Summaries of scientific publications

CF Research news is a joint project of the European Cystic Fibrosis Society (ECFS) and Journal of CF (JCF). CF Research news are layman’s language summaries of recent work published in JCF. This initiative aims to bridge the gap between people with CF and the researchers investigating CF, and bring research closer to those with the condition. This is done by providing access to patients, parents, relatives, friends and caregivers of patients with CF to high-impact scientific work published in JCF.

CF Europe wants to facilitate this initiative by sharing CF Research News on its site. Via the Google Translate bar, the summaries can be directly translated in your own language.

You can find the most recent summaries via the links below. For more CF Research News, please go to https://www.ecfs.eu/publications/cf-research-news

MUCOID PSEUDOMONAS AERUGINOSA AND REGIONAL INFLAMMATION IN THE CYSTIC FIBROSIS LUNG

Authors: Sankalp Malhotra a,b, Don Hayes, Jr b,c,d, Daniel J. Wozniak a,b,e Affiliations: a Department of Microbial Infection and Immunity, The Ohio State University, Columbus, OH, USA b The Ohio […]

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SPEEDING UP ACCESS TO NEW DRUGS FOR CYSTIC FIBROSIS: CONSIDERATIONS FOR CLINICAL TRIAL DESIGN AND DELIVERY

Authors: On behalf of the European CF Society (ECFS) Strategic Planning Task Force on ‘Speeding up access to new drugs for CF’:  Margarida Amaral6, Kris de Boeck7, Jane C Davies1, […]

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THERANOSTICS BY TESTING CFTR MODULATORS IN PATIENT-DERIVED MATERIALS: THE CURRENT STATUS AND A PROPOSAL FOR SUBJECTS WITH RARE CFTR MUTATIONS

Authors: Margarida Amaral1, Kris De Boeck2 On behalf of the ECFS Strategic Planning Task Force on ‘Speeding up access to new drugs for CF’: Margarida Amaral1, Kris De Boeck2, Jane […]

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LIKELIHOOD-BASED ANALYSIS OF OUTCOME-DEPENDENT SAMPLING DESIGNS WITH LONGITUDINAL DATA

Authors: Zelnick LR, Schildcrout JS, Heagerty PJ   What was your research question? Identifying new biomarkers is important to the CF research community, and it is natural to use already […]

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INHALED ANTIBIOTIC USE IS ASSOCIATED WITH SCEDOSPORIUM/LOMENTOSPORA SPECIES ISOLATION IN CYSTIC FIBROSIS

Authors: Hong G, Lechtzin N, Hadjiliadis D, Kawut S   What was your research question? We wanted to better understand what factors may be contributing to the rise of fungal […]

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A SEMIPARAMETRIC APPROACH TO ESTIMATE RAPID LUNG FUNCTION DECLINE IN CYSTIC FIBROSIS

Authors: RD Szczesniak, GL McPhail, LL Duan, M Macaluso, RS Amin, JP Clancy   What was your research question? We wanted to understand how calculating the timing and degree of […]

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RATE OF UPTAKE OF IVACAFTOR USE AFTER U.S. FOOD AND DRUG ADMINISTRATION APPROVAL AMONG PATIENTS ENROLLED IN THE U.S. CYSTIC FIBROSIS FOUNDATION PATIENT REGISTRY

Authors: Sawicki GS, Dasenbrook E, Fink AK, Schechter MS   What was your research question? We wanted to examine the rate of uptake and patterns of documented ivacaftor use among […]

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DO CURRENT CLINICAL TRIALS IN CYSTIC FIBROSIS MATCH THE PRIORITIES OF PATIENTS AND CLINICIANS? A SYSTEMATIC REVIEW

Authors: Izabella S Kalaitzis, Nicola J Rowbotham, Sherie J Smith, Alan R Smyth Affiliations: Evidence Based Child Health Group, University of Nottingham, UK   What was your research question? Do […]

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NON-FASTING BIOELECTRICAL IMPEDANCE ANALYSIS IN CYSTIC FIBROSIS: IMPLICATIONS FOR CLINICAL PRACTICE AND RESEARCH

Authors: F.M. Hollander-Kraaijeveld1, Y. Lindeman2, N.M. de Roos2, M. Burghard3, E.A. van de Graaf4, H.G.M. Heijerman4 Affiliations: 1Division of Internal Medicine and Dermatology, Department of Dietetics, University Medical Center Utrecht, […]

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SUPERSONIC SHEAR-WAVE ELASTOGRAPHY AND APRI FOR THE DETECTION AND STAGING OF LIVER DISEASE IN PAEDIATRIC CYSTIC FIBROSIS

Authors: Diego A. Calvopina1,2*, Charlton Noble3*, Anna Weis1,2, Gunter F. Hartel4, Louise E. Ramm1, Fariha Balouch3, Manuel A. Fernandez-Rojo2,5, Miranda A. Coleman1, Peter J. Lewindon2,3 and Grant A. Ramm1,2Ŧ Affiliations: […]

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BLOOD BIOMARKERS TO PREDICT SHORT-TERM PULMONARY EXACERBATION RISK IN CHILDREN AND ADOLESCENTS WITH CF: A PILOT STUDY

Authors: Amrit Singh1,2,3, Mary He3, Virginia Chen2,3, Zsuzsanna Hollander2,3, Scott J. Tebbutt2,3,4, Raymond T. Ng2,3, Bruce M. McManus1,2,3, Don D. Sin2,3,4, Felix Ratjen5, Bradley S. Quon2,3,4. Affiliations: 1Department of Pathology […]

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GLPG1837, A CFTR POTENTIATOR, IN p.Gly551Asp (G551D)-CF PATIENTS: AN OPEN-LABEL, SINGLE-ARM, PHASE 2a STUDY (SAPHIRA1)

Authors: Jane C Daviesa, Olivier Van de Steenb, Silke van Koningsbruggen-Rietschelc, Pavel Drevinekd, Nico Derichse, Edward F McKonef, Desirée Kantersb, Lisa Allamasseyb, Florence Namourg, Herman de Kockb, Katja Conrathb Affiliations: […]

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ABNORMAL PRO-GLY-PRO PATHWAY AND AIRWAY NEUTROPHILIA IN PEDIATRIC CYSTIC FIBROSIS

Authors: Andrew R. Turnbull1,2, Chloe J. Pyle3, Dhiren F. Patel3, Patricia L. Jackson4,5, Tom N. Hilliard1,2, Nicolas Regamey6, Hui-Leng Tan2, Sarah Brown2,3, Rebecca Thursfield7, Christopher Short1, Megan McFie3, Eric W.F.W. […]

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PRECISION MEDICINE IN ACTION: THE IMPACT OF IVACAFTOR ON CYSTIC FIBROSIS-RELATED HOSPITALIZATIONS

Authors: Feng LB, Grosse SD, Green RF, Fink AK, Sawicki GS   What was your research question? We wanted to assess the impact of the drug ivacaftor (brand name Kalydeco) […]

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CLINICAL OUTCOMES IN U.S. INFANTS WITH CYSTIC FIBROSIS FROM 2001 TO 2012

Authors: Hoch H, Sontag MK, Scarbro S, Juarez-Colunga E, McLean C, Kempe A, Sagel SD   What was your research question? How have clinical outcomes for children with cystic fibrosis […]

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VARIATION IN LUNG FUNCTION AND NUTRITIONAL DECLINE IN CYSTIC FIBROSIS BY GENOTYPE: AN ANALYSIS OF THE CANADIAN CYSTIC FIBROSIS REGISTRY

Authors: Garvin J. Leung1, Ting J. Cho2, Thomas Kovesi1,2,3, Jemila S. Hamid1,2, Dhenuka Radhakrishnan*1,2,3 Affiliations: 1 Faculty of Medicine, University of Ottawa, Ottawa, ON, K1H 8M5, Canada 2 Children’s Hospital […]

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DISEASE PROGRESSION IN PATIENTS WITH CYSTIC FIBROSIS TREATED WITH IVACAFTOR: DATA FROM NATIONAL US AND UK REGISTRIES

Authors: Nataliya Volkovaa, Kristin Moya, Jennifer Evansb, Daniel Campbella, Simon Tiana, Christopher Simarda, Mark Higginsc, Michael W. Konstand, Gregory S. Sawickie, Alexander Elbertf, Susan C. Charmang, Bruce C. Marshallf, Diana […]

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ANTISENSE OLIGONUCLEOTIDE ELUFORSEN IS SAFE AND IMPROVES RESPIRATORY SYMPTOMS IN F508DEL CYSTIC FIBROSIS

Authors: Pavel Drevineka, Tacjana Presslerb, Marco Cipollic,d, Kris De Boecke, Carsten Schwarzf, Florilene Bouissetg,h, Marie Boffg, Noreen Henigi,j, Nicolas Paquette-Lamontagnei,k, Sonya Montgomeryi,l, Jaakko Perquini, Nigel Tomkinsoni, Wilhelmina den Hollanderi, and […]

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DISSOCATION OF SYSTEMIC AND MUCOSAL AUTOIMMUNITY IN CYSTIC FIBROSIS

Authors: Theprungsirikul, Ja, Skopelja-Gardner, Sa, Meagher, R. Ea, Clancy, J. Pb, Zemanick, E.Tc, Ashare, Aa,e, Rigby, W. F. Ca, d Affiliations: aDepartment of Microbiology and Immunology, Geisel School of Medicine […]

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SURVIVAL COMPARISON OF PATIENTS WITH CYSTIC FIBROSIS IN CANADA AND THE UNITED STATES: A POPULATION-BASED COHORT STUDY

Authors: Stephenson AL, Sykes J, Stanojevic S, Quon BS, Marshall BC, Petren K, Ostrenga J, Fink AK, Elbert A, Goss CH.   What was your research question? In 2011, the […]

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CHARACTERIZATION OF MISSING DATA IN CLINICAL REGISTRY STUDIES

Authors: Mendelsohn AB, Dreyer NA, Mattox PW, Su Z, Swenson A, Li R, Turner JR, Velentgas P.   What was your research question? The primary objective of this study was […]

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SEASON IS ASSOCIATED WITH PSEUDOMONAS AERUGINOSA ACQUISITION IN YOUNG CHILDREN WITH CYSTIC FIBROSIS

Authors: Psoter KJ, DeRoos AJ, Wakefield J, Mayer J, Rosenfeld M.   What was your research question? The objectives of this study were to: (i) evaluate the seasonal acquisition of […]

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OUTCOMES OF INFANTS WITH INDETERMINATE DIAGNOSIS DETECTED BY CYSTIC FIBROSIS NEWBORN SCREENING

Authors: Ren CL, Fink AK, Petren K, Borowitz DS, McColley SA, Sanders DB, Rosenfeld M, Marshall BC.   What was your research question? The goal of this study was to […]

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DISPARITIES IN MORTALITY OF HISPANIC CYSTIC FIBROSIS PATIENTS IN THE UNITED STATES: A NATIONAL AND REGIONAL COHORT STUDY

Authors: Rho J, Ahn C, Gao A, Sawicki G, Keller A, Jain R.   What was your research question? Is there a difference in survival rates in Hispanic people with […]

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EARLY LIFE GROWTH TRAJECTORIES IN CYSTIC FIBROSIS ARE ASSOCIATED WITH PULMONARY FUNCTION AT AGE 6 YEARS

Authors: Sanders DB, Fink A, Mayer-Hamblett N, Schechter M, Sawicki GS, Rosenfeld M, Flume PA, Morgan WJ.   What was your research question? Is there a link between the severity […]

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THE USE OF ANTIMICROBIAL SUSCEPTIBILITY TESTING IN PEDIATRIC CYSTIC FIBROSIS PULMONARY EXACERBATIONS

Authors: Jonathan D. Cogen1, Kathryn B. Whitlock2, Ronald L. Gibson1, Lucas R. Hoffman1, Donald R. VanDevanter3 Affiliations: 1Division of Pulmonary & Sleep Medicine, Department of Pediatrics, University of Washington, Seattle, […]

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PILOT STUDY TO TEST INHALED NITRIC OXIDE IN CYSTIC FIBROSIS PATIENTS WITH REFRACTORY MYCOBACTERIUM ABSCESSUS LUNG INFECTION

Authors: Lea Bentura,b, Michal Gura,b, Moshe Ashkenazic,g, Galit Livnat-Levanond, Mark Mizrahie, Asher Tale, Abdi Ghaffarie, Yuval Geffenf, Micha Aviramg, Ori Efratic,h Affiliations: a Pediatric Pulmonary Institute and CF Center, Ruth […]

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SWATH LABEL-FREE PROTEOMICS FOR CYSTIC FIBROSIS RESEARCH

Authors: Clarissa Braccia1,4, Valeria Tomati2, Emanuela Caci2, Nicoletta Pedemonte2 and Andrea Armirotti3 Affiliations: 1D3Pharmachemistry, Fondazione Istituto Italiano di Tecnologia, Via Morego 30, 16163 Genova, Italy; 2U.O.C. Genetica Medica, IRCCS Istituto […]

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ALIGNING PATIENTS’ NEEDS TOWARDS A COMPREHENSIVE CF RESEARCH PROGRAM

Authors: Jacquelien J. Noordhoek a, Vincent A.M. Gulmans a, Harry G.M. Heijerman b, Cornelis K. van der Ent b Affiliations: a Dutch Cystic Fibrosis Foundation (NCFS) b University Medical Center […]

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METHYLOMIC CORRELATES OF AUTOPHAGY ACTIVITY IN CYSTIC FIBROSIS

Authors: Kyle Caution1,8, Alexander Pan2,8, Kathrin Krause1,8, Asmaa Badr1,8, Kaitlin Hamilton1,8, Anup Vaidya1,8, Hawin Gosu1,8, Kylene Daily1,8, Shady Estfanous1,8, Mikhail A. Gavrilin5,8, Mark E. Drew1,8, Estelle Cormet-Boyaka3,8, Xi Chen3,8, David […]

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ASSESSMENT OF ENDOTHELIAL FUNCTION IS REPRODUCIBLE IN PATIENTS WITH CYSTIC FIBROSIS

Authors: Cassandra C. Derella1,2, Nichole Lee2, Reva Crandall3, Marsha Blackburn2, Jacob Looney2, Amie Mangieri2, Paula Rodriguez-Miguelez4, Matthew A. Tucker2, Ryan A. Harris1,2,5 Affiliations: 1Department of Physiology, Augusta University, Augusta GA […]

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THE EFFECTS OF CYCLED INHALED AZTREONAM ON THE CYSTIC FIBROSIS (CF) LUNG MICROBIOME

Authors: Alya A. Heirali1; Nicole Acosta1; Douglas G. Storey1,2; Matthew L. Workentine3; Ranjani Somayaji1,4; Isabelle Laforest-Lapointe5,6, Winnie Leung7; Bradley S. Quon8; Yves Berthiaum9; Harvey R. Rabin1,4; Barbara J. Waddell1, Laura […]

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DEPLETION OF BAFF CYTOKINE EXACERBATES INFECTION IN PSEUDOMONAS AERUGINOSA INFECTED MICE

Authors: Dušan Garić1,4, Shao Tao2,4,Eisha Ahmed2,4, Mina Youssef1,4, Cynthia Kanagaratham1, 3, 4,Juhi Shah5, Bruce Mazer2, 4 and Danuta Radzioch1, 4 Affiliations: 1Department of Human Genetics, McGill University, Montreal, Quebec, Canada; […]

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MEASURING RECOVERY IN HEALT-RELATED QUALITY OF LIFE DURING AND AFTER PULMONARY EXACERBATIONS IN PATIENTS WITH CYSTIC FIBROSIS

Authors: Patrick A. Flume,a Ellison D. Suthoff,b Mark Kosinski,c Gautham Marigowda,d Alexandra L. Quittnere Affiliations: aMedical University of South Carolina, 96 Jonathan Lucas Street, 812-CSB, Charleston, SC 29425 USA; bFormerly […]

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STUDY OF 109 ACHROMOBACTER SPP. ISOLATES FROM 9 FRENCH CF CENTRES REVEALS THE CIRCULATION OF A MULTIRESISTANT CLONE OF A. XYLOSOXIDANS BELONGING TO ST 137

Authors: Lucie Amoureux1,2, Juliette Sauge1, Benoit Sarret1, Matthieu Lhoumeau1, Audrey Bajard1, Jennifer Tetu1, Julien Bador1,2, Catherine Neuwirth1,2; MucoMicrobes group3 Affiliations: 1Department of Bacteriology, University Hospital of Dijon, BP 37013, 21070 […]

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PHYSICAL ACTIVITY AND ASSOCIATIONS WITH CLINICAL OUTCOME MEASURES IN ADULTS WITH CYSTIC FIBROSIS, A SYSTEMATIC REVIEW

Authors: James Shelley1, Lynne M Boddy1, Zoe R Knowles1, Claire E Stewart1, & Ellen A Dawson1. Affiliations: 1Research Institute for Sport and Exercise Sciences, Liverpool John Moores University, Liverpool, L3 […]

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CIRRHOSIS ASSOCIATED WITH DECREASED SURVIVAL AND A 10-YEAR LOWER MEDIAN AGE AT DEATH OF CYSTIC FIBROSIS PATIENTS IN THE NETHERLANDS

Authors: Pals, FH1, Verkade HJ1, Gulmans VAM2, de Koning BAE3, Koot BGP4, de Meij TGJ5, Hendriks DM6, Gierenz N7, Vreugdenhil ACE8, Houwen RHJ9, Bodewes FAJA1 Affiliations: 1The University Medical Center […]

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CT EVALUATION OF HYPERATTENUATING MUCUS TO DIAGNOSE ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS IN THE SPECIAL CONDITION OF CYSTIC FIBROSIS

Authors: John Refait1, Julie Macey1,2,3, Stephanie Bui1, Michaël Fayon1,2,3, Patrick Berger1,2,3, Laurence Delhaes1,2,3, François Laurent1,2,3, Gaël Dournes1,2,3 Affiliations: 1CHU de Bordeaux, Service d’Imagerie Thoracique et Cardiovasculaire, Service des Maladies Respiratoires, […]

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ASSESSMENT OF STABILITY AND FLUCTUATIONS OF CULTURED LOWER AIRWAY BACTERIAL COMMUNITIES IN PEOPLE WITH CYSTIC FIBROSIS

Authors: Laura J. Sherrarda,1, Gisli G. Einarssonb,1, Elinor Johnstonb, Katherine O’Neillb, Leanne McIlreaveyb, Stephanie J. McGratha, Deirdre F. Gilpina, Damian G. Downeyb,c, Alastair Reidc, Noel G. McElvaneyd, Richard C. Bouchere, […]

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3D PRINTING AND THE CYSTIC FIBROSIS LUNG

Authors: Alicia A Mirza, Terry E Robinson, Kyle Gifford, Haiwei Henry Guo Affiliations: Stanford University, Lucile Packard Children’s Hospital Stanford   What was your research question? Can 3D printing technology […]

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THE IMPACT OF CYSTIC FIBROSIS-RELATED DIABETES ON HEALTH-RELATED QUALITY OF LIFE

Authors: Eugenie Kwonga, Sameer Desaia, Louise Chongb, Kathleen Leea, Jie Zhenga, Pearce G. Wilcoxa,c, Bradley S. Quona,c Affiliations: aDivision of Respiratory Medicine, Department of Medicine, University of British Columbia, Vancouver, […]

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IMPACT OF A PROGRAM ENSURING CONSISTENT RESPONSE TO ACUTE DROPS IN LUNG FUNCTION IN CHILDREN WITH CYSTIC FIBROSIS

Authors: Michael S. Schechter, H. Joel Schmidt, Ron Williams, Robert Norton, Deanna Taylor, Andrea Molzhon Affiliations: Children’s Hospital of Richmond at Virginia Commonwealth University 1000 East Broad Street, Richmond, VA […]

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INTERLEUKIN-1 IS ASSOCIATED WITH INFLAMMATION AND STRUCTURAL LUNG DISEASE IN YOUNG CHILDREN WITH CYSTIC FIBROSIS

Authors: Samuel T. Montgomery1, A. Susanne Dittrich2,3, Luke W. Garratt4, Lidija Turkovic4, Dario L. Frey2,3, Stephen M. Stick1,4,5,6, Marcus A. Mall2,7,8, Anthony Kicic1,4,5,6,9, & AREST CF1,5,10,11. Affiliations: 1School of Paediatrics […]

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AGTR2 ABSENCE OR ANTAGONISM PREVENTS CYSTIC FIBROSIS PULMONARY MANIFESTATIONS

Authors: Rebecca J. Darrah1,2, Frank J. Jacono3,4, Neha Joshi5, Anna L. Mitchell2, Abdus Sattar6, Cara K. Campanaro3, Paul Litman1, Jennifer Frey2, David E. Nethery4, Eric S. Barbato1, Craig A. Hodges2,5, […]

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ELECTROCHEMICAL MEASUREMENT OF MEMBRANE CHOLESTEROL CORRELATES WITH CFTR FUNCTION AND IS HDAC6-DEPENDENT

Authors: Binyu Lu1, Li Li Molly Schneider2, Craig A. Hodges2, James A. Burgess3, and Thomas J. Kelley1,2 Affiliations: 1Department of Chemistry, Case Western Reserve University 2Department of Pediatrics, Case Western […]

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ESTABLISHMENT OF A F508DEL-CF PROMYELOCYTIC CELL LINE FOR CYSTIC FIBROSIS RESEARCH AND DRUG SCREENING

Authors: Scott Jennings, Hong Pong Ng and Guoshun Wang Affiliations: Department of Microbiology, Immunology and Parasitology, Louisiana State University Health Sciences Center, New Orleans, LA 70122, USA   What was […]

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A SPECIALIZED METHOD OF SPUTUM COLLECTION AND PROCESSING FOR THERAPEUTIC INTERVENTIONS IN CYSTIC FIBROSIS

Authors: McElvaney OJ, Gunaratnam C, Reeves EP, McElvaney NG. Affiliations: Irish Centre for Genetic Lung Disease, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin, Ireland.   What was your […]

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EMERGENCE OF LIVESTOCK-ASSOCIATED MRSA ISOLATED FROM CYSTIC FIBROSIS PATIENTS: RESULTS OF A BELGIAN NATIONAL SURVEY

Authors: M. Dodémont1, M. A. Argudín1, J. Willekens2, E. Vanderhelst2, D. Pierard3, V.Y. Miendje Deyi4, L. Hanssens5, H. Franckx6, P. Schelstraete7, I. Leroux-Roels8, C. Nonhoff1, A. Deplano1, C. Knoop9, A. […]

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PREVALENCE OF HYPOGLYCEMIA DURING ORAL GLUCOSE TOLERANCE TESTING IN ADULTS WITH CYSTIC FIBROSIS AND RISK OF DEVELOPING CYSTIC FIBROSIS-RELATED DIABETES

Authors: Lisa A Mannik1, Kristy A Chang1, Pascalyn QK Annoh1, Jenna Sykes1, Julie Gilmour1, Ronalee Robert1, Anne L Stephenson1,2,3 Affiliations: 1Department of Respirology, St. Michael’s Hospital, 30 Bond Street, Toronto, […]

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RESCUE OF CFTR NBD2 MUTANTS N1303K AND S1235R IS INFLUENCED BY THE FUNCTIONING OF THE AUTOPHAGOSOME

Authors: Qiangni Liu, Inna Sabirzhanova, Murali K. Yanda, Emily A.S. Bergbower, Clément Boinot, William B. Guggino, Liudmila Cebotaru Affiliations: Departments of Medicine and Physiology, Johns Hopkins University, Baltimore, MD, USA […]

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