With the CF Research News, we aim to bridge the gap between people with CF and the researchers investigating CF and how best to treat those with the condition.
We want to provide access to patients, parents, relatives, friends and caregivers of patients with CF to all scientific work mainly published in the Journal of Cystic Fibrosis (JCF).

All PDF files published hereunder are lay version summaries of work published in the JCF.

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
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Scientific Abstract

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
Theme
CFTR/Basic Science
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Scientific Abstract
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
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Scientific Abstract

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
Scientific Abstract
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
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Scientific Abstract
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
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Scientific Abstract

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
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Scientific Abstract

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
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Scientific Abstract
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
Theme
Newborn screening
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Scientific Abstract

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
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Scientific Abstract

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
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Scientific Abstract
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
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Scientific Abstract

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
Theme
Diabetes
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Scientific Abstract

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
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Scientific Abstract

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
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Scientific Abstract