CF Reference List Antimicrobials

(compiled between October 2019 – January 2020) Baklouti S., Massip C., Mane C., Guet-Revillet H., Murris M., Concordet D., Gandia P. Adults & Adolescents Comment on: In vitro activity of seven beta-lactams including ceftolozane/tazobactam and ceftazidime/avibactam against Elborn JS. Burkholderia cepacia complex, Burkholderia gladioli and other non- Adult Care in Cystic Fibrosis fermentative Gram-negative bacilli isolated from cystic fibrosis Journal of Antimicrobial Chemotherapy 2019; 74: 3122 - 3123 Seminars in Respiratory and Critical Care Medicine 2019; 40: 857 - 868 Bilal H., Bergen PJ., Kim TH., Chung SE., Peleg AY., Oliver A.,

Flewelling KD., Sellers DE., Sawicki GS., Robinson WM., Dill EJ. Nation RL., Landersdorfer CB. Male gender and unemployment are associated with lower levels of Synergistic Meropenem-Tobramycin Combination Dosage Regimens perceived social support in adults with cystic fibrosis against Clinical Hypermutable Pseudomonas aeruginosa at Simulated Journal of Psychosomatic Research 2019; 127: ArNo: 109858 Epithelial Lining Fluid Concentrations in a Dynamic Biofilm Model Antimicrobial Agents and Chemotherapy 2019; 63: 11:e01293-19 Hart LC., Patel-Nguyen SV., Merkley MG., Jonas DE. An Evidence Map for Interventions Addressing Transition from Buil JB., Hare RK., Zwaan BJ., Arendrup MC., Melchers WJG., Pediatric to Adult Care: A Systematic Review of Systematic Reviews Verweij PE. Journal of Pediatric Nursing-Nursing Care of Children & Families The fading boundaries between patient and environmental routes of 2019; 48: 18 - 34 triazole resistance selection in Aspergillus fumigatus PloS Pathogens 2019; 15: 8:e1007858 Linnemann RW., Friedman D., Altstein LL., Islam S., Bach KT., Georgiopoulos AM., Moskowitz SM., Yonker LM. Caverly LJ., Spilker T., Kalikin LM., Stillwell T., Young C., Advance Care Planning Experiences and Preferences among People Huang DB., LiPuma JJ. with Cystic Fibrosis In Vitro Activities of beta-Lactam-beta-Lactamase Inhibitor Journal of Palliative Medicine 2019; 22: 138 - 144 Antimicrobial Agents against Cystic Fibrosis Respiratory Pathogens Antimicrobial Agents and Chemotherapy 2020; 64: 1:e01595-19 Marmor M., Jonas A., Mirza A., Rad E., Wong H., Aslakson RA., Aslakson R., Ast K., Carroll T., Dzeng E., Harrison KL., Kaye Day BJ., Bratcher PE., Chandler JD., Kilgore MB., Min E., EC., LeBlanc TW., Lo SS., McKenna K., Nageswaran S., Powers LiPuma JJ., Hondal RJ., Nichols DP. J., Rotella J., Ullrich C., Vickey T. The thiocyanate analog selenocyanate is a more potent antimicrobial Opportunities to Improve Utilization of Palliative Care Among Adults prodrug that also is selectively detoxified by the host Free Radical Biology and Medicine 2020; 146: 324 - 332 With Cystic Fibrosis: A Systematic Review Journal of Pain and Symptom Management 2019; 58: 1100 - + Dong K., Singh A., Ng RT., Sin DD., Tebbutt SJ., Ratjen F., Quon

Peeters MAC., Sattoe JNT., van Staa A., Versteeg SE., Heeres I., BS. Rutjes NW., Janssens HM. Proteomic Profiling to Identify Blood Biomarkers Predictive of Controlled evaluation of a transition clinic for Dutch young people Response to Azithromycin in Children and Adolescents With Cystic with cystic fibrosis Fibrosis Chest 2019; 156: 667 - 673 Pediatric Pulmonology 2019; 54: 1811 - 1820

Shteinberg M., Ben Lulu A., Downey DG., Blumenfeld Z., Epps QJ., Epps KL., Young DC., Zobell JT. Rousset-Jablonski C., Perceval M., Colombo A., Stein N., Livnat State of the art in cystic fibrosis pharmacology-Optimization of G., Gur M., Bentur L., Mussaffi H., Blau H., Sarouk I., Dagan .A., antimicrobials in the treatment of cystic fibrosis pulmonary Kerem E., Aviram M., Picard E., Aliberti S. et al exacerbations: I. Anti-methicillin-resistant Staphylococcus aureus Failure to conceive in women with CF is associated with pancreatic (MRSA) antibiotics Pediatric Pulmonology 2019; 123: 24643 - 24650 insufficiency and advancing age Journal of Cystic Fibrosis 2019; 18: 525 - 529 Fusco NM., Meaney CJ., Frederick CA., Prescott WA.

Stayer DL., Culver ED., Brosius HM., Stamm JA. Comparative Effectiveness of Vancomycin Versus Linezolid for the Case Report of Nursing Care for a Pregnant Woman With Cystic Treatment of Acute Pulmonary Exacerbations of Cystic Fibrosis Annals of Pharmacotherapy 2019; 116: 22275 - 22281 Fibrosis Journal of Obstetric Gynecologic and Neonatal Nursing 2019; 48: Han CD., Goodwine J., Romero N., Steck KS., Sauer K., 674 - 682 Doiron A. Enzyme-encapsulating polymeric nanoparticles: A potential adjunctive therapy in Pseudomonas aeruginosa biofilm-associated Animal Model infection treatment Colloids and Surfaces B-Biointerfaces 2019; 184: ArNo: 110512 Cooney AL., Thornell IM., Singh BK., Shah VS., Stoltz DA., McCray PB., Zabner J., Sinn PL. Harrison LB., Fowler RC., Abdalhamid B., Selmecki A., A Novel AAV-mediated Gene Delivery System Corrects CFTR Hanson ND. Function in Pigs lptG contributes to changes in membrane permeability and the American Journal of Respiratory Cell and Molecular Biology 2019; emergence of multidrug hypersusceptibility in a cystic fibrosis isolate 61: 747 - 754 of Pseudomonas aeruginosa Microbiologyopen 2019; 8: 11:e844 Ikpa PT., Doktorova M., Meijsen KF., Nieuwenhuijze NDA., Verkade HJ., Jonker JW., de Jonge HR., Bijvelds MJC. Heirali AA., Acosta N., Storey DG., Workentine ML., Somayaji Impaired Intestinal Farnesoid X Receptor Signaling in Cystic Fibrosis R., Laforest-Lapointe I., Leung W., Quon BS., Berthiaume Y., Mice Rabin HR., Waddell BJ., Rossi L., Surette MG., Parkins MD. Cellular and Molecular Gastroenterology and Hepatology 2020; 9: The effects of cycled inhaled aztreonam on the cystic fibrosis (CF) 47 - 60 lung microbiome Journal of Cystic Fibrosis 2019; 18: 829 - 837

Kidd JM., Sakon CM., Oleksiuk LM., Cies JJ., Pettit RS., Nicolau DP., Kuti JL. Pharmacokinetics of Telavancin in Adult Patients with Cystic Fibrosis during Acute Pulmonary Exacerbation Antimicrobial Agents and Chemotherapy 2020; 64: 1:e01914-19

Lababidi N., Kissi EO., Elgaher WAM., Sigal V., Haupenthal J., Waters VJ., Kidd TJ., Canton R., Ekkelenkamp MB., Johansen Schwarz BC., Hirsch AKH., Rades T., Schneider M. HK., LiPuma JJ., Bell SC., Elborn JS., Flume PA., VanDevanter Spray-drying of inhalable, multifunctional formulations for the DR., Gilligan P., Bullington W., Burgel PR., Byrnes C., Drevinek treatment of biofilms formed in cystic fibrosis P., Holmes A., Kahl B., Maples H., et al Journal of Controlled Release 2019; 314: 62 - 71 Reconciling Antimicrobial Susceptibility Testing and Clinical Lin Y., Chang RYK., Britton WJ., Morales S., Kutter E., Li J., Response in Antimicrobial Treatment of Chronic Cystic Fibrosis Chan HK. Lung Infections Inhalable combination powder formulations of phage and Clinical Infectious Diseases 2019; 69: 1812 - 1816 ciprofloxacin for P. aeruginosa respiratory infections European Journal of Pharmaceutics and Biopharmaceutics 2019; 142: 543 - 552 Cardiology

Loebinger MR., Polverino E., Blasi F., Elborn SJ., Chalmers JD., Derella CC., Lee N., Crandall R., Blackburn M., Looney J., Tiddens HAWM., Goossens H., Tunney M., Zhou WC., Mangieri A., Rodriguez-Miguelez P., Tucker MA., Harris RA. Angyalosi G., Hill AT., Haworth CS. Assessment of endothelial function is reproducible in patients with Efficacy and safety of tobramycin inhalation powder in bronchiectasis cystic fibrosis patients with P. aeruginosa infection: Design of a dose-finding study Journal of Cystic Fibrosis 2019; 18: 772 - 777 (iBEST-1) Pulmonary Pharmacology & Therapeutics 2019; 58: ArNo: 101834 Ghaderian M., Keivanfar M., Beiranvand SB., Reisi M. Cardiac Involvement in Mild Cystic Fibrosis Lung Involvement Lora-Tamayo J., Murillo O., Ariza J. Assessed by Tissue-Doppler Echocardiography Clinical Use of Colistin in Biofilm-Associated Infections International Journal of Pediatrics-mashhad 2019; 7: 10205 - 10214 Polymyxin Antibiotics: From Laboratory Bench To Bedside 2019; 1145: 181 - 195 Nowak JK., Szczepanik M., Wojsyk-Banaszak I., Madry E., Wykretowicz A., Krzyanowska-Jankowska P., Drzymala-Czyz S., McKinzie CJ., Chen L., Ehlert K., Grisso AG., Linafelter A., Nowicka A., Pogorzelski A., Sapiejka E., Skorupa W., Lubsch L., O'Brien CE., Pan AC., Wright BA., Elson EC. Miskiewicz-Chotnicka A., Lisowska A., Walkowiak J. Off-label use of intravenous antimicrobials for inhalation in patients Cystic fibrosis dyslipidaemia: A cross-sectional study with cystic fibrosis Journal of Cystic Fibrosis 2019; 18: 566 - 571 Pediatric Pulmonology 2019; 54: Ferreira DP., Chaves CRMD., da Costa ACC. Nation RL., Landersdorfer CB. Adherence of adolescents with cystic fibrosis to enzyme replacement Optimization of dosing regimens of intravenous colistin in patients therapy: associated factors with cystic fibrosis: What data are required? Ciencia & Saude Coletiva 2019; 24: 4717 - 4726 Pediatric Pulmonology 2019; 54: 1497 - 1498 Ratte MT., Jones AE., Witt DM., Young DC. Peixoto AO., Marson FAL., Souza TH., Fraga ADA., Ribeiro JD. Survey of current treatment practices for venous thromboembolism in Lung ultrasound assessment of response to antibiotic therapy in cystic patients with cystic fibrosis fibrosis exacerbations: a study of two cases Pediatric Pulmonology 2019; 19: 1:795 Jornal Brasileiro de Pneumologia 2019; 45: 6:e20190128 Sciatti E., Vizzardi E., Bonadei I., Valentini F., Menotti E., Prati Rajkumari J., Borkotoky S., Reddy D., Mohanty SK., F., Dallapellegrina L., Berlendis M., Poli P., Padoan R., Metra M. Kumavath R., Murali A., Suchiang K., Busi S. Focus on echocardiographic right ventricular strain analysis in cystic Anti-quorum sensing and anti-biofilm activity of 5- fibrosis adults without cardiovascular risk factors: a case-control hydroxymethylfurfural against Pseudomonas aeruginosa PAO1: study Insights from in vitro, in vivo and in silico studies Internal and Emergency Medicine 2019; 14: 1279 - 1285 Microbiological Research 2019; 226: 19 - 26 Vizzardi E., Sciatti E., Bonadei I., Cani DS., Menotti E., Prati F., Rosales-Reyes R., Esposito F., Fuga B., Cerdeira L., Gayosso- Dallapellegrina L., Metra M., Berlendis M., Poli P., Padoan R. Vazquez C., Lezana-Fernandez JL., Lascurain R., Valvano MA., Macro- and microvascular functions in cystic fibrosis adults without Lincopan N., Santos-Preciado JI. cardiovascular risk factors: A case-control study Draft Genome Sequence of a Pseudomonas aeruginosa Sequence Monaldi Archives for Chest Disease 2019; 89: 2:UNSP 1035 Type 3351 Strain Exhibiting High-Level Resistance to Polymyxins in a Pediatric Patient with Cystic Fibrosis in Mexico Microbiology Resource Announcements 2020; 9: 2:e01261-19 Cell Biology

Stewart PS., White B., Boegli L., Hamerly T., Williamson KS., Franklin MJ., Bothner B., James GA., Fisher S., Vital-Lopez FG., Ahmadi S., Wu YS., Li MY., Ip W., Lloyd-Kuzik A., Di Paola M., Wallqvist A. Du K., Xia S., Lew A., Bozoky Z., Forman-Kay J., Bear CE., Conceptual Model of Biofilm Antibiotic Tolerance That Integrates Gonska T. Phenomena of Diffusion, Metabolism, Gene Expression, and Augmentation of Cystic Fibrosis Transmembrane Conductance Physiology Regulator Function in Human Bronchial Epithelial Cells via Journal of Bacteriology 2019; 201: 22:e00307-19 SLC6A14-Dependent Amino Acid Uptake Implications for Treatment of Cystic Fibrosis Stultz JS., Arnold SR., Shelton CM., Bagga B., Lee KR. American Journal of Respiratory Cell and Molecular Biology 2019; Antimicrobial stewardship impact on Pseudomonas aeruginosa 61: 755 - 764 susceptibility to meropenem at a tertiary pediatric institution American Journal of Infection Control 2019; 47: 1513 - 1515 Bellisola G., Caldrer S., Cestelli-Guidi M., Cinque G. Infrared biomarkers of impaired cystic fibrosis transmembrane Talebi G., Hakemi-Vala M. regulator protein biogenesis Survey on Some Carbapenems and Colistin Resistance Genes Among Journal of Biophotonics 2020; 13: 1:e201900174 Pseudomonas aeruginosa Isolates from Burn and Cystic Fibrosis Patients, Tehran, Iran Braccia C., Tomati V., Caci E., Pedemonte N., Armirotti A. Archives of Clinical Infectious Diseases 2019; 14: 5:e93651 SWATH label-free proteomics for cystic fibrosis research Journal of Cystic Fibrosis 2019; 18: 501 - 506 Tueffers L., Barbosa C., Bobis I., Schubert S., Hoppner M., Ruhlemann M., Franke A., Rosenstiel P., Friedrichs A., Krenz- Declercq M., Treps L., Carmeliet R., Witters P. Weinreich A., Fickenscher H., Bewig B., Schreiber S., The role of endothelial cells in cystic fibrosis Journal of Cystic Fibrosis 2019; 18: 752 - 761 Schulenburg H. Pseudomonas aeruginosa populations in the cystic fibrosis lung lose susceptibility to newly applied beta-lactams within 3 days Journal of Antimicrobial Chemotherapy 2019; 74: 2916 - 2925 Favia M., de Bari L., Bobba A., Atlante A. Degrugillier F., Simon S., Aissat A., Remus N., Mekki C., Decrouy An Intriguing Involvement of Mitochondria in Cystic Fibrosis X., Hatton A., Hinzpeter A., Hoffmann B., Sermet-Gaudelus I., Journal of Clinical Medicine 2019; 32: ArNo: 101461 Callebaut I., Fanen P., Pruliere-Escabasse V.

Ideozu JE., Zhang X., Rangaraj V., McColley S., Levy H. Unsolved severe chronic rhinosinusitis elucidated by extensive CFTR Microarray profiling identifies extracellular circulating miRNAs genotyping Clinical Case Reports 2019; 9: ArNo: 13729 dysregulated in cystic fibrosis Scientific Reports 2019; 9: ArNo: 15483 Duan TY., Cil O., Tse CM., Sarker R., Lin RX., Donowitz M.,

Li HY., Valkenier H., Thorne AG., Dias CM., Cooper JA., Kieffer Verkman AS. M., Busschaert N., Gale PA., Sheppard DN., Davis AP. Inhibition of CFTR-mediated intestinal chloride secretion as potential Anion carriers as potential treatments for cystic fibrosis: transport in therapy for bile acid diarrhea FASEB Journal 2019; 33: 10924 - 10934 cystic fibrosis cells, and additivity to channel-targeting drugs Chemical Science 2019; 10: 9663 - 9672 Feng JR., Wu X., Zhang YN., Yang XJ., Ma GC., Chen ST., Luo

Matos AM., Pinto FR., Barros P., Amaral MD., Pepperkok R., SG., Zhang Y. Matos P. A novel mutation (-195C > A) in the promoter region of CFTR gene Inhibition of calpain 1 restores plasma membrane stability to is associated with Chinese Congenital Bilateral Absence of Vas pharmacologically rescued Phe508del-CFTR variant Deferens (CBAVD) Gene 2019; 719: ArNo: UNSP 144 Journal of Biological Chemistry 2019; 47: 436 - 445

Mroz MS., Harvey BJ. Holt GT., Jou JD., Gil NP., Lowegard AU., Martin JW., Madden Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to DR., Donald BR. restore airway surface liquid height in cystic fibrosis bronchial Computational Analysis of Energy Landscapes Reveals Dynamic epithelial cells Features That Contribute to Binding of Inhibitors to CFTR- Steroids 2019; 151: ArNo: 108461 Associated Ligand Journal of Physical Chemistry B 2019; 123: 10441 - 10455 Ridley C., Lockhart-Cairns MP., Collins RF., Jowitt TA., Subramani DB., Kesimer M., Baldock C., Thornton DJ. Kim D., Huang JW., Billet A., Abu-Arish A., Goepp J., Matthes The C-terminal dimerization domain of the respiratory mucin E., Tewfik MA., Frenkiel S., Hanrahan JW. MUC5B functions in mucin stability and intracellular packaging Pendrin Mediates Bicarbonate Secretion and Enhances Cystic Fibrosis before secretion Transmembrane Conductance Regulator Function in Airway Surface Journal of Biological Chemistry 2019; 294: 17105 - 17116 Epithelia American Journal of Respiratory Cell and Molecular Biology 2019; Stevens EM., Vladar EK., Alanin MC., Christensen ST., von 60: 705 - 716 Buchwald C., Milla C. Ciliary Localization of the Intraflagellar Transport Protein IFT88 Is Kim Y., Jun I., Shin DH., Yoon JG., Piao H., Jung J., Park HW., Disrupted in Cystic Fibrosis Cheng MH., Bahar I., Whitcomb DC., Lee MG. American Journal of Respiratory Cell and Molecular Biology 2020; Regulation of CFTR Bicarbonate Channel Activity by WNK1: 62: 120 - 123 Implications for Pancreatitis and CFTR-Related Disorders Cellular and Molecular Gastroenterology and Hepatology 2020; 9: Valley HC., Bukis KM., Bell A., Cheng Y., Wong E., Jordan NJ., 79 - 103 Allaire NE., Sivachenko A., Liang F., Bihler H., Thomas PJ., Mahiou J., Mense M. Marklew AJ., Patel W., Moore PJ., Tan CD., Smith AJ., Sassano Isogenic cell models of cystic fibrosis-causing variants in natively MF., Gray MA., Tarran R. expressing pulmonary epithelial cells Cigarette Smoke Exposure Induces Retrograde Trafficking of CFTR Journal of Cystic Fibrosis 2019; 18: 476 - 483 to the Endoplasmic Reticulum Scientific Reports 2019; 9: ArNo: 13655 Zoso A., Sofoluwe A., Bacchetta M., Chanson M. Transcriptomic profile of cystic fibrosis airway epithelial cells Michels M., Matte U., Fraga LR., Mancuso ACB., Ligabue-Braun undergoing repair R., Berneira EFR., Siebert M., Sanseverino MT. Scientific Data 2019; 10: ArNo: 2497 Determining the pathogenicity of CFTR missense variants: Multiple comparisons of in silico T predictors and variant annotation databases Genetics and Molecular Biology 2019; 42: 560 - 570

CFTR Pascucci C., De Biase RV., Savi D., Quattrucci S., Gnessi L., Lubrano C., Lenzi A. Bergougnoux A., Deletang K., Pommier A., Varilh J., Hpuriez F., Impact of CFTR-modulating drugs on GH-IGF-1 axis impairment in Altieri JP., Koenig M., Ferec C., Claustres M., Lalau G., adult patients with cystic fibrosis Bienvenu T., Audrezet MP., Pagin A., Girodon E., Raynal C., Journal of Endocrinological Investigation 2019; 42: 1361 - 1363 Taulan-Cadars M. Functional characterization and phenotypic spectrum of three Sasaki S., Sun R., Bui HH., Crosby JR., Monia BP., Guo SL. recurrent I Check for disease-causing deep intronic variants of the Steric Inhibition of 5 ' UTR Regulatory Elements Results in CFTR gene Upregulation of Human CFTR Molecular Therapy 2019; 27: 1749 - 1757 Journal of Cystic Fibrosis 2019; 18: 468 - 475

Caldrer S., Bergamini G., Sandri A., Vercellone S., Rodella L., Scanio MJC., Searle XB., Liu B., Koenig JR., Altenbach R., Cerofolini A., Tomba F., Catalano F., Frulloni L., Buffelli M., Gfesser GA., Bogdan A., Greszler S., Zhao G., Singh A., Fan YH., Tridello G., de Jonge H., Assael BM., Sorio C., Melotti P. Swensen AM., Vortherms T., Manelli A., Balut G., Jia Y., Gao Cystic fibrosis transmembrane conductance regulator functional WQ., Yong H., Schrimpf M., Tse C., Kym P., et al evaluations in a G542X+/-IVS8Tn:T7/9 patient with acute recurrent Discovery of ABBV/GLPG-3221, a Potent Corrector of CFTR for the pancreatitis Treatment of Cystic Fibrosis ACS Medicinal Chemistry Letters 2019; 10: 1543 - 1548 World Journal of Clinical Cases 2019; 7: 3757 - 3764

Chen KG., Zhong PY., Zheng W., Beekman JM. Schneider-Futschik EK. Pharmacological analysis of CFTR variants of cystic fibrosis using Beyond cystic fibrosis transmembrane conductance regulator therapy: stem cell-derived organoids a perspective on gene therapy and small molecule treatment for cystic Drug Discovery Today 2019; 24: 2126 - 2138 fibrosis Gene Therapy 2019; 26: 354 - 362

Singh AK., Fan YH., Balut C., Alani S., Manelli AM., Swensen Ellis S., Rang C., Kotsimbos T., Keating D., Finlayson F., Stark AM., Jia Y., Neelands TR., Vortherms TA., Liu B., Searle XB., R., Thyagarajan D., Wilson J. Wang XQ., Gao WQ., Hwang TC., Ren HY., Cyr D., Kym PR., CNS imaging studies in cystic fibrosis patients presenting with Conrath K., Tse C. sudden neurological events Biological Characterization of F508delCFTR Protein Processing by BMJ Open Respiratory Research 2019; 6: 1:UNSP e000456 the CFTR Corrector ABBV-2222/GLPG2222 Hammoudeh S., Gadelhak W., AbdulWahab A., Al-Langawi M., Journal of Pharmacology and Experimental Therapeutics 2020; 372: Janahi IA. 107 - 118 Approaching two decades of cystic fibrosis research in Qatar: a Smits RM., Oud MS., Vissers LELM., Lugtenberg D., Braat historical perspective and future directions DDM., Fleischer K., Ramos L., D'Hauwers KWM. Multidisciplinary Respiratory Medicine 2019; 14: 1:29

Improved detection of CFTR variants by targeted next-generation Kern-Goldberger AS., Hill-Ricciuti AC., Zhou JYJ., Savant AP., sequencing in male infertility: a case series Rugg L., Dozor AJ., Welter J., Saiman L., Quittell LM. Reproductive Biomedicine Online 2019; 39: 963 - 968 Perceptions of safety monitoring in CF clinical studies and potential Spano V., Montalbano A., Carbone A., Scudieri P., Galietta LJV., impact on future study participation Barraja P. Journal of Cystic Fibrosis 2019; 18: 530 - 535

An overview on chemical structures as Delta F508-CFTR correctors McGarry ME., Williams WA., McColley SA. European Journal of Medicinal Chemistry 2019; 180: 430 - 448 The demographics of adverse outcomes in cystic fibrosis Villamizar O., Waters SA., Scott T., Saayman S., Grepo N., Urak Pediatric Pulmonology 2019; 54:

R., Davis A., Jaffe A., Morris KV. Mohindru B., Turner D., Sach T., Bilton D., Carr S., Archangelidi Targeted Activation of Cystic Fibrosis Transmembrane Conductance O., Bhadhuri A., Whitty JA. Regulator Health economic modelling in Cystic Fibrosis: A systematic review Molecular Therapy 2019; 27: 1737 - 1748 Journal of Cystic Fibrosis 2019; 18: 452 - 460

Wahabi I., Fredj SH., Sahli C., Dabboubi R., Siala H., Hadj IB., Morrison CB., Markovetz MR., Ehre C. Bousetta K., Messaoud T. Mucus, mucins, and cystic fibrosis Analysis of IVS8 CFTR gene polymorphism in asthmatic children in Pediatric Pulmonology 2019; 54: a Tunisian population Revue Francaise D Allergologie 2019; 59: 363 - 368 Pawlaczyk-Kamienska T., Borysewicz-Lewicka M., Sniatala R., Batura-Gabryel H., Cofta S. Yeh JT., Hwang TC. Dental and periodontal manifestations in patients with cystic fibrosis - Positional effects of premature termination codons on the biochemical A systematic review and biophysical properties of CFTR Journal of Cystic Fibrosis 2019; 18: 762 - 771 Journal of Physiology-London 2019; 86: 987 - 994 Pawlaczyk-Kamienska T., Sniatala R., Batura-Gabryel H., Yu B., Zhu XJ., Yang XY., Jin LL., Xu J., Ma TH., Yang H. Borysewicz-Lewicka M., Cofta S. Plumbagin Prevents Secretory Diarrhea by Inhibiting CaCC and Periodontal Status and Subgingival Biofilms in Cystic Fibrosis Adults CFTR Channel Activities Polish Journal of Microbiology 2019; 68: 377 - 382 Frontiers in Pharmacology 2019; 10: ArNo: 1181 Sag E., Kamasak T., Kaya G., Cakir M. Zhou D., Bai R., Wang L. A rare clinical association: Barth syndrome and cystic fibrosis The Cystic Fibrosis Transmembrane Conductance Regulator 470 Met Turkish Journal of Pediatrics 2019; 61: 134 - 138 Allele Is Associated with an Increased Risk of Chronic Pancreatitis in Both Asian and Caucasian Populations: A Meta-Analysis Sznurkowska K., Kazmierska K., Sledzinski T., Zagierski M., Genetic Testing and Molecular Biomarkers 2020; 24: 24 - 32 Liberek A., Szlagatys-Sidorkiewicz A. Serum chemerin level, cytokine profile and nutritional status in children with cystic fibrosis Clinical Acta Biochimica Polonica 2019; 66: 445 - 449

Barczi E., Meszaros M., Bohacs A., Geczi L., Vereczkey I., Muller V. Databases & Registries Testicular Cancer in a Lung Transplant Patient With Cystic Fibrosis: A Case Report Dogru D., Cakir E., Sismanlar T., Cobanoglu N., Pekcan S., Cinel Transplantation Proceedings 2019; 51: 1293 - 1295 G., Yalcin E., Kiper N., Sen V., Sen HS., Ercan O., Keskin O., Eltan SB., Al Shadfan LM., Yazan H., Altintas DU., Basile M., Andrews J., Wang J., Hadjiliadis D., Henthorne K., Sasihuseyinoglu S., Sapan N., Cekic S., Cokugras H. Fields S., Kozikowski A., Huamantla J., Hajizadeh N. Cystic fibrosis in Turkey: First data from the national registry Using qualitative methods to inform the design of a decision aid for Pediatric Pulmonology 2019; 9: ArNo: 16259 people with advanced cystic fibrosis: The Informed Choices CF patient decision aid Rodriguez-Cala F., Suarez-Medina R., Venero-Fernandez SJ., Patient Education and Counseling 2019; 102: 1985 - 1990 Smyth A., Carr SB., Fogarty AW. The prevalence, clinical status and genotype of cystic fibrosis patients Bell SC., Mall MA., Gutierrez H., Macek M., Madge S., Davies living in Cuba using national registry data JC., Burgel PR., Tullis E., Castaos C., Castellani C., Byrnes CA., Journal of Cystic Fibrosis 2019; 18: 522 - 524 Cathcart F., Chotirmall SH., Cosgriff R., Eichler I., Fajac I., Goss CH., Drevinek P., Farrell PM., et al The future of cystic fibrosis care: a global perspective Diabetes Lancet Respiratory Medicine 2020; 8: 65 - 124

Calthorpe RJ., Smith S., Gathercole K., Smyth AR. Choudhury M., Taylor P., Morgan PH., Duckers J., Lau D., Using digital technology for home monitoring, adherence and self- George L., Ketchell RI., Wong FS. management in cystic fibrosis: a state-of-the-art review Association between HbA(1c) and the development of cystic fibrosis- Thorax 2020; 75: 72 - 77 related diabetes Diabetic Medicine 2019; 36: 1251 - 1255 Coton J., Le HH., Veuillet V., Janiaud P., Cucherat M., Kassai- Koupai B., Gueyffier F., Reix P. Colomba J., Boudreau V., Lehoux-Dubois C., Desjardins K., Do patients with cystic fibrosis participating in clinical trials Coriati A., Tremblay F., Rabasa-Lhoret R. demonstrate placebo response? A meta-analysis The main mechanism associated with progression of glucose Journal of Cystic Fibrosis 2019; 18: 461 - 467 intolerance in older patients with cystic fibrosis is insulin resistance and not reduced insulin secretion capacity Journal of Cystic Fibrosis 2019; 18: 551 - 556

Frost F., Jones GH., Dyce P., Jackson V., Nazareth D., Walshaw MJ. Walshaw MJ. Cystic fibrosis: Diagnosis and management - NICE guideline 78 Paediatric Respiratory Reviews 2019; 31: 12 - 14 Loss of incretin effect contributes to postprandial hyperglycaemia in cystic fibrosis-related diabetes Yuan Z., Hou L., Bariya M., Nyein HYY., Tai LC., Ji WB., Liabc Diabetic Medicine 2019; 9: ArNo: 13460 L., Javey A.

Gan HW., Bhatt JM., Denvir L., Randell T., Sachdev P. A multi-modal sweat sensing patch for cross-verification of sweat Monogenic diabetes mellitus in cystic fibrosis rate, total ionic charge, and Na+ concentration Lab On a Chip 2019; 19: 3179 - 3189 Archives of Disease in Childhood 2019; 104: 887 - 889

Lam ATN., Aksit MA., Vecchio-Pagan B., Shelton CA., Osorio Zhou ZP., Alvarez D., Milla C., Zare RN. DL., Anzmann AF., Goff LA., Whitcomb DC., Blackman SM., Proof of concept for identifying cystic fibrosis from perspiration Cutting GR. samples Increased expression of anion transporter SLC26A9 delays diabetes Proceedings of the National Academy of Sciences of the United States onset in cystic fibrosis 2019; 116: 24408 - 24412 Journal of Clinical Investigation 2020; 130: 272 - 286

Monteiro KS., Azevedo MD., Jales LM., da Silva FEP., Arrais Endocrinology RF., de Mendonca KMPP. Effects of aerobic interval training on glucose tolerance in children Moheet A., Chan CL., Granandos A., Putman M., Moheet A. and adolescents with cystic fibrosis: a randomized trial protocol Endocrine Complications of Cystic Fibrosis: A Multisystem Disease Trials 2019; 294: 18269 - 18284 of the Endocrine Organs

Perrem L., Stanojevic S., Solomon M., Carpenter S., Ratjen F. Seminars in Respiratory and Critical Care Medicine 2019; 40: 810 - Incidence and risk factors of paediatric cystic fibrosis-related diabetes 824 Journal of Cystic Fibrosis 2019; 18: 874 - 878

Prentice BJ., Ooi CY., Strachan RE., Hameed S., Ebrahimkhani Epidemiology S., Waters SA., Verge CF., Widger J. Early glucose abnormalities are associated with pulmonary Pasterkamp H., Menzies KJ., Bayomi DJ. inflammation in young children with cystic fibrosis Cystic fibrosis in Canadian Hutterites Journal of Cystic Fibrosis 2019; 18: 869 - 873 Pediatric Pulmonology 2019; 9: ArNo: 17877

Reynaud Q., Boudreau V., Touzet S., Desjardins K., Bourdy SP., Blond E., Berthiaume Y., Rabasa-Lhoret R., Durieu I. Exercise Glucose tolerance in Canadian and French cystic fibrosis adult patients Denford S., Hill DM., Mackintosh KA., McNarry MA., Barker Scientific Reports 2019; 9: ArNo: 4763 AR., Williams CA., Williams C., Williams C., Barker A., Denford Sun XS., Yi YL., Liang B., Yang Y., He N., Ode KL., Uc A., S., Main E., Rand S., Douglas H., Byron M., Holland A., Cox N., Wang K., Gibson-Corley KN., Engelhardt JF., Norris AW. O'Halloran P., Mackintosh K., McNarry M., et al Incretin dysfunction and hyperglycemia in cystic fibrosis: Role of Using photo-elicitation to explore perceptions of physical activity acyl-ghrelin among young people with cystic fibrosis Journal of Cystic Fibrosis 2019; 18: 557 - 565 BMC Pulmonary Medicine 2019; 19: 1:220

Tommerdahl KL., Brinton JT., Vigers T., Nadeau KJ., Zeitler Gambazza S., Guarise R., Carta F., Ambrogi F., Mirabella M., PS., Chan CL. Brivio A., Colombo C. Screening for cystic fibrosis-related diabetes and prediabetes: Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A Evaluating 1,5-anhydroglucitol, fructosamine, glycated albumin, and cross-sectional study hemoglobin A1c Pediatric Pulmonology 2020; 55: 394 - 400

Pediatric Diabetes 2019; 63: 602 - 609 Kampouras A., Hatziagorou E., Avramidou V., Georgopoulou V., Kirvassilis F., Hebestreit H., Tsanakas J. Diagnosis Ventilation efficiency to exercise in patients with cystic fibrosis Pediatric Pulmonology 2019; 54: 1584 - 1590

Albu CC., Stancu IG., Grigore LG., Albu DF., Albu SD., Patrascu Lopez-de-Uralde-Villanueva I., Visa TS., Marichalar PM., del A., Goganau AM. Corral T. Impact of genetic testing and family health history of cystic fibrosis in Minimal detectable change in six-minute walk test in children and the early prenatal diagnosis and prevention of a new case of genetic adolescents with cystic fibrosis disorder Disability and Rehabilitation 2019; 20: 1:212

Romanian Journal of Morphology and Embryology 2019; 60: 667 - Rudolf I., Pieper K., Nolte H., Junge S., Dopfer C., Sauer- 671 Heilborn A., Ringshausen FC., Tummler B., von Jan U., Albrecht Bensliman S., Lefevre N., Duchateau J., Hanssens L. UV., Fuge J., Hansen G., Dittrich AM. Review of the sweat test indications in a Brussels' cystic fibrosis Assessment of a Mobile App by Adolescents and Young Adults With reference center Cystic Fibrosis: Pilot Evaluation Annales de Biologie Clinique 2019; 77: 687 - 692 JMIR mHealth and uHealth 2019; 7: 11:e12442

Dubot P., Liang J., Dubs J., Missiak Y., Sarazin C., Couderc F., Shei RJ., Mackintosh KA., Lever JEP., McNarry MA., Krick S. Causse E. Exercise Physiology Across the Lifespan in Cystic Fibrosis Sweat chloride quantification using capillary electrophoresis Frontiers in Physiology 2019; 10: ArNo: 1382 Practical Laboratory Medicine 2019; 83: 205 - 283

Goncalves AC., Marson FAL., Mendonca RMH., Bertuzzo CS., Gastroenterology Paschoal IA., Ribeiro JD., Ribeiro AF., Levy CE. Chloride and sodium ion concentrations in saliva and sweat as a Ellery KM., Kopp B., Conwell DL., Gariepy C. method to diagnose cystic fibrosis Secondhand Smoke Exposure and Serum Trypsinogen in Cystic Jornal de Pediatria 2019; 95: 443 - 450 Fibrosis Carriers Simmonds NJ. Pancreas 2019; 48: 1155 - 1159 Is it cystic fibrosis? The challenges of diagnosing cystic fibrosis Paediatric Respiratory Reviews 2019; 31: 6 - 8

Gabel ME., Galante GJ., Freedman SD. Zeiger AM., McGarry ME., Mak ACY., Medina V., Salazar S., Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis Eng C., Liu AK., Oh SS., Nuckton TJ., Jain D., Blackwell TW., Seminars in Respiratory and Critical Care Medicine 2019; 40: 825 - Kang HM., Abecasis G., Onate LC., Seibold MA., Burchard EG., 841 Rodriguez-Santana J.

Masip E., Donat E., Polo B., Oltra S., Ortega P., Ribes-Koninckx Identification of CFTR variants in Latino patients with cystic fibrosis C. from the Dominican Republic and Puerto Rico Recurrent Metabolic Alkalosis in a Cystic Fibrosis Patient: Pediatric Pulmonology 2019; 129: 725 - 727 Coexistence with Congenital Chloride Diarrhea Journal of Pediatric Genetics 2020; 9: 44 - 47 Growth & Development Mavilia M. Distal intestinal obstructive syndrome (DIOS): a gastrointestinal Calella P., Valerio G., Brodlie M., Taylor J., Donini LM., complication of cystic fibrosis in adults Siervo M. Clinical Journal of Gastroenterology 2019; 12: 571 - 573 Tools and Methods Used for the Assessment of Body Composition in

Nazareth D., Mohan K., Fewins H., Walshaw M. Patients With Cystic Fibrosis: A Systematic Review Nutrition in Clinical Practice 2019; 34: 701 - 714 Evaluation of Gastric Emptying in Cystic Fibrosis Using Bedside Ultrasonography Chedevergne F., Sermet-Gaudelus I. Journal of Ultrasound in Medicine 2019; 38: 2955 - 2962 Prevention of osteoporosis in cystic fibrosis Current Opinion in Pulmonary Medicine 2019; 25: 660 - 665 Padoan R., Cirilli N., Falchetti D., Cesana BM. Risk factors for adverse outcome in infancy in meconium ileus cystic Stahl M., Holfelder C., Kneppo C., Kieser M., Kasperk C., fibrosis infants: A multicentre Italian study Schoenau E., Sommerburg O., Tonshoff B. Journal of Cystic Fibrosis 2019; 18: 863 - 868 FS7BJ Multiple prevalent fractures in relation to macroscopic bone

Pellegrino C., Molinaro F., Ferrara F., Bindi E., Fusi G., Messina architecture in patients with cystic fibrosis Journal of Cystic Fibrosis 2018; 17: 114 - 120 M., Angotti R. Meconium-related ileum in preterm and low birth weight children Ubago-Guisado E., Cavero-Redondo I., Alvarez-Bueno C., Chirurgia-Italy 2019; 32: 236 - 239 Vlachopoulos D., Martinez-Vizcaino V., Gracia-Marco L.

Tan SMJ., Coffey MJ., Ooi CY. Bone Health in Children and Youth with Cystic Fibrosis: A Differences in clinical outcomes of paediatric cystic fibrosis patients Systematic Review and Meta-Analysis of Matched Cohort Studies with and without meconium ileus Journal of Pediatrics 2019; 215: 178 Journal of Cystic Fibrosis 2019; 18: 857 - 862 van Mourik P., Beekman JM., van der Ent CK. Immunology & Inflammation Intestinal organoids to model cystic fibrosis European Respiratory Journal 2019; 54: 1:1802379 Berkebile AR., Bartlett JA., Abou Alaiwa M., Varga SM., Power

Yeh KM., Johansson O., Le H., Rao K., Markus I., Perera DS., UF., McCray PB. Lubowski DZ., King DW., Zhang L., Chen HZ., Liu L. Airway Surface Liquid Has Innate Antiviral Activity That Is Reduced Cystic fibrosis transmembrane conductance regulator modulates in Cystic Fibrosis enteric cholinergic activities and is abnormally expressed in the American Journal of Respiratory Cell and Molecular Biology 2020; 62: 104 - 111 enteric ganglia of patients with slow transit constipation Journal of Gastroenterology 2019; 54: 994 – 1006 Cappiello F., Ranieri D., Carnicelli V., Casciaro B., Chen HT., Ferrera L., Di YP., Mangoni ML. Bronchial epithelium repair by Esculentin-1a-derived antimicrobial Gene Therapy peptides: involvement of metalloproteinase-9 and interleukin-8, and evaluation of peptides' immunogenicity Brothers KB., Devereaux M., Sade RM. Scientific Reports 2019; 9: ArNo: 18988 Bespoke Babies: Genome Editing in Cystic Fibrosis Embryos Annals of Thoracic Surgery 2019; 108: 995 - 999 Castellani S., D'Oria S., Diana A., Polizzi AM., Di Gioia S., Mariggio MA., Guerra L., Favia M., Vinella A., Leonetti G., De Delhove J., Osenk I., Prichard I., Donnelley M. Venuto D., Gallo C., Montemurro P., Conese M. Public Acceptability of Gene Therapy and Gene Editing for Human G-CSF and GM-CSF Modify Neutrophil Functions at Concentrations Use: A Systematic Review found in Cystic Fibrosis Human Gene Therapy 2020; 31: 20 - 46 Scientific Reports 2019; 9: ArNo: 12937

Caution K., Pan A., Krause K., Badr A., Hamilton K., Vaidya A., Genetics Gosu H., Daily K., Estfanous S., Gavrilin MA., Drew ME., Cormet-Boyaka E., Chen X., Frankhouser DE., Bundschuh R., Arslan AB., Zamani AG., Pekcan S., Yildirim MS. Yan P., Dakhlallah D., Amer AO. A Novel Pathogenic Variant of the CFTR Gene in a Patient with Methylomic correlates of autophagy activity in cystic fibrosis Cystic Fibrosis Phenotype-c.4096A > T Journal of Cystic Fibrosis 2019; 18: 491 - 500

Journal of Pediatric Genetics 2020; 9: 40 - 43 Chen G., Sun L., Kato T., Okuda K., Martino MB., Abzhanova Chen Y., Li XL., Liao HJ., Leung XT., He JB., Wang X., Li FP., A., Lin JM., Gilmore RC., Batson BD., O'Neal YK., Volmer AS., Yue HX., Xu WM. Dang H., Deng YM., Randell SH., Button B., Livraghi-Butrico A., CFTR mutation compromises spermatogenesis by enhancing miR-15b Kesimer M., Ribeiro CMP., O'Neal WK., et al maturation and suppressing its regulatory target CDC25A IL-1 beta dominates the promucin secretory cytokine profile in cystic Biology of Reproduction 2019; 101: 50 - 62 fibrosis Journal of Clinical Investigation 2019; 129: 4433 - 4450 Rafeey M., Jabarpoor-Bonyadi M., Vahedi L. Genotype-Phenotype Correlation for Cystic Fibrosis According to Cogen JD., Whitlock KB., Gibson RL., Hoffman LR., Registry Center of Cystic Fibrosis VanDevanter DR. Crescent Journal of Medical and Biological Sciences 2020; 7: 124 - The use of antimicrobial susceptibility testing in fibrosis pulmonary 129 exacerbations Journal of Cystic Fibrosis 2019; 18: 851 - 856

Guney E., Emiralioglu N., Cinel G., Yalcin E., Dogru D., Kiper Scholte BJ., Horati H., Veltman M., Vreeken RJ., Garratt LW., N., Ozcelik HU. Tiddens HAWM., Janssens HM., Stick SM. Nasal nitric oxide levels in primary ciliary dyskinesia, cystic fibrosis Oxidative stress and abnormal bioactive lipids in early cystic fibrosis and healthy children lung disease Turkish Journal of Pediatrics 2019; 61: 20 - 25 Journal of Cystic Fibrosis 2019; 18: 781 - 789

Hall JR., Maloney SE., Jin HB., Taylor JB., Schoenfisch MH. Tucker MA., Fox BM., Seigler N., Rodriguez-Miguelez P., Looney Nitric oxide diffusion through cystic fibrosis-relevant media and lung J., Thomas J., McKie KT., Forseen C., Davison GW., Harris RA. tissue Endothelial Dysfunction in Cystic Fibrosis: Role of Oxidative Stress RSC Advances 2019; 40: 701 - 714 Oxidative Medicine and Cellular Longevity 2019; 2019: ArNo: 1629638 Hassanzad M., Hajiesmail G., Farnia P., Ghanavi J., Khanbabayi G., Feshangchi-Bonab M., Razeghi E., Velayati AA. Zulueta A., Peli V., Dei Cas M., Colombo M., Paroni R., Falleni Investigating the Effect of TNF alpha (-863) and TNF alpha (-308) M., Baisi A., Bollati V., Chiaramonte R., Del Favero E., Ghidoni genes Polymorphism on the Progression of Disease in Patients with R., Caretti A. Cystic Fibrosis Inflammatory role of extracellular sphingolipids in Cystic Fibrosis International Journal of Pediatrics-Mashhad 2019; 7: 10335 - 10341 International Journal of Biochemistry & Cell Biology 2019; 116:

Leung ST., Khoury O., Barrios C., Ortega VE., Atala A., Murphy ArNo: UNSP 105 SV. Immune and Cytokine Dysfunction in Cystic Fibrosis Liver Disease American Journal of Respiratory Cell and Molecular Biology 2019; 61: 656 - 658 Boelle PY., Debray D., Guillot L., Corvol H. Malhotra S., Hayes D., Wozniak DJ. SERPINA1 Z allele is associated with cystic fibrosis liver disease Mucoid Pseudomonas aeruginosa and regional inflammation in the Genetics in Medicine 2019; 21: 2151 - 2155 cystic fibrosis lung Lewindon PJ., Puertolas-Lopez MV., Ramm LE., Noble C., Journal of Cystic Fibrosis 2019; 18: 796 - 803 Pereira TN., Wixey JA., Hartel GF., Calvopina DA., Leung DH., McElvaney OJ., Wade P., Murphy M., Reeves EP., McElvaney Ramm GA. NG. Accuracy of Transient Elastography Data Combined With APRI in Targeting airway inflammation in cystic fibrosis Detection and Staging of Liver Disease in Pediatric Patients With Expert Review of Respiratory Medicine 2019; 286: 1912:20191794 Cystic Fibrosis Clinical Gastroenterology and Hepatology 2019; 17: 2561 McElvaney OJ., Zaslona Z., Becker-Flegler K., Palsson- McDermott EM., Boland F., Gunaratnam C., Gulbins E., O'Neill Ling SC., Ye W., Leung DH., Navarro OM., Weymann A., LA., Reeves EP., McElvaney NG. Karnsakul W., Freeman AJ., Magee JC., Narkewicz MR. Specific Inhibition of the NLRP3 Inflammasome as an Liver Ultrasound Patterns in Children With Cystic Fibrosis Correlate Antiinflammatory Strategy in Cystic Fibrosis With Noninvasive Tests of Liver Disease American Journal of Respiratory and Critical Care Medicine 2019; Journal of Pediatric Gastroenterology and Nutrition 2019; 69: 351 - 200: 1381 - 1391 357

Merakou C., Schaefers MM., Priebe GP. Sellers ZM., Lee LW., Barth RA., Milla C. Progress Toward the Elusive Pseudomonas aeruginosa Vaccine New Algorithm for the Integration of Ultrasound Into Cystic Fibrosis Surgical Infections 2018; 19: 757 - 768 Liver Disease Screening

Oriano M., Terranova L., Sotgiu G., Saderi L., Bellofiore A., Journal of Pediatric Gastroenterology and Nutrition 2019; 69: 404 - 410 Retucci M., Marotta C., Gramegna A., Miglietta D., Carnini C., Marchisio P., Chalmers JD., Aliberti S., Blasi F. Wong A., Cheung B., Nejad C., Gantier M., Graudins A. Evaluation of active neutrophil elastase in sputum of bronchiectasis Hepatotoxicity after paracetamol overdose in a patient with cystic and cystic fibrosis patients: A comparison among different techniques fibrosis despite early acetylcysteine and utility of microRNA to Pulmonary Pharmacology & Therapeutics 2019; 59: ArNo: 101856 predict hepatotoxicity

Perrem L., Ratjen F. Clinical Toxicology 2018; 56: 904 - 906 Anti-inflammatories and mucociliary clearance therapies in the age of CFTR modulators Microbiology Pediatric Pulmonology 2019; 54:

Ravnholt C., Qvist T., Kolpen M., Pressler T., Skov M., Hoiby N. Amoureux L., Sauge J., Sarret B., Lhoumeau M., Bajard A., Tetu Antibody response against Mycobacterium avium complex in cystic J., Bador J., Neuwirth C., Caillon J., Cardot-Martin E., Cattoir fibrosis patients measured by a novel IgG ELISA test V., Doleans-Jordheim A., Ferroni A., Guet-Revillet H., Hery- Journal of Cystic Fibrosis 2019; 18: 516 - 521 Arnaud G., Segonds C., Thomas E., Plesiat P.

Rosenow T., Mok LC., Turkovic L., Berry LJ., Sly PD., Study of 109 Achromobacter spp. isolates from 9 French CF centres Ranganathan S., Tiddens HAWM., Stick SM. reveals the circulation of a multiresistant clone of A. xylosoxidans The cumulative effect of inflammation and infection on structural belonging to ST 137 Journal of Cystic Fibrosis 2019; 18: 804 - 807 lung disease in early cystic fibrosis European Respiratory Journal 2019; 54: 1:1801771 Bandyopadhaya A., Tzika AA., Rahme LG.

Scambler T., Jarosz-Griffiths HH., Lara-Reyna S., Pathak S., Pseudomonas aeruginosa Quorum Sensing Molecule Alters Skeletal Wong C., Holbrook J., Martinon F., Savic S., Peckham D., Muscle Protein Homeostasis by Perturbing the Antioxidant Defense McDermott MF. System mBio 2019; 10: 5:e02211-19 ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in cystic fibrosis Bayjanov JR., Ekkelenkamp MB., Rogers MRC., Canton R., eLife 2019; 8: ArNo: e9248 Benaissa-Trouw BJ., Diez-Aguilar M., Tunney M., Fluit AC.

Simpson T., Elston C., Macedo P., Perrin F. Whole-genome analysis of Pandoraea species strains from cystic Amyloidosis in cystic fibrosis fibrosis patients Future Microbiology 2019; 14: 1357 - 1368 Paediatric Respiratory Reviews 2019; 31: 32 - 34 Blanchard AC., Tang L., Tadros M., Muller M., Spilker T., Waters VJ., LiPuma JJ., Tullis E. Burkholderia cenocepacia ET12 transmission in adults with cystic fibrosis Thorax 2020; 75: 88 - 90

Blanchard AC., Waters VJ. Forton JT. Microbiology of Cystic Fibrosis Airway Disease Detecting respiratory infection in children with cystic fibrosis: Cough Seminars in Respiratory and Critical Care Medicine 2019; 40: 727 - swab, sputum induction or bronchoalveolar lavage 736 Paediatric Respiratory Reviews 2019; 31: 28 - 31

Bouchara JP., Le Govic Y., Kabbara S., Cimon B., Zouhair R., Gohil S., Donaghy B., Tature D., Kowal J., Lea S., Lai FYL., Hamze M., Papon N., Nevez G. Range S., Tang JW. Advances in understanding and managing Scedosporium respiratory Seasonal respiratory virus testing in management of adult cystic infections in patients with cystic fibrosis fibrosis patients Expert Review of Respiratory Medicine 2019; 116: 25697 - 25706 Journal of Hospital Infection 2019; 103: 360 - 361

Boukerb AM., Simon M., Pernet E., Jouault A., Portier E., Persyn Gross JE., Martiniano SL., Nick JA. E., Bouffartigues E., Bazire A., Chevalier S., Feuilloley MGJ., Prevention of transmission of Mycobacterium abscessus among Lesouhaitier O., Caillon J., Dufour A. patients with cystic fibrosis Draft Genome Sequences of Four Pseudomonas aeruginosa Clinical Current Opinion in Pulmonary Medicine 2019; 25: 646 - 653

Strains with Various Biofilm Phenotypes Herzog S., Dach F., de Buhr N., Niemann S., Schlagowski J., Microbiology Resource Announcements 2020; 9: 1:e01286-19 Chaves-Moreno D., Neumann C., Goretzko J., Schwierzeck V., Caverly LJ., Lu JN., Carmody LA., Kalikin LM., Shedden K., Mellmann A., Dubbers A., Kuster P., Schultingkemper H., Opron K., Azar M., Cahalan S., Foster B., VanDevanter DR., Rescher U., Pieper DH., von Kockritz-Blickwede M., Kahl BC Simon RH., LiPuma JJ. High Nuclease Activity of Long Persisting Staphylococcus aureus Measures of Cystic Fibrosis Airway Microbiota during Periods of Isolates Within the Airways of Cystic Fibrosis Patients Protects Clinical Stability Against NET-Mediated Killing Annals of the American Thoracic Society 2019; 16: 1534 - 1542 Frontiers in Immunology 2019; 10: ArNo: 2552

Chirgwin ME., Dedloff MR., Holban AM., Gestal MC. Ibberson CB., Whiteley M. Novel Therapeutic Strategies Applied to Pseudomonas aeruginosa The Staphylococcus aureus Transcriptome during Cystic Fibrosis Infections in Cystic Fibrosis Lung Infection Materials 2019; 74: 1168 - 1173 mBio 2019; 10: 6:e02774

Cipoll L., Rocca F., Armitano RI., Martinez C., Almuzara M., Iribarren ME., Moreno RMG., Caceres LD., Sanz MTP., Moreno Faccone D., Vay C., Prieto M. BB., Cavero TA., Torrecillas SG., Cruz NDZ., Bermudez JA. Development and evaluation of an in-house database for quick Study of a Cohort of Patients With Cystic Fibrosis and Isolation of identification of Burkholderia contaminans by MALDI-TOF MS Scedosporium spp. Archivos de Bronconeumologia 2019; 55: 559 - Revista Argentina de Microbiologia 2019; 51: 255 - 258 564

Coffey MJ., Nielsen S., Wemheuer B., Kaakoush NO., Garg M., Jorth P., Spero MA., Livingston J., Newman DK. Needham B., Pickford R., Jaffe A., Thomas T., Ooi CY. Quantitative Visualization of Gene Expression in Mucoid and Gut Microbiota in Children With Cystic Fibrosis: A Taxonomic and Nonmucoid Pseudomonas aeruginosa Aggregates Reveals Localized Functional Dysbiosis Peak Expression of Alginate in the Hypoxic Zone Scientific Reports 2019; 9: ArNo: 18593 mBio 2019; 10: 6:e02622-19

Cowman S., van Ingen J., Griffith DE., Loebinger MR. Kevat AC., Carzino R., Vidmar S., Ranganathan S. Non-tuberculous mycobacterial pulmonary disease Glycoprotein A as a biomarker of pulmonary infection and European Respiratory Journal 2019; 54: 1:1900250 inflammation in children with cystic fibrosis Pediatric Pulmonology 2019; 74: 386 - 392 Davies JC., Alton E., Simbo A., Murphy R., Seth I., Williams K., Somerville M., Jolly L., Morant S., Guest C. Khil PP., Chiang AD., Ho J., Youn JH., Lemon JK., Gea- Training dogs to differentiate Pseudomonas aeruginosa from other Banacloche J., Frank KM., Parta M., Bonomo RA., Dekker JP. cystic fibrosis bacterial pathogens: not to be sniffed at? Dynamic mergence of Mismatch Repair Deficiency Facilitates Rapid European Respiratory Journal 2019; 54: 5:1900970 Evolution of Ceftazidime-Avibactam Resistance in Pseudomonas

Degiacomi G., Sammartino JC., Chiarelli LR., Riabova O., aeruginosa Acute Infection mBio 201; 10: 5:e01822-19 Makarov V., Pasca MR. Mycobacterium abscessus, an Emerging and Worrisome Pathogen Lennartz E., Schwartbeck B., Dubbers A., Grosse-Onnebrink J., among Cystic Fibrosis Patients Kessler C, Kuster P., Schultingkemper H., Peters G., Kahl BC. International Journal of Molecular Sciences 2019; 29: 6:e96977 The prevalence of Staphylococcus aureus with mucoid phenotype in

Deschamp AR., Hatch JE., Slaven JE., Gebregziabher N., Storch the airways of patients with cystic fibrosis-A prospective study International Journal of Medical Microbiology 2019; 309: 283 - 287 G., Hall GL., Stick S., Ranganathan S., Ferkol TW., Davis SD. Early respiratory viral infections in infants with cystic fibrosis Li KW., Gifford AH., Hampton TH., O'Toole GA. Journal of Cystic Fibrosis 2019; 18: 844 - 850 Availability of Zinc Impacts Interactions between Streptococcus

Dogan O., Tunckanat F., Cinel G., Sener B., Ozcelik HU., Yalcin sanguinis and Pseudomonas aeruginosa in Coculture Journal of Bacteriology 2020; 202: 2:e00618-19 EE., Ersoz DD., Kiper EN. Investigation of role of anaerobic bacteria in cystic fibrosis patients Lightly TJ., Frejuk KL., Groleau MC., Chiarelli LR., Ras C., Tuberkuloz ve Torak-Tuberculosis and Thorax 2019; 67: 151 - 161 Buroni S., Deziel E., Sorensen JL., Cardona ST.

Esoda CN., Kuehn MJ. Phenylacetyl Coenzyme A, Not Phenylacetic Acid, Attenuates CepIR- Pseudomonas aeruginosa Leucine Aminopeptidase Influences Early Regulated Virulence in Burkholderia cenocepacia Applied and Environmental Microbiology 2019; 85: 24:e01594-19 Biofilm Composition and Structure via Vesicle-Associated Antibiofilm Activity Magalhaes AP., Jorge P., Pereira MO. mBio 2019; 10: 6:e02548-19 Pseudomonas aeruginosa and Staphylococcus aureus communication

Ferreira MR., Gomes SC., Moreira LM. in biofilm infections: insights through network and database Mucoid switch in Burkholderia cepacia complex bacteria: Triggers, construction Critical Reviews in Microbiology 2019; 45: 712 - 728 molecular mechanisms and implications in pathogenesis Advances in Applied Microbiology, Vol 107 2019; 107: 113 - 140 Mainz JG., Baier M., Jaudszus A., Tabori H., Ribeiro JD., Lorenz

Fischer AJ., Kilgore SH., Singh SB., Allen PD., Hansen AR., M. Limoli DH., Schlievert PM. Pseudomonas aeruginosa colonization in the upper and lower airways High Prevalence of Staphylococcus aureus Enterotoxin Gene Cluster of a child with cystic fibrosis: a father's meticulous approach to Superantigens in Cystic Fibrosis Clinical Isolates successful eradication Jornal Brasileiro de Pneumologia 2019; 45: 6:e20190191 Genes 2019; 40: 1215 - 1221 Melnik AV., Vazquez-Baeza Y., Aksenov AA., Hyde E., McAvoy Sousa SA., Soares-Castro P., Seixas AMM., Feliciano JR., AC., Wang MX., da Silva RR., Protsyuk I., Wu JV., Bouslimani Balugas B., Barreto C., Pereira L., Santos PM., Leitao JH. A., Lim YW., Luzzatto-Knaan T., Comstock W., Quinn RA., New insights into the immunoproteome of B. cenocepacia J2315 Wong R., Humphrey G., Ackermann G., Spivey T., Brouha S using serum samples from cystic fibrosis patients Molecular and Microbial Microenvironments in Chronically Diseased New Biotechnology 2020; 54: 62 - 70

Lungs Associated with Cystic Fibrosis Spinler JK., Raza S., Runge JK., Luna RA. mSystems 2019; 4: 5:e00375-19 Complete Genome Sequence of the Multidrug-Resistant Mirzahosseini HK., Hadadi-Fishani M., Morshedi K., Khaledi A. Pseudomonas aeruginosa Endemic Houston-1 Strain, Isolated from a Meta-Analysis of Biofilm Formation, Antibiotic Resistance Pattern, Pediatric Patient with Cystic Fibrosis and Assembled Using Oxford and Biofilm-Related Genes in Pseudomonas aeruginosa Isolated from Nanopore and Illumina Sequencing Clinical Samples Microbiology Resource Announcements 2019; 8: 43:UNSP e00903 Microbial Drug Resistance 2020; 16: 37 - 50 Strelingerova J., Lengyelova L., Boldis M., Rybansky L., Nain Z., Bin Sayed S., Karim MM., Islam MA., Adhikari UK. Mrazova M., Mraz M., Zelenak A. Energy-optimized pharmacophore coupled virtual screening in the Colonisation of Staphylococcus aureus in patients with Nasal discovery of quorum sensing inhibitors of LasR protein of Polyposis Pseudomonas aeruginosa Neuroendocrinology Letters 2019; 40: Journal of Biomolecular Structure & Dynamics 2019; 18: 413 - 431 Tan X., Coureuil M., Ramond E., Euphrasie D., Dupuis M., Tros O'Brien TJ., Welch M. F., Meyer J., Nemazanyy I., Chhuon C., Guerrera IC., Ferroni A., A Continuous-Flow Model for in vitro Cultivation of Mixed Sermet-Gaudelus I., Nassif X., Charbit A., Jamet A. Microbial Populations Associated With Cystic Fibrosis Airway Chronic Staphylococcus aureus Lung Infection Correlates With Infections Proteogenomic and Metabolic Adaptations Leading to an Increased Frontiers in Microbiology 2019; 10: ArNo: 2713 Intracellular Persistence Clinical Infectious Diseases 2019; 69: 1937 - 1945 O'Brien TJ., Welch M. Recapitulation of polymicrobial communities associated with cystic Wardell SJT., Rehman A., Martin LW., Winstanley C., Patrick fibrosis airway infections: a perspective WM., Lamont IL. Future Microbiology 2019; 14: 1437 - 1450 A Large-Scale Whole-Genome Comparison Shows that Experimental

Palser S., Smith S., Nash EF., Agarwal A., Smyth AR. Evolution in Response to Antibiotics Predicts Changes in Naturally Treatments for preventing recurrence of infection with Pseudomonas Evolved Clinical Pseudomonas aeruginosa Antimicrobial Agents and Chemotherapy 2019; 63: 12:e01619-19 aeruginosa in people with cystic fibrosis Cochrane Database of Systematic Reviews 2019; : 12:CD012300 Warrier R., Skoric B., Vidmar S., Carzino R., Ranganathan S.

Patel G., Greenberger PA. The role of geographical location and climate on recurrent Allergic bronchopulmonary aspergillosis Pseudomonas infection in young children with Cystic Fibrosis Journal of Cystic Fibrosis 2019; 18: 817 - 822 Allergy and Asthma Proceedings 2019; 40: 421 - 424

Ponce MC., Svendsen E., Steed L., Flume PA. Webb K., Fogarty A., Barrett DA., Nash EF., Whitehouse JL., Impact of a Reduction in Susceptibility Testing for Pseudomonas Smyth AR., Stewart I., Knox A., Williams P., Halliday N., aeruginosa in a Cystic Fibrosis Program Camara M., Barr HL. American Journal of Respiratory and Critical Care Medicine 2020; Clinical significance of Pseudomonas aeruginosa 2-alkyl-4-quinolone 201: 114 - 116 quorum-sensing signal molecules for long-term outcomes in adults with cystic fibrosis Rani A., Azmi W. Journal of Medical Microbiology 2019; 68: 1823 - 1828 An overview on biosynthesis and applications of extracellular pyocyanin pigment and its role in Pseudomonas aeruginosa Wolter DJ., Onchiri FM., Emerson J., Precit MR., Lee M., pathogenesis McNamara S., Nay L., Blackledge M., Uluer A., Orenstein DM., Annals of Phytomedicine 2019; 8: 28 - 42 Mann M., Hoover W., Gibson RL., Burns JL., Hoffman LR. Prevalence and clinical associations of Staphylococcus aureus small- Reece E., McClean S., Greally P., Renwick J. colony variant respiratory infection in children with cystic fibrosis The prevalence of Aspergillus fumigatus in early cystic fibrosis (SCVSA): a multicentre, observational study disease is underestimated by culture-based diagnostic methods Lancet Respiratory Medicine 2019; 7: 1027 - 1038 Journal of Microbiological Methods 2019; 164: ArNo: 105683 Zemanick ET., Bell SC. Richards CJ., Olivier KN. Prevention of chronic infection with Pseudomonas aeruginosa Nontuberculous Mycobacteria in Cystic Fibrosis infection in cystic fibrosis Seminars in Respiratory and Critical Care Medicine 2019; 40: 737 - Current Opinion in Pulmonary Medicine 2019; 25: 636 - 645 750 Zhu K., Chen S., Sysoeva TA., You LC. Saiman L. Universal antibiotic tolerance arising from antibiotic-triggered Improving outcomes of infections in cystic fibrosis in the era of accumulation of pyocyanin in Pseudomonas aeruginosa CFTR modulator therapy PloS Biology 2019; 17: 12:e3000573 Pediatric Pulmonology 2019; 54: Zuttion F., Sicard D., Dupin L., Vergoten G., Girard-Bock C., Shaw LP., Doyle RM., Kavaliunaite E., Spencer H., Balloux F., Madaoui M., Chevolot Y., Morvan F., Vidal S., Vasseur JJ., Dixon G., Harris KA. Souteyrand E., Phaner-Goutorbe M. Children With Cystic Fibrosis Are Infected With Multiple Deciphering multivalent glycocluster-lectin interactions through AFM Subpopulations of Mycobacterium abscessus With Different characterization of the self-assembled nanostructures Antimicrobial Resistance Profiles Soft Matter 2019; 15: 7211 - 7218 Clinical Infectious Diseases 2019; 69: 1678 - 1686

Sherrard LJ., Einarsson GG., Johnston E., O'Neill K., McIlreavey L., McGrath SJ., Gilpin DF., Downey DG., Reid A., Nutrition McElvaney NG., Boucher RC., Muhlebach MS., Elborn JS., Tunney MM. Altman K., McDonald CM., Michel SH., Maguiness K. Assessment of stability and fluctuations of cultured lower airway Nutrition in cystic fibrosis: From the past to the present and into the bacterial communities in people with cystic fibrosis future Pediatric Pulmonology 2019; 54: Journal of Cystic Fibrosis 2019; 18: 808 - 816

Brownell JN., Bashaw H., Stallings VA. Psychosocial Growth and Nutrition in Cystic Fibrosis Seminars in Respiratory and Critical Care Medicine 2019; 40: 775 - Bianco B., Horsley A., Brennan A. 791 Implications of fatherhood in cystic fibrosis Ciofu O., Smith S., Lykkesfeldt J. Paediatric Respiratory Reviews 2019; 31: 18 - 20

Antioxidant supplementation for lung disease in cystic fibrosis Drabble SJ., O'Cathain A., Arden MA., Hutchings M., Beever D., Cochrane Database of Systematic Reviews 2019; : 10:CD007020 Wildman M. Ciuca IM., Pop LL., Dediu M., Tanasescu SA., Ardelean F., When Is Forgetting Not Forgetting? A Discursive Analysis of Iovanescu G., Boeriu E., Vlad CD., Marc MS., Guta BA. Differences in Forgetting Talk Between Adults With Cystic Fibrosis Vitamin D(25-OH-cholecalciferol) in Cystic Fibrosis and the With Different Levels of Adherence to Nebulizer Treatments Relations with Cholesterol and Proteins Qualitative Health Research 2019; 29: 2119 - 2131 Revista de Chimie 2019; 70: 3185 - 3187 Ferreira DP., Chaves CRMD., da Costa ACC. Koniarek-Maniecka A., Olszowiec-Chlebna M., Podlecka D., Adherence of adolescents with cystic fibrosis to enzyme replacement Jerzynska J., Stelmach I. therapy: associated factors The effect of treatment with calcitriol and cholecalciferol on calcium Ciencia & Saude Coletiva 2019; 24: 4717 - 4726 and phosphate metabolism in patients with cystic fibrosis Flewelling KD., Sellers DE., Sawicki GS., Robinson WM., Dill EJ. Pediatria I Medycyna Rodzinna-paediatrics and Family Medicine Social support is associated with fewer reported symptoms and 2019; 15: 152 - 156 decreased treatment burden in adults with cystic fibrosis Leveille P., Knoth IS., Denis MH., Morin G., Barlaam F., Journal of Cystic Fibrosis 2019; 18: 572 - 576

Nyalendo C., Daneault C., Marcotte JE., Des Rosiers C., Ferland Havermans T., Willem L. G., Lippe S., Mailhot G. Prevention of anxiety and depression in cystic fibrosis Association between fat-soluble nutrient status and auditory and Current Opinion in Pulmonary Medicine 2019; 25: 654 - 659 visual related potentials in newly diagnosed non-screened infants with cystic fibrosis: A case-control study Keyte R., Egan H., Nash EF., Regan A., Jackson C., Mantzios M. Prostaglandins Leukotrienes and Essential Fatty Acids 2019; 150: 21 An exploration into experiences and attitudes regarding risky health - 30 behaviours in an adult cystic fibrosis population Psychology Health & Medicine 2019; 7: ArNo: 406 Miller T., Antos NJ., Brock LA., Wade T., Goday PS. Lactation Consultation Sustains Breast Milk Intake in Infants With McIlwaine M., Button B., Nevitt SJ. Cystic Fibrosis Positive expiratory pressure physiotherapy for airway clearance in Journal of Pediatric Gastroenterology and Nutrition 2019; 69: 358 - people with cystic fibrosis Cochrane Database of Systematic Reviews 2019; : 11:CD003147 362

Mulherin DW., Bensink J., Zuckerwise L., Murphree JN., Tolle McLeod C., Norman R., Schultz A., Mascaro S., Webb S., J., Seidner DL. Snelling T. Long-Term Parenteral Nutrition in a Patient With Cystic Fibrosis Discrete choice experiment to evaluate preferences of patients with During Pregnancy cystic fibrosis among alternative treatment-related health outcomes: a Journal of Parenteral and Enteral Nutrition 2019; 43: 1065 - 1068 protocol BMJ Open 2019; 9: Norsa L., Zazzeron L., Cuomo M., Claut L., Bulfamante AMC., Biffi A., Colombo C. Murray TS., O'Rourke TK., Feinn R., Drapeau G., Collins MS. Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Nebulizer cleaning and disinfection practices in families with cystic Digestive System fibrosis: The relationship between attitudes, practice and microbe Nutrients 2019; 11: 8:a033928 colonization Journal of Cystic Fibrosis 2019; 18: 823 - 828 Pitman RT., Mui M., Michelson PH., Manary MJ. Ready-to-Use Supplemental Food for Nutritional Supplementation in Quick V., Chang G. Cystic Fibrosis Health care provider's experiences, practices, and recommendations Current Developments in Nutrition 2019; 3: 5:UNSP nzz016 for interventions and screening of cystic fibrosis patients with disordered eating: A qualitative analysis Riddle JD. Chronic Illness 2019; 9: ArNo: 14496 Vitamin E supplementation in people with cystic fibrosis: A Cochrane review summary Quittner AL., Eakin MN., Alpern AN., Ridge AK., McLean KA., International Journal of Nursing Studies 2019; 97: 132 - 133 Bilderback A., Criado KK., Chung SE., Riekert KA. Clustered randomized controlled trial of a clinic-based problem- Schonenberger KA., Reber E., Bally L., Geiser T., Lin D., Stanga solving intervention to improve adherence in adolescents with cystic Z. fibrosis Nutritional assessment in adults with cystic fibrosis Journal of Cystic Fibrosis 2019; 18: 879 - 885 Nutrition 2019; 67-68: ArNo: UNSP 110 Russell JK., Strodl E., Connolly J., Kavanagh DJ. Slae M., Wilschanski M. A Metacognitive Intervention of Narrative Imagery for young people Prevention of malnutrition in cystic fibrosis with cystic fibrosis: A feasibility study Current Opinion in Pulmonary Medicine 2019; 25: 674 - 679 Journal of Health Psychology 2019; 20: 18:4518

Suthoff E., Mainz JG., Cox DW., Thorat T., Grossoehme DH., Physiotherapy Fridman M., Sawicki GS., Rosenfeld M. Caregiver Burden Due to Pulmonary Exacerbations in Patients with Bourbeau J., McIvor RA., Devlin HM., Kaplan A. Cystic Fibrosis Oscillating positive expiratory pressure (OPEP) device therapy in Journal of Pediatrics 2019; 215: 164

Canadian respiratory disease management: Review, care gaps and Toprak D., Nay L., McNamara S., Rosenberg AR., Rosenfeld M., suggestion for use Yi-Frazier JP. Canadian Journal of Respiratory Critical Care and Sleep Medicine Resilience in adolescents and young adults with cystic fibrosis: A 2019; 3: 233 - 240 pilot feasibility study of the promoting resilience in stress management intervention Pediatric Pulmonology 2019; 129: 5236 - 5253

Trandel ET., Pilewski JM., Dellon EP., Moreines LT., Yabes JG., Lubovich S., Zaragoza S., Rodriguez V., Buendia J., Vargas BC., Jeong K., Arnold RM., Kavalieratos D. Moreira JA., Galanternik L., Ratto P., Teper A. Symptom Burden and Unmet Existential Needs in Adults With Cystic Risk factors associated with pulmonary exacerbations in pediatric Fibrosis patients with cystic fibrosis Western Journal of Nursing Research 2019; 41: 1448 - 1464 Archivos Argentinos de Pediatria 2019; 117: E466 - E471

Werner S., Halpern A., Kurz S., Rosenne H. Lumertz MS., Pinto LA. Disclosure in Cystic Fibrosis: A Qualitative Study Sleep-disordered breathing in cystic fibrosis pediatric subjects Journal of Social Issues 2019; 75: 881 - 903 Sleep Science 2019; 12: 165 - 170

Marsteller NL., Nussbaum E., Morphew T., Randhawa IS. Pulmonology Cystic fibrosis patients at risk for disease progression marked by decline in FEV1% predicted: development of the cystic fibrosis risk Balazs A., Mall MA. of disease progression score Mucus obstruction and inflammation in early cystic fibrosis lung Journal of Thoracic Disease 2019; 11: 5557 - 5565 disease: Emerging role of the IL-1 signaling pathway Milross MA., Piper AJ., Dwyer TJ., Bye PTP. Pediatric Pulmonology 2019; 54: Non-invasive ventilation versus oxygen therapy in cystic fibrosis: Balinotti JE., Chang DV., Lubovich S., Rodriguez V., Zaragoza Long-term effects Reply S., Escobar N., Kofman C., Perez GL., Ardiles V., Teper A. Respirology 2019; 38: 2127 - 2135

Course of lung function in children with cystic fibrosis in their first 3 Milross MA., Piper AJ., Dwyer TJ., Wong K., Bell SC., Bye PTP., years of life Robbins L., Dobbin C., Moriarty C., Willson G., Norman M., Archivos Argentinos de Pediatria 2019; 117: 323 - 328 Regnis J., Sullivan C., Grunstein R., Douglas J. Benlala I., Hocke F., Macey J., Bui S., Berger P., Laurent F., Non-invasive ventilation versus oxygen therapy in cystic fibrosis: A Dournes G. 12-month randomized trial Quantification of MRI T2-weighted High Signal Volume in Cystic Respirology 2019; 24: 1191 - 1197

Fibrosis: A Pilot Study Olivenca DV., Fonseca LL., Voit EO., Pinto FR. Radiology 2020; 294: 186 - 196 Thickness of the airway surface liquid layer in the lung is affected in Bergeron C., Cantin AM. cystic fibrosis by compromised synergistic regulation of the ENaC ion Cystic Fibrosis: Pathophysiology of Lung Disease channel Seminars in Respiratory and Critical Care Medicine 2019; 40: 715 - Journal of the Royal Society Interface 2019; 16: 157:20190187 726 Parisi GF., Papale M., Tardino L., Nenna R., Midulla F., Couch MJ., Morgado F., Kanhere N., Kowalik K., Rayment JH., Leonardi S. Ratjen F., Santyr G. Biomarkers in Pediatric Lung Diseases Including Cystic Fibrosis Assessing the feasibility of hyperpolarized Xe-129 multiple-breath Current Respiratory Medicine Reviews 2019; 15: 163 - 173 washout MRI in pediatric cystic fibrosis Sarc I., Ziherl K., Esquinas AM. Magnetic Resonance in Medicine 2019; 9: ArNo: 17535 Non-invasive ventilation versus oxygen therapy in cystic fibrosis: Garcia B., Flume PA. Long-term effects Pulmonary Complications of Cystic Fibrosis Respirology 2019; 24: 1222

Seminars in Respiratory and Critical Care Medicine 2019; 40: 804 - Schmid-Mohler G., Yorke J., Spirig R., Benden C., Caress AL. 809 Adult patients' experiences of symptom management during Gojsina BZ., Rodic M., Visekruna J., Trajkovic GZ., Sovtic AD., pulmonary exacerbations in cystic fibrosis: A thematic synthesis of Minic PB. qualitative research Pulmonary exacerbations as a risk factor for lung function decline - Chronic Illness 2019; 15: 245 - 263 experiences of the National Cystic Fibrosis Center Seidl E., Kiermeier H., Liebisch G., Ballmann M., Hesse S., Paul- Vojnosanitetski Pregled 2019; 76: 1110 - 1114 Buck K., Ratjen F., Rietscherl E., Griese M. Gold LS., Patrick DL., Hansen RN., Goss CH., Kessler L. Lavage lipidomics signatures in children with cystic fibrosis and Correspondence between lung function and symptom measures from protracted bacterial bronchitis the Cystic Fibrosis Respiratory Symptom Diary-Chronic Respiratory Journal of Cystic Fibrosis 2019; 18: 790 - 795

Infection Symptom Score (CFRSD-CRISS) Suthoff E., Mainz JG., Cox DW., Thorat T., Grossoehme DH., Journal of Cystic Fibrosis 2019; 18: 886 - 893 Fridman M., Sawicki GS., Rosenfeld M. Gomes A., Hutcheon D., Ziegler J. Caregiver Burden Due to Pulmonary Exacerbations in Patients with Association Between Fat-Free Mass and Pulmonary Function in Cystic Fibrosis Patients With Cystic Fibrosis: A Narrative Review Journal of Pediatrics 2019; 215: 164 - + Nutrition in Clinical Practice 2019; 34: 715 - 727 Wagener JS., VanDevanter DR., Konstan MW., Pasta DJ., Millar Goss CH. SJ., Morgan WJ. Acute Pulmonary Exacerbations in Cystic Fibrosis Lung function changes before and after pulmonary exacerbation Seminars in Respiratory and Critical Care Medicine 2019; 40: 792 - antimicrobial treatment in cystic fibrosis 803 Pediatric Pulmonology 2019; 9: ArNo: 16564

Hough J., Christensen H. Yousefzadegan S., Shirzadi R., Navaie S., Takzare A., Pediatric hospital in the home: clinical outcomes for treatment of a Ghaempanah M., Modaresi M. cystic fibrosis respiratory exacerbation Cast Bronchitis in a Cystic Fibrosis Child: Experience with Direct Physiotherapy Theory and Practice 2020; 23: ArNo: 102113 Instillation of Dornase Alfa as an Adjunct Treatment Agent Iranian Journal of Pediatrics 2019; 29: 5:e89703 Jang JH., Panariti A., O'Sullivan MJ., Pyrch M., Wong C., Lauzon AM., Martin JG. Zang XL., Monge ME., Gaul DA., McCarty NA., Stecenko A., Characterization of cystic fibrosis airway smooth muscle cell Fernandez FM. proliferative and contractile activities Early Detection of Cystic Fibrosis Acute Pulmonary Exacerbations by American Journal of Physiology-Lung Cellular and Molecular Exhaled Breath Condensate Metabolomics Physiology 2019; 317: L690 - L701 Journal of Proteome Research 2020; 19: 144 - 152

Karanth TK., Karanth VKLKL., Ward BK., Woodworth BA., Karanth L. Medical interventions for chronic rhinosinusitis in cystic fibrosis Cochrane Database of Systematic Reviews 2019; : 10:CD012979 Radiology Krishnananthan T., Pao C. The implications and management of cystic fibrosis screen positive, Abrami M., Maschio M., Conese M., Confalonieri M., Di Gioia S., inconclusive diagnosis patients Paediatric Respiratory Reviews 2019; 31: 21 - 24 Gerin F., Dapas B., Tonon F., Farra R., Murano E., Zanella G., Salton F., Torelli L., Grassi G., Grassi M. Ooi CY., Sutherland R., Castellani C., Keenan K., Boland M., Use of low field nuclear magnetic resonance to monitor lung Reisman J., Bjornson C., Chilvers MA., van Wylick R., Kent S., inflammation and the amount of pathological components in the Price A., Mateos-Corral D., Hughes D., Solomon M., Zuberbuhler sputum of cystic fibrosis patients P., Brusky J., Durie PR., Ratjen F., et al Magnetic Resonance in Medicine 2019; 25: 1110 - 1122 Immunoreactive trypsinogen levels in newborn screened infants with

Bouma NR., Janssens HM., Andrinopoulou ER., Tiddens an inconclusive diagnosis of cystic fibrosis BMC Pediatrics 2019; 16: ArNo: UNSP 147 HAWM. Airway disease on chest computed tomography of preschool children Sadik I., de Algaba IP., Jimenez R., Benito C., Blasco-Alonso J., with cystic fibrosis is associated with school-age bronchiectasis Caro P., Navas-Lopez VM., Perez-Frias J., Perez E., Serrano J., Pediatric Pulmonology 2019; 9: ArNo: 12896 Yahyaoui R.

Cohen RWF., Folescu TW., Boechat MCB., Fonseca VM., Initial Evaluation of Prospective and Parallel Assessments of Cystic Marques EA., Leao RS. Fibrosis Newborn Screening Protocols in Eastern Andalusia: IRT/IRT High-resolution computed tomography findings in young infants with versus IRT/PAP/IRT International Journal of Neonatal Screening 2019; 74: 2657 - 2665 cystic fibrosis detected by new-born screening Clinics 2019; 74: ArNo: e1399 Southern KW., Barben J., Gartner S., Munck A., Castellani C.,

Nyilas S., Bauman G., Pusterla O., Ramsey K., Singer F., Mayell SJ., Davies JC., Winters V., Murphy J., Salinas D., Stranzinger E., Yammine S., Casaulta C., Bieri O., Latzin P. McColley SA., Ren CL., Farrell PM. Ventilation and perfusion assessed by functional MRI in children with Inconclusive diagnosis after a positive newborn bloodspot screening CF: reproducibility in comparison to lung function result for cystic fibrosis; clarification of the harmonised international Journal of Cystic Fibrosis 2019; 18: 543 - 550 definition Journal of Cystic Fibrosis 2019; 18: 778 - 780 Oda M., Staziaki PV., Qureshi MM., Andreu-Arasa VC., Li BJ., Takumi K., Chapman MN., Wang A., Salama AR., Sakai O. Terlizzi V., Mergni G., Buzzetti R., Centrone C., Zavataro L., Using CT texture analysis to differentiate cystic and cystic-appearing Braggion C. odontogenic lesions Cystic fibrosis screen positive inconclusive diagnosis (CFSPID): European Journal of Radiology 2019; 120: ArNo: UNSP 108 Experience in Tuscany, Italy Journal of Cystic Fibrosis 2019; 18: 484 - 490 Strzelczuk-Judka L., Wojsyk-Banaszak I., Zakrzewska A., Jonczyk-Potoczna K. Diagnostic value of chest ultrasound in children with cystic fibrosis - Therapy (Non Antimicrobial) Pilot study PLoS One 2019; 14: 7:e0215786 Akshintala VS., Kamal A., Faghih M., Cutting GR., Cebotaru L.,

Woods JC., Wild JM., Wielputz MO., Clancy JP., Hatabu H., West NE., Jennings MT., Dezube R., Whitcomb DC., Lechtzin N., Kauczor HU., van Beek EJR., Altes TA. Merlo CA., Singh VK. Current state of the art MRI for the longitudinal assessment of cystic Cystic fibrosis transmembrane conductance regulator modulators fibrosis reduce the risk of recurrent acute pancreatitis among adult patients Journal of Magnetic Resonance Imaging 2019; 200: 1466 - 1471 with pancreas sufficient cystic fibrosis Pancreatology 2019; 19: 1023 - 1026

Calzetta L., Matera MG., Rogliani P., Cazzola M. Screening Ensifentrine Dual phosphodiesterase PDE3/4 inhibitor Treatment of COPD Treatment of cystic fibrosis Basaran AE., Basaran A., Kocacik Uygun DF., Alper O., Acican Drugs of the Future 2019; 44: 845 - 854 D., Bingol A. Initial regional evaluation of the Cystic Fibrosis Newborn Screening Carbone A., Montalbano A., Spano V., Musante I., Galietta LJV., program: data from the Mediterranean coast of Turkey Barraja P. Turkish Journal of Medical Sciences 2019; 49: 1655 - 1661 Furocoumarins as multi-target agents in the treatment of cystic fibrosis Beauchamp KA., Taber KAJ., Grauman PV., Spurka L., Lim- European Journal of Medicinal Chemistry 2019; 180: 283 - 290 Harashima J., Svenson A., Goldberg JD., Muzzey D. Sequencing as a first-line methodology for cystic fibrosis carrier Charbek E., Kamel G., Nayak RP. screening Ivacaftor for the Treatment of Cystic Fibrosis Coexisting with Genetics in Medicine 2019; 21: 2569 - 2576 Trisomy 21: A Case Report Cureus 2019; 51: 421 - + Ben-Shachar R., Svenson A., Goldberg JD., Muzzey D. A data-driven evaluation of the size and content of expanded carrier Cuyx S., De Boeck K. screening panels Treating the Underlying Cystic Fibrosis Transmembrane Conductance Genetics in Medicine 2019; 21: 1931 - 1939 Regulator Defect in Patients with Cystic Fibrosis Seminars in Respiratory and Critical Care Medicine 2019; 40: 762 - Course CW., Hanks R. 774 Newborn screening for cystic fibrosis: Is there benefit for everyone? Paediatric Respiratory Reviews 2019; 31: 3 - 5 D'Ursi P., Uggeri M., Urbinati C., Millo E., Paiardi G., Milanesi L., Ford RC., Clews J., Meng X., Bergese P., Ridolfi A., Doull I. Pedemonte N., Fossa P., Orro A., Rusnati M. Devil in the detail of newborn screening for cystic fibrosis Exploitation of a novel biosensor based on the full-length human Archives of Disease in Childhood 2019; 104: 938 F508de1-CFTR with computational studies, biochemical and Johnson F., Southern KW., Ulph F. biological assays for the characterization of a new Psychological Impact on Parents of an Inconclusive Diagnosis Lumacaftor/Tezacaftor analogue Following Newborn Bloodspot Screening for Cystic Fibrosis: A Sensors and Actuators B-chemical 2019; 125: ArNo: UNSP 108

Qualitative Study Davies JC. International Journal of Neonatal Screening 2019; 19: 921 - 928 Trials and tribulations: The highs and lows of running cystic fibrosis drug studies Paediatric Respiratory Reviews 2019; 31: 25 - 27

Frost FJ., Nazareth DS., Charman SC., Winstanley C., Walshaw Sermet-Gaudelus I., Clancy JP., Nichols DP., Nick JA., De Boeck MJ. K., Solomon GM., Mall MA., Bolognese J., Bouisset F., den Ivacaftor Is Associated with Reduced Lung Infection by Key Cystic Hollander W., Paquette-Lamontagne N., Tomkinson N., Henig N., Fibrosis Pathogens A Cohort Study Using National Registry Data Elborn JS., Rowe SM. Annals of the American Thoracic Society 2019; 16: 1375 - 1382 Antisense oligonucleotide eluforsen improves CFTR function in

Hamilton JL., Zobell JT., Robson J. F508del cystic fibrosis Journal of Cystic Fibrosis 2019; 18: 536 - 542 Pancreatic insufficiency converted to pancreatic sufficiency with ivacaftor Sun T., Sun Z., Jiang YL., Ferguson AA., Pilewski JM., Kolls JK., Pediatric Pulmonology 2019; 54: 1654 Chen W., Chen K.

Heijerman HGM., McKone EF., Downey DG., Van Braeckel E., Transcriptomic Responses to Ivacaftor and Prediction of Ivacaftor Rowe SM., Tullis E., Mall MA., Welter JJ., Ramsey BW., Mckee Clinical Responsiveness CM., Marigowda G., Moskowitz SM., Waltz D., Sosnay PR., American Journal of Respiratory Cell and Molecular Biology 2019; 61: 643 - 652 Simard C., Ahluwalia N., Xuan FJ., Zhang YH., et al Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor Tassini S., Langron E., Delang L., Mirabelli C., Lanko K., combination regimen in people with cystic fibrosis homozygous for Crespan E., Kissova M., Tagliavini G., Fonto G., Bertoni S., the F508del mutation: a double-blind, randomised, phase 3 trial Palese S., Giorgio C., Ravanetti F., Ragionieri L., Zamperini C., Lancet 2019; 394: 1940 - 1948 Mancini A., Dreassi E., Maga G., Vergani P.,

Hoy SM. Multitarget CFTR Modulators Endowed with Multiple Beneficial Elexacaftor/Ivacaftor/Tezacaftor: First Approval Side Effects for Cystic Fibrosis Patients: Toward a Simplified Drugs 2019; 79: 2001 - 2007 Therapeutic Approach Journal of Medicinal Chemistry 2019; 62: 10833 - 10847 Hughes DL. Patent Review of Synthetic Routes and Crystalline Forms of the Toprak D., Davis C., Rosenfeld M. CFTR-Modulator Drugs Ivacaftor, Lumacaftor, Tezacaftor, and Treating the Airway Consequences of Cystic Fibrosis Transmembrane Elexacaftor Conductance Regulator Dysfunction Organic Process Research & Development 2019; 23: 2302 - 2322 Seminars in Respiratory and Critical Care Medicine 2019; 40: 751 - 761 Joshi D., Ehrhardt A., Hong JS., Sorscher EJ. Cystic fibrosis precision therapeutics: Emerging considerations Tosco A., Villella VR., Raia V., Kroemer G., Maiuri L. Pediatric Pulmonology 2019; 54: Cystic Fibrosis: New Insights into Therapeutic Approaches Current Respiratory Medicine Reviews 2019; 15: 174 - 186 Kutney K., Donnola SB., Flask CA., Gubitosi-Klug R., O'Riordan M., McBennett K., Sferra TJ., Kaminski B. Yan ZY., McCray PB., Engelhardt JF. Lumacaftor/ivacaftor therapy is associated with reduced hepatic Advances in gene therapy for cystic fibrosis lung disease Human Molecular Genetics 2019; 28: R88 - R94 steatosis in cystic fibrosis patients World Journal of Hepatology 2019; 11: 761 - 772 van der Meer R., Touw DJ., Heijerman HGM.

Middleton PG., Mall MA., Drevinek P., Lands LC., McKone EF., Prevention of drug-related complications in cystic fibrosis Current Opinion in Pulmonary Medicine 2019; 25: 666 - 673 Polineni D., Ramsey BW., Taylor-Cousar JL., Tullis E., Vermeulen F., Marigowda G., McKee CM., Moskowitz SM., Nair Yousefzadegan S., Shirzadi R., Navaie S., Takzare A., N., Savage J., Simard C., Tian S., Waltz D., Xuan F., et al Ghaempanah M., Modaresi M. Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Cast Bronchitis in a Cystic Fibrosis Child: Experience with Direct Phe508del Allele Instillation of Dornase Alfa as an Adjunct Treatment Agent New England Journal of Medicine 2019; 381: 1809 - 1819 Iranian Journal of Pediatrics 2019; 29: 5:e89703

Phuan PW., Tan JA., Rivera AA., Zlock L., Nielson DW., Zhao JQ., Huang WX., Zhang SJ., Xu J., Xue W., He B., Zhang Finkbeiner WE., Haggie PM., Verkman AS. Y. Nanomolar-potency 'co-potentiator' therapy for cystic fibrosis caused Efficacy of Glutathione for Patients With Cystic Fibrosis: A Meta- by a defined subset of minimal function CFTR mutants analysis of Randomized-Controlled Studies Scientific Reports 2019; 9: ArNo: 17640 American Journal of Rhinology & Allergy 2020; 34: 115 - 121

Pritchard MF., Oakley JL., Brilliant CD., Rye PD., Forton J., Doull IJM., Ketchell I., Hill KE., Thomas DW., Lewis PD. Transplantation Mucin structural interactions with an alginate oligomer mucolytic in cystic fibrosis sputum Benden C., Goldfarb SB., Stehlik J. Vibrational Spectroscopy 2019; 103: ArNo: 102932 An aging population of patients with cystic fibrosis undergoes lung Reisi M., Modaresi MR., Aghaii Z., Mirlohi SH., Rafiemanesh H., transplantation: An analysis of the ISHLT Thoracic Transplant Azizi G., Sayedi SJ. Registry Efficacy and safety of oral sildenafil in cystic fibrosis children with Journal of Heart and Lung Transplantation 2019; 38: 1162 - 1169 mild to moderate lung disease Morrell MR., Kiel SC., Pilewski JM. Pediatric Pulmonology 2019; 17: 714 - 725 Organ Transplantation for Cystic Fibrosis Rodriguez-Miguelez P., Ishii H., Seigler N., Crandall R., Thomas Seminars in Respiratory and Critical Care Medicine 2019; 40: 842 - J., Forseen C., McKie KT., Harris RA. 856

Sildenafil improves exercise capacity in patients with cystic fibrosis: a Ruszel N., Kubisa B., Lisowski P., Piotrowska M., Kubisa MJ., proof-of-concept clinical trial Brykczynski M., Wojtys M., Pierog J., Czarnecka M., Wojcik J., Therapeutic Advances in Chronic Disease 2019; 10: ArNo: UNSP Wojcik N., Sielicki P., Bielewicz M., Grodzki T. 204 Bilateral lung transplantation in cystic fibrosis with hepatitis C Rosenfeld M., Cunningham S., Harris WT., Lapey A., Regelmann infection - a study of two cases WE., Sawicki GS., Southern KW., Chilvers M., Higgins M., Tian Kardiochirurgia I Torakochirurgia Polska-polish Journal of Thoracic S., Cooke J., Davies JC. 2019; 16: 133 - 135

An open-label extension study of ivacaftor in children with CF and a Xiao X., Tian HP., Cheng XH., Li GM., Zhou JY., Peng ZY., Li CFTR gating mutation initiating treatment at age 2-5 years (KLIMB) YR. Journal of Cystic Fibrosis 2019; 18: 838 - 843 Pandoraea sputorum Bacteremia In A Patient Who Had Undergone Allogeneic Liver Transplantation Plus Immunosuppressive Therapy: A Case Report Infection and Drug Resistance 2019; 12: 3359 - 3364

Urology

Crass RL., Pai MP. Optimizing Estimated Glomerular Filtration Rate to Support Adult to Pediatric Pharmacokinetic Bridging Studies in Patients with Cystic Fibrosis Clinical Pharmacokinetics 2019; 58: 1323 - 1332

Kopp BT., Joseloff E., Goetz D., Ingram B., Heltshe SL., Leung DH., Ramsey BW., McCoy K., Borowitz D. Urinary metabolomics reveals unique metabolic signatures in infants with cystic fibrosis Journal of Cystic Fibrosis 2019; 18: 507 - 515

Stoyanov GS., Popov H., Petkova L., Dzhenkov DL. The Sixty-five Roses of Cystic Fibrosis: A Report of Two Autopsy Cases with Kidney Involvement Cureus 2019; 5: 1:143