DK089507 - Search Results

Year	Number of Results
2010	1
2011	6
2012	21
2013	23
2014	36
2015	51
2016	44
2017	58
2018	56
2019	54
2020	53
2021	60
2022	53
2023	59
2024	49
2025	29
2026	9

1

Conserved and context-dependent changes in pathogen and host cell gene expression during Staphylococcus aureus intracellular infection.

Holmes EA, Waalkes A, Lewis JD, Penewit K, Long DR, Salipante SJ. Holmes EA, et al. BMC Microbiol. 2026 Jun 15. doi: 10.1186/s12866-026-05297-w. Online ahead of print. BMC Microbiol. 2026. PMID: 42298412 Free article.

2

Contributors to the heterogeneity of response to CFTR modulators.

Downey DG, Houston C, Mayer-Hamblett N, Taylor-Cousar JL, Taggart CC. Downey DG, et al. Lancet Respir Med. 2026 Apr 21:S2213-2600(26)00060-3. doi: 10.1016/S2213-2600(26)00060-3. Online ahead of print. Lancet Respir Med. 2026. PMID: 42030963 Review.

3

Risk factors for longitudinal estimated glomerular filtration rate decline in adults with cystic fibrosis.

Nacharaju D, Zelnick LR, Ramos KJ, de Boer IH, Flume PA, Goss CH, Heltshe SL, Bhatraju PK. Nacharaju D, et al. Ann Am Thorac Soc. 2026 May 1;23(5):737-744. doi: 10.1093/annalsats/aaoag013. Ann Am Thorac Soc. 2026. PMID: 41734009 Free PMC article.

4

Impact of elexacaftor/tezacaftor/ivacaftor on gastrointestinal outcomes, inflammation, exocrine pancreatic function and fat malabsorption: Report of PROMISE pediatric substudy.

Green N, Vu P, Xie J, Schwarzenberg SJ, Freedman S, Narkewicz M, Pittman J, Ratjen F, Rosenfeld M, Pope C, Duong J, Hoffman L, Heltshe S, Lusman SS; PROMISE Study Group. Green N, et al. J Cyst Fibros. 2026 May;25(3):489-494. doi: 10.1016/j.jcf.2026.02.005. Epub 2026 Feb 14. J Cyst Fibros. 2026. PMID: 41692662 Free PMC article.

5

Evaluating the association of antibiotic spectrum and treatment responses of cystic fibrosis pulmonary exacerbations.

Somayaji R, Cogen JD, VanDevanter D, LiPuma JJ, West NE, Sanders DB, Gerber JS, Kronman MP, Goss CH, Flume PA, Heltshe SL. Somayaji R, et al. J Cyst Fibros. 2026 Mar;25(2):218-224. doi: 10.1016/j.jcf.2025.12.003. Epub 2025 Dec 17. J Cyst Fibros. 2026. PMID: 41412872 Free PMC article.

6

Correlates of FEV₁ deficit in pulmonary exacerbations of cystic fibrosis and associated clinical outcomes.

McElvaney OJ, Heltshe SL, Sanders DB, West NE, Fogarty B, VanDevanter DR, Flume PA, Goss CH; STOP2 Investigators. McElvaney OJ, et al. J Cyst Fibros. 2026 Mar;25(2):210-217. doi: 10.1016/j.jcf.2025.12.007. Epub 2025 Dec 12. J Cyst Fibros. 2026. PMID: 41387145 Free PMC article. Clinical Trial.

7

Tertiary lymphoid structures support the development of allergen-specific progenitor CD4+ T cells.

Bangs DJ, Hondowicz B, Abadie K, Savard R, Weiss M, Karakousi T, Skene PJ; ImmGenT Consortium; Lund AW, Goldrath AW, Heeg M, Pepper M. Bangs DJ, et al. bioRxiv [Preprint]. 2025 Oct 5:2025.10.03.680350. doi: 10.1101/2025.10.03.680350. bioRxiv. 2025. PMID: 41256356 Free PMC article. Preprint.

8

Considerations in the Diagnosis and Management of Bronchiectasis in Older Adults.

Pollack LR, Goss CH, Olivier KN. Pollack LR, et al. Clin Geriatr Med. 2025 Nov;41(4):539-551. doi: 10.1016/j.cger.2025.07.006. Epub 2025 Aug 7. Clin Geriatr Med. 2025. PMID: 41198259 Free PMC article. Review.

9

Culture and Adaptation of Pseudomonas aeruginosa During Early Infection in Children With Cystic Fibrosis.

Mitra SD, Soneji M, Axline CMR, Hayden HS, Radey MC, Forsberg CM, Ozer EA, Wolter DJ, Pope CE, Burns J, Hoffman LR, Mayer-Hamblett N, Singh P, Hauser AR. Mitra SD, et al. J Infect Dis. 2026 Jan 17;233(1):e67-e76. doi: 10.1093/infdis/jiaf527. J Infect Dis. 2026. PMID: 41170776 Free PMC article.

10

Exploring reduced pulmonary exacerbation rates in cystic fibrosis: a pandemic or modulator effect?

Stephenson AL, Sykes J, Stanojevic S, Ma X, Ostrenga J, Todd J, Faro A, Elbert A, Marshall B, Ramos KJ, Cromwell E, Quon BS, Goss CH, Somayaji R. Stephenson AL, et al. ERJ Open Res. 2025 Oct 27;11(5):00111-2025. doi: 10.1183/23120541.00111-2025. eCollection 2025 Sep. ERJ Open Res. 2025. PMID: 41158496 Free PMC article.

11

Long-term reductions in inflammation in people with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor.

Sagel SD, Poore TS, Wagner BD, Xie J, Heltshe SL, Cross M, Bratcher PE, Taylor-Cousar JL, Wilson A, McBennett K, Morgan SJ, Singh PK, Nichols DP, Kelly A, Solomon GM; PROMISE Study Group. Sagel SD, et al. Ann Am Thorac Soc. 2026 Feb 1;23(2):241-251. doi: 10.1513/AnnalsATS.202507-817OC. Ann Am Thorac Soc. 2026. PMID: 40961158 Free PMC article.

12

Cystic Fibrosis: Huge Gains, but Still a Work in Progress.

Bush A, Ramsey BW. Bush A, et al. Am J Respir Crit Care Med. 2025 Oct;211(10):1731-1732. doi: 10.1164/rccm.202508-1983ED. Am J Respir Crit Care Med. 2025. PMID: 40920903 Free PMC article. No abstract available.

13

Evaluating Long-Term Effectiveness of Cystic Fibrosis Modulator Therapies after Rapid Adoption: A Dual-Approach Study.

Miranda Afonso P, Zhou GC, Su W, Burgel PR, Cromwell E, Goss CH, Keogh RH, Liou TG, Marshall BC, Mayer-Hamblett N, Morgan WJ, Ostrenga JS, Pasta DJ, Schechter MS, Stanojevic S, Wainwright CE, Szczesniak RD, Andrinopoulou ER. Miranda Afonso P, et al. Ann Am Thorac Soc. 2026 Jan 1;23(1):89-99. doi: 10.1513/AnnalsATS.202503-267OC. Ann Am Thorac Soc. 2026. PMID: 40920855 Free PMC article.

14

An alternative view of changes in lung function among people with cystic fibrosis.

McElvaney OJ, Goss CH. McElvaney OJ, et al. J Cyst Fibros. 2025 Sep;24(5):821-822. doi: 10.1016/j.jcf.2025.08.009. Epub 2025 Sep 1. J Cyst Fibros. 2025. PMID: 40897594 Free PMC article. No abstract available.

15

A dramatic decline in lung transplantation for cystic fibrosis in the United States.

Milinic T, Ramos KJ, Gill ER, Burdis N, Goss CH, Kapnadak SG. Milinic T, et al. CHEST Pulm. 2024 Dec;2(4):100077. doi: 10.1016/j.chpulm.2024.100077. Epub 2024 Jun 22. CHEST Pulm. 2024. PMID: 40821006 Free PMC article. No abstract available.

16

Pseudomonas infections persisting after CFTR modulators are widespread throughout the lungs and drive lung inflammation.

Durfey SL, Kapnadak SG, Pena T, Willmering MM, Godwin JD, Teresi ME, Heltshe SL, Vo AT, Villacreses RA, Aliukonyte I, Boyken L, Stroik MR, Morgan SJ, Wang GM, Betts HL, Zhang S, Goss CH, Clancy JP, Aitken ML, Steele C, Feder AF, Esther CR Jr, Tiddens HAWM, Woods JC, Stoltz DA, Singh PK. Durfey SL, et al. Cell Host Microbe. 2025 Aug 13;33(8):1428-1445.e4. doi: 10.1016/j.chom.2025.07.009. Epub 2025 Aug 5. Cell Host Microbe. 2025. PMID: 40769150 Free PMC article.

17

Maximising opportunity for therapeutic success: sequential participation in cystic fibrosis nucleic acid-based therapy trials.

Mayer-Hamblett N, Kerper NR, Clancy JP, Davies JC, Taylor-Cousar JL, Donaldson SH, Bell SC, Jain R, Simmonds NJ, Mall MA, Goss CH, Rayment JH, Setiawan L, Pilewski JM. Mayer-Hamblett N, et al. Lancet Respir Med. 2025 Dec;13(12):1108-1118. doi: 10.1016/S2213-2600(25)00206-1. Epub 2025 Jul 30. Lancet Respir Med. 2025. PMID: 40752498 Free PMC article. Review.

18

Validating the Data Completeness and Accuracy of the Canadian Cystic Fibrosis Registry.

Somayaji R, Cheng SY, Abdulrahem N, Stanojevic S, Eckford P, Quon BS, Cromwell E, Faro A, Goss CH, Stephenson AL. Somayaji R, et al. Can Respir J. 2025 Jul 1;2025:8893074. doi: 10.1155/carj/8893074. eCollection 2025. Can Respir J. 2025. PMID: 40661671 Free PMC article.

19

Airway complications after lung transplantation: Perioperative risk factors and clinical outcomes.

Kapnadak SG, Ramos KJ, Flodin R, Mansoor S, Bilodeau K, Beidler P, Lease ED, Thomas R, Dubois R, Pal J, Mulligan MS. Kapnadak SG, et al. JHLT Open. 2025 Jun 6;9:100315. doi: 10.1016/j.jhlto.2025.100315. eCollection 2025 Aug. JHLT Open. 2025. PMID: 40635784 Free PMC article.

20

Research coordinators' perspectives on recruitment of minoritized people with cystic fibrosis into clinical trials.

Milinic T, Burdis N, Gill E, Burks P, Jarosz ME, Hoffman AJ, Kapnadak SG, Ramos KJ, Goss CH. Milinic T, et al. BMC Pulm Med. 2025 Jul 4;25(1):325. doi: 10.1186/s12890-025-03707-9. BMC Pulm Med. 2025. PMID: 40615798 Free PMC article.

21

Elexacaftor/tezacaftor/ivacaftor prescription in lung transplant recipients with cystic fibrosis in the US.

Burdis NC, Bradford MC, Heltshe SL, Milinic T, McElvaney OJ, Lease ED, Goss CH, Kapnadak SG, Guthrie BL, Ramos KJ. Burdis NC, et al. J Cyst Fibros. 2025 Sep;24(5):929-934. doi: 10.1016/j.jcf.2025.06.005. Epub 2025 Jul 1. J Cyst Fibros. 2025. PMID: 40603159 Free PMC article.

22

Lung Donor Utilization Decisions: A Data-Free Zone.

Milinic T, Ramos KJ. Milinic T, et al. Ann Am Thorac Soc. 2025 Aug;22(8):1132-1133. doi: 10.1513/AnnalsATS.202506-646ED. Ann Am Thorac Soc. 2025. PMID: 40569202 Free PMC article. No abstract available.

23

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in 6- to 11-Year-Olds with Cystic Fibrosis: An Observational Study.

Pittman JE, Morgan S, McCreary M, Vu PT, Jorth P, Heltshe S, Hoffman LR, Kelly A, Sagel SD, Singh PK, Solomon GM, Rosenfeld M, Ratjen F; PROMISE Pediatric substudy Investigators. Pittman JE, et al. Ann Am Thorac Soc. 2025 Nov;22(11):1696-1708. doi: 10.1513/AnnalsATS.202501-103OC. Ann Am Thorac Soc. 2025. PMID: 40540670 Free PMC article.

24

Cystic Fibrosis Microbiome-directed Antibiotic Therapy Trial in Exacerbations Results Stratified (CFMATTERS): results of a multicentre randomised controlled trial.

Plant BJ, Einarsson GG, Deasy KF, Dahly D, Singh PK, Barry PJ, Goss CH, Fajac I, Vagg T, Durieu I, Flanagan E, O'Callaghan G, Martin C, Burgel PR, Haworth CS, Floto RA, Downey DG, Dupont LJ, Jones AM, Elborn JS, Eustace JA, Mall MA, Tunney MM. Plant BJ, et al. Eur Respir J. 2025 Aug 8;66(2):2402443. doi: 10.1183/13993003.02443-2024. Print 2025 Aug. Eur Respir J. 2025. PMID: 40506211 Free PMC article. Clinical Trial.

25

Fecal microbiota changes in people with cystic fibrosis after 6 months of elexacaftor/tezacaftor/ivacaftor: Findings from the promise study.

Duong JT, Hayden HS, Verster AJ, Pope CE, Miller C, Kelsi Penewit, Salipante SJ, Rowe SM, Solomon GM, Nichols D, Kelly A, Schwarzenberg SJ, Freedman SD, Hoffman LR. Duong JT, et al. J Cyst Fibros. 2025 Jul;24(4):792-800. doi: 10.1016/j.jcf.2025.05.006. Epub 2025 Jun 6. J Cyst Fibros. 2025. PMID: 40483244 Free PMC article.

26

Human intestinal enteroids: Nonclinical applications for predicting oral drug disposition, toxicity, and efficacy.

O'Mahony ET, Arian CM, Aryeh KS, Wang K, Thummel KE, Kelly EJ. O'Mahony ET, et al. Pharmacol Ther. 2025 Sep;273:108879. doi: 10.1016/j.pharmthera.2025.108879. Epub 2025 May 19. Pharmacol Ther. 2025. PMID: 40398537 Free PMC article. Review.

27

Accounting for Inconsistent Use of Covariate Adjustment in Group Sequential Trials.

Bannick MS, Heltshe SL, Simon N. Bannick MS, et al. Stat Med. 2025 May;44(10-12):e70082. doi: 10.1002/sim.70082. Stat Med. 2025. PMID: 40377247 Free PMC article.

28

Monoclonal antibodies derived from B cells in subjects with cystic fibrosis reduce Pseudomonas aeruginosa burden in mice.

Hale M, Takehara KK, Thouvenel CD, Moustafa DA, Repele A, Fontana MF, Netland J, McNamara S, Gibson RL, Goldberg JB, Rawlings DJ, Pepper M. Hale M, et al. Elife. 2025 Apr 24;13:RP98851. doi: 10.7554/eLife.98851. Elife. 2025. PMID: 40272253 Free PMC article.

29

Elexacaftor-tezacaftor-ivacaftor pharmacokinetics with concurrent tacrolimus administration after lung transplant.

Guimbellot JS, Chalamalla A, Baker E, Ryan KJ, Dowell A, Abouelenein S, Bartlett LE, Bergeron J, Turner G, Acosta EP, Ramos KJ. Guimbellot JS, et al. J Cyst Fibros. 2025 May;24(3):534-541. doi: 10.1016/j.jcf.2025.03.010. Epub 2025 Mar 21. J Cyst Fibros. 2025. PMID: 40121139 Free PMC article.

30

Effects of Therapeutic Antibiotic Exposure on the Oropharyngeal and Fecal Microbiota in Infants With Cystic Fibrosis.

Hayden HS, Nelson MT, Ross SE, Verster AJ, Bouzek DC, Eng A, Waalkes A, Penewit K, Kopp BT, Siracusa C, Rock MJ, Salipante SJ, Hoffman LR, Sanders DB. Hayden HS, et al. Pediatr Pulmonol. 2025 Mar;60(3):e71024. doi: 10.1002/ppul.71024. Pediatr Pulmonol. 2025. PMID: 40042126 Free PMC article.

31

The impact of highly effective modulator therapy on sinusitis and dysosmia in young children with cystic fibrosis: a prospective study protocol.

Liu CM, Fischer JL, Zemanick ET, Woods JC, Markarian KK, Fain SB, Froh D, Heltshe SL, Hoffman LR, Humphries SM, Kramer EL, Ode KL, Lewis M, Li DA, Mata J, Milla SS, Niedbalski PJ, Sawatzky BD, Sim MS, Sullivan JS, Trout AT, Goss CH, Taylor-Cousar JL, Beswick DM. Liu CM, et al. ERJ Open Res. 2025 Jan 13;11(1):00137-2024. doi: 10.1183/23120541.00137-2024. eCollection 2025 Jan. ERJ Open Res. 2025. PMID: 39811548 Free PMC article.

32

Vanzacaftor-tezacaftor-deutivacaftor for children aged 6-11 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial.

Hoppe JE, Kasi AS, Pittman JE, Jensen R, Thia LP, Robinson P, Tirakitsoontorn P, Ramsey B, Mall MA, Taylor-Cousar JL, McKone EF, Tullis E, Salinas DB, Zhu J, Chen YC, Rodriguez-Romero V, Sosnay PR, Davies G; VX21-121-105 Study Group. Hoppe JE, et al. Lancet Respir Med. 2025 Mar;13(3):244-255. doi: 10.1016/S2213-2600(24)00407-7. Epub 2025 Jan 2. Lancet Respir Med. 2025. PMID: 39756425 Free PMC article. Clinical Trial.

33

Vanzacaftor-tezacaftor-deutivacaftor versus elexacaftor-tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years and older (SKYLINE Trials VX20-121-102 and VX20-121-103): results from two randomised, active-controlled, phase 3 trials.

Keating C, Yonker LM, Vermeulen F, Prais D, Linnemann RW, Trimble A, Kotsimbos T, Mermis J, Braun AT, O'Carroll M, Sutharsan S, Ramsey B, Mall MA, Taylor-Cousar JL, McKone EF, Tullis E, Floreth T, Michelson P, Sosnay PR, Nair N, Zahigian R, Martin H, Ahluwalia N, Lam A, Horsley A; VX20-121-102 Study Group; VX20-121-103 Study Group. Keating C, et al. Lancet Respir Med. 2025 Mar;13(3):256-271. doi: 10.1016/S2213-2600(24)00411-9. Epub 2025 Jan 2. Lancet Respir Med. 2025. PMID: 39756424 Free PMC article. Clinical Trial.

34

CHP2 Modifies Chronic Pseudomonas aeruginosa Airway Infection Risk in Cystic Fibrosis.

Faino AV, Gordon WW, Buckingham K, Stilp AM, Pace RG, Raraigh KS, Collaco JM, Zhou YH, Dang H, O'Neal W, Knowles MK, Cutting GR, Rosenfeld M, Bamshad MJ, Gibson RL, Blue EE. Faino AV, et al. Ann Am Thorac Soc. 2025 May;22(5):715-723. doi: 10.1513/AnnalsATS.202408-868OC. Ann Am Thorac Soc. 2025. PMID: 39746161 Free PMC article.

35

The prognostic value of single and repeated ≥10% FEV₁ declines for chronic lung allograft dysfunction.

Lu K, Bradford MC, Wang X, Liu T, Schmitz C, Lease ED, Kapnadak S, Hachem R, Ramos KJ, Morrell ED. Lu K, et al. J Heart Lung Transplant. 2025 Apr;44(4):681-685. doi: 10.1016/j.healun.2024.11.031. Epub 2024 Dec 3. J Heart Lung Transplant. 2025. PMID: 39637927 Free PMC article.

36

Research Coordinators' Perspectives on Recruitment of Minoritized People with Cystic Fibrosis into Clinical Trials.

Milinic T, Burdis N, Gill E, Burks P, Jarosz ME, Hoffman AJ, Kapnadak SG, Ramos KJ, Goss CH. Milinic T, et al. Res Sq [Preprint]. 2024 Nov 13:rs.3.rs-5195002. doi: 10.21203/rs.3.rs-5195002/v1. Res Sq. 2024. Update in: BMC Pulm Med. 2025 Jul 4;25(1):325. doi: 10.1186/s12890-025-03707-9. PMID: 39606463 Free PMC article. Updated. Preprint.

37

Aging with CF: Characteristics of people with CF aged 40 and older in the United States.

Ostrenga JS, Robinson K, Brown AW, Goss CH, Cromwell EA. Ostrenga JS, et al. J Cyst Fibros. 2025 Jan;24(1):183-186. doi: 10.1016/j.jcf.2024.10.009. Epub 2024 Nov 4. J Cyst Fibros. 2025. PMID: 39500648 Free PMC article.

38

Factors associated with prescription of elexacaftor/tezacaftor/ivacaftor among people with cystic fibrosis aged 12 years or older with at least one F508del allele.

Hergenroeder GE, Todd JV, Ostrenga JS, Goss CH, Jain R, Morgan W, Sawicki GS, Schechter MS, Cromwell EA, Ren CL. Hergenroeder GE, et al. J Cyst Fibros. 2025 Jan;24(1):135-141. doi: 10.1016/j.jcf.2024.10.006. Epub 2024 Oct 29. J Cyst Fibros. 2025. PMID: 39472230 Free PMC article.

39

Cystic fibrosis-related diabetes is associated with reduced islet protein expression of GLP-1 receptor and perturbation of cell-specific transcriptional programs.

Gharib SA, Vemireddy R, Castillo JJ, Fountaine BS, Bammler TK, MacDonald JW, Hull-Meichle RL, Zraika S. Gharib SA, et al. Sci Rep. 2024 Oct 28;14(1):25689. doi: 10.1038/s41598-024-76722-1. Sci Rep. 2024. PMID: 39463434 Free PMC article.

40

Mechanisms of Staphylococcus aureus survival of trimethoprim-sulfamethoxazole-induced thymineless death.

Gonsalves LJ, Tran A, Gardiner T, Freeman T, Dutta A, Miller CJ, McNamara S, Waalkes A, Long DR, Salipante SJ, Hoffman LR, Wolter DJ. Gonsalves LJ, et al. mBio. 2024 Nov 13;15(11):e0163424. doi: 10.1128/mbio.01634-24. Epub 2024 Oct 24. mBio. 2024. PMID: 39445807 Free PMC article.

41

Species- and subspecies-level characterization of health-associated bacterial consortia that colonize the human gut during infancy.

Minot SS, Mayer-Blackwell K, Fiore-Gartland A, Johnson A, Self S, Bhatti P, Yao L, Liu L, Sun X, Jinfa Y, Kublin J. Minot SS, et al. Gut Microbes. 2024 Jan-Dec;16(1):2414975. doi: 10.1080/19490976.2024.2414975. Epub 2024 Oct 20. Gut Microbes. 2024. PMID: 39428758 Free PMC article.

42

A search for bacteria identified from cerebrospinal fluid shunt infections in previous surgical events.

Hodor P, Pope CE, Whitlock KB, McDonald PJ, Hauptman J, Hoffman LR, Limbrick DD Jr, Simon TD; Cerebrospinal FLuId MicroBiota in Shunts (CLIMB) Study Group. Hodor P, et al. PLoS One. 2024 Oct 10;19(10):e0311605. doi: 10.1371/journal.pone.0311605. eCollection 2024. PLoS One. 2024. PMID: 39388396 Free PMC article.

43

Impact of sinus surgery in people with cystic fibrosis and chronic rhinosinusitis in the era of highly effective modulator therapy: Protocol for a prospective observational study.

Liu CM, Fischer JL, Alt JA, Bodner TE, Chowdhury NI, Getz AE, Hwang PH, Kimple AJ, Mace JC, Smith TL, Soler ZM, Goss CH, Taylor-Cousar JL, Saavedra MT, Beswick DM. Liu CM, et al. PLoS One. 2024 Sep 26;19(9):e0310986. doi: 10.1371/journal.pone.0310986. eCollection 2024. PLoS One. 2024. PMID: 39325787 Free PMC article.

44

A survey of cystic fibrosis physicians' views on lung transplant referral in the era of elexacaftor/tezacaftor/ivacaftor.

Burdis N, Milinic T, Bartlett LE, Goss L, Tallarico E, Boyle A, Thaxton A, Turner GA, Benvenuto LJ, Faro A, Goss CH, Kapnadak SG, Ramos KJ. Burdis N, et al. Pediatr Pulmonol. 2024 Dec;59(12):3641-3649. doi: 10.1002/ppul.27273. Epub 2024 Sep 18. Pediatr Pulmonol. 2024. PMID: 39291796 Free PMC article.

45

Genetic modifiers of body mass index in individuals with cystic fibrosis.

Ling H, Raraigh KS, Pugh EW, Aksit MA, Zhang P, Pace RG, Faino AV, Bamshad MJ, Gibson RL, O'Neal W, Knowles MR, Blackman SM, Cutting GR; CF Genome Project. Ling H, et al. Am J Hum Genet. 2024 Oct 3;111(10):2203-2218. doi: 10.1016/j.ajhg.2024.08.004. Epub 2024 Sep 10. Am J Hum Genet. 2024. PMID: 39260370 Free PMC article.

46

Elexacaftor/tezacaftor/ivacaftor's effects on cystic fibrosis infections are maintained, but not increased, after 3.5 years of treatment.

Morgan SJ, Coulter E, Betts HL, Solomon GM, Clancy JP, Rowe SM, Nichols DP, Singh PK; PROMISE-Micro Study Group. Morgan SJ, et al. J Clin Invest. 2024 Sep 5;134(20):e184171. doi: 10.1172/JCI184171. J Clin Invest. 2024. PMID: 39235967 Free PMC article. No abstract available.

47

Longitudinal changes in BMD in adults with cystic fibrosis.

Jad R, Ma X, Stanojevic S, Illango A, Tullis E, Gilmour J, Goss CH, Strug LJ, Stephenson AL. Jad R, et al. J Bone Miner Res. 2024 Nov 29;39(12):1716-1721. doi: 10.1093/jbmr/zjae139. J Bone Miner Res. 2024. PMID: 39221749 Free PMC article.

48

Elexacaftor/Tezacaftor/Ivacaftor Markedly Reduces Aspergillus fumigatus in Cystic Fibrosis.

Morgan SJ, Nichols DP, Ni W, Hong G, Salipante SJ, Solomon GM, Rowe SM, Clancy JP, Cramer RA, Singh PK. Morgan SJ, et al. Am J Respir Crit Care Med. 2024 Nov 1;210(9):1155-1158. doi: 10.1164/rccm.202406-1128RL. Am J Respir Crit Care Med. 2024. PMID: 39189854 Free PMC article. No abstract available.

49

Impact of day-to-day variation in FEV1 on measures of change: A conceptual description.

Magaret AS, Graham E, Caverly LJ, Cromwell EA, Paynter A, Rosenfeld M, Thornton CS, Goss CH. Magaret AS, et al. J Cyst Fibros. 2024 Sep;23(5):943-946. doi: 10.1016/j.jcf.2024.07.005. Epub 2024 Aug 14. J Cyst Fibros. 2024. PMID: 39147620 Free PMC article.

50

Predicting return of lung function after a pulmonary exacerbation using the cystic fibrosis respiratory symptom diary-chronic respiratory infection symptom scale.

Gill ER, Goss CH, Sagel SD, Wright ML, Horner SD, Zuñiga JA. Gill ER, et al. BMC Pulm Med. 2024 Jul 24;24(1):360. doi: 10.1186/s12890-024-03148-w. BMC Pulm Med. 2024. PMID: 39049032 Free PMC article.

51

Impact of Discontinuing Both Hypertonic Saline and Dornase Alfa after Elexacaftor-Tezacaftor-Ivacaftor in Cystic Fibrosis.

Mayer-Hamblett N, Gifford AH, Kloster M, Russell R, Braun AT, Gibson RL, Hoppe JE, Jain R, Linnemann RW, Liou TG, Lysinger J, Milla C, Riekert KA, Sawicki GS, Young J, Nichols D. Mayer-Hamblett N, et al. Ann Am Thorac Soc. 2024 Nov;21(11):1507-1515. doi: 10.1513/AnnalsATS.202404-366OC. Ann Am Thorac Soc. 2024. PMID: 39041864 Free PMC article. Clinical Trial.

52

Attitudes toward and preparedness for lung transplantation among individuals with cystic fibrosis in the era of highly effective modulators.

Burdis N, Kapnadak SG, Bartlett LE, McElvaney O, Milinic T, Wai TH, Lange AV, Reid N, Dunitz JM, Billings JL, Pilewski JM, Saavedra M, Goss CH, Hartzler AL, Ramos KJ. Burdis N, et al. BMC Pulm Med. 2024 Jul 18;24(1):348. doi: 10.1186/s12890-024-03163-x. BMC Pulm Med. 2024. PMID: 39026320 Free PMC article.

53

Clinical and in vitro models identify distinct adaptations enhancing Staphylococcus aureus pathogenesis in human macrophages.

Long DR, Holmes EA, Lo HY, Penewit K, Almazan J, Hodgson T, Berger NF, Bishop ZH, Lewis JD, Waalkes A, Wolter DJ, Salipante SJ. Long DR, et al. PLoS Pathog. 2024 Jul 11;20(7):e1012394. doi: 10.1371/journal.ppat.1012394. eCollection 2024 Jul. PLoS Pathog. 2024. PMID: 38991026 Free PMC article.

54

Statistical approaches for the integration of external controls in a cystic fibrosis clinical trial: a simulation and an application.

Warden MN, Heltshe SL, Simon N, Mooney SJ, Mayer-Hamblett N, Magaret AS. Warden MN, et al. Am J Epidemiol. 2024 Dec 2;193(12):1796-1804. doi: 10.1093/aje/kwae148. Am J Epidemiol. 2024. PMID: 38918020 Free PMC article.

55

The association between cystic fibrosis-related diabetes and periodontitis in adults: A pilot cross-sectional study.

Alkhateeb AA, Mancl LA, Ramos KJ, Rothen ML, Kotsakis GA, Trence DL, Chi DL. Alkhateeb AA, et al. PLoS One. 2024 Jun 25;19(6):e0305975. doi: 10.1371/journal.pone.0305975. eCollection 2024. PLoS One. 2024. PMID: 38917148 Free PMC article.

56

Symptom phenotyping in people with cystic fibrosis during acute pulmonary exacerbations using machine-learning K-means clustering analysis.

Gill ER, Dill C, Goss CH, Sagel SD, Wright ML, Horner SD, Zuñiga JA. Gill ER, et al. J Cyst Fibros. 2024 Nov;23(6):1106-1111. doi: 10.1016/j.jcf.2024.05.014. Epub 2024 Jun 8. J Cyst Fibros. 2024. PMID: 38851922 Free PMC article.

57

Monoclonal antibodies derived from B cells in subjects with cystic fibrosis reduce Pseudomonas aeruginosa burden in mice.

Hale M, Takehara KK, Thouvenel CD, Moustafa DA, Repele A, Fontana MF, Netland J, McNamara S, Gibson RL, Goldberg JB, Rawlings DJ, Pepper M. Hale M, et al. bioRxiv [Preprint]. 2024 Oct 9:2024.04.08.588618. doi: 10.1101/2024.04.08.588618. bioRxiv. 2024. Update in: Elife. 2025 Apr 24;13:RP98851. doi: 10.7554/eLife.98851. PMID: 38645147 Free PMC article. Updated. Preprint.

58

"We'll deal with it as it comes": A Qualitative Analysis of Romantic Partners' Dyadic Coping in Cystic Fibrosis.

Lau N, Ramos KJ, Aitken ML, Goss CH, Barton KS, Kross EK, Engelberg RA. Lau N, et al. J Soc Pers Relat. 2024 Apr;41(4):689-705. doi: 10.1177/02654075231190617. Epub 2023 Jul 21. J Soc Pers Relat. 2024. PMID: 38638205 Free PMC article.

59

Genetics of cystogenesis in base-edited human organoids reveal therapeutic strategies for polycystic kidney disease.

Vishy CE, Thomas C, Vincent T, Crawford DK, Goddeeris MM, Freedman BS. Vishy CE, et al. Cell Stem Cell. 2024 Apr 4;31(4):537-553.e5. doi: 10.1016/j.stem.2024.03.005. Cell Stem Cell. 2024. PMID: 38579684 Free PMC article.

60

Genetic variation in severe cystic fibrosis liver disease is associated with novel mechanisms for disease pathogenesis.

Stonebraker JR, Pace RG, Gallins PJ, Dang H, Aksit MA, Faino AV, Gordon WW, MacParland S, Bamshad MJ, Gibson RL, Cutting GR, Durie PR, Wright FA, Zhou YH, Blackman SM, O'Neal WK, Ling SC, Knowles MR. Stonebraker JR, et al. Hepatology. 2024 Nov 1;80(5):1012-1025. doi: 10.1097/HEP.0000000000000863. Epub 2024 Mar 27. Hepatology. 2024. PMID: 38536042 Free PMC article.

61

Alterations in the fecal microbiota in patients with advanced cystic fibrosis liver disease after 6 months of elexacaftor/tezacaftor/ivacaftor.

Duong JT, Pope CE, Hayden HS, Miller C, Salipante SJ, Rowe SM, Solomon GM, Nichols D, Hoffman LR, Narkewicz MR, Green N. Duong JT, et al. J Cyst Fibros. 2024 May;23(3):490-498. doi: 10.1016/j.jcf.2024.02.015. Epub 2024 Mar 6. J Cyst Fibros. 2024. PMID: 38448281 Free PMC article.

62

Massively parallel mutant selection identifies genetic determinants of Pseudomonas aeruginosa colonization of Drosophila melanogaster.

Miles J, Lozano GL, Rajendhran J, Stabb EV, Handelsman J, Broderick NA. Miles J, et al. mSystems. 2024 Mar 19;9(3):e0131723. doi: 10.1128/msystems.01317-23. Epub 2024 Feb 21. mSystems. 2024. PMID: 38380971 Free PMC article.

63

Heterogeneity of CFTR modulator-induced sweat chloride concentrations in people with cystic fibrosis.

Zemanick ET, Emerman I, McCreary M, Mayer-Hamblett N, Warden MN, Odem-Davis K, VanDevanter DR, Ren CL, Young J, Konstan MW; CHEC-SC Study Group. Zemanick ET, et al. J Cyst Fibros. 2024 Jul;23(4):676-684. doi: 10.1016/j.jcf.2024.02.001. Epub 2024 Feb 15. J Cyst Fibros. 2024. PMID: 38360461 Free PMC article.

64

The effect of discontinuing hypertonic saline or dornase alfa on mucociliary clearance in elexacaftor/tezacaftor/ivacaftor treated people with cystic fibrosis: The SIMPLIFY-MCC Study.

Donaldson SH, Corcoran TE, Pilewski JM, Laube BL, Mogayzel P, Ceppe A, Wu J, Zeman K, Rowe SM, Nichols DP, Gifford AH, Bennett WD, Mayer-Hamblett N; SIMPLIFY MCC Study teams. Donaldson SH, et al. J Cyst Fibros. 2024 May;23(3):457-460. doi: 10.1016/j.jcf.2024.02.003. Epub 2024 Feb 13. J Cyst Fibros. 2024. PMID: 38355350 Free PMC article. Clinical Trial.

65

MEK1/2 inhibition decreases pro-inflammatory responses in macrophages from people with cystic fibrosis and mitigates severity of illness in experimental murine methicillin-resistant Staphylococcus aureus infection.

De M, Serpa G, Zuiker E, Hisert KB, Liles WC, Manicone AM, Hemann EA, Long ME. De M, et al. Front Cell Infect Microbiol. 2024 Jan 30;14:1275940. doi: 10.3389/fcimb.2024.1275940. eCollection 2024. Front Cell Infect Microbiol. 2024. PMID: 38352056 Free PMC article.

66

Pancreatic enzyme prescription following ivacaftor licensing: A retrospective analysis of the US and UK cystic fibrosis registries.

Calthorpe R, Rosenfeld M, Goss CH, Green N, Derleth M, Carr SB, Smyth A, Stewart I. Calthorpe R, et al. J Cyst Fibros. 2024 Jul;23(4):746-753. doi: 10.1016/j.jcf.2024.01.011. Epub 2024 Feb 10. J Cyst Fibros. 2024. PMID: 38342635 Free PMC article.

67

Longitudinal profiling of the intestinal microbiome in children with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor.

Reasoner SA, Bernard R, Waalkes A, Penewit K, Lewis J, Sokolow AG, Brown RF, Edwards KM, Salipante SJ, Hadjifrangiskou M, Nicholson MR. Reasoner SA, et al. mBio. 2024 Feb 14;15(2):e0193523. doi: 10.1128/mbio.01935-23. Epub 2024 Jan 26. mBio. 2024. PMID: 38275294 Free PMC article.

68

Qualitative Analysis of Perspectives on Lung Transplant among People with Cystic Fibrosis.

Milinic T, Hobler MR, Bartlett LE, Gill E, Burdis N, Engelberg RA, Curtis JR, Hartzler AL, Aitken ML, Kapnadak SG, Goss CH, Smith PJ, Ramos KJ. Milinic T, et al. Ann Am Thorac Soc. 2024 Jul;21(7):1044-1052. doi: 10.1513/AnnalsATS.202307-625OC. Ann Am Thorac Soc. 2024. PMID: 38259137 Free PMC article.

69

Strain-level characterization of health-associated bacterial consortia that colonize the human gut during infancy.

Minot SS, Mayer-Blackwell K, Fiore-Gartland A, Johnson A, Self S, Bhatti P, Yao L, Liu L, Sun X, Jinfa Y, Kublin J. Minot SS, et al. medRxiv [Preprint]. 2023 Dec 17:2023.12.16.23300077. doi: 10.1101/2023.12.16.23300077. medRxiv. 2023. Update in: Gut Microbes. 2024 Jan-Dec;16(1):2414975. doi: 10.1080/19490976.2024.2414975. PMID: 38168439 Free PMC article. Updated. Preprint.

70

The cost of simplifying treatments for cystic fibrosis: Implications of the SIMPLIFY trial.

Gold LS, Hansen RN, Mayer-Hamblett N, Nichols DP, Gifford AH, Kloster M, Goss CH, Kessler L. Gold LS, et al. J Manag Care Spec Pharm. 2024 Jan;30(1):26-33. doi: 10.18553/jmcp.2024.30.1.26. J Manag Care Spec Pharm. 2024. PMID: 38153868 Free PMC article.

71

Adjunctive Systemic Corticosteroids for Pulmonary Exacerbations of Cystic Fibrosis.

McElvaney OJ, Heltshe SL, Odem-Davis K, West NE, Sanders DB, Fogarty B, VanDevanter DR, Flume PA, Goss CH. McElvaney OJ, et al. Ann Am Thorac Soc. 2024 May;21(5):716-726. doi: 10.1513/AnnalsATS.202308-673OC. Ann Am Thorac Soc. 2024. PMID: 38096105 Free PMC article. Clinical Trial.

72

Pancreas and islet morphology in cystic fibrosis: clues to the etiology of cystic fibrosis-related diabetes.

Malik SS, Padmanabhan D, Hull-Meichle RL. Malik SS, et al. Front Endocrinol (Lausanne). 2023 Nov 23;14:1269139. doi: 10.3389/fendo.2023.1269139. eCollection 2023. Front Endocrinol (Lausanne). 2023. PMID: 38075070 Free PMC article. Review.

73

REC domain stabilizes the active heptamer of σ⁵⁴-dependent transcription factor, FleR from Pseudomonas aeruginosa.

Sahoo PK, Sheenu, Jain D. Sahoo PK, et al. iScience. 2023 Nov 4;26(12):108397. doi: 10.1016/j.isci.2023.108397. eCollection 2023 Dec 15. iScience. 2023. PMID: 38058307 Free PMC article.

74

Massively parallel mutant selection identifies genetic determinants of Pseudomonas aeruginosa colonization of Drosophila melanogaster.

Miles J, Lozano GL, Rajendhran J, Stabb EV, Handelsman J, Broderick NA. Miles J, et al. bioRxiv [Preprint]. 2023 Nov 21:2023.11.20.567573. doi: 10.1101/2023.11.20.567573. bioRxiv. 2023. Update in: mSystems. 2024 Mar 19;9(3):e0131723. doi: 10.1128/msystems.01317-23. PMID: 38045230 Free PMC article. Updated. Preprint.

75

Assessing Safety of Discontinuing Hypertonic Saline in Those with Lower Forced Expiratory Volume in 1 Second after Elexacaftor/Tezacaftor/Ivacaftor.

Nichols D, Gifford A, Russell R, Odem-Davis K, Young J, Amaro-Galvez R, Billings J, Mukadam Z, Mayer-Hamblett N. Nichols D, et al. Ann Am Thorac Soc. 2024 Feb;21(2):360-362. doi: 10.1513/AnnalsATS.202308-735RL. Ann Am Thorac Soc. 2024. PMID: 38019099 Free PMC article. No abstract available.

76

A longitudinal analysis of respiratory symptoms in people with cystic fibrosis with advanced lung disease on and off ETI.

Gill ER, Bartlett LE, Milinic T, Burdis N, Pilewski JM, Dunitz JM, Kapnadak SG, Goss CH, Ramos KJ. Gill ER, et al. J Cyst Fibros. 2024 Jan;23(1):161-164. doi: 10.1016/j.jcf.2023.11.008. Epub 2023 Nov 25. J Cyst Fibros. 2024. PMID: 38008684 Free PMC article.

77

Olfactory loss in people with cystic fibrosis: Community perceptions and impact.

Miller JE, Liu CM, Zemanick ET, Woods JC, Goss CH, Taylor-Cousar JL, Beswick DM. Miller JE, et al. J Cyst Fibros. 2024 Nov;23(6):1195-1198. doi: 10.1016/j.jcf.2023.11.006. Epub 2023 Nov 18. J Cyst Fibros. 2024. PMID: 37981480 Free PMC article.

78

Prevalence and Clinical Impact of Respiratory Viral Infections from the STOP2 Study of Cystic Fibrosis Pulmonary Exacerbations.

Thornton CS, Caverly LJ, Kalikin LM, Carmody LA, McClellan S, LeBar W, Sanders DB, West NE, Goss CH, Flume PA, Heltshe SL, VanDevanter DR, LiPuma JJ. Thornton CS, et al. Ann Am Thorac Soc. 2024 Apr;21(4):595-603. doi: 10.1513/AnnalsATS.202306-576OC. Ann Am Thorac Soc. 2024. PMID: 37963297 Free PMC article. Clinical Trial.

79

Identification of prognostic biomarkers for antibiotic associated nephrotoxicity in cystic fibrosis.

Hart A, Cesar F, Zelnick LR, O'Connor N, Bailey Z, Lo J, Van Ness K, Stanaway IB, Bammler TK, MacDonald JW, Thau MR, Himmelfarb J, Goss CH, Aitken M, Kelly EJ, Bhatraju PK. Hart A, et al. J Cyst Fibros. 2024 Mar;23(2):293-299. doi: 10.1016/j.jcf.2023.10.021. Epub 2023 Nov 10. J Cyst Fibros. 2024. PMID: 37949747 Free PMC article.

80

Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study.

Daines CL, Tullis E, Costa S, Linnemann RW, Mall MA, McKone EF, Polineni D, Quon BS, Ringshausen FC, Rowe SM, Selvadurai H, Taylor-Cousar JL, Withers NJ, Ahluwalia N, Moskowitz SM, Prieto-Centurion V, Tan YV, Tian S, Weinstock T, Xuan F, Zhang Y, Ramsey B, Griese M; VX17-445-105 Study Group. Daines CL, et al. Eur Respir J. 2023 Dec 7;62(6):2202029. doi: 10.1183/13993003.02029-2022. Print 2023 Dec. Eur Respir J. 2023. PMID: 37945033 Free PMC article. Clinical Trial.

81

Limited effects of azithromycin on the oropharyngeal microbiome in children with CF and early pseudomonas infection.

Wagner BD, Zemanick ET, Sagel SD, Robertson CE, Stevens MJ, Mayer-Hamblett N, Retsch-Bogart G, Ramsey BW, Harris JK. Wagner BD, et al. BMC Microbiol. 2023 Oct 27;23(1):312. doi: 10.1186/s12866-023-03073-8. BMC Microbiol. 2023. PMID: 37891457 Free PMC article.

82

Elexacaftor/Tezacaftor/Ivacaftor Treatment and Depression-related Events.

Ramsey B, Correll CU, DeMaso DR, McKone E, Tullis E, Taylor-Cousar JL, Chu C, Volkova N, Ahluwalia N, Waltz D, Tian S, Mall MA. Ramsey B, et al. Am J Respir Crit Care Med. 2024 Feb 1;209(3):299-306. doi: 10.1164/rccm.202308-1525OC. Am J Respir Crit Care Med. 2024. PMID: 37890129 Free PMC article. Review.

83

Lung Allocation Score Exceptions in Persons with Cystic Fibrosis Undergoing Lung Transplant.

Bayomy OF, Bradford MC, Milinic T, Kapnadak SG, Morrell ED, Lease ED, Goss CH, Ramos KJ. Bayomy OF, et al. Ann Am Thorac Soc. 2024 Feb;21(2):271-278. doi: 10.1513/AnnalsATS.202306-509OC. Ann Am Thorac Soc. 2024. PMID: 37878995 Free PMC article.

84

Characteristics associated with cystic fibrosis-related pulmonary exacerbation treatment location.

Gold LS, Hansen RN, Heltshe SL, Flume PA, Goss CH, West NE, Sanders DB, Kessler L. Gold LS, et al. J Cyst Fibros. 2024 Mar;23(2):278-281. doi: 10.1016/j.jcf.2023.10.004. Epub 2023 Oct 5. J Cyst Fibros. 2024. PMID: 37805355 Free PMC article. Clinical Trial.

85

Patient perspectives on elexacaftor/tezacaftor/ivacaftor after lung transplant.

Young D, Bartlett LE, Guimbellot J, Milinic T, Burdis N, Gill ER, Lease ED, Goss CH, Kapnadak SG, Ramos KJ. Young D, et al. J Cyst Fibros. 2024 May;23(3):545-548. doi: 10.1016/j.jcf.2023.09.017. Epub 2023 Oct 3. J Cyst Fibros. 2024. PMID: 37798159 Free PMC article.

86

Predicting return of lung function after a pulmonary exacerbation using the cystic fibrosis respiratory symptom diary-chronic respiratory infection symptom scale.

Gill ER, Goss CH, Sagel SD, Wright ML, Horner SD, Zuñiga JA. Gill ER, et al. Res Sq [Preprint]. 2023 Sep 13:rs.3.rs-3232522. doi: 10.21203/rs.3.rs-3232522/v1. Res Sq. 2023. Update in: BMC Pulm Med. 2024 Jul 24;24(1):360. doi: 10.1186/s12890-024-03148-w. PMID: 37790510 Free PMC article. Updated. Preprint.

87

Phoenix from the Ashes: Celebrating the 2023 North American Cystic Fibrosis Conference.

Bush A, Ramsey B, Shteinberg M, Martinez F. Bush A, et al. Am J Respir Crit Care Med. 2023 Nov 1;208(9):909-910. doi: 10.1164/rccm.202309-1603ED. Am J Respir Crit Care Med. 2023. PMID: 37756479 Free PMC article. No abstract available.

88

Mutation of putative glycosyl transferases PslC and PslI confers susceptibility to antibiotics and leads to drastic reduction in biofilm formation in Pseudomonas aeruginosa.

Ruhal R, Ghosh M, Kumar V, Jain D. Ruhal R, et al. Microbiology (Reading). 2023 Sep;169(9):001392. doi: 10.1099/mic.0.001392. Microbiology (Reading). 2023. PMID: 37702709 Free PMC article.

89

Advancing the pipeline of cystic fibrosis clinical trials: a new roadmap with a global trial network perspective.

Mayer-Hamblett N, Clancy JP, Jain R, Donaldson SH, Fajac I, Goss CH, Polineni D, Ratjen F, Quon BS, Zemanick ET, Bell SC, Davies JC, Jain M, Konstan MW, Kerper NR, LaRosa T, Mall MA, McKone E, Pearson K, Pilewski JM, Quittell L, Rayment JH, Rowe SM, Taylor-Cousar JL, Retsch-Bogart G, Downey DG. Mayer-Hamblett N, et al. Lancet Respir Med. 2023 Oct;11(10):932-944. doi: 10.1016/S2213-2600(23)00297-7. Epub 2023 Sep 9. Lancet Respir Med. 2023. PMID: 37699421 Free PMC article. Review.

90

Safety and Reactogenicity of COVID-19 Vaccination in Severe Alpha-1 Antitrypsin Deficiency.

McElvaney OJ, Cleary B, Fraughen DD, Kelly G, McElvaney OF, Murphy MP, Branagan P, Gunaratnam C, Carroll TP, Goss CH, McElvaney NG. McElvaney OJ, et al. Chronic Obstr Pulm Dis. 2024 Jan 25;11(1):3-12. doi: 10.15326/jcopdf.2023.0432. Chronic Obstr Pulm Dis. 2024. PMID: 37676644 Free PMC article.

91

Transposon sequencing identifies genes impacting Staphylococcus aureus invasion in a human macrophage model.

Lo H-Y, Long DR, Holmes EA, Penewit K, Hodgson T, Lewis JD, Waalkes A, Salipante SJ. Lo H-Y, et al. Infect Immun. 2023 Oct 17;91(10):e0022823. doi: 10.1128/iai.00228-23. Epub 2023 Sep 7. Infect Immun. 2023. PMID: 37676013 Free PMC article.

92

Prospective evaluation of nontuberculous mycobacteria disease in cystic fibrosis: The design of the PREDICT study.

Martiniano SL, Caceres SM, Poch K, Rysavy NM, Lovell VK, Armantrout E, Jones M, Anthony M, Keck A, Nichols DP, Vandalfsen JM, Sagel SD, Wagner B, Xie J, Weaver K, Heltshe SL, Daley CL, Davidson RM, Nick JA; Investigators of the Cystic Fibrosis NTM Consortium. Martiniano SL, et al. J Cyst Fibros. 2024 Jan;23(1):50-57. doi: 10.1016/j.jcf.2023.08.007. Epub 2023 Sep 4. J Cyst Fibros. 2024. PMID: 37666709 Free PMC article.

93

Longitudinal Profiling of the Intestinal Microbiome in Children with Cystic Fibrosis Treated with Elexacaftor-Tezacaftor-Ivacaftor.

Reasoner SA, Bernard R, Waalkes A, Penewit K, Lewis J, Sokolow AG, Brown RF, Edwards KM, Salipante SJ, Hadjifrangiskou M, Nicholson MR. Reasoner SA, et al. medRxiv [Preprint]. 2023 Nov 21:2023.08.11.23293949. doi: 10.1101/2023.08.11.23293949. medRxiv. 2023. Update in: mBio. 2024 Feb 14;15(2):e0193523. doi: 10.1128/mbio.01935-23. PMID: 37645804 Free PMC article. Updated. Preprint.

94

Stalled but not forgotten: Bacterial exotoxins inhibit translation to activate NLRP1.

Tibble R, Yonemitsu MA, Mitchell PS. Tibble R, et al. J Exp Med. 2023 Oct 2;220(10):e20231160. doi: 10.1084/jem.20231160. Epub 2023 Aug 29. J Exp Med. 2023. PMID: 37642998 Free PMC article.

95

Intracellular replication of Pseudomonas aeruginosa in epithelial cells requires suppression of the caspase-4 inflammasome.

Kroken AR, Klein KA, Mitchell PS, Nieto V, Jedel EJ, Evans DJ, Fleiszig SMJ. Kroken AR, et al. mSphere. 2023 Oct 24;8(5):e0035123. doi: 10.1128/msphere.00351-23. Epub 2023 Aug 17. mSphere. 2023. PMID: 37589460 Free PMC article.

96

Multicompartmental analysis of the murine pulmonary immune response by spectral flow cytometry.

Chang MY, Brune JE, Black M, Altemeier WA, Frevert CW. Chang MY, et al. Am J Physiol Lung Cell Mol Physiol. 2023 Oct 1;325(4):L518-L535. doi: 10.1152/ajplung.00317.2022. Epub 2023 Aug 15. Am J Physiol Lung Cell Mol Physiol. 2023. PMID: 37581225 Free PMC article.

97

Cystic fibrosis survival outcomes following second lung transplant: The north American experience.

Alshehri M, Ramos KJ, Sykes J, Ma X, Stanojevic S, Quon BS, Marshall BC, Cromwell E, Ostrenga JS, Faro A, Elbert A, Todd J, Chaparro C, Goss CH, Stephenson AL. Alshehri M, et al. Clin Transplant. 2023 Nov;37(11):e15097. doi: 10.1111/ctr.15097. Epub 2023 Aug 10. Clin Transplant. 2023. PMID: 37563332 Free PMC article.

98

The impact of elexacaftor/tezacaftor/ivacaftor on fat-soluble vitamin levels in people with cystic fibrosis.

Hergenroeder GE, Faino A, Bridges G, Bartlett LE, Cogen JD, Green N, McNamara S, Nichols DP, Ramos KJ. Hergenroeder GE, et al. J Cyst Fibros. 2023 Nov;22(6):1048-1053. doi: 10.1016/j.jcf.2023.08.002. Epub 2023 Aug 8. J Cyst Fibros. 2023. PMID: 37563007 Free PMC article.

99

Cell-Free DNA Detects Pseudomonas aeruginosa Lung Infection in Modulator-treated People with Cystic Fibrosis.

Long DR, Holmes EA, Goss CH, Singh PK, Waalkes A, Salipante SJ. Long DR, et al. Am J Respir Crit Care Med. 2023 Nov 1;208(9):944-947. doi: 10.1164/rccm.202305-0844LE. Am J Respir Crit Care Med. 2023. PMID: 37540570 Free PMC article. No abstract available.

100

Lung function decline is mitigated following liver transplantation in people with cystic fibrosis: A retrospective cohort study.

Albaiz FA, Ramos KJ, Sykes J, Stanojevic S, Ma X, Quon BS, Marshall BC, Cromwell EA, Ostrenga JS, Faro A, Elbert A, Goss CH, Stephenson AL. Albaiz FA, et al. Liver Transpl. 2024 Mar 1;30(3):302-310. doi: 10.1097/LVT.0000000000000232. Epub 2023 Aug 3. Liver Transpl. 2024. PMID: 37530842 Free PMC article.

101

Genetic Determinants of Acinetobacter baumannii Serum-Associated Adaptive Efflux-Mediated Antibiotic Resistance.

Young M, Chojnacki M, Blanchard C, Cao X, Johnson WL, Flaherty D, Dunman PM. Young M, et al. Antibiotics (Basel). 2023 Jul 11;12(7):1173. doi: 10.3390/antibiotics12071173. Antibiotics (Basel). 2023. PMID: 37508269 Free PMC article.

102

Discovery of a glutathione utilization pathway in Francisella that shows functional divergence between environmental and pathogenic species.

Wang Y, Ledvina HE, Tower CA, Kambarev S, Liu E, Charity JC, Kreuk LSM, Tang Q, Chen Q, Gallagher LA, Radey MC, Rerolle GF, Li Y, Penewit KM, Turkarslan S, Skerrett SJ, Salipante SJ, Baliga NS, Woodward JJ, Dove SL, Peterson SB, Celli J, Mougous JD. Wang Y, et al. Cell Host Microbe. 2023 Aug 9;31(8):1359-1370.e7. doi: 10.1016/j.chom.2023.06.010. Epub 2023 Jul 14. Cell Host Microbe. 2023. PMID: 37453420 Free PMC article.

103

Impact of lumacaftor/ivacaftor and tezacaftor/ivacaftor on treatment response in pulmonary exacerbations of F508del/F508del cystic fibrosis.

McElvaney OJ, Heltshe SL, Odem-Davis K, West NE, Sanders DB, Fogarty B, VanDevanter DR, Flume PA, Goss CH; STOP2 Investigators. McElvaney OJ, et al. J Cyst Fibros. 2023 Sep;22(5):875-879. doi: 10.1016/j.jcf.2023.06.012. Epub 2023 Jul 6. J Cyst Fibros. 2023. PMID: 37407341 Free PMC article.

104

Role of hyperglycemia in cystic fibrosis pulmonary exacerbations.

Merjaneh L, Sidhaye AR, Vu PT, Heltshe SL, Goss CH, Flume PA, Kelly A, Rosenfeld M on behalf of the STOP2 investigators. Merjaneh L, et al. J Cyst Fibros. 2023 Sep;22(5):868-874. doi: 10.1016/j.jcf.2023.06.011. Epub 2023 Jun 30. J Cyst Fibros. 2023. PMID: 37394317 Free PMC article.

105

Characterization of cerebrospinal fluid (CSF) microbiota at the time of initial surgical intervention for children with hydrocephalus.

Pandey S, Whitlock KB, Test MR, Hodor P, Pope CE, Limbrick DD Jr, McDonald PJ, Hauptman JS, Hoffman LR, Simon TD; Cerebrospinal FLuId MicroBiota in Shunts (CLIMB) Study Group. Pandey S, et al. PLoS One. 2023 Jun 21;18(6):e0280682. doi: 10.1371/journal.pone.0280682. eCollection 2023. PLoS One. 2023. PMID: 37342995 Free PMC article.

106

Antibiotic Regimen Changes during Cystic Fibrosis Pediatric Pulmonary Exacerbation Treatment.

Cogen JD, Sanders DB, Slaven JE, Faino AV, Somayaji R, Gibson RL, Hoffman LR, Ren CL. Cogen JD, et al. Ann Am Thorac Soc. 2023 Sep;20(9):1293-1298. doi: 10.1513/AnnalsATS.202301-078OC. Ann Am Thorac Soc. 2023. PMID: 37327485 Free PMC article.

107

Potential implicit bias in attribution of adverse events in randomized controlled trials in cystic fibrosis.

Somayaji R, Wessels ME, Milinic T, Ramos KJ, Mayer-Hamblett N, Ramsey BW, Heltshe S, Khan U, Goss CH. Somayaji R, et al. J Cyst Fibros. 2023 Jul;22(4):669-673. doi: 10.1016/j.jcf.2023.05.014. Epub 2023 Jun 5. J Cyst Fibros. 2023. PMID: 37286384 Free PMC article.

108

Metabolism and pharmacokinetics of vitamin D in patients with cystic fibrosis.

Bergagnini-Kolev MC, Hsu S, Aitken ML, Goss CH, Hoofnagle AN, Zelnick LR, Lum D, Best CM, Thummel KE, Kestenbaum BR, de Boer IH, Lin YS. Bergagnini-Kolev MC, et al. J Steroid Biochem Mol Biol. 2023 Sep;232:106332. doi: 10.1016/j.jsbmb.2023.106332. Epub 2023 May 20. J Steroid Biochem Mol Biol. 2023. PMID: 37217104 Free PMC article.

109

Cystic Fibrosis: From Tragedy to Triumph.

Ramsey B, Bush A. Ramsey B, et al. Am J Respir Crit Care Med. 2023 Jul 1;208(1):9-11. doi: 10.1164/rccm.202305-0785ED. Am J Respir Crit Care Med. 2023. PMID: 37167625 Free PMC article. No abstract available.

110

A Refined, Controlled 16S rRNA Gene Sequencing Approach Reveals Limited Detection of Cerebrospinal Fluid Microbiota in Children with Bacterial Meningitis.

Pope CE, Whitlock KB, Hodor P, Limbrick DD Jr, McDonald PJ, Hauptman J, Hoffman LR, Simon TD. Pope CE, et al. Microbiol Spectr. 2023 Jun 15;11(3):e0036123. doi: 10.1128/spectrum.00361-23. Epub 2023 May 4. Microbiol Spectr. 2023. PMID: 37140368 Free PMC article.

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Upper airway microbiota development in infants with cystic fibrosis diagnosed by newborn screen.

Harris JK, Wagner BD, Robertson CE, Stevens MJ, Lingard C, Borowitz D, Leung DH, Heltshe SL, Ramsey BW, Zemanick ET. Harris JK, et al. J Cyst Fibros. 2023 Jul;22(4):644-651. doi: 10.1016/j.jcf.2023.04.017. Epub 2023 May 1. J Cyst Fibros. 2023. PMID: 37137746 Free PMC article.

112

Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.

Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, Tuggle KL, Clancy JP, Eggerman TL, Engelhardt JF, Kelly A. Putman MS, et al. Diabetes Care. 2023 Jun 1;46(6):1112-1123. doi: 10.2337/dc23-0380. Diabetes Care. 2023. PMID: 37125948 Free PMC article. Review.

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Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.

Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, Tuggle KL, Clancy JP, Eggerman TL, Engelhardt JF, Kelly A. Putman MS, et al. Diabetes. 2023 Jun 1;72(6):677-689. doi: 10.2337/db22-0949. Diabetes. 2023. PMID: 37125945 Free PMC article. Review.

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Willingness of people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor (ETI) to participate in randomized modulator and inhaled antimicrobial clinical trials.

VanDevanter DR, Zemanick ET, Konstan MW, Ren CL, Odem-Davis K, Emerman I, Young J, Mayer-Hamblett N; CHEC-SC Study Group. VanDevanter DR, et al. J Cyst Fibros. 2023 Jul;22(4):652-655. doi: 10.1016/j.jcf.2023.04.007. Epub 2023 Apr 24. J Cyst Fibros. 2023. PMID: 37100705 Free PMC article.

115

Integrated Physiology of the Exocrine and Endocrine Compartments in Pancreatic Diseases: Workshop Proceedings.

Mastracci TL, Apte M, Amundadottir LT, Alvarsson A, Artandi S, Bellin MD, Bernal-Mizrachi E, Caicedo A, Campbell-Thompson M, Cruz-Monserrate Z, El Ouaamari A, Gaulton KJ, Geisz A, Goodarzi MO, Hara M, Hull-Meichle RL, Kleger A, Klein AP, Kopp JL, Kulkarni RN, Muzumdar MD, Naren AP, Oakes SA, Olesen SS, Phelps EA, Powers AC, Stabler CL, Tirkes T, Whitcomb DC, Yadav D, Yong J, Zaghloul NA, Sander M, Pandol SJ. Mastracci TL, et al. Pancreas. 2022 Oct 1;51(9):1061-1073. doi: 10.1097/MPA.0000000000002170. Pancreas. 2022. PMID: 37078927 Free PMC article.

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Periodontitis Risk Factors in Adults with Cystic Fibrosis: A pilot study.

Alkhateeb AA, Mancl LA, Ramos KJ, Rothen ML, Kotsakis GA, Trence DL, Chi DL. Alkhateeb AA, et al. J Dent Hyg. 2023 Apr;97(2):7-21. J Dent Hyg. 2023. PMID: 37068884 Free PMC article.

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Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist.

Nichols DP, Morgan SJ, Skalland M, Vo AT, Van Dalfsen JM, Singh SB, Ni W, Hoffman LR, McGeer K, Heltshe SL, Clancy JP, Rowe SM, Jorth P, Singh PK; PROMISE-Micro Study Group. Nichols DP, et al. J Clin Invest. 2023 May 15;133(10):e167957. doi: 10.1172/JCI167957. J Clin Invest. 2023. PMID: 36976651 Free PMC article.

118

Integrated Physiology of the Exocrine and Endocrine Compartments in Pancreatic Diseases: Workshop Proceedings.

Mastracci TL, Apte M, Amundadottir LT, Alvarsson A, Artandi S, Bellin MD, Bernal-Mizrachi E, Caicedo A, Campbell-Thompson M, Cruz-Monserrate Z, El Ouaamari A, Gaulton KJ, Geisz A, Goodarzi MO, Hara M, Hull-Meichle RL, Kleger A, Klein AP, Kopp JL, Kulkarni RN, Muzumdar MD, Naren AP, Oakes SA, Olesen SS, Phelps EA, Powers AC, Stabler CL, Tirkes T, Whitcomb DC, Yadav D, Yong J, Zaghloul NA, Pandol SJ, Sander M. Mastracci TL, et al. Diabetes. 2023 Apr 1;72(4):433-448. doi: 10.2337/db22-0942. Diabetes. 2023. PMID: 36940317 Free PMC article. Review.

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Cystic fibrosis prevalence in the United States and participation in the Cystic Fibrosis Foundation Patient Registry in 2020.

Cromwell EA, Ostrenga JS, Todd JV, Elbert A, Brown AW, Faro A, Goss CH, Marshall BC. Cromwell EA, et al. J Cyst Fibros. 2023 May;22(3):436-442. doi: 10.1016/j.jcf.2023.02.009. Epub 2023 Mar 13. J Cyst Fibros. 2023. PMID: 36922288 Free PMC article.

120

Genetic Modifiers of Cystic Fibrosis Lung Disease Severity: Whole-Genome Analysis of 7,840 Patients.

Zhou YH, Gallins PJ, Pace RG, Dang H, Aksit MA, Blue EE, Buckingham KJ, Collaco JM, Faino AV, Gordon WW, Hetrick KN, Ling H, Liu W, Onchiri FM, Pagel K, Pugh EW, Raraigh KS, Rosenfeld M, Sun Q, Wen J, Li Y, Corvol H, Strug LJ, Bamshad MJ, Blackman SM, Cutting GR, Gibson RL, O'Neal WK, Wright FA, Knowles MR. Zhou YH, et al. Am J Respir Crit Care Med. 2023 May 15;207(10):1324-1333. doi: 10.1164/rccm.202209-1653OC. Am J Respir Crit Care Med. 2023. PMID: 36921087 Free PMC article.

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Phase 3 Open-Label Clinical Trial of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged 2-5 Years with Cystic Fibrosis and at Least One F508del Allele.

Goralski JL, Hoppe JE, Mall MA, McColley SA, McKone E, Ramsey B, Rayment JH, Robinson P, Stehling F, Taylor-Cousar JL, Tullis E, Ahluwalia N, Chin A, Chu C, Lu M, Niu T, Weinstock T, Ratjen F, Rosenfeld M. Goralski JL, et al. Am J Respir Crit Care Med. 2023 Jul 1;208(1):59-67. doi: 10.1164/rccm.202301-0084OC. Am J Respir Crit Care Med. 2023. PMID: 36921081 Free PMC article.

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Lung Function Decline in Cystic Fibrosis: Impact of Data Availability and Modeling Strategies on Clinical Interpretations.

Szczesniak R, Andrinopoulou ER, Su W, Afonso PM, Burgel PR, Cromwell E, Gecili E, Ghulam E, Goss CH, Mayer-Hamblett N, Keogh RH, Liou TG, Marshall B, Morgan WJ, Ostrenga JS, Pasta DJ, Stanojevic S, Wainwright C, Zhou GC, Fernandez G, Fink AK, Schechter MS. Szczesniak R, et al. Ann Am Thorac Soc. 2023 Jul;20(7):958-968. doi: 10.1513/AnnalsATS.202209-829OC. Ann Am Thorac Soc. 2023. PMID: 36884219 Free PMC article.

123

In Vitro Selection Identifies Staphylococcus aureus Genes Influencing Biofilm Formation.

Long DR, Penewit K, Lo HY, Almazan J, Holmes EA, Bryan AB, Wolter DJ, Lewis JD, Waalkes A, Salipante SJ. Long DR, et al. Infect Immun. 2023 Mar 15;91(3):e0053822. doi: 10.1128/iai.00538-22. Epub 2023 Feb 27. Infect Immun. 2023. PMID: 36847490 Free PMC article.

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Safety and efficacy of vanzacaftor-tezacaftor-deutivacaftor in adults with cystic fibrosis: randomised, double-blind, controlled, phase 2 trials.

Uluer AZ, MacGregor G, Azevedo P, Indihar V, Keating C, Mall MA, McKone EF, Ramsey BW, Rowe SM, Rubenstein RC, Taylor-Cousar JL, Tullis E, Yonker LM, Chu C, Lam AP, Nair N, Sosnay PR, Tian S, Van Goor F, Viswanathan L, Waltz D, Wang LT, Xi Y, Billings J, Horsley A; VX18-121-101; VX18-561-101 Study Groups. Uluer AZ, et al. Lancet Respir Med. 2023 Jun;11(6):550-562. doi: 10.1016/S2213-2600(22)00504-5. Epub 2023 Feb 23. Lancet Respir Med. 2023. PMID: 36842446 Free PMC article. Clinical Trial.

125

Intracellular replication of Pseudomonas aeruginosa in epithelial cells requires suppression of the caspase-4 inflammasome.

Kroken AR, Klein KA, Mitchell PS, Nieto V, Jedel EJ, Evans DJ, Fleiszig SMJ. Kroken AR, et al. bioRxiv [Preprint]. 2023 Apr 28:2023.02.13.528260. doi: 10.1101/2023.02.13.528260. bioRxiv. 2023. Update in: mSphere. 2023 Oct 24;8(5):e0035123. doi: 10.1128/msphere.00351-23. PMID: 36824932 Free PMC article. Updated. Preprint.

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Re-examining baseline lung function recovery following IV-treated pulmonary exacerbations.

Heltshe SL, Russell R, VanDevanter DR, Sanders DB. Heltshe SL, et al. J Cyst Fibros. 2023 Sep;22(5):864-867. doi: 10.1016/j.jcf.2023.02.006. Epub 2023 Feb 16. J Cyst Fibros. 2023. PMID: 36803635 Free PMC article.

127

Diagnosis and Management of Cystic Fibrosis Exacerbations.

Milinic T, McElvaney OJ, Goss CH. Milinic T, et al. Semin Respir Crit Care Med. 2023 Apr;44(2):225-241. doi: 10.1055/s-0042-1760250. Epub 2023 Feb 6. Semin Respir Crit Care Med. 2023. PMID: 36746183 Free PMC article. Review.

128

Transforming the nutrition care model for infants with cystic fibrosis: A qualitative study of clinicians' perspectives.

Ong T, Bell S, Britto MT, Gamel B, McNamara S, Ramsey B, Barton KS. Ong T, et al. Pediatr Pulmonol. 2023 May;58(5):1380-1390. doi: 10.1002/ppul.26330. Epub 2023 Feb 8. Pediatr Pulmonol. 2023. PMID: 36695543 Free PMC article.

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Pseudomonas aeruginosa aggregation and Psl expression in sputum is associated with antibiotic eradication failure in children with cystic fibrosis.

Morris AJ, Yau YCW, Park S, Eisha S, McDonald N, Parsek MR, Howell PL, Hoffman LR, Nguyen D, DiGiandomenico A, Rooney AM, Coburn B, Grana-Miraglia L, Wang P, Guttman DS, Wozniak DJ, Waters VJ. Morris AJ, et al. Sci Rep. 2022 Dec 12;12(1):21444. doi: 10.1038/s41598-022-25889-6. Sci Rep. 2022. PMID: 36509824 Free PMC article.

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Eradication of early MRSA infection in cystic fibrosis: a novel study design for the STAR-ter trial.

Cunningham F, Caldwell E, Mayer-Hamblett N, Goss CH, Muhlebach MS. Cunningham F, et al. ERJ Open Res. 2022 Dec 5;8(4):00190-2022. doi: 10.1183/23120541.00190-2022. eCollection 2022 Oct. ERJ Open Res. 2022. PMID: 36478917 Free PMC article.

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Hypermutator strains of Pseudomonas aeruginosa reveal novel pathways of resistance to combinations of cephalosporin antibiotics and beta-lactamase inhibitors.

Dulanto Chiang A, Patil PP, Beka L, Youn JH, Launay A, Bonomo RA, Khil PP, Dekker JP. Dulanto Chiang A, et al. PLoS Biol. 2022 Nov 18;20(11):e3001878. doi: 10.1371/journal.pbio.3001878. eCollection 2022 Nov. PLoS Biol. 2022. PMID: 36399436 Free PMC article.

132

Contemporary cystic fibrosis incidence rates in Canada and the United States.

Stephenson AL, Swaleh S, Sykes J, Stanojevic S, Ma X, Quon BS, Faro A, Marshall B, Ramos KJ, Ostrenga J, Elbert A, Desai S, Cromwell E, Goss CH. Stephenson AL, et al. J Cyst Fibros. 2023 May;22(3):443-449. doi: 10.1016/j.jcf.2022.10.008. Epub 2022 Nov 10. J Cyst Fibros. 2023. PMID: 36371312 Free PMC article.

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Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials.

Mayer-Hamblett N, Ratjen F, Russell R, Donaldson SH, Riekert KA, Sawicki GS, Odem-Davis K, Young JK, Rosenbluth D, Taylor-Cousar JL, Goss CH, Retsch-Bogart G, Clancy JP, Genatossio A, O'Sullivan BP, Berlinski A, Millard SL, Omlor G, Wyatt CA, Moffett K, Nichols DP, Gifford AH; SIMPLIFY Study Group. Mayer-Hamblett N, et al. Lancet Respir Med. 2023 Apr;11(4):329-340. doi: 10.1016/S2213-2600(22)00434-9. Epub 2022 Nov 4. Lancet Respir Med. 2023. PMID: 36343646 Free PMC article. Clinical Trial.

134

Rate of Lung Function Decline in People with Cystic Fibrosis Having a Residual Function Gene Mutation.

Sawicki GS, Konstan MW, McKone EF, Moss RB, Lubarsky B, Suthoff E, Millar SJ, Pasta DJ, Mayer-Hamblett N, Goss CH, Morgan WJ, Duncan ME, Yang Y. Sawicki GS, et al. Pulm Ther. 2022 Dec;8(4):385-395. doi: 10.1007/s41030-022-00202-y. Epub 2022 Nov 1. Pulm Ther. 2022. PMID: 36319933 Free PMC article.

135

Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis.

Moshiree B, Freeman AJ, Vu PT, Khan U, Ufret-Vincenty C, Heltshe SL, Goss CH, Schwarzenberg SJ, Freedman SD, Borowitz D, Sathe M; GALAXY Study Group. Moshiree B, et al. J Cyst Fibros. 2023 Mar;22(2):266-274. doi: 10.1016/j.jcf.2022.10.006. Epub 2022 Oct 29. J Cyst Fibros. 2023. PMID: 36319569 Free PMC article.

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Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI.

Schwarzenberg SJ, Vu PT, Skalland M, Hoffman LR, Pope C, Gelfond D, Narkewicz MR, Nichols DP, Heltshe SL, Donaldson SH, Frederick CA, Kelly A, Pittman JE, Ratjen F, Rosenfeld M, Sagel SD, Solomon GM, Stalvey MS, Clancy JP, Rowe SM, Freedman SD; Promise Study Group. Schwarzenberg SJ, et al. J Cyst Fibros. 2023 Mar;22(2):282-289. doi: 10.1016/j.jcf.2022.10.003. Epub 2022 Oct 21. J Cyst Fibros. 2023. PMID: 36280527 Free PMC article.

137

T-cell deficiency and hyperinflammatory monocyte responses associate with Mycobacterium avium complex lung disease.

Lindestam Arlehamn CS, Benson B, Kuan R, Dill-McFarland KA, Peterson GJ, Paul S, Nguyen FK, Gilman RH, Saito M, Taplitz R, Arentz M, Goss CH, Aitken ML, Horne DJ, Shah JA, Sette A, Hawn TR. Lindestam Arlehamn CS, et al. Front Immunol. 2022 Oct 3;13:1016038. doi: 10.3389/fimmu.2022.1016038. eCollection 2022. Front Immunol. 2022. PMID: 36263044 Free PMC article.

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Pleiotropic modifiers of age-related diabetes and neonatal intestinal obstruction in cystic fibrosis.

Aksit MA, Ling H, Pace RG, Raraigh KS, Onchiri F, Faino AV, Pagel K, Pugh E, Stilp AM, Sun Q, Blue EE, Wright FA, Zhou YH, Bamshad MJ, Gibson RL, Knowles MR, Cutting GR, Blackman SM; CF Genome Project. Aksit MA, et al. Am J Hum Genet. 2022 Oct 6;109(10):1894-1908. doi: 10.1016/j.ajhg.2022.09.004. Am J Hum Genet. 2022. PMID: 36206743 Free PMC article.

139

Take on transplant: human-centered design of a patient education tool to facilitate informed discussions about lung transplant among people with cystic fibrosis.

Hartzler AL, Bartlett LE, Hobler MR, Reid N, Pryor JB, Kapnadak SG, Berry DL, Lober WB, Goss CH, Ramos KJ; Take on Transplant Study Group. Hartzler AL, et al. J Am Med Inform Assoc. 2022 Dec 13;30(1):26-37. doi: 10.1093/jamia/ocac176. J Am Med Inform Assoc. 2022. PMID: 36173364 Free PMC article.

140

Genome Capture Sequencing Selectively Enriches Bacterial DNA and Enables Genome-Wide Measurement of Intrastrain Genetic Diversity in Human Infections.

Hayden HS, Joshi S, Radey MC, Vo AT, Forsberg C, Morgan SJ, Waalkes A, Holmes EA, Klee SM, Emond MJ, Singh PK, Salipante SJ. Hayden HS, et al. mBio. 2022 Oct 26;13(5):e0142422. doi: 10.1128/mbio.01424-22. Epub 2022 Sep 19. mBio. 2022. PMID: 36121157 Free PMC article.

141

Drugs, Drugs, Drugs: Current Treatment Paradigms in Cystic Fibrosis Airway Infections.

Cogen JD, Nichols DP, Goss CH, Somayaji R. Cogen JD, et al. J Pediatric Infect Dis Soc. 2022 Sep 7;11(Supplement_2):S32-S39. doi: 10.1093/jpids/piac061. J Pediatric Infect Dis Soc. 2022. PMID: 36069901 Free PMC article. Review.

142

Systemic Corticosteroids in the Management of Pediatric Cystic Fibrosis Pulmonary Exacerbations.

Davis CS, Faino AV, Onchiri F, Gibson RL, Merjaneh L, Ramsey BW, Rosenfeld M, Cogen JD. Davis CS, et al. Ann Am Thorac Soc. 2023 Jan;20(1):75-82. doi: 10.1513/AnnalsATS.202203-201OC. Ann Am Thorac Soc. 2023. PMID: 36044723 Free PMC article.

143

An in vivo Biomarker to Characterize Ototoxic Compounds and Novel Protective Therapeutics.

Bellairs JA, Redila VA, Wu P, Tong L, Webster A, Simon JA, Rubel EW, Raible DW. Bellairs JA, et al. Front Mol Neurosci. 2022 Jul 18;15:944846. doi: 10.3389/fnmol.2022.944846. eCollection 2022. Front Mol Neurosci. 2022. PMID: 35923755 Free PMC article.

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Addressing Missing Data in GC × GC Metabolomics: Identifying Missingness Type and Evaluating the Impact of Imputation Methods on Experimental Replication.

Davis TJ, Firzli TR, Higgins Keppler EA, Richardson M, Bean HD. Davis TJ, et al. Anal Chem. 2022 Aug 9;94(31):10912-10920. doi: 10.1021/acs.analchem.1c04093. Epub 2022 Jul 26. Anal Chem. 2022. PMID: 35881554 Free PMC article.

145

Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study.

Mayer-Hamblett N, Zemanick ET, Odem-Davis K, VanDevanter D, Warden M, Rowe SM, Young J, Konstan MW, For-The-Chec-Sc-Study-Group. Mayer-Hamblett N, et al. J Cyst Fibros. 2023 Jan;22(1):79-88. doi: 10.1016/j.jcf.2022.07.008. Epub 2022 Jul 21. J Cyst Fibros. 2023. PMID: 35871974 Free PMC article.

146

Hemoptysis and the Risk for Lung Transplant or Death without Transplant in Individuals with Cystic Fibrosis in the United States.

Bayomy OF, Ramos KJ, Hee Wai T, Kapnadak SG, Morrell ED, Nomitch JT, Pollack LR, Lease ED, Aitken ML, Stephenson AL, Goss CH. Bayomy OF, et al. Ann Am Thorac Soc. 2022 Dec;19(12):1986-1992. doi: 10.1513/AnnalsATS.202202-110OC. Ann Am Thorac Soc. 2022. PMID: 35759341 Free PMC article. Review.

147

The lung allocation score and other available models lack predictive accuracy for post-lung transplant survival.

Brahmbhatt JM, Hee Wai T, Goss CH, Lease ED, Merlo CA, Kapnadak SG, Ramos KJ. Brahmbhatt JM, et al. J Heart Lung Transplant. 2022 Aug;41(8):1063-1074. doi: 10.1016/j.healun.2022.05.008. Epub 2022 May 20. J Heart Lung Transplant. 2022. PMID: 35690561 Free PMC article.

148

Genome-wide protein-DNA interaction site mapping in bacteria using a double-stranded DNA-specific cytosine deaminase.

Gallagher LA, Velazquez E, Peterson SB, Charity JC, Radey MC, Gebhardt MJ, Hsu F, Shull LM, Cutler KJ, Macareno K, de Moraes MH, Penewit KM, Kim J, Andrade PA, LaFramboise T, Salipante SJ, Reniere ML, de Lorenzo V, Wiggins PA, Dove SL, Mougous JD. Gallagher LA, et al. Nat Microbiol. 2022 Jun;7(6):844-855. doi: 10.1038/s41564-022-01133-9. Epub 2022 Jun 1. Nat Microbiol. 2022. PMID: 35650286 Free PMC article.

149

Accounting for population structure in genetic studies of cystic fibrosis.

Kingston H, Stilp AM, Gordon W, Broome J, Gogarten SM, Ling H, Barnard J, Dugan-Perez S, Ellinor PT, Gabriel S, Germer S, Gibbs RA, Gupta N, Rice K, Smith AV, Zody MC; Cystic Fibrosis Genome Project; NHLBI Trans-Omics for Precision Medicine (TOPMed) Consortium; Blackman SM, Cutting G, Knowles MR, Zhou YH, Rosenfeld M, Gibson RL, Bamshad M, Fohner A, Blue EE. Kingston H, et al. HGG Adv. 2022 May 12;3(3):100117. doi: 10.1016/j.xhgg.2022.100117. eCollection 2022 Jul 14. HGG Adv. 2022. PMID: 35647563 Free PMC article.

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Development and Internal Validation of a Prognostic Model of the Probability of Death or Lung Transplantation Within 2 Years for Patients With Cystic Fibrosis and FEV₁ ≤ 50% Predicted.

Ramos KJ, Hee Wai T, Stephenson AL, Sykes J, Stanojevic S, Rodriguez PJ, Bansal A, Mayer-Hamblett N, Goss CH, Kapnadak SG. Ramos KJ, et al. Chest. 2022 Oct;162(4):757-767. doi: 10.1016/j.chest.2022.05.021. Epub 2022 May 26. Chest. 2022. PMID: 35643116 Free PMC article.

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Association between stool consistency and clinical variables among infants with cystic fibrosis: Findings from the BONUS study.

Freeman AJ, Huang R, Heltshe SL, Gelfond D, Leung DH, Ramsey BR, Borowitz D, Sathe M; BONUS Study Investigators. Freeman AJ, et al. J Cyst Fibros. 2022 Sep;21(5):830-836. doi: 10.1016/j.jcf.2022.05.003. Epub 2022 May 16. J Cyst Fibros. 2022. PMID: 35585010 Free PMC article.

152

Tissue remodeling by an opportunistic pathogen triggers allergic inflammation.

Agaronyan K, Sharma L, Vaidyanathan B, Glenn K, Yu S, Annicelli C, Wiggen TD, Penningroth MR, Hunter RC, Dela Cruz CS, Medzhitov R. Agaronyan K, et al. Immunity. 2022 May 10;55(5):895-911.e10. doi: 10.1016/j.immuni.2022.04.001. Epub 2022 Apr 27. Immunity. 2022. PMID: 35483356 Free PMC article.

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Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients.

Ramos KJ, Guimbellot JS, Valapour M, Bartlett LE, Wai TH, Goss CH, Pilewski JM, Faro A, Diamond JM; CFLTC Study Group. Ramos KJ, et al. J Cyst Fibros. 2022 Sep;21(5):745-752. doi: 10.1016/j.jcf.2022.04.009. Epub 2022 Apr 23. J Cyst Fibros. 2022. PMID: 35474016 Free PMC article.

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Genetic and Transcriptomic Characteristics of RhlR-Dependent Quorum Sensing in Cystic Fibrosis Isolates of Pseudomonas aeruginosa.

Asfahl KL, Smalley NE, Chang AP, Dandekar AA. Asfahl KL, et al. mSystems. 2022 Apr 26;7(2):e0011322. doi: 10.1128/msystems.00113-22. Epub 2022 Apr 11. mSystems. 2022. PMID: 35471121 Free PMC article.

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Antipseudomonal treatment decisions during CF exacerbation management.

VanDevanter DR, West NE, Sanders DB, Skalland M, Goss CH, Flume PA, Heltshe SL. VanDevanter DR, et al. J Cyst Fibros. 2022 Sep;21(5):753-758. doi: 10.1016/j.jcf.2022.04.006. Epub 2022 Apr 22. J Cyst Fibros. 2022. PMID: 35466039 Free PMC article.

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Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D.

Hoppe JE, Wagner BD, Kirk Harris J, Rowe SM, Heltshe SL, DeBoer EM, Sagel SD. Hoppe JE, et al. J Cyst Fibros. 2022 Nov;21(6):950-958. doi: 10.1016/j.jcf.2022.03.012. Epub 2022 Apr 16. J Cyst Fibros. 2022. PMID: 35440409 Free PMC article.

157

Club cell secretory protein and lung function in children with cystic fibrosis.

Zhai J, Emond MJ, Spangenberg A, Stern DA, Vasquez MM, Blue EE, Buckingham KJ, Sherrill DL, Halonen M, Gibson RL, Rosenfeld M, Sagel SD, Bamshad MJ, Morgan WJ, Guerra S. Zhai J, et al. J Cyst Fibros. 2022 Sep;21(5):811-820. doi: 10.1016/j.jcf.2022.03.007. Epub 2022 Mar 31. J Cyst Fibros. 2022. PMID: 35367162 Free PMC article.

158

Health care costs in a randomized trial of antimicrobial duration among cystic fibrosis patients with pulmonary exacerbations.

Gold LS, Hansen RN, Patrick DL, Tabah A, Heltshe SL, Flume PA, Goss CH, West NE, Sanders DB, VanDevanter DR, Kessler L. Gold LS, et al. J Cyst Fibros. 2022 Jul;21(4):594-599. doi: 10.1016/j.jcf.2022.03.001. Epub 2022 Mar 14. J Cyst Fibros. 2022. PMID: 35300932 Free PMC article. Clinical Trial.

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Impact of azithromycin on serum inflammatory markers in children with cystic fibrosis and new Pseudomonas.

Pittman JE, Skalland MS, Sagel SD, Ramsey BW, Mayer-Hamblett N, Retsch-Bogart GZ. Pittman JE, et al. J Cyst Fibros. 2022 Nov;21(6):946-949. doi: 10.1016/j.jcf.2022.02.015. Epub 2022 Mar 5. J Cyst Fibros. 2022. PMID: 35260354 Free PMC article. Clinical Trial.

160

A Framework for Using Real-World Data and Health Outcomes Modeling to Evaluate Machine Learning-Based Risk Prediction Models.

Rodriguez PJ, Veenstra DL, Heagerty PJ, Goss CH, Ramos KJ, Bansal A. Rodriguez PJ, et al. Value Health. 2022 Mar;25(3):350-358. doi: 10.1016/j.jval.2021.11.1360. Epub 2021 Dec 22. Value Health. 2022. PMID: 35227445 Free PMC article.

161

Discovery of coordinately regulated pathways that provide innate protection against interbacterial antagonism.

Ting SY, LaCourse KD, Ledvina HE, Zhang R, Radey MC, Kulasekara HD, Somavanshi R, Bertolli SK, Gallagher LA, Kim J, Penewit KM, Salipante SJ, Xu L, Peterson SB, Mougous JD. Ting SY, et al. Elife. 2022 Feb 17;11:e74658. doi: 10.7554/eLife.74658. Elife. 2022. PMID: 35175195 Free PMC article.

162

Leveraging TOPMed imputation server and constructing a cohort-specific imputation reference panel to enhance genotype imputation among cystic fibrosis patients.

Sun Q, Liu W, Rosen JD, Huang L, Pace RG, Dang H, Gallins PJ, Blue EE, Ling H, Corvol H, Strug LJ, Bamshad MJ, Gibson RL, Pugh EW, Blackman SM, Cutting GR, O'Neal WK, Zhou YH, Wright FA, Knowles MR, Wen J, Li Y; Cystic Fibrosis Genome Project. Sun Q, et al. HGG Adv. 2022 Jan 11;3(2):100090. doi: 10.1016/j.xhgg.2022.100090. eCollection 2022 Apr 14. HGG Adv. 2022. PMID: 35128485 Free PMC article.

163

Improving lung transplant outcomes in France: the high emergency lung transplantation programme.

Bayomy OF, Ramos KJ, Goss CH. Bayomy OF, et al. Eur Respir J. 2022 Jan 27;59(1):2102209. doi: 10.1183/13993003.02209-2021. Print 2022 Jan. Eur Respir J. 2022. PMID: 35086845 Free PMC article. No abstract available.

164

Cystic Fibrosis: A Disease in Transformation, yet More Work to Be Done!

Ramsey BW, Bell SC. Ramsey BW, et al. Am J Respir Crit Care Med. 2022 Mar 1;205(5):487-489. doi: 10.1164/rccm.202112-2782ED. Am J Respir Crit Care Med. 2022. PMID: 35073504 Free PMC article. No abstract available.

165

Body Mass Index Recovery after Lung Transplant for Cystic Fibrosis.

Pryor JB, Bradford MC, Jennerich AL, Wai TYH, Pilewski JM, Kapnadak SG, Aitken ML, Goss CH, Ramos KJ. Pryor JB, et al. Ann Am Thorac Soc. 2022 Jul;19(7):1130-1138. doi: 10.1513/AnnalsATS.202108-969OC. Ann Am Thorac Soc. 2022. PMID: 35044894 Free PMC article.

166

Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood.

Marks MP, Heltshe SL, Baines A, Ramsey BW, Hoffman LR, Stalvey MS. Marks MP, et al. Nutrients. 2021 Dec 10;13(12):4414. doi: 10.3390/nu13124414. Nutrients. 2021. PMID: 34959966 Free PMC article.

167

A population-level strain genotyping method to study pathogen strain dynamics in human infections.

Morgan SJ, Durfey SL, Ravishankar S, Jorth P, Ni W, Skerrett DT, Aitken ML, McKone EF, Salipante SJ, Radey MC, Singh PK. Morgan SJ, et al. JCI Insight. 2021 Dec 22;6(24):e152472. doi: 10.1172/jci.insight.152472. JCI Insight. 2021. PMID: 34935640 Free PMC article.

168

C-reactive protein (CRP) as a biomarker of pulmonary exacerbation presentation and treatment response.

VanDevanter DR, Heltshe SL, Skalland M, West NE, Sanders DB, Goss CH, Flume PA. VanDevanter DR, et al. J Cyst Fibros. 2022 Jul;21(4):588-593. doi: 10.1016/j.jcf.2021.12.003. Epub 2021 Dec 18. J Cyst Fibros. 2022. PMID: 34933824 Free PMC article. Clinical Trial.

169

Low body mass index as a barrier to lung transplant in cystic fibrosis.

Jennerich AL, Pryor JB, Wai TYH, Kapnadak SG, Aitken ML, Goss CH, Ramos KJ. Jennerich AL, et al. J Cyst Fibros. 2022 May;21(3):475-481. doi: 10.1016/j.jcf.2021.12.001. Epub 2021 Dec 23. J Cyst Fibros. 2022. PMID: 34922852 Free PMC article.

170

The role of modulators in cystic fibrosis related diabetes.

Merjaneh L, Hasan S, Kasim N, Ode KL. Merjaneh L, et al. J Clin Transl Endocrinol. 2021 Dec 7;27:100286. doi: 10.1016/j.jcte.2021.100286. eCollection 2022 Mar. J Clin Transl Endocrinol. 2021. PMID: 34917484 Free PMC article.

171

Eating disorders and body image in cystic fibrosis.

Darukhanavala A, Merjaneh L, Mason K, Le T. Darukhanavala A, et al. J Clin Transl Endocrinol. 2021 Nov 26;26:100280. doi: 10.1016/j.jcte.2021.100280. eCollection 2021 Dec. J Clin Transl Endocrinol. 2021. PMID: 34917482 Free PMC article.

172

A randomized double-blind placebo controlled pilot study of probiotics in adolescents with severe obesity.

Verma A, Nelson MT, DePaolo WR, Hampe C, Roth CL. Verma A, et al. J Diabetes Metab Disord. 2021 Aug 8;20(2):1289-1300. doi: 10.1007/s40200-021-00855-7. eCollection 2021 Dec. J Diabetes Metab Disord. 2021. PMID: 34900780 Free PMC article.

173

A new path for CF clinical trials through the use of historical controls.

Magaret AS, Warden M, Simon N, Heltshe S, Retsch-Bogart GZ, Ramsey BW, Mayer-Hamblett N. Magaret AS, et al. J Cyst Fibros. 2022 Mar;21(2):293-299. doi: 10.1016/j.jcf.2021.11.007. Epub 2021 Dec 5. J Cyst Fibros. 2022. PMID: 34879997 Free PMC article.

174

Association of site of treatment with clinical outcomes following intravenous antimicrobial treatment of a pulmonary exacerbation.

Sanders DB, Khan U, Heltshe SL, Skalland M, West NE, VanDevanter DR, Goss CH, Flume PA; STOP2 Investigators. Sanders DB, et al. J Cyst Fibros. 2022 Jul;21(4):574-580. doi: 10.1016/j.jcf.2021.11.009. Epub 2021 Nov 29. J Cyst Fibros. 2022. PMID: 34857494 Free PMC article. Clinical Trial.

175

Variability in azithromycin practices among lung transplant providers in the International Society for Heart and Lung Transplantation Community.

Kapnadak SG, Morrell ED, Wai TH, Goss CH, Shah PD, Merlo CA, Hachem RR, Ramos KJ. Kapnadak SG, et al. J Heart Lung Transplant. 2022 Jan;41(1):20-23. doi: 10.1016/j.healun.2021.10.008. Epub 2021 Oct 22. J Heart Lung Transplant. 2022. PMID: 34785136 Free PMC article.

176

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial.

Nichols DP, Paynter AC, Heltshe SL, Donaldson SH, Frederick CA, Freedman SD, Gelfond D, Hoffman LR, Kelly A, Narkewicz MR, Pittman JE, Ratjen F, Rosenfeld M, Sagel SD, Schwarzenberg SJ, Singh PK, Solomon GM, Stalvey MS, Clancy JP, Kirby S, Van Dalfsen JM, Kloster MH, Rowe SM; PROMISE Study group. Nichols DP, et al. Am J Respir Crit Care Med. 2022 Mar 1;205(5):529-539. doi: 10.1164/rccm.202108-1986OC. Am J Respir Crit Care Med. 2022. PMID: 34784492 Free PMC article.

177

Heterogeneity of Diabetes: β-Cells, Phenotypes, and Precision Medicine: Proceedings of an International Symposium of the Canadian Institutes of Health Research's Institute of Nutrition, Metabolism and Diabetes and the U.S. National Institutes of Health's National Institute of Diabetes and Digestive and Kidney Diseases.

Cefalu WT, Andersen DK, Arreaza-Rubín G, Pin CL, Sato S, Verchere CB, Woo M, Rosenblum ND; Symposium planning committee, moderators, and speakers. Cefalu WT, et al. Diabetes Care. 2022 Jan 1;45(1):3-22. doi: 10.2337/dci21-0051. Diabetes Care. 2022. PMID: 34782355 Free PMC article. Review.

178

Testing the effects of combining azithromycin with inhaled tobramycin for P. aeruginosa in cystic fibrosis: a randomised, controlled clinical trial.

Nichols DP, Singh PK, Baines A, Caverly LJ, Chmiel JF, GIbson RL, Lascano J, Morgan SJ, Retsch-Bogart G, Saiman L, Sadeghi H, Billings JL, Heltshe SL, Kirby S, Kong A, Nick JA, Mayer-Hamblett N; TEACH Study Group. Nichols DP, et al. Thorax. 2022 Jun;77(6):581-588. doi: 10.1136/thoraxjnl-2021-217782. Epub 2021 Oct 27. Thorax. 2022. PMID: 34706982 Free PMC article. Clinical Trial.

179

Effects of Tham Nasal Alkalinization on Airway Microbial Communities: A Pilot Study in Non-CF and CF Adults.

Holliday ZM, Launspach JL, Durairaj L, Singh PK, Zabner J, Stoltz DA. Holliday ZM, et al. Ann Otol Rhinol Laryngol. 2022 Sep;131(9):1013-1020. doi: 10.1177/00034894211051814. Epub 2021 Oct 21. Ann Otol Rhinol Laryngol. 2022. PMID: 34674574 Free PMC article. Clinical Trial.

180

The antiactivator FleN uses an allosteric mechanism to regulate σ⁵⁴-dependent expression of flagellar genes in Pseudomonas aeruginosa.

Chanchal, Banerjee P, Raghav S, Goswami HN, Jain D. Chanchal, et al. Sci Adv. 2021 Oct 22;7(43):eabj1792. doi: 10.1126/sciadv.abj1792. Epub 2021 Oct 20. Sci Adv. 2021. PMID: 34669473 Free PMC article.

181

COVID-19 outcomes in people with cystic fibrosis.

Burgel PR, Goss C. Burgel PR, et al. Curr Opin Pulm Med. 2021 Nov 1;27(6):538-543. doi: 10.1097/MCP.0000000000000823. Curr Opin Pulm Med. 2021. PMID: 34620788 Free PMC article. Review.

182

Infants with cystic fibrosis have altered fecal functional capacities with potential clinical and metabolic consequences.

Eng A, Hayden HS, Pope CE, Brittnacher MJ, Vo AT, Weiss EJ, Hager KR, Leung DH, Heltshe SL, Raftery D, Miller SI, Hoffman LR, Borenstein E. Eng A, et al. BMC Microbiol. 2021 Sep 15;21(1):247. doi: 10.1186/s12866-021-02305-z. BMC Microbiol. 2021. PMID: 34525965 Free PMC article.

183

A comparison of clinic and home spirometry as longtudinal outcomes in cystic fibrosis.

Paynter A, Khan U, Heltshe SL, Goss CH, Lechtzin N, Hamblett NM. Paynter A, et al. J Cyst Fibros. 2022 Jan;21(1):78-83. doi: 10.1016/j.jcf.2021.08.013. Epub 2021 Aug 31. J Cyst Fibros. 2022. PMID: 34474987 Free PMC article.

184

A Randomized Clinical Trial of Antimicrobial Duration for Cystic Fibrosis Pulmonary Exacerbation Treatment.

Goss CH, Heltshe SL, West NE, Skalland M, Sanders DB, Jain R, Barto TL, Fogarty B, Marshall BC, VanDevanter DR, Flume PA; STOP2 Investigators. Goss CH, et al. Am J Respir Crit Care Med. 2021 Dec 1;204(11):1295-1305. doi: 10.1164/rccm.202102-0461OC. Am J Respir Crit Care Med. 2021. PMID: 34469706 Free PMC article. Clinical Trial.

185

Validation of the French 3-year prognostic score for death or lung transplant in the United States cystic fibrosis population.

Ramos KJ, Wai TH, Sykes J, Ma X, Stephenson AL, Jennerich AL, Kapnadak SG, Mayer-Hamblett N, Goss CH. Ramos KJ, et al. J Cyst Fibros. 2022 May;21(3):471-474. doi: 10.1016/j.jcf.2021.08.008. Epub 2021 Aug 28. J Cyst Fibros. 2022. PMID: 34465539 Free PMC article.

186

Impact of guideline-recommended dietitian assessments on weight gain in infants with cystic fibrosis.

Ong T, Onchiri FM, Britto MT, Heltshe SL, Kessler LG, Seid M, Ramsey BW. Ong T, et al. J Cyst Fibros. 2022 Jan;21(1):115-122. doi: 10.1016/j.jcf.2021.08.005. Epub 2021 Aug 26. J Cyst Fibros. 2022. PMID: 34454845 Free PMC article.

187

Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.

Barry PJ, Mall MA, Álvarez A, Colombo C, de Winter-de Groot KM, Fajac I, McBennett KA, McKone EF, Ramsey BW, Sutharsan S, Taylor-Cousar JL, Tullis E, Ahluwalia N, Jun LS, Moskowitz SM, Prieto-Centurion V, Tian S, Waltz D, Xuan F, Zhang Y, Rowe SM, Polineni D; VX18-445-104 Study Group. Barry PJ, et al. N Engl J Med. 2021 Aug 26;385(9):815-825. doi: 10.1056/NEJMoa2100665. N Engl J Med. 2021. PMID: 34437784 Free PMC article. Clinical Trial.

188

Utilization of electronic patient-reported outcome measures in cystic fibrosis research: Application to the GALAXY study.

Sathe M, Moshiree B, Vu PT, Khan U, Heltshe SL, Romasco M, Freedman SD, Schwarzenberg SJ, Goss CH, Freeman AJ. Sathe M, et al. J Cyst Fibros. 2021 Jul;20(4):605-611. doi: 10.1016/j.jcf.2021.07.002. Epub 2021 Jul 22. J Cyst Fibros. 2021. PMID: 34305007 Free PMC article.

189

Identification of small molecules that interfere with c-di-GMP signaling and induce dispersal of Pseudomonas aeruginosa biofilms.

Andersen JB, Hultqvist LD, Jansen CU, Jakobsen TH, Nilsson M, Rybtke M, Uhd J, Fritz BG, Seifert R, Berthelsen J, Nielsen TE, Qvortrup K, Givskov M, Tolker-Nielsen T. Andersen JB, et al. NPJ Biofilms Microbiomes. 2021 Jul 9;7(1):59. doi: 10.1038/s41522-021-00225-4. NPJ Biofilms Microbiomes. 2021. PMID: 34244523 Free PMC article.

190

The β Cell in Diabetes: Integrating Biomarkers With Functional Measures.

Kahn SE, Chen YC, Esser N, Taylor AJ, van Raalte DH, Zraika S, Verchere CB. Kahn SE, et al. Endocr Rev. 2021 Sep 28;42(5):528-583. doi: 10.1210/endrev/bnab021. Endocr Rev. 2021. PMID: 34180979 Free PMC article. Review.

191

Phenotypic characteristics of incident and chronic MRSA isolates in cystic fibrosis.

Gilpin D, Hoffman LR, Ceppe A, Muhlebach MS. Gilpin D, et al. J Cyst Fibros. 2021 Jul;20(4):692-698. doi: 10.1016/j.jcf.2021.05.015. Epub 2021 Jun 6. J Cyst Fibros. 2021. PMID: 34103251 Free PMC article.

192

Association Between Number of Intravenous Antipseudomonal Antibiotics and Clinical Outcomes of Pediatric Cystic Fibrosis Pulmonary Exacerbations.

Cogen JD, Faino AV, Onchiri F, Hoffman LR, Kronman MP, Nichols DP, Rosenfeld M, Gibson RL. Cogen JD, et al. Clin Infect Dis. 2021 Nov 2;73(9):1589-1596. doi: 10.1093/cid/ciab525. Clin Infect Dis. 2021. PMID: 34100912 Free PMC article.

193

Gastrointestinal Factors Associated With Hospitalization in Infants With Cystic Fibrosis: Results From the Baby Observational and Nutrition Study.

Sathe M, Huang R, Heltshe S, Eng A, Borenstein E, Miller SI, Hoffman L, Gelfond D, Leung DH, Borowitz D, Ramsey B, Freeman AJ. Sathe M, et al. J Pediatr Gastroenterol Nutr. 2021 Sep 1;73(3):395-402. doi: 10.1097/MPG.0000000000003173. J Pediatr Gastroenterol Nutr. 2021. PMID: 34016873 Free PMC article.

194

Lung function in children with cystic fibrosis in the USA and UK: a comparative longitudinal analysis of national registry data.

Schlüter DK, Ostrenga JS, Carr SB, Fink AK, Faro A, Szczesniak RD, Keogh RH, Charman SC, Marshall BC, Goss CH, Taylor-Robinson D. Schlüter DK, et al. Thorax. 2022 Feb;77(2):136-142. doi: 10.1136/thoraxjnl-2021-216849. Epub 2021 May 11. Thorax. 2022. PMID: 33975926 Free PMC article.

195

Reconstructing a Wild-Type Pseudomonas aeruginosa Reference Strain PAO1.

Lee S, Gallagher L, Manoil C. Lee S, et al. J Bacteriol. 2021 Jun 22;203(14):e0017921. doi: 10.1128/JB.00179-21. Epub 2021 Jun 22. J Bacteriol. 2021. PMID: 33972355 Free PMC article.

196

Characteristics and Outcomes of Children With Cystic Fibrosis Hospitalized With Cirrhosis in the United States.

Thornton C, Somayaji R, Parkins M, Swain MG, Ramos KJ, Goss CH, Shaheen AA. Thornton C, et al. Am J Gastroenterol. 2021 Aug 1;116(8):1734-1737. doi: 10.14309/ajg.0000000000001275. Am J Gastroenterol. 2021. PMID: 33927124 Free PMC article.

197

Assessing Practices, Beliefs, and Attitudes about Palliative Care among People with Cystic Fibrosis, Their Caregivers, and Clinicians: Results of a Content Analysis.

Basile M, Jojan L, Hobler MR, Dellon EP, Georgiopoulos AM, Goggin JL, Chen E, Goss CH, Hempstead SE, Faro A, Kavalieratos D. Basile M, et al. J Palliat Med. 2021 Nov;24(11):1650-1656. doi: 10.1089/jpm.2020.0725. Epub 2021 Apr 20. J Palliat Med. 2021. PMID: 33885355 Free PMC article.

198

Survival and Lung Transplant Outcomes for Individuals With Advanced Cystic Fibrosis Lung Disease Living in the United States and Canada: An Analysis of National Registries.

Ramos KJ, Sykes J, Stanojevic S, Ma X, Ostrenga JS, Fink A, Quon BS, Marshall BC, Faro A, Petren K, Elbert A, Goss CH, Stephenson AL. Ramos KJ, et al. Chest. 2021 Sep;160(3):843-853. doi: 10.1016/j.chest.2021.04.010. Epub 2021 Apr 17. Chest. 2021. PMID: 33878343 Free PMC article.

199

Use of proton pump inhibitors is associated with lower hemoglobin levels in people with cystic fibrosis.

Gifford AH, Sanville JL, Sathe M, Heltshe SL, Goss CH. Gifford AH, et al. Pediatr Pulmonol. 2021 Jul;56(7):2048-2056. doi: 10.1002/ppul.25431. Epub 2021 Apr 26. Pediatr Pulmonol. 2021. PMID: 33860641 Free PMC article.

200

Acute hyperglycaemia in cystic fibrosis pulmonary exacerbations.

Merjaneh L, Toprak D, McNamara S, Nay L, Sullivan E, Rosenfeld M. Merjaneh L, et al. Endocrinol Diabetes Metab. 2020 Nov 30;4(2):e00208. doi: 10.1002/edm2.208. eCollection 2021 Apr. Endocrinol Diabetes Metab. 2020. PMID: 33855211 Free PMC article.

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