{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T06:22:36Z","timestamp":1773296556422,"version":"3.50.1"},"reference-count":100,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2007,3,1]],"date-time":"2007-03-01T00:00:00Z","timestamp":1172707200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Paediatric Respiratory Reviews"],"published-print":{"date-parts":[[2007,3]]},"DOI":"10.1016\/j.prrv.2007.02.008","type":"journal-article","created":{"date-parts":[[2007,3,27]],"date-time":"2007-03-27T11:17:49Z","timestamp":1174994269000},"page":"30-39","source":"Crossref","is-referenced-by-count":28,"title":["Other mucoactive agents for cystic fibrosis"],"prefix":"10.1016","volume":"8","author":[{"given":"Peter T.P.","family":"Bye","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark R.","family":"Elkins","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.prrv.2007.02.008_bib1","first-page":"129","article-title":"Taxonomy of mucoactive medications","volume":"vol 188","author":"Rubin","year":"2004"},{"key":"10.1016\/j.prrv.2007.02.008_bib2","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1085\/jgp.118.2.223","article-title":"The relative roles of passive surface forces and active ion transport in the modulation of airway surface liquid volume and composition","volume":"118","author":"Tarran","year":"2001","journal-title":"J Gen Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib3","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.4049\/jimmunol.175.2.1090","article-title":"Reduced three-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces","volume":"175","author":"Matsui","year":"2005","journal-title":"J Immunol"},{"key":"10.1016\/j.prrv.2007.02.008_bib4","first-page":"288","article-title":"The high molecular weight components of sputum","volume":"41","author":"Brogen","year":"1960","journal-title":"Br J Exp Pathol"},{"key":"10.1016\/j.prrv.2007.02.008_bib5","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1183\/09031936.93.03010019","article-title":"The origin of DNA associated with mucus glycoproteins in cystic fibrosis sputum","volume":"3","author":"Lethem","year":"1990","journal-title":"Eur Respir J"},{"key":"10.1016\/j.prrv.2007.02.008_bib6","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1111\/j.1749-6632.1963.tb16677.x","article-title":"Human pulmonary secretions in health and disease","volume":"106","author":"Potter","year":"1963","journal-title":"Ann N Y Acad Sci"},{"key":"10.1016\/j.prrv.2007.02.008_bib7","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1016\/S0022-3476(88)80165-3","article-title":"Sputum changes associated with therapy for endobronchial exacerbation in cystic fibrosis","volume":"112","author":"Smith","year":"1988","journal-title":"J Pediatr"},{"key":"10.1016\/j.prrv.2007.02.008_bib8","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1016\/S0169-409X(02)00144-8","article-title":"An overview of the pathogenesis of cystic fibrosis lung disease","volume":"54","author":"Boucher","year":"2002","journal-title":"Adv Drug Del Rev"},{"key":"10.1016\/j.prrv.2007.02.008_bib9","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1097\/00063198-200311000-00007","article-title":"Update on pathogenesis of cystic fibrosis lung disease","volume":"9","author":"Donaldson","year":"2003","journal-title":"Curr Opin Pulm Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib10","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1126\/science.7543698","article-title":"CFTR as a cAMP-dependent regulator of sodium channels","volume":"269","author":"Stutts","year":"1995","journal-title":"Science"},{"key":"10.1016\/j.prrv.2007.02.008_bib11","first-page":"L372","article-title":"Inhibition of airway liquid secretion and its effect on the physical properties of airway mucus","volume":"272","author":"Trout","year":"1997","journal-title":"Am J Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib12","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1016\/S0092-8674(00)81724-9","article-title":"Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease","volume":"95","author":"Matsui","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.prrv.2007.02.008_bib13","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1172\/JCI2687","article-title":"Coordinated clearance of periciliary liquid and mucus from airway surfaces","volume":"102","author":"Matsui","year":"1998","journal-title":"J Clin Invest"},{"key":"10.1016\/j.prrv.2007.02.008_bib14","doi-asserted-by":"crossref","first-page":"1633","DOI":"10.1164\/ajrccm\/148.6_Pt_1.1633","article-title":"Elemental composition of human airway surface fluid in healthy and diseased airways","volume":"148","author":"Joris","year":"1993","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.prrv.2007.02.008_bib15","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/S0092-8674(00)81099-5","article-title":"Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid","volume":"85","author":"Smith","year":"1996","journal-title":"Cell"},{"key":"10.1016\/j.prrv.2007.02.008_bib16","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1016\/S0092-8674(00)81895-4","article-title":"Human \u03b2-defensin-1 is a salt-sensitive antibiotic in lung that is inactivated in cystic fibrosis","volume":"88","author":"Goldman","year":"1997","journal-title":"Cell"},{"key":"10.1016\/j.prrv.2007.02.008_bib17","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1016\/S1097-2765(00)80284-1","article-title":"Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro","volume":"2","author":"Zabner","year":"1998","journal-title":"Mol Cell"},{"key":"10.1016\/j.prrv.2007.02.008_bib18","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1172\/JCI2410","article-title":"Human \u03b2-defensin 2 is a salt-sensitive peptide antibiotic expressed in human lung","volume":"102","author":"Bals","year":"1998","journal-title":"J Clin Invest"},{"key":"10.1016\/j.prrv.2007.02.008_bib19","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1016\/S1097-2765(01)00286-6","article-title":"The CF salt controversy: In vivo observations and therapeutic approaches","volume":"8","author":"Tarran","year":"2001","journal-title":"Mol Cell"},{"key":"10.1016\/j.prrv.2007.02.008_bib20","doi-asserted-by":"crossref","first-page":"C469","DOI":"10.1152\/ajpcell.1999.276.2.C469","article-title":"Airway surface fluid volume and Cl content in cystic fibrosis and normal bronchial xenografts","volume":"276","author":"Zhang","year":"1999","journal-title":"Am J Physiol Cell Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib21","doi-asserted-by":"crossref","first-page":"2588","DOI":"10.1172\/JCI119802","article-title":"Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects","volume":"100","author":"Knowles","year":"1997","journal-title":"J Clin Invest"},{"key":"10.1016\/j.prrv.2007.02.008_bib22","doi-asserted-by":"crossref","first-page":"1633","DOI":"10.1164\/ajrccm\/148.6_Pt_1.1633","article-title":"Elemental composition of human airway surface fluid in health and diseased airways","volume":"148","author":"Joris","year":"1993","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.prrv.2007.02.008_bib23","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1164\/ajrccm.157.1.9703045","article-title":"Elemental content of airway surface liquid from infants with cystic fibrosis","volume":"157","author":"Hull","year":"1998","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib24","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1172\/JCI11154","article-title":"Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH","volume":"107","author":"Jayaraman","year":"2001","journal-title":"J Clin Invest"},{"key":"10.1016\/j.prrv.2007.02.008_bib25","doi-asserted-by":"crossref","first-page":"1286","DOI":"10.1152\/japplphysiol.00362.2005","article-title":"Epithelial lining fluid solute concentrations in chronic obstructive lung disease patients and normal subjects","volume":"99","author":"Effros","year":"2005","journal-title":"J Appl Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib26","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1085\/jgp.117.5.423","article-title":"Airway surface liquid osmolality measured using fluorophore-encapsulated liposomes","volume":"117","author":"Jayaraman","year":"2001","journal-title":"J Gen Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib27","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1378\/chest.107.3.747","article-title":"Effects of guaifenesin on nasal mucociliary clearance and ciliary beat frequency in healthy volunteers","volume":"107","author":"Sisson","year":"1995","journal-title":"Chest"},{"key":"10.1016\/j.prrv.2007.02.008_bib28","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1136\/thx.28.6.742","article-title":"A preliminary study of the effect of guaifenesin on mucociliary clearance from the human lung","volume":"28","author":"Thomson","year":"1973","journal-title":"Thorax"},{"key":"10.1016\/j.prrv.2007.02.008_bib29","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1378\/chest.109.2.348","article-title":"Iodinated glycerol has no effect on pulmonary function, symptom score, or sputum properties in patients with stable bronchitis","volume":"109","author":"Rubin","year":"1996","journal-title":"Chest"},{"key":"10.1016\/j.prrv.2007.02.008_bib30","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1002\/ppul.1950010209","article-title":"The effect of systemic hydration on normal and impaired mucociliary function","volume":"1","author":"Marchette","year":"1985","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib31","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1164\/arrd.1980.122.5P2.79","article-title":"Clinical indications for and effects of bland, mucolytic and antimicrobial aerosols","volume":"122","author":"Wanner","year":"1980","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.prrv.2007.02.008_bib32","first-page":"73","article-title":"Clinical aspects of mucociliary transport","volume":"116","author":"Wanner","year":"1977","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.prrv.2007.02.008_bib33","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1164\/ajrccm\/137.1.119","article-title":"Bronchodilator responsiveness in infants and young children with cystic fibrosis","volume":"137","author":"Hiatt","year":"1988","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.prrv.2007.02.008_bib34","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1183\/09031936.93.03101114","article-title":"Bronchial response to nebulized antibiotics in children with cystic fibrosis","volume":"3","author":"Chua","year":"1990","journal-title":"Eur Respir J"},{"key":"10.1016\/j.prrv.2007.02.008_bib35","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1136\/thx.48.8.812","article-title":"Effect of amiloride and saline on nasal mucociliary clearance and potential difference in cystic fibrosis and normal subjects","volume":"48","author":"Middleton","year":"1993","journal-title":"Thorax"},{"key":"10.1016\/j.prrv.2007.02.008_bib36","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1164\/rccm.200204-293OC","article-title":"Increasing concentration of inhaled saline with or without amiloride: Effect on mucociliary clearance in normal subjects","volume":"167","author":"Sood","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib37","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1097\/00005537-199704000-00013","article-title":"Mucociliary clearance and buffered hypertonic saline solution","volume":"107","author":"Talbot","year":"1997","journal-title":"Laryngoscope"},{"key":"10.1016\/j.prrv.2007.02.008_bib38","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1378\/chest.103.3.805","article-title":"Reduced effect of inhaled beta 2-adrenergic agonists on lung mucociliary clearance in patients with cystic fibrosis","volume":"103","author":"Mortensen","year":"1993","journal-title":"Chest"},{"key":"10.1016\/j.prrv.2007.02.008_bib39","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1056\/NEJMoa043891","article-title":"Mucus clearance and lung function in cystic fibrosis with hypertonic saline","volume":"354","author":"Donaldson","year":"2006","journal-title":"N Engl J Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib40","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1164\/ajrccm.156.1.9512074","article-title":"Rheology of cystic fibrosis sputum after in vitro treatment with hypertonic saline alone and in combination with recombinant human deoxyribonuclease I","volume":"156","author":"King","year":"1997","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib41","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1172\/JCI119138","article-title":"Sodium chloride increases the ciliary transportability of cystic fibrosis and bronchiectasis sputum on the mucus-depleted bovine trachea","volume":"99","author":"Wills","year":"1997","journal-title":"J Clin Invest"},{"key":"10.1016\/j.prrv.2007.02.008_bib42","first-page":"319","article-title":"Reduction of Pseudomonas aeruginosa biofilm formation under osmotic stress","volume":"S29","author":"Anderson","year":"2006","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib43","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1164\/ajrccm.153.5.8630593","article-title":"Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis","volume":"153","author":"Robinson","year":"1996","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib44","doi-asserted-by":"crossref","first-page":"900","DOI":"10.1136\/thx.52.10.900","article-title":"Effect of increasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis","volume":"52","author":"Robinson","year":"1997","journal-title":"Thorax"},{"key":"10.1016\/j.prrv.2007.02.008_bib45","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1113\/jphysiol.1986.sp016295","article-title":"The effect of airflow on mucus secretion into the trachea of the cat","volume":"380","author":"Peatfield","year":"1986","journal-title":"J Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib46","doi-asserted-by":"crossref","first-page":"2788","DOI":"10.1152\/jappl.1993.74.6.2788","article-title":"Osmolality affects ion and water fluxes and secretion in the ferret trachea","volume":"74","author":"Price","year":"1993","journal-title":"J Appl Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib47","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1165\/rcmb.2003-0345OC","article-title":"MUC5AC and MUC5B mucins are decreased in cystic fibrosis airway secretions","volume":"31","author":"Henke","year":"2004","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.prrv.2007.02.008_bib48","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1002\/ppul.10079","article-title":"Mucociliary clearance in cystic fibrosis","volume":"33","author":"Robinson","year":"2002","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib49","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1002\/(SICI)1099-0496(199602)21:2<77::AID-PPUL3>3.0.CO;2-M","article-title":"Short-term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis","volume":"21","author":"Eng","year":"1996","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib50","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1056\/NEJMoa043900","article-title":"The National Hypertonic Saline in Cystic Fibrosis Study Group. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis","volume":"354","author":"Elkins","year":"2006","journal-title":"N Engl J Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib51","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1097\/00005650-199206000-00002","article-title":"The MOS 36-item short-form health survey: conceptual framework and item selection","volume":"30","author":"Ware","year":"1992","journal-title":"Med Care"},{"key":"10.1016\/j.prrv.2007.02.008_bib52","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1093\/jpepsy\/25.6.403","article-title":"Translation and linguistic validation of a disease-specific quality of life measure for cystic fibrosis","volume":"25","author":"Quittner","year":"2000","journal-title":"J Pediatr Psychol"},{"key":"10.1016\/j.prrv.2007.02.008_bib53","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1055\/s-2004-815667","article-title":"Aerosolized antibiotics in cystic fibrosis","volume":"24","author":"Sexauer","year":"2003","journal-title":"Semin Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib54","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1002\/(SICI)1099-0496(199605)21:5<282::AID-PPUL3>3.0.CO;2-P","article-title":"Airway responsiveness to hyperosmolar saline challenge in cystic fibrosis: a pilot study","volume":"21","author":"Rodwell","year":"1996","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib55","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/S1569-1993(01)00009-1","article-title":"Hypertonic saline and recombinant human DNase: a randomised cross-over pilot study in patients with cystic fibrosis","volume":"1","author":"Ballmann","year":"2002","journal-title":"J Cystic Fibrosis"},{"key":"10.1016\/j.prrv.2007.02.008_bib56","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1034\/j.1399-3003.2000.16a16.x","article-title":"Sputum induction in young cystic fibrosis patients","volume":"16","author":"De Boeck","year":"2000","journal-title":"Eur Respir J"},{"key":"10.1016\/j.prrv.2007.02.008_bib57","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1136\/thorax.56.4.306","article-title":"Sputum induction as a research tool for sampling the airways of subjects with cystic fibrosis","volume":"56","author":"Henig","year":"2001","journal-title":"Thorax"},{"key":"10.1016\/j.prrv.2007.02.008_bib58","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1164\/rccm.200306-731OC","article-title":"Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis","volume":"168","author":"Ordonez","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib59","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1016\/S0140-6736(01)06412-1","article-title":"Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: a randomised trial","volume":"358","author":"Suri","year":"2001","journal-title":"Lancet"},{"key":"10.1016\/j.prrv.2007.02.008_bib60","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1097\/01.mcp.0000245714.89632.b2","article-title":"Inhaled hypertonic saline as a therapy for cystic fibrosis","volume":"12","author":"Elkins","year":"2006","journal-title":"Curr Opin Pulm Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib61","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1164\/ajrccm.156.3.9701113","article-title":"A new method for bronchial-provocation testing in asthmatic subjects using a dry powder of mannitol","volume":"156","author":"Anderson","year":"1997","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib62","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1152\/jappl.2001.90.3.821","article-title":"Ion transport and regulation of respiratory tract fluid output in dogs","volume":"90","author":"Chen","year":"2001","journal-title":"J Appl Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib63","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1016\/0091-6749(83)90626-7","article-title":"Effects of chemical mediators of anaphylaxis on ciliary function","volume":"72","author":"Wanner","year":"1983","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.prrv.2007.02.008_bib64","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1183\/09031936.03.00113403","article-title":"Evidence of mast cell activation and leukotriene release after mannitol inhalation","volume":"22","author":"Brannan","year":"2003","journal-title":"Eur Respir J"},{"key":"10.1016\/j.prrv.2007.02.008_bib65","doi-asserted-by":"crossref","first-page":"1475","DOI":"10.1016\/S0169-409X(02)00156-4","article-title":"Pharmacological approaches to discovery and development of new mucolytic agents","volume":"54","author":"King","year":"2002","journal-title":"Adv Drug Delivery Rev"},{"key":"10.1016\/j.prrv.2007.02.008_bib66","series-title":"Cilia, mucus and mucociliary interactions","first-page":"281","article-title":"Ciliary transportability is governed by its osmolality","author":"Wills","year":"1998"},{"key":"10.1016\/j.prrv.2007.02.008_bib67","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1034\/j.1399-3003.1999.14c30.x","article-title":"The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: a pilot study","volume":"14","author":"Robinson","year":"1999","journal-title":"Eur Respir J"},{"key":"10.1016\/j.prrv.2007.02.008_bib68","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1165\/ajrcmb.15.1.8679217","article-title":"Pseudomonas aeruginosa and epithelial permeability: role of virulence factors elastase and exotoxin A","volume":"15","author":"Azghani","year":"1996","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"Suppl 1","key":"10.1016\/j.prrv.2007.02.008_bib69","doi-asserted-by":"crossref","first-page":"S11","DOI":"10.1016\/S1569-1993(06)80042-1","article-title":"Inhaled dry powder mannitol (Bronchitol) improves FEV1 in cystic fibrosis","volume":"5","author":"Charlton","year":"2006","journal-title":"J Cystic Fibrosis"},{"key":"10.1016\/j.prrv.2007.02.008_bib70","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/0003-9861(70)90371-1","article-title":"Transport and phosphorylation of glucose, fructose and mannitol by Pseudomonas aeruginosa","volume":"138","author":"Phibbs","year":"1970","journal-title":"Arch Biochem Biophys"},{"key":"10.1016\/j.prrv.2007.02.008_bib71","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1139\/o71-151","article-title":"Kinetics of transport of glucose, fructose and mannitol by Pseudomonas aeruginosa","volume":"49","author":"Eagon","year":"1971","journal-title":"Can J Biochem"},{"key":"10.1016\/j.prrv.2007.02.008_bib72","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1128\/IAI.37.2.662-669.1982","article-title":"Utilization of human respiratory secretions by mucoid Pseudomonas aeruginosa of cystic fibrosis origin","volume":"37","author":"Ohman","year":"1982","journal-title":"Infect Immun"},{"key":"10.1016\/j.prrv.2007.02.008_bib73","doi-asserted-by":"crossref","first-page":"11614","DOI":"10.1073\/pnas.97.21.11614","article-title":"The osmolyte xylitol reduces the salt concentration of airway surface liquid and may enhance bacterial killing","volume":"97","author":"Zabner","year":"2000","journal-title":"Proc Nat Acad Sci USA"},{"key":"10.1016\/j.prrv.2007.02.008_bib74","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1089\/jam.2006.19.100","article-title":"Hyperosmolar agents and clearance of mucus in the diseased airway","volume":"19","author":"Daviskas","year":"2006","journal-title":"J Aerosol Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib75","doi-asserted-by":"crossref","first-page":"1445","DOI":"10.1016\/S0169-409X(02)00161-8","article-title":"Altering airway surface liquid volume: inhalation therapy with amiloride and hyperosmotic agents","volume":"54","author":"Hirsh","year":"2002","journal-title":"Adv Drug Del Rev"},{"key":"10.1016\/j.prrv.2007.02.008_bib76","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1056\/NEJM199108223250802","article-title":"Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis","volume":"325","author":"Knowles","year":"1991","journal-title":"N Engl J Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib77","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1165\/ajrcmb.23.6.4207","article-title":"Inhibition of amiloride-sensitive epithelial Na+ absorption by extracellular nucleotides in human normal and cystic fibrosis airways","volume":"23","author":"Mall","year":"2000","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.prrv.2007.02.008_bib78","doi-asserted-by":"crossref","first-page":"C827","DOI":"10.1152\/ajpcell.1999.276.4.C827","article-title":"UTP inhibits Na+ absorption in wild-type and Delta F508 CFTR-expressing human bronchial epithelia","volume":"276","author":"Devor","year":"1999","journal-title":"Am J Physiol Cell Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib79","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1183\/09031936.96.09030542","article-title":"Nucleotide-induced mucin release from primary hamster tracheal surface epithelial cells involves the P2u purinoceptor","volume":"9","author":"Kim","year":"1996","journal-title":"Eur Respir J"},{"key":"10.1016\/j.prrv.2007.02.008_bib80","doi-asserted-by":"crossref","first-page":"C1485","DOI":"10.1152\/ajpcell.2001.280.6.C1485","article-title":"Differential effects of UTP, ATP, and adenosine on ciliary activity of human nasal epithelial cells","volume":"280","author":"Morse","year":"2001","journal-title":"Am J Physiol Cell Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib81","doi-asserted-by":"crossref","first-page":"L625","DOI":"10.1152\/ajplung.1994.267.5.L625","article-title":"P2u purinoceptor stimulation of surfactant secretion coupled to phosphatidylcholine hydrolysis in type II cells","volume":"267","author":"Gobran","year":"1994","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib82","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1124\/jpet.102.035485","article-title":"Pharmacology of INS37217 [P1-(uridine 5\u2032)-P4- (2\u2032-deoxycytidine 5\u2032)tetraphosphate, tetrasodium salt], a next-generation P2Y2 receptor agonist for the treatment of cystic fibrosis","volume":"302","author":"Yerxa","year":"2002","journal-title":"J Pharmacol Exp Ther"},{"key":"10.1016\/j.prrv.2007.02.008_bib83","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1002\/ppul.20192","article-title":"Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: Results of a phase 1\/phase 2 multicenter study in mild to moderate cystic fibrosis","volume":"39","author":"Deterding","year":"2005","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib84","first-page":"249","article-title":"Safety and efficacy of INS37217 inhalation solution, a novel P2Y2 receptor agonist, in patients with mild cystic fibrosis: Results of a phase 2 multi-center study","volume":"38","author":"Deterding","year":"2004","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib85","first-page":"306","article-title":"Safety and tolerability of denufosol inhalation solution: Results of a 28-day phase 2 study in 5-7 year old patients in CF","volume":"41","author":"Deterding","year":"2006","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.prrv.2007.02.008_bib86","doi-asserted-by":"crossref","first-page":"1776","DOI":"10.1152\/jappl.1985.58.6.1776","article-title":"Clearance of mucus by simulated cough","volume":"58","author":"King","year":"1985","journal-title":"J Appl Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib87","doi-asserted-by":"crossref","first-page":"737","DOI":"10.3233\/BIR-1989-26406","article-title":"The role of mucus gel viscosity, spinnability, and adhesive properties in clearance simulated cough","volume":"26","author":"King","year":"1989","journal-title":"Biorheology"},{"key":"10.1016\/j.prrv.2007.02.008_bib88","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1172\/JCI112708","article-title":"Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation","volume":"78","author":"Boucher","year":"1986","journal-title":"J Clin Invest"},{"key":"10.1016\/j.prrv.2007.02.008_bib89","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1113\/jphysiol.1988.sp017322","article-title":"Evidence for reduced Cl\u2212 and increased Na+ permeability in cystic fibrosis human primary cell cultures","volume":"405","author":"Boucher","year":"1988","journal-title":"J Physiol"},{"key":"10.1016\/j.prrv.2007.02.008_bib90","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1038\/322467a0","article-title":"Chloride and potassium channels in cystic fibrosis airway epithelia","volume":"322","author":"Welsh","year":"1986","journal-title":"Nature"},{"key":"10.1016\/j.prrv.2007.02.008_bib91","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1164\/ajrccm.154.1.8680672","article-title":"The effects of albuterol on the lung function of hospitalized patients with cystic fibrosis","volume":"154","author":"Hordvik","year":"1996","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib92","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1136\/thorax.57.8.742","article-title":"\u201cCF asthma\u201d: what is it and what do we do about it?","volume":"57","author":"Balfour-Lynn","year":"2002","journal-title":"Thorax"},{"key":"10.1016\/j.prrv.2007.02.008_bib93","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1385\/CRIAI:23:1:077","article-title":"Airways reactivity in patients with CF","volume":"23","author":"Weinberger","year":"2002","journal-title":"Clin Rev Allergy Immunol"},{"key":"10.1016\/j.prrv.2007.02.008_bib94","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1016\/S0022-3476(78)81070-1","article-title":"Bronchial hyperreactivity in cystic fibrosis and asthma","volume":"93","author":"Mitchell","year":"1978","journal-title":"J Pediatr"},{"key":"10.1016\/j.prrv.2007.02.008_bib95","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1136\/adc.56.6.455","article-title":"Variability of airways hyper-reactivity and allergy in cystic fibrosis","volume":"56","author":"Holzer","year":"1981","journal-title":"Arch Dis Child"},{"key":"10.1016\/j.prrv.2007.02.008_bib96","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1016\/S0022-3476(73)80085-X","article-title":"The variable effect of a bronchodilating agent on pulmonary function in cystic fibrosis","volume":"82","author":"Landau","year":"1973","journal-title":"J Pediatr"},{"key":"10.1016\/j.prrv.2007.02.008_bib97","doi-asserted-by":"crossref","first-page":"1356","DOI":"10.1164\/rccm.200511-1808OC","article-title":"Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis","volume":"173","author":"Balfour-Lynn","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.prrv.2007.02.008_bib98","unstructured":"Halfhide C, Evans HJ, Couriel J. Inhaled bronchodilators for cystic fibrosis. The Cochrane Library 2007(1)."},{"key":"10.1016\/j.prrv.2007.02.008_bib99","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1164\/arrd.1985.131.4.537","article-title":"Bronchodilators increase airway instability in cystic fibrosis","volume":"131","author":"Zach","year":"1985","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.prrv.2007.02.008_bib100","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/S0140-6736(03)12352-5","article-title":"Design of clinical trials in cystic fibrosis","volume":"361","author":"Southern","year":"2003","journal-title":"Lancet"}],"container-title":["Paediatric Respiratory Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1526054207000097?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1526054207000097?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2023,5,11]],"date-time":"2023-05-11T05:32:14Z","timestamp":1683783134000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1526054207000097"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,3]]},"references-count":100,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2007,3]]}},"alternative-id":["S1526054207000097"],"URL":"https:\/\/doi.org\/10.1016\/j.prrv.2007.02.008","relation":{},"ISSN":["1526-0542"],"issn-type":[{"value":"1526-0542","type":"print"}],"subject":[],"published":{"date-parts":[[2007,3]]}}}