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How abnormal is the normal? Clinical characteristics of CF patients with normal FEV1
  • +11
  • Malena Cohen-Cymberknoh,
  • ELAD BEN MEIR,
  • Silvia Gartner,
  • Joel Reiter,
  • angeles spangenberg,
  • Laura Garriga,
  • Iris Eisenstadt,
  • Tomer Israeli,
  • Reuven Tsabari,
  • David Shoseyov,
  • Alex Gileles-Hillel,
  • Oded Breuer,
  • Natalia Simanovski,
  • Eitan Kerem
Malena Cohen-Cymberknoh
Hadassah Medical Center
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ELAD BEN MEIR
Hadassah Medical Center
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Silvia Gartner
Vall d'Hebron Hospital Universitari
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Joel Reiter
Hadassah Medical Center
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angeles spangenberg
Vall d'Hebron Hospital Universitari
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Laura Garriga
Vall d'Hebron Hospital Universitari
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Iris Eisenstadt
Hadassah Medical Center
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Tomer Israeli
Hadassah Medical Center
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Reuven Tsabari
Hadassah Medical Center
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David Shoseyov
Hadassah Medical Center
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Alex Gileles-Hillel
Hadassah Medical Center
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Oded Breuer
Hadassah Medical Center
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Natalia Simanovski
Hadassah Medical Center
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Eitan Kerem
Hadassah Medical Center
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Abstract

Background Normal values (>80%) of Forced Expiratory Volume in one second (FEV1) in patients with Cystic fibrosis (CF) may lead to the interpretation that there is no lung disease. This study is a comprehensive analysis of lung involvement in CF patients having normal FEV1. Methods Patients were recruited from two CF Centers: Hadassah Medical Center, Jerusalem and Vall d’ Hebron Hospital, Barcelona. Lung disease was assessed by lung clearance index (LCI), chest CT-Brody Score, respiratory cultures, number of pulmonary exacerbations (PEx) and days of antibiotic treatment in the year prior to the assessment. Results Of the 247 patients, 89 (36%) had FEV1 ≥80% and were included in the study (mean age 17.6 range 4.25-49 years). Chronic P. aeruginosa infection was found in 21%, and 31% had at least one major PEx in the year prior to the study. Abnormally elevated LCI was found in 86% of patients, ranging between 7.52-18.97, and total Brody score (TBS) was abnormal in 92% (range 5.0-96.5). Patients with chronic P. aeruginosa had significantly higher LCI (p=0.01) and TBS (p=0.01) which were associated with more major PEx (p = 0.04 and p<0.001, respectively) and more days of intravenous (IV) antibiotic treatment in the preceding year (p=0.03 and p=0.002, respectively). Conclusions Most CF patients with normal FEV1 have already physiological and structural lung abnormalities which were associated with more PEx and IV antibiotic treatment.

Peer review status:ACCEPTED

05 Nov 2020Submitted to Pediatric Pulmonology
06 Nov 2020Submission Checks Completed
06 Nov 2020Assigned to Editor
07 Nov 2020Reviewer(s) Assigned
26 Nov 2020Review(s) Completed, Editorial Evaluation Pending
16 Dec 2020Editorial Decision: Revise Major
23 Feb 20211st Revision Received
24 Feb 2021Submission Checks Completed
24 Feb 2021Assigned to Editor
24 Feb 2021Reviewer(s) Assigned
25 Feb 2021Review(s) Completed, Editorial Evaluation Pending
25 Feb 2021Editorial Decision: Accept