RT Journal Article
SR Electronic
T1 Quantitative imaging of airway liquid absorption in cystic fibrosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 675
OP 684
DO 10.1183/09031936.00220513
VO 44
IS 3
A1 Landon W. Locke
A1 Michael M. Myerburg
A1 Matthew R. Markovetz
A1 Robert S. Parker
A1 Lawrence Weber
A1 Michael R. Czachowski
A1 Thomas J. Harding
A1 Stefanie L. Brown
A1 Joseph A. Nero
A1 Joseph M. Pilewski
A1 Timothy E. Corcoran
YR 2014
UL http://erj.ersjournals.com/content/44/3/675.abstract
AB New measures are needed to rapidly assess emerging treatments for cystic fibrosis (CF) lung disease. Using an imaging approach, we evaluated the absorptive clearance of the radiolabeled small molecule probe diethylene triamine penta-acetic acid (DTPA) as an in vivo indicator of changes in airway liquid absorption. DTPA absorption and mucociliary clearance rates were measured in 21 patients with CF (12 adults and nine children) and nine adult controls using nuclear imaging. The effect of hypertonic saline on DTPA absorption was also studied. In addition, in vitro studies were conducted to identify the determinants of transepithelial DTPA absorption. CF patients had significantly increased rates of DTPA absorption compared with control subjects but had similar mucociliary clearance rates. Treatment with hypertonic saline resulted in a decrease in DTPA absorption and an increase in mucociliary clearance in 11 out of 11 adult CF patients compared with treatment with isotonic saline. In vitro studies revealed that ∼50% of DTPA absorption can be attributed to transepithelial fluid transport. Apically applied mucus impedes liquid and DTPA absorption. However, mucus effects become negligible in the presence of an osmotic stimulus. Functional imaging of DTPA absorption provides a quantifiable marker of immediate response to treatments that promote airway surface liquid hydration. Functional imaging detects liquid absorption and mucociliary clearance in cystic fibrosis airways http://ow.ly/uPuX2