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Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis.
Hardaker, K M; Panda, H; Hulme, K; Wong, A; Coward, E; Cooper, P; Fitzgerald, D A; Pandit, C; Towns, S; Selvadurai, H; Robinson, P D.
Affiliation
  • Hardaker KM; Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
  • Panda H; Sydney Medical School, University of Sydney, Australia.
  • Hulme K; Sydney Medical School, University of Sydney, Australia.
  • Wong A; Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
  • Coward E; Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
  • Cooper P; Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
  • Fitzgerald DA; 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.
  • Pandit C; Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
  • Towns S; Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
  • Selvadurai H; 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.
  • Robinson PD; 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.
J Cyst Fibros ; 18(5): 721-727, 2019 09.
Article in En | MEDLINE | ID: mdl-30827846
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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Respiratory Function Tests / Spirometry / Breath Tests / Mucociliary Clearance / Cystic Fibrosis Type of study: Diagnostic_studies / Etiology_studies / Prognostic_studies / Risk_factors_studies / Screening_studies Limits: Child / Child, preschool / Female / Humans / Male Language: En Journal: J Cyst Fibros Year: 2019 Type: Article Affiliation country: Australia
Fulltext
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Respiratory Function Tests / Spirometry / Breath Tests / Mucociliary Clearance / Cystic Fibrosis Type of study: Diagnostic_studies / Etiology_studies / Prognostic_studies / Risk_factors_studies / Screening_studies Limits: Child / Child, preschool / Female / Humans / Male Language: En Journal: J Cyst Fibros Year: 2019 Type: Article Affiliation country: Australia