March 2025
Title: How sweat chloride levels relate to health improvements with CFTR modulators
Authors Edith T Zemanick, Bonnie Ramsey, Dorota Sands, Edward F McKone, Isabelle Fajac, Jennifer L Taylor-Cousar, Marcus A Mall, Michael W Konstan, Nitin Nair, Jiaqiang Zhu, Emilio Arteaga-Solis, Fredrick Van Goor, Lisa McGarry, Valentin Prieto-Centurion,, Patrick R Sosnay, Carmen Bozic, David Waltz, Nicole Mayer-Hamblett
References J Cyst Fibros. 2025 Mar;24(2):246-254. doi: 10.1016/j.jcf.2024.12.006. Epub 2025 Jan 3.
Keywords CFTR function, CFTR modulators, clinical outcomes, sweat chloride
Title: AI-Powered Home Monitoring for Cystic Fibrosis Flare-Ups in Kids and Adults
Authors Henryk Mazurek, Andrzej Emeryk, Kamil Janeczek, Eric Derom, Barbara Kuźnar-Kamińska, Tomasz Grzywalski, Adam Biniakowski, Krzysztof Szarzyński, Anna Pastusiak, Dominika Kaminiarczyk-Pyzałka, Dick Botteldooren, Honorata Hafke-Dys, Jędrzej Kociński
References J Cyst Fibros. 2025 Mar;24(2):390-397. doi: 10.1016/j.jcf.2025.02.011. Epub 2025 Feb 18.
Keywords AI-aided CF management, Cystic fibrosis exacerbation, Cystic fibrosis monitoring, Remote management of CF, hOme healthcare
Title: Mental health is related to adherence through self-efficacy and perceived barriers
Authors Robin S Everhart, Milene T Saavedra, Christine R Ford, Sidney L Gibson, Felicia Reid, Emily F Muther, Christina L Duncan, Rachel Cravens, Angela Green, Kristin A Riekert
References J Cyst Fibros. 2025 Mar;24(2):334-340. doi: 10.1016/j.jcf.2025.02.016. Epub 2025 Feb 22.
Keywords Adherence, Barriers, mental health, Self-efficacy
March 2025
Title: Treating cystic fibrosis before birth: What we learned from an unsuccessful case
Authors M Destoop, C Brantner, E B Wilms, Shaj Tytgat, B Peels, R van der Graaf, T B Y Liem, K M de Winter-de Groot
References J Cyst Fibros. 2025 Mar 20:S1569-1993(25)00074-8. doi: 10.1016/j.jcf.2025.03.006.
Keywords CFTR modulator therapy, In utero exposure, meconium ileus, pregnancy
March 2025
Title: Drug allergy to CFTR modulators – an international survey
Authors Ruth M Urbantat, Laura Behan, Sebastian Wisniewski, Joshua Gardner, Mirjam Stahl, Marcus A Mall, Daniel Peckham, Dean J Naisbitt, Jobst F Roehmel
References J Cyst Fibros. 2025 Mar 18:S1569-1993(25)00071-2. doi: 10.1016/j.jcf.2025.03.003.
Keywords CFTR modulator withdrawal, Drug allergy, Drug allergy to CFTR modulator therapy, Immunogenic adverse events, Real-world data
March 2025
Title: Tracking the journey of elexacaftor-tezacaftor-ivacaftor in the bodies of children with cystic fibrosis
Authors Steffie E M Vonk, Suzanne W J Terheggen-Lagro, Eric G Haarman, S Hashimoto, Anke H Maitland-van der Zee, Ron A A Mathôt, E Marleen Kemper; Amsterdam Mucociliary Clearance Disease (AMCD) research group
References J Cyst Fibros. 2025 Mar 14:S1569-1993(25)00076-1. doi: 10.1016/j.jcf.2025.03.008.
Keywords CFTR modulators, Elexacaftor-tezacaftor-ivacaftor, Pediatrics, Population
March 2025
Title: Lower inflammation is related to greater energy levels in adults with cystic fibrosis on elexacaftor/tezacaftor/ivacaftor
Authors Jacob Gravelle, Sameer Desai, Kang Dong, Bradley S Quon
References J Cyst Fibros. 2025 Mar 11:S1569-1993(25)00073-6. doi: 10.1016/j.jcf.2025.03.004.
Keywords Elexacaftor, Fatigue, Systemic inflammation, Vitality
March 2025
Title: Do people with cystic fibrosis experience side effects from Elexacaftor/Tezacaftor/Ivacaftor because of high drug levels?
Authors Syendon B C Baromeo, Renske van der Meer, Richard C J M van Rossen, Erik B Wilms
References J Cyst Fibros. 2025 Mar 10:S1569-1993(25)00061-X. doi: 10.1016/j.jcf.2025.02.013.
Keywords adverse events, Dose reduction, Drug concentration levels, Elexacaftor/tezacaftor/ivacaftor, Pharmacology
March 2025
Title: CFTR modulators and pregnancy outcomes: early findings from a nationwide cohort study
Authors Laurent Chouchana, Mathis Collier, Clémence Martin, Pierre-Régis Burgel, Jean-Marc Treluyer
References J Cyst Fibros. 2025 Mar 8:S1569-1993(25)00070-0. doi: 10.1016/j.jcf.2025.03.002.
Keywords Birth defect, CFTR modulators, Drug safety, pregnancy, fetus
February 2025
Title: How people with CF access CFTR modulators in countries where coverage is not provided by health systems
Authors Jonathan Guo, Grace Hennessy, Benedict Young, Andrew Hill
References J Cyst Fibros. 2025 Feb 21:S1569-1993(25)00057-8. doi: 10.1016/j.jcf.2025.02.010.
Keywords CFTR modulator, ETI, Elexacaftor/tezacaftor/ivacaftor, Kaftrio, Trikafta
February 2025
Title: Reassurance in Pregnancy and Cystic Fibrosis
Authors Paul K Mohabir, Fatma Gunturkun, Jennifer Cannon, Yaowei Deng, Ariadna Garcia, Eahsan Shahriary, Alicia Mirza
References J Cyst Fibros. 2025 Feb 20:S1569-1993(25)00053-0. doi: 10.1016/j.jcf.2025.02.004.
Keywords Lung function, Outcomes, pregnancy, Survival
February 2025
Title: Response to CFTR modulators of the rare CFTR variants L383S, I507del, L1065P and R1066H
Authors Jessica Conti, Dora Angyal, Karina Kleinfelder, Roberta Valeria Latorre, Martina Calicchia, Alessia Farinazzo, Luca Rodella, Francesco Tomba, Arianna Massella, Luca Frulloni, Giovanni Taccetti, Vito Terlizzi, Anny Leung, Tessa A Groeneweg,, Marcel J C Bijvelds, Paola Melotti, Claudio Sorio
References J Cyst Fibros. 2025 Feb 19:S1569-1993(25)00059-1. doi: 10.1016/j.jcf.2025.02.008.
Keywords CFTR variants, Bicarbonate secretion, Personalized medicine, Rectal organoids, Theratyping, Ussing chamber
February 2025
Title: Viral infection causes an extreme immune response and loss of cilia function in airway cells of people with CF
Authors Jennifer A Bartlett, Eric D Huntemann, Sateesh Krishnamurthy, Stacey M Hartwig, Alvin Pewa, Andrew L Thurman, Michael S Chimenti, Eric B Taylor, Steven M Varga, Paul B McCray Jr Antiviral, Cilia, Interferon, RNAseq, Viral infection
References J Cyst Fibros. 2025 Feb 15:S1569-1993(25)00055-4. doi: 10.1016/j.jcf.2025.02.003.
Keywords Antiviral, Cilia, Interferon, RNAseq, Viral infection
February 2025
Title: Changes in how immune cells and lung cells communicate in people with cystic fibrosis
Authors Marijn Berg, Lisette Krabbendam, Esmee K van der Ploeg, Menno van Nimwegen, Tjeerd van der Veer, Martin Banchero, Orestes A Carpaij, Remco Hoogenboezem, Maarten van den Berge, Eric Bindels, Joachim G J V Aerts, Antoine Collin, Pascal Barbry,, Lieke S Kamphuis, Rudi W Hendriks, Martijn C Nawijn, Ralph Stadhouders
References J Cyst Fibros. 2025 Feb 12:S1569-1993(25)00049-9. doi: 10.1016/j.jcf.2025.01.016.
Keywords Bronchial biopsies, Single-cell RNA-sequencing, structural-immune cell crosstalk
February 2025
Title: The relationship between ivacaftor and lung function variability
Authors Rhonda D Szczesniak, Eleni-Rosalina Andrinopoulou, Hancheng Li, Raksha Jain, Nicole Mayer-Hamblett, Josh Ostrenga, Anushka K Palipana, David J Pasta, Margaret Rosenfeld, Jonathan Todd, Elizabeth A Cromwell, Wayne J Morgan
References J Cyst Fibros. 2025 Feb 9:S1569-1993(25)00047-5. doi: 10.1016/j.jcf.2025.01.014.
Keywords FEV1, Highly effective modulator, Longitudinal, mutation, patient registry, Stability
January 2025
Title: Tracking the effects of ETI treatment on pancreas in an infant over 1.7 years using stool tests
Authors Dorothea Appelt, Teresa Fuchs, Johannes Eder, Katharina Niedermayr, Anja Siedl, Helmut Ellemunter
References J Cyst Fibros. 2025 Jan 28:S1569-1993(25)00012-8. doi: 10.1016/j.jcf.2025.01.012
Keywords ETI, Pancreatic sufficiency, Faecal elastase, pancreatic insufficiency
Title: Male Sexual and Reproductive Health in Cystic Fibrosis: A Concept Mapping Study
Authors Kelly A Prangley, Olivia M Stransky, Jessica G Burke, Sigrid L Ladores, Kara S Hughan, Gregory S Sawicki, Traci M Kazmerski
References J Cyst Fibros. 2025 Jan 28:S1569-1993(25)00011-6. doi: 10.1016/j.jcf.2025.01.011.
Keywords Concept mapping, Sexual and reproductive health
January 2025
Title: Pain in adults with cystic fibrosis – are we painfully unaware?
Authors Anastasia Ward, Ramil Mauleon, Gretel Davidson, Chee Y Ooi, Nedeljka Rosić
References J Cyst Fibros. 2025 Jan 25:S1569-1993(25)00009-8. doi: 10.1016/j.jcf.2025.01.009.
Keywords Burden of disease, CFTR, Measurement, Online survey, Prevalence of pain, Symptom perception
January 2025
Title: Changes in eye development in baby rats exposed to ETI when in the womb
Authors Yimin Zhu, Mengliang Wu, Danni Li, Mark Habgood, Holly R Chinnery, Elena K Schneider-Futschik
References J Cyst Fibros. 2025 Jan;24(1):21-25. doi: 10.1016/j.jcf.2024.11.014. Epub 2024 Dec 5
Keywords ETI, Eye development, Ocular development, pregnancy, Trikafta
January 2025
Title: Kaftrio® quickly modifies the composition of breath in children with CF.
Authors Emmanuelle Bardin, Nicolas Hunzinger, Elodie Lamy, Camille Roquencourt, Bingqing Zhou, Yasmine Tabache, Laurence Le Clainche, Natascha Remus, Charlotte Roy, Philippe Devillier, Thao Nguyen-Khoa, Frédérique Chedevergne, Clément Pontoizeau, Mairead Kelly,, Stanislas Grassin Delyle, Isabelle Sermet-Gaudelus
References J Cyst Fibros. 2025 Jan 21:S1569-1993(25)00004-9. doi: 10.1016/j.jcf.2025.01.004.
Keywords CFTR modulators, Lung function, Volatile organic compounds, Biomarkers, breathomics
January 2025
Title: Racial Disparities in Lung Transplantation for People with Cystic
Authors Jessica M Ruck, Shi Nan Feng, Alexandra H Toporek, Pali D Shah, Erin Tallarico, Noah Lechtzin, Allan B Massie, Dorry L Segev, Errol L Bush, Christian A Merlo
References J Cyst Fibros. 2025 Jan 16:S1569-1993(25)00008-6. doi: 10.1016/j.jcf.2025.01.006.
Keywords Epidemiology, Highly effective modulator therapy, Lung transplantation, Race
January 2025
Title: Improvements in tummy and bowel symptoms after short and longer term Elexacaftor / Tezacaftor / Ivacaftor therapy (ETI)
Authors L.R. Caley , L. Gillgrass , C. Zagoya, H. Saumtally, F. Duckstein, H. White, J.G. Mainz, D.G. Peckham
References J Cyst Fibros. 2025 Jan 14:S1569-1993(25)00010-4. doi: 10.1016/j.jcf.2025.01.010.
Keywords CFAbd-Score, Elexacaftor/tezacaftor/ivacaftor (ETI) therapy, Gastrointestinal symptoms, abdominal
January 2025
Title: Understanding How a Simple Lung Test Can Guide Care for Children with Cystic Fibrosis
Authors Lucy Perrem, Stephanie Jeanneret-Manning, Stephanie D Davis, Margaret Rosenfeld, Todd Edwards, Sanja Stanojevic, Felix Ratjen
References J Cyst Fibros. 2025 Jan 11:S1569-1993(24)01852-6. doi: 10.1016/j.jcf.2024.12.001.
Keywords Clinical utilty, LCI, Lung function, MBW, Maintenance therapy, Modulators, Withdrawal
January 2025
Title: Clinical characteristics of people with CF experiencing food insecurity
Authors Heather Boas, Jesse Y Hsu, Allen Koshy, Semret Seyoum, Marsha Regenstein, Gina Hong, Olivia Dieni, Anne Willis, Clement L Ren
References J Cyst Fibros. 2025 Jan 9:S1569-1993(24)01855-1. doi: 10.1016/j.jcf.2024.12.005
Keywords Epidemiology, Health equity, Health-related social need, Nutrition, Pulmonary exacerbation, Social determinants of health
January 2025
Title: Understanding the Connection Between the Lungs and Kidneys in Cystic Fibrosis
Authors Grace M Rosner, Himanshu B Goswami, Katherine Sessions, Lindsay K Mendyka, Brenna Kerin, Irma Vlasac, Diane Mellinger, Lorraine Gwilt, Thomas H Hampton, Martha Graber, Alix Ashare, William T Harris, Brock Christensen, Bruce A Stanton,, Agnieszka Swiatecka-Urban, Sladjana Skopelja-Gardner
References J Cyst Fibros. 2025 Jan 2:S1569-1993(24)01858-7. doi: 10.1016/j.jcf.2024.12.007.
Keywords Kidney injury, Lung-kidney, Neutrophils, Urine analysis
December 2024
Title: What do people with CF think about their health since starting ETI?
Authors Robin S Everhart, Emma McWilliams, Jill Maggs, Gregory S Sawicki, Takeera Sconiers, Kyle Smith, Dana Yablon, Jennifer Butcher, Michelle Prickett, Callie Bacon, Andrea Goodman, Alex H Gifford, Nicole Mayer-Hamblett, David P Nichols, Kristin A Riekert
References J Cyst Fibros. 2024 Nov 21:S1569-1993(24)01811-3. doi: 10.1016/j.jcf.2024.11.003.
Keywords Health, Modulator therapy, Qualitative
December 2024
Title: Use of Elexacaftor/Tezacaftor/Ivacaftor in Pregnant Mothers without CF to Treat Meconium Ileus in Fetuses with Cystic Fibrosis
Authors Angela Metcalf, Stacey L Martiniano, Scott D Sagel, Michael V Zaretsky, Edith T Zemanick, Jordana E Hoppe
References J Cyst Fibros. 2024 Dec 6:S1569-1993(24)01843-5. doi: 10.1016/j.jcf.2024.11.011.
Keywords CFTR modulator, Carrier, Elexacaftor/texacaftor/ivacaftor, Fetal, meconium ileus, pregnancy, Prenatal
December 2024
Title: Elexacaftor/tezacaftor/ivacaftor can be effective for people with cystic fibrosis with the 3849 gene variant
Authors Moshe Heching, Michal Shteinberg, Inbal Golan-Tripto, Galit Livnat-Levanon, Karin Yaacoby-Bianu, Liora Boehm Cohen, Guy Hazan, Liora Slomianski, Dario Prais, Huda Mussaffi, Joel Weinberg, Mordechai R Kramer
References J Cyst Fibros. 2024 Dec 5:S1569-1993(24)01845-9. doi: 10.1016/j.jcf.2024.11.010.
Keywords 3849+10kbC->T variant, CFTR modulators, Elexacaftor, Residual function variant
December 2024
Title: Daily changes in the amount of Pseudomonas in CF sputum
Authors Lisa A Carmody, Lindsay J Caverly, Linda M Kalikin, Christina S Thornton, Richard H Simon, Donald R VanDevanter, John J LiPuma
References J Cyst Fibros. 2024 Dec 2:S1569-1993(24)01848-4. doi: 10.1016/j.jcf.2024.11.013.
Keywords Bacterial density in sputum, Pseudomonas airway infection, Response biomarker
November 2024
Title: The relation between the physical activity behaviour of youth with Cystic Fibrosis and their parents
Authors Manon Kinaupenne, Stephanie Van Biervliet, Kim Van Hoorenbeeck, Heidi Schaballie, Kristof Vandekerckhove, Marieke De Craemer, Heleen Demeyer
References J Cyst Fibros. 2024 Nov 28:S1569-1993(24)01815-0. doi: 10.1016/j.jcf.2024.11.007.
Keywords Accelerometer, Parental behaviour, Physical activity, Youth
November 2024
Title: A review of research about Trikafta and mental health: a report from Cystic Fibrosis Foundation’s Prioritizing Research in Mental Health Working Group
Authors Bathgate CJ, Fedele DA, Tillman EM, He J, Everhart RS, Reznikov LR, Liu FF, Kirby K, Raffensperger K, Traver K, Riekert KA, Powers SW, Georgiopoulos AM
References J Cyst Fibros. 2024 Nov 26:S1569-1993(24)01814-9. doi: 10.1016/j.jcf.2024.11.006.
Keywords adverse events, anxiety, CFTR modulators, Cognitive, Mood, Neuropsychological
November 2024
Title: Coping and Learning to Manage Stress (CALM): A telehealth intervention to help reduce depression and anxiety symptoms in adults with CF
Authors Christina J Bathgate, Elizabeth D Smith, Nora H Murphy, Alexandra L Quittner, Kristin A Riekert, Jennifer L Goralski, Kristen E Holm
References J Cyst Fibros. 2024 Nov 24:S1569-1993(24)01810-1. doi: 10.1016/j.jcf.2024.11.002.
Keywords anxiety, Cognitive behavioral Stress management, Cognitive behavioral therapy, depression, Psychosocial intervention, Skill building
November 2024
Title: Effect of elexacaftor/tezacaftor/ivacaftor on the use of routine therapies in Danish people with cystic fibrosis
Authors Hans Kristian Råket, Camilla Bjørn Jensen, Joanna Nan Wang, Tacjana Pressler, Hanne Vebert Olesen, Marianne Skov, Søren Jensen-Fangel, Janne Petersen, Espen Jimenez-Solem; TransformCF study group
References J Cyst Fibros. 2024 Nov 23:S1569-1993(24)01813-7. doi: 10.1016/j.jcf.2024.11.004.
Keywords Drug utilization, registry, Treatment Outcome
Title: People with cystic fibrosis and care teams share how CF care can change to meet patient needs.
Authors Quynh T Tran, Enid Aliaj, Murrey G Olmsted, Sarah E Hempstead, Paula H Lomas, Rebekah F Brown, Patrick A Flume, A Whitney Brown
References J Cyst Fibros. 2024 Nov;23(6):1066-1071. doi: 10.1016/j.jcf.2024.09.002. Epub 2024 Sep 25.
Keywords CF care model, Healthcare delivery, interdisciplinary, Remote monitoring, Specialized care, Telehealth, Telemedicine
November 2024
Title: Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis
Authors Carlo Castellani, Nicholas J Simmonds, Jürg Barben, Charlotte Addy, Amanda Bevan, Pierre-Régis Burgel, Pavel Drevinek, Silvia Gartner, Andrea Gramegna, Elise Lammertyn, Eddie Edwina C Landau, Peter G Middleton, Barry J Plant, Alan R Smyth, , Silke van Koningsbruggen-Rietschel, Emmanuelle Girodon, Nataliya Kashirskaya, Anne Munck, Lutz Nährlich, Karen Raraigh, Isabelle Sermet-Gaudelus, Olaf Sommerburg, Kevin W Southern
References J Cyst Fibros. 2023 Nov;22(6):963-968. doi: 10.1016/j.jcf.2023.09.008. Epub 2023 Sep 27.
Keywords CFSPID, CFTR, CFTR-related disorder, CRMS/CFSPID, Extended Gene Analysis (EGA), Newborn Bloodspot Screening (NBS)
November 2024
Title: A mouse model containing the W1282X mutation can be used to test cystic fibrosis therapies
Authors J Cyst Fibros. 2024 Nov 11:S1569-1993(24)01797-1. doi: 10.1016/j.jcf.2024.10.008.
References Margaret Michicich, Zachary Traylor, Caitlan McCoy, Dana M Valerio, Alma Wilson, Molly Schneider, Sakeena Davis, Amanda Barabas, Rachel J Mann, David F LePage, Weihong Jiang, Mitchell L Drumm, Thomas J Kelley, Ronald A Conlon, Craig A Hodges
Keywords Mouse model, Nonsense mutation, Organoids, W1282X
November 2024
Title: How Does Income Level Affect the Health of People with Cystic Fibrosis?
Authors Neval Metin Cakar, Seyda Karabulut, Mine Yuksel Kalyoncu, Merve Selcuk Balcı, Ceren Ayça Yıldız, Damla Kocaman, Burcu Uzunoglu, Gamze Tastan, Almala Pınar Ergenekon, Ela Erdem Eralp, Yasemin Gokdemir, Fazilet Karakoc, Bulent Karadag
References J Cyst Fibros. 2024 Nov 7:S1569-1993(24)01803-4. doi: 10.1016/j.jcf.2024.10.010.
Keywords Health outcomes, Income level, Social determinant of health, Your current life situation
November 2024
Title: Aging and CF in the United States
Authors Joshua S. Ostrenga, Kristina Robinson, A. Whitney Brown, Christopher H. Goss, Elizabeth A. Cromwell
References J Cyst Fibros. 2024 Nov 4:S1569-1993(24)01802-2. doi: 10.1016/j.jcf.2024.10.009.
October 2024
Title: Understanding rates of and factors associated with elexacaftor/tezacaftor/ivacaftor (ETI) prescription
Authors Georgene E Hergenroeder, Jonathan V Todd, Josh S Ostrenga, Christopher H Goss, Raksha Jain, Wayne Morgan, Gregory S Sawicki, Michael S Schechter, Elizabeth A Cromwell, Clement L Ren
References J Cyst Fibros. 2024 Oct 28:S1569-1993(24)01801-0. doi: 10.1016/j.jcf.2024.10.006.
Keywords CFTR modulator, Elexacaftor/tezacaftor/ivacaftor
October 2024
Title: Presence of bacteriophage is associated with lung function decline in people with cystic fibrosis and a pseudomonas infection
Authors Elizabeth B Burgener, Aditi Gupta, Kayo Nakano, Sophia L Gibbs, Maya E Sommers, Arya Khosravi, Michelle S Bach, Colleen Dunn, Jacquelyn Spano, Patrick R Secor, Lu Tian, Paul L Bollyky, Carlos E Milla
References J Cyst Fibros. 2024 Oct 25:S1569-1993(24)01786-7. doi: 10.1016/j.jcf.2024.09.018.
Keywords Bacteriophage, Pseudomonas
October 2024
Title: How a new CF treatment affects the throat bacteria in teenagers
Authors Ruth Steinberg, Alexander Moeller, Amanda Gisler, Nadja Mostacci, Markus Hilty, Jakob Usemann
References J Cyst Fibros. 2024 Oct 14:S1569-1993(24)01794-6. doi: 10.1016/j.jcf.2024.10.001.
Keywords Children, Elexacaftor/tezacaftor/ivacaftor, Metagenomics, Microbiome, Modulator therapy, strain-level
October 2024
Title: How have prescriptions for inhaled antibiotics changed in recent years in the U.S.
Authors Marianne S Muhlebach, Jane She, Eric Y Zhang, Jonathan D Cogen, Michael R Kosorok
References J Cyst Fibros. 2024 Oct 9:S1569-1993(24)01785-5. doi: 10.1016/j.jcf.2024.09.017.
Keywords Antimicrobial stewardship, Aspergillus, CFTR modulator, Inhaled antibiotics, Pseudomonas aeruginosa
September 2024
Title: Assessing novel modulator therapy in children with cystic fibrosis using xenon gas MRI during multiple-breath washout
Authors Faiyza Alam, Samal Munidasa, Brandon Zanette, Sharon Braganza, Daniel Li, Renee Jensen, Marie-Pier Dumas, Felix Ratjen, Giles Santyr
References J Cyst Fibros. 2024 Sep 30:S1569-1993(24)01789-2. doi: 10.1016/j.jcf.2024.09.021.
Keywords Fractional ventilation, Hyperpolarized (129)Xe, Lung clearance index, Multiple-breath washout, Pediatric; elexacaftor/tezacaftor/ivacaftor
September 2024
Title: Cardiac Structure and Function in People with Cystic Fibrosis.
Authors Lisa Steen Duus, Maria Dons, Rebekka Faber Thudium, Susanne Dam Nielsen, Mette F Olsen, Tavs Qvist, Mats C Højbjerg Lassen, Kristoffer Grundtvig Skaarup, Niklas Dyrby Johansen, Thomas Mørk-Strøm Bluhme, Terese L Katzenstein, Tacjana Pressler,, Daniel Faurholt-Jepsen, Tor Biering-Sørensen
References J Cyst Fibros. 2024 Sep 28:S1569-1993(24)01781-8. doi: 10.1016/j.jcf.2024.09.012.
Keywords Cardiac function, Cardiac structure, Cardiovascular disease, Echocardiography, Myocardial impairment
September 2024
Title: What is the best approach to bring drugs to all people with CF?
Authors Margarida D Amaral, Ines Pankonien
References J Cyst Fibros. 2024 Sep 25:S1569-1993(24)01782-X. doi: 10.1016/j.jcf.2024.09.013.
Keywords CFTR modulators, Highly effective CFTR modulator therapies, Orphan mutations, Rare diseases
September 2024
Title: Guidance for Changing the CF Care Team
Authors Rebekah F Brown, Charlotte T Close, Molly G Mailes, Luis J Gonzalez, Danielle M Goetz, Stephanie S Filigno, Rebecca Preslar, Quynh T Tran, Sarah E Hempstead, Paula Lomas, A Whitney Brown, Patrick A Flume; CFF Care Model Committee
References J Cyst Fibros. 2024 Sep 24:S1569-1993(24)01778-8. doi: 10.1016/j.jcf.2024.09.011.
Keywords Collaborative care, Interdisciplinary team
September 2024
Title: A study on how the newest modulator medication elexacaftor/tezacaftor/ivacaftor affects the gut over time using MRI scans
Authors Alexander Yule, Christabella Ng, Arantxa Recto, Florence Lockwood, Neele S Dellschaft, Caroline L Hoad, Carlos Zagoya, Jochen G Mainz, Giles Major, Helen L Barr, Penny A Gowland, Iain Stewart, Luca Marciani, Robin C Spiller, Alan R Smyth
References J Cyst Fibros. 2024 Sep;23(5):984-990. doi: 10.1016/j.jcf.2024.08.001.
Keywords CFTR modulator, ETI, GI, MRI, Elexacaftor/tezacaftor/ivacaftor, Gastrointestinal, gut, Magnetic Resonance Imaging
Title: Comparing the money spent on healthcare up to age 2 years in children with CF who are screen detected and those who are diagnosed later due to sickness.
Authors Rebecca Somerville, Catherine Fitzgerald, Ricardo Segurado, Kanika Kapur, Sherly George, Nancy Bhardwaj, Barry Linnane, Alan O’Ceilleachair, Anthony Staines, Patricia Fitzpatrick
References J Cyst Fibros. 2024 Sep;23(5):896-902. doi: 10.1016/j.jcf.2024.06.015.
Keywords Cost, Economic, Ireland, screening
Title: Evaluating the Effectiveness of a Novel CF Medication for People Without Common Cystic Fibrosis-causing Genetic Mutations
Authors Daniel Lupas, Frank Y Chou, Mohammad Abdullah Al Hakani, Ishita Kuthiala, Arjuna Srikrishnaraj, Xuan Li, Naomi Potter, Bradley S Quon
References J Cyst Fibros. 2024 Sep;23(5):950-958. doi: 10.1016/j.jcf.2024.07.012. Epub 2024 Jul 23.
Keywords CFTR modulator, Elexacaftor/tezacaftor/ivacaftor, Non-F508del mutation
September 2024
Title: Do clinical outcomes differ between white and non-white Canadians with cystic fibrosis?
Authors Rachel Holland, Hanna Stewart, Stephanie Y Cheng, Miriam Schroeder, Sanja Stanojevic
References J Cyst Fibros. 2024 Sep 21:S1569-1993(24)01777-6. doi: 10.1016/j.jcf.2024.09.009.
September 2024
Title: CF Care Model- Guidance for an Evolving Model
Authors DM Goetz, RF Brown, SS Filigno, SL Bichl, AL Nelson, CA Merlo, R Juel, P Lomas, SE Hempstead, Q Tran, AW Brown, & PA Flume on behalf of the CFF Care Model Committee
References J Cyst Fibros. 2024 Sep 19:S1569-1993(24)00838-5. doi: 10.1016/j.jcf.2024.08.007.
Keywords CF care model, Healthcare delivery, mental health, Telemedicine
August 2024
Title: What is the intimate quality of life of women with Cystic Fibrosis?
Authors Françoise Simonnet Bisson, Mathieu Fauvernier, Chantal Belleguic, Isabelle Danner Boucher, Dominique Grenet, Rebecca Hamidfar, Dominique Hubert, Julie Macey, Marlène Murris-Espin, Michele Porzio, Sophie Ramel, Quitterie Reynaud, Martine Reynaud-Gaubert, P
References J Cyst Fibros. 2024 Aug 24:S1569-1993(24)00829-4. doi: 10.1016/j.jcf.2024.08.003.
Keywords Sex life, Sexual disorders, Sexual quality of life
Title: Differences between the United States and the European Union in the time of approval of CFTR modulators for the treatment of cystic fibrosis
Authors Enrico Costa, Silvia Girotti, Clément Mathieu, Carlo Castellani, Joseph S Ross, Jennifer L. Taylor-Cousar, Hubert G. M. Leufkens
References J Cyst Fibros. 2024 Aug 24:S1569-1993(24)00828-2. doi: 10.1016/j.jcf.2024.08.002.
Keywords CFTR modulators, European Medicines Agency, Food and Drug Administration, Times of approval, Times of submission
August 2024
Title: How Race and Ethnicity Affect the Development of Diabetes in People with Cystic Fibrosis
Authors Maria S Rayas, Blessed Mbogo, Andrea Kelly, Phuong Vu, Amalia Magaret, Tanicia Daley
References J Cyst Fibros. 2024 Aug 3:S1569-1993(24)00803-8. doi: 10.1016/j.jcf.2024.07.018. Online ahead of print.
Keywords Cystic fibrosis related diabetes, Disparities, Race and ethnicity
July 2024
Title: Evaluating the treatment outcomes of the bacteria, Mycobacterium abscessus, in people with cystic fibrosis at the Royal Papworth Hospital, UK.
Authors Ieuan Evans, Aaron Weimann, Timothy Baird, Charles Haworth, Andres Floto
References J Cyst Fibros. 2024 Jul 26:S1569-1993(24)00801-4. doi: 10.1016/j.jcf.2024.07.016.
July 2024
Title: The impact of elexacaftor/tezacaftor/ivacaftor on anxiety and depression in adults with Cystic Fibrosis
Authors Minh Nguyen, Pat MacDiarmid, April Tanzler, Renée Dagenais, Carolina Bevanda, Bradley S Quon
References J Cyst Fibros. 2024 Jul 20:S1569-1993(24)00793-8. doi: 10.1016/j.jcf.2024.07.008.
Keywords anxiety, depression, Elexacaftor/tezacaftor/ivacaftor, Trikafta
July 2024
Title: Measuring Abnormal Lung Microstructure in People with Cystic Fibrosis Using Xenon Magnetic Resonance Imaging
Authors Abdullah S Bdaiwi, Alexandra M Svoboda, Kyle E Murdock, Alexandra Hendricks, Md M Hossain, Elizabeth L Kramer, John J Brewington, Matthew M Willmering, Jason C Woods, Laura L Walkup, Zackary I Cleveland
References J Cyst Fibros. 2024 Jul 11:S1569-1993(24)00786-0. doi: 10.1016/j.jcf.2024.07.002.
Keywords Diffusion, Hyperpolarized (129)Xe, Linear binning, Lung morphometry
Title: A survey of how the CF team use online learning.
Authors C Smith, H K Chadwick, K Hill, D G Peckham; European Cystic Fibrosis Society Education Committee
References J Cyst Fibros. 2024 Jul 11:S1569-1993(24)00787-2. doi: 10.1016/j.jcf.2024.07.003.
Keywords E learning, Education, Multidisciplinary.
June 2024
Title: Evaluating Fertility Preservation Telehealth Counseling for Males with Cystic Fibrosis
Authors Brittany M Woods, Leigh A Bray, Sukhkamal B Campbell, Peng Li, Traci M Kazmerski, Cade Hovater, Leslie N Pitts, Sigrid Ladores
References J Cyst Fibros. 2024 Jun 27:S1569-1993(24)00780-X. doi: 10.1016/j.jcf.2024.06.011.
Keywords Fertility preservation, Patient education, Telehealth
Title: Could glucose tolerance tests used to detect cystic fibrosis related diabetes be performed at home?
Authors Laure Alexandre-Heymann, Valérie Boudreau, Noémie Bélanger, Agata Mostkowska, Anne Bonhoure, Annick Lavoie, Rémi Rabasa-Lhoret, Adèle Coriati
References J Cyst Fibros. 2024 Jun 27:S1569-1993(24)00777-X. doi: 10.1016/j.jcf.2024.06.009
Keywords Cystic fibrosis related diabetes, Oral glucose tolerance test, Quality of life, screening
June 2024
Title: How Personalized Genetic Testing for Medication Metabolism May Reduce Problems with Medications for People with Cystic Fibrosis
Authors Anindit Chhibber, Alexandre Hikiji Watanabe, Hollyann Jacobs, Aditi Kharat, Sakil M Syeed, Catherine M Sherwin, Nathorn Chaiyakunapruk, Joseph Biskupiak, Venkata K Yellepeddi, Diana Brixner, David C Young
References J Cyst Fibros. 2024 Jun 26:S1569-1993(24)00775-6. doi: 10.1016/j.jcf.2024.06.006.
Keywords Adverse drug reactions, Drug therapy problems, Genomics, Pharmacogenomics, Pharmacotherapy, Precision medicine
June 2024
Title: Lung function and imaging outcomes in children with cystic fibrosis diagnosed after newborn screening
Authors Bettina S Frauchiger, Corin Willers, Jasna Cotting, Elisabeth Kieninger, Insa Korten, Carmen Casaulta, Yasmin Salem, Enno Stranzinger, Ben Brabandt, Jakob Usemann, Nicolas Regamey, Alena Kuhn, Sylvain Blanchon, Isabelle Rochat, Grzegorz Bauman, , Dominik Müller-Suter, Alexander Moeller, Philipp Latzin, Kathryn A Ramsey; BILD and SCILD study groups
References J Cyst Fibros. 2024 Jun 25:S1569-1993(24)00074-2. doi: 10.1016/j.jcf.2024.05.010.
Keywords Children, Lung clearance index, Magnetic Resonance Imaging, Multiple breath washout
June 2024
Title: Beyond Insulin: Unraveling the Complex Interplay of Cell Stress and Cell Communication in CFRD
Authors Bala Umashankar, Lena Eliasson, Chee Y Ooi, Ki Wook Kim, James A M Shaw, Shafagh A Waters
References J Cyst Fibros. 2024 Jun 18:S1569-1993(24)00082-1. doi: 10.1016/j.jcf.2024.06.004.
Title: Beyond Insulin: Unraveling the Complex Interplay of Cell Stress and Cell Communication in CFRD
Authors Bala Umashankar, Lena Eliasson, Chee Y Ooi, Ki Wook Kim, James A M Shaw, Shafagh A Waters
References J Cyst Fibros. 2024 Jun 18:S1569-1993(24)00082-1. doi: 10.1016/j.jcf.2024.06.004.
Keywords CFRD, ER stress, Exosomes, oxidative stress, miRNA
June 2024
Title: Presentation of two siblings with CF born to a mother with CF taking Trikafta (Kaftrio) throughout both pregnancies. A baby boy has normal vas deferens after birth.
Authors Aleksandra Kowalik, Emma Roberts, Anna Hedborg Harris, Marie Sund, Sara Wird, Ola Kvist, Lena Hjelte
References J Cyst Fibros. 2024 Jun 13:S1569-1993(24)00080-8. doi: 10.1016/j.jcf.2024.06.003.
June 2024
Title: Development and Initial Use of the Personalized Cystic Fibrosis Medication Questionnaire (PCF-MQ)
Authors Hanna Phan, Cori L Daines, Ti Woo, Kevin J. Psoter, Andrea Goodman, Emma McWilliams, Carla Frederick, Carlos Milla, Gabriela Oates, Gregory S. Sawicki, Kristin A. Riekert
References J Cyst Fibros. 2024 Jun 7:S1569-1993(24)00073-0. doi: 10.1016/j.jcf.2024.05.011.
Keywords Medication education, Medication knowledge, Personalized medicine
May 2024
Title: Standards for the care of people with cystic fibrosis (CF); Planning for a longer life
Authors Andrea Gramegna, Charlotte Addy, Lorna Allen, Egil Bakkeheim, Catherine Brown, Thomas Daniels, Gwyneth Davies, Jane C Davies, Ken De Marie, Damian Downey, Imogen Felton, Sylvia Hafkemeyer, Samia Hamouda, Victoria Kendall, Ulrika Lindberg, Milan Macek,, Sarah Mayell, Oren Pearlsman, Michael S Schechter, Lidia Salvatori, Dorota Sands, Carsten Schwarz, Michal Shteinberg, Julia Taylor, Jennifer L Taylor-Cousar, David Taylor-Robinson, Bethan Watkins, Marieke Verkleij, Amanda Bevan, Carlo Castellani,, Pavel Drevinek, Silvia Gartner, Elise Lammertyn, Eddie Edwina C Landau, Peter G Middleton, Barry J Plant, Alan R Smyth, Silke van Koningsbruggen-Rietschel, Pierre-Régis Burgel, Kevin W Southern
References J Cyst Fibros. 2024 May;23(3):375-387. doi: 10.1016/j.jcf.2024.05.007.
Keywords Family, Longer life, standards of care
Title: Improved growth in young children with Cystic Fibrosis
Authors Karlen Bader-Larsen, Daniel Faurholt-Jepsen, Thomas Bryrup,Esben Herborg Henriksen, Hanne Vebert Olesen, Tacjana Pressler, Marianne Skov, Mette Frahm Olsen
References J Cyst Fibros. 2024 May 23:S1569-1993(24)00072-9. doi: 10.1016/j.jcf.2024.05.009.
Keywords Body-mass-index, Height, Length, Malnourishment, malnutrition, Newborn screening, Overweight, Stunting, Underweight, Weight, Z-score
May 2024
Title: Modulatory therapy discontinuation of children with cystic fibrosis
Authors Burcu Capraz Yavuz, Ebru Yalcin, Halime Nayir Buyuksahin, Birce Sunman, Ismail Guzelkas, Didem Alboga, Meltem Akgul Erdal, Havva Ipek Demir, Raziye Atan, Nagehan Emiralioglu, Deniz Dogru, Ugur Ozcelik, Nural Kiper
References J Cyst Fibros. 2024 May 18:S1569-1993(24)00069-9. doi: 10.1016/j.jcf.2024.05.006.
Keywords CFTR modulator drugs, Pseudomonas aeruginosa
May 2024
Title: In-depth examination of the CFTR gene finds more disease-causing genetic changes than conventional clinical genetic testing
Authors Molly B Sheridan, Melis A Aksit, Kymberleigh Pagel, Kurt Hetrick, Hannah Shultz-Lutwyche, Ben Myers, Kati J Buckingham, Rhonda G Pace, Hua Ling, Elizabeth Pugh, Wanda K O’Neal, Michael J Bamshad, Ronald L. Gibson, Michael R Knowles, Scott M Blackman, , Garry R Cutting, Karen S Raraigh
References J Cyst Fibros. 2024 May 10:S1569-1993(24)00062-6. doi: 10.1016/j.jcf.2024.04.018.
Keywords CFTR, genetic testing, Genome sequencing, Whole-gene sequencing
May 2024
Title: Accurate identification of people living with cystic fibrosis (CF) in the Danish National Patient Registry
Authors Hans Kristian Råket, Joanna Nan Wang, Janne Petersen, Tacjana Pressler, Hanne Vebert Olesen, Søren Jensen-Fangel, Thomas Bryrup, Espen Jimenez-Solem, Camilla Bjørn Jensen
References J Cyst Fibros. 2024 May 9:S1569-1993(24)00066-3. doi: 10.1016/j.jcf.2024.05.003.
Keywords Databases, Factual, registries, Validation studies
May 2024
Title: Oversight of Rare Disease Studies: Experience of the CF Foundation DSMB
Authors Lynne M. Quittell, Richard H. Simon, Wayne Morgan
References J Cyst Fibros. 2024 May 7:S1569-1993(24)00065-1. doi: 10.1016/j.jcf.2024.05.001.
Keywords clinical trials, DSMB, Data safety, Data safety monitoring board, Rare disease
May 2024
Title: The structure of the blood immune system in people with cystic fibrosis and how this affects their lung capacity and the microorganisms colonizing their airways.
Authors Elio Rossi, Mads Lausen, Nina Friesgaard Øbro, Antonella Colque, Bibi Uhre Nielsen, Rikke Møller, Camilla de Gier, Annemette Hald, Marianne Skov, Tacjana Pressler, Søren Molin, Sisse Rye Ostrowski, Hanne Vibeke Marquart, Helle Krogh Johansen
References J Cyst Fibros. 2024 May 2:S1569-1993(24)00059-6. doi: 10.1016/j.jcf.2024.04.015.
Keywords Adaptive immunity, Immune dysregulation, Infections, Innate immunity, Lung microbiome
May 2024
Title: Exercise capacity after one year of Kaftrio® treatment in people with Cystic Fibrosis
Authors Lue Katrine Drasbæk Philipsen, Hanne Vebert Olesen, Janne Hastrup Jensen, Mette Frahm Olsen, Daniel Faurholt-Jepsen, Frederik Buchvald, Kim Gjerum Nielsen, Marianne Skov, Tacjana Pressler
References J Cyst Fibros. 2024 May 1:S1569-1993(24)00055-9. doi: 10.1016/j.jcf.2024.04.010.
April 2024
Title: Do adult patients with cystic fibrosis suffer from sexual dysfunction?
Authors Sophie Ramel, Laetitia Gueganton, Emmanuel Nowak, Jean Le Bihan, Baptiste Arnouat, Chantal Belleguic, Isabelle Danner-Boucher, Julie Mankikian, Annabelle Payet, Thierry Urban, Marion Buyse, Katelyne Hubeaux
References J Cyst Fibros. 2024 Apr 29:S1569-1993(24)00056-0. doi: 10.1016/j.jcf.2024.04.011.
Keywords Erectile dysfunction, Faecal incontinence, Sexual function, Urinary incontinence
April 2024
Title: 11-year evaluation of newborn screening for Cystic Fibrosis in Switzerland
Authors Esl Pedersen, C M de Jong Carmen, M Jurca, D O Berger, J Sanz, Shm Sluka, M Poms, M R Baumgartner, N Regamey, C E Kuehni, J Barben, C S Rueegg; Swiss Cystic Fibrosis Newborn Screening Group
References J Cyst Fibros. 2024 Apr 23:S1569-1993(24)00053-5. doi: 10.1016/j.jcf.2024.04.008.
Keywords evaluation, Newborn screening, performance, Positive predictive value, Safety net, Screening algorithm, Sensitivity, Simulation, Specificity
March 2024
Title: Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues
Authors Pierre-Régis Burgel, Kevin W Southern, Charlotte Addy, Alberto Battezzati, Claire Berry, Jean-Philippe Bouchara, Edwin Brokaar, Whitney Brown, Pilar Azevedo, Isabelle Durieu, Miquel Ekkelenkamp, Felicity Finlayson, Julian Forton, Johanna Gardecki,, Pavla Hodkova, Gina Hong, Jacqueline Lowdon, Su Madge, Clémence Martin, Edward McKone, Anne Munck, Chee Y Ooi, Lucy Perrem, Amanda Piper, Andrew Prayle, Felix Ratjen, Margaret Rosenfeld, Don B Sanders, Carsten Schwarz, Giovanni Taccetti, Claire Wainwright, Natalie E West, Michael Wilschanski, Amanda Bevan, Carlo Castellani, Pavel Drevinek, Silvia Gartner, Andrea Gramegna, Elise Lammertyn, Eddie Edwina C Landau, Barry J Plant, Alan R Smyth, Silke van Koningsbruggen-Rietschel, Peter G Middleton
References J Cyst Fibros. 2024 Mar;23(2):187-202. doi: 10.1016/j.jcf.2024.01.005.
Keywords breathing, CFRD, Complications, End of life;, liver, lung infection, mental health, Pulmonary, Transplant
March 2024
Title: Analysis of cell mechanisms influencing Covid-19 disease in cystic fibrosis patients and in the general population
Authors Fabiana Ciciriello, Francesco Panariello, Paola Medino, Arianna Biffi, Federico Alghisi, Chiara Rosazza, Patrizia Annunziata, Valentina Bouchè, Antonio Grimaldi, Daniela Guidone, Arianna Venturini, Gianfranco Alicandro, Massimo Oggioni, Pellegrino Cerino,, Giulia Paiola, Andrea Gramegna, Alessandro Fiocchi, Alessandra Bandera, Vincenzina Lucidi, Davide Cacchiarelli, Luis J V Galietta, Carla Colombo
References J Cyst Fibros. 2024 Mar 19:S1569-1993(24)00036-5. doi: 10.1016/j.jcf.2024.03.006.
Keywords COVID-19, Gene expression, RNA sequencing, SARS-CoV-2, Virus-host interactions
March 2024
Title: Correlation between trough concentration and exposure during the day for elexacaftor, tezacaftor and ivacaftor
Authors Steffie E M Vonk, Josje Altenburg, Ron A A Mathôt, E Marleen Kemper; Amsterdam Mucociliary Clearance Disease (AMCD) Research Group
References J Cyst Fibros. 2024 Mar 16:S1569-1993(24)00038-9. doi: 10.1016/j.jcf.2024.03.010.
Keywords Area under the curve, CFTR modulators, Elexacaftor-tezacaftor-ivacaftor, Therapeutic drug monitoring, Trough concentration
March 2024
Title: Education, employment, and income among people living with cystic fibrosis across three decades
Authors Camilla Bjørn Jensen, Kristoffer Jarlov Jensen, Tacjana Pressler, Terese L Katzenstein, Marianne Skov, Tavs Qvist, Mette Frahm Olsen, Majbritt Jeppesen, Søren Jensen-Fangel, Hanne Vebert Olesen, Simon Bertram Reuter, Hans Kristian Råket Pedersen,, Joanna Nan Wang, Steven Michalopoulos, Lisa McGarry, Heike Wöhling, Janne Petersen, Espen Jimenez-Solem; TransformCF study group
References J Cyst Fibros. 2024 Mar 14:S1569-1993(24)00039-0. doi: 10.1016/j.jcf.2024.03.009.
Keywords Education, Employment, Income
Title: Impact of modulator therapy on iron deficiency in cystic fibrosis
Authors Shijing Jia, Yizhuo Wang, Melissa H Ross, Jonathan B Zuckerman, Susan Murray, MeiLan K Han, Shannon E Cahalan, Blair E Lenhan, Ryan N Best, Jennifer L Taylor-Cousar, Richard H Simon, Linda J Fitzgerald, Jonathan P Troost, Suman L Sood, Alex H Gifford
References J Cyst Fibros. 2024 Mar 14:S1569-1993(24)00030-4. doi: 10.1016/j.jcf.2024.03.002.
Keywords anemia, CFTR modulator therapies, Iron deficiency, Nutrition
March 2024
Title: Use of health care before lung transplantation or death in people with cystic fibrosis
Authors Erika Guyot, Quitterie Reynaud, Manon Belhassen, Marjorie Bérard, Clémence Dehillotte, Lydie Lemonnier, Marie Viprey, Eric Van Ganse, Pierre-Régis Burgel, Isabelle Durieu
References J Cyst Fibros. 2024 Mar 12:S1569-1993(24)00031-6. doi: 10.1016/j.jcf.2024.03.001
Keywords Claims data, Costs, Healthcare resource utilization, National registry, transplantation
March 2024
Title: What symptoms are most common and most bothersome for people living with cystic fibrosis?
Authors Natalia Smirnova, Jane Lowers, Alexandre Cammarata-Mouchtouris, Elisabeth P Dellon, Anne Fitzpatrick, Dio Kavalieratos
References J Cyst Fibros. 2024 Mar 8:S1569-1993(24)00033-X. doi: 10.1016/j.jcf.2024.03.005.
Keywords Palliative care, Quality of life, Symptom management
March 2024
Title: CFTR modulators in people with CF and the I1234V mutation
Authors Bat El Bar Aluma, Joel Reiter, Ori Efrati, Yael Bezalel, Shlomit Keler, Moshe Ashkenazi, Adi Dagan, Yael Buchnik, Ido Sadras, Malena Cohen-Cymberknoh
References J Cyst Fibros. 2024 Mar 4:S1569-1993(24)00019-5. doi: 10.1016/j.jcf.2024.02.008.
Keywords CFTR modulators, Elexacaftor/tezacaftor/ivacaftor, I1234V CFTR mutation
March 2024
Title: 2023 Year in Review Literature Review
Authors Luca Cristiani, Flávia Fonseca Fernandes
References J Cyst Fibros. 2024 Mar 1:S1569-1993(24)00022-5. doi: 10.1016/j.jcf.2024.02.007.
February 2024
Title: Remote endpoints for research in cystic fibrosis
Authors Jordana E Hoppe, Jacquelyn Sjoberg, Gina Hong, Katie Poch, Edith T Zemanick, Stephanie Thee, Claire Edmondson, Dhiren Patel, Meghana Sathe, Drucy Borowitz, Melissa S Putman, Noah Lechtzin, Kristin A Riekert, Melissa Basile, Christopher H Goss,, Mary Elizabeth Jarosz, Margaret Rosenfeld Clinical trials, Home, Remote monitoring.
References J Cyst Fibros. 2024 Feb 29:S1569-1993(24)00023-7. doi: 10.1016/j.jcf.2024.02.011.
Keywords clinical trials, Home, Remote monitoring
February 2024
Title: Role of dormant bacteria in people with cystic fibrosis patients
Authors Natalia Cirilli, Valentina Schiavoni, Valentina Tagliabracci, Rosaria Gesuita, Luca Tiano, Benedetta Fabrizzi, Anastasia D'Antuono, Arianna Peruzzi, Nicholas Cedraro, Flavia Carle, Marco Moretti, Luigi Ferrante, Carla Vignaroli, Francesca Biavasco, Gianma
References J Cyst Fibros. 2024 Feb 28:S1569-1993(24)00026-2. doi: 10.1016/j.jcf.2024.02.013.
Title: Role of dormant bacteria in people with cystic fibrosis patients
Authors Natalia Cirilli, Valentina Schiavoni, Valentina Tagliabracci, Rosaria Gesuita, Luca Tiano, Benedetta Fabrizzi, Anastasia D'Antuono, Arianna Peruzzi, Nicholas Cedraro, Flavia Carle, Marco Moretti, Luigi Ferrante, Carla Vignaroli, Francesca Biavasco, Gianma
References J Cyst Fibros. 2024 Feb 28:S1569-1993(24)00026-2. doi: 10.1016/j.jcf.2024.02.013.
Keywords CF lung pathogens, lung infection, Non-cultural methods, VBN
February 2024
Title: Repurposing enzymes to degrade complex biofilms containing two bacteria and improve antibiotic activity
Authors Zhifen Wang, Rita Vanbever, Joseph H Lorent, Jessica Solis, Christiane Knoop, Françoise Van Bambeke
References J Cyst Fibros. 2024 Feb 23:S1569-1993(24)00027-4. doi: 10.1016/j.jcf.2024.02.012.
Keywords Alginate lyase, Artificial sputum medium, DNase I, Dual-species biofilm, Meropenem, Tobramycin, Pseudomonas aeruginosa, Staphylococcus aureus
February 2024
Title: A review of medications and medical devices used for preventing pregnancy in people with cystic fibrosis
Authors E. Claire Elson, Taylor Imburgia, Kevin Lonabaugh, Lindsey McCoy, Nicole E. Omecene, Shannon M. Rotolo
References NA 21 February 2024
February 2024
Title: Amount of chloride in the sweat of people with CF taking CFTR modulators
Authors ET Zemanick, I Emerman, M McCreary, N Mayer-Hamblett, MN Warden, K Odem-Davis, DR VanDevanter, CL Ren, J Young, MW Konstan for the CHEC-SC Study Group
References J Cyst Fibros. 2024 Feb 14:S1569-1993(24)00015-8. doi: 10.1016/j.jcf.2024.02.001.
Keywords CFTR function, CFTR modulators, Epidemiology, sweat chloride
Title: Use of inhaled antibiotics to treat Pseudomonas aeruginosa infections in Europe.
Authors Callum M. Sloan, Laura J. Sherrard, Gisli G. Einarsson, Lieven J. Dupont, Silke van Koningsbruggen-Rietschel, Nicholas J. Simmonds, Damian G. Downey
References J Cyst Fibros. 2024 Feb 14:S1569-1993(23)01683-1. doi: 10.1016/j.jcf.2023.11.012.
Keywords Inhaled antibiotics, Pseudomonas aeruginosa
February 2024
Title: Effect of stopping inhaled therapies on mucus clearance while on elexacaftor/tezacaftor/ivacaftor
Authors Scott H Donaldson, Timothy E Corcoran, Joseph M Pilewski, Beth L Laube, Peter Mogayzel, Agathe Ceppe, Jihong Wu, Kirby Zeman, Steven M Rowe, David P Nichols, Alex H Gifford, William D Bennett, Nicole Mayer-Hamblett; SIMPLIFY MCC Study teams
References J Cyst Fibros. 2024 Feb 13:S1569-1993(24)00018-3. doi: 10.1016/j.jcf.2024.02.003
Keywords Clinical trial, Dornase alfa, Hypertonic saline, Mucociliary clearance, Withdrawal
February 2024
Title: Nationwide records suggest some people with CF have improved digestion with ivacaftor
Authors Rebecca Calthorpe, Margaret Rosenfeld, Christopher H. Goss, Nicole Green, Mark Derleth, Siobhán B Carr, Alan Smyth, Iain Stewart
References J Cyst Fibros. 2024 Feb 10:S1569-1993(24)00012-2. doi: 10.1016/j.jcf.2024.01.011
Keywords Gastrointestinal, Ivacaftor, PERT, pancreas, Prevalence, registry
January 2024
Title: Is it safe to use Elexacaftor/tezacaftor/ivacaftor if you have had a kidney or liver transplant?
Authors Renske van der Meer, Erik B Wilms, Margot N Eggermont, Helena M Paalvast, Matthijs van Luin, Richard C J M van Rossen, Harry G M Heijerman
References J Cyst Fibros. 2024 Jan 29:S1569-1993(24)00007-9. doi: 10.1016/j.jcf.2024.01.008.
Keywords Drug-drug interaction, Elexacaftor/tezacaftor/ivacaftor, Solid organ transplant, Tacrolimus
January 2024
Title: Standards for the care of people with cystic fibrosis; establishing and maintaining health
Authors Kevin W Southern, Charlotte Addy, Scott C Bell, Amanda Bevan, Urzula Borawska, Catherine Brown, Pierre-Régis Burgel, Brenda Button, Carlo Castellani, Audrey Chansard, Mark A Chilvers, Gwyneth Davies, Jane C Davies, Kris De Boeck, Dimitri Declercq,, Michael Doumit, Pavel Drevinek, Isabelle Fajac, Silvia Gartner, Anna M Georgiopoulos, Sandra Gursli, Andrea Gramegna, Carina Me Hansen, Martin J Hug, Elise Lammertyn, Edwina Eddie C Landau, Ross Langley, Nicole Mayer-Hamblett, Anna Middleton, , Peter Middleton, Monika Mielus, Lisa Morrison, Anne Munck, Barry Plant, Maarten Ploeger, Dominique Pougheon Bertrand, Tacjana Pressler, Bradley S Quon, Thomas Radtke, Zoe L Saynor, Ilan Shufer, Alan Smyth, Chris Smith, Silke van Koningsbruggen-Rietschel
References J Cyst Fibros. 2024 Jan;23(1):12-28. doi: 10.1016/j.jcf.2023.12.002.
Keywords ECFS, guidance, Health, Standards
Title: Sleep breathing disorders in adults with cystic fibrosis (CF)
Authors Asma Maqsood, Xiayi Ma, Elizabeth Tullis, Clodagh M Ryan, Anju Anand, Anne L Stephenson, Nicholas T Vozoris
References Cyst Fibros. 2024 Jan;23(1):144-149. doi: 10.1016/j.jcf.2023.12.005. Epub 2023 Dec 20.
Keywords Epidemiology, Positive airway pressure, Sleep breathing disorder
January 2024
Title: Markers of inflammation in lung fluid can predict lung damage in young children with Cystic Fibrosis (CF)?
Authors Hamed Horati, Camilla Margaroli, Joshua D Chandler, Matthew B Kilgore, Badies Manai, Eleni-Rosalina Andrinopoulou, Limin Peng , Lokesh Guglani, Harm A M W Tiddens, Daan Caudri, Bob J Scholte, Rabindra Tirouvanziam, HettieM Janssens
References J Cyst Fibros. 2024 Jan 20:S1569-1993(24)00002-X. doi: 10.1016/j.jcf.2024.01.002.
Keywords Bronchiectasis, Bronchoalveolar lavage, Children, Inflammatory markers
January 2024
Title: CF adults with private insurance receive equivalent preventive care to the general population
Authors Sankalp Malhotra, J Madison Hyer, Djhenne Dalmacy, Don Hayes Jr, Dmitry Tumin, Stephen E Kirkby, Daniel E Jonas, Seuli Bose-Brill, Susan S Li
References J Cyst Fibros. 2024 Jan 13:S1569-1993(23)01698-3. doi: 10.1016/j.jcf.2023.11.013.
Keywords colonoscopy, mammogram, pap smear, Primary care, screening
January 2024
Title: Elexacaftor/Tezacaftor/Ivacaftor Improves Chronic Rhinosinusitis Detected by Magnetic Resonance Imaging in Children with Cystic Fibrosis on Long-Term Therapy with Lumacaftor/Ivacaftor
Authors Lena Wucherpfennig, Johanna K Z Becker, Felix Wuennemann, Monika Eichinger, Angelika Seitz, Ingo Baumann, Mirjam Stahl, Simon Y Graeber, Shengkai Zhao, Jaehi Chung, Jens-Peter Schenk, Abdulsattar Alrajab, Hans-Ulrich Kauczor, Marcus A Mall, , Olaf Sommerburg, Mark O Wielpütz
References J Cyst Fibros. 2024 Jan 12:S1569-1993(24)00004-3. doi: 10.1016/j.jcf.2024.01.00
Keywords Airway disease, Chronic rhinosinusitis, Magnetic Resonance Imaging, Mucus obstruction
https://pubmed.ncbi.nlm.nih.gov/38218661/
