| Literature DB >> 26135832 |
Andrew Bush1, Steve Cunningham2, Jacques de Blic3, Angelo Barbato4, Annick Clement5, Ralph Epaud6, Meike Hengst7, Nural Kiper8, Andrew G Nicholson9, Martin Wetzke10, Deborah Snijders4, Nicolaus Schwerk11, Matthias Griese12.
Abstract
Interstitial lung disease in children (chILD) is rare, and most centres will only see a few cases/year. There are numerous possible underlying diagnoses, with specific and non-specific treatment possibilities. The chILD-EU collaboration has brought together centres from across Europe to advance understanding of these considerations, and as part of this process, has created standard operating procedures and protocols for the investigation of chILD. Where established consensus documents exist already, for example, for the performance of bronchoalveolar lavage and processing of lung biopsies, these have been adopted. This manuscript reports our proposals for a staged investigation of chILD, starting from when the condition is suspected to defining the diagnosis, using pathways dependent on the clinical condition and the degree of illness of the child. These include the performance of genetic testing, echocardiography, high-resolution CT, bronchoscopy when appropriate and the definitive investigation of lung biopsy, in order to establish a precise diagnosis. Since no randomised controlled trials of treatment have ever been performed, we also report a Delphi consensus process to try to harmonise treatment protocols such as the use of intravenous and oral corticosteroids, and add-on therapies such as hydroxychloroquine and azithromycin. The aim is not to dictate to clinicians when a therapeutic trial should be performed, but to offer the possibility to collaborators of having a unified approach when a decision to treat has been made. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.Entities:
Keywords: ABCA3; GM-CSF Receptor; Hypersensitivity pneumonitis; Idiopathic pulmonary fibrosis; NEHI; PIG; Surfactant protein; TTF-1
Mesh:
Year: 2015 PMID: 26135832 DOI: 10.1136/thoraxjnl-2015-207349
Source DB: PubMed Journal: Thorax ISSN: 0040-6376 Impact factor: 9.139