Absorptive Clearance in the Cystic Fibrosis Airway

University of Pittsburgh

Status: Completed

Conditions: Cystic Fibrosis

Treatments: Other: Technetium [Tc-99m] sulfur colloid and Indium [In-111] DTPA

Study type: Interventional

Funder types: Other

Identifiers

NCT00541190

PRO07060240

NIH K25 HL081533-02

Details and patient eligibility

About

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.

Enrollment: 21 patients

Sex: All

Ages: 18+ years old

Volunteers: Accepts Healthy Volunteers

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.

Trial design

Primary purpose: Basic Science

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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21 participants in 2 patient groups

cystic fibrosis

Experimental group

Description:

Cystic fibrosis patients

Treatment:

Other: Technetium [Tc-99m] sulfur colloid and Indium [In-111] DTPA

healthy controls

Experimental group

Description:

Healthy control subjects

Treatment:

Other: Technetium [Tc-99m] sulfur colloid and Indium [In-111] DTPA