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

Autor:	Fletcher C; Paediatric Pulmonology Department and Reference Center for Rare Lung Diseases, RespiRare, Sorbonne University, AP-HP, Armand Trousseau Hospital, Paris, France.; Laboratory of Childhood Genetic Diseases, UMR_S933, Sorbonne University, INSERM, Armand Trousseau Hospital, Paris, France., Hadchouel A; AP-HP, Service de Pneumologie Pédiatrique and Reference center for rare lung diseases RespiRare, Necker-Enfants Malades Hospital, Paris, France.; INSERM U1151 INEM, Université Paris Cité, INSERM, Paris, France., Thumerelle C; Pediatric Pulmonology Department, Lille University Hospital, Lille, France., Mazenq J; Pediatric Pulmonology Department and Reference Center for Rare Lung Diseases RespiRare, Aix-Marseille University, AP-HM, Marseille, France.; INRAE, C2VN, INSERM, Marseille, France., Fleury M; APHP, Armand Trousseau Hospital, Pediatric Pulmonology Department and Reference Center for Rare Lung Diseases RespiRare, Sorbonne University, Paris, France., Corvol H; Paediatric Pulmonology Department and Reference Center for Rare Lung Diseases, RespiRare, Sorbonne University, AP-HP, Armand Trousseau Hospital, Paris, France.; CDR Saint-Antoine, Paris, France., Jedidi N; Paediatric Pulmonology Department and Reference Center for Rare Lung Diseases, RespiRare, Sorbonne University, AP-HP, Armand Trousseau Hospital, Paris, France., Benhamida M; Pediatric Department, University Hospital Centre Nantes, Nantes, France., Bessaci K; Pediatric Pulmonology Department, University Hospital Centre Reims, Reims, France., Bilhouee T; Pediatric Department, University Hospital Centre Nantes, Nantes, France., Borie R; APHP, Bichat Hospital, Pulmonology Department A, Université Paris Cité, Paris, France.; Inserm UMR-S 1152 PHERE, INSERM, Paris, France., Brouard J; Medical Pediatric Department, Inserm UMRS 1311, DYNAMICURE, UNICAEN, University Hospital Centre Caen, Caen, France., Cantais A; Pediatric Department, Saint-Etienne University Hospital, Saint-Etienne, France., Clement A; Plateforme d'expertise maladies rares, AP-HP.Sorbonne University, Paris, France., Coutier L; Pediatric Pulmonology Department, University Hospital Lyon, Lyon, France., Cisterne C; Pediatric Pulmonology Department, Lille University Hospital, Lille, France., Cros P; Pediatric Department, Centre Hospitalier Universitaire de Brest, Brest, France., Dalphin ML; Pediatric Pulmonology Department, Centre Hospitalier Universitaire de Besancon, Besancon, France., Delacourt C; AP-HP, Service de Pneumologie Pédiatrique and Reference center for rare lung diseases RespiRare, Necker-Enfants Malades Hospital, Paris, France.; INSERM U1151 INEM, Université Paris Cité, INSERM, Paris, France., Deneuville E; Pediatric Pulmonology Department, University Hospital Centre Rennes, Rennes, France., Dubus JC; Pediatric Pulmonology Department and Reference Center for Rare Lung Diseases RespiRare, Aix-Marseille University, AP-HM, Marseille, France.; IRD, MEPHI, IHU Méditerranée-Infection, Aix-Marseille Université, Marseille, France., Egron C; University Hospital Centre Clermont-Ferrand, Clermont-Ferrand, France., Epaud R; Pédiatrie, Centre Hospitalier Intercommunal de Créteil, Creteil, France.; FHU SENEC; University Paris Est Créteil, INSERM, IMRB, Créteil, France., Fayon M; Pediatric Pulmonology Department, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France.; Clinical Investigation Center (CIC 1401); Bordeaux University, Cardio-Thoracic Research Center of Bordeaux, Inserm, U1045, INSERM, Bordeaux, France., Forgeron A; Pediatric Pulmonology Department, Hospital Centre Le Mans, Le Mans, France., Gachelin E; Pediatric Pulmonology Department, CHU Nord Réunion, Saint-Denis, France., Galode F; Pediatric Pulmonology Department, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France., Gertini I; Pediatric Pulmonology Department, Tours University hospital, Tours, France., Giovannini-Chami L; Pediatric Pulmonology Department, Hôpitaux Pédiatriques de Nice CHU-LENVAL, Nice, France., Gourdan P; Pediatric Pulmonology Department, Hôpitaux Pédiatriques de Nice CHU-LENVAL, Nice, France., Guiddir T; Pediatric Pulmonology Department, AP-HP - Université Paris Saclay, Hospital Bicetre, Le Kremlin-Bicetre, France., Herzog A; Pediatric Pulmonology Department, CHU de Strasbourg, Strasbourg, France., Houdouin V; Pediatric Pulmonology Department, AP-HP - Paris University, Robert Debré Hospital, Paris, France., Hullo É; Pediatric Pulmonology Department, University Grenoble Alpes, CHU Grenoble Alpes, Grenoble, France., Jarreau PH; Néonatal intensive care unit, Cochin Hospital, Université Paris Cité, Paris, France., Labbé G; University Hospital Centre Clermont-Ferrand, Clermont-Ferrand, France., Labouret G; Pediatric Pulmonology Department, CHU Toulouse, Toulouse, France., Ladaurade A; Pediatric Pulmonology Department, Centre Hospitalier Universitaire de Besancon, Besancon, France., Le Clainche Viala L; Pediatric Pulmonology Department, AP-HP - Paris University, Robert Debré Hospital, Paris, France., Marguet C; Pediatric Pulmonology Department, CHU de Rouen, Rouen, France., Masson-Rouchaud A; Pediatric Pulmonology Department, CHU Limoges, Limoges, France., Perisson C; Pediatric Pulmonology Department, CHU de La Réunion Sites Sud, Saint-Pierre, Réunion., Rames C; Pediatric Department, CHU Amiens-Picardie, Amiens, France., Reix P; Pediatric Pulmonology Department, University Hospital Lyon, Lyon, France., Renoux MC; Pediatric Pulmonology Department, CHRU Montpellier, Montpellier, France., Roditis L; Pediatric Pulmonology Department, CHU Toulouse, Toulouse, France., Schweitzer C; Pediatric Pulmonology Department, CHU de Nancy, Nancy, France., Tatopoulos A; Pediatric Pulmonology Department, CHU de Nancy, Nancy, France., Trioche-Eberschweiler P; Pediatric Department, APHP.Paris-Saclay University, Antoine-Beclere Hospital, Clamart, France., Troussier F; Pediatric Department, CHU Angers, Angers, France., Vigier C; Pediatric Pulmonology Department, University Hospital Centre Rennes, Rennes, France., Weiss L; Pediatric Pulmonology Department, CHU de Strasbourg, Strasbourg, France., Legendre M; Laboratory of Childhood Genetic Diseases, UMR_S933, Sorbonne University, INSERM, Armand Trousseau Hospital, Paris, France.; APHP, Armand Trousseau Hospital, Molecular Genetics Department, Sorbonne University, Paris, France., Louvrier C; Laboratory of Childhood Genetic Diseases, UMR_S933, Sorbonne University, INSERM, Armand Trousseau Hospital, Paris, France.; APHP, Armand Trousseau Hospital, Molecular Genetics Department, Sorbonne University, Paris, France., de Becdelievre A; Molecular Genetics Department, Centre Hospitalier Universitaire Henri Mondor, Creteil, France.; INSERM U-955, Université Paris Est Creteil, INSERM, Créteil, France., Coulomb A; Pathology Department, Sorbonne University, AP-HP, Armand-Trousseau Hospital, Paris, France., Sileo C; APHP, Sorbonne University, Radiology Department, Armand-Trousseau Hospital, Paris, France., Ducou le Pointe H; APHP, Sorbonne University, Radiology Department, Armand-Trousseau Hospital, Paris, France., Berteloot L; Pediatric Radiology Department, APHP, Université Paris Cité, Necker-Enfants Malades Hospitals, Paris, France., Delestrain C; Pédiatrie, Centre Hospitalier Intercommunal de Créteil, Creteil, France.; FHU SENEC; University Paris Est Créteil, INSERM, IMRB, Créteil, France., Nathan N; Paediatric Pulmonology Department and Reference Center for Rare Lung Diseases, RespiRare, Sorbonne University, AP-HP, Armand Trousseau Hospital, Paris, France nadia.nathan@aphp.fr.; Laboratory of Childhood Genetic Diseases, UMR_S933, Sorbonne University, INSERM, Armand Trousseau Hospital, Paris, France.
Jazyk:	angličtina
Zdroj:	Thorax [Thorax] 2024 Aug 19; Vol. 79 (9), pp. 842-852. Date of Electronic Publication: 2024 Aug 19.
DOI:	10.1136/thorax-2023-221325
Abstrakt:	Introduction: 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. Competing Interests: Competing interests: None declared. (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)
Databáze:	MEDLINE
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