Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

Charles R. Esther; Marianne S. Muhlebach; Camille Ehre; David B. Hill; Matthew C. Wolfgang; Mehmet Kesimer; Kathryn A. Ramsey; Matthew R. Markovetz; Ian C. Garbarine; M. Gregory Forest; Ian Seim; Bryan Zorn; Cameron B. Morrison; Martial F. Delion; William R. Thelin; Diane Villalon; Juan R. Sabater; Lidija Turkovic; Sarath Ranganathan; Stephen M. Stick; Richard C. Boucher

doi:10.1126/scitranslmed.aav3488

Research ArticleCystic Fibrosis

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

Charles R. Esther Jr.¹,²,*,†,
View ORCID ProfileMarianne S. Muhlebach¹,²,†,
View ORCID ProfileCamille Ehre ¹,²,
David B. Hill ²,³,
Matthew C. Wolfgang²,
Mehmet Kesimer²,
View ORCID ProfileKathryn A. Ramsey ²,⁴,
View ORCID ProfileMatthew R. Markovetz²,
Ian C. Garbarine²,
M. Gregory Forest⁵,
View ORCID ProfileIan Seim⁵,
Bryan Zorn²,
View ORCID ProfileCameron B. Morrison²,
Martial F. Delion²,
View ORCID ProfileWilliam R. Thelin⁶,
Diane Villalon⁶,
Juan R. Sabater⁷,
Lidija Turkovic⁴,
Sarath Ranganathan⁸,
View ORCID ProfileStephen M. Stick ⁴,⁹,¹⁰,‡ and
Richard C. Boucher ²,‡

¹Division of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
²Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
³Department of Physics and Astronomy, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
⁴Telethon Kids Institute, University of Western Australia, Perth 6009, Australia.
⁵Departments of Mathematics, Biomedical Engineering, and Applied Physical Sciences, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
⁶Parion Sciences, Durham, NC 27713, USA.
⁷Department of Research, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.
⁸Murdoch Children’s Research Institute, University of Melbourne, Parkville 3052, Australia.
⁹Division of Paediatrics and Child Health, University of Western Australia, Perth 6009, Australia.
¹⁰Princess Margaret Hospital for Children, Perth 6009, Australia.

↵*Corresponding author. Email: charles_esther{at}med.unc.edu

↵† These authors contributed equally to this work.
↵‡ These authors contributed equally to this work as senior authors.

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Science Translational Medicine 03 Apr 2019:
Vol. 11, Issue 486, eaav3488
DOI: 10.1126/scitranslmed.aav3488

Charles R. Esther Jr.

1Division of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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ORCID record for Charles R. Esther Jr.
For correspondence: charles_esther@med.unc.edu

Marianne S. Muhlebach

1Division of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Camille Ehre

1Division of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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David B. Hill

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

3Department of Physics and Astronomy, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Matthew C. Wolfgang

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Mehmet Kesimer

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Kathryn A. Ramsey

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

4Telethon Kids Institute, University of Western Australia, Perth 6009, Australia.

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Matthew R. Markovetz

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Ian C. Garbarine

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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M. Gregory Forest

5Departments of Mathematics, Biomedical Engineering, and Applied Physical Sciences, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Ian Seim

5Departments of Mathematics, Biomedical Engineering, and Applied Physical Sciences, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Bryan Zorn

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Cameron B. Morrison

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Martial F. Delion

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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William R. Thelin

6Parion Sciences, Durham, NC 27713, USA.

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Diane Villalon

6Parion Sciences, Durham, NC 27713, USA.

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Juan R. Sabater

7Department of Research, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.

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Lidija Turkovic

4Telethon Kids Institute, University of Western Australia, Perth 6009, Australia.

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Sarath Ranganathan

8Murdoch Children’s Research Institute, University of Melbourne, Parkville 3052, Australia.

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Stephen M. Stick

4Telethon Kids Institute, University of Western Australia, Perth 6009, Australia.

9Division of Paediatrics and Child Health, University of Western Australia, Perth 6009, Australia.

10Princess Margaret Hospital for Children, Perth 6009, Australia.

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Richard C. Boucher

2Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

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Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

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