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Hyperinflation is Associated with Increased Respiratory Rate and is a More Sensitive Measure of Cystic Fibrosis Lung Disease During Infancy Compared to Forced Expiratory Measures
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  • Heather Muston,
  • James Slaven,
  • Christina Tiller,
  • Charles Clem,
  • Thomas Ferkol,
  • Sarath Ranganathan,
  • Stephanie Davis,
  • Clement Ren
Heather Muston
Riley Hospital for Children at Indiana University Health

Corresponding Author:[email protected]

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James Slaven
Indiana University School of Medicine
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Christina Tiller
James Whitcomb Riley Hospital for Children
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Charles Clem
James Whitcomb Riley Hospital for Children
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Thomas Ferkol
Washington University in Saint Louis School of Medicine
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Sarath Ranganathan
Royal Children's Hospital, Melbourne
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Stephanie Davis
University of North Carolina at Chapel Hill School of Medicine
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Clement Ren
Indiana University System
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ABSTRACT Background: The goal of this study was to identify clinical features associated with abnormal infant pulmonary function tests (iPFTs), specifically functional residual capacity (FRC), in infants with cystic fibrosis (CF) diagnosed via newborn screen (NBS). We hypothesized that poor nutritional status in the first 6-12 months would be associated with increased FRC at 12-24 months. Methods: This study utilized a combination of retrospectively and prospectively collected data from ongoing research studies and iPFTs performed for clinical indications. Demographic and clinical features were obtained from the electronic medical record. Forced expiratory flows and volumes were obtained using the raised volume rapid thoracoabdominal technique (RVRTC) and FRC was measured via plethysmography. Results: A total of 45 CF NBS infants had iPFTs performed between 12-24 months. Mean forced vital capacity, forced expiratory volume in 0.5 second, and forced expiratory flows were all within normal limits. In contrast, the mean FRC z-score was 2.18 (95%CI=1.48, 2.88) and the mean respiratory rate (RR) z-score was 1.42 (95%CI=0.95, 1.89). There was no significant association between poor nutritional status and abnormal lung function. However, there was a significant association between higher RR and increased FRC, and a RR cutoff of 36 breaths/min resulted in 92% sensitivity to detect hyperinflation with 32% specificity. Conclusions: These results suggest that FRC is a more sensitive measure of early CF lung disease than RVRTC measurements and that RR may be a simple, non-invasive clinical marker to identify CF NBS infants with hyperinflation.
23 Oct 2020Submitted to Pediatric Pulmonology
26 Oct 2020Submission Checks Completed
26 Oct 2020Assigned to Editor
01 Nov 2020Reviewer(s) Assigned
27 Nov 2020Review(s) Completed, Editorial Evaluation Pending
01 Dec 2020Editorial Decision: Revise Major
06 Feb 20211st Revision Received
08 Feb 2021Submission Checks Completed
08 Feb 2021Assigned to Editor
08 Feb 2021Reviewer(s) Assigned
01 Mar 2021Review(s) Completed, Editorial Evaluation Pending
07 Mar 2021Editorial Decision: Revise Minor
12 Apr 20212nd Revision Received
12 Apr 2021Submission Checks Completed
12 Apr 2021Assigned to Editor
12 Apr 2021Reviewer(s) Assigned
02 May 2021Review(s) Completed, Editorial Evaluation Pending
03 May 2021Editorial Decision: Revise Minor
24 May 20213rd Revision Received
25 May 2021Submission Checks Completed
25 May 2021Assigned to Editor
25 May 2021Reviewer(s) Assigned
02 Jun 2021Review(s) Completed, Editorial Evaluation Pending
02 Jun 2021Editorial Decision: Accept
Sep 2021Published in Pediatric Pulmonology volume 56 issue 9 on pages 2854-2860. 10.1002/ppul.25538