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

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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MEASURED FETAL AND NEONATAL EXPOSURE TO LUMACAFTOR AND IVACAFTOR DURING PREGNANCY AND WHILE BREASTFEEDING

Authors: Aaron T Trimble1, Cameron McKinzie2, Mary Terrell3, Elizabeth Stringer4, Charles Esther Jr3 Affiliations: 1Department of Medicine, Division of Pulmonary & Critical Care Medicine, 2Eshelman School of Pharmacy, 3Department of […]

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CONTINUOUS GLUCOSE MONITORING ABNORMALITIES IN CYSTIC FIBROSIS YOUTH CORRELATES WITH PULMONARY FUNCTION DECLINE

Authors: Christine L. Chan1, Timothy Vigers1, Laura Pyle1,2, Philip S. Zeitler1, Scott D. Sagel3, Kristen J. Nadeau1 Affiliations: 1Department of Pediatrics, Division of Pediatric Endocrinology, Children’s Hospital Colorado and University […]

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RESPIRATORY RATE IN INFANTS WITH CYSTIC FIBROSIS THROUGHOUT THE FIRST YEAR OF LIFE AND ASSOCIATION WITH LUNG CLEARANCE INDEX MEASURED SHORTLY AFTER BIRTH

Authors: Insa Korten1,2,3, Elisabeth Kieninger1, Sophie Yammine1, Giulia Cangiano4, Sylvia Nyilas1,2, Pinelopi Anagnostopoulou1, Florian Singer1, Claudia E. Kuehni5, Nicolas Regamey1,6, Urs Frey3, Carmen Casaulta1, Ben D. Spycher1,5, Philipp Latzin1 on […]

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STRATEGIES FOR NEWBORN SCREENING FOR CYSTIC FIBROSIS: A SYSTEMATIC REVIEW OF HEALTH ECONOMIC EVALUATIONS

Authors: Masja Schmidta, Amber Werbrouckb,d, Nick Verhaeghea,b, Elke De Wachterc, Steven Simoensd, Lieven Annemansb, Koen Putmana Affiliations: a Interuniversity Center for Health Economics Research, Vrije Universiteit Brussel, Brussels, Belgium b […]

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CONTINUOUS GLUCOSE MONITORING IN YOUTH WITH CYSTIC FIBROSIS TREATED WITH LUMACAFTOR-IVACAFTOR

Authors: Angel Li1, Tim Vigers2, Laura Pyle3, Edith Zemanick4, Kristen Nadeau2, Scott D. Sagel4, Christine L. Chan2 Affiliations: 1University of Colorado School of Medicine, University of Colorado Anschutz Medical Campus, […]

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CORRESPONDENCE BETWEEN SYMPTOMS AND PREFERENCE-BASED HEALTH STATUS MEASURES IN THE STOP STUDY

Authors: Laura S. Golda, Donald L. Patrickb, Ryan N. Hansenc, Valeria Beckettd, Christopher H. Gosse and Larry Kesslerb Affiliations: aDepartment of Radiology, University of Washington, Seattle, Washington, United States bDepartment […]

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ANTISENSE OLIGONUCLEOTIDE TARGETING OF MRNAS ENCODING ENAC SUBUNITS ALPHA, BETA, AND GAMMA IMPROVES CYSTIC FIBROSIS-LIKE DISEASE IN MICE

Authors: Chenguang Zhao, Jeff Crosby, Tinghong Lv, Dong Bai, Brett P. Monia, and Shuling Guo Affiliations: Ionis Pharmaceuticals, 2855 Gazelle Court, Carlsbad, CA 92010 USA   What was your research […]

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RESISTIN IS ELEVATED IN CYSTIC FIBROSIS SPUTUM AND CORRELATES NEGATIVELY WITH LUNG FUNCTION

Authors: Osric A. Forrest1,2, Daniel M. Chopyk3, Yael Gernez4, Milton R. Brown1,2, Carol K. Conrad4, 6, Richard B. Moss4, Vin Tangpricha3,5, Limin Peng6, Rabindra Tirouvanziam1,2 Affiliations: 1Department of Pediatrics, Emory […]

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R560S: A CLASS II CFTR MUTATION THAT IS NOT RESCUED BY CURRENT MODULATORS

Authors: Nikhil T Awatade1, Sofia Ramalho1, Iris AL Silva1, Verónica Felício1, Hugo M Botelho1, Eyleen de Poel2, Annelotte Vonk2, Jeffrey M Beekman2, Carlos M Farinha1, Margarida D Amaral1 Affiliations: 1University […]

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THE Q359K/T360K MUTATION CAUSES CYSTIC FIBROSIS IN GEORGIAN JEWS

Authors: Mei-Zahav M1,2, Stafler P1,2, Senderowitz H3, Bentur L4,5, Livnat G5,6, Shteinberg M5,6, Orenstein N2,6, Bazak L2,7, Prais D1,2, Levine H1,2, Gur M4, Khazanov N3, Simhaev L3, Eliyahu H8, Cohen […]

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ERADICATION OF PERSISTENT METHICILLIN-RESISTANT STAPHYLOCOCCUS AUREUS INFECTION IN CYSTIC FIBROSIS

Authors: Rebecca Dezube1*, Mark T. Jennings1*, Mary Rykiel1, Marie Diener-West2, Michael P. Boyle1,3, James F. Chmiel4, Elliott C. Dasenbrook5 *Co-First Authors  Affiliations: 1Division of Pulmonary and Critical Care Medicine, The […]

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BREATHLESSNESS CATASTROPHIZING RELATES TO POORER QUALITY OF LIFE IN ADULTS WITH CYSTIC FIBROSIS

Authors: Danijela Maras1,2 MA, Louise Balfour1,2,3,4 PhD, C.Psych, Giorgio A. Tasca1,2,4  PhD, C.Psych, Ena Gaudet2 RN, Shawn D. Aaron2,4,5 MD, FRCPC, William D. Cameron2,4,5 MD, FRCPC, Smita Pakhale2,4,5 MD, FRCPC, […]

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ASSESSING DIFFERENCES IN MORTALITY RATES AND RISK FACTORS BETWEEN HISPANIC AND NON-HISPANIC PATIENTS WITH CYSTIC FIBROSIS IN CALIFORNIA

Article citation: Buu M, Sanders L, Mayo J, Milla C, Wise P. Assessing differences in mortality rates and risk factors between Hispanic and non-Hispanic patients with cystic fibrosis in California. […]

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GENETIC MODIFIERS OF CYSTIC FIBROSIS-RELATED DIABETES

Article citation: Blackman SM, Commander CW, Watson C, Arcara KM, Strug LJ, Stonebraker JR, Wright FA, Rommens JM, Sun L, Pace RG, Norris SA, Durie PR, Drumm ML, Knowles MR, […]

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SHORT-TERM AND LONG-TERM RESPONSE TO PULMONARY EXACERBATION TREATMENT IN CYSTIC FIBROSIS

Authors: Heltshe SL, Goss CH, Thompson V, Sagel SD, Sanders DB, Marshall BC, Flume PA   What was your research question? We looked at differences in pulmonary exacerbation care in […]

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RATE AND PREDICTORS OF PRESCRIPTION OF LUMACAFTOR-IVACAFTOR IN THE 18 MONTHS FOLLOWING APPROVAL IN THE UNITED STATES

Authors: Gregory S. Sawicki1, Aliza K. Fink2, Michael S. Schechter3, Deena R. Loeffler2, Nicole Mayer-Hamblett4  Affiliations: 1Division of Respiratory Diseases, Boston Children’s Hospital, Boston, Massachusetts 2Cystic Fibrosis Foundation, Bethesda MD […]

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LUMACAFTOR/IVACAFTOR REDUCES PULMONARY EXACERBATIONS IN PATIENTS IRRESPECTIVE OF INITIAL CHANGES IN FEV1

Authors: Susanna A. McColley,a Michael W. Konstan,b Bonnie W. Ramsey,c J. Stuart Elborn,d Michael P. Boyle,e Claire E. Wainwright,f David Waltz,g Montserrat Vera-Llonch,g Gautham Marigowda,g John G. Jiang,g Jaime L. […]

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MENTAL HEALTH SCREENING IN CYSTIC FIBROSIS CENTERS ACROSS EUROPE

Authors: J. Abbott1, T. Havermans2, S. Jarvholm3, E. Landau4, Y. Prins5, U. Smrekar6, D. Staab7, L. Verity1, M. Verkleij8 on behalf of the ECFS Mental Health Working Group. Affiliations: 1University […]

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HUMAN EPIDIDYMIS PROTEIN 4 (HE4) LEVELS INVERSELY CORRELATE WITH LUNG FUNCTION IMPROVEMENT (DELTA FEV1) IN CYSTIC FIBROSIS PATIENTS RECEIVING IVACAFTOR TREATMENT

Authors: Béla Nagy Jr1, Zsolt Bene2, Zsolt Fejes1, Sonya L. Heltshe3, David Reid4, Nicola J. Ronan5, 3, Yvonne McCarthy5, Daniel Smith4, Attila Nagy6, Elizabeth Joseloff7, György Balla2,8, János Kappelmayer1, Milan […]

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INVESTIGATING THE EFFECTS OF LONG-TERM DORNASE ALFA USE ON LUNG FUNCTION USING REGISTRY DATA

Authors: Newsome SJ1, Daniel RM2, Carr SB3, Bilton D4 and Keogh RH1 Affiliations: 1Department of Medical Statistics, London School of Hygiene & Tropical Medicine, London, UK 2Division of Population Medicine, […]

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HIGHER INTERLEUKIN-7 SERUM CONCENTRATIONS IN PATIENTS WITH CYSTIC FIBROSIS CORRELATES WITH IMPAIRED LUNG FUNCTION

Authors: Julia Seyfarth1, Sutharsan Sivagurunathan2, Sarah Ricken2, Gerhard Weinreich², Laura Olbrich1, Christian Taube², Ertan Mayatepek1, Dirk Schramm1, Marc Jacobsen1 Affiliations: 1 Department of General Pediatrics, Neonatology, and Pediatric Cardiology, University […]

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ITALIAN AND NORTH AMERICAN DIETARY INTAKE AFTER IVACAFTOR TREATMENT FOR CYSTIC FIBROSIS GATING MUTATIONS

Authors: Nina Sainath a, Joan Schall a, Chiara Bertolaso b, Carolyn McAnlis a, Virginia A. Stallings a,c Affiliations: aDivision of Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, […]

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PHENOTYPIC SPECTRUM OF PATIENTS WITH CYSTIC FIBROSIS AND CYSTIC FIBROSIS-RELATED DISEASE CARRYING P.ARG117HIS

Authors: Katherine Keenan1; Annie Dupuis2,3; Katherine Griffin4; Carlo Castellani5,6; Elizabeth Tullis4,7; Tanja Gonska1,8. Affiliations: 1Physiology and Experimental Medicine, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada 2Clinical Research […]

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PREVALENCE, GEOGRAPHIC RISK FACTORS, AND DEVELOPMENT OF A STANDARDIZED PROTOCOL FOR FUNGAL ISOLATION IN CYSTIC FIBROSIS: RESULTS FROM THE INTERNATIONAL PROSPECTIVE STUDY “MFIP”

Authors: Laurence Delhaes1,2, Kada Touati1, Odile Faure-Cognet3, Muriel Cornet3, Françoise Botterel4, Eric DannaouiI5, Florent Morio6, Patrice Lepape6, Fréderic Grenouillet7, Loic Favennec8, Solène Le Gal9, Gilles Nevez9, Alain Duhamel1, Andrew Borman10, […]

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NEWBORN BLOOD SPOT SCREENING FOR CYSTIC FIBROSIS WITH A FOUR-STEP SCREENING STRATEGY IN THE NETHERLANDS

Authors: Jeannette E. Dankert-Roelse1, Marelle J. Bouva2, Bernadette S. Jakobs3, Hettie M. Janssens4, Karin M. de Winter-de Groot5, Yvonne Schönbeck6, Johan J.P. Gille7, Vincent A. M. Gulmans8, Rendelien K. Verschoof-Puite9, […]

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FAILURE TO CONCEIVE IN WOMEN WITH CF IS ASSOCIATED WITH PANCREATIC INSUFFICIENCY AND ADVANCING AGE

Authors: Michal Shteinberg1,2,3, Adi Ben Lulu1,2,3, Damian G Downey14, Zeev Blumenfeld3,4, Christine Rousset-Jablonski13, Marie Percival13, Alessandra Colombo15, Nili Stein5, Galit Livnat2,3, Michal Gur3,6, Lea Bentur3,6, Huda Mussaffi7,8, Hannah Blau7,8, Ifat […]

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THE MAIN MECHANISM ASSOCIATED WITH PROGRESSION OF GLUCOSE INTOLERANCE IN OLDER PATIENTS WITH CYSTIC FIBROSIS IS INSULIN RESISTANCE AND NOT REDUCED INSULIN SECRETION CAPACITY

Authors: Johann Colomba1,2, Valérie Boudreau1,2, Catherine Lehoux-Dubois1,2, Katherine Desjardins1, Adèle Coriati1, François Tremblay3,4, Rémi Rabasa-Lhoret1-4 Affiliations: 1Institut de Recherches Cliniques de Montréal, 110 avenue des Pins Ouest, Montréal H2W 1R7, […]

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THE CYSTIC FIBROSIS FOUNDATION PATIENT REGISTRY. DESIGN AND METHODS OF A NATIONAL OBSERVATIONAL DISEASE REGISTRY

Citation: Knapp EA, Fink AK, Goss CH, Sewall A, Ostrenga J, Dowd C, Elbert A, Petren KM, Marshall BC. Annals of the American Thoracic Society. 2016 Jul;13(7):1173-9.   What was […]

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GENOME-WIDE ASSOCIATION META-ANALYSIS IDENTIFIES FIVE MODIFIER LOCI OF LUNG DISEASE SEVERITY IN CYSTIC FIBROSIS

Citation: Corvol H, Blackman SM, Boëlle PY, et al. Nat Commun. 2015 Sep 29; 6:8382   What was your research question? What are non-cystic fibrosis transmembrane conductance regulator (CFTR) genes […]

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GENDER DIFFERENCES IN OUTCOMES OF PATIENTS WITH CYSTIC FIBROSIS

Citation: Harness-Brumley CL, Elliott AC, Rosenbluth DB, Raghavan D, Jain R.  Journal of Women’s Health. 2014 Dec; 23 (12): 1012-1020. PMID: 25495366   What was your research question? Does gender […]

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THE DYNAMICS OF DISEASE PROGRESSION IN CYSTIC FIBROSIS

Citation: Adler FR, Liou TG. PLOS ONE. 2016;11(6):e0156752.   What was your research question? How do lung function (FEV1%) and lung infections in CF interact and affect patient health and […]

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APPLICATION OF MULTIPLE EVENT ANALYSIS AS AN ALTERNATIVE APPROACH TO STUDYING PULMONARY EXACERBATIONS AS AN OUTCOME MEASURE

Authors: Elizabeth Juarez-Colunga1,2, Rosenfeld Margaret3, Edith T. Zemanick4, Brandie Wagner1,4 Affiliations: 1Department of Biostatistics and Informatics, University of Colorado School of Public Health, 2Adult and Child Consortium for Health Outcomes […]

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