Characterizing mucociliary clearance in young children with cystic fibrosis.


Journal

Pediatric research
ISSN: 1530-0447
Titre abrégé: Pediatr Res
Pays: United States
ID NLM: 0100714

Informations de publication

Date de publication:
02 2022
Historique:
received: 14 08 2020
accepted: 18 02 2021
revised: 10 02 2021
pubmed: 24 3 2021
medline: 12 4 2022
entrez: 23 3 2021
Statut: ppublish

Résumé

This research characterized mucociliary clearance (MCC) in young children with cystic fibrosis (CF). Fourteen children (5-7 years old) with CF underwent: two baseline MCC measurements (Visits 1 and 2); one MCC measurement approximately 1 year later (Visit 3); and measurements of lung clearance index (LCI), a measure of ventilation inhomogeneity. Median (range) percent MCC through 60 min (MCC60) was similar on Visits 1 and 2 with 11.0 (0.9-33.7) and 12.8 (2.7-26.8), respectively (p = 0.95), and reproducible (Spearman Rho = 0.69; p = 0.007). Mucociliary clearance did not change significantly over 1 year with median percent MCC60 on Visit 3 [12.8 (3.7-17.6)] similar to Visit 2 (p = 0.58). Lower percent MCC60 on Visit 3 was significantly associated with higher LCI scores on Visit 3 (N = 14; Spearman Rho = -0.56; p = 0.04). Tests of MCC were reproducible and reliable over a 2-week period and stable over a 1-year period in 5-7-year-old children with CF. Lower MCC values were associated with increased ventilation inhomogeneity. These results suggest that measurements of MCC could be used in short-term clinical trials of interventions designed to modulate MCC and as a new, non-invasive test to evaluate early lung pathology in children with CF. This is the first study to characterize mucociliary clearance (MCC) in children with cystic fibrosis (CF) who were 5-7 years old. Measurements of mucociliary clearance were reproducible and reliable over a 2-week period and stable over a 1-year period. Variability in MCC between children was associated with differences in ventilation homogeneity, such that children with lower MCC values had increased ventilation inhomogeneity. These results suggest that measurements of MCC could be used in short-term clinical trials of interventions designed to modulate MCC and as a new, non-invasive test to evaluate early lung pathology in children with CF.

Sections du résumé

BACKGROUND
This research characterized mucociliary clearance (MCC) in young children with cystic fibrosis (CF).
METHODS
Fourteen children (5-7 years old) with CF underwent: two baseline MCC measurements (Visits 1 and 2); one MCC measurement approximately 1 year later (Visit 3); and measurements of lung clearance index (LCI), a measure of ventilation inhomogeneity.
RESULTS
Median (range) percent MCC through 60 min (MCC60) was similar on Visits 1 and 2 with 11.0 (0.9-33.7) and 12.8 (2.7-26.8), respectively (p = 0.95), and reproducible (Spearman Rho = 0.69; p = 0.007). Mucociliary clearance did not change significantly over 1 year with median percent MCC60 on Visit 3 [12.8 (3.7-17.6)] similar to Visit 2 (p = 0.58). Lower percent MCC60 on Visit 3 was significantly associated with higher LCI scores on Visit 3 (N = 14; Spearman Rho = -0.56; p = 0.04).
CONCLUSIONS
Tests of MCC were reproducible and reliable over a 2-week period and stable over a 1-year period in 5-7-year-old children with CF. Lower MCC values were associated with increased ventilation inhomogeneity. These results suggest that measurements of MCC could be used in short-term clinical trials of interventions designed to modulate MCC and as a new, non-invasive test to evaluate early lung pathology in children with CF.
IMPACT
This is the first study to characterize mucociliary clearance (MCC) in children with cystic fibrosis (CF) who were 5-7 years old. Measurements of mucociliary clearance were reproducible and reliable over a 2-week period and stable over a 1-year period. Variability in MCC between children was associated with differences in ventilation homogeneity, such that children with lower MCC values had increased ventilation inhomogeneity. These results suggest that measurements of MCC could be used in short-term clinical trials of interventions designed to modulate MCC and as a new, non-invasive test to evaluate early lung pathology in children with CF.

Identifiants

pubmed: 33753897
doi: 10.1038/s41390-021-01453-2
pii: 10.1038/s41390-021-01453-2
pmc: PMC8455702
mid: NIHMS1676943
doi:

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

612-620

Subventions

Organisme : NCATS NIH HHS
ID : UL1 TR001079
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL128475
Pays : United States
Organisme : NIEHS NIH HHS
ID : R21 ES023384
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL080396
Pays : United States
Organisme : NHLBI NIH HHS
ID : R35 HL140039
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL129925
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL130938
Pays : United States

Informations de copyright

© 2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.

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Auteurs

Beth L Laube (BL)

Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA. blaube@jhmi.edu.

Kathryn A Carson (KA)

Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA.

Christopher M Evans (CM)

Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA.

Melis A Aksit (MA)

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Joseph M Collaco (JM)

Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Vanessa L Richardson (VL)

Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA.

Gail Sharpless (G)

Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Pamela L Zeitlin (PL)

Department of Pediatrics, National Jewish Health, Denver, CO, USA.

Garry R Cutting (GR)

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Peter J Mogayzel (PJ)

Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

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