September 2022
Title: The CFTR protein impacts energy usage in mouse brown fat
Authors Kyung-Mi Choi, Sung-Hee Cho, Jung Hak Kim, Ae-Rhee Lilian Kim, Xiangmudong Kong, John C Yoon
References J Cyst Fibros. 2022 Sep 7;S1569-1993(22)00649-X. doi: 10.1016/j.jcf.2022.08.012.
Keywords CFTR, brown adipocyte, cAMP-PKA pathway, thermogenesis
Theme CFTR/Basic Science, Endocrinology, Nutrition/Gastroenterology
August 2022
Title: How does early eradication of Pseudomonas affect lung function?
Authors Isabel Gascon Casaredi, Michelle Shaw, Valerie Waters, Ryan Seeto, Ana Blanchard, Felix Ratjen
References J Cyst Fibros. 2022 Aug 19;S1569-1993(22)00645-2. doi: 10.1016/j.jcf.2022.08.007.
Keywords antibiotic therapy, eradication, Pseudomonas aeruginosa
Theme Care of patients with CF, Lung Disease and Infection
August 2022
Title: How do people with CF experience pain and other symptoms?
Authors Ethan Dubin, Jane Lowers, Elisabeth P Dellon, Sarah Hempstead, Albert Faro, Erin Tallarico, Anne Fitzpatrick, William R Hunt, Dio Kavalieratos
References J Cyst Fibros. 2022 Aug 13;S1569-1993(22)00643-9. doi: 10.1016/j.jcf.2022.08.006.
Keywords Pain, Palliative care, Perceived discrimination, Signs and symptoms, Symptom assessment
Theme Care of patients with CF
August 2022
Title: Partner Perspectives on Women’s Sexual and Reproductive Healthcare in Cystic Fibrosis
Authors Sarah Ehrenberg, Olivia M Stransky, Catherine E Wright, Raksha Jain, Traci M Kazmerski
References J Cyst Fibros. 2022 Aug 12;S1569-1993(22)00641-5. doi: 10.1016/j.jcf.2022.08.003.
Keywords Partner perspectives, Sexual and reproductive healthcare
Theme Care of patients with CF, Pregnancy with CF, Psychosocial
August 2022
Title: Predicting Risk-Adjusted Incidence Rates of Methicillin-Resistant Staphylococcus aureus and Pseudomonas aeruginosa in the Cystic Fibrosis Programs in the United States
Authors United States William Stoudemire, Xiaotong Jiang, Juyan J Zhou, Michael R Kosorok, Lisa Saiman, Marianne S Muhlebach
References J Cyst Fibros. 2022 Aug 10;S1569-1993(22)00639-7. doi: 10.1016/j.jcf.2022.08.002.
Keywords Cystic fibrosis transmembrane conductance regulator, Lung transplantation, Trikafta
Theme Care of patients with CF, Epidemiology/Models of care, Lung Disease and Infection
July 2022
Title: Comparing continuous glucose monitoring to self-monitoring of blood glucose in the management of cystic fibrosis related diabetes; a systematic review and meta-analysis
Authors Shanal Kumar, Georgia Soldatos, Sanjeeva Ranasinha, Helena Teede, Michael Pallin
References J Cyst Fibros. 2022 Jul 26;S1569-1993(22)00633-6. doi: 10.1016/j.jcf.2022.07.013.
Keywords continuous glucose monitoring, Diabetes, Management
Theme Care of patients with CF, Diabetes, Epidemiology/Models of care
July 2022
Title: How might cystic fibrosis care change because of telehealth and CFTR modulators
Authors Michelle H. Prickett, Patrick A. Flume, Kathryn A. Sabadosa, Quynh T. Tran, Bruce C. Marshall
References J Cyst Fibros. 2022 Jul 22;S1569-1993(22)00600-2. doi: 10.1016/j.jcf.2022.07.002.
Keywords CFTR modulators, Telehealth
Theme Care of patients with CF, Epidemiology/Models of care
July 2022
Title: A Study Characterizing Sweat Chloride after CFTR Modulators in People with Cystic Fibrosis
Authors N Mayer-Hamblett, ET Zemanick, K Odem-Davis, D VanDevanter, M Warden, SM Rowe, J Young, MW Konstan for the CHEC-SC Study Group
References J Cyst Fibros. 2022 Jul 21;S1569-1993(22)00625-7. doi: 10.1016/j.jcf.2022.07.008.
Keywords CFTR function, CFTR modulators, Epidemiology, sweat chloride
Title: Are we missing Aspergillus in airway samples due to bacteria that may also be present?
Authors Dominic A Hughes, Mark Rosenthal, Leah Cuthbertson, Newara Ramadan, Imogen Felton, Nicholas J Simmonds, Michael R Loebinger, Henry Price, Darius Armstrong-James, J Stuart Elborn, William O Cookson, Miriam F Moffatt, Jane C Davies
References J Cyst Fibros. 2022 Jul 21;S1569-1993(22)00627-0. doi: 10.1016/j.jcf.2022.07.009.
Keywords Aspergillus, Chronic suppurative lung disease, Co-infection, Pseudomonas; Sequencing
Theme Care of patients with CF, Microbiology
July 2022
Title: The assessment of quality of care from the patient perspective
Authors Jacquelien J. Noordhoek, Joshena Jeyaratnam , Domenique Zomer, Vincent A.M. Gulmans, Cornelis K. van der Ent , Harry G.M. Heijerman
References J Cyst Fibros. 2022 Jul 13;S1569-1993(22)00622-1. doi: 10.1016/j.jcf.2022.07.004.
Keywords Patient involvement quality mark, Patient participation, Patient perspective, Quality of care
Theme Care of patients with CF
July 2022
Title: CFTR modulators: A case where Triple therapy induced rash resolved on its own, without stopping the drug
Authors Divyalakshmi Bhaskaran, Kathryn Bateman
References J Cyst Fibros. 2022 Jul 12;S1569-1993(22)00598-7. doi: 10.1016/j.jcf.2022.06.011.
Theme Care of patients with CF
July 2022
Title: People with cystic fibrosis-related diabetes do not have a lower exercise capacity compared to those without diabetes: data from an international multicenter study
Authors Thomas Radtke, Susi Kriemler, Lothar Stein, Chantal Karila, Don S Urquhart, David M Orenstein, Larry C Lands, Christian Schindler, Ernst Eber, Sarah R Haile, Helge Hebestreit, ACTIVATE-CF Study Working Group
References J Cyst Fibros. 2022 Jul 5;S1569-1993(22)00599-9. doi: 10.1016/j.jcf.2022.06.012.
Keywords Cystic fibrosis related diabetes, Dysglycaemia, Exercise capacity, Peak oxygen uptake, Physical activity
July 2022
Title: Vitamin D and liver stiffness measurements – Are they players in Cystic Fibrosis liver disease?
Authors Elidottir H, Hansen CR, Diemer S, Eklund EA
References J Cyst Fibros. 2022 Jul 3;S1569-1993(22)00596-3. doi: 10.1016/j.jcf.2022.06.009.
Keywords 2D Shear wave elastography, Cystic fibrosis liver disease screening, Liver stiffness, Vitamin D
Theme Care of patients with CF, Diagnosis/Screening, Nutrition/Gastroenterology
June 2022
Title: ivacaftor does not change gut bacteria, inflammation or pancreas function
Authors N J Ronan, G G Einarsson, J Deane, F Fouhy, M Rea, C Hill, F Shanahan, J S Elborn, R P Ross, M McCarthy, D M Murphy, J A Eustace, Tunney Mm, C Stanton, B J Plant
References J Cyst Fibros. 2022 Jun 25;S1569-1993(22)00589-6. doi: 10.1016/j.jcf.2022.06.002
Keywords G551D, Gut microbiota, Inflammation, Ivacaftor
Theme Care of patients with CF, Microbiology, Nutrition/Gastroenterology
June 2022
Title: Eruption of brown moles following Trikafta/Kaftrio treatment for cystic fibrosis
Authors Michael Atkinson, Oliver Johnson, Niall Wilson, Martin Walshaw, Thomas Simon FitzMaurice
References J Cyst Fibros. 2022 Jun 16;S1569-1993(22)00591-4. doi: 10.1016/j.jcf.2022.06.003.
Keywords Elexacaftor, Eruptive melanocytic naevi, Ivacaftor, Tezacaftor
Theme Care of patients with CF
Title: Elexacaftor-Tezacaftor-Ivacaftor (ETI) improve stomach, gullet, nose and throat symptoms in people with advanced CF
Authors Sufyan Shakir, Carlos Echevarria, Simon Doe, Malcolm Brodlie, Christopher Ward, Stephen J Bourke
References J Cyst Fibros. 2022 Jun 16;S1569-1993(22)00591-4. doi: 10.1016/j.jcf.2022.06.003.
Keywords CFTR modulators, Gastro-oesophageal reflux, Sinonasal symptoms
Theme Care of patients with CF, Nutrition/Gastroenterology
May 2022
Title: How does chronic rhinosinusitis impact quality of life of cystic fibrosis patients?
Authors Daniel J Lee, Jenna Sykes, Katherine Griffin, Christopher W Noel, Brian Hyung, Kamalprit Chokar, Christopher Mkl Yao, Elizabeth Tullis, John M Lee
References J Cyst Fibros. 2022 May 31;S1569-1993(22)00146-1. doi: 10.1016/j.jcf.2022.05.008.
Keywords Chronic rhinosinusitis, Epidemiology, Prevalence, Quality of life
Theme Care of patients with CF, Pulmonology
May 2022
Title: Long-term health effects of real-world use of ivacaftor by people with cystic fibrosis who have non-G551D-CFTR gating mutations
Authors Nicholas J. Simmonds, C. Kors van der Ent, Carla Colombo, Nils Kinnman, Cynthia DeSouza, Teja Thorat, Marci L. Chew, Keval Chandarana, Carlo Castellani
References J Cyst Fibros. 2022 May 22;S1569-1993(22)00141-2. doi: 10.1016/j.jcf.2022.05.007.
Keywords CFTR gating mutation, Healthcare resource utilization, Ivacaftor, Lung function, Real-world experience
Theme Care of patients with CF, Pulmonology
May 2022
Title: Evaluation of a biochemical cystic fibrosis newborn screening protocol using IRT and PAP
Authors Olaf Sommerburg, Mirjam Stahl, Susanne Hämmerling, Gwendolyn Gramer, Martina U Muckenthaler, Jürgen Okun, Dirk Kohlmüller, Margit Happich, Andreas E Kulozik, Marcus A Mall, Georg F Hoffmann
References J Cyst Fibros. 2022 May;21(3):422-433. doi: 10.1016/j.jcf.2021.10.007. Epub 2021 Nov 9.
Keywords Biochemical screening, genetic testing, Immuno reactive trypsinogene (IRT), Newborn screening (NBS), Pancreatitis associated protein (PAP)
Theme False-positives, Newborn screening
May 2022
Title: Adjusting the dose of triple therapy (Elexacaftor/Tezacaftor/Ivacaftor) to reduce mental health side effects
Authors Spoletini G, Gillgrass L, Pollard K, Shaw N, Williams E, Etherington C, Clifton Ij, Peckham Dg
References J Cyst Fibros. 2022 May 15;S1569-1993(22)00111-4. doi: 10.1016/j.jcf.2022.05.001. Online ahead of print.
Theme Care of patients with CF, Psychosocial, Pulmonology
May 2022
Title: A rapid sweat test for chloride and bicarbonate to confirm the diagnosis of infants with cystic fibrosis
Authors Stellena Mathiaparanam, Adriana Nori de Macedo, Biban Gill, Katherine Keenan, Tanja Gonska, Linda Pedder, Stephen Hill, Philip Britz-McKibbin
References J Cyst Fibros. 2022 May 13;S1569-1993(22)00112-6. doi: 10.1016/j.jcf.2022.05.004.
Keywords Bicarbonate, Capillary electrophoresis, Chloride, Confirmatory testing, Coulometric titration, Newborn screening, pilocarpine iontophoresis, Sweat
Theme Diagnosis/Screening
May 2022
Title: Breakdown of lung proteins causes structural damage in cystic fibrosis lungs
Authors Meghan R Pinezich, Manuel A Tamargo, Sharon Fleischer, Jonathan A Reimer, Maria R Hudock, Ahmed E Hozain, Sarah R Kaslow, Yuliya Tipograf, Rajesh Kumar Soni, Olimpia P Gavaudan, Brandon A Guenthart, Charles C Marboe, Matthew Bacchetta, John D O'Neill, N V
References J Cyst Fibros. 2022 May 4;S1569-1993(22)00106-0. doi: 10.1016/j.jcf.2022.04.016.
Keywords Basement membrane, Biomarkers, Extracellular matrix, Lung proteases
Theme Imaging, Lung Disease and Infection
Title: Patients with F508del/5T;TG12 CFTR genotype
Authors Antonella Tosco, Alice Castaldo, Carla Colombo, Laura Claut, Vincenzo Carnovale, Paola Iacotucci, Marco Lucarelli, Giuseppe Cimino, Benedetta Fabrizzi, Nicole Caporelli, Fabio Majo, Fabiana Ciciriello, Rita Padoan, Piercarlo Poli,, Giovanni Taccetti, Claudia Centrone, Rosaria Casciaro, Carlo Castellani, Donatello Salvatore, Carmela Colangelo, Paolo Bonomi, Giuseppe Castaldo, Vito Terlizzi
References J Cyst Fibros. 2022 May 4;S1569-1993(22)00109-6. doi: 10.1016/j.jcf.2022.04.020.
Keywords CBAVD, CFTR-RD, CRMS/CFSPID, FEV1, Outcomes, Spirometry
Theme Care of patients with CF, Diagnosis/Screening
May 2022
Title: Respiratory viral infections and chest CT (computer tomography scan) changes in infants with CF
Authors Don B Sanders, Ashley R Deschamp, Joseph E Hatch, James E Slaven, Netsanet Gebregziabher, Mariette Kemner-van de Corput, Harm A W M Tiddens, Tim Rosenow, Gregory A Storch, Graham L Hall, Stephen M Stick, Sarath Ranganathan, Thomas W Ferkol, Stephanie D D
References J Cyst Fibros. 2022 May 3;S1569-1993(22)00103-5. doi: 10.1016/j.jcf.2022.04.014.
Keywords Chest computed tomography, Newborn screening, Respiratory infection, Viruses
Theme Care of patients with CF, Imaging, Lung Disease and Infection
April 2022
Title: Use of the CFTR modulator combination elexacaftor/tezacaftor/ivacaftor after lung transplant
Authors Kathleen J. Ramos, Jennifer S. Guimbellot, Maryam Valapour, Lauren E. Bartlett, Travis Hee Wai, Joseph M. Pilewski, Albert Faro, Joshua M. Diamond for the CFLTC Study Group
References J Cyst Fibros. 2022 Apr 23;S1569-1993(22)00098-4. doi: 10.1016/j.jcf.2022.04.009.
Keywords Cystic fibrosis transmembrane conductance regulator, Lung transplantation, Trikafta
Theme Care of patients with CF, Diabetes, Lung Disease and Infection, Nutrition/Gastroenterology, Transplantation
April 2022
Title: How the number of kinds of antibiotics chosen to treat Pseudomonas during an exacerbation affect recovery
Authors D.R. VanDevanter, N.E. West, D.B. Sanders, M. Skalland, C.H. Goss, P.A. Flume, S.L. Heltshe
References J Cyst Fibros. 2022 Apr 21;S1569-1993(22)00096-0. doi: 10.1016/j.jcf.2022.04.006.
Keywords antipseudomonal classes, P. aeruginosa, Pulmonary exacerbation
Theme Care of patients with CF, Lung Disease and Infection, Pulmonology
April 2022
Title: Coping and Length of Life in Cystic Fibrosis
Authors Janice Abbott, Margaret A. Hurley, Helen Chadwick, Daniel Peckham
References J Cyst Fibros. 2022 Apr 20;S1569-1993(22)00100-X. doi: 10.1016/j.jcf.2022.04.011.
Keywords Cox model, Coping, optimism, Survival
Theme Care of patients with CF, Psychosocial
April 2022
Title: Current Prices versus Minimum Costs of Production for CFTR Modulators
Authors Jonathan Guo, Junzheng Wang, Jingchun Zhang, Joseph Fortunak, Andrew Hill
References J Cyst Fibros. 2022 Apr 16;S1569-1993(22)00090-X. doi: 10.1016/j.jcf.2022.04.007.
Keywords CFTR modulator, ; Cystic fibrosis transmembrane conductance regulator, Elexacaftor/tezacaftor/ivacaftor, Kaftrio, Trikafta
Theme Care of patients with CF, Psychosocial
Title: How ivacaftor changes inflammation and protein levels in the bloodstream of people with CF and the G551D mutation
Authors Jordana E. Hoppe, Brandie D. Wagner, J. Kirk Harris, Steven M. Rowe, Sonya L Heltshe, Emily M. DeBoer, Scott D. Sagel
References J Cyst Fibros. 2022 Apr 16;S1569-1993(22)00089-3. doi: 10.1016/j.jcf.2022.03.012.
Keywords CFTR modulation, Extrapulmonary, Inflammation, Proteomics
Theme Care of patients with CF, Endocrinology, Nutrition/Gastroenterology, Pulmonology
April 2022
Title: Inflammation and infection change what mucus is made of, how much gets made, and how much it can dissolve
Authors Matthew R Markovetz, Ian C Garbarine, Cameron B Morrison, William J Kissner, Ian Seim, M Gregory Forest, Micah J Papanikolas, Ronit Freeman, Agathe Ceppe, Andrew Ghio, Neil E Alexis, Stephen M Stick, Camille Ehre, Richard C Boucher, Charles R Esther, Marianne S Muhlebach, David B Hill
References J Cyst Fibros. 2022 Apr 15;S1569-1993(22)00097-2. doi: 10.1016/j.jcf.2022.04.008.
Keywords Airway mucus, Gel-forming mucins, Infection, Microrheology, Mucus flakes
Theme CFTR/Basic Science, Lung Disease and Infection
April 2022
Title: Immune response after COVID-19 vaccination in cystic fibrosis patients
Authors Athanasios Michos, Filippos Filippatos, Elisabeth-Barbara Tatsi, Charilaos Dellis, Vasiliki Efthymiou, Ioanna Zarkada, Evgenia Troupi, Vasiliki Syriopoulou, Ioanna Loukou
References J Cyst Fibros. 2022 Apr 14;S1569-1993(22)00094-7. doi: 10.1016/j.jcf.2022.04.004.
Keywords Antibody, BNT162b2, COVID-19, Neutralizing, SARS-CoV-2, Vaccine
Theme Care of patients with CF, Immunology
April 2022
Title: Maternal Trikafta use during pregnancy and breastfeeding improved outcomes for a baby with CF
Authors Sylvia Szentpetery, Kimberly Foil, Sara Hendrix, Sue Gray, Christina Mingora, Barbara Head, Donna Johnson, Patrick A Flume
References J Cyst Fibros. 2022 Apr 11;S1569-1993(22)00095-9. doi: 10.1016/j.jcf.2022.04.005.
Keywords CFTR modulators, pregnancy
Theme Care of patients with CF, Parents, Pregnancy with CF
April 2022
Title: Magnetic Resonance Imaging Detects Improvements of Pulmonary and Paranasal Sinus Abnormalities in Response to Elexacaftor/Tezacaftor/Ivacaftor Therapy in Adults with Cystic Fibrosis
Authors Lena Wucherpfennig, Simon M. F. Triphan, Sabine Wege, Hans-Ulrich Kauczor, Claus P. Heussel, Niclas Schmitt, Felix Wuennemann, Victoria L. Mayer, Olaf Sommerburg, Marcus A. Mall, Monika Eichinger, Mark O. Wielpütz
References J Cyst Fibros. 2022 Apr 7;S1569-1993(22)00088-1. doi: 10.1016/j.jcf.2022.03.011.
Keywords Airway disease, Functional MRI, Lung perfusion, Mucus obstruction, CFTR-modulator
March 2022
Title: High blood eosinophil counts are linked with worse symptoms in adults with cystic fibrosis
Authors Si Cong Ye, Sameer Desai, Emma Karlsen, Eugenie Kwong, Pearce G. Wilcox, Bradley S. Quon
References J Cyst Fibros. 2022 Mar 31;S1569-1993(22)00086-8. doi: 10.1016/j.jcf.2022.03.009.
Keywords Biomarkers, Eosinophils, Outcomes, Patient-reported symptoms
Theme Biomarkers, Care of patients with CF, Lung Disease and Infection
March 2022
Title: Club cell secretory protein is reduced in children with cystic fibrosis who have severe lung disease
Authors Jing Zhai, Mary J Emond, Amber Spangenberg, Debra A Stern, Monica M Vasquez, Elizabeth E Blue, Kati J Buckingham, Duane L Sherrill, Marilyn Halonen, Ronald L Gibson, Margaret Rosenfeld, Scott D Sagel, Michael J Bamshad, Wayne J Morgan, Stefano Guerra
References J Cyst Fibros. 2022 Mar 30;S1569-1993(22)00084-4. doi: 10.1016/j.jcf.2022.03.007.
Keywords Club cell secretory protein, Lung function
Theme Biomarkers, Care of patients with CF, Lung Disease and Infection
March 2022
Title: What makes lung function fall between childhood and adolescence
Authors Nelufa Begum, Catherine A Byrnes, Joyce Cheney, Peter J Cooper, Emmanuelle Fantino, Nicholas Gailer, Keith Grimwood, Diana GutierrezCardenas, John Massie, Colin F Robertson, Peter D Sly, Harm Awm Tiddens, Claire E Wainwright, Robert S Ware
References J Cyst Fibros. 2022 Mar 24;S1569-1993(22)00085-6. doi: 10.1016/j.jcf.2022.03.008.
Keywords ACFBAL study, Aspergillus, CF-FAB study, Hospitalisation, Neutrophilic inflammation, Respiratory exacerbations
Theme Care of patients with CF, Lung Disease and Infection, Pulmonology
March 2022
Title: Cystic fibrosis liver disease – A clinical challenge in the era of improved outcomes.
Authors Ao Sasame, Diarmuid Stokes, Billy Bourke, Lucy Connolly, Emer Fitzpatrick, Marion Rowland
References J Cyst Fibros. 2022 Mar;21(2):202-211. doi: 10.1016/j.jcf.2021.07.014. Epub 2021 Aug 8.
Keywords Cystic fibrosis liver disease, mortality, Outcome, Systematic review
Theme Care of patients with CF, Nutrition/Gastroenterology
Title: Serodiagnostic as a complement to bacterial culture in identification of Mycobacterium abscessus infections in cystic fibrosis patients
Authors Vincent Le Moigne , Anne-Laure Roux, Hélène Mahoudo, Gaëtan Christien, Agnès Ferroni, Oana Dumitrescu, Gérard Lina, Jean-Philippe Bouchara, Patrick Plésiat, Jean-Louis Gaillard, Stéphane Canaan, Geneviève Héry-Arnaud, Jean-Louis Herrmann
References J Cyst Fibros. 2022 Mar;21(2):353-360. doi: 10.1016/j.jcf.2021.08.019. Epub 2021 Sep 10.
Keywords ELISA, Non-tuberculous mycobacteria, Serodiagnosis, Serology
Theme Biomarkers, Lung Disease and Infection, Microbiology
Title: Multi-Organ MRI in Cystic Fibrosis
Authors Kimberly McBennett, Christina J MacAskill, Elise Keshock, Maryam Ghadimi Mahani, Jaime Mata, Alexander J Towbin, Senthilkumar Sankararaman, Mitchell L Drumm, Xin Yu, Clement L Ren, Samya Z Nasr, Katherine Kutney, Chris A Flask
References J Cyst Fibros. 2022 Mar;21(2):e148-e157. doi: 10.1016/j.jcf.2021.11.006.
Keywords Heart, kidney, liver, lung, Magnetic Resonance Imaging, pancreas, Review
Theme Care of patients with CF, Imaging
March 2022
Title: Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal sinus in cystic fibrosis
Authors Amanda L Stapleton, Adam J Kimple, Jennifer L Goralski, S Mehdi Nouraie, Barton F Branstetter, Amber D Shaffer, Joseph M Pilewski, Brent A Senior, Stella E Lee, Anna C Zemke
References J Cyst Fibros. 2022 Mar 14;S1569-1993(22)00051-0. doi: 10.1016/j.jcf.2022.03.002.
Keywords Highly effective modulator therapy, Sinus disease, Sinusitis
Theme Care of patients with CF
March 2022
Title: Reduced levels of nitric oxide in cystic fibrosis airways may be caused by interleukin-1 beta
Authors Gyde Nissen, Elad Ben-Meir, Matthias Kopp, Michelle Shaw, Felix Ratjen, Hartmut Grasemann
References J Cyst Fibros. 2022 Mar 5;S1569-1993(22)00049-2. doi: 10.1016/j.jcf.2022.02.017.
Keywords Airway inflammation, Ivacaftor, Nitric oxide
Theme Care of patients with CF, Immunology
March 2022
Title: Intracranial mucous benign cyst causing bulging eye in a child with cystic fibrosis: a case report.
Authors Cristina de Manuel Gómez, Ana Cecilia Morales Palacios, Olga de la Serna Blázquez, Pablo Vicente Morillo Carnero, María Elena Pérez Arenas, Sonia Milkova Ivanova, Marta Ruiz de Valbuena Maiz
References J Cyst Fibros. 2022 Mar 1;S1569-1993(22)00048-0. doi: 10.1016/j.jcf.2022.02.016.
Keywords Mucocele, Mucopyocele, Proptosis, Escherichia coli
Theme Care of patients with CF
February 2022
Title: Evaluation of a novel compound, AZD5634, in healthy volunteers and patients with cystic fibrosis
Authors Cecilia Kristensson, Annika Åstrand, Scott Donaldson, Ron Goldwater, Raolat Abdulai, Naimish Patel, Philip Gardiner, Ulrika Tehler, Anne-Kristina Mercier, Marita Olsson, Eva Ersdal, Jukka Mäenpää, Tobias Bramer, Anna Malmgren, William Bennett,, Christina Keen
References J Cyst Fibros. 2022 Feb 26;S1569-1993(22)00042-X. doi: 10.1016/j.jcf.2022.02.010.
Keywords ENaC inhibitors, clinical trials, Mucociliary clearance, Pharmacokinetics
Theme Clinical Trials, Pulmonology
February 2022
Title: The impact of the first wave of the COVID-19 pandemic on people with cystic fibrosis in New York
Authors Joseph L Simonson, Christine Esposito, Theresa Frantzen, Katherine Henthorne, Aileen Espinal, Serena Romano, Ramona Ramdeo, Jessica Trentacoste, Donna Tsang, Geralyn LaVecchia, Robert Abdullah, Maria Berdella, Lynn Bonitz, Rany Condos,, Andrei Constantinescu, Joan K DeCelie-Germana, Emily DiMango, Myah Draine, Tara Gimeli, Robert Giusti, Jessenia Guzman, Soumia Hammouda, Claire Keating, Catherine Kier, Alison T Lennox, Carmen Liriano, Zachary Messer, Amy Plachta, Hossein Sadeghi, , Elinor Schwind, Teresa Stables-Carney, Patricia Walker, Janice Wang
References J Cyst Fibros. 2022 Feb 21;S1569-1993(22)00044-3. doi: 10.1016/j.jcf.2022.02.012.
Keywords Coronavirus, Covid-19 SARS-CoV2
Theme Care of patients with CF, Lung Disease and Infection, Psychosocial
February 2022
Title: Human airway cell models to study genes contributing to CF lung disease
Authors Gengming He, Naim Panjwani, Julie Avolio, Hong Ouyang, Shaf Keshavjee, Johanna M. Rommens, Tanja Gonska, Theo J. Moraes, Lisa J. Strug
References J Cyst Fibros. 2022 Feb 18;S1569-1993(22)00039-X. doi: 10.1016/j.jcf.2022.02.007.
Keywords Co-expression relationships, Cystic fibrosis modifier genes, Differential expression analysis, Epithelia, Equivalence testing
Title: Tezacaftor/Ivacaftor in Children ≥6 Years of Age With F508del and Residual Function Changes in CFTR.
Authors Gregory S Sawicki, Mark Chilvers, John McNamara, Lutz Naehrlich, Clare Saunders, Isabelle Sermet-Gaudelus, Claire E Wainwright, Neil Ahluwalia, Daniel Campbell, R Scott Harris, Hildegarde Paz-Diaz, Judy L Shih, Jane C Davies
References J Cyst Fibros. 2022 Feb 18;S1569-1993(22)00033-9. doi: 10.1016/j.jcf.2022.02.003.
Keywords Ivacaftor, Tezacaftor
Theme Care of patients with CF, Clinical Trials, Pulmonology
February 2022
Title: Measuring CFTR function and relating it to health.
References J Cyst Fibros. 2022 Feb 17;S1569-1993(22)00035-2. doi: 10.1016/j.jcf.2022.02.001.
Theme Care of patients with CF
February 2022
Title: Health Changes in People with CF the Year After Having a Child
Authors Traci M. Kazmerski, Raksha Jain, MinJae Lee, Jennifer L. Taylor-Cousar
References J Cyst Fibros. 2022 Feb 15;S1569-1993(22)00038-8. doi: 10.1016/j.jcf.2022.02.006.
Keywords Fatherhood, Motherhood, Parenthood, pregnancy
Theme Care of patients with CF, Pregnancy with CF, Pulmonology
February 2022
Title: Men’s Sexual and Reproductive Health in Cystic Fibrosis in the Era Highly Effective Modulator Therapies: A Qualitative Study
Authors Alison R Clarke, Olivia M Stransky, Miriam Bernard, Kara S Hughan, Sigrid Ladores, Gregory S Sawicki, Michael S Stalvey, Traci M Kazmerski
References J Cyst Fibros. 2022 Feb 12;S1569-1993(22)00034-0. doi: 10.1016/j.jcf.2022.02.002.
Keywords Assisted reproductive technology, Infertility, Masculinity, Sexual and reproductive health
Theme Care of patients with CF, Psychosocial
Title: CFTR modulator therapy alters sphingolipid profiles in blood from people with cystic fibrosis
Authors Dirk Westhölter, Fabian Schumacher, Nuria Wülfinghoff, Sivagurunathan Sutharsan, Svenja Strassburg, Burkhard Kleuser, Peter A Horn, Sebastian Reuter, Erich Gulbins, Christian Taube1, Matthias Welsner
References J Cyst Fibros. 2022 Feb 12;S1569-1993(22)00037-6. doi: 10.1016/j.jcf.2022.02.005.
Keywords CFTR modulator therapy, Ceramide, Elexacaftor/tezacaftor/ivacaftor, Plasma sphingolipids
Theme Nutrition/Gastroenterology
Title: Cystic fibrosis outcomes with Tricare Insurance
Authors Joseph M Collaco, Lori L Vanscoy, Kevin J Psoter, Kristin A Riekert, Kimberly M Dickinson
References J Cyst Fibros. 2022 Feb 12;S1569-1993(22)00036-4. doi: 10.1016/j.jcf.2022.02.004.
Theme Care of patients with CF, Psychosocial
February 2022
Title: Worldwide Estimates of Cystic Fibrosis Diagnosis and Treatment Access
Authors Jonathan Guo, Anna Garratt, Andrew Hill
References J Cyst Fibros. 2022 Feb 3;S1569-1993(22)00031-5. doi: 10.1016/j.jcf.2022.01.009.
Keywords CFTR modulator, Cystic fibrosis transmembrane conductance regulator, Epidemiology, Ivacaftor/tezacaftor/elexacaftor, Treatment cascade, Trikafta
Theme Care of patients with CF, Diagnosis/Screening, Epidemiology/Models of care
February 2022
Title: Elexacaftor-tezacaftor-ivacaftor provides continued health improvement in lung transplant candidates with cystic fibrosis
Authors Clémence Martin, Martine Reynaud-Gaubert, Rebecca Hamidfar, Isabelle Durieu, Marlène Murris-Espin, Isabelle Danner-Boucher, Raphaël Chiron, Sylvie Leroy, Benoit Douvry, Dominique Grenet, Laurent Mely, Sophie Ramel, Sylvie Montcouquiol, Lydie Lemonnier, , Espérie Burnet, Jean-Louis Paillasseur, Jennifer Da Silva, Pierre-Régis Burgel
References J Cyst Fibros. 2022 Feb 2;S1569-1993(22)00032-7. doi: 10.1016/j.jcf.2022.01.012.
Theme Care of patients with CF, Transplantation
January 2022
Title: Use of swelling tests in mini-guts to help determine eligibility for treatment with Trikafta for a CF patient with a rare mutation
Authors B L Aalbers, J E Brunsveld, C K van der Ent, J C van den Eijnden, J M Beekman, H G M Heijerman
References J Cyst Fibros. 2022 Jan 30;S1569-1993(22)00028-5. doi: 10.1016/j.jcf.2022.01.008.
Theme Care of patients with CF
January 2022
Title: Measuring how the drug elexacaftor/tezacaftor/ivacaftor affects the muscles of breathing, using dynamic chest radiography
Authors Thomas S FitzMaurice, Caroline McCann, Dilip Nazareth, Matthew Shaw, Paul S McNamara, Martin J Walshaw
References J Cyst Fibros. 2022 Jan 28;S1569-1993(22)00027-3. doi: 10.1016/j.jcf.2022.01.007.
Keywords CFTR modulators, Cystic fibrosis in adults, chest imaging, Diaphragm, dynamic chest radiography, Pulmonary physiology
Theme Care of patients with CF, Imaging, Pulmonology
January 2022
Title: Patient and program characteristics which influence screening for bone mineral disease in people with Cystic Fibrosis
Authors Gregory A Ratti, Gabriela S Fernandez, Michael S Schechter, Michael S Stalvey, Josh Ostrenga, Aliza K Fink, Raksha Jain
References J Cyst Fibros. 2022 Jan 24;S1569-1993(22)00029-7. doi: 10.1016/j.jcf.2022.01.011.
Keywords Cystic fibrosis related bone disease, DEXA scan, Osteopenia, Osteoporosis
Theme Bone health, Care of patients with CF, Diagnosis/Screening
Title: Results from a clinical trial of the natural therapeutic alginate OligoG against Burkholderia lung infection in people with cystic fibrosis.
Authors Rianald Fischer, Carsten Schwarz, Rebecca Weiser, Eshwar Mahenthiralingam, Knut Smerud, Nils Meland, Hugo Flaten, Philip D Rye
References J Cyst Fibros. 2022 Jan 24;S1569-1993(22)00024-8. doi: 10.1016/j.jcf.2022.01.003.
Keywords Alginate oligosaccharide, Burkholderia cepacia complex (Bcc) bacteria, FEV1, OligoG
Theme Clinical Trials, Lung Disease and Infection, Microbiology
January 2022
Title: Using safe sampling and DNA tests to diagnose lung infections in children with cystic fibrosis
Authors Rebecca Weiser, Juliette Oakley, Katherine Ronchetti, Jo-Dee Tame, Sven Hoehn, Tomasz P Jurkowski, Eshwar Mahenthiralingam, Julian T Forton
References J Cyst Fibros. 2022 Jan 22;S1569-1993(22)00023-6. doi: 10.1016/j.jcf.2022.01.006.
Keywords Induced sputum, Microbiome, microbiota, Sputum induction
Theme Care of patients with CF, Lung Disease and Infection, Microbiology
January 2022
Title: Description of children who changed diagnosis from indeterminate newborn screening to cystic fibrosis
Authors Daniella Ginsburg, Choo Phei Wee, Maria Carmen Reyes, John J Brewington, Danieli B Salinas
References J Cyst Fibros. 2022 Jan;21(1):e23-e27. doi: 10.1016/j.jcf.2021.06.012.
Keywords CFSPID, CRMS, Diagnosis, Newborn screening, reclassification, sweat chloride
Title: Comparing four different definitions of chronic pseudomonas infection in people with cystic fibrosis
Authors Margaret Rosenfeld, Anna V Faino, Frankline Onchiri, Melis A Aksit, Scott M Blackman, Elizabeth E Blue, Joseph M Collaco, William W Gordon, Rhonda G Pace, Karen S Raraigh, Yi-Hui Zhou, Garry R Cutting, Michael R Knowles, Michael J Bamshad, Ronald L Gibson
References J Cyst Fibros. 2022 Jan;21(1):40-44. doi: 10.1016/j.jcf.2021.07.020. Epub 2021 Aug 13.
Keywords Pseudomonas aeruginosa, Chronic infection, definitions
Theme Epidemiology/Models of care, Lung Disease and Infection, Microbiology
January 2022
Title: Guidance for Sweat Testing in Europe
Authors N Cirilli, K W Southern, J Barben, F Vermeulen, A Munck, M Wilschanski, Thao Nguyen-Khoa, M Aralica, N J Simmonds, E De Wachter, ECFS Diagnostic Network Working Group
References J Cyst Fibros. 2022 Jan 19;S1569-1993(22)00025-X. doi: 10.1016/j.jcf.2022.01.004.
Keywords CF diagnosis, Conductivity, QPIT, sweat chloride, sweat testing, Sweat test
Theme Diagnosis/Screening
January 2022
Title: Wrinkling of the palms on exposure to water in cystic fibrosis patients treated with ivacaftor
Authors Eyal Jacobi, Melinda Solomon, Julie Avolio, Michelle Shaw, Tanja Gonska, Felix Ratjen, Hartmut Grasemann
References J Cyst Fibros. 2022 Jan 18;S1569-1993(22)00026-1. doi: 10.1016/j.jcf.2022.01.005.
Keywords Aquagenic wrinkling, Ivacaftor
January 2022
Title: Exploring health care provider attitudes and perspectives related to men’s sexual and reproductive health in cystic fibrosis
Authors Alison R Clarke, Olivia M Stransky, Miriam Bernard, Kara S Hughan, Sigrid Ladores, Gregory S Sawicki, Michael Stalvey, Traci M Kazmerski
References J Cyst Fibros. 2022 Jan 5;S1569-1993(21)02176-7. doi: 10.1016/j.jcf.2021.12.016.
Keywords Assisted reproductive technology, Infertility, Men's health, Sexual and reproductive health
Theme Care of patients with CF, Psychosocial
January 2022
Title: Neutrophils do not produce more oxidants when stimulated to fight infections in cystic fibrosis
Authors Dean Kelk, Jayden Logan, Isabella Andersen, Diana Gutierrez Cardenas, Scott C Bell, Claire E Wainwright, Peter D Sly, Emmanuelle Fantino
References J Cyst Fibros. 2022 Jan 3;S1569-1993(21)02175-5. doi: 10.1016/j.jcf.2021.12.015.
Keywords Adults, Children, Inflammation, oxidative stress, Reactive oxygen species, hsCRP
Theme Lung Disease and Infection
December 2021
Title: Treatment of Idiopathic Chronic Pancreatitis (ICP) with ivacaftor in a Cystic Fibrosis (CF) carrier who also had a second inherited risk factor for pancreatitis called Methylmalonic Acidemia
Authors Tin-Yun Tang, Vincent B Cruz, Laura L Konczal
References J Cyst Fibros. 2021 Dec 30;S1569-1993(21)02174-3. doi: 10.1016/j.jcf.2021.12.013.
Theme Nutrition/Gastroenterology
December 2021
Title: Antisense oligonucleotide as a novel therapy for people with CF affected by the W1282X mutation
Authors Yifat S. Oren, Ofra Avizur-Barchad, Efrat Ozeri-Galai, Renana Elgrabli, Meital R. Schirelman, Tehilla Blinder, Chava D. Stampfer, Merav Ordan, Onofrio Laselva, Malena Cohen-Cymberknoh, Eitan Kerem, Christine E Bear, Batsheva Kerem
References J Cyst Fibros. 2021 Dec 28;S1569-1993(21)02172-X. doi: 10.1016/j.jcf.2021.12.012.
Keywords Antisense oligonucleotides, drug development, Exon skipping
Title: Is Body Mass Index Associated with Better Lung Function in Children with Pancreatic-Sufficient Cystic Fibrosis?
Authors Ankitha Madde, William Okoniewski, Don B Sanders, Clement L Ren, Daniel J Weiner, Erick Forno
References J Cyst Fibros. 2021 Dec 28;S1569-1993(21)02173-1. doi: 10.1016/j.jcf.2021.12.014.
Keywords BMI, FEV1, Lung function, Nutrition, pancreatic insufficiency
Theme Care of patients with CF, Lung Disease and Infection, Nutrition/Gastroenterology
December 2021
Title: Looking at lung disease over time in young children with CF
Authors Alan S Brody, Rui Huang, Bin Zhang, Frederick R Long, Scott W Powers
References J Cyst Fibros. 2021 Dec 24;S1569-1993(21)02168-8. doi: 10.1016/j.jcf.2021.12.009.
Theme Imaging, Lung Disease and Infection, Pulmonology
Title: Looking at lung disease over time in young children with CF
Authors Alan S Brody, Rui Huang, Bin Zhang, Frederick R Long, Scott W Powers
References J Cyst Fibros. 2021 Dec 24;S1569-1993(21)02168-8. doi: 10.1016/j.jcf.2021.12.009.
Keywords Bronchiectasis, Child, Computed tomography, Outcome measures, Pulmonary cystic fibrosis, Preschool
Title: Results of a clinical evaluation of the safety and efficacy of the therapeutic drug candidate Roscovitine in people with cystic fibrosis.
Authors Laurent Meijer, Geneviève Hery-Arnaud, Cyril Leven, Emmanuel Nowak, Sophie Hillion, Yves Renaudineau, Isabelle Durieu, Raphaël Chiron, Anne Prevotat, Isabelle Fajac, Dominique Hubert, Marlène Murris-Espin, Sandrine Huge, Isabelle Danner-Boucher, , Bruno Ravoninjatovo, Sylvie Leroy, Julie Macey, Thierry Urban, Gilles Rault, Dominique Mottier, Rozenn Le Berre
References J Cyst Fibros. 2021 Dec 24;S1569-1993(21)02167-6. doi: 10.1016/j.jcf.2021.10.013.
Keywords Clinical trial, Inflammation, Pseudomonas aeruginosa, Roscovitine, Seliciclib
Theme Lung Disease and Infection, Microbiology
December 2021
Title: A simple blood test to detect how severe liver disease is in children with CF
Authors Diego A Calvopina, Peter J Lewindon, Louise E Ramm, Charlton Noble, Gunter F Hartel, Daniel H Leung, Grant A Ramm
References J Cyst Fibros. 2021 Dec 22;S1569-1993(21)02171-8. doi: 10.1016/j.jcf.2021.10.014.
Keywords Children, cystic fibrosis-associated liver disease, Liver fibrosis, multilobular cirrhosis, Serum biomarker
Theme Biomarkers, Nutrition/Gastroenterology
December 2021
Title: Advanced but not mild liver disease is a predictor of decreased survival in children with cystic fibrosis, with far greater impact in females: A 27-year real-life cohort study
Authors Marilisa Baldissera, Peter J Lewindon, Louise E Ramm, Gunter F Hartel, Rita Mattiello, Grant A Ramm
References J Cyst Fibros. 2021 Dec 20;S1569-1993(21)02156-1. doi: 10.1016/j.jcf.2021.11.015.
Keywords Children, Cohort studies, liver disease, mortality
Theme Diabetes, Nutrition/Gastroenterology
Title: Improving conclusions after newborn screening for babies with no clear diagnosis
Authors Aurelie Hatton, Anne Bergougnoux, Katarzyna Zybert, Benoit Chevalier, Myriam Mesbahi, Jean Pierre Altéri, Katarzyna Walicka-Serzysko, Magdalena Postek, Magali Taulan-Cadars, Aleksander Edelman, Alexandre Hinzpeter, Mireille Claustres, Emmanuelle Girodon, , Caroline Raynal, Isabelle Sermet-Gaudelus, Dorota Sands
References J Cyst Fibros. 2021 Dec 20;S1569-1993(21)02169-X. doi: 10.1016/j.jcf.2021.12.010
Keywords CFTR, Functional tests, neonatal screening
December 2021
Title: Measuring inflammation in blood during exacerbation treatment
Authors D R VanDevanter, S L Heltshe, M Skalland, N E West, D B Sanders, C H Goss, P A Flume
References J Cyst Fibros. 2021 Dec 18;S1569-1993(21)02160-3. doi: 10.1016/j.jcf.2021.12.003.
Keywords C-reactive protein, Clinical response, Pulmonary exacerbation
Theme Biomarkers, Care of patients with CF, Lung Disease and Infection, Pulmonology
December 2021
Title: Magnetic resonance imaging of the gastrointestinal tract shows reduced small bowel motility and altered chyme in cystic fibrosis compared to controls
Authors Neele S Dellschaft, Christabella Ng, Caroline Hoad, Luca Marciani, Robin Spiller, Iain Stewart, Alex Menys, Helen Barr, Penny A Gowland, Giles Major, Alan R Smyth
References J Cyst Fibros. 2021 Dec 15;S1569-1993(21)02166-4. doi: 10.1016/j.jcf.2021.12.007.
Keywords Gastrointestinal tract, Magnetic Resonance Imaging
Theme Imaging, Nutrition/Gastroenterology
Title: Nutritional health and lung transplantation in cystic fibrosis
Authors Ann L. Jennerich, Joseph B. Pryor, Travis Y. Hee Wai, Siddhartha G. Kapnadak, Moira L. Aitken, Christopher H. Goss, Kathleen J. Ramos
References J Cyst Fibros. 2021 Dec 15;S1569-1993(21)02159-7. doi: 10.1016/j.jcf.2021.12.001.
Keywords Lung diseases, Lung transplantation, nutritional status, Survival analysis
Theme Care of patients with CF, Nutrition/Gastroenterology, Transplantation
December 2021
Title: Two cases of heart attacks in young women with cystic fibrosis and diabetes
Authors Sirisha Reddy Thambuluru, Sayyad Kyazimzade, Katherine A Despotes, Deepa Kirk, Jennifer L Goralski
References J Cyst Fibros. 2021 Dec 12;S1569-1993(21)02154-8. doi: 10.1016/j.jcf.2021.11.013.
Keywords Coronary artery disease, Cystic fibrosis related diabetes, Dyslipidemia, myocardial infarction
December 2021
Title: Bacterial communities relate to gut function and transit in the cystic fibrosis gut
Authors Ryan Marsh, Helen Gavillet, Liam Hanson, Christabella Ng, Mandisa Mitchell-Whyte, Giles Major, Alan R Smyth, Damian Rivett, Christopher van der Gast
References J Cyst Fibros. 2021 Dec 8;S1569-1993(21)02155-X. doi: 10.1016/j.jcf.2021.11.014.
Keywords Antibiotics, Dysbiosis, Gut microbiome, Gut microbiota, Intestinal physiology, MRI
Theme Imaging, Microbiology, Nutrition/Gastroenterology
December 2021
Title: A new path for CF clinical trials through the use of historical controls
Authors Amalia S Magaret, Mark Warden, Noah Simon, Sonya Heltshe, George Z Retsch-Bogart, Bonnie W Ramsey, Nicole Mayer-Hamblett
References J Cyst Fibros. 2021 Dec 5;S1569-1993(21)02138-X. doi: 10.1016/j.jcf.2021.11.007.
Keywords Clinical development, External controls, Pulmonary exacerbations, Real world data
Theme Clinical Trials, Epidemiology/Models of care
November 2021
Title: Effects of CFTR Modulators on Metabolic Parameters
Authors Max C Petersen, Lauren Begnel, Michael Wallendorf, Marina Litvin
References J Cyst Fibros. 2021 Nov 30;S1569-1993(21)02153-6. doi: 10.1016/j.jcf.2021.11.012
Keywords BMI, Body weight, CFRD, Elexacaftor-tezacaftor-ivacaftor, metabolism, obesity
Theme Care of patients with CF, Diabetes, Endocrinology, Nutrition/Gastroenterology
November 2021
Title: Outcomes after IV antibiotics for pulmonary exacerbations at home or in the hospital
Authors D B Sanders, U Khan, S L Heltshe, M Skalland, N E West, D R VanDevanter, C H Goss, P A Flume
References J Cyst Fibros. 2021 Nov 29;S1569-1993(21)02151-2. doi: 10.1016/j.jcf.2021.11.009.
Keywords Antimicrobials, clinical trials, Exacerbations
Theme Care of patients with CF, Lung Disease and Infection
November 2021
Title: Using inhaled aztreonam in the treatment of exacerbations of cystic fibrosis
Authors Freddy Frost, Gregory R Young, Laura Wright, Nahida Miah, Darren L Smith, Craig Winstanley, Martin J Walshaw, Joanne L Fothergill, Dilip Nazareth
References J Cyst Fibros. 2021 Nov;20(6):994-1002. doi: 10.1016/j.jcf.2020.12.012. Epub 2021 Jan 7.
Theme Care of patients with CF, Lung Disease and Infection
Title: Changes in inpatient episodes following the introduction of modulator therapy across different age groups in the USA
Authors Nga TQ. Nguyen, Grainne Crealey, Ciaran O'Neill
References J Cyst Fibros. 2021 Nov;20(6):e114-e117. doi: 10.1016/j.jcf.2021.08.003. Epub 2021 Sep 25.
November 2021
Title: Evaluating the sodium status in relation to your age will be possible with a single spot urine.
Authors Dimitri Declercq, Lieselot Peremans, Michiel Glorieus, Yannick Vande Weygaerde, Heidi Schaballie, Eva Van Braeckel, Evelien Snauwaert, Sabine Van Daele, Stephanie Van Biervliet
References J Cyst Fibros. 2021 Nov 18;S1569-1993(21)02130-5. doi: 10.1016/j.jcf.2021.11.002.
Keywords Adults, Children, Fractional sodium excretion, Salt, Sodium, Sodium status, sodium depletion
Theme Care of patients with CF, Nutrition/Gastroenterology
November 2021
Title: A microengineered laboratory model of human CF lung
Authors Roberto Plebani, Ratnakar Potla, Mercy Soong, Haiqing Bai, Zohreh Izadifar, Amanda Jiang, Renee N Travis, Chaitra Belgur, Alexandre Dinis, Mark J Cartwright, Rachelle Prantil-Baun, Pawan Jolly, Sarah E Gilpin, Mario Romano, Donald E Ingber
References J Cyst Fibros. 2021 Nov 16;S1569-1993(21)02106-8. doi: 10.1016/j.jcf.2021.10.004.
Keywords Microfluidics, Neutrophils, Organ chip, Pseudomonas
November 2021
Title: Cystic fibrosis patients and health care professionals’ evaluation of a remote follow up solution with digital questionnaires and phone consultations developed due to the COVID19 pandemic.
Authors Lotte Rodkjær, Majbritt Jeppesen, Liv Schougaard
References J Cyst Fibros. 2021 Nov 13;S1569-1993(21)02112-3. doi: 10.1016/j.jcf.2021.10.010.
Keywords COVID-19, Patient Reported Outcome Measure, Remote follow-up, CFTR-m therapy
Theme Care of patients with CF
November 2021
Title: An in-depth study of the CFTR gene enables selection of CFTR therapies for almost all individuals with CF
Authors Karen S Raraigh, Melis A Aksit, Kurt Hetrick, Rhonda G Pace, Hua Ling, Wanda O'Neal, Elizabeth Blue, Yi-Hui Zhou, Michael J Bamshad, Scott M Blackman, Ronald L Gibson, Michael R Knowles, Garry R Cutting, for the CF Genome Project
References J Cyst Fibros. 2021 Nov 12;S1569-1993(21)02113-5. doi: 10.1016/j.jcf.2021.10.011.
Keywords CFTR, Modulator therapy, Variant annotation, Variant identification, Whole genome sequencing
Theme Care of patients with CF, CFTR/Basic Science
November 2021
Title: What do we already know and what do we need to study next when looking at markers of inflammation in sputum in CF trials?
Authors Agathe Lepissier, Charlotte Addy, Kate Hayes, Sabrina Noel, Stéphanie Bui, Pierre-Régis Burgel, Lieven Dupont, Olaf Eickmeier, Michael Fayon, Teresinha Leal, Carlos Lopes, Damian G Downey, Isabelle Sermet-Gaudelus
References J Cyst Fibros. 2021 Nov 9;S1569-1993(21)02111-1. doi: 10.1016/j.jcf.2021.10.009.
Keywords Inflammation, sputum biomarkers
November 2021
Title: How effective are exercise interventions at increasing physical activity in people with Cystic Fibrosis?
Authors M Curran, A C Tierney, B Button, L Collins, L Kennedy, C McDonnell, B Casserly, R Cahalan
References J Cyst Fibros. 2021 Nov 6;S1569-1993(21)02110-X. doi: 10.1016/j.jcf.2021.10.008.
Keywords Exercise, Interventions, Physical activity promotion, Training
Theme Care of patients with CF, Exercise
October 2021
Title: Arm muscle area as an early indicator of worsening nutritional status in children - a single centre experience
Authors Helmut Ellemunter, Markus Dumke, Gratiana Steinkamp
References J Cyst Fibros. 2021 Oct 30;S1569-1993(21)02108-1. doi: 10.1016/j.jcf.2021.10.006.
Keywords Anthropometric parameters, Arm muscle area, Fat-free mass, nutritional status, Skinfold measurements
Theme Care of patients with CF, Nutrition/Gastroenterology
October 2021
Title: Clofazimine has no influence on the blood concentrations of tezacaftor-ivacaftor in a patient with cystic fibrosis.
Authors S E M Vonk, S W J Terheggen-Lagro, L M Mouissie, R A A Mathôt, E M Kemper, Amsterdam Mucociliary Clearance Disease (AMCD) research group
References J Cyst Fibros. 2021 Oct 29;S1569-1993(21)02105-6. doi: 10.1016/j.jcf.2021.10.003.
Keywords Clofazimine, Drug-drug interaction, Tezacaftor-ivacaftor
Theme Care of patients with CF, Lung Disease and Infection
October 2021
Title: The effect of CFTR modulation with elexacaftor-tezacaftor-ivacaftor on the anxiety sweat rate in people with cystic fibrosis
Authors Sophia Theres Pallenberg, Sibylle Junge, Felix C Ringshausen, Annette Sauer-Heilborn, Gesine Hansen, Anna Maria Dittrich, Burkhard Tümmler, Manuel Nietert
References J Cyst Fibros. 2021 Oct 28;S1569-1993(21)02107-X. doi: 10.1016/j.jcf.2021.10.005.
Keywords CFTR modulator, Sweat rate assay, Sweat test
Theme Diagnosis/Screening
October 2021
Title: Exploring new treatment options for people with cystic fibrosis with severe stop mutations
Authors E de Poel, S Spelier, S W F Suen, E Kruisselbrink, S Y Graeber, M A Mall, E J M Weersink, M M van der Eerden, G H Koppelman, C K van der Ent, J M Beekman
References J Cyst Fibros. 2021 Oct 16;S1569-1993(21)01425-9. doi: 10.1016/j.jcf.2021.09.020.
Keywords CFTR modulation, CFTR nonsense mutation, Intestinal organoids, nonsense mRNA mediated decay inhibition, Premature termination codon, Read-through
October 2021
Title: Damage in the cell lining of the airways in cystic fibrosis
Authors Amandine M Collin, Marylène Lecocq, Bruno Detry, François M Carlier, Caroline Bouzin, Philippe de Sany, Delphine Hoton, Stijn Verleden, Antoine Froidure, Charles Pilette, Sophie Gohy
References J Cyst Fibros. 2021 Oct 14;S1569-1993(21)01424-7. doi: 10.1016/j.jcf.2021.09.019.
Keywords Epithelial differentiation, Epithelial-to-mesenchymal transition, Respiratory epithelium
Theme CFTR/Basic Science, Lung Disease and Infection
October 2021
Title: Is it possible to monitor young people with cystic fibrosis at home? Results of the CLIMB-CF study
Authors C Edmondson, N Westrupp, P Seddon, C Olden, C Wallis, C Dawson, M Brodlie, F Baxter, J McCormick, S MacFarlane, D Rice, A Macleod, R Brooker, M Connon, S Ghayyda, L Blaikie, R Thursfield, L Brown, A Price, E Fleischer, J Itterman, D Hughes, P Barrett, , M Surette, C Donnelly, D Mateos-Corral, G Padley, J Wallenburg, K Brownlee, E W F W Alton, A Bush, J C Davies
References J Cyst Fibros. 2021 Oct 8;S1569-1993(21)01423-5. doi: 10.1016/j.jcf.2021.09.018.
Keywords anxiety, depression, Digital technology, Feasibility, Home monitoring
Theme Care of patients with CF, Clinical Trials
October 2021
Title: Lung infection by Pseudomonas aeruginosa in children with cystic fibrosis: risk factors and impact on lung function
Authors Julie Mésinèle, Manon Ruffin, Astrid Kemgang, Loïc Guillot, Pierre-Yves Boëlle, Harriet Corvol
References J Cyst Fibros. 2021 Oct 7;S1569-1993(21)01422-3. doi: 10.1016/j.jcf.2021.09.017.
Keywords Children, Genetic modifiers, Lung function, Pseudomonas aeruginosa, Risk factors
Theme Lung Disease and Infection
October 2021
Title: A review of sweat testing in Ireland in the era of modern CF care
Authors Barrie Maguire, Ophelia Blake, Gerard Boran, Ingrid Borovickova, Sabah Abdelfadil, Caroline Murray, Basil Elnazir, Barry Linnane
References J Cyst Fibros. 2021 Oct 4;S1569-1993(21)01417-X. doi: 10.1016/j.jcf.2021.09.012.
Keywords National survey, Newborn screening, Quantity not sufficient, sweat testing
Theme Diagnosis/Screening
October 2021
Title: Features of Salt Imbalance and Pseudo Barter Syndrome in hospitalized Children and Young Adults with Cystic Fibrosis.
Authors Danish Abdul Aziz, Fatima Siddiqui, Quratulain Abbasi, Haissan Iftikhar, Shahira Shahid, Fatima Mir
References J Cyst Fibros. 2021 Oct 2;S1569-1993(21)01418-1. doi: 10.1016/j.jcf.2021.09.013.
Keywords Hypokalemic alkalosis, Hyponatremia, Pseudo-Bartter syndrom
Theme Care of patients with CF, Endocrinology
September 2021
Title: Sense of Smell in People with Cystic Fibrosis: Impact of Modulator Therapy
Authors Daniel M Beswick, Stephen M Humphries, Connor D Balkissoon, Matthew Strand, Eszter K Vladar, Vijay R Ramakrishnan, Jennifer L Taylor-Cousar
References J Cyst Fibros. 2021 Sep 28;S1569-1993(21)01419-3. doi: 10.1016/j.jcf.2021.09.014.
Keywords CFTR modulator therapy, Olfaction, Olfactory cleft
Theme Care of patients with CF, Nutrition/Gastroenterology
September 2021
Title: A summary of previously-published research looking at the length of treatment of acute infections and exacerbations in people with cystic fibrosis
Authors Trevor Nicholson, Aisling Smith, Edward McKone, Charles Gallagher
References J Cyst Fibros. 2021 Sep 27;S1569-1993(21)01358-8. doi: 10.1016/j.jcf.2021.08.017.
Keywords antibiotic, Duration of treatment, acute exacerbations
Theme Care of patients with CF, Lung Disease and Infection
