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Items: 1 to 50 of 152

1.

Amikacin liposome inhalation suspension for chronic Pseudomonas aeruginosa infection in cystic fibrosis.

Bilton D, Pressler T, Fajac I, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Sole A, Quittner AL, Liu K, McGinnis JP 2nd, Eagle G, Gupta R, Konstan MW; CLEAR-108 Study Group.

J Cyst Fibros. 2019 Aug 23. pii: S1569-1993(19)30833-1. doi: 10.1016/j.jcf.2019.08.001. [Epub ahead of print]

PMID:
31451351
2.

Dissociation of systemic and mucosal autoimmunity in cystic fibrosis.

Theprungsirikul J, Skopelja-Gardner S, Meagher RE, Clancy JP, Zemanick ET, Ashare A, Rigby WFC.

J Cyst Fibros. 2019 Jun 28. pii: S1569-1993(19)30807-0. doi: 10.1016/j.jcf.2019.06.006. [Epub ahead of print]

PMID:
31262645
3.

Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.

Borcherding DC, Siefert ME, Lin S, Brewington J, Sadek H, Clancy JP, Plafker SM, Ziady AG.

J Clin Invest. 2019 May 30;129(8):3448-3463. doi: 10.1172/JCI96273.

4.

Prospective multicenter randomized patient recruitment and sample collection to enable future measurements of sputum biomarkers of inflammation in an observational study of cystic fibrosis.

Liou TG, Adler FR, Argel N, Asfour F, Brown PS, Chatfield BA, Daines CL, Durham D, Francis JA, Glover B, Heynekamp T, Hoidal JR, Jensen JL, Keogh R, Kopecky CM, Lechtzin N, Li Y, Lysinger J, Molina O, Nakamura C, Packer KA, Poch KR, Quittner AL, Radford P, Redway AJ, Sagel SD, Sprandel S, Taylor-Cousar JL, Vroom JB, Yoshikawa R, Clancy JP, Elborn JS, Olivier KN, Cox DR.

BMC Med Res Methodol. 2019 Apr 26;19(1):88. doi: 10.1186/s12874-019-0705-0.

5.

Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections in Children with Cystic Fibrosis.

Jorth P, Ehsan Z, Rezayat A, Caldwell E, Pope C, Brewington JJ, Goss CH, Benscoter D, Clancy JP, Singh PK.

Cell Rep. 2019 Apr 23;27(4):1190-1204.e3. doi: 10.1016/j.celrep.2019.03.086.

6.

A glycopolymer improves vascoelasticity and mucociliary transport of abnormal cystic fibrosis mucus.

Fernandez-Petty CM, Hughes GW, Bowers HL, Watson JD, Rosen BH, Townsend SM, Santos C, Ridley CE, Chu KK, Birket SE, Li Y, Leung HM, Mazur M, Garcia BA, Evans TIA, Libby EF, Hathorne H, Hanes J, Tearney GJ, Clancy JP, Engelhardt JF, Swords WE, Thornton DJ, Wiesmann WP, Baker SM, Rowe SM.

JCI Insight. 2019 Apr 18;4(8). pii: 125954. doi: 10.1172/jci.insight.125954. eCollection 2019 Apr 18.

7.

Aerosolized agents for airway clearance in cystic fibrosis.

Southern KW, Clancy JP, Ranganathan S.

Pediatr Pulmonol. 2019 Jun;54(6):858-864. doi: 10.1002/ppul.24306. Epub 2019 Mar 18.

PMID:
30884217
8.

Improving Detection of Rapid Cystic Fibrosis Disease Progression-Early Translation of a Predictive Algorithm Into a Point-of-Care Tool.

Szczesniak RD, Brokamp C, Su W, Mcphail GL, Pestian J, Clancy JP.

IEEE J Transl Eng Health Med. 2018 Nov 9;7:2800108. doi: 10.1109/JTEHM.2018.2878534. eCollection 2019.

9.

Sweat Chloride Testing.

McCarthy C, Clancy JP, Brewington J.

JAMA. 2019 Feb 19;321(7):701-702. doi: 10.1001/jama.2018.21998. No abstract available.

PMID:
30698611
10.

Current Status and Future Opportunities in Lung Precision Medicine Research with a Focus on Biomarkers. An American Thoracic Society/National Heart, Lung, and Blood Institute Research Statement.

Wu AC, Kiley JP, Noel PJ, Amur S, Burchard EG, Clancy JP, Galanter J, Inada M, Jones TK, Kropski JA, Loyd JE, Nogee LM, Raby BA, Rogers AJ, Schwartz DA, Sin DD, Spira A, Weiss ST, Young LR, Himes BE.

Am J Respir Crit Care Med. 2018 Dec 15;198(12):e116-e136. doi: 10.1164/rccm.201810-1895ST.

PMID:
30640517
11.

A Comparison between Two Pathophysiologically Different yet Microbiologically Similar Lung Diseases: Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

Fenker DE, McDaniel CT, Panmanee W, Panos RJ, Sorscher EJ, Sabusap C, Clancy JP, Hassett DJ.

Int J Respir Pulm Med. 2018;5(2). pii: 098. doi: 10.23937/2378-3516/1410098. Epub 2018 Nov 29.

12.

Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis.

Sermet-Gaudelus I, Clancy JP, Nichols DP, Nick JA, De Boeck K, Solomon GM, Mall MA, Bolognese J, Bouisset F, den Hollander W, Paquette-Lamontagne N, Tomkinson N, Henig N, Elborn JS, Rowe SM.

J Cyst Fibros. 2019 Jul;18(4):536-542. doi: 10.1016/j.jcf.2018.10.015. Epub 2018 Nov 19.

13.

Rapid therapeutic advances in CFTR modulator science.

Clancy JP.

Pediatr Pulmonol. 2018 Nov;53(S3):S4-S11. doi: 10.1002/ppul.24157. Review.

14.

Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies.

Brewington JJ, Filbrandt ET, LaRosa FJ 3rd, Moncivaiz JD, Ostmann AJ, Strecker LM, Clancy JP.

JCI Insight. 2018 Jul 12;3(13). pii: 99385. doi: 10.1172/jci.insight.99385.

15.

CFTR modulator theratyping: Current status, gaps and future directions.

Clancy JP, Cotton CU, Donaldson SH, Solomon GM, VanDevanter DR, Boyle MP, Gentzsch M, Nick JA, Illek B, Wallenburg JC, Sorscher EJ, Amaral MD, Beekman JM, Naren AP, Bridges RJ, Thomas PJ, Cutting G, Rowe S, Durmowicz AG, Mense M, Boeck KD, Skach W, Penland C, Joseloff E, Bihler H, Mahoney J, Borowitz D, Tuggle KL.

J Cyst Fibros. 2019 Jan;18(1):22-34. doi: 10.1016/j.jcf.2018.05.004. Epub 2018 Jun 20. Review.

16.

Subacute TGFβ expression drives inflammation, goblet cell hyperplasia, and pulmonary function abnormalities in mice with effects dependent on CFTR function.

Kramer EL, Hardie WD, Madala SK, Davidson C, Clancy JP.

Am J Physiol Lung Cell Mol Physiol. 2018 Sep 1;315(3):L456-L465. doi: 10.1152/ajplung.00530.2017. Epub 2018 Jun 7.

17.

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study.

Brewington JJ, Filbrandt ET, LaRosa FJ 3rd, Moncivaiz JD, Ostmann AJ, Strecker LM, Clancy JP.

J Vis Exp. 2018 Apr 11;(134). doi: 10.3791/57492.

PMID:
29708545
18.

Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial.

Sagel SD, Khan U, Jain R, Graff G, Daines CL, Dunitz JM, Borowitz D, Orenstein DM, Abdulhamid I, Noe J, Clancy JP, Slovis B, Rock MJ, McCoy KS, Strausbaugh S, Livingston FR, Papas KA, Shaffer ML.

Am J Respir Crit Care Med. 2018 Sep 1;198(5):639-647. doi: 10.1164/rccm.201801-0105OC.

19.

Early Detection of Rapid Cystic Fibrosis Disease Progression Tailored to Point of Care: A Proof-of-Principle Study.

Szczesniak R, Brokamp C, Su W, McPhail GL, Pestian J, Clancy JP.

Health Innov Point Care Conf. 2017 Nov;2017:204-207. doi: 10.1109/HIC.2017.8227620. Epub 2017 Dec 21.

20.

Joint hierarchical Gaussian process model with application to personalized prediction in medical monitoring.

Duan LL, Wang X, Clancy JP, Szczesniak RD.

Stat (Int Stat Inst). 2018;7(1). pii: e178. doi: 10.1002/sta4.178. Epub 2018 Mar 4.

21.

Personalised CFTR pharmacotherapeutic response testing and therapy of cystic fibrosis.

McCarthy C, Brewington JJ, Harkness B, Clancy JP, Trapnell BC.

Eur Respir J. 2018 Jun 7;51(6). pii: 1702457. doi: 10.1183/13993003.02457-2017. Print 2018 Jun. No abstract available.

PMID:
29563174
22.

Chronic β2AR stimulation limits CFTR activation in human airway epithelia.

Brewington JJ, Backstrom J, Feldman A, Kramer EL, Moncivaiz JD, Ostmann AJ, Zhu X, Lu LJ, Clancy JP.

JCI Insight. 2018 Feb 22;3(4). pii: 93029. doi: 10.1172/jci.insight.93029. eCollection 2018 Feb 22.

23.

Assessment of pulmonary structure-function relationships in young children and adolescents with cystic fibrosis by multivolume proton-MRI and CT.

Pennati F, Roach DJ, Clancy JP, Brody AS, Fleck RJ, Aliverti A, Woods JC.

J Magn Reson Imaging. 2018 Aug;48(2):531-542. doi: 10.1002/jmri.25978. Epub 2018 Feb 19.

24.

An update on new and emerging therapies for cystic fibrosis.

Hudock KM, Clancy JP.

Expert Opin Emerg Drugs. 2017 Dec;22(4):331-346. doi: 10.1080/14728214.2017.1418324. Epub 2017 Dec 22. Review.

PMID:
29264936
25.

MicroRNA-145, Cystic Fibrosis Transmembrane Conductance Regulator, and Transforming Growth Factor-β. An (Un)tangled Regulatory Web.

Kramer EL, Clancy JP.

Am J Respir Crit Care Med. 2018 Mar 1;197(5):551-552. doi: 10.1164/rccm.201711-2297ED. No abstract available.

PMID:
29253345
26.

Flexible semiparametric joint modeling: an application to estimate individual lung function decline and risk of pulmonary exacerbations in cystic fibrosis.

Li D, Keogh R, Clancy JP, Szczesniak RD.

Emerg Themes Epidemiol. 2017 Nov 14;14:13. doi: 10.1186/s12982-017-0067-1. eCollection 2017.

27.

TGFβ as a therapeutic target in cystic fibrosis.

Kramer EL, Clancy JP.

Expert Opin Ther Targets. 2018 Feb;22(2):177-189. doi: 10.1080/14728222.2018.1406922. Epub 2017 Dec 13. Review.

28.

Airway microbiota across age and disease spectrum in cystic fibrosis.

Zemanick ET, Wagner BD, Robertson CE, Ahrens RC, Chmiel JF, Clancy JP, Gibson RL, Harris WT, Kurland G, Laguna TA, McColley SA, McCoy K, Retsch-Bogart G, Sobush KT, Zeitlin PL, Stevens MJ, Accurso FJ, Sagel SD, Harris JK.

Eur Respir J. 2017 Nov 16;50(5). pii: 1700832. doi: 10.1183/13993003.00832-2017. Print 2017 Nov.

29.

Guanylate cyclase 2C agonism corrects CFTR mutants.

Arora K, Huang Y, Mun K, Yarlagadda S, Sundaram N, Kessler MM, Hannig G, Kurtz CB, Silos-Santiago I, Helmrath M, Palermo JJ, Clancy JP, Steinbrecher KA, Naren AP.

JCI Insight. 2017 Oct 5;2(19). pii: 93686. doi: 10.1172/jci.insight.93686.

30.

Take it to the Bank.

Clancy JP, Joseloff E.

J Cyst Fibros. 2017 Sep;16(5):532-533. doi: 10.1016/j.jcf.2017.08.005. No abstract available.

31.

Detection of CFTR function and modulation in primary human nasal cell spheroids.

Brewington JJ, Filbrandt ET, LaRosa FJ 3rd, Ostmann AJ, Strecker LM, Szczesniak RD, Clancy JP.

J Cyst Fibros. 2018 Jan;17(1):26-33. doi: 10.1016/j.jcf.2017.06.010. Epub 2017 Jul 13.

32.

Home Monitoring of Patients with Cystic Fibrosis to Identify and Treat Acute Pulmonary Exacerbations. eICE Study Results.

Lechtzin N, Mayer-Hamblett N, West NE, Allgood S, Wilhelm E, Khan U, Aitken ML, Ramsey BW, Boyle MP, Mogayzel PJ Jr, Gibson RL, Orenstein D, Milla C, Clancy JP, Antony V, Goss CH; eICE Study Team.

Am J Respir Crit Care Med. 2017 Nov 1;196(9):1144-1151. doi: 10.1164/rccm.201610-2172OC.

33.

Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood.

Szczesniak RD, Li D, Su W, Brokamp C, Pestian J, Seid M, Clancy JP.

Am J Respir Crit Care Med. 2017 Aug 15;196(4):471-478. doi: 10.1164/rccm.201612-2574OC.

34.

Doxycycline improves clinical outcomes during cystic fibrosis exacerbations.

Xu X, Abdalla T, Bratcher PE, Jackson PL, Sabbatini G, Wells JM, Lou XY, Quinn R, Blalock JE, Clancy JP, Gaggar A.

Eur Respir J. 2017 Apr 5;49(4). pii: 1601102. doi: 10.1183/13993003.01102-2016. Print 2017 Apr.

36.

Biomarkers for cystic fibrosis drug development.

Muhlebach MS, Clancy JP, Heltshe SL, Ziady A, Kelley T, Accurso F, Pilewski J, Mayer-Hamblett N, Joseloff E, Sagel SD.

J Cyst Fibros. 2016 Nov;15(6):714-723. doi: 10.1016/j.jcf.2016.10.009. Epub 2016 Oct 27. Review.

37.

CFTR Modulator Therapies in Pediatric Cystic Fibrosis: Focus on Ivacaftor.

Kramer EL, Clancy JP.

Expert Opin Orphan Drugs. 2016 Oct;4(10):1033-1042. doi: 10.1080/21678707.2016.1226800. Epub 2016 Sep 13.

38.

Feasibility, tolerability and safety of pediatric hyperpolarized 129Xe magnetic resonance imaging in healthy volunteers and children with cystic fibrosis.

Walkup LL, Thomen RP, Akinyi TG, Watters E, Ruppert K, Clancy JP, Woods JC, Cleveland ZI.

Pediatr Radiol. 2016 Nov;46(12):1651-1662. Epub 2016 Aug 5.

39.

Constrictive Bronchiolitis in Cystic Fibrosis Adolescents with Refractory Pulmonary Decline.

Harris WT, Boyd JT, McPhail GL, Brody AS, Szczesniak RD, Korbee LL, Baker ML, Clancy JP.

Ann Am Thorac Soc. 2016 Dec;13(12):2174-2183.

40.

PharmGKB summary: ivacaftor pathway, pharmacokinetics/pharmacodynamics.

Fohner AE, McDonagh EM, Clancy JP, Whirl Carrillo M, Altman RB, Klein TE.

Pharmacogenet Genomics. 2017 Jan;27(1):39-42. No abstract available.

41.

Urinary kidney injury biomarkers and tobramycin clearance among children and young adults with cystic fibrosis: a population pharmacokinetic analysis.

Downes KJ, Dong M, Fukuda T, Clancy JP, Haffner C, Bennett MR, Vinks AA, Goldstein SL.

J Antimicrob Chemother. 2017 Jan;72(1):254-260. Epub 2016 Sep 1.

42.

Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease.

Roach DJ, Crémillieux Y, Fleck RJ, Brody AS, Serai SD, Szczesniak RD, Kerlakian S, Clancy JP, Woods JC.

Ann Am Thorac Soc. 2016 Nov;13(11):1923-1931.

43.

Bayesian Ensemble Trees (BET) for Clustering and Prediction in Heterogeneous Data.

Duan LL, Clancy JP, Szczesniak RD.

J Comput Graph Stat. 2016;25(3):748-761. Epub 2016 Aug 5.

44.

Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.

Thomen RP, Walkup LL, Roach DJ, Cleveland ZI, Clancy JP, Woods JC.

J Cyst Fibros. 2017 Mar;16(2):275-282. doi: 10.1016/j.jcf.2016.07.008. Epub 2016 Jul 29.

45.

Continuous alternating inhaled antibiotics for chronic pseudomonal infection in cystic fibrosis.

Flume PA, Clancy JP, Retsch-Bogart GZ, Tullis DE, Bresnik M, Derchak PA, Lewis SA, Ramsey BW.

J Cyst Fibros. 2016 Nov;15(6):809-815. doi: 10.1016/j.jcf.2016.05.001. Epub 2016 May 24.

46.

Cystic Fibrosis and Its Management Through Established and Emerging Therapies.

Spielberg DR, Clancy JP.

Annu Rev Genomics Hum Genet. 2016 Aug 31;17:155-75. doi: 10.1146/annurev-genom-090314-050024. Epub 2016 Feb 22. Review.

PMID:
26905785
47.

Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Smooth Muscle. A Novel Role in Cystic Fibrosis Airway Obstruction.

Clancy JP.

Am J Respir Crit Care Med. 2016 Feb 15;193(4):352-3. doi: 10.1164/rccm.201510-2088ED. No abstract available.

PMID:
26871669
48.

Diagnostic Testing in Cystic Fibrosis.

Brewington J, Clancy JP.

Clin Chest Med. 2016 Mar;37(1):31-46. doi: 10.1016/j.ccm.2015.10.005. Epub 2015 Dec 28. Review.

PMID:
26857766
49.
50.

Predicting future lung function decline in cystic fibrosis patients: Statistical methods and clinical connections.

Szczesniak RD, McPhail GL, Li D, Amin RS, Clancy JP.

Pediatr Pulmonol. 2016 Feb;51(2):217-8. doi: 10.1002/ppul.23357. Epub 2015 Dec 17. No abstract available.

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