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  • Clinical Research Article
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Isolated abnormal FEF75% detects unsuspected bronchiolar obstruction in CF children

Pediatric Research volume 94, pages 1051–1056 (2023)Cite this article

Abstract

Background

Physiologic detection of bronchiolar obstruction in children with cystic fibrosis (CF) may be clinically unsuspected because of normal routine spirometry despite bronchiectasis on lung CT.

Methods

Children from two accredited CF facilities had spirometry obtained every 3 months when clinically stable. Pre-bronchodilator maximum expiratory flow volume curves were retrospectively analyzed over 16 years to detect an isolated abnormal FEF75%, despite normal routine spirometry.

Results

At Miller Children’s and Women’s Hospital (MCWH), an abnormal FEF75% was initially detected in 26 CF children at age 7.5 ± 4 (SD) years despite normal routine spirometry initially. FEF75% remained an isolated abnormality for 2.5 ± 1.5 years after it was initially detected in these 26 CF children. At Cohen Children’s Medical Center (CCMC), despite normal routine spirometry initially, abnormal FEF75% occurred in 13 children at age 11.7 ± 4.5 years, and abnormal FEF25–75% in 10 children at age 11.8 ± 5.3 years.

Conclusions

FEF75% was most sensitive spirometric test for diagnosing both early and isolated progressive bronchiolar obstruction. Data from CCMC in older children demonstrated the simultaneous detection of abnormal FEF75% and FEF25–75% values consistent with greater bronchiolar obstruction when serial spirometry was initiated at an older age.

Impact

  • There is very little published spirometric data regarding diagnosis of isolated small airways obstruction in CF children.

  • FEF75% can easily detect unsuspected small airways obstruction in CF children with normal routine spirometry and bronchiectasis on lung CT and optimize targeted modulatory therapies.

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Fig. 1: Patient distribution of CF children at MCWH and CCMC.
Fig. 2: Mean ± SD age (years) at first detection of abnormal expiratory airflow in 26 cases of CF at MCWH.
Fig. 3: Mean ± SD age (years) at first detection of abnormal expiratory airflow in 13 cases of CF at CCMC.

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Author information

Author notes

  1. Vicki K. Masson

    Present address: Stony Brook University Medical Center, Children’s Hospital, Stony Brook, NY, USA

Authors and Affiliations

  1. Pulmonary Division, Miller Children’s and Women’s Hospital, Long Beach Memorial Hospital, Long Beach, CA, USA

    Vicki K. Masson, Eliezer Nussbaum, Arthur F. Gelb, Inderpal Randhawa & Yaron Fireizen

  2. University of California, Irvine, School of Medicine, Irvine, CA, USA

    Vicki K. Masson

  3. Geffen School of Medicine, Pulmonary and Critical Care Division, Department of Medicine, UCLA Medical Center, Los Angeles, CA, USA

    Arthur F. Gelb & Donald P. Tashkin

  4. Lakewood Regional Medical Center, Lakewood, CA, USA

    Arthur F. Gelb

  5. Cardiovascular Research Institute, Pulmonary and Critical Care Division, Department of Medicine, University of California, San Francisco Medical Center, San Francisco, CA, USA

    Jay A. Nadel

  6. Cohen Children’s Medical Center, Northwell Health, New York City, NY, USA

    Joan K. De Celie-Germana & Danielle Madera

  7. University of Southern California, Los Angeles, CA, USA

    Dinithi Senanayake

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  1. Vicki K. Masson
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Contributions

E.N., A.F.G., and J.A.N. contributed to conception and design of the study. V.K.M., E.N., A.F.G., Y.F., J.K.D.C.-G., and D.M. contributed to acquisition of the data. V.K.M., E.N., A.F.G., Y.F., J.K.D.C.-G., D.M., and D.S. contributed to analysis and interpretation of data. V.K.M., E.N., A.F.G, and A.F.G. drafted the article, and all authors have revised it critically for important intellectual content. All authors have approved the final version to be published.

Corresponding author

Correspondence to Arthur F. Gelb.

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Competing interests

The authors declare no competing interests.

Ethics approval and consent to participate

This study was approved by Miller Children’s and Women’s Hospital, Long Beach Memorial Hospital, Long Beach, California, MHS IRB Number 843-18, and Cohen Children’s Medical Center, Northwell Health, Lake Success, New York, IRB Number 20-0581 and Clinical Trials.gov NCT 03839992. Parents/guardians of patients consented to inclusion of data in the study.

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Masson, V.K., Nussbaum, E., Gelb, A.F. et al. Isolated abnormal FEF75% detects unsuspected bronchiolar obstruction in CF children. Pediatr Res 94, 1051–1056 (2023). https://doi.org/10.1038/s41390-023-02532-2

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  • DOI: https://doi.org/10.1038/s41390-023-02532-2

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