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Mucus Removal Is Impaired in Children with Cystic Fibrosis Who Have Been Infected by Pseudomonas aeruginosa - 19/03/14

Doi : 10.1016/j.jpeds.2013.11.031 
Beth L. Laube, PhD , Gail Sharpless, BS, Jane Benson, MD, Kathryn A. Carson, ScM, Peter J. Mogayzel, MD, PhD
 The Johns Hopkins Medical Institutions, Baltimore, MD 

Reprint requests: Beth L. Laube, PhD, The Johns Hopkins Medical Institutions, The David M. Rubenstein Building, 200 North Wolfe St, Ste 3015, Baltimore, MD 21287-2533.

Abstract

Objective

To determine if mucus removal is impaired in children with cystic fibrosis (CF) who have been recently infected with Pseudomonas aeruginosa.

Study design

We compared mucociliary clearance (MCC), cough clearance (CC), lung morphology, and forced expiratory volume in 1 second (FEV1) in 7- to 14-year-old children with CF and mild lung disease (FEV1 ≥ 80%). Children were either P aeruginosa negative (n = 8), or P aeruginosa positive (P aeruginosa obtained from at least 1 airway culture in the preceding 18 months) (n = 10). MCC and CC were quantified from gamma camera imaging of the right lung immediately after inhalation of 99mtechnetium sulfur-colloid (time 0), over the next 60 minutes (average percent clearance over the first 60 minutes [AveMCC60]), 60-90 minutes (average percent clearance between 70 and 90 minutes [AveMCC/CC90]), and after 24 hours (percent clearance after 24 hours [MCC24hrs]). Children coughed 30 times between 60 and 90 minutes. Lung morphology was assessed by high resolution computed tomography (HRCT) scores of both lungs (total score) and of the right lung, using the Brody scale. Percent AveMCC60, AveMCC/CC90, MCC24hrs, FEV1, and HRCT scores were compared across the 2 groups using unpaired t tests. Associations were assessed using Spearman correlation.

Results

There were no differences between the 2 groups in AveMCC60, MCC24hrs, mean HRCT total scores, right lung HRCT scores, or mean FEV1. AveMCC/CC90 was significantly decreased in children with P aeruginosa compared with those without (16.2% ± 11.0% vs 28.6% ± 7.8%, respectively; P = .016). There was a significant negative correlation of AveMCC60 and AveMCC/CC90 with total lung HRCT score (all P < .05) but not with FEV1.

Conclusions

Infection with P aeruginosa is associated with a significant slowing of MCC/CC in children with mild CF and may be a more sensitive indicator of the effects of P aeruginosa than measurements of FEV1.

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Keyword : AveMCC60, AveMCCCC90, CC, CF, CT, FEV1, FVC, HRCT, MCC, MCC24Hrs, O:I


Plan


 Funded by Gilead Sciences, Inc, which had initial input in study design, but no involvement in the collection, analysis or interpretation of the data, writing of the report, or the decision to submit the manuscript for publication. Supported by Johns Hopkins Institute for Clinical and Translational Research (ICTR), which is funded in part by the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health (NIH), and NIH Roadmap for Medical Research (UL1 TR 000424-06). Its contents are solely the responsibility of the authors and do not necessarily represent the official view of the Johns Hopkins ICTR, NCATS, or NIH. The authors declare no conflicts of interest.


© 2014  Mosby, Inc. Tous droits réservés.
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Vol 164 - N° 4

P. 839-845 - avril 2014 Retour au numéro
Article précédent Article précédent
  • Predictors and Outcome of Low Initial Forced Expiratory Volume in 1 Second Measurement in Children with Cystic Fibrosis
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