Other authors

Institut Català de la Salut

[Nathan N] AP-HP, Sorbonne Université, Pediatric Pulmonology Department and Reference Center for Rare Lung Disease RespiRare, Armand Trousseau Hospital, Paris, France. Sorbonne Université, Inserm Laboratory of Childhood Genetic Diseases, Armand Trousseau Hospital, Paris, France. [Griese M, Michel K] Department of Paediatric Pneumology, Dr von Hauner Children’s Hospital, German Centre for Lung Research, University of Munich, Munich, Germany. [Carlens J] Clinic for Pediatric Pneumology, Hannover Medical School, Hannover, Germany. [Gilbert C] Institute of Population Health, University of Liverpool, Liverpool, UK. [Emiralioglu N] Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey. [Torrent-Vernetta A] Unitat de Pneumologia Pediàtrica i Fibrosi Quística, Servei de Pediatria, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III (ISCIII), Madrid, Spain

Vall d'Hebron Barcelona Hospital Campus

Publication date

2023-03-09T10:55:07Z

2023-03-09T10:55:07Z

2023-03-31



Abstract

Interstitial and orphan lung disease


Enfermedad pulmonar intersticial y huérfana


Malaltia pulmonar intersticial i orfe


Childhood interstitial lung diseases (chILDs) are rare and heterogeneous diseases with significant morbidity and mortality. An accurate and quick aetiological diagnosis may contribute to better management and personalised treatment. On behalf of the European Respiratory Society Clinical Research Collaboration for chILD (ERS CRC chILD-EU), this review summarises the roles of the general paediatrician, paediatric pulmonologists and expert centres in the complex diagnostic workup. Each patient's aetiological chILD diagnosis must be reached without prolonged delays in a stepwise approach from medical history, signs, symptoms, clinical tests and imaging, to advanced genetic analysis and specialised procedures including bronchoalveolar lavage and biopsy, if necessary. Finally, as medical progress is fast, the need to revisit a diagnosis of “undefined chILD” is stressed.

Document Type

Article


Published version

Language

English

Publisher

European Respiratory Society

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European Respiratory Review;32(167)

https://doi.org/10.1183/16000617.0188-2022

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Attribution-NonCommercial 4.0 International

http://creativecommons.org/licenses/by-nc/4.0/

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