Evidence map›Paper›PMID 40642924›Full record

Observational studyPediatric pulmonology2025

Repeatability of Multiple Breath Washout in Pediatric Primary Ciliary Dyskinesia.

Wallace B Wee, Layan M Bashi, Renee Jensen, Jonathan H Rayment, Teresa To, Felix Ratjen, Giles Santyr, Sharon D Dell

Abstract readMulticenter StudyObservational Study
PubMed Full text at PMC Publisher
In one paragraph

Observational study in Pediatric pulmonology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Wallace B WeeRespiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.ORCID http://orcid.org/0000-0003-4291-3513
Layan M BashiRespiratory Medicine, Department of Pediatrics, BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Renee JensenTranslational Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.
Jonathan H RaymentRespiratory Medicine, Department of Pediatrics, BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.ORCID http://orcid.org/0000-0003-4955-8876
Teresa ToChild Health Evaluative Sciences, Hospital for Sick Children, Toronto, Ontario, Canada.
Felix RatjenRespiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.ORCID http://orcid.org/0000-0003-4057-6592
Giles SantyrTranslational Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.
Sharon D DellChild Health Evaluative Sciences, Hospital for Sick Children, Toronto, Ontario, Canada.ORCID https://orcid.org/0000-0003-2169-9407

Funding

Longitudinal Characterization of Respiratory Tract Exacerbations and Treatment Responses in Primary Ciliary DyskinesiaU54HL096458 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI OLIVIER, KENNETH N. · 2009 to 2023
$20.0M
NHLBI NIH HHS U54 HL096458The Genetic Disorders of Mucociliary Clearance Consortium (U54HL096458) is part of the National Center for Advancing Translational Sciences (NCATS) Rare Diseases Clinical Research Network (RDCRN) and supported by the RDCRN Data Management and Coordinating Center (DMCC) (U2CTR002818). RDCRN is an initiative of the Office of Rare Diseases Research (ORDR) funded through a collaboration between NCATS and the National Heart, Lung, and Blood Institute (NHLBI).
6 · The paper itself

Abstract

backgroundPrimary ciliary dyskinesia (PCD) is a motile ciliopathy characterized by abnormal mucociliary clearance and progressive lung disease. Spirometry is commonly used to monitor lung health and response to treatment, but it is known to be insensitive to early subclinical lung disease in PCD. Multiple breath washout is more sensitive than spirometry, but its repeatability in PCD has not been assessed.

objectivesTo evaluate the (i) same-day and (ii) 28-day repeatability of lung clearance index 2.5% (LCI) in PCD.

methodsParticipants > 6 years old with a confirmed PCD diagnosis were recruited from two Canadian PCD centers. Participants completed baseline lung function tests to measure their forced expiratory volume in 1-second, z-score (FEV1z), and LCI. Tests were repeated either on the same day or after 28 days. No clinical interventions were performed during the same-day repeat testing. Outpatient therapies were unchanged during 28-day repeat testing. Repeatability was assessed using intraclass correlation (ICC), and Bland-Altman plots (B&A).

resultsTwenty-three participants were enrolled (same-day: 16; 28-day: 13). The same-day and 28-day repeat testing ICC for FEV1z were 0.9 and 0.92, and LCI were 0.95 and 0.71, respectively. Baseline testing showed that most participants had abnormal LCI (18 of 29 tests), even in those with FEV1z in the normal range. FEV1z and LCI exhibited a weak inverse correlation.

conclusionsLCI is a repeatable and sensitive lung function measure in PCD patients, and may be a suitable outcome metric for clinical trials, particularly in patients with early subclinical lung disease.

Indexed as

Kartagener SyndromeAdolescentBreath TestsChildCiliary Motility DisordersFemaleForced Expiratory VolumeHumansMaleReproducibility of ResultsRespiratory Function TestsSpirometrymeasurement theorymultiple breath washoutpediatricprimary ciliary dyskinesiaspirometry

Identifiers

PMID40642924
PMCPMC12247151

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.