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Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort

dc.rights.licenseopenen_US
dc.contributor.authorFLETCHER, Camille
dc.contributor.authorHADCHOUEL, Alice
dc.contributor.authorTHUMERELLE, Caroline
dc.contributor.authorMAZENQ, Julie
dc.contributor.authorFLEURY, Manon
dc.contributor.authorCORVOL, Harriet
dc.contributor.authorJEDIDI, Nouha
dc.contributor.authorBENHAMIDA, Myriam
dc.contributor.authorBESSACI, Katia
dc.contributor.authorBILHOUEE, Tiphaine
dc.contributor.authorBORIE, Raphael
dc.contributor.authorBROUARD, Jacques
dc.contributor.authorCANTAIS, Aurelie
dc.contributor.authorCLEMENT, Annick
dc.contributor.authorCOUTIER, Laurianne
dc.contributor.authorCISTERNE, Camille
dc.contributor.authorCROS, Pierrick
dc.contributor.authorDALPHIN, Marie-Laure
dc.contributor.authorDELACOURT, Christophe
dc.contributor.authorDENEUVILLE, Eric
dc.contributor.authorDUBUS, Jean-Christophe
dc.contributor.authorEGRON, Carole
dc.contributor.authorEPAUD, Ralph
hal.structure.identifierBordeaux population health [BPH]
dc.contributor.authorFAYON, Michael
dc.contributor.authorFORGERON, Aude
dc.contributor.authorGACHELIN, Elsa
dc.contributor.authorGALODE, Francois
dc.contributor.authorGERTINI, Isabelle
dc.contributor.authorGIOVANNINI-CHAMI, Lisa
dc.contributor.authorGOURDAN, Pierre
dc.contributor.authorGUIDDIR, Tamazoust
dc.contributor.authorHERZOG, Audrey
dc.contributor.authorHOUDOUIN, Veronique
dc.contributor.authorHULLO, Eglantine
dc.contributor.authorJARREAU, Pierre-Henri
dc.contributor.authorLABBE, Guillame
dc.contributor.authorLABOURET, Geraldine
dc.contributor.authorLADAURADE, Alice
dc.contributor.authorLE CLAINCHE VIALA, Laurence
dc.contributor.authorMARGUET, Christophe
dc.contributor.authorMASSON-ROUCHAUD, Alexandra
dc.contributor.authorPERISSON, Caroline
dc.contributor.authorRAMES, Cinthia
dc.contributor.authorREIX, Philippe
dc.contributor.authorRENOUX, Marie-Catherine
dc.contributor.authorRODITIS, Lea
dc.contributor.authorSCHWEITZER, Cyril
dc.contributor.authorTATOPOULOS, Aurelie
dc.contributor.authorTRIOCHE-EBERSCHWEILER, Pascale
dc.contributor.authorTROUSSIER, Francoise
dc.contributor.authorVIGIER, Clementine
dc.contributor.authorWEISS, Laurence
dc.contributor.authorLEGENDRE, Marie
dc.contributor.authorLOUVRIER, Camille
dc.contributor.authorDE BECDELIEVRE, Alix
dc.contributor.authorCOULOMB, Aurore
dc.contributor.authorSILEO, Chiara
dc.contributor.authorDUCOU LE POINTE, Hubert
dc.contributor.authorBERTELOOT, Laureline
dc.contributor.authorDELESTRAIN, Celine
dc.contributor.authorNATHAN, Nadia
dc.date.accessioned2024-10-11T07:16:48Z
dc.date.available2024-10-11T07:16:48Z
dc.date.issued2024-08-19
dc.identifier.issn1468-3296en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/202416
dc.description.abstractEnINTRODUCTION: Interstitial lung disease in children (chILD) are rare and mostly severe lung diseases. Very few epidemiological data are available in limited series of patients. The aim of this study was to assess the prevalence and incidence of chILD in France. METHODS: We performed within the RespiRare network a multicentre retrospective observational study in patients with chILD from 2000 to 2022 and a prospective evaluation of chILD's incidence between February 2022 and 2023. RESULTS: chILD was reported in 790 patients in 42 centres. The estimated 2022 prevalence in France was 44 /million children (95% CI 40.76 to 47.46) and the computed incidence was 4.4 /million children (95% CI 3.44 to 5.56). The median age at diagnosis was 3 months with 16.9% of familial forms. Lung biopsy and genetic analyses were performed in 23.4% and 76.9%, respectively. The most frequent chILD aetiologies in the <2 years group were surfactant metabolism disorders (16.3%) and neuroendocrine cell hyperplasia of infancy (11.8%), and in the 2-18 years group diffuse alveolar haemorrhage (12.2%), connective tissue diseases (11.4%), hypersensitivity pneumonitis (8.8%) and sarcoidosis (8.8%). The management included mainly oxygen therapy (52%), corticosteroid pulses (56%), oral corticosteroids (44%), azithromycin (27.2%), enteral nutrition (26.9%), immunosuppressants (20.3%) and hydroxychloroquine (15.9%). The 5-year survival rate was 57.3% for the patients diagnosed before 2 years and 86% between 2 and 18 years. CONCLUSION: This large and systematic epidemiological study confirms a higher incidence and prevalence of chILD than previously described. In order to develop international studies, efforts are still needed to optimise the case collection and to harmonise diagnostic and management practices.
dc.language.isoENen_US
dc.subject.enChild
dc.subject.enClinical Epidemiology
dc.subject.enPaediatric Lung Disaese
dc.subject.enPaediatric interstitial lung disease
dc.subject.enRare lung diseases
dc.title.enEpidemiology of childhood interstitial lung disease in France: the RespiRare cohort
dc.title.alternativeThoraxen_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1136/thorax-2023-221325en_US
dc.subject.halSciences du Vivant [q-bio]/Santé publique et épidémiologieen_US
dc.identifier.pubmed38964860en_US
bordeaux.journalThoraxen_US
bordeaux.page842-852en_US
bordeaux.volume79en_US
bordeaux.hal.laboratoriesBordeaux Population Health Research Center (BPH) - UMR 1219en_US
bordeaux.issue9en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.institutionINSERMen_US
bordeaux.teamLEHA_BPHen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
bordeaux.identifier.funderIDAssistance publique-Hôpitaux de Parisen_US
bordeaux.identifier.funderIDEuropean Lung Foundationen_US
bordeaux.identifier.funderIDEuropean Respiratory Societyen_US
hal.popularnonen_US
hal.audienceInternationaleen_US
hal.exportfalse
dc.rights.ccPas de Licence CCen_US
bordeaux.COinSctx_ver=Z39.88-2004&amp;rft_val_fmt=info:ofi/fmt:kev:mtx:journal&amp;rft.jtitle=Thorax&amp;rft.date=2024-08-19&amp;rft.volume=79&amp;rft.issue=9&amp;rft.spage=842-852&amp;rft.epage=842-852&amp;rft.eissn=1468-3296&amp;rft.issn=1468-3296&amp;rft.au=FLETCHER,%20Camille&amp;HADCHOUEL,%20Alice&amp;THUMERELLE,%20Caroline&amp;MAZENQ,%20Julie&amp;FLEURY,%20Manon&amp;rft.genre=article


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