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Sci Transl Med. 2019 Aug 7;11(504). pii: eaav3505. doi: 10.1126/scitranslmed.aav3505.

Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies.

Author information

1
Wellman Center for Photomedicine, Massachusetts General Hospital, Boston, MA 02114, USA.
2
Harvard Medical School, Boston, MA 02114, USA.
3
Gregory Fleming James Cystic Fibrosis Research Center, Birmingham, AL 35294, USA.
4
Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
5
Nanyang Technological University, Singapore 639798, Singapore.
6
Biostatistics Center, Massachusetts General Hospital, Boston, MA 02114, USA.
7
Mucosal Immunology and Biology Research Center, Massachusetts General Hospital, Boston, MA 02114, USA.
8
Gregory Fleming James Cystic Fibrosis Research Center, Birmingham, AL 35294, USA. gtearney@partners.org srowe@peds.uab.edu.
9
Wellman Center for Photomedicine, Massachusetts General Hospital, Boston, MA 02114, USA. gtearney@partners.org srowe@peds.uab.edu.
10
Department of Pathology, Massachusetts General Hospital, Boston, MA 02114, USA.

Abstract

Cystic fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Although impairment of mucociliary clearance contributes to severe morbidity and mortality in people with CF, a clear understanding of the pathophysiology is lacking. This is, in part, due to the absence of clinical imaging techniques capable of capturing CFTR-dependent functional metrics at the cellular level. Here, we report the clinical translation of a 1-μm resolution micro-optical coherence tomography (μOCT) technology to quantitatively characterize the functional microanatomy of human upper airways. Using a minimally invasive intranasal imaging approach, we performed a clinical study on age- and sex-matched CF and control groups. We observed delayed mucociliary transport rate at the cellular level, depletion of periciliary liquid layer, and prevalent loss of ciliation in subjects with CF. Distinctive morphological differences in mucus and various forms of epithelial injury were also revealed by μOCT imaging and had prominent effects on the mucociliary transport apparatus. Elevated mucus reflectance intensity in CF, a proxy for viscosity in situ, had a dominant effect. These results demonstrate the utility of μOCT to determine epithelial function and monitor disease status of CF airways on a per-patient basis, with applicability for other diseases of mucus clearance.

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