April 2023
Title: Impact of unplanned pregnancies in cystic fibrosis
Authors Giselle Peng, Jennifer L Taylor-Cousar, MinJae Lee, Ashley Keller, Natalie E West, Traci M Kazmerski, Jennifer L Goralski, Moira L Aitken, Andrea H Roe, Denis Hadjiliadis, Ahmet Uluer, Patrick A Flume, Sheila Mody, Leigh Ann Bray, Raksha Jain
References J Cyst Fibros. 2023 Apr 5;S1569-1993(23)00084-X. doi: 10.1016/j.jcf.2023.03.020.
Keywords Contraception, pregnancy, Pulmonary exacerbations, Unplanned
Theme Care of patients with CF, Pregnancy with CF, Pulmonology
March 2023
Title: How the discovery of new white blood cell subtypes can improve our understanding of lung disease progression in adult CF patients?
Authors Murru Andréa, Vadeboncoeur Nathalie, Therrien Andrée-Anne, Coderre Lise, Vaillancourt Myriam, Labrecque Marie-Michèle, Berthiaume Yves, Bouvet Guillaume, Adam Damien, Brochiero Emmanuelle, Lesage Sylvie, Flamand Nicolas, Bilodeau Lara, Maria J Fernandes
References J Cyst Fibros. 2023 Mar 25;S1569-1993(23)00072-3. doi: 10.1016/j.jcf.2023.03.009.
Keywords Lung function, Neutrophil, Neutrophil sub-populations, Pulmonary exacerbation
Theme CFTR/Basic Science, Immunology, Lung Disease and Infection
March 2023
Title: Autoimmunity (a condition where the body's immune system mistakes its own healthy tissues as foreign and attacks them) in People with Cystic Fibrosis
Authors Christina Chadwick, Heather Lehman, Shelby Luebbert, Rabheh Abdul-Aziz, Drucy Borowitz
References J Cyst Fibros. 2023 Mar 23;S1569-1993(23)00071-1. doi: 10.1016/j.jcf.2023.03.007.
Keywords Arthritis, Autoimmune disease, autoimmunity, Celiac disease, Crohn's disease, Vasculitis
Title: Cervical mucus quality in females with and without cystic fibrosis
Authors Andrea H Roe, Nathanael Koelper, Arden McAllister, Kurt T Barnhart, Courtney A Schreiber, Denis Hadjiliadis
References J Cyst Fibros. 2023 Mar 23;S1569-1993(23)00077-2. doi: 10.1016/j.jcf.2023.03.013.
Theme Endocrinology, Pregnancy with CF
Title: What do dietitians, doctors and physiotherapists think about the management of people with CF who are living with overweight and obesity?
Authors Joanna E Snowball, William G Flight, Laura Heath, Dimitrios A Koutoukidis
References J Cyst Fibros. 2023 Mar 23;S1569-1993(23)00075-9. doi: 10.1016/j.jcf.2023.03.011.
Keywords CFTR modulators, obesity, Overweight, Qualitative interviews, Weight management
Theme Care of patients with CF, Nutrition/Gastroenterology
March 2023
Title: Year in Review (2022): Modulators and COVID-19: the story does on
Authors Stefanie Dillenhoefer, Dorothy Grogono, Ana Morales-Tirado
References J Cyst Fibros. 2023 Mar;22(2):188-192. doi: 10.1016/j.jcf.2023.03.001. Epub 2023 Mar 6.
Theme Bone health, Care of patients with CF, Imaging, Lung Disease and Infection, Nutrition/Gastroenterology, Psychosocial, Pulmonology, Transplantation
Title: Effects of elexacaftor/tezacaftor/ivacaftor on liver tests in cystic fibrosis
Authors Daniel H Tewkesbury, Varinder Athwal, Rowland J Bright-Thomas, Andrew M Jones, Peter J Barry
References J Cyst Fibros. 2023 Mar;22(2):256-262. doi: 10.1016/j.jcf.2023.01.007. Epub 2023 Jan 19.
Theme CFTR/Basic Science, Nutrition/Gastroenterology
Title: Sharing decisions about family goals: A study exploring women’s experiences and needs for support.
Authors Denitza Williams, Oluwaseun B Esan, Daniela K Schlüter, David Taylor-Robinson, Shantini Paranjothy, Jamie Duckers, Natalie Goodchild, Rhiannon Phillips
References J Cyst Fibros. 2023 Mar;22(2):207-216. doi: 10.1016/j.jcf.2023.02.007. Epub 2023 Feb 28.
Keywords Mixed-method, Person-centred care, Pre-conception, Reproductive goals, Shared decision-making
Theme Care of patients with CF, Pregnancy with CF, Psychosocial
Title: An overview of CFTR modulators and pancreas function
Authors Mitchell L Ramsey, Susan S Li, Luis F Lara, Yevgeniya Gokun, Venkata S Akshintala, Darwin L Conwell, John Heintz, Stephen E Kirkby, Karen S McCoy, Georgios I Papachristou, Alpa Patel, Vikesh K Singh, Phil A Hart
References J Cyst Fibros. 2023 Mar;22(2):193-200. doi: 10.1016/j.jcf.2022.08.008.
Keywords Acute pancreatitis, Exocrine pancreatic insufficiency, Fecal elastase, Lipase, Trypsin
Theme Care of patients with CF, Nutrition/Gastroenterology
Title: Two-year follow-up results from an ongoing study on the safety and effectiveness of elexacaftor/tezacaftor/ivacaftor when used in real-world settings by people with cystic fibrosis
Authors Julie K Bower, Nataliya Volkova, Neil Ahluwalia, Gurvaneet Sahota, Fengjuan Xuan, Anna Chin, Tanya G Weinstock, Josh Ostrenga, Alexander Elbert
References J Cyst Fibros. 2023 Mar 22;S1569-1993(23)00066-8. doi: 10.1016/j.jcf.2023.03.002.
Keywords Elexacaftor, Ivacaftor, Real-world, registry, Tezacaftor
Theme Clinical Trials, Pulmonology
March 2023
Title: A letter in response to the recently published article entitled “Effect of elexacaftor/tezacaftor/ivacaftor on annual rate of lung function decline in people with cystic fibrosis
Authors Zhe Hui Hoo, Lana Yh Lai, Robert D Sandler, Tracey E Daniels, Sophie Dawson, Marlene Hutchings, Martin J Wildman
References J Cyst Fibros. 2023 Mar 20;S1569-1993(23)00076-0. doi: 10.1016/j.jcf.2023.03.012.
March 2023
Title: Postage of sputum samples from home to clinic can be used for microbiology surveillance
Authors Lauren Hatfield, Brooke Bianco, Helen Gavillet, Phillipa Burns, Damian Rivett, Matthew Smith, Andrew Jones, Christopher van der Gast, Alexander Horsley
References J Cyst Fibros. 2023 Mar 16;S1569-1993(23)00073-5. doi: 10.1016/j.jcf.2023.03.008.
Keywords Airway infection, microbiology, Remote monitoring, Sputum, Telemedicine
March 2023
Title: Understanding Medical Traumatic Stress in Cystic Fibrosis from the Viewpoints of Patients, Families, and Providers
Authors Addison A Cuneo, Simon Outram, Meghan L Marsac, Siena Vendlinski, Melvin B Heyman, Ngoc Ly, Emily von Scheven, Emily R Perito
References J Cyst Fibros. 2023 Mar 14;S1569-1993(22)01427-8. doi: 10.1016/j.jcf.2022.12.008.
Keywords Chronic illness, Iatrogenic trauma, Medical traumatic stress, Trauma-informed medical care
March 2023
Title: Association between nebulizer therapies adherence and visit-to-visit variability of lung function in patients with cystic fibrosis
Authors David Drummond, Pauline Whelan, Matthew Sperrin
References J Cyst Fibros. 2023 Mar 13;S1569-1993(23)00070-X. doi: 10.1016/j.jcf.2023.03.006.
Keywords Adherence, Forced expiratory volume in one second, Lung function tests
March 2023
Title: How does telehealth impact the health of children with cystic fibrosis
Authors Kristene Rimbaldo, Katherine B Frayman, Shivanthan Shanthikumar
References J Cyst Fibros. 2023 Mar 6;S1569-1993(23)00068-1. doi: 10.1016/j.jcf.2023.03.003.
Keywords COVID-19, Spirometry, Telehealth.
Theme Care of patients with CF, Epidemiology/Models of care
March 2023
Title: Models of Cystic Fibrosis care after lung transplant
Authors Edward McKone, Kathleen Ramos, Cecilia Chaparro, Joshua Blatter, Ramsey Hachem, Michael Anstead, Fanny Vlahos, Abby Thaxton, Sarah Hempstead, Thomas Daniels, Michelle Murray, Amparo Sole, Robin Vos, Erin Tallarico, Albert Faro, Joseph M Pilewski
References J Cyst Fibros. 2023 Mar 5;S1569-1993(23)00059-0. doi: 10.1016/j.jcf.2023.02.011.
Theme Care of patients with CF, Epidemiology/Models of care, Transplantation
February 2023
Title: Investigating the diagnostic potential of small cell particles in the blood of people with CF
Authors Anne Trappe, Navya Lakkappa, Suzanne Carter, Eugene Dillon, Kieran Wynne, Edward McKone, Paul McNally, Judith A Coppinger
References J Cyst Fibros. 2023 Feb 27;S1569-1993(23)00056-5. doi: 10.1016/j.jcf.2023.02.005.
Keywords Extracellular vesicles, Inflammation, Serum
Title: More favorable growth profiles in early childhood are associated with higher lung function at age six
Authors Kevin J Psoter, Kimberly M Dickinson, Kristin A Riekert, Joseph M Collaco
References J Cyst Fibros. 2023 Feb 27;S1569-1993(23)00060-7. doi: 10.1016/j.jcf.2023.02.008.
Keywords BMI, Early childhood, FEV(1)pp, Growth trajectories, registry, WFL
Theme Care of patients with CF, Nutrition/Gastroenterology
February 2023
Title: Suicide Attempts in Adolescents with Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor Therapy
Authors Meliksah Arslan, Sarah Chalmers, Kelly Rentfrow, Janelle M Olson, Vicki Dean, Mark E Wylam, Nadir Demirel
References J Cyst Fibros. 2023 Feb 7;S1569-1993(23)00023-1. doi: 10.1016/j.jcf.2023.01.015.
Keywords Adolescent, Covid-19 pandemic, depression, Elexacaftor/tezacaftor/ivacaftor, Mental adverse effects, mental health, Suicide
Theme Care of patients with CF, Psychosocial
February 2023
Title: CFTR modulators: a new therapeutic approach but not for all CF patients
Authors Carla Colombo, Grant A Ramm, Anders Lindblad, Fabiola Corti, Luigi Porcaro, Federico Alghisi, Irina Asherova, Helen Evans, Nataliya Kashirskaya, Elena Kondratyeva, Peter J Lewindon, Isabelle de Monestrol, Mark Oliver, Chee Y Ooi, Rita Padoan, Sahana Shank
References J Cyst Fibros. 2023 Feb 2;S1569-1993(23)00020-6. doi: 10.1016/j.jcf.2023.01.012.
Keywords CFTR genotype, CFTR modulators, Cirrhosis, liver disease, portal hypertension
Theme Care of patients with CF, Nutrition/Gastroenterology
January 2023
Title: Diabetes is associated with an increased burden of tummy and bowel symptoms in adults with cystic fibrosis
Authors L.R. Caley, C. Zagoya, F. Duckstein, H. White, D. Shimmin, A. M. Jones, J. Barrett, J. L. Whitehouse, R. A. Floto, J. G. Mainz, D.G. Peckham
References J Cyst Fibros. 2023 Jan 27;S1569-1993(23)00018-8. doi: 10.1016/j.jcf.2023.01.010.
Keywords CFAbd-Score©, Cystic fibrosis-related diabetes, Gastrointestinal symptoms
Theme Care of patients with CF, Diabetes, Nutrition/Gastroenterology
January 2023
Title: The Completion of Airway Clearance Therapy at School in a Group of Pediatric Patients with Cystic Fibrosis
Authors David J Byrwa, Geovanny F Perez, Christine M Roach, Sandra Prentice, Danielle M Goetz
References J Cyst Fibros. 2023 Jan 24;S1569-1993(23)00007-3. doi: 10.1016/j.jcf.2023.01.006.
Keywords Adherence, Airway clearance therapy (ACT), High-frequency chest wall oscillation (HFCWO)
Theme Care of patients with CF, Parents, Psychosocial, Pulmonology
January 2023
Title: Bariatric surgery in a patient with cystic fibrosis and diabetes: a case report
Authors N R A Bruijn, M A E M Wagenmakers, M van Hoek, J A Apers, M M van der Eerden, B Özcan
References J Cyst Fibros. 2023 Jan 22;S1569-1993(23)00009-7. doi: 10.1016/j.jcf.2023.01.008.
Keywords Bariatric surgery, Diabetes, obesity
Theme Care of patients with CF, Diabetes, Endocrinology, Nutrition/Gastroenterology
January 2023
Title: Bone health after lung transplantation in patients with cystic fibrosis
Authors Triet Vincent M Tran, Xilong Li, Naim M Maalouf
References J Cyst Fibros. 2023 Jan 18;S1569-1993(23)00005-X. doi: 10.1016/j.jcf.2023.01.003.
Keywords Bone health, Fractures, Lung transplantation, Osteoporosis
Theme Bone health, Care of patients with CF, Pulmonology, Transplantation
January 2023
Title: Examining the potential for drug interactions with Trikafta®/Kaftrio® in people with cystic fibrosis
Authors Debanjali Purkayastha, Kyla Agtarap, Kristy Wong, Onella Pereira, Jannie Co, Smita Pakhale, Salmaan Kanji
References J Cyst Fibros. 2023 Jan 16;S1569-1993(23)00004-8. doi: 10.1016/j.jcf.2023.01.005.
Keywords CFTR modulator, Drug interactions, Elexacaftor, Ivacaftor, Pharmacokinetics, Tezacaft
Theme Care of patients with CF
January 2023
Title: Lung infection in people with advanced cystic fibrosis lung disease
Authors Christina S. Thornton, Lisa A. Carmody, Linda M. Kalikin, Kristopher Opron, Lindsay J. Caverly, and John J. LiPuma
References J Cyst Fibros. 2023 Jan 8;S1569-1993(23)00002-4. doi: 10.1016/j.jcf.2023.01.001.
Keywords Bacterial community diversity, Microbiome
Theme CFTR/Basic Science, Microbiology
January 2023
Title: CFTR in the pancreas, the intestine and the kidney: Function, dysfunction and potential future remedies
Authors Peter Hegyi, Ursula Seidler, Karl Kunzelmann
References J Cyst Fibros. 2023 Mar;22 Suppl 1:S17-S22. doi: 10.1016/j.jcf.2022.12.017. Epub 2023 Jan 6.
Keywords Bicarbonate secretion, Collecting duct, DIOS, Intestine, kidney, Metabolic alkalosis, Mucoviscidosis, NHE3, pancreas, Pancreatitis, Tenapanor
Theme CFTR/Basic Science, Nutrition/Gastroenterology
Title: What happens with CFTR inside human cells affected by cystic fibrosis and how the latest CF drugs change the effects
Authors Alessandra Ghigo, Chiara De Santi, Merrill Hart, Nilay Mitash, Agnieszka Swiatecka-Urban
References J Cyst Fibros. 2023 Jan 6;S1569-1993(22)01435-7. doi: 10.1016/j.jcf.2022.12.015.
December 2022
Title: The Presence of Clostridioides difficile in Stool Samples of Children with Cystic Fibrosis
Authors Seth A Reasoner, Irtiqa F Fazili, Rachel Bernard, Jacob M Parnell, Andrew G Sokolow, Kelly F Thomsen, Kathryn M Edwards, Rebekah F Brown, Maribeth R Nicholson
References J Cyst Fibros. 2022 Dec 28;S1569-1993(22)01434-5. doi: 10.1016/j.jcf.2022.12.014.
Keywords Clostridioides difficile, colonization, Infection, Pediatrics
Theme Microbiology, Nutrition/Gastroenterology
Title: Fundamental and translational research in Cystic Fibrosis – why we still need it
Authors Carlos M Farinha, Jeffrey L Brodsky, Nicoletta Pedemonte
References J Cyst Fibros. 2022 Dec 26;S1569-1993(22)01430-8. doi: 10.1016/j.jcf.2022.12.010.
December 2022
Title: Safety and efficacy of extended treatment with tezacaftor/ivacaftor
Authors Patrick A Flume, R Scott Harris, Hildegarde Paz-Diaz, Neil Ahluwalia, Mark Higgins, Daniel Campbell, Indrias Berhane, Judy L Shih, Gregory Sawicki
References J Cyst Fibros. 2022 Dec 27;S1569-1993(22)01426-6. doi: 10.1016/j.jcf.2022.12.006
Keywords F508del, Ivacaftor, Phase 3, Tezacaftor
December 2022
Title: Pulmonary exacerbation groupings based on blood inflammation in Cystic Fibrosis
Authors Kang Dong, Sung Moon Huh, Grace Y Lam, Jiah Jang, Alessandro N. Franciosi, Pearce G Wilcox, Bradley S Quon
References J Cyst Fibros. 2022 Dec 24;S1569-1993(22)01433-3. doi: 10.1016/j.jcf.2022.12.013.
Keywords Inflammatory phenotype, Pulmonary exacerbations, Treatment response
Theme Care of patients with CF, Lung Disease and Infection, Pulmonology
December 2022
Title: Effect of variants in the CFTR gene on the CFTR transcripts and protein to improve patients care.
Authors A. Bergougnoux, A. Billet, C. Ka, M. Heller, F. Degrugillier, M.-L. Vuillaume, V. Thoreau, S. Sasorith, C. Bareil, C. Thèze, C. Ferec, G. Le Gac, T. Bienvenu, E. Bieth, V. Gaston, G. Lalau, A. Pagin, M.-C. Malinge, F. Dufernez, L. Lemonnier, M. Koenig, , P. Fergelot, M. Claustres, M. Taulan-Cadars, A. Kitzis, M.-P. Reboul, F. Becq, P. Fanen, C. Mekki, M.-P. Audrezet, E. Girodon, C. Raynal
References J Cyst Fibros. 2022 Dec 23;S1569-1993(22)01423-0. doi: 10.1016/j.jcf.2022.12.003.
Theme Care of patients with CF, CFTR/Basic Science
December 2022
Title: Experiences of people with cystic fibrosis living without Trikafta/Kaftrio
Authors Francesco Milo, Fabiana Ciciriello, Federico Alghisi, Paola Tabarini
References J Cyst Fibros. 2022 Dec 20;S1569-1993(22)01428-X. doi: 10.1016/j.jcf.2022.12.007.
Keywords CFTR modulator therapies, Elexacaftor-tezacaftor-ivacaftor, Thematic analysis
Theme Care of patients with CF, Psychosocial
December 2022
Title: Study of how the G1244E cystic fibrosis mutation modifies the CFTR function and response to available CFTR modulators
Authors Valeria Tomati, Stefano Costa, Valeria Capurro, Emanuela Pesce, Cristina Pastorino, Mariateresa Lena, Elvira Sondo, Marco Di Duca, Federico Cresta, Simona Cristadoro, Federico Zara, Luis J V Galietta, Renata Bocciardi, Carlo Castellani, Maria Cristina Lucanto, Nicoletta Pedemonte
References J Cyst Fibros. 2022 Dec 19;S1569-1993(22)01425-4. doi: 10.1016/j.jcf.2022.12.005.
Keywords Chloride secretion, Corrector, Gating; Modulators, Potentiator, Stabilizer, Theratype
December 2022
Title: Risk of Human papilloma virus infection and of cervical lesions in females with cystic fibrosis
Authors C Rousset-Jablonski, Y Mekki, A Denis, Q Reynaud, R Nove-Josserand, S Durupt, S Touzet, M Perceval, I Ray-Coquard, F Golfier, I Durieu
References J Cyst Fibros. 2022 Dec 14;S1569-1993(22)01415-1. doi: 10.1016/j.jcf.2022.11.005.
Theme Care of patients with CF, Microbiology, Transplantation
December 2022
Title: Clinician Perspectives on Assessing for Disordered Eating and Body Image Disturbance in Adolescents and Young Adults with Cystic Fibrosis
Authors Alexandra P. Kass, Traci M. Kazmerski, Elana Bern, Sabina Sabharwal, Jessica Leonard, Moira Harrison, Katherine Barnico, Tracy Richmond, Gregory S. Sawicki
References J Cyst Fibros. 2022 Dec 2;S1569-1993(22)01416-3. doi: 10.1016/j.jcf.2022.11.006.
Keywords Body image, Eating disorder
Theme Care of patients with CF, Psychosocial
November 2022
Title: Mutations carrying premature termination mutations are improved by ELX-02
Authors Máire Curran, Audrey C Tierney, Louise Collins, Lauren Kennedy, Ciara McDonnell, Andrew J Jurascheck, Ali Sheikhi, Cathal Walsh, Brenda Button, Brian Casserly, Roisin Cahalan
References J Cyst Fibros. 2022 Nov 16;S1569-1993(22)01411-4. doi: 10.1016/j.jcf.2022.11.002.
Keywords Exercise, Physical activity, Step count, Wearable technology
Theme Care of patients with CF, Exercise
November 2022
Title: The effects of Trikafta/Kaftrio on people with CF who have mutations other than the most common CF mutation (F508del)
Authors Galit Livnat, Adi Dagan, Moshe Heching, Einat Shmueli, Dario Prais, Karin Yaacoby-Bianu, Nili Stein, Meir Mei-Zahav, Michal Gur, Malena Cohen-Cymberknoh, Michal Shteinberg
References J Cyst Fibros. 2022 Nov 10;S1569-1993(22)01393-5. doi: 10.1016/j.jcf.2022.10.011.
Keywords Elexacaftor/tezacaftor/ivacaftor, Non-F508del mutations, treatment
Theme Care of patients with CF, Lung Disease and Infection, Pulmonology
October 2022
Title: Best therapeutic approaches to treat everyone with CF
Authors Margarida D Amaral, Patrick T Harrison
References J Cyst Fibros. 2022 Oct 30;S1569-1993(22)01389-3. doi: 10.1016/j.jcf.2022.10.007.
Keywords CFTR, Genome editing, Theratypes
October 2022
Title: Gastrointestinal symptom burden in cystic fibrosis
Authors Baha Moshiree, A Jay Freeman, Phuong T Vu, Umer Khan, Carmen Ufret-Vincenty, Sonya L Heltshe, Christopher H Goss, Sarah Jane Schwarzenberg, Steven D Freedman, Drucy Borowitz, Meghana Sathe, GALAXY Study Group
References J Cyst Fibros. 2022 Oct 29;S1569-1993(22)01388-1. doi: 10.1016/j.jcf.2022.10.006.
Keywords Constipation, Gastrointestinal questionnaires, Gastrointestinal symptoms, Patient-reported outcome measures
Theme Care of patients with CF, Nutrition/Gastroenterology
October 2022
Title: Effect of ETI on CF Gastrointestinal Disease
Authors Sarah Jane Schwarzenberg, Phuong T Vu, Michelle Skalland, Lucas R Hoffman, Christopher Pope, Daniel Gelfond, Michael R Narkewicz, David P Nichols, Sonya L Heltshe, Scott H Donaldson, Carla A Frederick, Andrea Kelly, Jessica E Pittman, Felix Ratjen,, Margaret Rosenfeld, Scott D Sagel, George M Solomon, Michael S Stalvey, John P Clancy, Steven M Rowe, Steven D Freedman, Promise Study Group
References J Cyst Fibros. 2022 Oct 21;S1569-1993(22)01384-4. doi: 10.1016/j.jcf.2022.10.003.
Keywords Inflammation, Modulators, pancreatic insufficiency, Patient-reported outcomes measures
Theme Care of patients with CF, Nutrition/Gastroenterology
October 2022
Title: The risk of developing bowel cancer in people with cystic fibrosis, or cystic fibrosis causing mutations.
Authors Rebecca J. Birch, Daniel Peckham, Henry M. Wood, Philip Quirke, Rob Konstant-Hambling, Keith Brownlee, Rebecca Cosgriff, Genomics England Research Consortium, Nicholas Burr, Amy Downing
References J Cyst Fibros. 2022 Oct 14;S1569-1993(22)01383-2. doi: 10.1016/j.jcf.2022.10.001.
Theme Care of patients with CF
October 2022
Title: New guidelines on non CF disorders related to the CFTR gene
Authors Castellani C, De Boeck K, De Wachter E, Sermet-Gaudelus I, Simmonds NJ, Southern KW, on behalf of the ECFS Diagnostic Network Working Group
References J Cyst Fibros. 2022 Oct 8;S1569-1993(22)00691-9. doi: 10.1016/j.jcf.2022.09.011.
Keywords CFTR, CFTR biomarkers, CFTR related disorders, CFTR sequence variations, Follow-up, Intestinal current measurement, Nasal potential difference, Sweat test, treatment
Theme Diagnosis/Screening
September 2022
Title: What happened to outcomes of Australians with CF during the first year of the COVID pandemic?
Authors Michael Doumit, Sandra Chuang, Peter Middleton, Hiran Selvadurai, Sheila Sivam, Rasa Ruseckaite, Susannah Ahern, Kylie Ann Mallitt, Verity Pacey, Kelly Gray, Adam Jaffe
References J Cyst Fibros. 2022 Sep 16;S1569-1993(22)00685-3. doi: 10.1016/j.jcf.2022.09.006.
Keywords COVID-19, Telehealth
Theme Care of patients with CF, Epidemiology/Models of care, Lung Disease and Infection
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
September 2022
Title: Increased blood glucose during the night is associated with a lower pulmonary function in adults but not in children with cystic fibrosis.
Authors Dimitri Declercq, Simon Helleputte, Sophie Marchand, Sara Van Aken, Eva Van Braeckel, Sabine Van Daele, Guy T'Sjoen, Stephanie Van Biervliet, Bruno Lapauw
References J Cyst Fibros. 2022 Sep 3;S1569-1993(22)00653-1. doi: 10.1016/j.jcf.2022.08.016.
Theme Care of patients with CF, Diabetes, Pulmonology
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: A step forward in the development of a safe anti-inflammatory molecule for the chronic respiratory disease of people with CF
Authors Alice Rossi, Alessandra Bragonzi, Melessike Medede, Ida De Fino, Giuseppe Lippi, Marco Prosdocimi, Anna Tamanini, Giulio Cabrini and Maria Cristina Dechecchi
References J Cyst Fibros. 2022 Aug 13;S1569-1993(22)00642-7. doi: 10.1016/j.jcf.2022.08.005.
Keywords Beta-sitosterol, Host response, Natural medicine, Neutrophils, Pseudomonas aeruginosa
Theme CFTR/Basic Science, Lung Disease and Infection
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: Two mutations in one CFTR gene copy can influence each other and thereby disease characteristics
Authors Senne Cuyx, Sofia S Ramalho, Isabelle Callebaut, Harry Cuppens, Arthur Kmit, Kaline Arnauts, Marc Ferrante, Catherine Verfaillie, Marjolein Ensinck, Marianne S Carlon, Mieke Boon, Marijke Proesmans, Lieven Dupont, Kris De Boeck, Carlos M Farinha,, François Vermeulen, Anabela S Ramalho
References J Cyst Fibros. 2022 Jul;21(4):644-651. doi: 10.1016/j.jcf.2022.05.013. Epub 2022 Jun 9.
Keywords CFTR, Complex allele, F508C, FIS assay, Organoids, S1251N
Theme Care of patients with CF, CFTR/Basic Science
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: The Association of Area Deprivation and State Child Health with Respiratory Outcomes of Pediatric Patients with Cystic Fibrosis in the United States
Authors Gabriela Oates, Sarah Rutland, Lucia Juarez, Annabelle Friedman, Michael S Schechter
References Pediatr Pulmonol. 2021 May;56(5):883-890. doi: 10.1002/ppul.25192. Epub 2020 Dec 14.
Keywords social dimensions of pulmonary medicine
Theme Care of patients with CF, Psychosocial, Pulmonology
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
