Potential of anti-inflammatory treatment for cystic fibrosis lung disease

References

• DavisPBDrummMKonstanMWCystic fibrosis: state of the artAm J Respir Crit Care Med1996154122912568912731
   PubMed Web of Science ®Google Scholar
• BedrossianCWGreenbergSDSingerDBThe lung in cystic fibrosis. A quantitative study including prevalence or pathologic findings among different age groupsHum Pathol1976721952041262016
   PubMed Web of Science ®Google Scholar
• KhanTZWagenerJSBostTEarly pulmonary inflammation in infants with cystic fibrosisAm J Respir Crit Care Med1995151107510827697234
   PubMed Web of Science ®Google Scholar
• BonfieldTLPanuskaJRKonstanMWInflammatory cytokines in cystic fibrosis lungsAm J Respir Crit Care Med1995152211121188520783
   PubMed Web of Science ®Google Scholar
• KonstanMWHilliardKANorvellTMBronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammationAm J Respir Crit Care Med19941504484548049828
   PubMed Web of Science ®Google Scholar
• BaloughKMcCubbinMWeinbergerMThe relationship between infection and inflammation in the early stages of lung disease from cystic fibrosisPediatr Pulmonol19952063708570304
   PubMed Web of Science ®Google Scholar
• ArmstrongDSGrimwoodKCarlinJBLower airway inflammation in infants and young children with cystic fibrosisAm J Respir Crit Care Med1997156119712049351622
   PubMed Web of Science ®Google Scholar
• MuhlebachMSStewartPWLeighMWQuantitation of inflammatory response to bacteria in young cystic fibrosis and control patientsAm J Respir Crit Care Med199916018619110390398
   PubMed Web of Science ®Google Scholar
• AuerbachHSWilliamsMKirkpatrickJAAlternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosisLancet1985284576866882863676
   PubMed Web of Science ®Google Scholar
• EigenHRosensteinBJFitzSimmonsSA multicenter study of alternate- day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial GroupJ Pediatr199512645155237699528
   PubMed Web of Science ®Google Scholar
• KonstanMWByardPJHoppelCLEffect of high-dose ibuprofen in patients with cystic fibrosisN Engl J Med19953328488447503838
   PubMed Web of Science ®Google Scholar
• DonatiMAHaverKGersonWLong-term alternate day prednisone therapy in cystic fibrosisPediatr Pulmonol.19905A322
   Google Scholar
• LandsLCMilnerRCantinAMHigh-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trialJ Pediatr200715122823017719925
   PubMed Web of Science ®Google Scholar
• KonstanMWSchluchterMDXueWClinical use of ibuprofen is associated with slower FEV1 decline in children with cystic fibrosisAm J Respir Crit Care Med20071761084108917872492
   PubMed Web of Science ®Google Scholar
• NoahTLBlackHRChengPWNasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosisJ Infect Dis19971756386479041336
   PubMed Web of Science ®Google Scholar
• PerezAIsslerACCottonCUCFTR inhibition mimics the cystic fibrosis inflammatory profileAm J Physiol Lung Cell Mol Physiol2007292L38339516920886
   PubMed Web of Science ®Google Scholar
• DechecchiMCNicolisEBezzerriVMPB-07 reduces the inflammatory response to Pseudomonas aeruginosa in cystic fibrosis bronchial cellsAm J Respir Cell Mol Biol20073661562417197571
   PubMed Web of Science ®Google Scholar
• van HeeckerenAWalengaRKonstanMWExcessive inflammatory response on cystic fibrosis mice to bronchopulmonary infection with P. aeruginosaJ Clin Invest1997100281028159389746
   PubMed Web of Science ®Google Scholar
• McMorranBJPalmerJSLunnDPG551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung diseaseAm J Physiol Lung Cel Mol Physiol2001281L740747
   PubMed Web of Science ®Google Scholar
• VerhaegheCRemouchampsCHennuyBRole of IKK and ERK pathways in intrinsic inflammation of cystic fibrosis airwaysBiochem Pharmacol2007731982199417466952
   PubMed Web of Science ®Google Scholar
• LiJJohnsonXDLazvovskaiaSSignaling intermediates required for NFκB activation and IL-8 expression in CF bronchial epithelial cellsAm J Physiol Lung Cell Mol Physiol2003284L30731512388360
   PubMed Web of Science ®Google Scholar
• EscotteSTabaryODusserDFluticasone reduces IL-6 and IL-8 production of cystic fibrosis bronchial epithelial cells via IKK-beta kinase pathwayEur Respir J20032157458112762338
   PubMed Web of Science ®Google Scholar
• RibeiroCMParadisoAMSchwabUChronic airway infection/ inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epitheliaJ Biol Chem2005280177981780615746099
   PubMed Web of Science ®Google Scholar
• BonfieldTLKonstanMWBurfeindPNormal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosisAm J Respir Cell Mol Biol1995132572617544594
   PubMed Web of Science ®Google Scholar
• BonfieldTLKonstanMWBergerMAltered respiratory epithelial cell cytokine production in cystic fibrosisJ Allergy Clin Immunol19991041727810400842
   PubMed Web of Science ®Google Scholar
• MooreKWde Waal-MalefytRCoffmanRLInterleukin-10 and the interleukin-10 receptorAnnu Rev Immunol20011968376511244051
   PubMed Web of Science ®Google Scholar
• OsikaECavaillonJMChadelatKDistinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway diseaseEur Respir J19991433934610515411
   PubMed Web of Science ®Google Scholar
• LentschABShanleyTPSarmaVIn vivo suppression of NFkB and preservation of IkBalpha by interleukin-10 and interleukin-13J Clin Invest1997100244324489366558
   PubMed Web of Science ®Google Scholar
• SchotteliusAJGMayoMWSartorRBInterleukin-10 signaling blocks inhibitor of kB kinase activity and nuclear factor kB DNA bindingJ Biol Chem1999274318683187410542212
   PubMed Web of Science ®Google Scholar
• AnderssonCZamanMMJonesABAlterations in immune respnse and PPAR/LXR regulation in cystic fibrosis macrophagesJ Cyst Fibros200871687817889625
   PubMed Web of Science ®Google Scholar
• OlleroMJunaidiOZamanMMDecreased expression of peroxisome proliferator activated receptor gamma in cftr−/− miceJ Cell Physiol200420023524415174093
   PubMed Web of Science ®Google Scholar
• KarpCLFlickLMParkKWDefective lipoxin-mediated antiinflammatory activity in the cystic fibrosis airwayNat Immunol2004535735815052263
   PubMed Web of Science ®Google Scholar
• StarostaVRatjenFRietschelEAnti-inflammatory cytokines in cystic fibrosis lung diseaseEur Respir J20062858158716807263
   PubMed Web of Science ®Google Scholar
• DubinPJMcAllisterFKollsJKIs cystic fibrosis a TH17 diseaseInflamm Res20075622122717607545
   PubMed Web of Science ®Google Scholar
• DubinPJKollsJKIL-23 mediates inflammatory responses to mucoid pseudomonas aeruginosa lung infection in miceAm J Physiol Lung Cel Mol Physiol2007292L519523
   PubMed Web of Science ®Google Scholar
• McAllisterFHenryAKreindlerJLRole of IL-17A, IL-17F, and the IL-17 receptor in regulating growth related oncogene-alpha and granulocyte colony-stimulating factor in bronchial epithelium: implications for airway inflammation in cystic fibrosisJ Immunol200517540441215972674
   PubMed Web of Science ®Google Scholar
• RousselLRousseauSIL-17 primes airway epithelial cells lacking functional CFTR in increase NOD1 responsesBiochem Biophys Res Commun2010391150550919931506
   PubMed Web of Science ®Google Scholar
• FujisawaTVelichkoSThaiPRegulation of airway MUC5AC expression by IL-1beta and IL-17A; the NF-kappaB paradigmJ Immunol2009183106236624319841186
   PubMed Web of Science ®Google Scholar
• FerrettiSBonneauODuboisGRIL-17, produced by lymphocytes and neutrophils, is necessary for lipopolysaccharide induced airway neutrophilia: IL-15 as a possible triggerJ Immunol20031702106211212574382
   PubMed Web of Science ®Google Scholar
• ChenJKinterMShankSDysfunction of Nrf-2 in CF epithelia leads to excess intracellular H2O2 and inflammatory cytokine productionPLoS ONE.20083e336718846238
   PubMed Web of Science ®Google Scholar
• LiQEngelhardtJFInterleukin-1B induction of NFkB is partially regulated by H2O2-mediated activation of NFkB-inducing kinaseJ Biol Chem20062811495150516286467
   PubMed Web of Science ®Google Scholar
• Balfour-LynnIMLavertyADinwiddieRReduced upper airway nitric oxide in cystic fibrosisArch Dis Child1996753193228984918
   PubMed Web of Science ®Google Scholar
• GrasemannHMichlerEWallotMDecreased concentration of exhaled nitric oxide (NO) in patients with cystic fibrosisPediatr Pulmonol1997241731779330413
   PubMed Web of Science ®Google Scholar
• RoumJHBuhlRMcElvaneyNGSystemic deficiency of glutathione in cystic fibrosisJ Appl Physiol199375241924248125859
   PubMed Web of Science ®Google Scholar
• GaoLKimKJYankaskasJRAbnormal glutathione transport in cystic fibrosis airway epitheliaAm J Physiol1999277L11311810409237
   PubMed Web of Science ®Google Scholar
• NicholsDPZiadyAGShankSLThe triterpenoid CDDO limits inflammation in preclinical models of cystic fibrosis lung diseaseAm J Physiol Lung Cel Mol Physiol20092975L828836
   Web of Science ®Google Scholar
• IshiiYItohKMorishimaYTranscription factor Nrf2 plays a pivotal role in protection against elastase-induced pulmonary inflammation and emphysemaJ Immunol20051756968697516272357
   PubMed Web of Science ®Google Scholar
• GaggarAJacksonPLNoeragerBDA novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammationJ Immunol20081805662566918390751
   PubMed Web of Science ®Google Scholar
• JacksonPLXuXWilsonLHuman neutrophil elastase-mediated cleavage sites on MMP-9 and TIMP-1: implications to cystic fibrosis proteolytic dysfunctionMol Med2010 Epub 2010 Jan 29
   Web of Science ®Google Scholar
• WeldonSMcNallyPMcElvaneyNGDecreased levels of secretory leucoprotease inhibitor in the pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degredationJ Immunol200918381468156
   Web of Science ®Google Scholar
• GoyotNButlerMWMcNallyPElafin, an elastase-specific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic fibrosisJ Biol Chem200828347323773238518799464
   PubMed Web of Science ®Google Scholar
• OermannCMSockriderMMKonstanMWThe use of anti-inflammatory medications in cystic fibrosis: trends, and physician attitudesChest19991151053105810208207
   PubMed Web of Science ®Google Scholar
• MatthewsWJWilliamsMOliphintBHypogammaglobulinemia in patients with cystic fibrosisN Engl J Med19803022452497350477
   PubMed Web of Science ®Google Scholar
• EigenHRosensteinBJFitzSimmonsSA multicenter study of alternate-day prednisone therapy in patients with cystic fibrosisJ Pediatr199512645155237699528
   PubMed Web of Science ®Google Scholar
• RosensteinBJEigenHRisks of alternate day prednisone in patients with cystic fibrosisPediatr199187245246
   PubMed Web of Science ®Google Scholar
• LaiHCFitzSimmonsSCAllenDBRisk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosisN Engl J Med200034285185910727589
   PubMed Web of Science ®Google Scholar
• GreallyPHussainMJVerganiDInterleukin-1alpha, soluble interleukin-2 receptor, and IgG concentrations in cystic fibrosis treated with prednisoloneArch Dis Child19947135398067791
   PubMed Web of Science ®Google Scholar
• Balfour-LynnIMWelchKInhaled corticosteroids for cystic fibrosisCochrane Database Syst Rev20091
   Web of Science ®Google Scholar
• KonstanMWVargoKMDavisPBIbuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in a rat model of chroic pulmonary infectionAm Rev Respir Dis19901411861922153353
   PubMed Web of Science ®Google Scholar
• ScheurenNBangHMunsterTModulation of transcription factor NF-kappaB by enantiomers of the nonsteroidal drug ibuprofenBr J Pharmacol19981236456529517383
   PubMed Web of Science ®Google Scholar
• TegederINiederbergerEIsrarEInhibition of NF-kB and AP-1 activation by R- and S-flurbiprofenFASEB J20011559559711291649
   PubMed Web of Science ®Google Scholar
• SaimanLMarshallBCMayer-HamblettNAzithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trialJAMA20032901749175614519709
   PubMed Web of Science ®Google Scholar
• FlorescuDFMurphyPJKalilACEffects of prolonged use of azithromycin in patients with cystic fibrosis: a meta-analysisPulm Pharmacol Ther200922646747219328860
   PubMed Web of Science ®Google Scholar
• EquiABalfour-LynnIMBushALong term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trialLancet200236097898412383667
   PubMed Web of Science ®Google Scholar
• VerledenGMVanaudenaerdeBMDupontLJAzithromycin reduces airway neutrophilia and interleukin-8 in patients with bronchiolitis obliterans syndromeAm J Respir Crit Care Med200617456657016741151
   PubMed Web of Science ®Google Scholar
• TsaiWCRodriguezMLYoungKSAzithromycin blocks neutrophil recruitment in pseudomonas endobronchial infectionAm J Respir Crit Care Med20041701331133915361366
   PubMed Web of Science ®Google Scholar
• CulicOErakovicVCepelakIAzithromycin modulates neutrophil function and circulating inflammatory mediators in healthy human subjectsEur J Pharmacol200245027728912208321
   PubMed Web of Science ®Google Scholar
• HoffmannNLeeBHentzerMAzithromycin blocks quorum sensing and alginate polymer formation an dincreases the sensitivity to serum and stationary-growth-phase killing of pseudomonas aeruginosa and attenuates chronic P. aeruginosa lung infection in cftr(−/−) miceAntimicrob Agents Chemother2007513677368717620382
   PubMed Web of Science ®Google Scholar
• GavilanesXHuauxFMeyerMAzithromycin fails to reduce increased expression of neutrophil-related cytokines in primary-cultured epithelial cells from cystic fibrosis miceJ Cyst Fibros20098320321019345617
   PubMed Web of Science ®Google Scholar
• RibeiroCMHurdHWuYAzithromycin treatment alters gene expression in inflammatory, lipid metabolism, and cell cycle pathways in well-differentiated human airway epitheliaPLoS ONE.200946e580619503797
   PubMed Web of Science ®Google Scholar
• Tramper-StrandersGAWolfsTFFleerAMaintenance azithromycin treatment in pediatric patients with cystic fibrosis: long-term outcomes related to macrolide resistance and pulmonary functionPediatr Infect Dis J20072681217195698
   PubMed Web of Science ®Google Scholar
• SaimanLAnsteadMMayer-HamblettNEffect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trialJAMA20103031707171520442386
   PubMed Web of Science ®Google Scholar
• GruenertDCWillemsMCassimanJJEstablished cell lines used in cystic fibrosis researchJ Cyst Fibros20043Suppl 219119615463957
   PubMedGoogle Scholar
• DoringGElbornJSJohannessonMClinical trials in cystic fibrosisJ Cyst Fibros200762859917350898
   PubMed Web of Science ®Google Scholar
• DiMangoERatnerAJBryanRActivation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cellsJ Clin Invest199810111259826059616231
   PubMed Web of Science ®Google Scholar
• KubeDSontichUFletcherDProinflammatory cytokine responses to P. aeruginosa infection in human airway epithelial cell linesAm J Physiol Lung Cell Mol Physiol20012803L49350211159033
   PubMed Web of Science ®Google Scholar
• KammouniWFigarellaCMarchandSAltered cytokine production by cystic fibrosis tracheal gland serous cellsInfect Immun19976512517651839393813
   PubMed Web of Science ®Google Scholar
• WeberAJSoongGBryanRActivation of NF-kappaB in airway epithelial cells is dependent on CFTR trafficking and Cl-channel functionAm J Physiol Lung Cell Mol Physiol20012811L717811404248
   PubMed Web of Science ®Google Scholar
• BorgattiMBezzerriVManciniIInduction of IL-6 gene expression in a CF bronchial epithelial cell line by Pseudomonas aeruginosa is dependent on transcription factors belonging to the Sp1 superfamilyBiochem Biophys Res Commun2007357497798317466942
   PubMed Web of Science ®Google Scholar
• LiJJohnsonXDIazvovskaiaSSignaling intermediates required for NF-kappa B activation and IL-8 expression in CF bronchial epithelial cellsAm J Physiol Lung Cell Mol Physiol20032842L30731512388360
   PubMed Web of Science ®Google Scholar
• BannerKHDe JongeHElbornSHighlights of a workshop to discuss targeting inflammation in cystic fibrosisJ Cyst Fibros2009811819022708
   PubMed Web of Science ®Google Scholar
• BorthwickDWWestJDKeighrenMAMurine submucosal glands are clonally derived and show a cystic fibrosis gene-dependent distribution patternAm J Respir Cell Mol Biol19992061181118910340937
   PubMed Web of Science ®Google Scholar
• PackRJAl-UgailyLHMorrisGThe distribution and structure of cells in the tracheal epithelium of the mouseCell Tissue Res1980208165846248229
   PubMed Web of Science ®Google Scholar
• ScholteBJDavidsonDJWilkeMAnimal models of cystic fibrosisJ Cyst Fibros20043Suppl 218319015463956
   PubMedGoogle Scholar
• HarrisATowards an ovine model of cystic fibrosisHum Mol Genet1997613219121949361022
   PubMed Web of Science ®Google Scholar
• SunXYanZYiYAdeno-associated virus-targeted disruption of the CFTR gene in cloned ferretsJ Clin Invest200811841578158318324338
   PubMed Web of Science ®Google Scholar
• RogersCSStoltzDAMeyerholzDKDisruption of the CFTR gene produces a model of cystic fibrosis in newborn pigsScience262008321589718371841
   Google Scholar
• RogersCSHaoYRokhlinaTProduction of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transferJ Clin Invest200811841571157718324337
   PubMed Web of Science ®Google Scholar
• StoltzDAEarly Pulmonary Findings in a CFTR −/−Pig North American Cystic Fibrosis ConferenceMinneapolis, MNWiley-Blackwell2009191
   Google Scholar
• StoltzDAMeyerholzDKPezzuloAACystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birthSci Transl Med.2010229ra31
   PubMed Web of Science ®Google Scholar
• FibrosisFCCystic Fibrosis Foundation National Patient RegistryAnnual Data ReportBethesda, MD2007
   Google Scholar
• FarrellPMThe prevalence of cystic fibrosis in the European UnionJ Cyst Fibros20087545045318442953
   PubMed Web of Science ®Google Scholar
• McCormickJMehtaGOlesenHVComparative demographics of the European cystic fibrosis population: a cross-sectional database analysisLancet202010375971910071013
   Google Scholar
• ParkerACPritchardPPrestonTEnhanced drug metabolism in young children with cystic fibrosisArch Dis Child19977732392419370904
   PubMed Web of Science ®Google Scholar
• PrandotaJClinical pharmacology of antibiotics and other drugs in cystic fibrosisDrugs19883555425783293970
   PubMed Web of Science ®Google Scholar
• KearnsGLHepatic drug metabolism in cystic fibrosis: recent developments and future directionsAnn Pharmacother199327174798431626
   PubMed Web of Science ®Google Scholar
• EquiABalfour-LynnIMBushALong term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trialLancet2820023609338978984
   Google Scholar
• WolterJSeeneySBellSEffect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trialThorax200257321221611867823
   PubMed Web of Science ®Google Scholar
• McPhailGLActonJDFenchelMCImprovements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa useJ Pediatr2008153675275718760423
   PubMed Web of Science ®Google Scholar
• RabinHRButlerSMWohlMEPulmonary exacerbations in cystic fibrosisPediatr Pulmonol200437540040615095322
   PubMed Web of Science ®Google Scholar
• Mayer-HamblettNAitkenMLAccursoFJAssociation between pulmonary function and sputum biomarkers in cystic fibrosisAm J Respir Crit Care Med2007175882282817234902
   PubMed Web of Science ®Google Scholar
• QuittnerALBuuAMesserMADevelopment and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosisChest200512842347235416236893
   PubMed Web of Science ®Google Scholar
• Mayer-HamblettNRamseyBWKronmalRAAdvancing outcome measures for the new era of drug development in cystic fibrosisProc Am Thorac Soc20074437037717652504
   PubMedGoogle Scholar
• HendryJElbornJSNixonLCystic fibrosis: inflammatory response to infection with Burkholderia cepacia and Pseudomonas aeruginosaEur Respir J199914243543810515426
   PubMed Web of Science ®Google Scholar
• KollerDYGotzMWojnarowskiCRelationship between disease severity and inflammatory markers in cystic fibrosisArch Dis Child19967564985019014602
   PubMed Web of Science ®Google Scholar
• NormanDElbornJSCordonSMPlasma tumour necrosis factor alpha in cystic fibrosisThorax199146291952014508
   PubMed Web of Science ®Google Scholar
• BrownMAMorganWJFinleyPRCirculating levels of tumor necrosis factor and interleukin-1 in cystic fibrosisPediatr Pulmonol199110286912030926
   PubMed Web of Science ®Google Scholar
• DorlochterLCarlssonMOlafsdottirEJAnti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosisJ Cyst Fibros20043317918315463905
   PubMedGoogle Scholar
• ColomboCCostantiniDRocchiACytokine levels in sputum of cystic fibrosis patients before and after antibiotic therapyPediatr Pulmonol2005401152115858808
   PubMed Web of Science ®Google Scholar
• RoderfeldMRathTSchulzRSerum matrix metalloproteinases in adult CF patients: relation to pulmonary exacerbationJ Cyst Fibros20098533834719604728
   PubMed Web of Science ®Google Scholar
• JonesAMMartinLBright-ThomasRJInflammatory markers in cystic fibrosis patients with transmissible Pseudomonas aeruginosaEur Respir J200322350350614516142
   PubMed Web of Science ®Google Scholar
• VallettaEARigoABonazziLModification of some markers of inflammation during treatment for acute respiratory exacerbation in cystic fibrosisActa Paediatr19928132272301511195
   PubMed Web of Science ®Google Scholar
• DowneyDGMartinSLDempsterMThe relationship of clinical and inflammatory markers to outcome in stable patients with cystic fibrosisPediatr Pulmonol200742321622017238189
   PubMed Web of Science ®Google Scholar
• WilmottRWFrenzkeMKocielaVPlasma interleukin-1 alpha and beta, tumor necrosis factor-alpha, and lipopolysaccharide concentrations during pulmonary exacerbations of cystic fibrosisPediatr Pulmonol199418121277970903
   PubMed Web of Science ®Google Scholar
• McColleySAStellmachVBoasSRSerum vascular endothelial growth factor is elevated in cystic fibrosis and decreases with treatment of acute pulmonary exacerbationAm J Respir Crit Care Med200016161877188010852760
   PubMed Web of Science ®Google Scholar
• DeanTPDaiYShuteJKInterleukin-8 concentrations are elevated in bronchoalveolar lavage, sputum, and sera of children with cystic fibrosisPediatr Res19933421591618233718
   PubMed Web of Science ®Google Scholar
• GreallyPHussainMJVerganiDSerum interleukin-1 alpha and soluble interleukin-2 receptor concentrations in cystic fibrosisArch Dis Child19936867857878333775
   PubMed Web of Science ®Google Scholar
• KronborgGHansenMBSvensonMCytokines in sputum and serum from patients with cystic fibrosis and chronic Pseudomonas aeruginosa infection as markers of destructive inflammation in the lungsPediatr Pulmonol19931552922978327288
   PubMed Web of Science ®Google Scholar
• EichlerINilssonMRathRHuman neutrophil lipocalin, a highly specific marker for acute exacerbation in cystic fibrosisEur Respir J19991451145114910596704
   PubMed Web of Science ®Google Scholar
• DowneyDGBrockbankSMartinSLBroncoscopy Study Group The effect of treatment of cystic fibrosis pulmonary exacerbations on airways and systemic inflammationPediatr Pulmonol200742872973517588254
   PubMed Web of Science ®Google Scholar
• SagelSDChmielJFKonstanMWSputum biomarkers of inflammation in cystic fibrosis lung diseaseProc Am Thorac Soc20074440641717652508
   PubMedGoogle Scholar
• WagenerJSRockMJMcCubbinMMAerosol delivery and safety of recombinant human deoxyribonuclease in young children with cystic fibrosis: a bronchoscopic studyPulmozyme PediatricJ Pediatr19981334486491
   Web of Science ®Google Scholar
• WojtczakHAKerbyGSWagenerJSBeclomethasone diproprionate reduced airway inflammation without adrenal suppression in young children with cystic fibrosis: a pilot studyPediatr Pulmonol200132429330211568990
   PubMed Web of Science ®Google Scholar
• DouglasTABrennanSGardSAcquisition and eradication of P. aeruginosa in young children with cystic fibrosisEur Respir J200933230531119010992
   PubMed Web of Science ®Google Scholar
• GibsonRLEmersonJMayer-HamblettNDuration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosisPediatr Pulmonol200742761062317534969
   PubMed Web of Science ®Google Scholar
• RatjenFPaulKvan KoningsbruggenSDNA concentrations in BAL fluid of cystic fibrosis patients with early lung disease: influence of treatment with dornase alphaPediatr Pulmonol20053911415532079
   PubMed Web of Science ®Google Scholar
• PaulKRietschelEBallmannMEffect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosisAm J Respir Crit Care Med2004169671972514684561
   PubMed Web of Science ®Google Scholar
• RatjenFHartogCMPaulKMatrix metalloproteases in BAL fluid of patients with cystic fibrosis and their modulation by treatment with dornase alphaThorax2002571193093412403873
   PubMed Web of Science ®Google Scholar
• MeyerKCLewandoskiJRZimmermanJJHuman neutrophil elastase and elastase/alpha 1-antiprotease complex in cystic fibrosis. Comparison with interstitial lung disease and evaluation of the effect of intravenously administered antibiotic therapyAm Rev Respir Dis19911443 Pt 15805851892298
   PubMed Web of Science ®Google Scholar
• McElvaneyNGHubbardRCBirrerPAerosol alpha 1-antitrypsin treatment for cystic fibrosisLancet199133787383923941671425
   PubMed Web of Science ®Google Scholar
• McElvaneyNGNakamuraHBirrerPModulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitorJ Clin Invest1992904129613011357002
   PubMed Web of Science ®Google Scholar
• CarpagnanoGEBarnesPJFrancisJBreath condensate pH in children with cystic fibrosis and asthma: a new noninvasive marker of airway inflammation?Chest200412562005201015189915
   PubMed Web of Science ®Google Scholar
• KarakocGBInalAYilmazMExhaled breath condensate MMP-9 levels in children with bronchiectasisPediatr Pulmonol200944101010101619725099
   PubMed Web of Science ®Google Scholar
• RobroeksCMRosiasPPvan VlietDBiomarkers in exhaled breath condensate indicate presence and severity of cystic fibrosis in childrenPediatr Allergy Immunol200819765265918312532
   PubMed Web of Science ®Google Scholar
• ThomasSRKharitonovSAScottSFNasal and exhaled nitric oxide is reduced in adult patients with cystic fibrosis and does not correlate with cystic fibrosis genotypeChest200011741085108910767244
   PubMed Web of Science ®Google Scholar
• BodiniAD’OrazioCPeroniDBiomarkers of neutrophilic inflammation in exhaled air of cystic fibrosis children with bacterial airway infectionsPediatr Pulmonol200540649449916229003
   PubMed Web of Science ®Google Scholar
• OjooJCMulrennanSAKastelikJAExhaled breath condensate pH and exhaled nitric oxide in allergic asthma and in cystic fibrosisThorax2005601222615618578
   PubMed Web of Science ®Google Scholar
• TateSMacGregorGDavisMAirways in cystic fibrosis are acidified: detection by exhaled breath condensateThorax2002571192692912403872
   PubMed Web of Science ®Google Scholar
• NewportSAminNDozorAJExhaled breath condensate pH and ammonia in cystic fibrosis and response to treatment of acute pulmonary exacerbationsPediatr Pulmonol200944986687219670404
   PubMed Web of Science ®Google Scholar
• OrdonezCLHenigNRMayer-HamblettNInflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosisAm J Respir Crit Care Med1520031681214711475
   Google Scholar
• GilljamHMalmborgASStrandvikBConformity of bacterial growth in sputum and contamination free endobronchial samples in patients with cystic fibrosisThorax19864186416463097864
   PubMed Web of Science ®Google Scholar
• AaronSDKottachchiDFerrisWJSputum versus bronchoscopy for diagnosis of Pseudomonas aeruginosa biofilms in cystic fibrosisEur Respir J200424463163715459143
   PubMed Web of Science ®Google Scholar
• SagelSDKapsnerROsbergIAirway inflammation in children with cystic fibrosis and healthy children assessed by sputum inductionAm J Respir Crit Care Med1520011648 Pt 11425143111704590
   PubMed Web of Science ®Google Scholar
• BaraldiEGhiroLPiovanVSafety and success of exhaled breath condensate collection in asthmaArch Dis Child200388435836012651772
   PubMed Web of Science ®Google Scholar
• de JongPALindbladARubinLProgression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosisThorax2006611808516244089
   PubMed Web of Science ®Google Scholar
• de JongPANakanoYLequinMHProgressive damage on high resolution computed tomography despite stable lung function in cystic fibrosisEur Respir J2004231939714738238
   PubMed Web of Science ®Google Scholar
• BrodyASSucharewHCampbellJDComputed tomography correlates with pulmonary exacerbations in children with cystic fibrosisAm J Respir Crit Care Med200517291128113216100015
   PubMed Web of Science ®Google Scholar
• NasrSZKuhnsLRBrownRWUse of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: a preliminary studyPediatr Pulmonol200131537738211340684
   PubMed Web of Science ®Google Scholar
• RobinsonTEGorisMLZhuHJDornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: a quantitative analysisChest200512842327233516236891
   PubMed Web of Science ®Google Scholar
• NakanoYWongJCde JongPAThe prediction of small airway dimensions using computed tomographyAm J Respir Crit Care Med2005171214214615516531
   PubMed Web of Science ®Google Scholar
• OrlandiIMoroniCCamiciottoliGChronic obstructive pulmonary disease: thin-section CT measurement of airway wall thickness and lung attenuationRadiology2005234260461015671010
   PubMed Web of Science ®Google Scholar
• de JongPANakanoYHopWCChanges in airway dimensions on computed tomography scans of children with cystic fibrosisAm J Respir Crit Care Med2005172221822415831838
   PubMed Web of Science ®Google Scholar
• NiimiAMatsumotoHAmitaniREffect of short-term treatment with inhaled corticosteroid on airway wall thickening in asthmaAm J Med20041161172573115144908
   PubMed Web of Science ®Google Scholar
• PuderbachMEichingerMGahrJProton MRI appearance of cystic fibrosis: comparison to CTEur Radiol200717371672416941092
   PubMed Web of Science ®Google Scholar
• EichingerMPuderbachMFinkCContrast-enhanced 3D MRI of lung perfusion in children with cystic fibrosis – initial resultsEur Radiol200616102147215216673092
   PubMed Web of Science ®Google Scholar
• LeySPuderbachMFinkCAssessment of hemodynamic changes in the systemic and pulmonary arterial circulation in patients with cystic fibrosis using phase-contrast MRIEur Radiol20051581575158015761712
   PubMed Web of Science ®Google Scholar
• AltesTAEichingerMPuderbachMMagnetic resonance imaging of the lung in cystic fibrosisProc Am Thorac Soc20074432132717652494
   PubMedGoogle Scholar
• MentoreKFrohDKde LangeEEHyperpolarized HHe 3 MRI of the lung in cystic fibrosis: assessment at baseline and after bronchodilator and airway clearance treatmentAcad Radiol200512111423142916253854
   PubMed Web of Science ®Google Scholar
• RamseyBWUse of lung imaging studies as outcome measures for development of new therapies in cystic fibrosisProc Am Thorac Soc20074435936317652502
   PubMedGoogle Scholar
• FoundationCFCFF Drug Development Pipeline Available from: http://www.cff.org/treatments/Pipeline/Accessed Apr 5, 2010
   Google Scholar
• WilliamsBRobinetteMSlovisBDereticVPerkettEHydroxychoroquine: pilot study of anti-inflammatory effects in cystic fibrosisPediatr Pulmonol200831A314
   Google Scholar
• KraynackNCCJXueWEffect of simvastatin on exhaled nitric oxide and inflammatory markers in sputum in patients with cystic fibrosisPediatr Pulmonol200831A300
   Google Scholar
• KonstanMWHKABucurCEffect of pioglitazone on sputum markers of inflammation in cystic fibrosisPediatr Pulmonol200831A310
   Google Scholar
• AshlockMABeallRJHamblettNMA pipeline of therapies for cystic fibrosisSemin Respir Crit Care Med200930561162619760548
   PubMed Web of Science ®Google Scholar
• TowardTJSmithNBroadleyKJEffect of phosphodiesterase-5 inhibitor, sildenafil (Viagra), in animal models of airways diseaseAm J Respir Crit Care Med2004169222723414597480
   PubMed Web of Science ®Google Scholar
• PoschetJFFazioJATimminsGSEndosomal hyperacidification in cystic fibrosis is due to defective nitric oxide-cylic GMP signalling cascadeEMBO Rep20067555355916612392
   PubMed Web of Science ®Google Scholar
• PoschetJFTimminsGSTaylor-CousarJLPharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosisAm J Physiol Lung Cell Mol Physiol20072933L71271917586695
   PubMed Web of Science ®Google Scholar
• TirouvanziamRConradCKBottiglieriTHigh-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosisProc Natl Acad Sci U S A2006103124628463316537378
   PubMed Web of Science ®Google Scholar