Journal article
Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis
CR Esther, MS Muhlebach, C Ehre, DB Hill, MC Wolfgang, M Kesimer, KA Ramsey, MR Markovetz, IC Garbarine, M Gregory Forest, I Seim, B Zorn, CB Morrison, MF Delion, WR Thelin, D Villalon, JR Sabater, L Turkovic, S Ranganathan, SM Stick Show all
Science Translational Medicine | AMER ASSOC ADVANCEMENT SCIENCE | Published : 2019
Abstract
Although destructive airway disease is evident in young children with cystic fibrosis (CF), little is known about the nature of the early CF lung environment triggering the disease. To elucidate early CF pulmonary pathophysiology, we performed mucus, inflammation, metabolomic, and microbiome analyses on bronchoalveolar lavage fluid (BALF) from 46 preschool children with CF enrolled in the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) program and 16 non-CF disease controls. Total airway mucins were elevated in CF compared to non-CF BALF irrespective of infection, and higher densities of mucus flakes containing mucin 5B and mucin 5AC were observed in samples fro..
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Awarded by National Institutes of Health
Funding Acknowledgements
C.R.E., L.T., M.S.M., and S.M.S. were supported by NIH/NHLBI R01-HL116228. C.R.E. was also supported by NC TraCS 50KR51009, NIH/NIEHS P30-ES10126, and NIH/NHLBI K23-HL089708. L.T. and S.M.S. were supported by NHMRC GNT1000896. S.M.S. was supported by a NHMRC Practitioner Fellowship GNT 111840. R.C.B. was supported by NIH/NHLBI UH3-HL123645, P01-HL108808, P30-DK065988, P01-HL110873, P50-HL107168, and R01-HL136961. M.G.F., D.B.H., and I.S. were supported by NSF-NIGMS DMS-1462992. K.A.R. and C.E. were supported by Cystic Fibrosis Foundation RAMSEY1610 and a NHMRC Early Career Fellowship (APP1088389) and EHRE 16XX0 and EHRE07XX0.