The Cystic Fibrosis Pulmonary Microbiome

Contact Information: 

Ryan Thomas, MD:
 @MSUPedsPulm

Robert Quinn, PhD:
 @Quinn_Labs

ATS Peds:
 @ATSPeds

Selected Relevant References: 

Lindsay Caverly: 

• Caverly LJ et al. Measures of Cystic Fibrosis Airway Microbiota During Periods of Clinical Stability. Ann Am Thorac Soc. 2019 Dec;16(12):1534-1542.
• Caverly LJ, Zhao J, LiPuma JJ. Cystic fibrosis lung microbiome: Opportunities to reconsider management of airway infection. Pediatr Pulmonol . 2015 Oct;50 Suppl 40:S31-8.
• Caverly LJ, Huang YJ, Sze MA. Past, Present, and Future Research on the Lung Microbiome in Inflammatory Airway Disease. Chest . 2019 Aug;156(2):376-382.
• Caverly LJ, LiPuma JJ. Cystic fibrosis respiratory microbiota: unraveling complexity to inform clinical practice. Expert Rev Respir Med. 2018 Oct;12(10):857-865.
• Carmody et al. Fluctuations in airway bacterial communities associated with clinical states and disease stages in cystic fibrosis. PLoS One. 2018 Mar 9;13(3):e0194060.

Robert Quinn: 

• Quinn RA et al. Microbial, host and xenobiotic diversity in the cystic fibrosis sputum metabolome. ISME J. 2016 Jun; 10(6): 1483–1498. 
• Quinn RA et al. A Winogradsky-based culture system shows an association between microbial fermentation and cystic fibrosis exacerbation. The ISME Journal volume 9, pages1024–1038. ISME J. 2015 Mar 17;9(4):1024-38.
• Quinn RA et al. Metabolomics of pulmonary exacerbations reveals the personalized nature of cystic fibrosis disease. PeerJ. 2016 Aug 11;4:e2174.
• Raghunashi R et al. High-Resolution Longitudinal Dynamics of the Cystic Fibrosis Sputum Microbiome and Metabolome through Antibiotic Therapy. mSystems. 2020 Jun 23;5(3):e00292-20.
• Quinn RA et al. Ecological networking of cystic fibrosis lung infections. NPJ Biofilms Microbiomes. 2016 Dec 2;2:4.

Other Referenced Works: 

• Muhlebach MS et al. Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children. PLoS Pathog. 2018 Jan 18;14(1):e1006798.
• Sly PD et al. Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med 2009; 180: pp. 146-152
• JM Flynn et al. Disruption of Cross-Feeding Inhibits Pathogen Growth in the Sputa of Patients with Cystic Fibrosis. 
• Muhlebach MS et al.  Anaerobic bacteria cultured from cystic fibrosis airways correlate to milder disease: a multisite study. Eur Respir J. 2018 Jul 11;52(1):1800242. mSphere. 2020 Apr 29;5(2):e00343-20.
• Pittman JE et al. Association of Antibiotics, Airway Microbiome, and Inflammation in Infants with Cystic Fibrosis. Ann Am Thorac Soc. 2017 Oct;14(10):1548-1555.
• Nelso MT et al. Maintenance tobramycin primarily affects untargeted bacteria in the CF sputum microbiome. Thorax. 2020 Jul 6
• Cowley SC et al. Pediatric Cystic Fibrosis Sputum Can Be Chemically Dynamic, Anoxic, and Extremely Reduced Due to Hydrogen Sulfide Formation. mBio. 2015 Jul 28;6(4):e00767.
• Heltshe SL et al. Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor. Clin Infect Dis. 2015 Mar 1;60(5):703-12.
• Singh SB. Pathogen acquisition in patients with cystic fibrosis receiving ivacaftor or lumacaftor/ivacaftor. Pediatr Pulmonol. 2019 Aug;54(8):1200-1208.
• Hisert KB et al. Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections. Am J Respir Crit Care Med. 2017 Jun 15;195(12):1617-1628.