Detection of Unsuspected Small Airways Obstruction in Cystic Fibrosis

Sponsor

Gelb, Arthur F., M.D. (Other)

Overall Status

Recruiting

CT.gov ID

NCT03839992

Collaborator

Miller Children's & Women's Hospital Long Beach (Other), Stony Brook University (Other), The Hospital for Sick Children (Other)

100

Enrollment

Locations

Anticipated Duration (Months)

Patients Per Site

0.7

Patients Per Site Per Month

Study Details

Study Description

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.

Condition or Disease	Intervention/Treatment	Phase
Cystic Fibrosis in Children	Diagnostic Test: spirometry

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 Design

Study Type:

Observational

Anticipated Enrollment :

100 participants

Observational Model:

Cohort

Time Perspective:

Retrospective

Official Title:

The Measurement and Analysis of Maximal Expiratory Flow Volume Loops at Low Lung Volumes in Children With Cystic Fibrosis and Normal Routine Lung Function.

Actual Study Start Date :

Apr 25, 2018

Anticipated Primary Completion Date :

Apr 25, 2023

Anticipated Study Completion Date :

Nov 25, 2023

Outcome Measures

Primary Outcome Measures

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

Eligibility Criteria

Criteria

Ages Eligible for Study:

5 Years to 40 Years

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

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

Contacts and Locations

Locations

	Site	City	State	Country	Postal Code
1	Arthur F Gelb MD	Lakewood	California	United States	90712
2	Miller Children's Hospital and Long Beach Medical Center	Long Beach	California	United States	90806

Sponsors and Collaborators

Gelb, Arthur F., M.D.
Miller Children's & Women's Hospital Long Beach
Stony Brook University
The Hospital for Sick Children

Investigators

Principal Investigator: Arthur Gelb, MD, University of California, Los Angeles

Study Documents (Full-Text)

None provided.

More Information

Publications

None provided.

Responsible Party:

Gelb, Arthur F., M.D.

ClinicalTrials.gov Identifier:

NCT03839992

Other Study ID Numbers:

MHS #843-18

First Posted:

Feb 15, 2019

Last Update Posted:

Feb 9, 2021

Last Verified:

Feb 1, 2021

Individual Participant Data (IPD) Sharing Statement:

Yes

Plan to Share IPD:

Yes

Studies a U.S. FDA-regulated Drug Product:

Studies a U.S. FDA-regulated Device Product:

Yes

Keywords provided by Gelb, Arthur F., M.D.

abnormal FEF75

bronchiectasis

lung CT

Additional relevant MeSH terms:

Cystic Fibrosis

Fibrosis

Study Results

No Results Posted as of Feb 9, 2021