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Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Updated: Aug 7, 2025

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Isolated abnormal FEF75% detects unsuspected bronchiolar obstruction in CF children.

Vicki K Masson1,2,3, Eliezer Nussbaum1, Arthur F Gelb4,5,6

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

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Early detection of small airways obstruction in children with cystic fibrosis (CF) is possible using FEF75% spirometry, even with normal routine tests. This finding aids in optimizing therapies for CF patients with suspected lung disease.

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Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Cystic Fibrosis Research

Background:

  • Physiologic detection of bronchiolar obstruction in children with cystic fibrosis (CF) can be challenging due to normal spirometry despite CT-confirmed bronchiectasis.
  • Small airways obstruction in CF may go clinically unsuspected.

Purpose of the Study:

  • To investigate the utility of specific spirometric measures, particularly FEF75%, in detecting early and isolated small airways obstruction in children with CF.
  • To assess if FEF75% can identify subclinical airway disease in CF patients with normal routine spirometry.

Main Methods:

  • Retrospective analysis of spirometry data from children at two CF facilities over 16 years.
  • Focus on pre-bronchodilator maximum expiratory flow volume curves to identify abnormal FEF75% despite normal routine spirometry.
  • Data collected every 3 months during clinically stable periods.

Main Results:

  • An abnormal FEF75% was detected in 26 CF children at a mean age of 7.5 years, persisting as an isolated abnormality for 2.5 years.
  • At another center, abnormal FEF75% occurred in 13 children (mean age 11.7 years), and abnormal FEF25-75% in 10 (mean age 11.8 years), despite initially normal spirometry.
  • FEF75% proved to be the most sensitive test for diagnosing early and isolated progressive bronchiolar obstruction.

Conclusions:

  • FEF75% is a sensitive spirometric marker for detecting unsuspected small airways obstruction in children with CF.
  • This test can identify subclinical disease in CF patients with normal routine spirometry and bronchiectasis, allowing for optimized targeted therapies.