Clinical trial NCT03839992: Detection of Unsuspected Small Airways Obstruction in Cystic Fibrosis
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.
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Cystic Fibrosis in Children
Keywords: abnormal FEF75
bronchiectasis
lung CT
Lead sponsor: Gelb, Arthur F., M.D.
Sponsor class: INDIV
Collaborators: name: Miller Children's & Women's Hospital Long Beach
name: Stony Brook University
name: The Hospital for Sick Children
First posted: 2019-02-15
Enrollment: 100
Sex: ALL
Minimum age (years): 5
Maximum age (years): 40
Standard ages: CHILD
ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: RETROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Review pulmonary function and radiologic studies in retrospective records of heterozygote or homozygote patients with cystic fibrosis

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.

Eligibility criteria: 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

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Primary outcomes:
measure: Detection of Unsuspected Small Airway Obstruction in Cystic Fibrosis
timeFrame: 5 years
Secondary outcomes:
Other outcomes:
Locations:
city: Lakewood
country: United States
facility: Arthur F Gelb MD
state: California
city: Long Beach
country: United States
facility: Miller Children's Hospital and Long Beach Medical Center
state: California
Study officials:
affiliation: University of California, Los Angeles
name: Arthur Gelb, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Mead J. The lung's "quiet zone". N Engl J Med. 1970 Jun 4;282(23):1318-9. doi: 10.1056/NEJM197006042822311. No abstract available.
citation: Gelb AF, Gold WM, Wright RR, Bruch HR, Nadel JA. Physiologic diagnosis of subclinical emphysema. Am Rev Respir Dis. 1973 Jan;107(1):50-63. doi: 10.1164/arrd.1973.107.1.50. No abstract available.
citation: Gelb AF, Zamel N. Simplified diagnosis of small-airway obstruction. N Engl J Med. 1973 Feb 22;288(8):395-8. doi: 10.1056/NEJM197302222880805. No abstract available.
citation: Hogg JC, Pare PD, Hackett TL. The Contribution of Small Airway Obstruction to the Pathogenesis of Chronic Obstructive Pulmonary Disease. Physiol Rev. 2017 Apr;97(2):529-552. doi: 10.1152/physrev.00025.2015.
citation: Lukic KZ, Coates AL. Does the FEF25-75 or the FEF75 have any value in assessing lung disease in children with cystic fibrosis or asthma? Pediatr Pulmonol. 2015 Sep;50(9):863-8. doi: 10.1002/ppul.23234. Epub 2015 Jun 16.