February 2022
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
September 2021
Title: A serious consideration about the wise use of the new, highly efficient, very expensive, treatment for cystic fibrosis (Trikafta©/Kaftrio©)
Authors Donatello Salvatore, Angela Pepe, Vincenzo Carnovale, Fabio Majo, Rita Padoan, Serena Quattrucci, Marco Salvatore, Domenica Taruscio, Annalisa Amato, Gianluca Ferrari, Giuseppe Campagna
References J Cyst Fibros. 2021 Sep 25;S1569-1993(21)01416-8. doi: 10.1016/j.jcf.2021.09.011.
Keywords CFTR modulator, CFTR-related disorders
September 2021
Title: Using a digital system, CFHealthHub, to automatically record the use of preventative inhaled treatments as to compare the amount of medicines collected from pharmacy to the amount actually used.
Authors Amanda Bevan and Martin Wildman for CFDigicare (https://www.cfhealthhub.com/cfdigicare/)
References J Cyst Fibros. 2021 Sep 23;S1569-1993(21)01408-9. doi: 10.1016/j.jcf.2021.09.007.
Keywords Adherence to therapy, Cost analysis, Measurement systems
Theme Care of patients with CF
September 2021
Title: Prevalence, management and outcome of Italian infants with cystic fibrosis screening positive inconclusive diagnosis
Authors Vito Terlizzi, Laura Claut, Antonella Tosco, Carla Colombo, Valeria Raia, Benedetta Fabrizzi, Marco Lucarelli, Antonio Angeloni, Giuseppe Cimino, Alice Castaldo, Laura Marsiglio, Silviana Timpano, Natalia Cirilli, Laura Moroni, Filippo Festini, , Pietro Piccinini, Lucia Zavataro, Paolo Bonomi, Giovanni Taccetti, Kevin W Southern, Rita Padoan
References J Cyst Fibros. 2021 Sep;20(5):828-834. doi: 10.1016/j.jcf.2021.03.015.
Title: Drug levels and dosing recommendations for antibiotics used to treat Mycobacterium avium complex in people with cystic fibrosis.
Authors Stacey L. Martiniano, Brandie D. Wagner, Laney Brennan, Michael F. Wempe, Peter L. Anderson, Charles L. Daley, Meg Anthony, Jerry A. Nick, and Scott D. Sagel
References J Cyst Fibros. 2021 Sep;20(5):772-778. doi: 10.1016/j.jcf.2021.04.011. Epub 2021 May 21.
Keywords Mycobacterium avium, nontuberculous mycobacteria, Pharmacokinetics, Therapeutic drug monitoring
Theme Care of patients with CF, Lung Disease and Infection, Microbiology
Title: Drug development for Cystic Fibrosis patients carrying the 3849+10kb C-to-T splicing mutation
Authors Yifat S Oren, Michal Irony-Tur Sinai, Anita Golec, Ofra Barchad-Avitzur, Venkateshwar Mutyam, Yao Li, Jeong Hong, Efrat Ozeri-Galai, Aurélie Hatton, Chen Leibson, Liran Carmel, Joel Reiter, Eric J Sorscher, Steve D Wilton, Eitan Kerem, Steven M Rowe, Isab
References J Cyst Fibros. 2021 Sep;20(5):865-875. doi: 10.1016/j.jcf.2021.06.003. Epub 2021 Jul 2.
Keywords 3849+10 kb C-to-T mutation, Antisense oligonucleotides, drug development, Splicing modulation
September 2021
Title: Testicular pain following initiation of elexacaftor/tezacaftor/ivacaftor in males with cystic fibrosis
Authors Shannon M Rotolo, Stephanie Duehlmeyer, Sarah M Slack, Hollyann R Jacobs, Brian Heckman
References J Cyst Fibros. 2020 Sep;19(5):e39-e41. doi: 10.1016/j.jcf.2020.04.017.
Keywords Adverse reactions, Congenital bilateral absence of the vas deferens, Drug-related side effects, Elexacaftor, Infertility, Male
Theme Care of patients with CF, Psychosocial
September 2021
Title: Can cough swabs be used to identify bacterial infections in patient with cystic fibrosis.
Authors Dominic Fenn, Mahmoud I Abdel-Aziz, Paul Brinkman, Renate Kos, Anne H Neerincx, Josje Altenburg, E Weersink, Eric G Haarman, Suzanne W J Terheggen-Lagro, Anke H Maitland-van der Zee, Lieuwe D J Bos, Amsterdam mucociliary clearance disease research group
References J Cyst Fibros. 2021 Sep 18;S1569-1993(21)01372-2. doi: 10.1016/j.jcf.2021.08.031.
Keywords Microbiome, Next generation sequencing, Pseudomonas aeruginosa, pulmonary infection
September 2021
Title: Study that tested the effects of a new drug called acebilustat in adultswith cystic fibrosis
Authors J. Stuart Elborn, Michael W. Konstan, Jennifer L. Taylor-Cousar, Isabelle Fajac, Alexander Horsley, Sivagurunathan Sutharsan, Shawn D. Aaron, Cori L. Daines, Ahmet Uluer, Damian G. Downey, Vincenzina V. Lucidi, Sanjeev Ahuja, Eric Springman, John Mershon, Ralph Grosswald, Steven M. Rowe, for the EMPIRE-CF study group.
References J Cyst Fibros. 2021 Sep 16;S1569-1993(21)01348-5. doi: 10.1016/j.jcf.2021.08.007.
Keywords CFTR, Inflammation, LTA4H, LTB(4), Neutrophils
September 2021
Title: The effect of ETI (Trikafta™) on blood sugars in adults with cystic fibrosis
Authors Kevin J Scully, Peter Marchetti, Gregory S Sawicki, Ahmet Uluer, Manuela Cernadas, Rebecca E Cagnina, John C Kennedy, Melissa S Putman
References J Cyst Fibros. 2021 Sep 13;S1569-1993(21)01377-1. doi: 10.1016/j.jcf.2021.09.001.
Keywords CFTR modulator, continuous glucose monitoring, Cystic fibrosis related diabetes, Elexacaftor-tezacaftor-ivacaftor
September 2021
Title: Defining constipation in patients with Cystic Fibrosis following criteria delineated by the European Society for Paediatric Gastroenterology Hepatology and Nutrition
Authors Marina Abib Stefano, Natascha Silva Sandy, Carlos Zagoya, Franziska Duckstein, Antonio Fernando Ribeiro, Jochen G Mainz, Elizete Aparecida Lomazi
References J Cyst Fibros. 2021 Sep 9;S1569-1993(21)01362-X. doi: 10.1016/j.jcf.2021.08.021.
Theme Care of patients with CF, Nutrition/Gastroenterology
Title: Defining constipation in patients with Cystic Fibrosis following criteria delineated by the European Society for Paediatric Gastroenterology Hepatology and Nutrition
Authors Marina Abib Stefano, Natascha Silva Sandy, Carlos Zagoya, Franziska Duckstein, Antonio Fernando Ribeiro, Jochen G Mainz, Elizete Aparecida Lomazi
References J Cyst Fibros. 2021 Sep 9;S1569-1993(21)01362-X. doi: 10.1016/j.jcf.2021.08.021.
September 2021
Title: A review of three studies examining different aspects of pulmonary exacerbation treatment
Authors Georgene E Hergenroeder, Jonathan D Cogen
References J Cyst Fibros. 2021 Sep 6;S1569-1993(21)01365-5. doi: 10.1016/j.jcf.2021.08.024.
Keywords Antimicrobial stewardship, Pulmonary exacerbation
Theme Care of patients with CF, Epidemiology/Models of care
September 2021
Title: Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?
Authors Emily Granger, Gwyneth Davies, Ruth H Keogh
References J Cyst Fibros. 2021 Sep 5;S1569-1993(21)01355-2. doi: 10.1016/j.jcf.2021.08.014.
Keywords Ivacaftor, Registry data, Treatment burden, Treatment patterns
Theme Care of patients with CF, Epidemiology/Models of care
September 2021
Title: New perspectives on physical exercise training in adults and children with cystic fibrosis
Authors Mathieu Gruet, Zoe L Saynor, Don S Urquhart, Thomas Radtke
References J Cyst Fibros. 2021 Sep 4;S1569-1993(21)01345-X. doi: 10.1016/j.jcf.2021.08.004
Keywords Aerobic training, CFTR modulator, Exercise preferences, Physical fitness, Resistance training
September 2021
Title: Needle-free bubble test
Authors Audrey Reynaerts, François Vermeulen, Angélique Mottais, Sophie Gohy, Patrick Lebecque, Raphaël Frédérick, Rita Vanbever, Teresinha Leal
References J Cyst Fibros. 2021 Sep 3;S1569-1993(21)01353-9. doi: 10.1016/j.jcf.2021.08.012.
Keywords CFTR, Iontophoresis, Nonlinear mixed-effects model, Sweat gland, Sweat test
Theme Care of patients with CF, Diagnosis/Screening
Title: Clinical presentation and basic defect of the CFTR genotype p.Phe508del / p.Arg117His in a mother and her monozygous twin daughters
Authors Christiane Lex, Rebecca Minso, Nadine Alfeis, Hendrik Rosewich, Sylvia Schucht, Burkhard Tümmler
References J Cyst Fibros. 2021 Sep 3;S1569-1993(21)01359-X. doi: 10.1016/j.jcf.2021.08.018.
Keywords Intestinal current measurement, Nasal potential difference, Twin research
August 2021
Title: Survival in cystic fibrosis after need for mechanical life support.
Authors Jenna M. Gibilaro, Claire Keating, Luke Benvenuto, Amanda Kramer, Ann Privorotskiy, Yuan Zheng, Cheng-Shiun Leu
References J Cyst Fibros. 2021 Aug 31;S1569-1993(21)01357-6. doi: 10.1016/j.jcf.2021.08.016.
Title: Clofazimine use for treatment of mycobacteria in children with and without cystic fibrosis
Authors L H Cameron, C A Peloquin, P Hiatt, M Mann, J R Starke, J Faircloth, J C McNeil, A Patel, F Ruiz
References J Cyst Fibros. 2021 Aug 31;S1569-1993(21)01351-5. doi: 10.1016/j.jcf.2021.08.010.
August 2021
Title: Comparing lung function testing at home vs in the clinic.
Authors Alex Paynter, Umer Khan, Sonya L Heltshe, Christopher H Goss, Noah Lechtzin, Nicole Mayer Hamblett
References J Cyst Fibros. 2021 Aug 30;S1569-1993(21)01354-0. doi: 10.1016/j.jcf.2021.08.013.
Keywords CF outcome measures, COVID-19, Coronavirus, Forced expiratory volume in one second (FEV1), Remote monitoring, Telehealth
August 2021
Title: The influence of cystic fibrosis on work ability of affected patients
Authors Veruscka Leso, Rosaria Romano, Carolina Santocono, Marilisa Caruso, Paola Iacotucci, Vincenzo Carnovale, Ivo Iavicoli
References J Cyst Fibros. 2021 Aug 29;S1569-1993(21)01352-7. doi: 10.1016/j.jcf.2021.08.011.
Keywords Employment, Occupation, Professional life, Risk assessment and management, Work ability
August 2021
Title: How did new infections with important bacteria change over time across U.S. CF care centers
Authors Marianne S Muhlebach, Xiaotong Jiang, Michael R Kosorok, Eili Y Klein, Lisa Saiman
References J Cyst Fibros. 2021 Aug 26;S1569-1993(21)01350-3. doi: 10.1016/j.jcf.2021.08.009.
Keywords CF center, Epidemiology, ethnicity, infection control, Methicillin resistant Staphylococcus aureus (MRSA), Pseudomonas aeruginosa
Title: Review of pregnancy in cystic fibrosis
Authors Raksha Jain, Traci M Kazmerski, Lisa C Zuckerwise, Natalie E West, Kristina Montemayor, Moira L Aitken, Edith Cheng, Andrea H Roe, Alexandra Wilson, Caitlin Mann, Sigrid Ladores, Jacqui Sjoberg, Madeline Poranski, Jennifer L Taylor-Cousar
References J Cyst Fibros. 2021 Aug 26;S1569-1993(21)01338-2. doi: 10.1016/j.jcf.2021.07.019.
Keywords Family planning, Fertility, Parenthood, pregnancy
August 2021
Title: Orkambi may improve liver disease in people with CF
Authors David Drummond, Jérémy Dana, Laureline Berteloot, Elena K. Schneider-Futschik, Frédérique Chedevergne, Céline Bailly-Botuha, Thao Nguyen-Khoa, Muriel Le Bourgeois, Dominique Debray, Muriel Girard, Isabelle Sermet-Gaudelus
References J Cyst Fibros. 2021 Aug 25;S1569-1993(21)01337-0. doi: 10.1016/j.jcf.2021.07.018.
Keywords CFTR modulator, Cystic-fibrosis liver disease, Cirrhosis, hepatobiliary, liver enzymes; pediatrics
August 2021
Title: A study of the drug riociguat as a possible treatment for people with cystic fibrosis
Authors Nico Derichs, Jennifer L Taylor-Cousar, Jane C Davies, Isabelle Fajac, Elizabeth Tullis, Dilip Nazareth, Damian G Downey, Daniel Rosenbluth, Anne Malfroot, Clare Saunders, Renee Jensen, George M Solomon, Francois Vermeulen, Andreas Kaiser, Stefan Willmann, Soundos Saleh, Karoline Droebner, Peter Sandner, Christine E. Bear, Anja Hoffmann, Felix Ratjen1, Steven M. Rowe
References J Cyst Fibros. 2021 Aug 19;S1569-1993(21)01329-1. doi: 10.1016/j.jcf.2021.07.015.
Keywords Phe508del, Riociguat
Title: Do cystic fibrosis patients have an abnormal innate immune response to pathogens?
Authors Isabelle Fleurot, Raquel López-Gálvez, Pascal Barbry, Antoine Guillon, Mustapha Si-Tahar, Andrea Bähr, Nikolai Klymiuk, Jean-Claude Sirard, Ignacio Caballero
References J Cyst Fibros. 2021 Aug 19;S1569-1993(21)01343-6. doi: 10.1016/j.jcf.2021.08.002.
August 2021
Title: Ivacaftor withdrawal syndrome: A rare but potentially life-threatening consequence from a life-saving medication
Authors James M Clegg, Kelsey W Malloy, Rebekah F Brown, Alison G Grisso, Andrew G Sokolow
References J Cyst Fibros. 2021 Aug 11;S1569-1993(21)01340-0. doi: 10.1016/j.jcf.2021.07.021.
Keywords Highly effective modulator therapy, Ivacaftor withdrawal syndrome
Theme Care of patients with CF, Pulmonology
August 2021
Title: Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis
Authors Cameron J McKinzie, Christina T Doligalski, Steven J Lobritto, Raymond D Coakley, W Adam Gower
References J Cyst Fibros. 2021 Aug 9;S1569-1993(21)01336-9. doi: 10.1016/j.jcf.2021.07.017.
Keywords CFTR modulator; Cystic fibrosis; Liver transplantation
August 2021
Title: “Coping and Learning to Manage Stress with CF” a Telehealth Pilot Intervention
Authors Christina J Bathgate, Kristin M Kilbourn, Nora H Murphy, Frederick S Wamboldt, Kristen E Holm
References J Cyst Fibros. 2021 Aug 5;S1569-1993(21)01326-6. doi: 10.1016/j.jcf.2021.07.012.
Keywords anxiety, Cognitive behavioral therapy, Coping, depression, pilot study, RCT, Stress
Title: Do bone active therapy and lung disease influence the skeletal outcome after transplantation?
Authors Grassi Giorgia, Chiodini Iacopo, Cairoli Elisa, Morlacchi Letizia Corinna, Rossetti Valeria, Rosso Lorenzo, Righi Ilaria, Nosotti Mario, Arosio Maura, Blasi Francesco, Eller-Vainicher Cristina
References J Cyst Fibros. 2021 Aug 5;S1569-1993(21)01327-8. doi: 10.1016/j.jcf.2021.07.013
Keywords Lung transplantation, Trabecular bone score, Vertebral fractures
August 2021
Title: Cancer cases in the UK Cystic Fibrosis Population
Authors Olga Archangelidi, Paul Cullinan, Nicholas J Simmonds, Emmanouil Mentzakis, Daniel Peckham, Diana Bilton, Siobhán B Carr
References J Cyst Fibros. 2021 Aug 1;S1569-1993(21)01302-3. doi: 10.1016/j.jcf.2021.07.004.
Keywords Breast, Cancer, Gastro-intestinal, registry, Risk, skin
Theme Care of patients with CF, Diabetes, Epidemiology/Models of care, Nutrition/Gastroenterology, Transplantation
Title: Is elexacaftor/tezacaftor/ivacaftor always better than ezacaftor/ivacaftor?
Authors Eva Furstova, Tereza Dousova, Jakub Beranek, Malgorzata Libik, Libor Fila, Martin Modrak, Ondrej Cinek, Milan Macek Jr, Pavel Drevinek
References J Cyst Fibros. 2021 Aug 1;S1569-1993(21)01304-7. doi: 10.1016/j.jcf.2021.07.006.
Keywords CFTR sequence, Elexacaftor, Intestinal organoids, Ivacaftor, Tezacaftor
July 2021
Title: Different approaches to Aspergillus-related conditions in people with cystic fibrosis
Authors Gina Hong, Sameer Desai, Richard B Moss, Patience Eschenhagen, Bradley S Quon, Carsten Schwarz
References J Cyst Fibros. 2021 Jul 28;S1569-1993(21)01306-0. doi: 10.1016/j.jcf.2021.07.008.
Keywords Allergic bronchopulmonary aspergillosis, Aspergillus bronchitis, Aspergillus colonization, Fungi
July 2021
Title: Urinary tract infections in cystic fibrosis patients
Authors Seth A Reasoner, Kyle T Enriquez, Benjamin Abelson, Steven Scaglione, Bennett Schneier, Michael G O'Connor, Gerald Van Horn, Maria Hadjifrangiskou
References J Cyst Fibros. 2021 Jul 27;S1569-1993(21)01303-5. doi: 10.1016/j.jcf.2021.07.005
Keywords Antibiotic resistance, Cystitis, Urinary tract infection
July 2021
Title: Utilization of Electronic Patient-Reported Outcome Measures in Cystic Fibrosis Research
Authors Meghana Sathe, Baha Moshiree, Phuong T Vu, Umer Khan, Sonya L Heltshe, Melita Romasco, Steven D Freedman, Sarah Jane Schwarzenberg, Christopher H Goss, A Jay Freeman
References J Cyst Fibros. 2021 Jul 22;S1569-1993(21)01299-6. doi: 10.1016/j.jcf.2021.07.002.
Keywords Electronic PROMs, Patient reported outcomes measures, gastrointestinal manifestations of cystic fibrosis
July 2021
Title: Tobacco Smoke Exposure Limits the Therapeutic Benefit of Tezacaftor-Ivacaftor in Pediatric Patients with Cystic Fibrosis.
Authors Elizabeth Baker, William T Harris, Steven M Rowe, Sarah B Rutland, Gabriela R Oates
References J Cyst Fibros. 2021 Jul;20(4):612-617. doi: 10.1016/j.jcf.2020.09.011. Epub 2020 Oct 3.
Theme Care of patients with CF, Pulmonology
Title: Review of abnormal Stomach and Intestinal Movement in Cystic Fibrosis
Authors Sara Henen, Christine Denton, Jeff Teckman, Drucy Borowitz, Dhiren Patel
References J Cyst Fibros. 2021 Jul;20(4):578-585. doi: 10.1016/j.jcf.2021.05.016. Epub 2021 Jun 17.
Keywords GI motility, dysmotility, Gastrointestinal, highly effective modulators, laxatives; motility
Title: Stopping exposure to smoke improves breathing and nutritional outcomes in children and adolescents with cystic fibrosis
Authors Gabriela R Oates, Elizabeth Baker, Joseph M Collaco, Steven M Rowe, Sarah B Rutland, Christopher M Fowler, William T Harris
References J Cyst Fibros. 2021 Jul;20(4):618-624. doi: 10.1016/j.jcf.2021.06.014. Epub 2021 Jul 17.
Keywords Secondhand smoke, Smoke exposure, Smoking cessation, Tobacco
July 2021
Title: Disease burden in people with cystic fibrosis heterozygous for F508del and a minimal function mutation
Authors Gregory S. Sawicki, Kate Van Brunt, Jason Booth, Evan Bailey, Stefanie J. Millar, Michael W. Konstan, Patrick A. Flume
References J Cyst Fibros. 2021 Jul 19;S1569-1993(21)01300-X. doi: 10.1016/j.jcf.2021.07.003.
July 2021
Title: Drug-Induced Liver Injury from Elexacaftor/Ivacaftor/Tezacaftor
Authors Sarah Lowry; Peter J. Mogayzel, Jr.; Kiyoko Oshima; Wikrom Karnsakul
References J Cyst Fibros. 2021 Jul 16;S1569-1993(21)01297-2. doi: 10.1016/j.jcf.2021.07.001.
Keywords CFTR modulator, Drug-induced liver injury
July 2021
Title: A Short extension to multiple breath washout provides additional indications of blocked and severely diseased airways in people with CF: a pilot study
Authors Christopher Short, Thomas Semple, Clare Saunders, Dominic Hughes, Samantha Irving, Laura Gardener, Mark Rosenthal, Paul D Robinson, Jane C Davies
References J Cyst Fibros. 2021 Jul 15;S1569-1993(21)01298-4. doi: 10.1016/j.jcf.2021.06.013.
Keywords Air Trapping, Biomarkers, Computed Tomography (CT), Lung clearance index (LCI), Lung Function Testing, Multiple breath washout (mbw), Pulmonary function testing (pft), Pulmonary physiology
July 2021
Title: There are two main types of problems with arteries in cystic fibrosis and existence of one or the other depends on pancreatic health
Authors Jan Krzysztof Nowak, Andrzej Wykrętowicz, Edyta Mądry, Tomasz Krauze, Sławomira Drzymała-Czyż, Patrycja Krzyżanowska-Jankowska, Paulina Sobkowia, Agata Schneider, Joanna Goździk-Spychalska, Szymon Kurek, Victoria Kononets, Nataliya Kashirskaya, Aleksandra Lisowska, Jarosław Walkowiak
References J Cyst Fibros. 2021 Jul 10;S1569-1993(21)01294-7. doi: 10.1016/j.jcf.2021.06.010.
Keywords Arterial stiffness, Atherosclerosis, Digital volume pulse, Intima-media, Lipids
Theme Care of patients with CF, Nutrition/Gastroenterology
Title: Opioid Pain Management Among Adults with CF: Survey Results
Authors Sarah Allgood, Jessica L Zemlak, Elisabeth Dellon, Siddhartha G Kapnadak, Jessica Goggin, Noah Lechtzin
References J Cyst Fibros. 2021 Jul 10;S1569-1993(21)01291-1. doi: 10.1016/j.jcf.2021.06.007.
Keywords Adults, Opioids, Pain
July 2021
Title: Genetics of immunity against the Pseudomonas aeruginosa bacteria in people with cystic fibrosis
Authors Janardan P Pandey, Aryan M Namboodiri, Paul J Nietert, Michael R Knowles, Rhonda G Pace, Gerald B Pier
References J Cyst Fibros. 2021 Jul 7;S1569-1993(21)01295-9. doi: 10.1016/j.jcf.2021.06.011.
Keywords GM/KM allotypes, IgG antibodies, Pseudomonas aeruginosa
June 2021
Title: How does the cystic fibrosis drug ivacaftor move in the body during pregnancy and breastfeeding?
Authors Fiona Qiu, Mark D Habgood, Yifan Huang, Katarzyna M Dziegielewska, Sam Toll, Elena K Schneider-Futschik
References J Cyst Fibros. 2021 Jun 27;S1569-1993(21)01289-3. doi: 10.1016/j.jcf.2021.06.005.
Keywords Breastfeeding, Cystic fibrosis transmembrane conductance regulator, Ivacaftor, pregnancy, blood brain barrier
June 2021
Title: Use of LCI to monitor CFTR Modulator effect in people with CF
Authors Philippe Reix, Aurélie Tatopoulos, Iulia Ioan, Muriel Le Bourgeois, Stéphanie Bui, Marie Luce Choukroun, Katia Bessaci-Kabouya, Michele Gerardin, Plamen Bokov, Jennifer Da Silva, Jean-Louis Paillasseur, Pierre Regis Burgel,, French Cystic Fibrosis Reference Network study group
References J Cyst Fibros. 2021 Jun 25;S1569-1993(21)01286-8. doi: 10.1016/j.jcf.2021.06.002.
Keywords CFTR modulators, Lumacaftor, Multiple breath washout
