Literature DB >> 29035122

Homogeneity of Aerosol Deposition and Mucociliary Clearance are Improved Following Ivacaftor Treatment in Cystic Fibrosis.

William D Bennett1, Kirby L Zeman1, Beth L Laube2, Jihong Wu1, Gail Sharpless2, Peter J Mogayzel2, Scott H Donaldson1.   

Abstract

BACKGROUND: Using planar gamma scintigraphy of inhaled radioaerosols, we have developed new analytical methods for assessing homogeneity of aerosol deposition and time-dependent particle clearance on a pixel-by-pixel basis, and applied them to a therapeutic cystic fibrosis (CF) study.
METHODS: At baseline and 1 month after beginning treatment with ivacaftor, a cystic fibrosis transmembrane regulator modulator for CF patients with at least one copy of the G551D mutation (n = 13), initial deposition and subsequent mucociliary clearance (MCC) of radiolabeled particles (99mTechnetium-sulfur colloid, 5 μm mass median aerodynamic diameter) inhaled under controlled breathing conditions were measured.
RESULTS: Improved homogeneity of deposition, that is, decreased areas of higher and lower particle deposition in the lungs, was observed following ivacaftor treatment. The mean number ratio (NR) of pixels with higher deposition, relative to lung size, decreased from 0.14 to 0.09 (p = 0.003) and mean NR of colder pixels decreased from 0.23 to 0.19 (p = 0.004). Particle clearance was also improved following treatment, with mean MCC through 60 minutes equal to 12% versus 24%, without and with treatment, respectively (p = 0.010). Pixel-level analysis of MCC showed that (1) the fraction of pixels clearing >30% at 60 minutes was increased from 0.13 to 0.32 (p = 0.007); and (2) the fraction of pixels clearing <5% at 60 minutes was decreased from 0.54 to 0.37 (p = 0.014), indicating an overall recruitment of more fast-clearing lung regions with ivacaftor treatment.
CONCLUSION: These detailed pixel analyses of deposition and clearance homogeneity may supplement traditional methods that use large regions of interest for assessing efficacy and mechanisms of therapeutic intervention in patients with airways disease.

Entities:  

Keywords:  gamma scintigraphy; mucociliary clearance; particle deposition

Mesh:

Substances:

Year:  2017        PMID: 29035122      PMCID: PMC6067688          DOI: 10.1089/jamp.2017.1388

Source DB:  PubMed          Journal:  J Aerosol Med Pulm Drug Deliv        ISSN: 1941-2711            Impact factor:   2.849


  7 in total

1.  Comparing lung regions of interest in gamma scintigraphy for assessing inhaled therapeutic aerosol deposition.

Authors:  Martyn F Biddiscombe; Sally N Meah; S Richard Underwood; Omar S Usmani
Journal:  J Aerosol Med Pulm Drug Deliv       Date:  2011-03-31       Impact factor: 2.849

2.  Heterogeneity of Particle Deposition by Pixel Analysis of 2D Gamma Scintigraphy Images.

Authors:  William D Bennett; Miao Xie; Kirby Zeman; Harry Hurd; Scott Donaldson
Journal:  J Aerosol Med Pulm Drug Deliv       Date:  2014-11-13       Impact factor: 2.849

3.  Multisite comparison of mucociliary and cough clearance measures using standardized methods.

Authors:  William D Bennett; Beth L Laube; Timothy Corcoran; Kirby Zeman; Gail Sharpless; Kristina Thomas; Jihong Wu; Peter J Mogayzel; Joseph Pilewski; Scott Donaldson
Journal:  J Aerosol Med Pulm Drug Deliv       Date:  2013-03-21       Impact factor: 2.849

4.  The characterization of radioaerosol deposition in the healthy lung by histogram distribution analysis.

Authors:  C S Garrard; T R Gerrity; J F Schreiner; D B Yeates
Journal:  Chest       Date:  1981-12       Impact factor: 9.410

5.  Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

Authors:  Steven M Rowe; Sonya L Heltshe; Tanja Gonska; Scott H Donaldson; Drucy Borowitz; Daniel Gelfond; Scott D Sagel; Umer Khan; Nicole Mayer-Hamblett; Jill M Van Dalfsen; Elizabeth Joseloff; Bonnie W Ramsey
Journal:  Am J Respir Crit Care Med       Date:  2014-07-15       Impact factor: 21.405

6.  Comparison of 133 xenon ventilation equilibrium scan (XV) and 99m technetium transmission (TT) scan for use in regional lung analysis by 2D gamma scintigraphy in healthy and cystic fibrosis lungs.

Authors:  Kirby L Zeman; Jihong Wu; Scott H Donaldson; William D Bennett
Journal:  J Aerosol Med Pulm Drug Deliv       Date:  2013-02-19       Impact factor: 2.849

7.  Standardization of techniques for using planar (2D) imaging for aerosol deposition assessment of orally inhaled products.

Authors:  Stephen Newman; William D Bennett; Martyn Biddiscombe; Sunalene G Devadason; Myrna B Dolovich; John Fleming; Sabine Haeussermann; Claudius Kietzig; Philip J Kuehl; Beth L Laube; Knut Sommerer; Glyn Taylor; Omar S Usmani; Kirby L Zeman
Journal:  J Aerosol Med Pulm Drug Deliv       Date:  2012-12       Impact factor: 2.849

  7 in total
  5 in total

1.  Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR.

Authors:  Scott H Donaldson; Beth L Laube; Timothy E Corcoran; Pradeep Bhambhvani; Kirby Zeman; Agathe Ceppe; Pamela L Zeitlin; Peter J Mogayzel; Michael Boyle; Landon W Locke; Michael M Myerburg; Joseph M Pilewski; Brian Flanagan; Steven M Rowe; William D Bennett
Journal:  JCI Insight       Date:  2018-12-20

2.  A Flow Cytometric Method for Isolating Cystic Fibrosis Airway Macrophages from Expectorated Sputum.

Authors:  Katherine B Hisert; W Conrad Liles; Anne M Manicone
Journal:  Am J Respir Cell Mol Biol       Date:  2019-07       Impact factor: 6.914

3.  Transduction of Surface and Basal Cells in Rhesus Macaque Lung Following Repeat Dosing with AAV1CFTR.

Authors:  William B Guggino; Murali K Yanda; Cristina V Cebotaru; Liudmila Cebotaru
Journal:  Hum Gene Ther       Date:  2020-09       Impact factor: 5.695

Review 4.  Mucus, mucins, and cystic fibrosis.

Authors:  Cameron Bradley Morrison; Matthew Raymond Markovetz; Camille Ehre
Journal:  Pediatr Pulmonol       Date:  2019-11

Review 5.  Aerosolised micro and nanoparticle: formulation and delivery method for lung imaging.

Authors:  Miftakul Munir; Herlan Setiawan; Rohadi Awaludin; Vicky L Kett
Journal:  Clin Transl Imaging       Date:  2022-09-29
  5 in total

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