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References
- Mucus clearance as a primary innate defense mechanism for mammalian airways.J Clin Invest. 2002; 109: 571-577
- Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis.Sci Transl Med. 2019; 11
- IL-1beta dominates the promucin secretory cytokine profile in cystic fibrosis.J Clin Invest. 2019; 129: 4433-4450
- Hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies.Eur Respir J. 2017; 49
- Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis.Science. 2014; 345: 818-822
- Therapeutic options for hydrating airway mucus in cystic fibrosis.Pharmacology. 2015; 95: 117-132
- Inhaled dry powder alginate oligosaccharide in cystic fibrosis: a randomised, double-blind, placebo-controlled, crossover phase 2b study.ERJ Open Res. 2020; 6
- Ion channels as targets to treat cystic fibrosis lung disease.J Cyst Fibros. 2018; 17: S22-S27
- pH modulates the activity and synergism of the airway surface liquid antimicrobials beta-defensin-3 and LL-37.Proc Natl Acad Sci U S A. 2014; 111: 18703-18708
- Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis.J Infect Dis. 2004; 190: 1245-1253
- Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients.J Cyst Fibros. 2013; 12: 390-398
- A novel host defense system of airways is defective in cystic fibrosis.Am J Respir Crit Care Med. 2007; 175: 174-183
- SPLUNC1: a novel marker of cystic fibrosis exacerbations.Eur Respir J. 2021; 58
- Airway Surface Liquid Has Innate Antiviral Activity That Is Reduced in Cystic Fibrosis.Am J Respir Cell Mol Biol. 2020; 62: 104-111
- Activity of hypothiocyanite and lactoferrin (ALX-009) against respiratory cystic fibrosis pathogens in sputum.J Antimicrob Chemother. 2018; 73: 3391-3397
- First clinical trials of novel ENaC targeting therapy, SPX-101, in healthy volunteers and adults with cystic fibrosis.Pulm Pharmacol Ther. 2019; 58: 101819
- Gallium disrupts bacterial iron metabolism and has therapeutic effects in mice and humans with lung infections.Sci Transl Med. 2018; 10: eaat7520
- Lysosomal-Enzymes from Polymorphonuclear Leukocytes and Proteinase-Inhibitors in Patients with Cystic-Fibrosis.Am Rev Respir Dis. 1986; 134: 49-56
- Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis.Am J Respir Crit Care Med. 2012; 186: 857-865
- miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production.Am J Respir Crit Care Med. 2014; 190: 165-174
- Proteinase 3, a potent secretagogue in airways, is present in cystic fibrosis sputum.Am J Respir Cell Mol Biol. 1999; 20: 729-736
- Australian Respiratory Early Surveillance Team for Cystic F. Matrix metalloproteinase activation by free neutrophil elastase contributes to bronchiectasis progression in early cystic fibrosis.Eur Respir J. 2015; 46: 384-394
- Changes in Airway Microbiome and Inflammation with Ivacaftor Treatment in Patients with Cystic Fibrosis and the G551D Mutation.Ann Am Thorac Soc. 2020; 17: 212-220
- Investigators W. Phase 2 Trial of the DPP-1 Inhibitor Brensocatib in Bronchiectasis.N Engl J Med. 2020; 383: 2127-2137
- Cathepsin C inhibitors as anti-inflammatory drug discovery: Challenges and opportunities.Eur J Med Chem. 2021; 225: 113818
- Single dose escalation studies with inhaled POL6014, a potent novel selective reversible inhibitor of human neutrophil elastase, in healthy volunteers and subjects with cystic fibrosis.J Cyst Fibros. 2020; 19: 299-304
- Neutrophil-targeted, protease-activated pulmonary drug delivery blocks airway and systemic inflammation.JCI Insight. 2019; 4
- Nitric oxide and the immune response.Nat Immunol. 2001; 2: 907-916
- Decreased concentration of exhaled nitric oxide (NO) in patients with cystic fibrosis.Pediatr Pulmonol. 1997; 24: 173-177
- Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells.J Clin Invest. 1998; 102: 1200-1207
- Increased arginase activity in cystic fibrosis airways.Am J Respir Crit Care Med. 2005; 172: 1523-1528
- Effect of ivacaftor therapy on exhaled nitric oxide in patients with cystic fibrosis.J Cyst Fibros. 2015;
- Pilot study to test inhaled nitric oxide in cystic fibrosis patients with refractory Mycobacterium abscessus lung infection.J Cyst Fibros. 2020; 19: 225-231
- Oxidative stress and antioxidant therapy in cystic fibrosis.Biochim Biophys Acta. 2012; 1822: 690-713
- CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.Front Physiol. 2021; 12: 619442
- Barrier functions and paracellular integrity in human cell culture models of the proximal respiratory unit.Eur J Pharm Biopharm. 2009; 72: 339-349
- Src signaling links mediators of inflammation to Cx43 gap junction channels in primary and transformed CFTR-expressing airway cells.Cell Commun Adhes. 2003; 10: 279-285
- Junctional abnormalities in human airway epithelial cells expressing F508del CFTR.Am J Physiol Lung Cell Mol Physiol. 2015; 309: L475-L487
- Paracellular permeability of bronchial epithelium is controlled by CFTR.Cell Physiol Biochem. 2011; 28: 289-296
- Novel effects of azithromycin on tight junction proteins in human airway epithelia.Antimicrob Agents Chemother. 2006; 50: 1805-1812
- Pathogen recognition and innate immunity.Cell. 2006; 124: 783-801
- Cystic Fibrosis Lung Immunity: The Role of the Macrophage.J Innate Immun. 2016; 8: 550-563
- Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations.Cell Mol Life Sci. 2020; 77: 4485-4503
- Pseudomonas aeruginosa Consumption of Airway Metabolites Promotes Lung Infection.Pathogens. 2021; 10
- Impaired innate host defense causes susceptibility to respiratory virus infections in cystic fibrosis.Immunity. 2003; 18: 619-630
- SARS-CoV-2 (COVID-19) and cystic fibrosis.Am J Physiol Lung Cell Mol Physiol. 2020; 319: L408-L415
- Anti-inflammatory effects of azithromycin in cystic fibrosis airway epithelial cells.Biochem Biophys Res Commun. 2006; 350: 977-982
- Looking beyond pulmonary disease in COVID-19: A lesson from patients with cystic fibrosis.Med Hypotheses. 2021; 147: 110481
- IL-1 receptor antagonist ameliorates inflammasome-dependent inflammation in murine and human cystic fibrosis.Nat Commun. 2016; 7: 10791
- Inflammasome activation and Th17 responses.Mol Immunol. 2019; 107: 142-164
- Bacterial Subversion of Autophagy in Cystic Fibrosis.Front Cell Infect Microbiol. 2021; 11: 760922
- Association of cystic fibrosis with abnormalities in fatty acid metabolism.N Engl J Med. 2004; 350: 560-569
- Roles, Actions, and Therapeutic Potential of Specialized Pro-resolving Lipid Mediators for the Treatment of Inflammation in Cystic Fibrosis.Front Pharmacol. 2019; 10: 252
- Metabolomic profiling of regulatory lipid mediators in sputum from adult cystic fibrosis patients.Free Radic Biol Med. 2012; 53: 160-171
- Reduced 15-lipoxygenase 2 and lipoxin A4/leukotriene B4 ratio in children with cystic fibrosis.Eur Respir J. 2014; 44: 394-404
- Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis.Am J Physiol Lung Cell Mol Physiol. 2007; 292: L476-L486
- Lipid metabolism in cystic fibrosis.Curr Opin Clin Nutr Metab Care. 2009; 12: 105-109
- Association of High-Dose Ibuprofen Use, Lung Function Decline, and Long-Term Survival in Children with Cystic Fibrosis.Ann Am Thorac Soc. 2018; 15: 485-493
- Treatment of Allergic Asthma with Fenretinide Formulation (LAU-7b) Downregulates ORMDL Sphingolipid Biosynthesis Regulator 3 (Ormdl3) Expression and Normalizes Ceramide Imbalance.J Pharmacol Exp Ther. 2020; 373: 476-487
- Specific Inhibition of the NLRP3 Inflammasome as an Antiinflammatory Strategy in Cystic Fibrosis.Am J Respir Crit Care Med. 2019; 200: 1381-1391
- Oral L-arginine supplementation in cystic fibrosis patients: a placebo-controlled study.Eur Respir J. 2005; 25: 62-68
- The triterpenoid CDDO limits inflammation in preclinical models of cystic fibrosis lung disease.Am J Physiol Lung Cell Mol Physiol. 2009; 297: L828-L836
- Antimicrobial Properties of Mesenchymal Stem Cells: Therapeutic Potential for Cystic Fibrosis Infection, and Treatment.Stem Cells Int. 2016; 2016: 5303048
- A comprehensive proteomics profiling identifies NRP1 as a novel identity marker of human bone marrow mesenchymal stromal cell-derived small extracellular vesicles.Stem Cell Res Ther. 2019; 10: 401
- Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation.Genes (Basel). 2019; 10
- Pseudomonas aeruginosa aggregates in cystic fibrosis sputum produce exopolysaccharides that likely impede current therapies.Cell Rep. 2021; 34: 108782
- CFTR Expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis.Biochemistry. 2006; 45: 10260-10269
- A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy.Blood. 2014; 124: 999-1009
- Neutrophil Adaptations upon Recruitment to the Lung: New Concepts and Implications for Homeostasis and Disease.Int J Mol Sci. 2020; 21
- Transcriptional firing represses bactericidal activity in cystic fibrosis airway neutrophils.Cell Rep Med. 2021; 2: 100239
- A randomized double blind, placebo controlled phase 2 trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis.J Cyst Fibros. 2014; 13: 148-155
- Empire-CF study: A phase 2 clinical trial of leukotriene A4 hydrolase inhibitor acebilustat in adult subjects with cystic fibrosis.J Cyst Fibros. 2021; 20: 1026-1034
- Targeting cystic fibrosis inflammation in the age of CFTR modulators: focus on macrophages.Eur Respir J. 2021; 57
- The heterogeneity of lung macrophages in the susceptibility to disease.Eur Respir Rev. 2015; 24: 505-509
- Distinct pattern of immune cell population in the lung of human fetuses with cystic fibrosis.J Allergy Clin Immunol. 2001; 108: 524-529
- Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis.Eur Respir J. 2009; 34: 655-661
- Single Cell Transcriptional Archetypes of Airway Inflammation in Cystic Fibrosis.Am J Respir Crit Care Med. 2020;
- Monocytes from cystic fibrosis patients are locked in an LPS tolerance state: down-regulation of TREM-1 as putative underlying mechanism.PLoS ONE. 2008; 3: e2667
- Pseudomonas aeruginosa colonization causes PD-L1 overexpression on monocytes, impairing the adaptive immune response in patients with cystic fibrosis.J Cyst Fibros. 2019; 18: 630-635
- Mutations of Cystic Fibrosis Transmembrane Conductance Regulator Gene Cause a Monocyte-Selective Adhesion Deficiency.Am J Respir Crit Care Med. 2016; 193: 1123-1133
- CFTR Modulator Therapy Enhances Peripheral Blood Monocyte Contributions to Immune Responses in People With Cystic Fibrosis.Front Pharmacol. 2020; 11: 1219
- Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis.Elife. 2020; 9
- Ivacaftor decreases monocyte sensitivity to interferon-gamma in people with cystic fibrosis.ERJ Open Res. 2020; 6
- Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis.J Clin Invest. 2002; 109: 661-670
- Neutrophil elastase-regulated macrophage sheddome/secretome and phagocytic failure.Am J Physiol Lung Cell Mol Physiol. 2021; 321: L555-L565
- Decreased expression of HLA-DQ and HLA-DR on cells of the monocytic lineage in cystic fibrosis.J Mol Med (Berl). 2014; 92: 1293-1304
- Low sphingosine-1-phosphate impairs lung dendritic cells in cystic fibrosis.Am J Respir Cell Mol Biol. 2013; 48: 250-257
- Inflammatory lung secretions inhibit dendritic cell maturation and function via neutrophil elastase.Am J Respir Crit Care Med. 2006; 174: 1189-1198
- Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses.Am J Respir Cell Mol Biol. 2011; 44: 922-929
- Th17/Treg imbalance in murine cystic fibrosis is linked to indoleamine 2,3-dioxygenase deficiency but corrected by kynurenines.Am J Respir Crit Care Med. 2013; 187: 609-620
- Patients with cystic fibrosis have inducible IL-17+IL-22+ memory cells in lung draining lymph nodes.J Allergy Clin Immunol. 2013; 131 (1129.e1-5): 1117-1129
- Updates on T helper type 17 immunity in respiratory disease.Immunology. 2019; 156: 3-8
- Interleukin-17 Pathophysiology and Therapeutic Intervention in Cystic Fibrosis Lung Infection and Inflammation.Infect Immun. 2016; 84: 2410-2421
- Inflammatory cytokines TNF-alpha and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators.J Clin Invest. 2021; 131
- Regulatory T-cell impairment in cystic fibrosis patients with chronic pseudomonas infection.Am J Respir Crit Care Med. 2015; 191: 914-923
- Regulatory T cells in cystic fibrosis lung disease. More answers, more questions.Am J Respir Crit Care Med. 2015; 191: 866-868
- Transcriptional consequences of impaired immune cell responses induced by cystic fibrosis plasma characterized via dual RNA sequencing.BMC Med Genomics. 2019; 12: 66
- Secretory IgA-mediated immune response in saliva and early detection of Pseudomonas aeruginosa in the lower airways of pediatric cystic fibrosis patients.Med Microbiol Immunol. 2019; 208: 205-213
- Assessment of IgG antibodies to Pseudomonas aeruginosa in patients with cystic fibrosis by an enzyme-linked immunosorbent assay (ELISA).Diagn Pathol. 2014; 9: 158
- The influence of developmental timing on B cell diversity.Curr Opin Immunol. 2018; 51: 7-13
- Mucosal humoral immunity in cystic fibrosis - a tangled web of failed proteostasis, infection and adaptive immunity.EBioMedicine. 2020; 60: 103035
- Dissociation of systemic and mucosal autoimmunity in cystic fibrosis.J Cyst Fibros. 2020; 19: 196-202
- Vasculitis in Cystic Fibrosis.Front Pediatr. 2020; 8: 585275
- The role for neutrophil extracellular traps in cystic fibrosis autoimmunity.JCI Insight. 2016; 1: e88912
- BPI-ANCA is expressed in the airways of cystic fibrosis patients and correlates to platelet numbers and Pseudomonas aeruginosa colonization.Respir Med. 2020; 170: 105994
- SPLUNC1 degradation by the cystic fibrosis mucosal environment drives airway surface liquid dehydration.Eur Respir J. 2018; 52
- The Innate Lymphoid System Is a Critical Player in the Manifestation of Mucoinflammatory Airway Disease in Mice.J Immunol. 2020; 205: 1695-1708
- Hallmarks of Tissue-Resident Lymphocytes.Cell. 2016; 164: 1198-1211
- Reduced mucosal associated invariant T-cells are associated with increased disease severity and Pseudomonas aeruginosa infection in cystic fibrosis.PLoS One. 2014; 9: e109891
- Severely Impaired Control of Bacterial Infections in a Patient With Cystic Fibrosis Defective in Mucosal-Associated Invariant T Cells.Chest. 2018; 153: e93-e96
- Gammadelta T lymphocytes from cystic fibrosis patients and healthy donors are high TNF-alpha and IFN-gamma-producers in response to Pseudomonas aeruginosa.Respir Res. 2003; 4: 9
- IL-17A from innate and adaptive lymphocytes contributes to inflammation and damage in cystic fibrosis lung disease.Eur Respir J. 2021; 57
- Invariant natural killer T (iNKT) cells prevent autoimmunity, but induce pulmonary inflammation in cystic fibrosis.Cell Physiol Biochem. 2014; 34: 56-70
- Increased platelet activation occurs in cystic fibrosis patients and correlates to clinical status.Thromb Res. 2018; 162: 32-37
- Cystic fibrosis transmembrane conductance regulator dysfunction in platelets drives lung hyperinflammation.J Clin Invest. 2020; 130: 2041-2053
- Stroke and myocardial infarction following bronchial artery embolization in a cystic fibrosis patient.J Cyst Fibros. 2017; 16: 161-162
- CNS imaging studies in cystic fibrosis patients presenting with sudden neurological events.BMJ Open Respir Res. 2019; 6: e000456
- Pseudomonas aeruginosa Airway Infection Recruits and Modulates Neutrophilic Myeloid-Derived Suppressor Cells.Front Cell Infect Microbiol. 2016; 6: 167
- Granulocytic Myeloid-Derived Suppressor Cells in Cystic Fibrosis.Front Immunol. 2021; 12: 745326
- CF monocyte-derived macrophages have an attenuated response to extracellular vesicles secreted by airway epithelial cells.Am J Physiol Lung Cell Mol Physiol. 2021; 320: L530-L544
- Toll-Like Receptor 4 Engagement Mediates Prolyl Endopeptidase Release from Airway Epithelia via Exosomes.Am J Respir Cell Mol Biol. 2016; 54: 359-369
- Outer-membrane vesicles from Gram-negative bacteria: biogenesis and functions.Nat Rev Microbiol. 2015; 13: 605-619
- Extracellular Vesicles from Pseudomonas aeruginosa Suppress MHC-Related Molecules in Human Lung Macrophages.Immunohorizons. 2020; 4: 508-519
- Mesenchymal Stem Cell Soluble Mediators and Cystic Fibrosis.J Stem Cell Res Ther. 2017; 7
- Lung mesenchymal stem cells-derived extracellular vesicles attenuate the inflammatory profile of Cystic Fibrosis epithelial cells.Cell Signal. 2018; 51: 110-118
- Enhancing Cystic Fibrosis Immune Regulation.Front Pharmacol. 2021; 12: 573065
- Donor-defined mesenchymal stem cell antimicrobial potency against nontuberculous mycobacterium.Stem Cells Transl Med. 2021; 10: 1202-1216
- Can we design a better anti-cytokine therapy?.J Leukoc Biol. 2017; 102: 783-790
- Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis.Nat Commun. 2015; 6: 8382
- Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2.Nat Genet. 2011; 43: 539-546
- Members of Canadian Consortium for CFGS. Modulatory effect of the SLC9A3 gene on susceptibility to infections and pulmonary function in children with cystic fibrosis.Pediatr Pulmonol. 2011; 46: 385-392
- Genome-wide association study in patients with pulmonary Mycobacterium avium complex disease.Eur Respir J. 2021; 58
- AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations.J Cyst Fibros. 2019; 18: 127-134
- Inflammasome Genetic Variants, Macrophage Function, and Clinical Outcomes in Cystic Fibrosis.Am J Respir Cell Mol Biol. 2021; 65: 157-166
- Genetic Association With Pseudomonas aeruginosa Acquisition in Cystic Fibrosis: Influence of Surfactant Protein D and Mannose-Binding Lectin.Front Immunol. 2021; 12: 587313
- Genetic influences on cystic fibrosis lung disease severity.Front Pharmacol. 2013; 4: 40
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