2022
Donaldson SH, Laube BL, Mogayzel P, Corcoran TE, Pilewski JM, Ceppe A, Wu J, Bhambhvani PG, Ratjen F, Sagel SD, Clancy JP, Rowe SM, Bennett WD. Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study J Cyst Fibros. 2022 Jan;21(1):143-145. PMID: 34083123; PMCID: PMC8630086.
Hebestreit H, Kriemler S, Schindler C, Stein L, Karila C, Urquhart DS, Orenstein DM, Lands LC, Schaeff J, Eber E, Radtke T; ACTIVATE-CF Study Working Group. Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF) Am J Respir Crit Care Med. 2022 Feb 1;205(3):330-339.: 34735776; PMCID: PMC8887001.
2021
Armbruster CR, Marshall CW, Garber AI, Melvin JA, Zemke AC, Moore J, Zamora PF, Li K, Fritz IL, Manko CD, Weaver ML, Gaston JR, Morris A, Methé B, DePas WH, Lee SE, Cooper VS, Bomberger JM. Adaptation and genomic erosion in fragmented Pseudomonas aeruginosa populations in the sinuses of people with cystic fibrosis Cell Rep. 2021 Oct 19;37(3):109829. PMID: 34686349; PMCID: PMC8667756.
Corbera-Hincapie MA, Kurland KS, Hincapie MR, Fabio A, Weiner DJ, Kim SC, Kazmerski TM. Geospatial Analysis of Food Deserts and Their Impact on Health Outcomes in Children with Cystic Fibrosis Nutrients. 2021 Nov 10;13(11):3996. PMID: 34836250; PMCID: PMC8621515.
Kumar P, Soni DK, Sen C, Larsen MB, Mazan-Mamczarz K, Piao Y, De S, Gorospe M, Frizzell RA, Biswas R. SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells Sci Rep. 2021 Aug 17;11(1):16645. PMID: 34404863; PMCID: PMC8371023.
Larsen MB, Choi JJ, Wang X, Myerburg MM, Frizzell RA, Bertrand CA. Separating the contributions of SLC26A9 and CFTR to anion secretion in primary human bronchial epithelia Am J Physiol Lung Cell Mol Physiol. 2021 Dec 1;321(6):L1147-L1160. PMID: 34668421; PMCID: PMC8715023.
Shah P, Lowery E, Chaparro C, Visner G, Hempstead SE, Abraham J, Bhakta Z, Carroll M, Christon L, Danziger-Isakov L, Diamond JM, Lease E, Leonard J, Litvin M, Poole R, Vlahos F, Werchan C, Murray MA, Tallarico E, Faro A, Pilewski JM, Hachem RR. Cystic fibrosis foundation consensus statements for the care of cystic fibrosis lung transplant recipients J Heart Lung Transplant. 2021 Jul;40(7):539-556. PMID: 34103223.
Weinstock MS, Shaffer AD, Stapleton AL. Predictors of sinonasal disease onset, progression, and severity in pediatric cystic fibrosis patients Am J Otolaryngol. 2021 Sep-Oct;42(5):103016. PMID: 33836483; PMCID: PMC8403133.
2020
Armbruster CR, Coenye T, Touqui L, Bomberger JM. Interplay between host-microbe and microbe-microbe interactions in cystic fibrosis J Cyst Fibros. 2020 Mar;19 Suppl 1(Suppl 1):S47-S53. PMID: 31685398; PMCID: PMC7035965.
Kapnadak SG, Dimango E, Hadjiliadis D, Hempstead SE, Tallarico E, Pilewski JM, Faro A, Albright J, Benden C, Blair S, Dellon EP, Gochenour D, Michelson P, Moshiree B, Neuringer I, Riedy C, Schindler T, Singer LG, Young D, Vignola L, Zukosky J, Simon RH. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease J Cyst Fibros. 2020 May;19(3):344-354. PMID: 32115388; PMCID: .
Kidd KM, Sequeira GM, Voss RV, Weiner DJ, Ramsey BW, Jain R, Kazmerski TM. Caring for gender diverse youth with cystic fibrosis J Cyst Fibros. 2020 Nov;19(6):1018-1020. PMID: 32268993; PMCID: PMC8746999.
Okoniewski W, Hughan KS, Weiner GA, Weiner DJ, Forno E. Glycemic control and FEV(1) recovery during pulmonary exacerbations in pediatric cystic fibrosis-related diabetes J Cyst Fibros. 2020 May;19(3):460-465. PMID: 31980357; PMCID: PMC7329580.
Ramos KJ, Kapnadak SG, Bradford MC, Somayaji R, Morrell ED, Pilewski JM, Lease ED, Mulligan MS, Aitken ML, Gries CJ, Goss CH. Underweight Patients With Cystic Fibrosis Have Acceptable Survival Following Lung Transplantation: A United Network for Organ Sharing Registry Study Chest. 2020 Apr;157(4):898-906. PMID: 31958441; PMCID: PMC7118242.
Ramos KJ, Pilewski JM, Faro A, Marshall BC. Improved Prognosis in Cystic Fibrosis: Consideration for Intensive Care during the COVID-19 Pandemic Am J Respir Crit Care Med. 2020 Jun 1;201(11):1434-1435. PMID: 32289235; PMCID: PMC7258637.
Smith PJ, Dunitz JM, Lucy A, Hempstead SE, Tallarico E, Faro A, Pilewski JM, Ramos KJ. Incorporating patient and caregiver feedback into lung transplant referral guidelines for individuals with cystic fibrosis-Preliminary findings from a novel paradigm Clin Transplant. 2020 Oct;34(10):e14038. PMID: 32654238; PMCID: PMC7868110.
Trandel ET, Pilewski JM, Dellon EP, Jeong K, Yabes JG, Moreines LT, Arnold RM, Hoydich ZP, Kavalieratos D. Prevalence of unmet palliative care needs in adults with cystic fibrosis J Cyst Fibros. 2020 May;19(3):394-401. PMID: 31862306; PMCID: PMC8662333.
2019
Corcoran TE, Huber AS, Myerburg MM, Weiner DJ, Locke LW, Lacy RT, Weber L, Czachowski MR, Johnston DJ, Muthukrishnan A, Lennox AT, Pilewski JM. Multiprobe Nuclear Imaging of the Cystic Fibrosis Lung as a Biomarker of Therapeutic Effect J Aerosol Med Pulm Drug Deliv. 2019 Aug;32(4):242-249. PMID: 30969149; PMCID: PMC6685188.
Harano T, Haidar G, Schaheen L, Morrell MR, Pilewski JM, D'Cunha J. Successful lung transplantation from a hepatitis C RNA-positive donor to a hepatitis C treatment-experienced recipient with cystic fibrosis J Thorac Cardiovasc Surg. 2019 Oct;158(4):e155-e157. PMID: 31229293.
Kumar P, Sen C, Peters K, Frizzell RA, Biswas R. Comparative analyses of long non-coding RNA profiles in vivo in cystic fibrosis lung airway and parenchyma tissues Respir Res. 2019 Dec 16;20(1):284. PMID: 31842871; PMCID: PMC6916039.
Morrell MR, Kiel SC, Pilewski JM. Organ Transplantation for Cystic Fibrosis Semin Respir Crit Care Med. 2019 Dec;40(6):842-856. PMID: 31887769.
Ramos KJ, Smith PJ, McKone EF, Pilewski JM, Lucy A, Hempstead SE, Tallarico E, Faro A, Rosenbluth DB, Gray AL, Dunitz JM; CF Lung Transplant Referral Guidelines Committee. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines J Cyst Fibros. 2019 May;18(3):321-333. PMID: 30926322; PMCID: PMC6545264.
Serrano Castillo F, Bertrand CA, Myerburg MM, Shapiro ME, Corcoran TE, Parker RS. A physiologically-motivated model of cystic fibrosis liquid and solute transport dynamics across primary human nasal epithelia J Pharmacokinet Pharmacodyn. 2019 Oct;46(5):457-472. PMID: 31494805.
Sun T, Sun Z, Jiang Y, Ferguson AA, Pilewski JM, Kolls JK, Chen W, Chen K. Transcriptomic Responses to Ivacaftor and Prediction of Ivacaftor Clinical Responsiveness Am J Respir Cell Mol Biol. 2019 Nov;61(5):643-652. PMID: 30995102; PMCID: PMC6827069.
Trandel ET, Pilewski JM, Dellon EP, Moreines LT, Yabes JG, Jeong K, Arnold RM, Kavalieratos D. Symptom Burden and Unmet Existential Needs in Adults With Cystic Fibrosis West J Nurs Res. 2019 Oct;41(10):1448-1464. PMID: 31267835.
Wolter DJ, Onchiri FM, Emerson J, Precit MR, Lee M, McNamara S, Nay L, Blackledge M, Uluer A, Orenstein DM, Mann M, Hoover W, Gibson RL, Burns JL, Hoffman LR; SCVSA study group. Prevalence and clinical associations of Staphylococcus aureus small-colony variant respiratory infection in children with cystic fibrosis (SCVSA): a multicentre, observational study Lancet Respir Med. 2019 Dec;7(12):1027-1038. PMID: 31727592; PMCID: PMC6924508.
Zemke AC, Nouraie SM, Moore J, Gaston JR, Rowan NR, Pilewski JM, Bomberger JM, Lee SE. Clinical predictors of cystic fibrosis chronic rhinosinusitis severity Int Forum Allergy Rhinol. 2019 Jul;9(7):759-765. PMID: 31162888; PMCID: PMC6715283.
2018
Abraham O, Li JS, Monangai KE, Feathers AM, Weiner D. The pharmacist's role in supporting people living with cystic fibrosis J Am Pharm Assoc (2003). 2018 May-Jun;58(3):246-249. PMID: 29459095.
Al Qatarneh S, Michel HK, Lindblad D, Ozolek J, Venkat V, Weiner DJ. Pancreatic Cystosis and Intrahepatic Biliopathy in a Young Adult with Cystic Fibrosis J Pediatr. 2018 Dec;203:457-457.e1. PMID: 30041939.
Dar HH, Tyurina YY, Mikulska-Ruminska K, Shrivastava I, Ting HC, Tyurin VA, Krieger J, St Croix CM, Watkins S, Bayir E, Mao G, Armbruster CR, Kapralov A, Wang H, Parsek MR, Anthonymuthu TS, Ogunsola AF, Flitter BA, Freedman CJ, Gaston JR, Holman TR, Pilewski JM, Greenberger JS, Mallampalli RK, Doi Y, Lee JS, Bahar I, Bomberger JM, Bayır H, Kagan VE. Pseudomonas aeruginosa utilizes host polyunsaturated phosphatidylethanolamines to trigger theft-ferroptosis in bronchial epithelium J Clin Invest. 2018 Oct 1;128(10):4639-4653. PMID: 30198910; PMCID: PMC6159971.
Donaldson SH, Laube BL, Corcoran TE, Bhambhvani P, Zeman K, Ceppe A, Zeitlin PL, Mogayzel PJ Jr, Boyle M, Locke LW, Myerburg MM, Pilewski JM, Flanagan B, Rowe SM, Bennett WD. Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR JCI Insight. 2018 Dec 20;3(24):e122695. PMID: 30568035; PMCID: PMC6338313.
Donaldson SH, Pilewski JM, Griese M, Cooke J, Viswanathan L, Tullis E, Davies JC, Lekstrom-Himes JA, Wang LT; VX11-661-101 Study Group. Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR Am J Respir Crit Care Med. 2018 Jan 15;197(2):214-224. PMID: 28930490; PMCID: PMC5768901.
Hebestreit H, Lands LC, Alarie N, Schaeff J, Karila C, Orenstein DM, Urquhart DS, Hulzebos EHJ, Stein L, Schindler C, Kriemler S, Radtke T; ACTIVATE-CF Study Working Group. Effects of a partially supervised conditioning programme in cystic fibrosis: an international multi-centre randomised controlled trial (ACTIVATE-CF): study protocol BMC Pulm Med. 2018 Feb 8;18(1):31. PMID: 29422091; PMCID: PMC5806352.
Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, Ladores S, Rubenstein RC, Sagel SD, Weiner DJ, Pilewski JM, Orenstein DM, Borrero S. Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis J Cyst Fibros. 2018 Jan;17(1):64-70. PMID: 28866451.
Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, Ladores S, Rubenstein RC, Sagel SD, Weiner DJ, Pilewski JM, Orenstein DM, Borrero S. Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis J Cyst Fibros. 2018 Jan;17(1):57-63. PMID: 28774749.
Kiedrowski MR, Gaston JR, Kocak BR, Coburn SL, Lee S, Pilewski JM, Myerburg MM, Bomberger JM. Staphylococcus aureus Biofilm Growth on Cystic Fibrosis Airway Epithelial Cells Is Enhanced during Respiratory Syncytial Virus Coinfection mSphere. 2018 Aug 15;3(4):e00341-18. PMID: 30111629; PMCID: PMC6094059.
Sagel SD, Khan U, Jain R, Graff G, Daines CL, Dunitz JM, Borowitz D, Orenstein DM, Abdulhamid I, Noe J, Clancy JP, Slovis B, Rock MJ, McCoy KS, Strausbaugh S, Livingston FR, Papas KA, Shaffer ML. Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial Am J Respir Crit Care Med. 2018 Sep 1;198(5):639-647. PMID: 29688760; PMCID: PMC6118015.
Taylor-Cousar JL, Jain M, Barto TL, Haddad T, Atkinson J, Tian S, Tang R, Marigowda G, Waltz D, Pilewski J; VX14-809-106 Investigator Group. Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR J Cyst Fibros. 2018 Mar;17(2):228-235. PMID: 29126871.