Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis

K M Hardaker; H Panda; K Hulme; A Wong; E Coward; P Cooper; D A Fitzgerald; C Pandit; S Towns; H Selvadurai; P D Robinson

doi:10.1016/j.jcf.2019.02.007

Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis

J Cyst Fibros. 2019 Sep;18(5):721-727. doi: 10.1016/j.jcf.2019.02.007. Epub 2019 Mar 1.

Authors

K M Hardaker¹, H Panda², K Hulme², A Wong¹, E Coward¹, P Cooper¹, D A Fitzgerald³, C Pandit¹, S Towns¹, H Selvadurai³, P D Robinson⁴

Affiliations

¹ Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
² Sydney Medical School, University of Sydney, Australia.
³ Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia; Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Australia.
⁴ Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia; Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Australia. Electronic address: paul.robinson1@health.nsw.gov.au.

PMID: 30827846
DOI: 10.1016/j.jcf.2019.02.007

Abstract

Background: Clinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear.

Methods: Initial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2-6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0).

Results: LCI was abnormal in 56% (28/50), with mean (SD) LCI 8.61(1.85) at age 4.71(1.3) years. Abnormal LCI was associated with higher dornase alfa use, previous positive bacterial cultures and pF508.del homozygous genotype. Later spirometry (n = 44; mean (SD) 2.3(0.5) years after MBW) demonstrated that abnormal initial preschool LCI was a strong predictor of lower later spirometry outcomes.

Conclusion: Abnormal preschool LCI was associated with concurrent measures of clinical status and later spirometry deficits, suggesting early prognostic utility of MBW testing in this age range.

Keywords: Cystic fibrosis; Multiple breath washout; Preschool; Spirometry.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Aftercare / methods
Breath Tests / methods*
Child
Child, Preschool
Cystic Fibrosis* / diagnosis
Cystic Fibrosis* / physiopathology
Early Diagnosis
Equipment Design
Female
Health Status Indicators
Humans
Male
Mucociliary Clearance*
Outcome Assessment, Health Care
Predictive Value of Tests
Prognosis
Respiratory Function Tests* / methods
Respiratory Function Tests* / statistics & numerical data
Spirometry* / methods
Spirometry* / statistics & numerical data