Clinical trial NCT00541190: Absorptive Clearance in the Cystic Fibrosis Airway
Official title: Absorptive Clearance in the Cystic Fibrosis Airway
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Cystic Fibrosis
Keywords: cystic fibrosis
mucociliary clearance
outcome measures
Lead sponsor: University of Pittsburgh
Sponsor class: OTHER
First posted: 2007-10-10
Enrollment: 21
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Primary purpose: BASIC_SCIENCE

Brief summary: The objective of this overall project is to develop a new aerosol-based technique for quantifying liquid absorption in the airways of subjects with cystic fibrosis(CF) that can be used to help develop new therapies. In CF, mutations in the CF gene result in dysfunction of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ion channel on the cells that line the airway epithelium, causing improper fluxes of ions such as sodium, chloride, and bicarbonate. The so called "low volume" hypothesis of CF pathogenesis contends that abnormal absorption of ions from the airways causes excessive absorption of liquid, resulting in an airway surface liquid layer that is dehydrated and difficult to clear. Here we are measuring the absorption rate of a radiolabeled small molecule (DTPA) from the lungs of cystic fibrosis patients and healthy controls. We hypothesize that the molecule will absorb more quickly in cystic fibrosis patients. Further studies will be performed to determine if DTPA absorption is related to liquid absorption in the airways.


Eligibility criteria: Inclusion Criteria:

* Cystic fibrosis subjects and healthy controls
* Age ≥ 18 years
* Diagnosis of cystic fibrosis as determined by sweat test or genotype and clinical symptoms (CF subjects)
* Clinically stable as determined by the investigator (pulmonologist)

Exclusion Criteria:

* Reactive airways disease
* Tobacco smokers (regular smoking within 6 months of enrollment)
* Positive urine pregnancy test on the day of testing
* FEV1p value of \< 50%
* SaO2 \< 92%, or if they require supplemental oxygen.
* Subjects performing other radioisotope studies within the last 2 weeks will be excluded.
* Healthy subjects with any history of lung disease will be excluded.
* Women currently breastfeeding
* Subjects not willing to stop treatments with inhaled hypertonic saline for 48 hours in advance of the study.

Primary outcomes:
measure: Absorptive Clearance Rate
timeFrame: single measurement
Secondary outcomes:
measure: Mucociliary Clearance Rate
timeFrame: single measurement
Other outcomes:
Locations:
city: Pittsburgh
country: United States
facility: University of Pittsburgh
state: Pennsylvania
Study officials:
affiliation: University of Pittsburgh
name: Timothy E Corcoran, Ph.D.
role: PRINCIPAL_INVESTIGATOR
References:
citation: Locke LW, Myerburg MM, Weiner DJ, Markovetz MR, Parker RS, Muthukrishnan A, Weber L, Czachowski MR, Lacy RT, Pilewski JM, Corcoran TE. Pseudomonas infection and mucociliary and absorptive clearance in the cystic fibrosis lung. Eur Respir J. 2016 May;47(5):1392-401. doi: 10.1183/13993003.01880-2015. Epub 2016 Mar 23.
citation: Corcoran TE, Thomas KM, Myerburg MM, Muthukrishnan A, Weber L, Frizzell R, Pilewski JM. Absorptive clearance of DTPA as an aerosol-based biomarker in the cystic fibrosis airway. Eur Respir J. 2010 Apr;35(4):781-6. doi: 10.1183/09031936.00059009. Epub 2009 Aug 28.