Detection of Unsuspected Small Airways Obstruction in Cystic Fibrosis

Recruiting

• Conditions: Cystic Fibrosis in Children

• Registration Number: NCT03839992
• Lead Sponsor: Gelb, Arthur F., M.D.

Brief Summary

Goal is to physiologically detect unsuspected small airways obstruction in children and adults with treated heterozygous and homozygous cystic fibrosis. Unsuspected refers to normal routine pre bronchodilator spirometry including normal FEV1(L), FVC (L). and FEV1/FVC%. This is a retrospective study.

Detailed Description

The current study is based on current investigators previously published patho-physiologic and lung CT studies in small airways intrinsic obstruction, emphysema, and asthma. Current investigators have demonstrated that despite the presence of normal routine spirometry including normal FEV1(L), FVC (L), and FEV1/FVC% that unsuspected small airways obstruction, and emphysema can be detected. This has been achieved by presence of isolated abnormal expiratory airflow limitation at low lung volumes on the maximal expiratory flow volume curves. This includes abnormal expiratory airflow at 75% and 80% expired lung volume. Current investigators believe investigators will be able to detect unsuspected small airways intrinsic obstruction, and peripheral airway bronchiectasis proven by lung CT, in patients with cystic fibrosis despite presence of pre bronchodilator normal routine spirometry.

Study Timeline

• Study Start: 2018-04-25
• Study First Submitted: 2019-02-11
• Study First Submitted QC: 2019-02-11
• Study First Posted: 2019-02-15
• Status Verified: 2023-10
• Last Update Submitted: 2023-10-25
• Last Update Posted: 2023-10-26
• Primary Completion: 2027-04-25
• Study Completion: 2027-11-25

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 100

Inclusion Criteria

Heterozygote or homozygote patients with cystic fibrosis with normal routine pre bronchodilator spirometry including normal FEV1(L), FVC(L) and FEV1/FVC%.

Exclusion Criteria

Heterozygote or homozygote patients with cystic fibrosis with ABNORMAL routine pre bronchodilator spirometry

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Detection of Unsuspected Small Airway Obstruction in Cystic Fibrosis	5 years	Retrospective analysis of pre bronchodilator spirometry, and lung CT study to detect abnormal FEF75% in the presence of normal spirometry and FEF25-75. Detect isolated abnormal pre bronchodilator spirometry at 75%FVC

Trial Locations

Locations (2)

Arthur F Gelb MD; 🇺🇸 Lakewood, California, United States

Miller Children's Hospital and Long Beach Medical Center; 🇺🇸 Long Beach, California, United States