 Cystic Fibrosis
"Cystic Fibrosis" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
| Descriptor ID |
D003550
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| MeSH Number(s) |
C06.689.202 C08.381.187 C16.320.190 C16.614.213
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| Concept/Terms |
Pancreatic Cystic Fibrosis- Pancreatic Cystic Fibrosis
- Cystic Fibrosis, Pancreatic
- Fibrocystic Disease of Pancreas
- Pancreas Fibrocystic Disease
- Pancreas Fibrocystic Diseases
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Below are MeSH descriptors whose meaning is more general than "Cystic Fibrosis".
Below are MeSH descriptors whose meaning is more specific than "Cystic Fibrosis".
This graph shows the total number of publications written about "Cystic Fibrosis" by people in UAMS Profiles by year, and whether "Cystic Fibrosis" was a major or minor topic of these publications.
To see the data from this visualization as text, click here.
| Year | Major Topic | Minor Topic | Total |
|---|
| 2024 | 3 | 0 | 3 | | 2023 | 4 | 0 | 4 | | 2022 | 8 | 0 | 8 | | 2021 | 9 | 1 | 10 | | 2020 | 5 | 0 | 5 | | 2019 | 4 | 0 | 4 | | 2018 | 6 | 1 | 7 | | 2017 | 3 | 0 | 3 | | 2016 | 1 | 1 | 2 | | 2015 | 4 | 0 | 4 | | 2014 | 5 | 0 | 5 | | 2013 | 8 | 1 | 9 | | 2012 | 3 | 2 | 5 | | 2011 | 4 | 0 | 4 | | 2010 | 5 | 0 | 5 | | 2009 | 3 | 0 | 3 | | 2008 | 1 | 1 | 2 | | 2007 | 1 | 1 | 2 | | 2006 | 2 | 0 | 2 | | 2005 | 1 | 0 | 1 | | 2004 | 1 | 0 | 1 | | 2003 | 2 | 1 | 3 | | 2002 | 1 | 0 | 1 | | 1999 | 1 | 0 | 1 | | 1991 | 0 | 1 | 1 | | 1989 | 2 | 0 | 2 |
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Below are the most recent publications written about "Cystic Fibrosis" by people in Profiles over the past ten years.
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Baker E, Harris WT, Guimbellot JS, Bliton K, Rowe SM, Raju SV, Oates GR. Association between biomarkers of tobacco smoke exposure and clinical efficacy of ivacaftor in the G551D observational trial (GOAL). J Cyst Fibros. 2024 Sep; 23(5):959-966.
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Liu Z, Anderson JD, Rose NR, Baker EH, Dowell AE, Ryan KJ, Acosta EP, Guimbellot JS. Differential distribution of ivacaftor and its metabolites in plasma and human airway epithelia. Pulm Pharmacol Ther. 2024 Sep; 86:102314.
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Rose NR, Chalamalla AR, Garcia BA, Krick S, Bergeron J, Sadeghi H, Schellhase DE, Ryan KJ, Dowell AE, Acosta EP, Guimbellot JS. Pharmacokinetic variability of CFTR modulators from standard and alternative regimens. Pulm Pharmacol Ther. 2024 Sep; 86:102301.
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Li C, Liu Z, Anderson J, Liu Z, Tang L, Li Y, Peng N, Chen J, Liu X, Fu L, Townes TM, Rowe SM, Bedwell DM, Guimbellot J, Zhao R. Prime editing-mediated correction of the CFTR W1282X mutation in iPSCs and derived airway epithelial cells. PLoS One. 2023; 18(11):e0295009.
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Young D, Bartlett LE, Guimbellot J, Milinic T, Burdis N, Gill ER, Lease ED, Goss CH, Kapnadak SG, Ramos KJ. Patient perspectives on elexacaftor/tezacaftor/ivacaftor after lung transplant. J Cyst Fibros. 2024 May; 23(3):545-548.
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Gushue C, Eisner M, Bai S, Johnson T, Holtzlander M, McCoy K, Sheikh S. Impact of Elexacaftor-Tezacaftor-Ivacaftor on lung disease in cystic fibrosis. Pediatr Pulmonol. 2023 Aug; 58(8):2308-2316.
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Oates GR, Mims C, Geurs R, Bergquist R, Hager A, Guimbellot JS, Hartzes AM, Gutierrez HH. Mobile health platform for self-management of pediatric cystic fibrosis: Impact on patient-centered care outcomes. J Cyst Fibros. 2023 09; 22(5):823-829.
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Zhang S, Shrestha CL, Robledo-Avila F, Jaganathan D, Wisniewski BL, Brown N, Pham H, Carey K, Amer AO, Hall-Stoodley L, McCoy KS, Bai S, Partida-Sanchez S, Kopp BT. Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor. Eur Respir J. 2023 04; 61(4).
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Anderson JD, Davis BH, Giang G, Jones A, Lee CR, Parker K, Searcy H, Benner K, Limdi NA, Guimbellot JS. Pharmacogenetic actionability and medication prescribing in people with cystic fibrosis. Clin Transl Sci. 2023 04; 16(4):662-672.
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McCoy KS, Blind J, Johnson T, Olson P, Raterman L, Bai S, Eisner M, Sheikh SI, Druhan S, Young C, Pasley K. Clinical change 2 years from start of elexacaftor-tezacaftor-ivacaftor in severe cystic fibrosis. Pediatr Pulmonol. 2023 04; 58(4):1178-1184.
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Guimbellot JS, Nichols DP, Brewington JJ. Novel Applications of Biomarkers and Personalized Medicine in Cystic Fibrosis. Clin Chest Med. 2022 12; 43(4):617-630.
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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. 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. Lancet Respir Med. 2023 04; 11(4):329-340.
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Ryan KJ, Guimbellot JS, Dowell AE, Reed-Walker KD, Kerstner-Wood CD, Anderson JD, Liu Z, Acosta EP. Quantitation of cystic fibrosis triple combination therapy, elexacaftor/tezacaftor/ivacaftor, in human plasma and cellular lysate. J Chromatogr B Analyt Technol Biomed Life Sci. 2022 Dec 15; 1213:123518.
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Lloyd EC, Cogen JD, Maples H, Bell SC, Saiman L. Antimicrobial Stewardship in Cystic Fibrosis. J Pediatric Infect Dis Soc. 2022 Sep 07; 11(Supplement_2):S53-S61.
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Lowers J, Dellon EP, Stephenson A, Arnold R, Althouse A, Jeong K, Dubin E, Soodalter J, Hovater C, Bakitas M, Goggin J, Hunt W, Ladores S, Curseen K, Winter G, Solomon G, Ailon J, Conrad D, Kavalieratos D. Integrating specialist palliative care to improve care and reduce suffering: cystic fibrosis (InSPIRe:CF) - study protocol for a multicentre randomised clinical trial. BMJ Open Respir Res. 2022 09; 9(1).
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Ramsey ML, Li SS, Lara LF, Gokun Y, Akshintala VS, Conwell DL, Heintz J, Kirkby SE, McCoy KS, Papachristou GI, Patel A, Singh VK, Hart PA. Cystic fibrosis transmembrane conductance regulator modulators and the exocrine pancreas: A scoping review. J Cyst Fibros. 2023 03; 22(2):193-200.
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Guimbellot JS, Ryan KJ, Anderson JD, Parker KL, Victoria Odom L, Rowe SM, Acosta EP. Plasma and cellular ivacaftor concentrations in patients with cystic fibrosis. Pediatr Pulmonol. 2022 11; 57(11):2745-2753.
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Ramos KJ, Guimbellot JS, Valapour M, Bartlett LE, Wai TH, Goss CH, Pilewski JM, Faro A, Diamond JM. Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients. J Cyst Fibros. 2022 09; 21(5):745-752.
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Butt AT, Banyard CD, Haldipurkar SS, Agnoli K, Mohsin MI, Vitovski S, Paleja A, Tang Y, Lomax R, Ye F, Green J, Thomas MS. The Burkholderia cenocepacia iron starvation s factor, OrbS, possesses an on-board iron sensor. Nucleic Acids Res. 2022 04 22; 50(7):3709-3726.
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Liu Z, Anderson JD, Natt J, Guimbellot JS. Culture and Imaging of Human Nasal Epithelial Organoids. J Vis Exp. 2021 12 17; (178).
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Ramsey ML, Gokun Y, Sobotka LA, Wellner MR, Porter K, Kirkby SE, Li SS, Papachristou GI, Krishna SG, Stanich PP, Hart PA, Conwell DL, Lara LF. Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis. Am J Gastroenterol. 2021 12 01; 116(12):2446-2454.
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Lester M, Eidson D, Blair S, Gray S, Sapp P, Zupancic FJ, Marshall BC, Berlinski A. Cystic Fibrosis Foundation Nebulizer and Compressor Accessibility Survey. Respir Care. 2021 Dec; 66(12):1840-1847.
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Ararat E, Sonawalla A, Berlinski A, Tas E. Nutritional status between 5-10 years is associated with cystic fibrosis-related diabetes in adolescence. Pediatr Pulmonol. 2021 10; 56(10):3217-3222.
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Smith M, Ryan KJ, Gutierrez H, Sanchez LHG, Anderson JN, Acosta EP, Benner KW, Guimbellot JS. Ivacaftor-elexacaftor-tezacaftor and tacrolimus combination in cystic fibrosis. J Cyst Fibros. 2022 01; 21(1):e8-e10.
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Anderson JD, Liu Z, Odom LV, Kersh L, Guimbellot JS. CFTR function and clinical response to modulators parallel nasal epithelial organoid swelling. Am J Physiol Lung Cell Mol Physiol. 2021 07 01; 321(1):L119-L129.
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Abraham EH, Guidotti G, Rapaport E, Bower D, Brown J, Griffin RJ, Donnelly A, Waitzkin ED, Qamar K, Thompson MA, Ethirajan S, Robinson K. Cystic fibrosis improves COVID-19 survival and provides clues for treatment of SARS-CoV-2. Purinergic Signal. 2021 09; 17(3):399-410.
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Guimbellot JS, Taylor-Cousar JL. Combination CFTR modulator therapy in children and adults with cystic fibrosis. Lancet Respir Med. 2021 07; 9(7):677-679.
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Mainz JG, Arnold C, Wittstock K, Hipler UC, Lehmann T, Zagoya C, Duckstein F, Ellemunter H, Hentschel J. Ivacaftor Reduces Inflammatory Mediators in Upper Airway Lining Fluid From Cystic Fibrosis Patients With a G551D Mutation: Serial Non-Invasive Home-Based Collection of Upper Airway Lining Fluid. Front Immunol. 2021; 12:642180.
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Sherman AC, Simonton-Atchley S, O'Brien CE, Campbell D, Reddy RM, Guinee B, Wagner LD, Anderson PJ. Associations Between Religious/Spiritual Coping and Depression Among Adults with Cystic Fibrosis: A 12-Month Longitudinal Study. J Relig Health. 2021 Aug; 60(4):2646-2661.
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Biesecker LG, Adam MP, Alkuraya FS, Amemiya AR, Bamshad MJ, Beck AE, Bennett JT, Bird LM, Carey JC, Chung B, Clark RD, Cox TC, Curry C, Dinulos MBP, Dobyns WB, Giampietro PF, Girisha KM, Glass IA, Graham JM, Gripp KW, Haldeman-Englert CR, Hall BD, Innes AM, Kalish JM, Keppler-Noreuil KM, Kosaki K, Kozel BA, Mirzaa GM, Mulvihill JJ, Nowaczyk MJM, Pagon RA, Retterer K, Rope AF, Sanchez-Lara PA, Seaver LH, Shieh JT, Slavotinek AM, Sobering AK, Stevens CA, Stevenson DA, Tan TY, Tan WH, Tsai AC, Weaver DD, Williams MS, Zackai E, Zarate YA. A dyadic approach to the delineation of diagnostic entities in clinical genomics. Am J Hum Genet. 2021 01 07; 108(1):8-15.
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Guimbellot JS, Baines A, Paynter A, Heltshe SL, VanDalfsen J, Jain M, Rowe SM, Sagel SD. Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation. J Cyst Fibros. 2021 03; 20(2):213-219.
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Hoppe JE, Guimbellot J, Martiniano SL, Toprak D, Davis C, Daines CL, Muhlebach MS, Esther CR, Dellon EP. Highlights from the 2019 North American Cystic Fibrosis Conference. Pediatr Pulmonol. 2020 09; 55(9):2225-2232.
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Liu Z, Anderson JD, Deng L, Mackay S, Bailey J, Kersh L, Rowe SM, Guimbellot JS. Human Nasal Epithelial Organoids for Therapeutic Development in Cystic Fibrosis. Genes (Basel). 2020 05 29; 11(6).
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Secunda KE, Guimbellot JS, Jovanovic B, Heltshe SL, Sagel SD, Rowe SM, Jain M. Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males. Am J Respir Crit Care Med. 2020 04 15; 201(8):996-998.
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Guimbellot JS, Ryan KJ, Anderson JD, Liu Z, Kersh L, Esther CR, Rowe SM, Acosta EP. Variable cellular ivacaftor concentrations in people with cystic fibrosis on modulator therapy. J Cyst Fibros. 2020 09; 19(5):742-745.
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McKinzie CJ, Chen L, Ehlert K, Grisso AG, Linafelter A, Lubsch L, O'Brien CE, Pan AC, Wright BA, Elson EC. Off-label use of intravenous antimicrobials for inhalation in patients with cystic fibrosis. Pediatr Pulmonol. 2019 11; 54 Suppl 3:S27-S45.
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Zhang X, Pan A, Jia S, Ideozu JE, Woods K, Murkowski K, Hessner MJ, Simpson PM, Levy H. Cystic Fibrosis Plasma Blunts the Immune Response to Bacterial Infection. Am J Respir Cell Mol Biol. 2019 09; 61(3):301-311.
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Sherman AC, Simonton-Atchley S, O'Brien CE, Campbell D, Reddy RM, Guinee B, Wagner LD, Anderson PJ. Longitudinal associations between gratitude and depression 1?year later among adult cystic fibrosis patients. J Behav Med. 2020 08; 43(4):596-604.
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Sherman AC, Simonton-Atchley S, Campbell D, Reddy RM, O'Brien CE, Guinee B, Wagner LD, Anderson PJ. Persistent Adherence to Airway Clearance Therapy in Adults With Cystic Fibrosis. Respir Care. 2019 Jul; 64(7):778-785.
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Levy H, Jia S, Pan A, Zhang X, Kaldunski M, Nugent ML, Reske M, Feliciano RA, Quintero D, Renda MM, Woods KJ, Murkowski K, Johnson K, Verbsky J, Dasu T, Ideozu JE, McColley S, Quasney MW, Dahmer MK, Avner E, Farrell PM, Cannon CL, Jacob H, Simpson PM, Hessner MJ. Identification of molecular signatures of cystic fibrosis disease status with plasma-based functional genomics. Physiol Genomics. 2019 01 01; 51(1):27-41.
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Eikani MS, Nugent M, Poursina A, Simpson P, Levy H. Clinical course and significance of nontuberculous mycobacteria and its subtypes in cystic fibrosis. BMC Infect Dis. 2018 07 06; 18(1):311.
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Poore TS, Virella-Lowell I, Guimbellot JS. Potential pathogenicity of Inquilinus limosus in a pediatric patient with cystic fibrosis. Pediatr Pulmonol. 2018 07; 53(7):E21-E23.
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Guimbellot J, Solomon GM, Baines A, Heltshe SL, VanDalfsen J, Joseloff E, Sagel SD, Rowe SM. Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations. J Cyst Fibros. 2019 01; 18(1):102-109.
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Guimbellot JS, Acosta EP, Rowe SM. Sensitivity of ivacaftor to drug-drug interactions with rifampin, a cytochrome P450 3A4 inducer. Pediatr Pulmonol. 2018 05; 53(5):E6-E8.
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Awad SM, Berlinski A. Crossover Evaluation of Compressors and Nebulizers Typically Used by Cystic Fibrosis Patients. Respir Care. 2018 Mar; 63(3):294-300.
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O'Brien CE, Com G, Fowlkes J, Tang X, James LP. Peripheral quantitative computed tomography detects differences at the radius in prepubertal children with cystic fibrosis compared to healthy controls. PLoS One. 2018; 13(1):e0191013.
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Guimbellot JS, Leach JM, Chaudhry IG, Quinney NL, Boyles SE, Chua M, Aban I, Jaspers I, Gentzsch M. Nasospheroids permit measurements of CFTR-dependent fluid transport. JCI Insight. 2017 11 16; 2(22).
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Butt AT, Thomas MS. Iron Acquisition Mechanisms and Their Role in the Virulence of Burkholderia Species. Front Cell Infect Microbiol. 2017; 7:460.
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Guimbellot J, Sharma J, Rowe SM. Toward inclusive therapy with CFTR modulators: Progress and challenges. Pediatr Pulmonol. 2017 Nov; 52(S48):S4-S14.
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Ideozu JE, Zhang X, Pan A, Ashrafi Z, Woods KJ, Hessner MJ, Simpson P, Levy H. Increased Expression of Plasma-Induced ABCC1 mRNA in Cystic Fibrosis. Int J Mol Sci. 2017 Aug 11; 18(8).
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Rowan NR, Wang EW, Kanaan A, Sahu N, Williams JV, Phillips CD, Lee SE. Respiratory viral detection in the paranasal sinuses of patients with cystic fibrosis. Am J Rhinol Allergy. 2017 Mar 01; 31(2):105-108.
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Perez-Marques F, Simpson P, Yan K, Quasney MW, Halligan N, Merchant D, Dahmer MK. Association of polymorphisms in genes of factors involved in regulation of splicing of cystic fibrosis transmembrane conductance regulator mRNA with acute respiratory distress syndrome in children with pneumonia. Crit Care. 2016 09 05; 20:281.
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Janhsen WK, Arnold C, Hentschel J, Lehmann T, Pfister W, Baier M, B?er K, H?nniger K, Kurzai O, Hipler UC, Mainz JG. Colonization of CF patients' upper airways with S. aureus contributes more decisively to upper airway inflammation than P. aeruginosa. Med Microbiol Immunol. 2016 Oct; 205(5):485-500.
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Raghavan D, Gao A, Ahn C, Kaza V, Finklea J, Torres F, Jain R. Lung transplantation and gender effects on survival of recipients with cystic fibrosis. J Heart Lung Transplant. 2016 12; 35(12):1487-1496.
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Campbell CT, McCaleb R, Manasco KB. New Inhaled Antimicrobial Formulations for Use in the Cystic Fibrosis Patient Population. Ann Pharmacother. 2016 Feb; 50(2):133-40.
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Harik NS, Com G, Tang X, Melguizo Castro M, Stemper ME, Carroll JL. Clinical characteristics and epidemiology of methicillin-resistant Staphylococcus aureus (MRSA) in children with cystic fibrosis from a center with a high MRSA prevalence. Am J Infect Control. 2016 Apr 01; 44(4):409-15.
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Stolarz AJ, Farris RA, Wiley CA, O'Brien CE, Price ET. Fenofibrate Attenuates Neutrophilic Inflammation in Airway Epithelia: Potential Drug Repurposing for Cystic Fibrosis. Clin Transl Sci. 2015 Dec; 8(6):696-701.
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Machogu E, Cao Y, Miller T, Simpson P, Levy H, Quintero D, Goday PS. Comparison of WHO and CDC growth charts in predicting pulmonary outcomes in cystic fibrosis. J Pediatr Gastroenterol Nutr. 2015 Mar; 60(3):378-83.
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Harness-Brumley CL, Elliott AC, Rosenbluth DB, Raghavan D, Jain R. Gender differences in outcomes of patients with cystic fibrosis. J Womens Health (Larchmt). 2014 Dec; 23(12):1012-20.
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