My Bibliography

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Zemke AC, Hilliam Y, Stapleton AL, Kimple AJ, Goralski JL, Shaffer AD, Pilewski JM, Senior BA, Lee SE, Cooper VS. Elexacaftor-tezacaftor-ivacaftor decreases pseudomonas abundance in the sinonasal microbiome in cystic fibrosis. Int Forum Allergy Rhinol. 2024 May;14(5):928-938. doi: 10.1002/alr.23288. Epub 2023 Oct 14. PubMed PMID: 37837613; PubMed Central PMCID: PMC11131353.

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Peng G, Taylor-Cousar JL, Lee M, Keller A, West NE, Kazmerski TM, Goralski JL, Aitken ML, Roe AH, Hadjiliadis D, Uluer A, Flume PA, Mody S, Bray LA, Jain R. Association between unplanned pregnancies and maternal exacerbations in cystic fibrosis. J Cyst Fibros. 2023 Sep;22(5):796-803. doi: 10.1016/j.jcf.2023.03.020. Epub 2023 Apr 5. PubMed PMID: 37029015.

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Chung SH, Huynh KM, Goralski JL, Chen Y, Yap PT, Ceppe AS, Powell MZ, Donaldson SH, Lee YZ. Feasibility of free-breathing (19) F MRI image acquisition to characterize ventilation defects in CF and healthy volunteers at wash-in. Magn Reson Med. 2023 Jul;90(1):79-89. doi: 10.1002/mrm.29630. Epub 2023 Mar 13. PubMed PMID: 36912481; PubMed Central PMCID: PMC10149612.

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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. 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. Am J Respir Crit Care Med. 2023 Jul 1;208(1):59-67. doi: 10.1164/rccm.202301-0084OC. PubMed PMID: 36921081; PubMed Central PMCID: PMC10870849.

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Fajac I, Daines C, Durieu I, Goralski JL, Heijerman H, Knoop C, Majoor C, Bruinsma BG, Moskowitz S, Prieto-Centurion V, Van Brunt K, Zhang Y, Quittner A. Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor. J Cyst Fibros. 2023 Jan;22(1):119-123. doi: 10.1016/j.jcf.2022.08.018. Epub 2022 Sep 14. PubMed PMID: 36114142.

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Goralski JL, Chung SH, Ceppe AS, Powell MZ, Sakthivel M, Handly BD, Lee YZ, Donaldson SH. Dynamic Perfluorinated Gas MRI Shows Improved Lung Ventilation in People with Cystic Fibrosis after Elexacaftor/Tezacaftor/Ivacaftor: An Observational Study. J Clin Med. 2022 Oct 19;11(20). doi: 10.3390/jcm11206160. PubMed PMID: 36294480; PubMed Central PMCID: PMC9604637.

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Stapleton AL, Kimple AJ, Goralski JL, Nouraie SM, Branstetter BF, Shaffer AD, Pilewski JM, Senior BA, Lee SE, Zemke AC. Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis. J Cyst Fibros. 2022 Sep;21(5):792-799. doi: 10.1016/j.jcf.2022.03.002. Epub 2022 Mar 14. PubMed PMID: 35300931; PubMed Central PMCID: PMC9470769.

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Raraigh KS, Paul KC, Goralski JL, Worthington EN, Faino AV, Sciortino S, Wang Y, Aksit MA, Ling H, Osorio DL, Onchiri FM, Patel SU, Merlo CA, Montemayor K, Gibson RL, West NE, Thakerar A, Bridges RJ, Sheppard DN, Sharma N, Cutting GR. CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor. JCI Insight. 2022 Mar 22;7(6). doi: 10.1172/jci.insight.148841. PubMed PMID: 35315358; PubMed Central PMCID: PMC8986068.

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Bacon DR, Stapleton A, Goralski JL, Ebert CS Jr, Thorp BD, Nouraie M, Shaffer AD, Senior BA, Lee SE, Zemke AC, Kimple AJ. Olfaction before and after initiation of elexacaftor-tezacaftor-ivacaftor in a cystic fibrosis cohort. Int Forum Allergy Rhinol. 2022 Feb;12(2):223-226. doi: 10.1002/alr.22891. Epub 2021 Oct 28. PubMed PMID: 34709729; PubMed Central PMCID: PMC8792160.

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Thambuluru SR, Kyazimzade S, Despotes KA, Kirk D, Goralski JL. Acute ST-elevation myocardial infarction in two young women with cystic fibrosis and cystic fibrosis-related diabetes. J Cyst Fibros. 2022 Jan;21(1):e44-e47. doi: 10.1016/j.jcf.2021.11.013. Epub 2021 Dec 12. PubMed PMID: 34911664.

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Goralski JL. Foretelling Early Lung Disease Progression in Cystic Fibrosis: The Combined Benefits of Magnetic Resonance Imaging and Newborn Screening. Am J Respir Crit Care Med. 2021 Oct 15;204(8):880-881. doi: 10.1164/rccm.202107-1727ED. PubMed PMID: 34384036; PubMed Central PMCID: PMC8534612.

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Todd SH, Sonntag EA, Buchanan ML, Jones BW, Dellon EP, Donaldson SH, Goralski JL. Development of an Advance Care Planning Protocol in a Cystic Fibrosis Outpatient Clinic. J Palliat Med. 2021 Sep;24(9):1383-1386. doi: 10.1089/jpm.2021.0186. Epub 2021 Jun 30. PubMed PMID: 34191614.

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McCallister A, Chung SH, Antonacci M, Z Powell M, Ceppe AS, Donaldson SH, Lee YZ, Branca RT, Goralski JL. Comparison of single breath hyperpolarized (129) Xe MRI with dynamic (19) F MRI in cystic fibrosis lung disease. Magn Reson Med. 2021 Feb;85(2):1028-1038. doi: 10.1002/mrm.28457. Epub 2020 Aug 8. PubMed PMID: 32770779; PubMed Central PMCID: PMC7689687.

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Goralski JL, Stewart NJ, Woods JC. Novel imaging techniques for cystic fibrosis lung disease. Pediatr Pulmonol. 2021 Feb;56 Suppl 1(Suppl 1):S40-S54. doi: 10.1002/ppul.24931. Review. PubMed PMID: 32592531; PubMed Central PMCID: PMC7808406.

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Haywood KB, Goralski JL. The specialist as primary care provider in CF. J Cyst Fibros. 2020 Sep;19(5):844-845. doi: 10.1016/j.jcf.2020.03.018. Epub 2020 Apr 22. PubMed PMID: 32335024.

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Goralski JL, Chung SH, Glass TM, Ceppe AS, Akinnagbe-Zusterzeel EO, Trimble AT, Boucher RC, Soher BJ, Charles HC, Donaldson SH, Lee YZ. Dynamic perfluorinated gas MRI reveals abnormal ventilation despite normal FEV1 in cystic fibrosis. JCI Insight. 2020 Jan 30;5(2). doi: 10.1172/jci.insight.133400. PubMed PMID: 31855577; PubMed Central PMCID: PMC7098716.

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Megalaa R, Gopalareddy V, Champion E, Goralski JL. Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use. Pediatr Pulmonol. 2019 Aug;54(8):E16-E18. doi: 10.1002/ppul.24353. Epub 2019 May 7. PubMed PMID: 31066218.

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Gunnell ET, Franceschi DK, Inscoe CR, Hartman A, Goralski JL, Ceppe A, Handly B, Sams C, Fordham LA, Lu J, Zhou O, Lee YZ. Initial clinical evaluation of stationary digital chest tomosynthesis in adult patients with cystic fibrosis. Eur Radiol. 2019 Apr;29(4):1665-1673. doi: 10.1007/s00330-018-5703-9. Epub 2018 Sep 25. PubMed PMID: 30255248; PubMed Central PMCID: PMC6896210.

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Bernstein AT, Leigh MW, Goralski JL, Esther CR Jr, McKinzie CJ. Use of telavancin in adolescent patients with cystic fibrosis and prior intolerance to vancomycin: A case series. J Cyst Fibros. 2018 Nov;17(6):e48-e50. doi: 10.1016/j.jcf.2018.08.003. Epub 2018 Aug 28. PubMed PMID: 30170755.

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Goralski JL, Wu D, Thelin WR, Boucher RC, Button B. The in vitro effect of nebulised hypertonic saline on human bronchial epithelium. Eur Respir J. 2018 May;51(5). doi: 10.1183/13993003.02652-2017. Print 2018 May. PubMed PMID: 29599187; PubMed Central PMCID: PMC6000726.

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Carrion A, Borowitz DS, Freedman SD, Siracusa CM, Goralski JL, Hadjiliadis D, Srinivasan S, Stokes DC. Reduction of Recurrence Risk of Pancreatitis in Cystic Fibrosis With Ivacaftor: Case Series. J Pediatr Gastroenterol Nutr. 2018 Mar;66(3):451-454. doi: 10.1097/MPG.0000000000001788. PubMed PMID: 29045347.

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Goralski JL, Glass T, Ceppe A, Trimble A, Boucher RC, Donaldson SH, Lee YZ. Visualizing Ventilation: Application of 19F MRI to CF Lung Disease. Pediatric pulmonology. Supplement. 2018; 53:207.

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Goralski JL, Nasr SZ, Uluer A. Overcoming barriers to a successful transition from pediatric to adult care. Pediatr Pulmonol. 2017 Nov;52(S48):S52-S60. doi: 10.1002/ppul.23778. Epub 2017 Sep 26. Review. PubMed PMID: 28950427.

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Corcoran TE, Godovchik JE, Donn KH, Busick DR, Goralski J, Locke LW, Markovetz MR, Myerburg MM, Muthukrishnan A, Weber L, Lacy RT, Pilewski JM. Overnight delivery of hypertonic saline by nasal cannula aerosol for cystic fibrosis. Pediatr Pulmonol. 2017 Sep;52(9):1142-1149. doi: 10.1002/ppul.23749. Epub 2017 Jul 24. PubMed PMID: 28737262; PubMed Central PMCID: PMC5561478.

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McKinzie CJ, Goralski JL, Noah TL, Retsch-Bogart GZ, Prieur MB. Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis. J Cyst Fibros. 2017 Jul;16(4):525-527. doi: 10.1016/j.jcf.2017.05.008. Epub 2017 Jun 8. PubMed PMID: 28602538.

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Goralski JL, Davis SD. Improving complex medical care while awaiting next-generation CFTR potentiators and correctors: The current pipeline of therapeutics. Pediatr Pulmonol. 2015 Oct;50 Suppl 40:S66-73. doi: 10.1002/ppul.23253. Review. PubMed PMID: 26335956.

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Hawkins A, Guttentag SH, Deterding R, Funkhouser WK, Goralski JL, Chatterjee S, Mulugeta S, Beers MF. A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy. Am J Physiol Lung Cell Mol Physiol. 2015 Jan 1;308(1):L33-47. doi: 10.1152/ajplung.00217.2014. Epub 2014 Oct 24. PubMed PMID: 25344067; PubMed Central PMCID: PMC4281696.

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Goralski JL, Davis SD. Breathing easier: addressing the challenges of aerosolizing medications to infants and preschoolers. Respir Med. 2014 Aug;108(8):1069-74. doi: 10.1016/j.rmed.2014.06.004. Epub 2014 Jun 25. Review. PubMed PMID: 25012949.

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Goralski JL, Davis SD. Challenges and limitations of testing efficacy of aerosol device delivery in young children. J Aerosol Med Pulm Drug Deliv. 2014 Aug;27(4):264-71. doi: 10.1089/jamp.2013.1097. Epub 2014 Jan 29. Review. PubMed PMID: 24476048.

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Goralski JL, Donaldson SH. Hypertonic saline for cystic fibrosis: worth its salt?. Expert Rev Respir Med. 2014 Jun;8(3):267-9. doi: 10.1586/17476348.2014.896203. Epub 2014 Mar 26. PubMed PMID: 24666113.

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Goralski JL, Lercher DM, Davis SD, Dellon ES. Eosinophilic esophagitis in cystic fibrosis: a case series and review of the literature. J Cyst Fibros. 2013 Jan;12(1):9-14. doi: 10.1016/j.jcf.2012.09.002. Epub 2012 Oct 22. Review. PubMed PMID: 23085252; PubMed Central PMCID: PMC3519943.

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Goralski JL, Boucher RC, Button B. Osmolytes and ion transport modulators: new strategies for airway surface rehydration. Curr Opin Pharmacol. 2010 Jun;10(3):294-9. doi: 10.1016/j.coph.2010.04.003. Epub 2010 May 1. Review. PubMed PMID: 20439165; PubMed Central PMCID: PMC8855330.

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Goralski JL, Bromberg PA, Haithcock B. Intrapleural hemorrhage after administration of tPA: a case report and review of the literature. Ther Adv Respir Dis. 2009 Dec;3(6):295-300. doi: 10.1177/1753465809350748. Review. PubMed PMID: 19934281.

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Waller AH, Goralski JL, Brelvi ZS. Agenesis of the gallbladder. Clin Gastroenterol Hepatol. 2008 May;6(5):A28. doi: 10.1016/j.cgh.2007.12.050. Epub 2008 Mar 5. PubMed PMID: 18325844.

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Goralski JL, Button B. CF Cells do not Hyperabsorb Sodium in the Setting of Nebulized Hypertonic Saline. The North American Cystic Fibrosis Conference; 2012 October 11; Orlando, FL, USA. Pediatric Pulmonology Supp, 2012, abstract #105; c00.

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Goralski JL, Birchard KR, Muhlebach MS. Evaluation of CF Lung Disease by MR: A Radiation Free Alternative. North American Cystic Fibrosis Conference; 2014 October 9; Atlanta, GA, USA. Pediatric Pulmonology Supp 38. 2014, abstract #228; c00.

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Goralski JL, Neuringer IP. Women’s Health Concerns Following Lung Transplant. North American Cystic Fibrosis Conference; 2011; Anaheim, CA, USA. Ped Pulm Supp 34, 2011. Abstract #412; c00.

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Goralski JL, Button B. An In Vitro Study of the Kinetics of Hypertonic Saline on ASL Height. North American Cystic Fibrosis Conference; 2010 October 21; Baltimore, MD, USA. Pediatric Pulmonology Supp 33, 2010. Abstract#205; c00.

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Goralski JL, Button B. Nebulized Hypertonic Saline Yields Disparate ASL Heights with Sequential Doses. North American Cystic Fibrosis Conference; 2013 October 11; Salt Lake City, UT, USA. Pediatric Pulmonology Supp 36. 2013, abstract #107; c00.

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Goralski JL, Button B. . Mucus Concentration Affects HBE Response to Nebulized Hypetonic Saline. North American Cystic Fibrosis Conference; 2014 October 9; Atlanta, GA, USA. Pediatric Pulmonology Supp 38, 2014, abstract #182; c00.

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Goralski JL, Button B. Nebulized Hypertonic Saline Causes Reversible Ciliostasis in Airway Epithelial Cells. North American Cystic Fibrosis Conference; 2010 October 21; Baltimore, MD, USA. . Pediatric Pulmonology Supp 33, 2010. Abstract#102; c00.