Abstract
The use of pulmonary function tests (PFTs) has been widely described in airway diseases like asthma and cystic fibrosis, but for children's interstitial lung disease (chILD), which encompasses a broad spectrum of pathologies, the usefulness of PFTs is still undetermined, despite widespread use in adult interstitial lung disease.
A literature review was initiated by the COST/Enter chILD working group aiming to describe published studies, to identify gaps in knowledge and to propose future research goals in regard to spirometry, whole-body plethysmography, infant and pre-school PFTs, measurement of diffusing capacity, multiple breath washout and cardiopulmonary exercise tests in chILD. The search revealed a limited number of papers published in the past three decades, of which the majority were descriptive and did not report pulmonary function as the main outcome.
PFTs may be useful in different stages of management of children with suspected or confirmed chILD, but the chILD spectrum is diverse and includes a heterogeneous patient group in all ages. Research studies in well-defined patient cohorts are needed to establish which PFT and outcomes are most relevant for diagnosis, evaluation of disease severity and course, and monitoring individual conditions both for improvement in clinical care and as end-points in future randomised controlled trials.
Abstract
Pulmonary function testing may be useful in different stages of management of children with suspected or confirmed chILD but this literature search revealed only a limited number of papers published in the last three decades. https://bit.ly/3cs0uZr
Footnotes
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Provenance: Submitted article, peer reviewed.
Conflict of interest: A.M. Ring has nothing to disclose.
Conflict of interest: J. Carlens has nothing to disclose.
Conflict of interest: A. Bush has nothing to disclose.
Conflict of interest: S. Castillo-Corullón has nothing to disclose.
Conflict of interest: S. Fasola has nothing to disclose.
Conflict of interest: M.P. Gaboli has nothing to disclose.
Conflict of interest: M. Griese reports grants and personal fees from Boehringer-Ingelheim, outside the submitted work.
Conflict of interest: V. Koucky reports non-financial support from Mylan Pharmaceuticals s.r.o., outside the submitted work.
Conflict of interest: S. La Grutta has nothing to disclose.
Conflict of interest: E. Lombardi reports personal fees from Boehringer-Ingelheim and grants from Restech, outside the submitted work.
Conflict of interest: M. Proesmans has nothing to disclose.
Conflict of interest: N. Schwerk has nothing to disclose.
Conflict of interest: D. Snijders reports grants from Cost Action Ca16126 ENTeR-chILD, during the conduct of the study.
Conflict of interest: K.G. Nielsen has nothing to disclose.
Conflict of interest: F. Buchvald has nothing to disclose.
Support statement: The COST Action 16125 working group” Enter chILD” and this work was funded by the EU COST action for research and innovation network. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received January 23, 2020.
- Accepted April 11, 2020.
- Copyright ©ERS 2020.
This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.