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Your search keyword '"Galeron, L"' showing total 3 results
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3 results on '"Galeron, L"'

1. Management of children with interstitial lung diseases: The difficult issue of acute exacerbations

Author

Clement, A, De Blic, J, Epaud, R, Galeron, L, Nathan, N, Hadchouel, A, Barbato, A, Snijders, D, Kiper, N, Cunningham, S, Griese, M, Bush, A, Schwerk, N, ChILD-EU collaboration, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Physiopathologie des maladies génétiques d'expression pédiatrique, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Intercommunal de Créteil (CHIC), Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Università degli Studi di Padova = University of Padua (Unipd), Hacettepe University = Hacettepe Üniversitesi, University of Edinburgh, Ludwig-Maximilians University [Munich] (LMU), Dr von Hauner Children's Hospital [Munich, Germany], Ludwig-Maximilians-Universität München (LMU), German Center for Lung Research, Royal Brompton Hospital, Imperial College London, Hannover Medical School [Hannover] (MHH), The project (Orphans Unite, and chILD better together – European Management Platform for Childhood Interstitial Lung Disease) was funded by the FP7-305653-chILD-EU grant. A. Bush is an NIHR SeniorInvestigator, and additionally was supported by the NIHR Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London (London, UK).

Subjects
MESH: Expert Testimony, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Exacerbation, Respiratory System, MEDLINE, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 03 medical and health sciences, 0302 clinical medicine, MESH: Child, 030225 pediatrics, Medicine, Humans, Respiratory system, Intensive care medicine, Child, Expert Testimony, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, MESH: Humans, Lung, business.industry, Disease progression, 11 Medical And Health Sciences, respiratory system, 3. Good health, medicine.anatomical_structure, 030228 respiratory system, Disease Progression, MESH: Disease Progression, business, Lung Diseases, Interstitial, MESH: Lung Diseases, Interstitial
Abstract

The study provides the first proposal for acute exacerbation definition in paediatric interstitial lung diseases http://ow.ly/emPs305mpJ6 Interstitial lung disease (ILD) in children (chILD) is an umbrella term for a wide spectrum of rare diseases affecting the lung parenchyma, the causes of which often remain undetermined. These diffuse lung disorders are chronic, and often have high morbidity and mortality [1–3]. The epidemiology of the various forms of chILD is difficult to establish. Extrapolations from small studies have suggested an approximate incidence of 0.5–0.8 cases per 100 000 children [4, 5] However, this is certainly an underestimation due to the lack of standardised definitions, the inadequacy of organised reporting systems, and the variety of pathological conditions. In addition, clinical presentation is often nonspecific, contributing to a poor recognition of these disorders and confusion with other chronic pulmonary diseases. Insufficient disease-specific knowledge creates particular challenges for medical professionals, caregivers and chILD patients. Within the international community of clinicians and researchers involved in paediatric parenchymal lung diseases, the need for multicentre collaborations has resulted in the formation of networks of expertise to improve and harmonise approaches to diagnosis and management of the various forms of chILD. In this context, the present article reports expert opinions on the definition and diagnosis of acute exacerbations, which are major unpredictable deleterious episodes of acute worsening with significant morbidity that punctuate disease course.

Published
2016
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3. Germline SFTPA1 mutation in familial idiopathic interstitial pneumonia and lung cancer.

Author

Nathan N, Giraud V, Picard C, Nunes H, Dastot-Le Moal F, Copin B, Galeron L, De Ligniville A, Kuziner N, Reynaud-Gaubert M, Valeyre D, Couderc LJ, Chinet T, Borie R, Crestani B, Simansour M, Nau V, Tissier S, Duquesnoy P, Mansour-Hendili L, Legendre M, Kannengiesser C, Coulomb-L'Hermine A, Gouya L, Amselem S, and Clement A

Subjects
Adult, Aged, Female, Gene Expression Regulation, Genetic Predisposition to Disease, Humans, Idiopathic Interstitial Pneumonias pathology, Lung Neoplasms pathology, Male, Middle Aged, Pedigree, Pulmonary Surfactant-Associated Protein A metabolism, Sequence Analysis, DNA, Germ-Line Mutation, Idiopathic Interstitial Pneumonias genetics, Lung Neoplasms genetics, Mutation, Missense, Pulmonary Surfactant-Associated Protein A genetics
Abstract

Idiopathic interstitial pneumonias (IIPs) comprise a heterogeneous group of rare lung parenchyma disorders with high morbidity and mortality, which can occur at all ages. In adults, the most common form of IIPs, idiopathic pulmonary fibrosis (IPF), has been associated with an increased frequency of lung cancer. The molecular basis of IIPs remains unknown in most cases. This study investigates IIP pathophysiology in 12 families affected by IPF and lung cancer. We identified, in a multigenerational family, nine members carrying a heterozygous missense mutation with evidence of pathogenicity in SFTPA1 that encodes the surfactant protein (SP)-A1. The mutation (p.Trp211Arg), which segregates with a disease phenotype characterized by either isolated IIP/IPF, or IPF associated with lung adenocarcinoma, is located in the carbohydrate recognition domain (CRD) of SP-A1 and involves a residue invariant throughout evolution, not only in SP-A1, but also in its close paralog SP-A2 and other CRD-containing proteins. As shown through functional studies, the p.Trp211Arg mutation impairs SP-A1 secretion. Immunohistochemistry studies on patient alveolar epithelium showed an altered SP-A expression pattern. Overall, this first report of a germline molecular defect in SFTPA1 unveils the key role of SP-A1 in the occurrence of several chronic respiratory diseases, ranging from severe respiratory insufficiency occurring early in life to the association of lung fibrosis and cancer in adult patients. These data also clearly show that, in spite of their structural and functional similarities, SP-A1 and SP-A2 are not redundant., (© The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Published
2016
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