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Page 1
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.
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.
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 Clinical Trial.
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
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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
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 article. Clinical Trial.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy.
Nichols DP, Donaldson SH, Frederick CA, Freedman SD, Gelfond D, Hoffman LR, Kelly A, Narkewicz MR, Pittman JE, Ratjen F, Sagel SD, Rosenfeld M, Schwarzenberg SJ, Singh PK, Solomon GM, Stalvey MS, Kirby S, VanDalfsen JM, Clancy JP, Rowe SM. Nichols DP, et al. J Cyst Fibros. 2021 Mar;20(2):205-212. doi: 10.1016/j.jcf.2021.02.003. Epub 2021 Feb 19. J Cyst Fibros. 2021. PMID: 33619012 Free PMC article. Review.
Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Clinical Trial Study Design.
Mayer-Hamblett N, Nichols DP, Odem-Davis K, Riekert KA, Sawicki GS, Donaldson SH, Ratjen F, Konstan MW, Simon N, Rosenbluth DB, Retsch-Bogart G, Clancy JP, VanDalfsen JM, Buckingham R, Gifford AH. Mayer-Hamblett N, et al. Ann Am Thorac Soc. 2021 Aug;18(8):1397-1405. doi: 10.1513/AnnalsATS.202010-1336SD. Ann Am Thorac Soc. 2021. PMID: 33465316 Free PMC article.
Designing the GALAXY study: Partnering with the cystic fibrosis community to optimize assessment of gastrointestinal symptoms.
Freeman AJ, Sathe M, Aliaj E, Borowitz D, Fogarty B, Goss CH, Freedman S, Heltshe SL, Khan U, Riva D, Roman C, Romasco M, Schwarzenberg SJ, Ufret-Vincenty CA, Moshiree B. Freeman AJ, et al. J Cyst Fibros. 2021 Jul;20(4):598-604. doi: 10.1016/j.jcf.2020.12.021. Epub 2021 Jan 13. J Cyst Fibros. 2021. PMID: 33451899 Free PMC article.
Changes in fecal microbiota with CFTR modulator therapy: A pilot study.
Pope CE, Vo AT, Hayden HS, Weiss EJ, Durfey S, McNamara S, Ratjen A, Grogan B, Carter S, Nay L, Parsek MR, Singh PK, McKone EF, Aitken ML, Rosenfeld MR, Hoffman LR. Pope CE, et al. J Cyst Fibros. 2021 Sep;20(5):742-746. doi: 10.1016/j.jcf.2020.12.002. Epub 2020 Dec 31. J Cyst Fibros. 2021. PMID: 33390317 Free PMC article.
Bridging the survival gap in cystic fibrosis: An investigation of lung transplant outcomes in Canada and the United States.
Stephenson AL, Ramos KJ, Sykes J, Ma X, Stanojevic S, Quon BS, Marshall BC, Petren K, Ostrenga JS, Fink AK, Faro A, Elbert A, Chaparro C, Goss CH. Stephenson AL, et al. J Heart Lung Transplant. 2021 Mar;40(3):201-209. doi: 10.1016/j.healun.2020.12.001. Epub 2020 Dec 7. J Heart Lung Transplant. 2021. PMID: 33386232 Free PMC article.
Functional SARS-CoV-2-Specific Immune Memory Persists after Mild COVID-19.
Rodda LB, Netland J, Shehata L, Pruner KB, Morawski PA, Thouvenel CD, Takehara KK, Eggenberger J, Hemann EA, Waterman HR, Fahning ML, Chen Y, Hale M, Rathe J, Stokes C, Wrenn S, Fiala B, Carter L, Hamerman JA, King NP, Gale M Jr, Campbell DJ, Rawlings DJ, Pepper M. Rodda LB, et al. Cell. 2021 Jan 7;184(1):169-183.e17. doi: 10.1016/j.cell.2020.11.029. Epub 2020 Nov 23. Cell. 2021. PMID: 33296701 Free PMC article.
Cystic Fibrosis Lung Function Decline after Within-Host Evolution Increases Virulence of Infecting Pseudomonas aeruginosa.
Jorth P, Durfey S, Rezayat A, Garudathri J, Ratjen A, Staudinger BJ, Radey MC, Genatossio A, McNamara S, Cook DA, Aitken ML, Gibson RL, Yahr TL, Singh PK. Jorth P, et al. Am J Respir Crit Care Med. 2021 Mar 1;203(5):637-640. doi: 10.1164/rccm.202007-2735LE. Am J Respir Crit Care Med. 2021. PMID: 33137262 Free PMC article. No abstract available.
Sex Steroids Induce Membrane Stress Responses and Virulence Properties in Pseudomonas aeruginosa.
Vidaillac C, Yong VFL, Aschtgen MS, Qu J, Yang S, Xu G, Seng ZJ, Brown AC, Ali MK, Jaggi TK, Sankaran J, Foo YH, Righetti F, Nedumaran AM, Mac Aogáin M, Roizman D, Richard JA, Rogers TR, Toyofuku M, Luo D, Loh E, Wohland T, Czarny B, Horvat JC, Hansbro PM, Yang L, Li L, Normark S, Henriques Normark B, Chotirmall SH. Vidaillac C, et al. mBio. 2020 Sep 29;11(5):e01774-20. doi: 10.1128/mBio.01774-20. mBio. 2020. PMID: 32994320 Free PMC article.
The Pseudomonas aeruginosa protease LasB directly activates IL-1β.
Sun J, LaRock DL, Skowronski EA, Kimmey JM, Olson J, Jiang Z, O'Donoghue AJ, Nizet V, LaRock CN. Sun J, et al. EBioMedicine. 2020 Oct;60:102984. doi: 10.1016/j.ebiom.2020.102984. Epub 2020 Sep 23. EBioMedicine. 2020. PMID: 32979835 Free PMC article.
Maintenance tobramycin primarily affects untargeted bacteria in the CF sputum microbiome.
Nelson MT, Wolter DJ, Eng A, Weiss EJ, Vo AT, Brittnacher MJ, Hayden HS, Ravishankar S, Bautista G, Ratjen A, Blackledge M, McNamara S, Nay L, Majors C, Miller SI, Borenstein E, Simon RH, LiPuma JJ, Hoffman LR. Nelson MT, et al. Thorax. 2020 Sep;75(9):780-790. doi: 10.1136/thoraxjnl-2019-214187. Epub 2020 Jul 6. Thorax. 2020. PMID: 32631930 Free PMC article.
Elevated exopolysaccharide levels in Pseudomonas aeruginosa flagellar mutants have implications for biofilm growth and chronic infections.
Harrison JJ, Almblad H, Irie Y, Wolter DJ, Eggleston HC, Randall TE, Kitzman JO, Stackhouse B, Emerson JC, Mcnamara S, Larsen TJ, Shendure J, Hoffman LR, Wozniak DJ, Parsek MR. Harrison JJ, et al. PLoS Genet. 2020 Jun 12;16(6):e1008848. doi: 10.1371/journal.pgen.1008848. eCollection 2020 Jun. PLoS Genet. 2020. PMID: 32530919 Free PMC article.
538 results