CF Research News for June 2020 | European Cystic Fibrosis Society (ECFS)

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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.

June 2020

Title: A disruption of innervation around CF tissues results in low amount of VIP before CF disease has produced damage to the lungs and small intestine.

Authors

Anna Semaniakou, Sarah Brothers, Grayson Gould, Mehrsa Zahiremani, Jamie Paton, Frederic Chappe, Audrey Li, Younes Anini, Roger P Croll, Valerie Chappe

References

J Cyst Fibros. 2020 Jun 26;S1569-1993(20)30777-3. doi: 10.1016/j.jcf.2020.06.013. Online ahead of print

Keywords

Acetylcholine, Innervation, Intestine, Mice, VIP

CF Research News Article

Scientific Abstract

June 2020

Title: Fibrocyte accumulation in the lungs of cystic fibrosis patients

Authors

Rajesh K Kasam, Prathibha R Gajjala, Anil G Jegga Jennifer A Courtney, Scott H Randell, Elizabeth L Kramer, John P Clancy, Satish K Madala

References

J Cyst Fibros. 2020 Jun 24;S1569-1993(20)30756-6. doi: 10.1016/j.jcf.2020.06.011. Online ahead of print.

Keywords

CFTR, Fibrocytes, lung, Pulmonary fibrosis

CF Research News Article

Scientific Abstract

June 2020

Title: Tezacaftor/ivacaftor in people with cystic fibrosis who stopped lumacaftor/ivacaftor due to respiratory adverse events

Authors

Carsten Schwarz, Sivagurunathan Sutharsan, Ralph Epaud, Ross C Klingsberg, Rainald Fischer, Steven M Rowe, Paul K Audhya, Neil Ahluwalia, Xiaojun You, Thomas J Ferro, Margaret E Duncan, Bote G Bruinsma

References

J Cyst Fibros. 2020 Jun 23;S1569-1993(20)30730-X. doi: 10.1016/j.jcf.2020.06.001.

Keywords

Clinical trial, Phase 3, Phe508del-CFTR

CF Research News Article

Scientific Abstract

Title: Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function

Authors

Pierre-Régis Burgel, Isabelle Durieu, Raphaël Chiron, Laurent Mely, Anne Prevotat, Marlene Murris-Espin, Michele Porzio, Michel Abely, Philippe Reix9, Christophe Marguet, Julie Macey, Isabelle Sermet-Gaudelus, Harriet Corvol, Stéphanie Bui, Tiphaine Biouhee, Dominique Hubert, Anne Munck, Lydie Lemonnier, Clémence Dehillotte, Jennifer Da Silva, Jean-Louis Paillasseur, Clémence Martin, French Cystic Fibrosis Reference Network study group

References

J Cyst Fibros. 2020 Jun 23;S1569-1993(20)30752-9. doi: 10.1016/j.jcf.2020.06.012. Online ahead of print.

CF Research News Article

Scientific Abstract

June 2020

Title: Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis

Authors

Monica Gelzo, Paola Iacotucci, Mafalda Caputo, Gustavo Cernera, Marika Comegna, Vincenzo Carnovale, Gaetano Corso,, Giuseppe Castaldoa

References

J Cyst Fibros. 2020 Jun 22;S1569-1993(20)30779-7. doi: 10.1016/j.jcf.2020.06.015. Online ahead of print

Keywords

Cholesterol homeostasis, Gas chromatography, Lumacaftor/ivacaftor, pancreatic insufficiency

CF Research News Article

Scientific Abstract

June 2020

Title: Influenza B outbreak at an adult cystic fibrosis centre - clinical impact and factors influencing spread

Authors

Jordan B Dennis, Andrew M Jones, Emma A Davies, William Welfare, Peter J Barry, Lisa Collier, Andrew Turner, Rowland J Bright-Thomas

References

J Cyst Fibros. 2020 Jun 19;S1569-1993(20)30124-7. doi: 10.1016/j.jcf.2020.04.011.

Keywords

Influenza, Lung function, Outbreak, Ventilation

CF Research News Article

Scientific Abstract

June 2020

Title: In vitro 3D culture lung model from expanded primary cystic fibrosis human airway cells

Authors

Rachael E Rayner, Jack Wellmerling, Wissam Osman, Sean Honesty, Maria Alfaro, Mark E Peeples, Estelle Cormet-Boyaka

References

J Cyst Fibros. 2020 Jun 18;S1569-1993(20)30151-X. doi: 10.1016/j.jcf.2020.05.007. Online ahead of print.

Keywords

CFTR ion channel, Cell expansion, In vitro, Lung model

CF Research News Article

Scientific Abstract

June 2020

Title: Antimicrobial resistance: concerns of healthcare providers and people with CF

Authors

Wendy Bullington, Sarah Hempstead, Alan R Smyth, Pavel Drevinek, Lisa Saiman, Valerie J Waters, Scott C Bell, Donald R VanDevanter, Patrick A Flume, Stuart Elborn, Marianne S Muhlebach, Antimicrobial Resistance International Working Group in Cystic Fibrosis

References

J Cyst Fibros . 2020 Jun 17;S1569-1993(20)30159-4. doi: 10.1016/j.jcf.2020.05.009

Keywords

Antimicrobial resistance, Survey

CF Research News Article

Scientific Abstract

June 2020

Title: Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging

Authors

Christabella Ng, Neele S Dellschaft, Caroline L Hoad, Luca Marciani, Lu Ban, Andrew P Prayle, Helen L Barr, Anke Jaudszus, Jochen G Mainz, Robin C Spiller, Penny Gowland, Giles Major, Alan R Smyth

References

J Cyst Fibros. 2020 Jun 16;S1569-1993(20)30733-5. doi: 10.1016/j.jcf.2020.06.004.

Keywords

Gastrointestinal function, Gastrointestinal symptoms, MRI

CF Research News Article

Scientific Abstract

June 2020

Title: Newborn screening alone insufficient to improve pulmonary outcomes for cystic fibrosis

Authors

Christina B Barreda, Philip M Farrell, Anita Laxova, Jens C Eickhoff, Andrew T Braun, Ryan J Coller, Michael J Rock

References

J Cyst Fibros . 2020 Jun 13;S1569-1993(20)30731-1. doi: 10.1016/j.jcf.2020.06.002.

CF Research News Article

Scientific Abstract

Title: The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis

Authors

Leah Cuthbertson, Imogen Felton, Phillip James, Michael J Cox, Diana Bilton, Silke Schelenz, Michael R Loebinger, William O C Cookson, Nicholas J Simmonds, Miriam F Moffatt

References

J Cyst Fibros. 2020 Jun 13;S1569-1993(20)30163-6. doi: 10.1016/j.jcf.2020.05.013.

Keywords

Chronic airway disease, Filamentous fungi, Fungal airway disease, Mycobiome

CF Research News Article

Scientific Abstract

Title: Immunotyping of clinically divergent p.Phe508del homozygous monozygous cystic fibrosis twins

Authors

Esther Schamschula, Wolfgang Hagmann, Yassen Assenov, Silke Hedtfeld, Ahmed K Farag, Lennart M Roesner, Lutz Wiehlmann, Frauke Stanke, Sebastian Fischer, Angela Risch, Burkhard Tümmler

References

J Cyst Fibros. 2020 Jun 13;S1569-1993(20)30754-2. doi: 10.1016/j.jcf.2020.06.009.

Keywords

DNA methylation, Epigenetics, T cell receptor repertoire, Twin research

CF Research News Article

Scientific Abstract

Title: Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations

Authors

Anne Munck, Eitan Kerem, Helmut Ellemunter, Daniel Campbell, Linda T Wang, Neil Ahluwalia, Caroline A Owen, Claire Wainwright

References

J Cyst Fibros . 2020 Jun 13;S1569-1993(20)30128-4. doi: 10.1016/j.jcf.2020.04.015.

Keywords

Ivacaftor; Tezacaftor

CF Research News Article

Scientific Abstract

June 2020

Title: Defective BACH1/HO-1 regulatory circuits in cystic fibrosis bronchial epithelial cells

Authors

Shashipavan Chillappagari, Virajith Garapati, Poornima Mahavadi, Lutz Naehrlich, Bernd T Schmeck, M Lienhard Schmitz, Andreas Guenther

References

J Cyst Fibros . 2020 Jun 11;S1569-1993(20)30150-8. doi: 10.1016/j.jcf.2020.05.006. Online ahead of print.

Keywords

ACH1, Hemeoxygenase-1, MicroRNAs, Regulatory circuits

CF Research News Article

Scientific Abstract

Title: Accumulation and persistence of Ivacaftor in airway epithelia with prolonged treatment

Authors

Tara N Guhr Lee, Deborah M Cholon, Nancy L Quinney, Martina Gentzsch, Charles R Esther Jr

References

J Cyst Fibros . 2020 Jun 11;S1569-1993(20)30123-5. doi: 10.1016/j.jcf.2020.04.010.

Keywords

CFTR modulator, F508del, Ivacaftor, Pharmacokinetics

CF Research News Article

Scientific Abstract

June 2020

Title: Clinimetric evaluation of muscle function tests for individuals with cystic fibrosis: A systematic review

Authors

Yann Combret, Clement Medrinal, Tristan Bonnevie, Francis-Edouard Gravier, Pascal Le Roux, Bouchra Lamia, Guillaume Prieur, Gregory Reychler

References

J Cyst Fibros . 2020 Jun 10;S1569-1993(20)30164-8. doi: 10.1016/j.jcf.2020.05.014.

Keywords

Clinimetric properties, Limb muscles, Muscle function, Respiratory muscles

CF Research News Article

Scientific Abstract

June 2020

Title: Building global development strategies for cf therapeutics during a transitional cftr modulator era

Authors

N Mayer-Hamblett, S van Koningsbruggen-Rietschel, D P Nichols, D R VanDevanter, J C Davies, T Lee, A G Durmowicz, F Ratjen, M W Konstan, K Pearson, S C Bell, J P Clancy, J L Taylor-Cousar, K De Boeck, S H Donaldson, D G Downey, P A Flume, P Drevinek, C H Goss, I Fajac, A S Magaret, B S Quon, S M Singleton, J M VanDalfsen, G Z Retsch-Bogart

References

J Cyst Fibros . 2020 Jun 7;S1569-1993(20)30161-2. doi: 10.1016/j.jcf.2020.05.011. Online ahead of print.

Keywords

CFTR modulators, clinical trials, drug development, Global perspective

CF Research News Article

Scientific Abstract

June 2020

Title: Pseudomonas aeruginosa antimicrobial susceptibility test (AST) results and pulmonary exacerbation treatment responses in cystic fibrosis

Authors

Donald R. VanDevanter, Sonya L. Heltshe, Jay B. Hilliard, Michael W. Konstan

References

J Cyst Fibros . 2020 Jun 4;S1569-1993(20)30152-1. doi: 10.1016/j.jcf.2020.05.008

Keywords

Pseudomonas aeruginosa, Pulmonary exacerbation, Susceptibility testing

CF Research News Article

Scientific Abstract

June 2020

Title: Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation

Authors

Gitte Berkers, Renske van der Meer, Peter van Mourik, Annelotte M Vonk, Evelien Kruisselbrink, Sylvia Wf Suen, Harry Gm Heijerman, Christof J Majoor, Gerard H Koppelman, Jolt Roukema, Hettie M Janssens, Yolanda B de Rijke, E Marleen Kemper, Jeffrey M Beekman, Cornelis K van der Ent, Hugo R de Jonge

References

J Cyst Fibros. 2020 Jun 1;S1569-1993(20)30127-2. doi: 10.1016/j.jcf.2020.04.014. Online ahead of print.

Keywords

CFTR, Curcumin, Genistein, Ivacaftor, Organoids

CF Research News Article

Scientific Abstract