Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis

Gustafsson, P M; De Jong, P A; Tiddens, H A W M; Lindblad, A

doi:10.1136/thx.2007.077784

Cited by 364Cystic Fibrosis Research AdvancesChronic Obstructive Pulmonary Disease (COPD) ResearchNeonatal Respiratory Health Research

Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis

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Gustafsson, P M, De Jong, P A, Tiddens, H A W M, Lindblad, A·2007·Thorax

DOI 10.1136/thx.2007.077784

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Abstract

BACKGROUND: A sensitive and valid non-invasive marker of early cystic fibrosis (CF) lung disease is sought. The lung clearance index (LCI) from multiple-breath washout (MBW) is known to detect abnormal lung function more readily than spirometry in children and teenagers with CF, but its relationship to structural lung abnormalities is unknown. A study was undertaken to determine the agreements between LCI and spirometry, respectively, with structural lung disease as measured by high-resolution computed tomography (HRCT) in children and teenagers with CF. METHODS: A retrospective study was performed in 44 consecutive patients with CF aged 5-19 years (mean 12 years). At an annual check-up inspiratory and expiratory HRCT scans, LCI and spirometric parameters (forced expiratory volume in 1 s (FEV1) and maximal expiratory flow when 75% of forced vital capacity was expired (FEF75)) were recorded. Abnormal structure was defined as a composite HRCT score of >5%, the presence of bronchiectasis or air trapping >30%. Abnormal lung function was defined as LCI above the predicted mean +1.96 residual standard deviations (RSD), or FEV1 or FEF75 below the predicted mean -1.96 RSD. Sensitivity/specificity assessments and correlation analyses were done. RESULTS: The sensitivity to detect abnormal lung structure was 85-94% for LCI, 19-26% for FEV1 and 62-75% for FEF75. Specificity was 43-65% for LCI, 89-100% for FEV1 and 75-88% for FEF75. LCI correlated better with HRCT scores (Rs +0.85) than FEV1 (-0.62) or FEF75 (-0.66). CONCLUSIONS: LCI is a more sensitive indicator than FEV1 or FEF75 for detecting structural lung disease in CF, and a normal LCI almost excludes HRCT abnormalities. The finding of an abnormal LCI in some patients with normal HRCT scans suggests that LCI may be even more sensitive than HRCT scanning for detecting lung involvement in CF.

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Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?
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Xenon-129 MRI detects ventilation deficits in paediatric stem cell transplant patients unable to perform spirometry
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Feasibility of lung clearance index in a clinical setting in pre-school children
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Long-term safety and efficacy of lumacaftor–ivacaftor therapy in children aged 6–11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study
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Tracking Lung Clearance Index and chest CT in mild cystic fibrosis lung disease over a period of three years
Fuchs, S. I., Gappa, M., Eder, J., Unsinn, K. M., Steinkamp, G., & Ellemunter, H. (2014) · Respiratory Medicine · doi:10.1016/j.rmed.2014.03.011 · Cited by 43 · see citing works
Multiple-breath washout and pulmonary function tests versus high-resolution computed tomography scores in adults with cystic fibrosis
Cherniak, A. V., Константиновна, Н. Ж., Krasovskiy, S., Михайличенко, К. Ю., & Nekludova, G. V. (2020) · European Respiratory Journal · doi:10.1183/13993003.congress-2020.4341

Authors: Gustafsson, P. M., De Jong, P. A., Tiddens, H. A. W. M., & Lindblad, A.
Affiliations: Drottning Silvias barn- och ungdomssjukhus (SE)
Erasmus MC - Sophia Children’s Hospital (NL)
Sahlgrenska University Hospital (SE)
Corresponding author: Gustafsson, P M
ORCID: Gustafsson, P M
Tiddens, H A W M
Lindblad, A
Venue: Thorax
Publisher: BMJ
Published: 2007-09-17
Volume: 63
Issue: 2
Pages: 129-134
ISSN: 0040-6376 (ISSN-L)
DOI: 10.1136/thx.2007.077784
OpenAlex: W2052470792
Cited by: 364
References: 55
Citations by year: 2026: 6 · 2025: 8 · 2024: 14 · 2023: 22 · 2022: 19 · 2021: 30 · 2020: 25 · 2019: 23 · 2018: 13 · 2017: 22 · 2016: 22 · 2015: 30 · 2014: 18 · 2013: 21 · 2012: 21
FWCI: 11.81
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: Thorax (publishedVersion)
PubMed (publishedVersion)
EUR Research Repository (Erasmus University Rotterdam) (publishedVersion)
EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
HighWire Press Open Archive (submittedVersion)
Funders: British Columbia Lung Association
Topics: Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Neonatal Respiratory Health Research
Keywords: cystic fibrosis, lung clearance index, multiple breath washout, spirometry, structural lung disease, high-resolution computed tomography, bronchiectasis, air trapping, FEV1, FEF75, children and adolescents
MeSH terms: Adolescent, Adult, Breath Tests, Bronchiectasis, Child, Child, Preschool, Cross-Sectional Studies, Cystic Fibrosis, Female, Forced Expiratory Volume, Humans, Lung, Male, Respiratory Function Tests, Retrospective Studies, Sensitivity and Specificity, Spirometry, Tomography, X-Ray Computed, Ultrasonography

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Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis

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