July 2023
Title: Response to oral antibiotic treatment in people with cystic fibrosis
Authors Ana Blanchard, Michelle Shaw, Felix Ratjen, Elizabeth Tullis, Nick Daneman, Valerie Waters
References J Cyst Fibros. 2023 Jul 18;S1569-1993(23)00829-9. doi: 10.1016/j.jcf.2023.06.015.
Keywords oral antibiotics, Pulmonary exacerbation
Theme Care of patients with CF, Lung Disease and Infection
July 2023
Title: Ivacaftor impacts nutrition and growth status in 4 to 24 month old children within the first 12 weeks
Authors Alyssa Tindall, Rosara Bass, Asim Maqbool, Virginia A Stallings
References J Cyst Fibros. 2023 Jul 10;S1569-1993(23)00114-5. doi: 10.1016/j.jcf.2023.04.010.
Keywords Bile acids, Fatty acids, Ivacaftor, nutritional status
Theme Care of patients with CF, Nutrition/Gastroenterology
July 2023
Title: Successful treatment of a blockage in the lower portion of the small intestine in patients with cystic fibrosis using a solution administered during a colonoscopy.
Authors Ikenna K Emelogu, Cynthia N Tran, Wendy R Greene, Joshua D Novak
References J Cyst Fibros. 2023 Jul 8;S1569-1993(23)00828-7. doi: 10.1016/j.jcf.2023.06.014
Keywords colonoscopy, DIOS, Distal intestinal obstruction syndrome, N-acetylcysteine, Polyethylene glycol
Theme Care of patients with CF, Imaging, Nutrition/Gastroenterology
July 2023
Title: CF Mutation Presents with Intestinal Obstruction in Newborns and Later Pancreatic Sufficiency
Authors Emma Lang, Bridget Kiernan, Eleanor D. Muise, Robert Giusti
References J Cyst Fibros. 2023 Jul 7;S1569-1993(23)00045-0. doi: 10.1016/j.jcf.2023.02.002.
Theme Care of patients with CF, Diagnosis/Screening, Newborn screening
July 2023
Title: Changes in weight and how much you eat after starting triple modulator therapy in adults with cystic fibrosis
Authors L R Caley, H H Jarosz-Griffiths, L Smith, L Gale, J Barrett, L Kinsey, V Davey, M Nash, A M Jones, J L Whitehouse, D Shimmin, R A Floto, H White, D G Peckham
References J Cyst Fibros. 2023 Jul 6;S1569-1993(23)00824-X. doi: 10.1016/j.jcf.2023.06.010.
Keywords Body mass index, CFTR modulators, Elexacaftor/ivacaftor/tezacaftor, Energy expenditure, Nutritional intake
Theme Nutrition/Gastroenterology
Title: Airway infection increases Pseudomonas aeruginosa acquisition
Authors Julie Mésinèle, Manon Ruffin, Loïc Guillot, Pierre-Yves Boëlle, Harriet Corvol
References J Cyst Fibros. 2023 Jul 6;S1569-1993(23)00822-6. doi: 10.1016/j.jcf.2023.06.007
Keywords Airway infections, Children, Pseudomonas aeruginosa, Risk factors
Theme Lung Disease and Infection, Microbiology
June 2023
Title: What happens to muscle, fat and body mass index between 8 and 18 years of age in children with cystic fibrosis
Authors Jade K. Tran, Chee Y. Ooi, Katrina Blazek, Tamarah Katz
References J Cyst Fibros. 2023 Jun 24;S1569-1993(23)00817-2. doi: 10.1016/j.jcf.2023.06.003.
Theme Care of patients with CF, Nutrition/Gastroenterology, Pulmonology
June 2023
Title: Anxiety and Depression Symptom Patterns over 6 years in one Pediatric CF Center
Authors Michelle Hjelm, Elizabeth Hente, Jamie Miller, Susan Moore, James Peugh, David V Swetland, Dawit G Tadesse, Md Monir Hossain, Christopher Siracusa, Stephanie S Filigno
References J Cyst Fibros. 2023 Jun 29;S1569-1993(23)00823-8. doi: 10.1016/j.jcf.2023.06.009.
Keywords anxiety, depression, Generalized anxiety disorder questionnaire, mental health, Patient health questionnaire
Theme Care of patients with CF, Psychosocial
Title: Weight A Minute: Exploring the effect on total weight, fat and fat-free weight after the initiation of Elexacaftor/ Tezacaftor/ Ivacaftor in adults with CF
Authors David Proud, Jamie Duckers
References J Cyst Fibros. . 2023 Jun 23;S1569-1993(23)00180-7. doi: 10.1016/j.jcf.2023.06.002.
Theme Care of patients with CF, Diabetes, Nutrition/Gastroenterology
June 2023
Title: Elexacaftor/Tezacaftor/Ivacaftor improves CFTR activity and pulmonary function in Cystic Fibrosis Patients carrying the N1303K Mutation
Authors Ido Sadras, Eitan Kerem, Galit Livnat, Ifat Sarouk, Oded Breuer, Joel Reiter, Alex Gileles-Hillel, Ori Inbar, Michael Cohen, Ayelet Gamliel, Noemie Stanleigh, Tarini Gunawardena, Claire Bartlett, Tanja Gonska, Theo Moraes, Paul D W Eckford, , Christine E Bear, Felix Ratjen, Batsheva Kerem, Michael Wilschanski, Michal Shteinberg, Malena Cohen-Cymberknoh
References J Cyst Fibros. 2023 Jun 16;S1569-1993(23)00178-9. doi: 10.1016/j.jcf.2023.06.001.
Title: Elexacaftor/Tezacaftor/Ivacaftor improves CFTR activity and pulmonary function in Cystic Fibrosis Patients carrying the N1303K Mutation
Authors Ido Sadras, Eitan Kerem, Galit Livnat, Ifat Sarouk, Oded Breuer, Joel Reiter, Alex Gileles-Hillel, Ori Inbar, Michael Cohen, Ayelet Gamliel, Noemie Stanleigh, Tarini Gunawardena, Claire Bartlett, Tanja Gonska, Theo Moraes, Paul D W Eckford,, Christine E Bear, Felix Ratjen, Batsheva Kerem, Michael Wilschanski, Michal Shteinberg, Malena Cohen-Cymberknoh
References J Cyst Fibros. 2023 Jun 16;S1569-1993(23)00178-9. doi: 10.1016/j.jcf.2023.06.001.
Theme Care of patients with CF, Clinical Trials, Pulmonology
June 2023
Title: Molecular fingerprinting of Pseudomonas aeruginosa from PWCF, and a UK reference laboratory investigation into frequency of cross-infection
Authors Dervla T D Kenna, Zoë Payne, David A Lee, Ann-Marie Keane, Jack Turton, Dania V Zamarreño, Ulf Schaefer, Katie L Hopkins, Danièle Meunier, Rishi Dhillon, Jamie Duckers, Lorraine Speight, Jane F Turton
References J Cyst Fibros. 2023 Jun 2;S1569-1993(23)00176-5. doi: 10.1016/j.jcf.2023.05.017.
Keywords Cross-infection, Liverpool epidemic strain, Pseudomonas aeruginosa, Whole genome sequencing
May 2023
Title: People with CF who take part in clinical trials do not represent the general CF population well
Authors Rebecca Dobra, Sophie Pinnell, Andy Jones, Susan Madge, Nicholas J Simmonds, Jane C Davies
May 2023
Title: How to interpret changes in home spirometry when people are feeling well?
Authors Christina S Thornton, Amalia S Magaret, Lisa A Carmody, Linda M Kalikin, Richard H Simon, John J LiPuma, Lindsay J Caverly
References J Cyst Fibros. 2023 May 25;S1569-1993(23)00141-8. doi: 10.1016/j.jcf.2023.05.011.
Keywords Baseline health, Exacerbation, Home spirometry, Lung function
Theme Care of patients with CF, Pulmonology
Title: Male female differences after elexacaftor/tezacaftor/ivacaftor
Authors Angela Wang, MinJae Lee, Ashley Keller, Sarah Jian, Karen Lowe, James D Finklea, Raksha Jain
References J Cyst Fibros. 2023 May 25;S1569-1993(23)00139-X. doi: 10.1016/j.jcf.2023.05.009.
Keywords Cystic fibrosis transmembrane conductance regulators, Elexacaftor/tezacaftor/ivacaftor, Estrogen, Sex
Theme Epidemiology/Models of care, Pulmonology
May 2023
Title: Simplified method for cystic fibrosis sweat testing using microneedle patches is effective in human study.
Authors Jonathan Yuxuan Chen, Song Li, George L. Silva, Joshua D. Chandler, Mark R. Prausnitz, Lokesh Guglani
References Cyst Fibros. 2023 May 24;S1569-1993(23)00120-0. doi: 10.1016/j.jcf.2023.04.014.
Keywords Iontophoresis, Microneedle patch, Pilocarpine, Sweat induction, Sweat test
Theme Diagnosis/Screening
May 2023
Title: Nutritional Considerations for a New Era: A CF Foundation Position Paper
Authors Amanda Leonard, Julianna Bailey, Amanda Bruce, Shijing Jia, Adam Stein, Judith Fulton, Meagan Helmick, Marina Litvin, Alpa Patel, Kate E Powers, Elizabeth Reid, Senthilkumar Sankararaman, Cristen Clemm, Kim Reno, Sarah E Hempstead, Emily DiMango
References J Cyst Fibros. 2023 May 23;S1569-1993(23)00140-6. doi: 10.1016/j.jcf.2023.05.010.
Keywords Eating behavior, Food Insecurity, Nutrition, Pancreatic enzymes, Sodium, Weight management
Theme Care of patients with CF, Exercise, Nutrition/Gastroenterology
May 2023
Title: People with CF face high costs of CF care which have implications for debt, access to health care, adequate food, and other necessities of life.
Authors Semret Seyoum, Marsha Regenstein, Marie Benoit, Olivia Dieni, Anne Willis, Kim Reno, Cristen Clemm
References J Cyst Fibros. 2023 May;22(3):471-477. doi: 10.1016/j.jcf.2023.01.002. Epub 2023 Jan 27.
Keywords Access, Cost, Debt, Financial burden, Food Insecurity, Insurance
Theme Care of patients with CF, Psychosocial
May 2023
Title: Understanding sleep in young adults with cystic fibrosis (CF) taking elexacaftor/tezacaftor/ivacaftor (ETI)
Authors Lisa J Meltzer, Jane E Gross
References J Cyst Fibros. 2023 May 18;S1569-1993(23)00135-2. doi: 10.1016/j.jcf.2023.05.005.
Title: Understanding sleep in young adults with cystic fibrosis (CF) taking elexacaftor/tezacaftor/ivacaftor (ETI)
Authors Lisa J Meltzer, Jane E Gross
References J Cyst Fibros. 2023 May 18;S1569-1993(23)00135-2. doi: 10.1016/j.jcf.2023.05.005.
May 2023
Title: Eradication of Nontuberculous Mycobacteria in People with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor
Authors Vered Wiesel, Micha Aviram, Meir Mei-Zahav, Miri Dotan, Dario Prais, Malena Cohen-Cymberknoh, Michal Gur, Ronen Bar-Yoseph, Galit Livnat, Aviv Goldbart, Guy Hazan, Itai Hazan, Inbal Golan-Tripto
References J Cyst Fibros. 2023 May 10;S1569-1993(23)00133-9. doi: 10.1016/j.jcf.2023.05.003.
Keywords Mycobacterium abscessus complex, Mycobacterium abscessus subsp. abscessus, Trikafta, Elexacaftor/tezacaftor/ivacaftor, nontuberculous mycobacteria
Theme Care of patients with CF, Microbiology
May 2023
Title: Use of Cystic Fibrosis inhaled medication after Elexacaftor/Tezacaftor/Ivacaftor in comparison to before
Authors Katerina Manika, Filia Diamantea, Anna Tsakona, Alexandros Kalokyris, Athina Sopiadou, Serafeim-Chrysovalantis Kotoulas, Maria Sionidou, Sirmo-Stiliani Kirvasili, Marija Hadji-Mitrova, Eleni Papadaki, Elisavet-Anna Chrysochoou, Elpis Hatziagorou
References J Cyst Fibros. 2023 May 9;S1569-1993(23)00131-5. doi: 10.1016/j.jcf.2023.05.001.
Keywords CFTR modulators, Inhaled medications, Medication Possession Ratio, multi-center study, Use of treatment
Theme Care of patients with CF, Epidemiology/Models of care, Pulmonology
May 2023
Title: Understanding Joint Problems in Cystic Fibrosis: Defining Stages of Arthritis with Ultrasound and Clinical Assessment
Authors F Holz, E Can, C Grehn, J Klotsche, B Materne, J Kruppa, T Kallinich, C Schwarz
References J Cyst Fibros. 2023 May 5;S1569-1993(23)00116-9. doi: 10.1016/j.jcf.2023.04.011.
Theme Care of patients with CF, Imaging
May 2023
Title: Understanding the relationship between secondhand smoke exposure and lung function in children with cystic fibrosis
Authors Jack Reifenberg, Emrah Gecili, Teresa Pestian, Eleni-Rosalina Andrinopoulou, Patrick H Ryan, Cole Brokamp, Joseph M Collaco, Rhonda D Szczesniak
References J Cyst Fibros. 2023 May 2;S1569-1993(23)00126-1. doi: 10.1016/j.jcf.2023.04.020.
Keywords Environmental health, FEV1, Lung function, Pediatric, Pulmonary function testing, Secondhand smoke exposure, Tobacco
Theme Care of patients with CF, Pulmonology
Title: Changes in blood levels of vitamins A, D and E after starting Kaftrio
Authors L Schembri, S Warraich, S Bentley, S B Carr, I M Balfour-Lynn
References J Cyst Fibros. 2023 May 2;S1569-1993(23)00125-X. doi: 10.1016/j.jcf.2023.04.019.
Keywords CFTR modulators, Vitamins
Theme Care of patients with CF, Nutrition/Gastroenterology
April 2023
Title: Inhaled mRNA therapy for treatment of Cystic Fibrosis: Results of an Early Phase Clinical Trial
Authors S M Rowe, J B Zuckerman, D Dorgan, J Lascano, K McCoy, M Jain, M S Schechter, S Lommatzsch, V Indihar, N Lechtzin, K McBennett, C Callison, C Brown, T G Liou, K D MacDonald, S Z Nasr, S Bodie, M Vaughn, E B Meltzer, A J Barbier
References J Cyst Fibros. 2023 Apr 28;S1569-1993(23)00112-1. doi: 10.1016/j.jcf.2023.04.008
Keywords Aerosol, Gene therapy, mRNA therapy
Theme Care of patients with CF, Clinical Trials, Pulmonology
April 2023
Title: Variability of the sweat test in children with Cystic Fibrosis previously with an inconclusive diagnosis Vito Terlizzi, Daniela Dolce
Authors Vito Terlizzi, Daniela Dolce
References J Cyst Fibros. 2023 Apr 26;S1569-1993(23)00122-4. doi: 10.1016/j.jcf.2023.04.018.
Keywords Coefficient of variation, inconclusive diagnosis, Outcomes, sweat chloride
Theme Care of patients with CF, Diagnosis/Screening, Parents, Psychosocial
April 2023
Title: Is it cost-effective to use genetic tools to predict how well patients with cystic fibrosis will respond to treatment?
Authors Yeva Sahakyan, Lusine Abrahamyan, Felix Ratjen, Christine Bear, Lisa Strug, Paul D W Eckford, John K Peel, Murray Krahn, Beate Sander
References J Cyst Fibros. 2023 Apr 24;S1569-1993(23)00119-4. doi: 10.1016/j.jcf.2023.04.013.
Theme Care of patients with CF, Epidemiology/Models of care
Title: Willingness of people taking elexacaftor/tezacaftor/ivacaftor to be in new CFTR modulator studies
Authors D R VanDevanter, E T Zemanick, M W Konstan, C L Ren, K Odem-Davis, I Emerman, J Young, N Mayer-Hamblett, CHEC-SC Study Group
References J Cyst Fibros. 2023 Apr 24;S1569-1993(23)00096-6. doi: 10.1016/j.jcf.2023.04.007.
April 2023
Title: The combination elexacaftor/tezacaftor/ivacaftor (eti) alters the lipid composition of the lung cells irrespective of CFTR mutation
Authors Nara Liessi, Valeria Tomati, Valeria Capurro, Nicoletta Loberto, Mar Garcia-Aloy, Pietro Franceschi, Massimo Aureli, Nicoletta Pedemonte, Andrea Armirotti
References J Cyst Fibros. 2023 Apr 21;S1569-1993(23)00115-7. doi: 10.1016/j.jcf.2023.04.012.
Keywords dihydroceramides, lipidomics, off-target effect, sphingolipids, triple combination ETI
Title: Cumulative ototoxicity of tobramycin
Authors Justine Madaule, Félix Valenzuela, Marie Mittaine, Yohan Gallois, Blandine Baladi, Marlène Murris, Marie-Noelle Calmels, Didier Concordet, Peggy Gandia
References J Cyst Fibros. 2023 Apr 21;S1569-1993(23)00092-9. doi: 10.1016/j.jcf.2023.04.002.
Keywords Modelling, Ototoxicity, Personalised medicine, Tobramycin
Theme Care of patients with CF, Lung Disease and Infection
April 2023
Title: A mobile app for self-management of CF and improved patient-centred care
Authors Gabriela R Oates, Cathy Mims, Robin Geurs, Rikard Bergquist, Andreas Hager, Jennifer S Guimbellot, Anastasia M Hartzes, Hector H Gutierrez
References J Cyst Fibros. 2023 Apr 19;S1569-1993(23)00113-3. doi: 10.1016/j.jcf.2023.04.009.
Keywords Mobile health, Patient-centered care, Patient-generated health data, Satisfaction with care, Shared decision-making
Theme Epidemiology/Models of care
April 2023
Title: Impact of the De-escalation of Supportive Therapies in Patients with Cystic Fibrosis Taking Elexacaftor, Tezacaftor, Ivacaftor
Authors Emma L Guenther, Karen S McCoy, Mariah Eisner, Shasha Bai, Christopher J Nemastil, Kimberly J Novak, Terri Johnson, Emily M Stephan
References J Cyst Fibros. 2023 Apr 15;S1569-1993(23)00082-6. doi: 10.1016/j.jcf.2023.03.018.
Theme Care of patients with CF
April 2023
Title: Children and Adolescents with CF who have a patchy liver on a research ultrasound (US) are at increased risk of developing advanced CF liver disease
Authors Marilyn J Siegel, Daniel H Leung, Jean P Molleston, Wen Ye, Shruti M Paranjape, A Jay Freeman, Joseph J Palermo, Janis Stoll, Prakash Masand, Boaz Karmazyn, Roger Harned, Simon C Ling, Oscar M Navarro, Wikrom Karnsakul, Adina Alazraki,, Sarah Jane Schwarzenberg, Alex J Towbin, Estella M Alonso, Jennifer L Nicholas, Nicole Green, Randolph K Otto, John C Magee, Michael R Narkewicz
References J Cyst Fibros. 2023 Apr 7;S1569-1993(23)00083-8. doi: 10.1016/j.jcf.2023.03.019
Keywords Cirrhosis, Cystic fibrosis liver disease
Theme Imaging, Nutrition/Gastroenterology
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
