update and improve the diagnosis of allergic bronchopulmonary aspergillosis.

Published
2017

41. Évaluation des connaissances en aérosolthérapie du personnel hospitalier

Author

M. Baravalle, Jean-Christophe Dubus, Ania Carsin, and S. Orfanos

Subjects
Pulmonary and Respiratory Medicine, Gynecology, 03 medical and health sciences, medicine.medical_specialty, 0302 clinical medicine, 030228 respiratory system, business.industry, 030225 pediatrics, medicine, Inhalation Devices, business, 3. Good health, Inhalation technique
Abstract

Resume Introduction L’aerosoltherapie est le traitement privilegie de l’asthme par son action locale et rapide. Cependant, son efficacite depend de la technique d’inhalation apprise aupres des professionnels de sante. Nous avons evalue les connaissances en aerosoltherapie du personnel hospitalier. Methodes Il s’agit d’une etude observationnelle multicentrique effectuee au sein des services de pneumologie et de pediatrie de Marseille. Les pratiques de service, les connaissances pratiques et theoriques sur l’aerosoltherapie, du personnel medical et paramedical etaient evaluees par l’intermediaire d’un questionnaire et d’une demonstration a l’utilisation d’une chambre d’inhalation. Resultats Quarante personnels medicaux et paramedicaux (9 medecins, 14 internes, 16 infirmieres, 1 kinesitherapeute) ont ete interroges dans 8 services de pediatrie et de pneumologie. Tous etaient impliques dans la mise en route de traitements inhales. Une formation a l’aerosoltherapie avait ete suivie par 42,5 %. Concernant les connaissances theoriques, on notait 54 % de reponses justes. On notait en moyenne 1,12 erreurs sur 7 sur la demonstration avec la chambre d’inhalation (45 % sans erreur). Les medecins connaissaient statistiquement mieux l’aerosoltherapie que les internes et les infirmieres. Conclusion Les connaissances parcellaires sur l’aerosoltherapie des personnels hospitaliers plaident pour une meilleure formation de ces personnels.

Published
2017

42. Air pollution and children’s asthma-related emergency hospital visits in southeastern France

Author

Denis Charpin, Jean-Christophe Dubus, Guilhem Noel, Jean Gaudart, Gilles Viudes, Julie Mazenq, Antoine Nougairède, Pédiatrie et oncologie pédiatrique [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Sciences Economiques et Sociales de la Santé & Traitement de l'Information Médicale (SESSTIM - U912 INSERM - Aix Marseille Univ - IRD), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Nord [CHU - APHM], Emergence des Pathologies Virales (EPV), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille), Regional Emergencies Observatory, Provence-Alpes-Cote d'Azur region (ORU PACA), Hyères, France., Médecine Infantile [Hôpital Nord - APMH], Gaudart, Jean, Institut de Recherche pour le Développement (IRD)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)

Subjects
Male, [MATH.MATH-PR] Mathematics [math]/Probability [math.PR], Pediatrics, Air pollution, 010501 environmental sciences, medicine.disease_cause, 01 natural sciences, 0302 clinical medicine, [STAT.AP] Statistics [stat]/Applications [stat.AP], Residence Characteristics, Risk Factors, [MATH.MATH-ST]Mathematics [math]/Statistics [math.ST], [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Odds Ratio, pollution, Child, [MATH.MATH-ST] Mathematics [math]/Statistics [math.ST], Children, [SDV.MHEP.ME] Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Air Pollutants, [STAT.AP]Statistics [stat]/Applications [stat.AP], [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, [STAT.ME] Statistics [stat]/Methodology [stat.ME], Air, Absolute risk reduction, Child, Preschool, Disease Progression, [SDV.MHEP.MI] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Female, France, Emergency Service, Hospital, [STAT.ME]Statistics [stat]/Methodology [stat.ME], [SDV.MP.PAR] Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, medicine.medical_specialty, Adolescent, [MATH.MATH-DS]Mathematics [math]/Dynamical Systems [math.DS], Protective factor, [MATH.MATH-DS] Mathematics [math]/Dynamical Systems [math.DS], Environment, Zip code, 03 medical and health sciences, Air Pollution, 030225 pediatrics, Environmental health, medicine, Humans, [SDV.EE.SANT] Life Sciences [q-bio]/Ecology, environment/Health, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Weather, Male gender, 0105 earth and related environmental sciences, Asthma, [SDV.EE.SANT]Life Sciences [q-bio]/Ecology, environment/Health, business.industry, Odds ratio, Protective Factors, medicine.disease, [SDE.ES]Environmental Sciences/Environmental and Society, [MATH.MATH-PR]Mathematics [math]/Probability [math.PR], [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, 13. Climate action, Case-Control Studies, Multivariate Analysis, Pediatrics, Perinatology and Child Health, Particulate Matter, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Residence, [SDE.ES] Environmental Sciences/Environmental and Society, business
Abstract

International audience; Children's asthma is multifactorial. Environmental factors like air pollution exposure, meteorological conditions, allergens, and viral infections are strongly implicated. However, place of residence has rarely been investigated in connection with these factors. The primary aim of our study was to measure the impact of particulate matter (PM), assessed close to the children's homes, on asthma-related pediatric emergency hospital visits within the Bouches-du-Rhône area in 2013. In a nested case-control study on 3- to 18-year-old children, each control was randomly matched on the emergency room visit day, regardless of hospital. Each asthmatic child was compared to 15 controls. PM10 and PM2.5, meteorological conditions, pollens, and viral data were linked to ZIP code and analyzed by purpose of emergency visit. A total of 68,897 visits were recorded in children, 1182 concerning asthma. Short-term exposure to PM10 measured near children's homes was associated with excess risk of asthma emergency visits (adjusted odds ratio 1.02 (95% CI 1.01-1.04; p = 0.02)). Male gender, young age, and temperature were other risk factors. Conversely, wind speed was a protective factor.CONCLUSION:PM10 and certain meteorological conditions near children's homes increased the risk of emergency asthma-related hospital visits in 3- to 18-year-old children in Bouches-du-Rhône. What is Known: • A relationship between short-term exposure to air pollution and increase in emergency room visits or hospital admissions as a result of increased pollution levels has already been demonstrated. What is New: • This study confirms these results but took into account confounding factors (viral data, pollens, and meteorological conditions) and is based on estimated pollution levels assessed close to the children's homes, rather than those recorded at the hospital. • The study area, the Mediterranean, is favorable to creation of secondary pollutants in these sunny and dry seasons.

Published
2017

43. Chambres d’inhalation pour délivrance de fluticasone chez le nourrisson au banc d’essai

Author

Gwendoline Sarry, Jean-Michel Vergnon, Jean-Christophe Dubus, Jérémie Pourchez, and Lara Leclerc

Subjects
Pulmonary and Respiratory Medicine, Gynecology, medicine.medical_specialty, business.industry, Bench test, 3. Good health, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, medicine, 030212 general & internal medicine, business, Fluticasone, medicine.drug
Abstract

Resume Introduction La delivrance de fluticasone via une chambre d’inhalation est delicate chez les jeunes nourrissons asthmatiques. Le choix du dispositif est souvent difficile pour le clinicien. Cette etude caracterise in vitro la granulometrie et la delivrance medicamenteuse de la fluticasone par neuf chambres d’inhalation. Methodes Nous avons utilise un modele anatomique in vitro de nourrisson avec deux debits inspiratoires differents (50 et 100 mL/s). La granulometrie de l’aerosol etait evaluee par diffractometrie laser et le depot tracheal par dosage spectrophotometrique. Resultats Des differences granulometriques importantes entre les chambres d’inhalation etaient observees (D50 identique mais D90 de 5,65 ± 0,65 μm a 8,80 ± 1,35 μm). Une fraction respirable superieure a 75 % n’etait obtenue qu’avec cinq chambres d’inhalation. Le debit a 50 mL/s permettait d’obtenir la meilleure delivrance medicamenteuse. Pour ce debit, Optichamber® (62 ± 3 %) et Vortex® (91 ± 8,5 %) avaient un depot tracheal superieur a 50 % de la dose initiale de fluticasone, les sept autres chambres d’inhalation presentant un depot tracheal inferieur a 25 %. Discussion Notre etude montre de fortes variations de delivrance entre les neuf chambres d’inhalation. Un debit inspiratoire faible et une chambre d’inhalation de nature antistatique semblent des elements favorisant cette delivrance.

Published
2017

44. Radiographie de thorax et bronchiolite aiguë : des indications en diminution ?

Author

Brigitte Chabrol, A. Carsin, A. Dabadie, Josette Mancini, Guillaume Gorincour, E. Bosdure, Jean-Christophe Dubus, V. Arnoux, and K. Retornaz

Subjects
First episode, Chronic condition, Pediatrics, medicine.medical_specialty, medicine.diagnostic_test, Heart disease, business.industry, Atelectasis, Retrospective cohort study, medicine.disease, 3. Good health, 03 medical and health sciences, Pneumonia, 0302 clinical medicine, 030225 pediatrics, Pediatrics, Perinatology and Child Health, medicine, 030212 general & internal medicine, Differential diagnosis, Chest radiograph, business
Abstract

Objective. A management protocol for infants hospitalized for acute bronchiolitis, established after the study conducted in our unit in 2012, recommends a chest X-ray when the clinical course is unusual or if a differential diagnosis is suspected. The goal of this study was to evaluate professional practices after the introduction of this new management protocol. Study design. Retrospective descriptive study in two pediatric units from October 2013 to March 2015, including infants (0-23 months) hospitalized for their first episode of acute bronchiolitis without any underlying chronic condition. Result. Overall, 599 infants were included (median age, 3.7 months, 54 % boys). Nearly six out of ten (n = 355, 59.3 %) had at least one chest radiograph (38.5 % fewer than in 2012). It was abnormal in 96.3 % of cases, revealing distension and/or bronchial wall thickening (56.7 %), focal opacity (23.5 %), or atelectasis (19.5 %). An X-ray was performed out of the recommendations in 42.5 % of cases. The chest X-ray result led to management changes in 52 infants with prescription of antibiotics for pneumonia (86.5 %) and allowed the diagnosis of heart disease in one case (0.2 %). Management of acute bronchiolitis (X-ray and antibiotics) was statistically different between the two pediatric units. Discussion. This protocol led to a significant decrease in the number of chest X-rays. However, many are still performed out of the recommendations, resulting in an increase of antibiotic use for pneumonia. Conclusion. The decrease in use of chest X-rays in acute bronchiolitis for hospitalized infants was significant but remains insufficient. (C) 2016 Elsevier Masson SAS. All rights reserved.

Published
2017

45. Cystic fibrosis in the modern therapeutic era: Give the shower a thought!

Author

M. Baravalle-Einaudi, Deborah Le Pennec, Jean-Christophe Dubus, Laurent Vecellio, Christian Dupont, Université de Tours (UT), Hôpital de la Timone [CHU - APHM] (TIMONE), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Microbes évolution phylogénie et infections (MEPHI), and Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)

Subjects
0301 basic medicine, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Mesh-nebulizer, Cystic fibrosis, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Administration, Inhalation, Tobramycin, medicine, Humans, In vitro study, Pseudomonas Infections, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, ComputingMilieux_MISCELLANEOUS, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, business.industry, Nebulizers and Vaporizers, Home use, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, Anti-Bacterial Agents, 3. Good health, Equipment Failure Analysis, Self Care, 030104 developmental biology, 030228 respiratory system, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Pediatrics, Perinatology and Child Health, Equipment Contamination, Equipment Failure, Radiology, business, medicine.drug
Abstract

International audience; Fifty-two meshes of e-flow rapid® were characterized for tobramycin delivery with a laser diffractometer after 6 months of home use by cystic fibrosis patients treated with various nebulized drugs. Three meshes were out of order and 30 considered to be defective for tobramycin delivery. The use of the specific mesh cleaning shower system permitted 14 defective meshes to be in the expected range of nebulized volume.

Published
2019

46. Pulmonary contusion after bumper car collision: Case report and review of the literature

Author

Karine Retornaz, Emi Cauchois, Jean-Christophe Dubus, Marjolaine Oger, Valérie Arnoux, Mahé Berland, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), and Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, Case Report, Chest injury, 03 medical and health sciences, 0302 clinical medicine, Bronchoscopy, Main Bronchus, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, 030225 pediatrics, medicine, Thoracic trauma, lcsh:RC705-779, Lung, medicine.diagnostic_test, business.industry, 030208 emergency & critical care medicine, lcsh:Diseases of the respiratory system, medicine.disease, 3. Good health, Surgery, Pulmonary contusion, medicine.anatomical_structure, Blunt trauma, Supportive psychotherapy, business
Abstract

International audience; Thoracic trauma is a major source of morbi-mortality in injured children. Their pliable chest wall makes pulmonary contusion the most common chest injury. It is most often secondary to blunt trauma caused by traffic accidents. We report a case of severe chest trauma caused by a bumper car collision in an 8-years old girl. She sustained right lung contusion that led to complete atelectasis. After a week of supportive therapy, bronchoscopy removed a mucous plug from the main bronchus, resulting in significant clinical improvement. We aim to raise awareness of the risk of severe chest injuries during bumper car collisions.

Published
2018

47. Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis

Author

Pierre-Régis Burgel, Anne Munck, Isabelle Durieu, Raphaël Chiron, Laurent Mely, Anne Prevotat, Marlene Murris-Espin, Michele Porzio, Michel Abely, Philippe Reix, Christophe Marguet, Julie Macey, Isabelle Sermet-Gaudelus, Harriet Corvol, Stéphanie Bui, Lydie Lemonnier, Clémence Dehillotte, Jennifer Da Silva, Jean-Louis Paillasseur, Dominique Hubert, Julie Mounard, Claire Poulet, Cinthia Rames, Christine Person, Françoise Troussier, Thierry Urban, Marie-Laure Dalphin, Jean-Claude Dalphin, Didier Pernet, Bénédicte Richaud-Thiriez, Mickael Fayon, Julie Macey-Caro, Karine Campbell, Muriel Laurans, Corinne Borderon, Marie-Christine Heraud, André Labbé, Sylvie Montcouquiol, Laurence Bassinet, Natascha Remus, Annlyse Fanton, Anne Houzel-Charavel, Frédéric Huet, Stéphanie Perez-Martin, Amale Boldron-Ghaddar, Manuela Scalbert, Boubou Camara, Catherine Llerena, Isabelle Pin, Sébastien Quétant, Aurélie Cottereau, Antoine Deschildre, Alice Gicquello, Thierry Perez, Lidwine Stervinou-Wemeau, Caroline Thumerelle, Benoit Wallaert, Nathalie Wizla, Jane Languepin, Céline Ménétrey, Magalie Dupuy-Grasset, Lucie Bazus, Clelia Buchs, Virginie Jubin, Marie-Christine Werck-Gallois, Catherine Mainguy, Thomas Perrin, Agnès Toutain-Rigolet, Stéphane Durupt, Quitterie Reynaud, Raphaele Nove-Josserand, Melisande Baravalle-Einaudi, Bérangère Coltey, Nadine Dufeu, Jean-Christophe Dubus, Nathalie Stremler, Davide Caimmi, Yves Billon, Jocelyne Derelle, Sébastien Kieffer, Anne-Sophie Pichon, Cyril Schweitzer, Aurélie Tatopoulos, Sarah Abbes, Tiphaine Bihouée, Isabelle Danner-Boucher, Valérie David, Alain Haloun, Adrien Tissot, Sylvie Leroy, Carole Bailly-Piccini, Annick Clément, Aline Tamalet, Isabelle Honoré, Reem Kanaan, Clémence Martin, Cécile Bailly, Frédérique Chédevergne, Jacques De Blic, Brigitte Fauroux, Murielle Le Bourgeois, Bertrand Delaisi, Michèle Gérardin, Michel Abély, Bruno Ravoninjatovo, Chantal Belleguic, Benoit Desrues, Graziella Brinchault, Michel Dagorne, Eric Deneuville, Sylvaine Lefeuvre, Anne Dirou, Jean Le Bihan, Sophie Ramel, Stéphane Dominique, Annabelle Payet, Romain Kessler, Vincent Rosner, Laurence Weiss, Sandra de Miranda, Dominique Grenet, Abdoul Hamid, Clément Picard, François Brémont, Alain Didier, Géraldine Labouret, Marie Mittaine, Marlène Murris-Espin, Laurent Têtu, Laure Cosson, Charlotte Giraut, Anne-Cécile Henriet, Julie Mankikian, Sophie Marchand, Sandrine Hugé, Véronique Storni, Emmanuelle Coirier-Duet, CHU Cochin [AP-HP], Service de Médecine Interne - Centre Hospitalier Lyon Sud, Hospices Civils de Lyon (HCL)-Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre de Ressources et de Compétences en Mucoviscidose [Lyon] (CRCM [Lyon]), Hospices Civils de Lyon (HCL)-CHU Lyon-Hôpital Renée Sabran [CHU - HCL], Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Toulouse [Toulouse], Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Service de pédiatrie médicale et médecine de l'adolescent [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre de recherche Croissance et signalisation (UMR_S 845), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de pneumologie [CHU Trousseau], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Centre de Ressources et de Compétences de la Mucoviscidose (CRCM), Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Association Vaincre La Mucoviscidose, Institut Cochin (IC UM3 (UMR 8104 / U1016)), EFFI-STAT, CIC Cochin Pasteur (CIC 1417), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP]-Hôtel-Dieu-Université Paris Descartes - Paris 5 (UPD5)-Groupe hospitalier Broca-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Mucoviscidose: physiopathologie et phénogénomique [CRSA], Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Centre de Ressources et de Compétences en Mucoviscidose [CHU Toulouse] (CRCM Toulouse), Service Pneumologie et allergologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôtel-Dieu-Université Paris Descartes - Paris 5 (UPD5)-Groupe hospitalier Broca-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Institut National de la Santé et de la Recherche Médicale (INSERM)-Groupe hospitalier Broca-Université Paris Descartes - Paris 5 (UPD5)-Hôtel-Dieu-Hôpital Cochin [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
Male, Cystic Fibrosis, Gastrointestinal Diseases, [SDV]Life Sciences [q-bio], Aminopyridines, Quinolones, Critical Care and Intensive Care Medicine, Logistic regression, Aminophenols, Cystic fibrosis, Body Mass Index, Ivacaftor, chemistry.chemical_compound, 0302 clinical medicine, Deprescriptions, Forced Expiratory Volume, Medicine, 030212 general & internal medicine, Fatigue, 2. Zero hunger, biology, Lumacaftor, Headache, lumacaftor–ivacaftor, Cystic fibrosis transmembrane conductance regulator, 3. Good health, Anti-Bacterial Agents, Drug Combinations, Treatment Outcome, Administration, Intravenous, Female, France, medicine.drug, Pulmonary and Respiratory Medicine, Adult, medicine.medical_specialty, Metrorrhagia, Adolescent, Nutritional Status, 03 medical and health sciences, Young Adult, Internal medicine, Product Surveillance, Postmarketing, Humans, Benzodioxoles, Adverse effect, Bronchial Spasm, business.industry, Editorials, Myalgia, medicine.disease, Discontinuation, Dyspnea, Logistic Models, 030228 respiratory system, chemistry, Cough, Multivariate Analysis, biology.protein, postmarketing study, business, Body mass index
Abstract

International audience; Rationale: Lumacaftor-ivacaftor is a CFTR (cystic fibrosis transmembrane conductance regulator) modulator combination recently approved for patients with cystic fibrosis (CF) homozygous for the Phe508del mutation.Objectives: To evaluate the safety and effectiveness of lumacaftor-ivacaftor in adolescents (≥12 yr) and adults (≥18 yr) in a real-life postapproval setting.Methods: The study was conducted in the 47 CF reference centers in France. All patients who initiated lumacaftor-ivacaftor from January 1 to December 31, 2016, were eligible. Patients were evaluated for lumacaftor-ivacaftor safety and effectiveness over the first year of treatment following the French CF Learning Society's recommendations.Measurements and Main Results: Among the 845 patients (292 adolescents and 553 adults) who initiated lumacaftor-ivacaftor, 18.2% (154 patients) discontinued treatment, often owing to respiratory (48.1%, 74 patients) or nonrespiratory (27.9%, 43 patients) adverse events. In multivariable logistic regression, factors associated with increased rates of discontinuation included adult age group, percent predicted FEV1 (ppFEV1) less than 40%, and numbers of intravenous antibiotic courses during the year before lumacaftor-ivacaftor initiation. Patients with continuous exposure to lumacaftor-ivacaftor showed an absolute increase in ppFEV1 (+3.67%), an increase in body mass index (+0.73 kg/m2), and a decrease in intravenous antibiotic courses by 35%. Patients who discontinued treatment had significant decrease in ppFEV1, without improvement in body mass index or decrease in intravenous antibiotic courses.Conclusions: Lumacaftor-ivacaftor was associated with improvement in lung disease and nutritional status in patients who tolerated treatment. Adults who discontinued lumacaftor-ivacaftor, often owing to adverse events, were found at high risk of clinical deterioration.

Published
2019

48. Use of high-flow nasal cannula in infants with viral bronchiolitis outside pediatric intensive care units

Author

Mélanie Panciatici, S. Tardieu, Marion Dequin, Emilie Sauvaget, Jean-Christophe Dubus, Nathalie Stremler-Le Bel, Candice Fabre, Emmanuelle Bosdure, Aix-Marseille Université - Faculté de médecine (AMU MED), Aix Marseille Université (AMU), Hôpital de la Timone [CHU - APHM] (TIMONE), Microbes évolution phylogénie et infections (MEPHI), and Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)

Subjects
medicine.medical_specialty, Vital signs, Hospitals, General, Intensive Care Units, Pediatric, medicine.disease_cause, Pediatrics, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Surveys and Questionnaires, 030225 pediatrics, Intensive care, medicine, Bronchiolitis, Viral, Cannula, Humans, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, 030212 general & internal medicine, Nose, ComputingMilieux_MISCELLANEOUS, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Continuous Positive Airway Pressure, Hospitals, Public, business.industry, Infant, University hospital, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, 3. Good health, medicine.anatomical_structure, Bronchiolitis, Pediatrics, Perinatology and Child Health, Emergency medicine, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Bronchitis, France, High flow, business, Nasal cannula
Abstract

High-flow nasal cannula (HFNC) is frequently used in infants with acute viral bronchiolitis outside pediatric intensive care units (PICU). A structured questionnaire was sent out to pediatricians of all public French hospitals with pediatric emergency and/or general pediatric departments on their use of HFNC outside PICU (department using HFNC, number of available devices, monitoring, criteria for initiating or stopping HFNC, and personal comments on HFNC). Of the 166 eligible hospitals, 135 answered (96 general and 39 university hospitals; 81.3%), for a total of 217 answering pediatricians. Seventy-two hospitals (53.3%) used HFNC in acute bronchiolitis outside PICU, particularly, general hospitals (59.4% vs 38.5%), and mostly in pediatric general departments (75%). Continuous patient monitoring with a cardiorespiratory monitor was usual (n = 58, 80%). Nursing staff was responsible for 2.7 children on HFNC and checked vital signs 8.6 times per day. Criteria for HFNC initiation and withdrawal were not standardized. Pediatricians had a positive opinion of HFNC and were willing to extend its use to other diseases.Conclusion: Use of HFNC outside PICU in infants with acute bronchiolitis is now usual, but urgently requires guidelines. What is Known: • Acute viral bronchiolitis treatment is only supportive • High-flow nasal cannula (HFNC) is a respiratory support accumulating convincing clinical evidence in bronchiolitis • This latter treatment is usually proposed in pediatric intensive care unit (PICU) What is New: • HFNC are increasingly used outside PICU in bronchiolitis, particularly, in general hospitals and in pediatric general departments • Pediatricians are enthusiastic about this device, but validated criteria for initiation and withdrawal are lacking • Guidelines for the use of HFNC outside PICU are urgently required.

Published
2019

49. Health-related quality of life in children interstitial lung disease

Author

Clara Lauby, Marie-Catherine Renoux, Pierre-Yves Boëlle, Isabelle Pin, Jean-Christophe Dubus, Michael Fayon, Christophe Marguet, Antoine Deschildre, Anne Houzel, Christophe Delacourt, Nadia Nathan, Laurence Weiss, Céline Delestrain, Cyril Schweitzer, Annick Clement, Lisa Giovannini-Chami, Ralph Epaud, Marie-Laure Dalphin, Véronique Houdouin, Jacques Brouard, Rola Abou Taam, Katia Bessaci, Aurélie Tatopoulos, Caroline Thumerelle, Philippe Reix, Françoise Troussier, and Stéphanie Wanin

Subjects
Health related quality of life, Pediatrics, medicine.medical_specialty, Quality of life, business.industry, Healthy population, Interstitial lung disease, Medicine, Disease, business, medicine.disease, humanities, Lung function
Abstract

Introduction: Health-related quality of life (HR-QoL) is an understudied area in rare diseases. Interstitial lung diseases in children (chILD) are heterogeneous disorders that are associated with a high morbidity and mortality. The impaired lung function, the nutritional impact and the burden of the treatments can impair the HR-QoL of these children. The aim of this study was to compare the HR-QoL in chILD to an aged-matched healthy population and to identify the factors that were associated to an altered QoL. Methods: Patients were recruited in the French RespiRare network. Patients with chILD of all causes, aged 1 month to 18 years were prospectively included. The children over 8 years old and the 2 parents of all children were asked to complete the PedsQLTM 4.0 Generic Core Scale (0 to 100 points). Results: A total of 78 children were included in the RespiRare centres. Total mean scores appeared to be significantly reduced in child self-report and in parent proxy-report (11.94 points (p=0.0003) and 14.08 points (p Conclusion: The study shows that HR-QoL is significantly impaired in chILD compared to a healthy population, using a validated generic QoL scale. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL. A longitudinal follow-up of HR-QoL in chILD is necessary to follow the impact of the disease and the treatments. To better appreciate chILD QoL, a specific scale is currently under study by the chILD-EU group.

Published
2019

50. Health‐related quality of life in infants and children with interstitial lung disease

Author

Aurélie Tatopoulos, Philippe Reix, Ralph Epaud, Jean-Christophe Dubus, Antoine Deschildre, Caroline Thumerelle, Christophe Delacourt, Lisa Giovannini-Chami, Katia Bessaci, Marie-Laure Dalphin, Nadia Nathan, Céline Delestrain, Pierre-Yves Boëlle, Stéphanie Wanin, Rola Abou Taam, Laurence Weiss, Annick Clement, Michael Fayon, Christophe Marguet, Cyril Schweitzer, Véronique Houdouin, Jacques Brouard, Françoise Troussier, Marie-Catherine Renoux, Anne Houzel, Isabelle Pin, Clara Lauby, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Epidémiologie, Systèmes d'Information, Modélisation, Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Centre Référence des Maladies Héréditaires du Métabolisme de l'Enfant et de l'Adulte [CHU Necker] (MaMEA Necker), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital universitaire Robert Debré [Reims], Service de Pédiatrie Médicale [Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Groupe de Recherche sur l'Adaptation Microbienne (GRAM 2.0), Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Laboratoire de Sciences de la Terre (LST), École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut national des sciences de l'Univers (INSU - CNRS)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Intercommunal de Créteil (CHIC), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de pédiatrie spécialisée et médecine infantile (neurologie, pneumologie, maladies héréditaires du métabolisme) [Hôpital de la Timone - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), CHU Bordeaux [Bordeaux], Centre Hospitalier Universitaire de Nice (CHU Nice), AP-HP Hôpital universitaire Robert-Debré [Paris], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de pédiatrie médicale et médecine de l'adolescent [Rouen], CHU Rouen, CHU Grenoble, Inflammatory Responses and Transcriptomic Networks in 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), Hospices Civils de Lyon (HCL), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de Médecine Infantile III et Génétique Clinique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Service d'immunologie [HEGP, Paris], Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Biomécanique cellulaire et respiratoire (BCR), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et la Recherche Médicale (INSERM), the European Union's Seventh Framework Program, Grant/Award Number: FP7‐ChILD‐EU 2007‐2013, Respirer c'est Grandir and Belleherbe Association, École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Service de Pneumologie pédiatrique [CHU Trousseau], Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Maladies génétiques d'expression pédiatrique [CHU Trousseau] (Inserm U933), Mzembaba, Sandy, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Université de Caen Normandie (UNICAEN), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Physiologie, biologie des organismes, populations, interactions, and Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Pulmonary and Respiratory Medicine, Male, Parents, Pediatrics, medicine.medical_specialty, chILD, Adolescent, medicine.medical_treatment, Disease, Severity of Illness Index, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, scale, 03 medical and health sciences, 0302 clinical medicine, Quality of life, children, 030225 pediatrics, Oxygen therapy, medicine, Humans, [SDV.MP] Life Sciences [q-bio]/Microbiology and Parasitology, ComputingMilieux_MISCELLANEOUS, Health related quality of life, [SDV.MP.VIR] Life Sciences [q-bio]/Microbiology and Parasitology/Virology, interstitial lung disease, Heterogeneous group, business.industry, Nutritional Support, Healthy population, Interstitial lung disease, Infant, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, humanities, Proxy, 3. Good health, Oxygen, [SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, 030228 respiratory system, quality of life, Child, Preschool, Pediatrics, Perinatology and Child Health, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Etiology, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Female, [SDV.MP.BAC] Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, business, Lung Diseases, Interstitial
Abstract

International audience; Introduction: Interstitial lung disease in children (chILD) is a highly heterogeneous group of rare and severe respiratory disorders. The disease by itself, the burden of the treatments (oxygen therapy, corticosteroid pulses, nutritional support) and recurrent hospitalizations may impair the quality of life (QoL) of these children. The aim of the study was to compare the health-related QoL (HR-QoL) in chILD compared to a healthy population and to find out the predictive factors of an altered QoL.Methods: Patients aged 1 month to 18 years with ILD of known or unknown etiology were prospectively included. Parents and children over 8 years old were asked to fill the PedsQL 4.0 Generic Core Scale ranging from 0 to 100 points.Results: A total of 78 children were recruited in 13 French pediatric centers. Total scores were 11.94 points (P = 0.0003) less for child self-report and 14.08 points ( P < 0.0001) less for parent proxy-report with respect to the healthy population. The clinical factors associated with a lower total score were: extrapulmonary expression of the disease, higher Fan severity score, long-term oxygen therapy, nutritional support, and a number of oral treatments.Conclusion: Using a validated quality of life (QoL) scale, we showed that health-related-QoL is significantly impaired in chILD compared with a healthy population. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL

Published
2019

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