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Items: 1 to 50 of 107

1.

Update in Cystic Fibrosis 2018.

Ramsey BW, Downey GP, Goss CH.

Am J Respir Crit Care Med. 2019 May 15;199(10):1188-1194. doi: 10.1164/rccm.201902-0310UP. No abstract available.

PMID:
30917288
2.

Urinary metabolomics reveals unique metabolic signatures in infants with cystic fibrosis.

Kopp BT, Joseloff E, Goetz D, Ingram B, Heltshe SL, Leung DH, Ramsey BW, McCoy K, Borowitz D.

J Cyst Fibros. 2018 Nov 23. pii: S1569-1993(18)30916-0. doi: 10.1016/j.jcf.2018.10.016. [Epub ahead of print]

PMID:
30477895
3.

VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.

Davies JC, Moskowitz SM, Brown C, Horsley A, Mall MA, McKone EF, Plant BJ, Prais D, Ramsey BW, Taylor-Cousar JL, Tullis E, Uluer A, McKee CM, Robertson S, Shilling RA, Simard C, Van Goor F, Waltz D, Xuan F, Young T, Rowe SM; VX16-659-101 Study Group.

N Engl J Med. 2018 Oct 25;379(17):1599-1611. doi: 10.1056/NEJMoa1807119. Epub 2018 Oct 18.

4.

VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.

Keating D, Marigowda G, Burr L, Daines C, Mall MA, McKone EF, Ramsey BW, Rowe SM, Sass LA, Tullis E, McKee CM, Moskowitz SM, Robertson S, Savage J, Simard C, Van Goor F, Waltz D, Xuan F, Young T, Taylor-Cousar JL; VX16-445-001 Study Group.

N Engl J Med. 2018 Oct 25;379(17):1612-1620. doi: 10.1056/NEJMoa1807120. Epub 2018 Oct 18.

5.

Lumacaftor/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV1.

McColley SA, Konstan MW, Ramsey BW, Stuart Elborn J, Boyle MP, Wainwright CE, Waltz D, Vera-Llonch M, Marigowda G, Jiang JG, Rubin JL.

J Cyst Fibros. 2019 Jan;18(1):94-101. doi: 10.1016/j.jcf.2018.07.011. Epub 2018 Aug 23.

6.

Reply to Shanthikumar et al.: Azithromycin for Early Pseudomonas Infection in Cystic Fibrosis: Do the Benefits Outweigh the Harms?

Retsch-Bogart G, Mayer-Hamblett N, Ramsey BW.

Am J Respir Crit Care Med. 2018 Nov 15;198(10):1349-1350. doi: 10.1164/rccm.201808-1462LE. No abstract available.

PMID:
30138567
7.

Azithromycin for Early Pseudomonas Infection in Cystic Fibrosis. The OPTIMIZE Randomized Trial.

Mayer-Hamblett N, Retsch-Bogart G, Kloster M, Accurso F, Rosenfeld M, Albers G, Black P, Brown P, Cairns A, Davis SD, Graff GR, Kerby GS, Orenstein D, Buckingham R, Ramsey BW; OPTIMIZE Study Group.

Am J Respir Crit Care Med. 2018 Nov 1;198(9):1177-1187. doi: 10.1164/rccm.201802-0215OC. Erratum in: Am J Respir Crit Care Med. 2019 Mar 15;199(6):809.

PMID:
29890086
8.

Islet Interleukin-1β Immunoreactivity Is an Early Feature of Cystic Fibrosis That May Contribute to β-Cell Failure.

Hull RL, Gibson RL, McNamara S, Deutsch GH, Fligner CL, Frevert CW, Ramsey BW, Sanda S.

Diabetes Care. 2018 Apr;41(4):823-830. doi: 10.2337/dc17-1387. Epub 2018 Feb 1.

9.

Pancreatic Enzyme Replacement Therapy Use in Infants With Cystic Fibrosis Diagnosed by Newborn Screening.

Gelfond D, Heltshe SL, Skalland M, Heubi JE, Kloster M, Leung DH, Ramsey BW, Borowitz D; BONUS Study Investigators.

J Pediatr Gastroenterol Nutr. 2018 Apr;66(4):657-663. doi: 10.1097/MPG.0000000000001829.

10.

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.

11.

Academic, Foundation, and Industry Collaboration in Finding New Therapies.

Ramsey BW, Nepom GT, Lonial S.

N Engl J Med. 2017 May 4;376(18):1762-1769. doi: 10.1056/NEJMra1612575. Review. No abstract available.

12.

AJRCCM: 100-Year Anniversary. Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis.

Ramsey BW, Welsh MJ.

Am J Respir Crit Care Med. 2017 May 1;195(9):1092-1099. doi: 10.1164/rccm.201702-0266ED. No abstract available.

13.

Effects of Diagnosis by Newborn Screening for Cystic Fibrosis on Weight and Length in the First Year of Life.

Leung DH, Heltshe SL, Borowitz D, Gelfond D, Kloster M, Heubi JE, Stalvey M, Ramsey BW; Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network.

JAMA Pediatr. 2017 Jun 1;171(6):546-554. doi: 10.1001/jamapediatrics.2017.0206.

14.

New Therapeutic Approaches to Modulate and Correct Cystic Fibrosis Transmembrane Conductance Regulator.

Ong T, Ramsey BW.

Pediatr Clin North Am. 2016 Aug;63(4):751-64. doi: 10.1016/j.pcl.2016.04.006. Review.

15.

Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis.

Elborn JS, Ramsey BW, Boyle MP, Konstan MW, Huang X, Marigowda G, Waltz D, Wainwright CE; VX-809 TRAFFIC and TRANSPORT study groups.

Lancet Respir Med. 2016 Aug;4(8):617-626. doi: 10.1016/S2213-2600(16)30121-7. Epub 2016 Jun 10.

PMID:
27298017
16.

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.

17.

Metagenomic evidence for taxonomic dysbiosis and functional imbalance in the gastrointestinal tracts of children with cystic fibrosis.

Manor O, Levy R, Pope CE, Hayden HS, Brittnacher MJ, Carr R, Radey MC, Hager KR, Heltshe SL, Ramsey BW, Miller SI, Hoffman LR, Borenstein E.

Sci Rep. 2016 Mar 4;6:22493. doi: 10.1038/srep22493.

18.

Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

Wainwright CE, Elborn JS, Ramsey BW.

N Engl J Med. 2015 Oct 29;373(18):1783-4. doi: 10.1056/NEJMc1510466. No abstract available.

PMID:
26510034
19.

Update in Cystic Fibrosis 2014.

Ong T, Ramsey BW.

Am J Respir Crit Care Med. 2015 Sep 15;192(6):669-75. doi: 10.1164/rccm.201504-0656UP. Review. No abstract available.

PMID:
26371812
20.

Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.

Solomon GM, Marshall SG, Ramsey BW, Rowe SM.

Pediatr Pulmonol. 2015 Oct;50 Suppl 40:S3-S13. doi: 10.1002/ppul.23240. Epub 2015 Jun 19. Review.

21.

Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K, Flume PA, Konstan MW, McColley SA, McCoy K, McKone EF, Munck A, Ratjen F, Rowe SM, Waltz D, Boyle MP; TRAFFIC Study Group; TRANSPORT Study Group.

N Engl J Med. 2015 Jul 16;373(3):220-31. doi: 10.1056/NEJMoa1409547. Epub 2015 May 17.

22.

Impact of Sustained Eradication of New Pseudomonas aeruginosa Infection on Long-term Outcomes in Cystic Fibrosis.

Mayer-Hamblett N, Kloster M, Rosenfeld M, Gibson RL, Retsch-Bogart GZ, Emerson J, Thompson V, Ramsey BW.

Clin Infect Dis. 2015 Sep 1;61(5):707-15. doi: 10.1093/cid/civ377. Epub 2015 May 13.

23.

Use of ibuprofen to assess inflammatory biomarkers in induced sputum: Implications for clinical trials in cystic fibrosis.

Chmiel JF, Konstan MW, Accurso FJ, Lymp J, Mayer-Hamblett N, VanDevanter DR, Rose LM, Ramsey BW; Assessment of Induced Sputum in Cystic Fibrosis Study Group.

J Cyst Fibros. 2015 Nov;14(6):720-6. doi: 10.1016/j.jcf.2015.03.007. Epub 2015 Apr 11.

24.

Remarkable progress toward new treatments for cystic fibrosis.

Eldredge LC, Ramsey BW.

Lancet Respir Med. 2014 Dec;2(12):962-4. doi: 10.1016/S2213-2600(14)70272-3. Epub 2014 Nov 19. No abstract available.

PMID:
25466347
25.

Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor.

Heltshe SL, Mayer-Hamblett N, Burns JL, Khan U, Baines A, Ramsey BW, Rowe SM; GOAL (the G551D Observation-AL) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network.

Clin Infect Dis. 2015 Mar 1;60(5):703-12. doi: 10.1093/cid/ciu944. Epub 2014 Nov 25.

26.

Study design considerations for evaluating the efficacy and safety of pancreatic enzyme replacement therapy in patients with cystic fibrosis.

Konstan MW, Borowitz D, Mayer-Hamblett N, Milla C, Hendeles L, Murray S, Kronmal RA, Casey S, Rose LM, Morgan WJ, Ramsey BW.

Clin Investig (Lond). 2013 Aug;3(8):731-741.

27.

Highly effective cystic fibrosis clinical research teams: critical success factors.

Retsch-Bogart GZ, Van Dalfsen JM, Marshall BC, George C, Pilewski JM, Nelson EC, Goss CH, Ramsey BW.

J Gen Intern Med. 2014 Aug;29 Suppl 3:S714-23. doi: 10.1007/s11606-014-2896-8.

28.

Pseudomonas aeruginosa in vitro phenotypes distinguish cystic fibrosis infection stages and outcomes.

Mayer-Hamblett N, Rosenfeld M, Gibson RL, Ramsey BW, Kulasekara HD, Retsch-Bogart GZ, Morgan W, Wolter DJ, Pope CE, Houston LS, Kulasekara BR, Khan U, Burns JL, Miller SI, Hoffman LR.

Am J Respir Crit Care Med. 2014 Aug 1;190(3):289-97. doi: 10.1164/rccm.201404-0681OC.

29.

Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, Sagel SD, Khan U, Mayer-Hamblett N, Van Dalfsen JM, Joseloff E, Ramsey BW; GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network.

Am J Respir Crit Care Med. 2014 Jul 15;190(2):175-84. doi: 10.1164/rccm.201404-0703OC.

30.

Pseudomonas aeruginosa phenotypes associated with eradication failure in children with cystic fibrosis.

Mayer-Hamblett N, Ramsey BW, Kulasekara HD, Wolter DJ, Houston LS, Pope CE, Kulasekara BR, Armbruster CR, Burns JL, Retsch-Bogart G, Rosenfeld M, Gibson RL, Miller SI, Khan U, Hoffman LR.

Clin Infect Dis. 2014 Sep 1;59(5):624-31. doi: 10.1093/cid/ciu385. Epub 2014 May 26.

31.

Inhaled antibiotics for lower airway infections.

Quon BS, Goss CH, Ramsey BW.

Ann Am Thorac Soc. 2014 Mar;11(3):425-34. doi: 10.1513/AnnalsATS.201311-395FR. Review.

32.

Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data.

Accurso FJ, Van Goor F, Zha J, Stone AJ, Dong Q, Ordonez CL, Rowe SM, Clancy JP, Konstan MW, Hoch HE, Heltshe SL, Ramsey BW, Campbell PW, Ashlock MA.

J Cyst Fibros. 2014 Mar;13(2):139-47.

33.

Escherichia coli dysbiosis correlates with gastrointestinal dysfunction in children with cystic fibrosis.

Hoffman LR, Pope CE, Hayden HS, Heltshe S, Levy R, McNamara S, Jacobs MA, Rohmer L, Radey M, Ramsey BW, Brittnacher MJ, Borenstein E, Miller SI.

Clin Infect Dis. 2014 Feb;58(3):396-9. doi: 10.1093/cid/cit715. Epub 2013 Oct 30.

34.

Expanding assessments of translational research programs: supplementing metrics with value judgments.

Scott CS, Nagasawa PR, Abernethy NF, Ramsey BW, Martin PJ, Hacker BM, Schwartz HD, Brock DM, Robins LS, Wolf FM, Carter-Dubois M, Disis ML.

Eval Health Prof. 2014 Mar;37(1):83-97. doi: 10.1177/0163278713500984. Epub 2013 Sep 23.

35.

Rationale and design of a randomized trial of home electronic symptom and lung function monitoring to detect cystic fibrosis pulmonary exacerbations: the early intervention in cystic fibrosis exacerbation (eICE) trial.

Lechtzin N, West N, Allgood S, Wilhelm E, Khan U, Mayer-Hamblett N, Aitken ML, Ramsey BW, Boyle MP, Mogayzel PJ Jr, Goss CH.

Contemp Clin Trials. 2013 Nov;36(2):460-9. doi: 10.1016/j.cct.2013.09.004. Epub 2013 Sep 19.

36.

Modifying disease in cystic fibrosis: current and future therapies on the horizon.

Ong T, Ramsey BW.

Curr Opin Pulm Med. 2013 Nov;19(6):645-51. doi: 10.1097/MCP.0b013e328365ab5f. Review.

PMID:
24048086
37.

Standard care versus protocol based therapy for new onset Pseudomonas aeruginosa in cystic fibrosis.

Mayer-Hamblett N, Rosenfeld M, Treggiari MM, Konstan MW, Retsch-Bogart G, Morgan W, Wagener J, Gibson RL, Khan U, Emerson J, Thompson V, Elkin EP, Ramsey BW; EPIC; ESCF Investigators.

Pediatr Pulmonol. 2013 Oct;48(10):943-53. doi: 10.1002/ppul.22693. Epub 2013 Jul 2.

38.

Staphylococcus aureus small-colony variants are independently associated with worse lung disease in children with cystic fibrosis.

Wolter DJ, Emerson JC, McNamara S, Buccat AM, Qin X, Cochrane E, Houston LS, Rogers GB, Marsh P, Prehar K, Pope CE, Blackledge M, Déziel E, Bruce KD, Ramsey BW, Gibson RL, Burns JL, Hoffman LR.

Clin Infect Dis. 2013 Aug;57(3):384-91. doi: 10.1093/cid/cit270. Epub 2013 Apr 26.

39.

Cystic fibrosis therapeutics: the road ahead.

Hoffman LR, Ramsey BW.

Chest. 2013 Jan;143(1):207-213. doi: 10.1378/chest.12-1639. Review.

40.

Incidence and clinical significance of elevated liver function tests in cystic fibrosis clinical trials.

Mayer-Hamblett N, Kloster M, Ramsey BW, Narkewicz MR, Saiman L, Goss CH.

Contemp Clin Trials. 2013 Mar;34(2):232-8. doi: 10.1016/j.cct.2012.11.005. Epub 2012 Nov 29.

41.

Progress in cystic fibrosis and the CF Therapeutics Development Network.

Rowe SM, Borowitz DS, Burns JL, Clancy JP, Donaldson SH, Retsch-Bogart G, Sagel SD, Ramsey BW.

Thorax. 2012 Oct;67(10):882-90. doi: 10.1136/thoraxjnl-2012-202550.

42.

Risk factors for age at initial Pseudomonas acquisition in the cystic fibrosis epic observational cohort.

Rosenfeld M, Emerson J, McNamara S, Thompson V, Ramsey BW, Morgan W, Gibson RL; EPIC Study Group.

J Cyst Fibros. 2012 Sep;11(5):446-53. doi: 10.1016/j.jcf.2012.04.003. Epub 2012 May 1.

43.

Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report.

Ramsey BW, Banks-Schlegel S, Accurso FJ, Boucher RC, Cutting GR, Engelhardt JF, Guggino WB, Karp CL, Knowles MR, Kolls JK, LiPuma JJ, Lynch S, McCray PB Jr, Rubenstein RC, Singh PK, Sorscher E, Welsh M.

Am J Respir Crit Care Med. 2012 Apr 15;185(8):887-92. doi: 10.1164/rccm.201111-2068WS. Epub 2012 Feb 3.

44.

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordoñez C, Elborn JS; VX08-770-102 Study Group.

N Engl J Med. 2011 Nov 3;365(18):1663-72. doi: 10.1056/NEJMoa1105185.

45.

Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis.

Treggiari MM, Retsch-Bogart G, Mayer-Hamblett N, Khan U, Kulich M, Kronmal R, Williams J, Hiatt P, Gibson RL, Spencer T, Orenstein D, Chatfield BA, Froh DK, Burns JL, Rosenfeld M, Ramsey BW; Early Pseudomonas Infection Control (EPIC) Investigators.

Arch Pediatr Adolesc Med. 2011 Sep;165(9):847-56. doi: 10.1001/archpediatrics.2011.136.

46.

Initial Pseudomonas aeruginosa treatment failure is associated with exacerbations in cystic fibrosis.

Mayer-Hamblett N, Kronmal RA, Gibson RL, Rosenfeld M, Retsch-Bogart G, Treggiari MM, Burns JL, Khan U, Ramsey BW; EPIC Investigators.

Pediatr Pulmonol. 2012 Feb;47(2):125-34. doi: 10.1002/ppul.21525. Epub 2011 Aug 9.

47.

Wouldn’t we all like to be a little more LEAN?

Disis ML, Schwartz HD, Ramsey BW.

Clin Transl Sci. 2010 Oct;3(5):207-9. No abstract available.

48.

Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function.

Accurso FJ, Moss RB, Wilmott RW, Anbar RD, Schaberg AE, Durham TA, Ramsey BW; TIGER-1 Investigator Study Group.

Am J Respir Crit Care Med. 2011 Mar 1;183(5):627-34. doi: 10.1164/rccm.201008-1267OC. Epub 2010 Dec 17.

PMID:
21169471
49.

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.

Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW.

N Engl J Med. 2010 Nov 18;363(21):1991-2003. doi: 10.1056/NEJMoa0909825.

50.

A pipeline of therapies for cystic fibrosis.

Ashlock MA, Beall RJ, Hamblett NM, Konstan MW, Penland CM, Ramsey BW, Van Dalfsen JM, Wetmore DR, Campbell PW 3rd.

Semin Respir Crit Care Med. 2009 Oct;30(5):611-26. doi: 10.1055/s-0029-1238919. Epub 2009 Sep 16.

PMID:
19760548

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