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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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2. Sftpa1 Mutation In Familial Idiopathic Interstitial Pneumonia And Lung ă Cancer

Author

Nathan, N., Giraud, V., Picard, C., Nunes, H., Ă Dastot Le Moal, F., Duquesnoy, P., Copin, B., Reynaud-Gaubert, M., Ă Valeyre, D., J Couderc, L., Chinet, T., Borie, R., Ă Crestani, B., Nau, V., Tissier, S., Galeron, L., Ă Ligniville, A., Kuziner, N., Simansour, M., Mansour Ă Hendili, L., Marie Legendre, Kannengiesser, C., Coulomb, A., Gouya, L., Ă Amselem, S., Annick Clément, Epicentre [Paris] [Médecins Sans Frontières], Etablissement Français du Sang - Alpes-Méditerranée (EFS - Alpes-Méditerranée), Etablissement Français du Sang, Anthropologie bio-culturelle, Droit, Ethique et Santé (ADES), Aix Marseille Université (AMU)-EFS ALPES MEDITERRANEE-Centre National de la Recherche Scientifique (CNRS), 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), Service de Radiologie et Echographie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Méthodes d'Analyses pour le Traitement d'Images et la Stéréorestitution (MATIS), Laboratoire des Sciences et Technologies de l'Information Géographique (LaSTIG), École nationale des sciences géographiques (ENSG), Institut National de l'Information Géographique et Forestière [IGN] (IGN)-Institut National de l'Information Géographique et Forestière [IGN] (IGN)-École nationale des sciences géographiques (ENSG), Institut National de l'Information Géographique et Forestière [IGN] (IGN)-Institut National de l'Information Géographique et Forestière [IGN] (IGN), Laboratoire de météorologie physique (LaMP), Centre National de la Recherche Scientifique (CNRS)-Université Blaise Pascal - Clermont-Ferrand 2 (UBP)-Institut national des sciences de l'Univers (INSU - CNRS), INSB-INSB-Centre National de la Recherche Scientifique (CNRS), Service de génétique et embryologie médicales [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), Laboratoire d'Hydrologie et de Géochimie de Strasbourg (LHyGeS), École Nationale du Génie de l'Eau et de l'Environnement de Strasbourg (ENGEES)-Université de Strasbourg (UNISTRA)-Institut national des sciences de l'Univers (INSU - CNRS)-Ecole et Observatoire des Sciences de la Terre (EOST), Université de Strasbourg (UNISTRA)-Institut national des sciences de l'Univers (INSU - CNRS)-Centre National de la Recherche Scientifique (CNRS)-Institut national des sciences de l'Univers (INSU - CNRS)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), and Université Blaise Pascal - Clermont-Ferrand 2 (UBP)-Institut national des sciences de l'Univers (INSU - CNRS)-Centre National de la Recherche Scientifique (CNRS)

Subjects
[SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases
Abstract

International Conference of the American-Thoracic-Society (ATS), San ă Francisco, CA, MAY 13-18, 2016; International audience; no abstract

4. 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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5. A national internet-linked based database for pediatric interstitial lung diseases: the French network.

Author

Nathan N, Taam RA, Epaud R, Delacourt C, Deschildre A, Reix P, Chiron R, de Pontbriand U, Brouard J, Fayon M, Dubus JC, Giovannini-Chami L, Bremont F, Bessaci K, Schweitzer C, Dalphin ML, Marguet C, Houdouin V, Troussier F, Sardet A, Hullo E, Gibertini I, Mahloul M, Michon D, Priouzeau A, Galeron L, Vibert JF, Thouvenin G, Corvol H, Deblic J, and Clement A

Subjects
Adolescent, Child, Child, Preschool, Female, France, Government Programs, Humans, Infant, Lung Diseases, Interstitial diagnosis, Lung Diseases, Interstitial genetics, Lung Diseases, Interstitial physiopathology, Male, Patient Care, Rare Diseases, Databases, Factual, Internet, Lung Diseases, Interstitial epidemiology
Abstract

Background: Interstitial lung diseases (ILDs) in children represent a heterogeneous group of rare respiratory disorders that affect the lung parenchyma. After the launch of the French Reference Centre for Rare Lung Diseases (RespiRare®), we created a national network and a web-linked database to collect data on pediatric ILD., Methods: Since 2008, the database has been set up in all RespiRare® centres. After patient's parents' oral consent is obtained, physicians enter the data of children with ILD: identity, social data and environmental data; specific aetiological diagnosis of the ILD if known, genetics, patient visits to the centre, and all medical examinations and tests done for the diagnosis and/or during follow up. Each participating centre has a free access to his own patients' data only, and cross-centre studies require mutual agreement. Physicians may use the system as a daily aid for patient care through a web-linked medical file, backed on this database., Results: Data was collected for 205 cases of ILD. The M/F sex ratio was 0.9. Median age at diagnosis was 1.5 years old [0-16.9]. A specific aetiology was identified in 149 (72.7%) patients while 56 (27.3%) cases remain undiagnosed. Surfactant deficiencies and alveolar proteinosis, haemosiderosis, and sarcoidosis represent almost half of the diagnoses. Median length of follow-up is 2.9 years [0-17.2]., Conclusions: We introduce here the French network and the largest national database in pediatric ILDs. The diagnosis spectrum and the estimated incidence are consistent with other European databases. An important challenge will be to reduce the proportion of unclassified ILDs by a standardized diagnosis work-up. This database is a great opportunity to improve patient care and disease pathogenesis knowledge. A European network including physicians and European foundations is now emerging with the initial aim of devising a simplified European database/register as a first step to larger European studies.

Published
2012
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