{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T16:42:39Z","timestamp":1790700159517,"version":"4.1.0"},"reference-count":94,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["K23-HL146970"],"award-info":[{"award-number":["K23-HL146970"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["POORE20D0"],"award-info":[{"award-number":["POORE20D0"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["ZEMANI17Y5"],"award-info":[{"award-number":["ZEMANI17Y5"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["HONG20A0-KB"],"award-info":[{"award-number":["HONG20A0-KB"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pathogens"],"abstract":"<jats:p>Fungi are frequently recovered from lower airway samples from people with cystic fibrosis (CF), yet the role of fungi in the progression of lung disease is debated. Recent studies suggest worsening clinical outcomes associated with airway fungal detection, although most studies to date are retrospective or observational. The presence of fungi can elicit a T helper cell type 2 (Th-2) mediated inflammatory reaction known as allergic bronchopulmonary aspergillosis (ABPA), particularly in those with a genetic atopic predisposition. In this review, we discuss the epidemiology of fungal infections in people with CF, risk factors associated with development of fungal infections, and microbiologic approaches for isolation and identification of fungi. We review the spectrum of fungal disease presentations, clinical outcomes after isolation of fungi from airway samples, and the importance of considering airway co-infections. Finally, we discuss the association between fungi and airway inflammation highlighting gaps in knowledge and future research questions that may further elucidate the role of fungus in lung disease progression.<\/jats:p>","DOI":"10.3390\/pathogens10050618","type":"journal-article","created":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T06:01:33Z","timestamp":1621317693000},"page":"618","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Fungal Infection and Inflammation in Cystic Fibrosis"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5229-2418","authenticated-orcid":false,"given":"T. Spencer","family":"Poore","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gina","family":"Hong","sequence":"additional","affiliation":[{"name":"Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7507-9337","authenticated-orcid":false,"given":"Edith T.","family":"Zemanick","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1016\/S0140-6736(16)00576-6","article-title":"Cystic fibrosis","volume":"388","author":"Elborn","year":"2016","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1056\/NEJMra043184","article-title":"Cystic fibrosis","volume":"352","author":"Rowe","year":"2005","journal-title":"N. Engl. J. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1164\/rccm.200304-505SO","article-title":"Pathophysiology and management of pulmonary infections in cystic fibrosis","volume":"168","author":"Gibson","year":"2003","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1086\/514631","article-title":"Microbiology of sputum from patients at cystic fibrosis centers in the United States","volume":"27","author":"Burns","year":"1998","journal-title":"Clin. Infect. Dis."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1016\/j.jcf.2015.03.003","article-title":"Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy","volume":"14","author":"Cantin","year":"2015","journal-title":"J. Cyst. Fibros."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1164\/rccm.201203-0507OC","article-title":"Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis","volume":"186","author":"Sagel","year":"2012","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1378\/chest.15-0676","article-title":"Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis","volume":"149","author":"Salsgiver","year":"2016","journal-title":"Chest"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1002\/ppul.10127","article-title":"Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis","volume":"34","author":"Emerson","year":"2002","journal-title":"Pediatr. Pulmonol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1002\/ppul.21525","article-title":"Initial Pseudomonas aeruginosa treatment failure is associated with exacerbations in cystic fibrosis","volume":"47","author":"Kronmal","year":"2012","journal-title":"Pediatr. Pulmonol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/j.jpeds.2008.08.001","article-title":"Impact of Pseudomonas and Staphylococcus infection on inflammation and clinical status in young children with cystic fibrosis","volume":"154","author":"Sagel","year":"2009","journal-title":"J. Pediatr."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1093\/cid\/cit270","article-title":"Staphylococcus aureus small-colony variants are independently associated with worse lung disease in children with cystic fibrosis","volume":"57","author":"Wolter","year":"2013","journal-title":"Clin. Infect. Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1001\/jama.293.5.581","article-title":"Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis","volume":"293","author":"Li","year":"2005","journal-title":"JAMA"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1002\/ppul.21374","article-title":"Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis","volume":"46","author":"Sanders","year":"2011","journal-title":"Pediatr. Pulmonol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1055\/s-0039-1697975","article-title":"Acute pulmonary exacerbations in cystic fibrosis","volume":"40","author":"Goss","year":"2019","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"S75","DOI":"10.1002\/ppul.24126","article-title":"The myriad challenges of respiratory fungal infection in cystic fibrosis","volume":"53","author":"Tracy","year":"2018","journal-title":"Pediatric Pulmonol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"S50","DOI":"10.1016\/j.jinf.2016.04.022","article-title":"Aspergillus infections in cystic fibrosis","volume":"72","author":"King","year":"2016","journal-title":"J. Infect."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1016\/j.jaci.2013.04.007","article-title":"Novel immunologic classification of aspergillosis in adult cystic fibrosis","volume":"132","author":"Baxter","year":"2013","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1007\/s11046-017-0185-x","article-title":"Fungal and bacterial diversity of airway microbiota in adults with cystic fibrosis: Concordance between conventional methods and ultra-deep sequencing, and their practical use in the clinical laboratory","volume":"183","author":"Botterel","year":"2018","journal-title":"Mycopathologia"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/s11046-017-0173-1","article-title":"Toward the standardization of mycological examination of sputum samples in cystic fibrosis: Results from a French multicenter prospective study","volume":"183","author":"Coron","year":"2018","journal-title":"Mycopathologia"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1378\/chest.09-1103","article-title":"The effect of chronic infection with Aspergillus fumigatus on lung function and hospitalization in patients with cystic fibrosis","volume":"137","author":"Amin","year":"2010","journal-title":"Chest"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/j.jcf.2019.08.008","article-title":"The presence of Aspergillus fumigatus is associated with worse respiratory quality of life in cystic fibrosis","volume":"19","author":"Hong","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"18999","DOI":"10.1038\/s41598-020-75886-w","article-title":"Risk factors for respiratory Aspergillus fumigatus in German Cystic Fibrosis patients and impact on lung function","volume":"10","author":"Duesberg","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"74","DOI":"10.4103\/atm.ATM_231_16","article-title":"Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis","volume":"12","author":"Janahi","year":"2017","journal-title":"Ann. Thorac. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/j.aller.2010.06.002","article-title":"Cystic fibrosis, atopy, asthma and ABPA","volume":"38","author":"Antunes","year":"2010","journal-title":"Allergol. Immunopathol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1016\/j.jcf.2018.10.001","article-title":"Prevalence, geographic risk factor, and development of a standardized protocol for fungal isolation in cystic fibrosis: Results from the international prospective study \u201cMFIP\u201d","volume":"18","author":"Delhaes","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.jcf.2018.11.012","article-title":"Prevalence and diversity of filamentous fungi in the airways of cystic fibrosis patients\u2014A Dutch, multicentre study","volume":"18","author":"Engel","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1007\/s11046-017-0190-0","article-title":"Aspergillus Bronchitis in Patients with Cystic Fibrosis","volume":"183","author":"Brandt","year":"2018","journal-title":"Mycopathologia"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1016\/j.jcf.2018.01.008","article-title":"Risk factors for persistent Aspergillus respiratory isolation in cystic fibrosis","volume":"17","author":"Hong","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/j.jcf.2009.11.010","article-title":"Prevalence and risk factors for recovery of filamentous fungi in individuals with cystic fibrosis","volume":"9","author":"Sudfeld","year":"2010","journal-title":"J. Cyst. Fibros."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1164\/rccm.201908-1585OC","article-title":"Aspergillus infections and progression of structural lung disease in children with cystic Fibrosis","volume":"201","author":"Breuer","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1136\/thoraxjnl-2018-211550","article-title":"Aspergillus and progression of lung disease in children with cystic fibrosis","volume":"74","author":"Harun","year":"2019","journal-title":"Thorax"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1002\/ppul.24210","article-title":"Inhaled antibiotic use is associated with Scedosporium\/Lomentospora species isolation in cystic fibrosis","volume":"54","author":"Hong","year":"2019","journal-title":"Pediatr. Pulmonol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1093\/mmy\/myw035","article-title":"Fungal epidemiology and diversity in cystic fibrosis patients over a 5-year period in a national reference center","volume":"54","author":"Ziesing","year":"2016","journal-title":"Med. Mycol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"S37","DOI":"10.3109\/13693786.2010.500627","article-title":"Clinical associations and prevalence of Scedosporium spp. in Australian cystic fibrosis patients: Identification of novel risk factors?","volume":"48","author":"Blyth","year":"2010","journal-title":"Med. Mycol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1111\/myc.12789","article-title":"Airway persistence by the emerging multi-azole-resistant Rasamsonia argillacea complex in cystic fibrosis","volume":"61","author":"Abdolrasouli","year":"2018","journal-title":"Mycoses"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"e45","DOI":"10.1016\/j.jcf.2016.02.006","article-title":"Clinical outcomes in cystic fibrosis patients with Trichosporon respiratory infection","volume":"15","author":"Esther","year":"2016","journal-title":"J. Cyst. Fibros."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1093\/mmy\/myx108","article-title":"Direct detection of Exophiala and Scedosporium species in sputa of patients with cystic fibrosis","volume":"56","author":"Chen","year":"2018","journal-title":"Med. Mycol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/j.jcf.2013.12.007","article-title":"Development of IgG antibodies to Exophiala dermatitidis is associated with inflammatory responses in patients with cystic fibrosis","volume":"13","author":"Kondori","year":"2014","journal-title":"J. Cyst. Fibros."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1002\/(SICI)1099-0496(199911)28:5<321::AID-PPUL3>3.0.CO;2-V","article-title":"Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis","volume":"28","author":"Rosenfeld","year":"1999","journal-title":"Pediatr. Pulmonol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1002\/ppul.23150","article-title":"Sputum induction improves detection of pathogens in children with cystic fibrosis","volume":"50","author":"Hoppe","year":"2015","journal-title":"Pediatr. Pulmonol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1164\/ajrccm\/144.2.331","article-title":"Predictive value of oropharyngeal cultures for identifying lower airway bacteria in cystic fibrosis patients","volume":"144","author":"Ramsey","year":"1991","journal-title":"Am. Rev. Respir. Dis."},{"key":"ref_42","first-page":"163","article-title":"Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: A randomized trial","volume":"306","author":"Wainwright","year":"2011","journal-title":"JAMA"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"S6","DOI":"10.1086\/503485","article-title":"Infection control recommendations for patients with cystic fibrosis: Microbiology, important pathogens, and infection control practices to prevent patient-to-patient transmission","volume":"24","author":"Saiman","year":"2003","journal-title":"Infect. Control Hosp. Epidemiol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1007\/s11046-017-0150-8","article-title":"Challenges in laboratory detection of fungal pathogens in the airways of cystic fibrosis patients","volume":"183","author":"Chen","year":"2018","journal-title":"Mycopathologia"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1016\/S2213-2600(18)30171-1","article-title":"The CF-Sputum Induction Trial (CF-SpIT) to assess lower airway bacterial sampling in young children with cystic fibrosis: A prospective internally controlled interventional trial","volume":"6","author":"Ronchetti","year":"2018","journal-title":"Lancet Respir. Med."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jcf.2013.09.004","article-title":"Culture-based diagnostic microbiology in cystic fibrosis: Can we simplify the complexity?","volume":"13","author":"Burns","year":"2014","journal-title":"J. Cyst. Fibros."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.1128\/JCM.02182-16","article-title":"Use of selective fungal culture media increases rates of detection of fungi in the respiratory tract of cystic fibrosis patients","volume":"55","author":"Hong","year":"2017","journal-title":"J. Clin. Microbiol."},{"key":"ref_48","doi-asserted-by":"crossref","unstructured":"Delhaes, L., Monchy, S., Frealle, E., Hubans, C., Salleron, J., Leroy, S., Prevotat, A., Wallet, F., Wallaert, B., and Dei-Cas, E. (2012). The airway microbiota in cystic fibrosis: A complex fungal and bacterial community--implications for therapeutic management. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0036313"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"89","DOI":"10.3389\/fmicb.2015.00089","article-title":"The lung mycobiome: An emerging field of the human respiratory microbiome","volume":"6","author":"Nguyen","year":"2015","journal-title":"Front. Microbiol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.jcf.2020.05.013","article-title":"The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis","volume":"20","author":"Cuthbertson","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"ref_51","doi-asserted-by":"crossref","unstructured":"Toor, A., Culibrk, L., Singhera, G.K., Moon, K.M., Prudova, A., Foster, L.J., Moore, M.M., Dorscheid, D.R., and Tebbutt, S.J. (2018). Transcriptomic and proteomic host response to Aspergillus fumigatus conidia in an air-liquid interface model of human bronchial epithelium. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0209652"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"30513","DOI":"10.1074\/jbc.M803149200","article-title":"Aspergillus fumigatus-induced interleukin-8 synthesis by respiratory epithelial cells is controlled by the phosphatidylinositol 3-kinase, p38 MAPK, and ERK1\/2 pathways and not by the toll-like receptor-MyD88 pathway","volume":"283","author":"Balloy","year":"2008","journal-title":"J. Biol. Chem."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/j.jinf.2004.12.012","article-title":"Interaction of airway epithelial cells (A549) with spores and mycelium of Aspergillus fumigatus","volume":"51","author":"Zhang","year":"2005","journal-title":"J. Infect."},{"key":"ref_54","doi-asserted-by":"crossref","unstructured":"Oosthuizen, J.L., Gomez, P., Ruan, J., Hackett, T.L., Moore, M.M., Knight, D.A., and Tebbutt, S.J. (2011). Dual organism transcriptomics of airway epithelial cells interacting with conidia of Aspergillus fumigatus. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0020527"},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Gomez, P., Hackett, T.L., Moore, M.M., Knight, D.A., and Tebbutt, S.J. (2010). Functional genomics of human bronchial epithelial cells directly interacting with conidia of Aspergillus fumigatus. BMC Genom., 11.","DOI":"10.1186\/1471-2164-11-358"},{"key":"ref_56","doi-asserted-by":"crossref","unstructured":"Botterel, F., Gross, K., Ibrahim-Granet, O., Khoufache, K., Escabasse, V., Coste, A., Cordonnier, C., Escudier, E., and Bretagne, S. (2008). Phagocytosis of Aspergillus fumigatus conidia by primary nasal epithelial cells in vitro. BMC Microbiol., 8.","DOI":"10.1186\/1471-2180-8-97"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1007\/s12281-016-0268-z","article-title":"Pathogenesis of fungal infections in cystic fibrosis","volume":"10","author":"Williams","year":"2016","journal-title":"Curr. Fungal Infect. Rep."},{"key":"ref_58","first-page":"2","article-title":"Biofilm formation by Aspergillus fumigatus","volume":"52","author":"Kaur","year":"2014","journal-title":"Med. Mycol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1016\/S1534-5807(02)00295-2","article-title":"Pseudomonas aeruginosa anaerobic respiration in biofilms: Relationships to cystic fibrosis pathogenesis","volume":"3","author":"Yoon","year":"2002","journal-title":"Dev. Cell"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"S229","DOI":"10.1080\/13693780600760773","article-title":"Th1\/Th2 in aspergillosis","volume":"44","author":"Stevens","year":"2006","journal-title":"Med. Mycol."},{"key":"ref_61","first-page":"1662","article-title":"CFTR modulators: The changing face of cystic fibrosis in the era of precision medicine","volume":"10","year":"2019","journal-title":"Front. Pharmacol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"e122695","DOI":"10.1172\/jci.insight.122695","article-title":"Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR","volume":"3","author":"Donaldson","year":"2018","journal-title":"JCI Insight"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1093\/cid\/ciu944","article-title":"Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor","volume":"60","author":"Heltshe","year":"2015","journal-title":"Clin. Infect. Dis."},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Bercusson, A., Jarvis, G., and Shah, A. (2021). CF fungal disease in the age of CFTR modulators. Mycopathologia.","DOI":"10.1007\/s11046-021-00541-5"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"S196","DOI":"10.1093\/mmy\/myy090","article-title":"The role of occupational Aspergillus exposure in the development of diseases","volume":"57","author":"Sabino","year":"2019","journal-title":"Med. Mycol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1378\/chest.130.1.222","article-title":"Aspergillus bronchitis in cystic fibrosis","volume":"130","author":"Shoseyov","year":"2006","journal-title":"Chest"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1053\/rmed.2000.0918","article-title":"Fungal atopy in adult cystic fibrosis","volume":"94","author":"Henry","year":"2000","journal-title":"Respir. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1136\/thx.35.11.807","article-title":"Atopy and bronchial reactivity in older patients with cystic fibrosis","volume":"35","author":"Tobin","year":"1980","journal-title":"Thorax"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1097\/MCP.0b013e32833e24a6","article-title":"Allergic bronchopulmonary aspergillosis and Aspergillus infection in cystic fibrosis","volume":"16","author":"Moss","year":"2010","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1111\/j.1469-0691.2010.03429.x","article-title":"Aspergillus fumigatus colonization in cystic fibrosis: Implications for lung function?","volume":"17","author":"Stellato","year":"2011","journal-title":"Clin. Microbiol. Infect."},{"key":"ref_71","doi-asserted-by":"crossref","unstructured":"AbdulWahab, A., Salah, H., Chandra, P., and Taj-Aldeen, S.J. (2017). Persistence of Candida dubliniensis and lung function in patients with cystic fibrosis. BMC Res. Notes, 10.","DOI":"10.1186\/s13104-017-2656-z"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1016\/j.ijid.2020.05.063","article-title":"A 16-year retrospective study on fungal prevalence and diversity in patients with cystic fibrosis: Candida dubliniensis was associated with a decline in lung function","volume":"96","author":"Klingspor","year":"2020","journal-title":"Int. J. Infect. Dis."},{"key":"ref_73","doi-asserted-by":"crossref","unstructured":"Aaron, S.D., Vandemheen, K.L., Freitag, A., Pedder, L., Cameron, W., Lavoie, A., Paterson, N., Wilcox, P., Rabin, H., and Tullis, E. (2012). Treatment of Aspergillus fumigatus in patients with cystic fibrosis: A randomized, placebo-controlled pilot study. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0036077"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"999","DOI":"10.1164\/rccm.201203-0478OC","article-title":"The effect of Aspergillus fumigatus infection on vitamin D receptor expression in cystic fibrosis","volume":"186","author":"Coughlan","year":"2012","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"e197","DOI":"10.1016\/j.ejrad.2011.01.114","article-title":"Radiological abnormalities associated with Aspergillus colonization in a cystic fibrosis population","volume":"81","author":"McMahon","year":"2012","journal-title":"Eur. J. Radiol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1513\/AnnalsATS.201902-122OC","article-title":"Ivacaftor is associated with reduced lung infection by key cystic fibrosis pathogens. A cohort study using national registry data","volume":"16","author":"Frost","year":"2019","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_77","doi-asserted-by":"crossref","unstructured":"Keown, K., Reid, A., Moore, J.E., Taggart, C.C., and Downey, D.G. (2020). Coinfection with Pseudomonas aeruginosa and Aspergillus fumigatus in cystic fibrosis. Eur. Respir. Rev., 29.","DOI":"10.1183\/16000617.0011-2020"},{"key":"ref_78","doi-asserted-by":"crossref","unstructured":"Reece, E., Segurado, R., Jackson, A., McClean, S., Renwick, J., and Greally, P. (2017). Co-colonisation with Aspergillus fumigatus and Pseudomonas aeruginosa is associated with poorer health in cystic fibrosis patients: An Irish registry analysis. BMC Pulm. Med., 17.","DOI":"10.1186\/s12890-017-0416-4"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1190","DOI":"10.1086\/314727","article-title":"Effect of chronic intermittent administration of inhaled tobramycin on respiratory microbial flora in patients with cystic fibrosis","volume":"179","author":"Burns","year":"1999","journal-title":"J. Infect. Dis."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1111\/j.1574-6968.2010.02130.x","article-title":"Pseudomonas aeruginosa and their small diffusible extracellular molecules inhibit Aspergillus fumigatus biofilm formation","volume":"313","author":"Mowat","year":"2010","journal-title":"FEMS Microbiol. Lett."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"866","DOI":"10.3389\/fmicb.2015.00866","article-title":"Pseudomonas aeruginosa inhibits the growth of Scedosporium aurantiacum, an opportunistic fungal pathogen isolated from the lungs of cystic fibrosis patients","volume":"6","author":"Kaur","year":"2015","journal-title":"Front. Microbiol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"e5931","DOI":"10.7717\/peerj.5931","article-title":"Interaction between Pseudomonas aeruginosa and Aspergillus fumigatus in cystic fibrosis","volume":"6","author":"Zhao","year":"2018","journal-title":"PeerJ"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1136\/thoraxjnl-2012-202412","article-title":"Intravenous antibiotics reduce the presence of Aspergillus in adult cystic fibrosis sputum","volume":"68","author":"Baxter","year":"2013","journal-title":"Thorax"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1513\/pats.200703-044BR","article-title":"Sputum biomarkers of inflammation in cystic fibrosis lung disease","volume":"4","author":"Sagel","year":"2007","journal-title":"Proc. Am. Thorac. Soc."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1002\/ppul.20165","article-title":"Induced sputum matrix metalloproteinase-9 correlates with lung function and airway inflammation in children with cystic fibrosis","volume":"39","author":"Sagel","year":"2005","journal-title":"Pediatr. Pulmonol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1093\/cid\/cir399","article-title":"Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis","volume":"53","author":"Gangell","year":"2011","journal-title":"Clin. Infect. Dis."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1002\/ppul.22934","article-title":"Asthma and cystic fibrosis: A tangled web","volume":"49","author":"Kent","year":"2014","journal-title":"Pediatr. Pulmonol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1016\/S0022-2143(98)90089-0","article-title":"The Th1\/Th2 paradigm in allergic bronchopulmonary aspergillosis","volume":"131","author":"Schuyler","year":"1998","journal-title":"J. Lab. Clin. Med."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1164\/rccm.201106-1027OC","article-title":"Cystic fibrosis transmembrane conductance regulator regulates epithelial cell response to Aspergillus and resultant pulmonary inflammation","volume":"185","author":"Chaudhary","year":"2012","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"5186","DOI":"10.4049\/jimmunol.177.8.5186","article-title":"Aspergillus fumigatus generates an enhanced Th2-biased immune response in mice with defective cystic fibrosis transmembrane conductance regulator","volume":"177","author":"Allard","year":"2006","journal-title":"J. Immunol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1038\/labinvest.3700379","article-title":"Enhanced IgE allergic response to Aspergillus fumigatus in CFTR-\/- mice","volume":"86","author":"Muller","year":"2006","journal-title":"Lab. Investig."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1988","DOI":"10.1086\/429964","article-title":"Influence of interleukin-10 on Aspergillus fumigatus infection in patients with cystic fibrosis","volume":"191","author":"Brouard","year":"2005","journal-title":"J. Infect. Dis."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/s40733-015-0003-5","article-title":"Vitamin D supplementation decreases Aspergillus fumigatus specific Th2 responses in CF patients with aspergillus sensitization: A phase one open-label study","volume":"1","author":"Nguyen","year":"2015","journal-title":"Asthma Res. Pract."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.1111\/cea.12595","article-title":"Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: Systematic review and meta-analysis","volume":"45","author":"Maturu","year":"2015","journal-title":"Clin. Exp. Allergy"}],"container-title":["Pathogens"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-0817\/10\/5\/618\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T06:03:08Z","timestamp":1760162588000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-0817\/10\/5\/618"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,18]]},"references-count":94,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2021,5]]}},"alternative-id":["pathogens10050618"],"URL":"https:\/\/doi.org\/10.3390\/pathogens10050618","relation":{},"ISSN":["2076-0817"],"issn-type":[{"value":"2076-0817","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,18]]}}}