Article
Multidisciplinary Sciences
Maria Pino-Argumedo, Anthony J. Fischer, Brieanna M. Hilkin, Nicholas D. Gansemer, Patrick D. Allen, Eric A. Hoffman, David A. Stoltz, Michael J. Welsh, Mahmoud H. Abou Alaiwa
Summary: Defective mucociliary transport in cystic fibrosis (CF) lungs is caused by abnormally elastic mucus strands that impair clearance. Breaking these mucus strands can improve mucociliary clearance in CF.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Medicine, General & Internal
Eva Steinke, Olaf Sommerburg, Simon Y. Graeber, Cornelia Joachim, Christiane Labitzke, Gyde Nissen, Isabell Ricklefs, Isa Rudolf, Matthias V. Kopp, Anna-Maria Dittrich, Marcus A. Mall, Mirjam Stahl
Summary: This study is a prospective, longitudinal cohort study aimed at determining influencing factors of early cystic fibrosis (CF) lung disease through the combined analysis of clinical information and biomaterials. The primary endpoints are the lung clearance index and magnetic resonance imaging scores, while secondary endpoints include pulmonary exacerbations, infection with pro-inflammatory pathogens, and anthropometric data.
FRONTIERS IN MEDICINE
(2023)
Article
Multidisciplinary Sciences
Wenjie Yu, Thomas O. Moninger, Andrew L. Thurman, Yuliang Xie, Akansha Jain, Keyan Zarei, Linda S. Powers, Alejandro A. Pezzulo, David A. Stoltz, Michael J. Welsh
Summary: Submucosal glands (SMGs) play a crucial role in protecting the lungs but can also contribute to disease. In cystic fibrosis (CF), SMGs produce abnormal mucus that disrupts mucociliary transport. This study used single-cell RNA sequencing, immunohistochemistry, and in situ hybridization to investigate the expression and distribution of ion transporters and cell types in SMGs. The results suggest that loss of epithelial anion secretion, rather than intrinsic cell defects, causes CF mucus abnormalities. The study also revealed different functions of acinar mucous cells and acinar serous cells in producing mucins and antimicrobials, respectively.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Respiratory System
Christina Thornton, Ranjani Somayaji, Angel Chu, Michael D. Parkins
Summary: Human papillomavirus (HPV) is a major risk factor for cervical cancer, and transplant recipients have a higher risk of HPV complications. A retrospective study on adult female cystic fibrosis (CF) lung transplant recipients found that 35.3% had abnormal pap smear results, including refractory anogenital warts, vulvectomy, and cervical cancer. The study also revealed that lung transplant recipients had a higher likelihood of cervical dysplasia compared to the control group, emphasizing the importance of HPV vaccination to prevent future morbidity and mortality.
Article
Psychology, Multidisciplinary
Deborah Friedman, Maysa M. Kaskas, Alexandra L. Quittner, Beth A. Smith, Anna M. Georgiopoulos
Summary: Individuals with cystic fibrosis (CF) are at high risk for depression and anxiety. This study developed a CBT-based intervention tailored to CF-specific needs, with positive feedback from patients.
FRONTIERS IN PSYCHOLOGY
(2022)
Article
Health Care Sciences & Services
Tomasz Hildebrandt, Elzbieta Swietochowska, Agata Trzcionka, Anna Zawilska, Henryk Mazurek, Dagmara Maczkowiak, Mansur Rahnama, Marta Tanasiewicz
Summary: This study assessed the oral hygiene status of adult cystic fibrosis patients and found that their oral hygiene was satisfactory. However, professional preventive treatments were needed.
Article
Mycology
John E. Moore, Jacqueline C. Rendall, Beverley C. Millar
Summary: This study examined the correlation between time to first isolation of A. fumigatus and patient gender and CFTR mutation type. The results showed a significant difference in time to first isolation between CFTR mutation groups, but not between genders. It is suggested that infection prevention education about A. fumigatus acquisition should be strengthened in pediatric clinics.
Article
Respiratory System
Anna Semaniakou, Frederic Chappe, Younes Anini, Valerie Chappe
Summary: Investigation revealed decreased VIP levels and reduced innervation in the pancreas of CF mice, leading to decreased insulin secretion, increased glucagon production, and elevated random blood glucose levels. Therefore, VIP deficiency may contribute to the development of CFRD.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Review
Pediatrics
Marie E. Egan
Summary: Effective CFTR modulator therapy may not work for all CF patients, leading to the need for genetic-based therapies; these new therapies have the potential for a one-time cure.
PEDIATRIC PULMONOLOGY
(2021)
Article
Chemistry, Medicinal
Marta Guerini, Giorgia Condro, Valeria Friuli, Lauretta Maggi, Paola Perugini
Summary: N-acetylcysteine is a powerful antioxidant and antidote for paracetamol overdose. It has also shown potential as a drug for diseases like cystic fibrosis, playing a role in reducing inflammation and restoring redox balance. Additionally, NAC has been found to prevent and eliminate biofilms caused by CF airway infections.
Article
Critical Care Medicine
Sameer Desai, Sanja Stanojevic, Grace Y. Lam, Anne L. Stephenson, Bradley S. Quon
Summary: Individuals diagnosed with cystic fibrosis (CF) as adults show stable lung function in early adulthood, a decline in middle adulthood, and a greater decline after 50 years of age. Older individuals and those with pulmonary symptoms at diagnosis experience a faster rate of lung function decline and should be monitored more closely.
Article
Critical Care Medicine
Helge Hebestreit, Susi Kriemler, Christian Schindler, Lothar Stein, Chantal Karila, Don S. Urquhart, David M. Orenstein, Larry C. Lands, Jonathan Schaeff, Ernst Eber, Thomas Radtke
Summary: This study aimed to evaluate the effects of a 12-month partially supervised physical activity intervention on lung function in cystic fibrosis patients. The results showed that the intervention increased vigorous physical activity and exercise capacity, but the control group had better FEV1 effects.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Article
Cell Biology
Eric Barbato, Rebecca Darrah, Thomas J. Kelley
Summary: Disordered sleep in people with cystic fibrosis (CF) may be associated with disrupted circadian regulation due to loss of cystic fibrosis transmembrane conductance regulator (Cftr) function. A CF mouse model showed significant alterations in locomotor activity timing and duration, as well as disrupted circadian gene expression and reduced melatonin production. Microtubules were found to play a role in regulating these outcomes, and targeting this microtubule-related mechanism may provide a therapeutic intervention for CF.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Maya Desai, Chris Hine, Joanna L. Whitehouse, Keith Brownlee, Susan C. Charman, Prasad Nagakumar
Summary: Patients from black, Asian and minority ethnic backgrounds are significantly less likely to be eligible for ivacaftor/tezacaftor/elexacaftor based on the current prescribing policy in the UK. These findings highlight the need for effective targeted therapies for cystic fibrosis patients without the F508del mutation.
RESPIRATORY MEDICINE
(2022)
Article
Endocrinology & Metabolism
Rebecca Hicks, Katie Larson Ode, Tim Vigers, Christine L. Chan
Summary: A study was conducted to examine the screening practices for cystic fibrosis related diabetes (CFRD) and identify barriers and strategies for improvement. Surveys were conducted among CF center directors and endocrinologists, and the results showed low screening rates and practice variations. Use of systematic reminders and further research are recommended to improve CFRD screening.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Article
Respiratory System
Isobel E. R. MacKenzie, Valerie Paquette, Frances Gosse, Sheenagh George, Frederic Chappe, Valerie Chappe
JOURNAL OF CYSTIC FIBROSIS
(2017)
Article
Medicine, Research & Experimental
Maral Aali, Alexa Caldwell, Kelsey House, Juan Zhou, Valerie Chappe, Christian Lehmann
MEDICAL HYPOTHESES
(2017)
Review
Pharmacology & Pharmacy
Diogo R. Poroca, Ryan M. Pelis, Valerie M. Chappe
FRONTIERS IN PHARMACOLOGY
(2017)
Article
Cell Biology
Gage Seavilleklein, Noha Amer, Alexandra Evagelidis, Frederic Chappe, Thomas Irvine, John W. Hanrahan, Valerie Chappe
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2008)
Article
Cell Biology
Nicole Alcolado, Dustin J. Conrad, Sara Rafferty, Frederic G. Chappe, Valerie M. Chappe
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2011)
Article
Cell Biology
Nicole G. Alcolado, Dustin J. Conrad, Diogo Poroca, Mansong Li, Walaa Alshafie, Frederic G. Chappe, Ryan M. Pelis, Younes Anini, Zhaolin Xu, Sayyed Hamidi, Sami I. Said, Valerie M. Chappe
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2014)
Article
Cell Biology
Walaa Alshafie, Frederic G. Chappe, Mansong Li, Younes Anini, Valerie M. Chappe
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2014)
Article
Pharmacology & Pharmacy
Frederic Chappe, Matthew E. Loewen, John W. Hanrahan, Valerie Chappe
JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS
(2008)
Article
Pharmacology & Pharmacy
Sara Rafferty, Nicole Alcolado, Caroline Norez, Frederic Chappe, Siegried Pelzer, Frederic Becq, Valerie Chappe
JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS
(2009)
Article
Critical Care Medicine
Marilyn Macdonald, Ariella Lang, Eileen Savage, Valerie Chappe, Andrea Murphy, Frances Gosse, Heather MacLean
Article
Respiratory System
Anna Semaniakou, Sarah Brothers, Grayson Gould, Mehrsa Zahiremani, Jamie Paton, Frederic Chappe, Audrey Li, Younes Anini, Roger P. Croll, Valerie Chappe
Summary: Vasoactive Intestinal Peptide (VIP) is a major physiological agonist of CFTR chloride channel activity, functioning as a vasodilator, bronchodilator, and regulator of exocrine gland secretions. Reduction of VIP in CF tissues may represent an early aggravating factor for cystic fibrosis progression.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Biochemistry & Molecular Biology
Christian Lehmann, Nazli Alizadeh-Tabrizi, Stefan Hall, Sufyan Faridi, Irene Euodia, Bruce Holbein, Juan Zhou, Valerie Chappe
Summary: The iron chelator, DIBI, exhibits beneficial anti-inflammatory effects in experimental acute lung injury by reducing histopathological damage, inflammatory mediator levels, and NF-kappa B p65 activation.
Review
Biochemistry & Molecular Biology
Bader N. Alamri, Kyungsoo Shin, Valerie Chappe, Younes Anini
HORMONE MOLECULAR BIOLOGY AND CLINICAL INVESTIGATION
(2016)
Article
Biochemistry & Molecular Biology
V Chappe, T Irvine, J Liao, A Evagelidis, JW Hanrahan