Results 71 to 80 of about 92,053 (154)

Cystic Fibrosis: Understanding Cystic Fibrosis Transmembrane Regulator Mutation Classification and Modulator Therapies

Advances in Respiratory Medicine
A common life-threatening hereditary disease, Cystic Fibrosis (CF), affects primarily Caucasian infants. High sweat-salt levels are observed as a result of a single autosomal mutation in chromosome 7 that affects the critical function of the cystic ...
Saba Anwar, Jin-Liang Peng, Kashif Rafiq Zahid, Yu-Ming Zhou, Qurban Ali, Chong-Rong Qiu   +5 more
doaj   +1 more source

Cystic Fibrosis and Colorectal Cancer Risk: Reprogramming of the Intestinal Epithelial Niche and Cell‐State Plasticity in the CFTR Modulator Era

Cell Proliferation, EarlyView.
In cystic fibrosis disorder, CFTR dysfunction drives multilayered intestinal niche disruption, including dysbiosis, inflammation, hypoxia, metabolic stress and genotoxic injury, promoting cell‐state plasticity and a pre‐neoplastic ecosystem. CFTR modulators and environmental factors may partially reshape these processes, highlighting opportunities for ...
Bala Umashankar, Ines Pankonien, Margarida Amaral, Chee Y. Ooi, Thierry Jardé, Shafagh A. Waters   +5 more
wiley   +1 more source

Olfactory Tuft Cells Are Critical to Basal Inflammation, Innate Immune Response to Viral Infection, and Modulation of Quiescent Stem Cell Activation, Proliferation and Differentiation

Cell Proliferation, EarlyView.
Olfactory tuft cells communicate with other mucosal cells in maintaining olfactory epithelial immunity and sensory function. Disruption of tuft cell signalling pathways causes a skewed immune response, leading to heightened inflammation and increased activation of the quiescent stem cells, which are further exacerbated by viral infection.
Sai‐Sai Zhang, Haiyan Wang, Yi‐Sen Yang, Yi‐Hong Li, Defu Yu, Yushi Yao, Ruhong Zhou, Liquan Huang   +7 more
wiley   +1 more source

Pharmacological Modulation of Ion Channels for the Treatment of Cystic Fibrosis

Journal of Experimental Pharmacology, 2021
Madalena C Pinto,1,* Iris AL Silva,1,* Miriam F Figueira,2 Margarida D Amaral,1 Miquéias Lopes-Pacheco1,* 1Biosystems & Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Lisboa, Portugal; 2Marsico Lung ...
Pinto MC, Silva IAL, Figueira MF, Amaral MD, Lopes-Pacheco M   +4 more
doaj  

Aligning clinical care with consumer priorities: an adult cystic fibrosis centre experience of co‐designing a model of care

Internal Medicine Journal, EarlyView.
Abstract Cystic fibrosis (CF) is a multi‐system genetic condition, and CF modulator therapies have transformed health outcomes promising improved longevity. Our aim was to co‐design and implement a nested endocrine and metabolic model of care called CF Endocrine for adults with CF that aligned with consumer and healthcare provider priorities and ...
Shanal Kumar, Vanessa Moore, Daniel Smith, Angela Matson   +3 more
wiley   +1 more source

Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey.

, 2020
BACKGROUND As their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR modulators in pregnancy and breastfeeding is currently unknown.
Taylor-Cousar, Jennifer L, Nash, Edward F, Middleton, Peter G   +2 more
core   +1 more source

Medication adherence to CFTR modulators in patients with cystic fibrosis: a systematic review

European Respiratory Review
Background In the last decade, a fundamental shift in the treatment of cystic fibrosis (CF) took place due to the introduction of CF transmembrane conductance regulator (CFTR) modulators.
Carina M.E. Hansen, Anna J. Breukelman, Patricia M.L.A. van den Bemt, Annelies M. Zwitserloot, Liset van Dijk, Job F.M. van Boven   +5 more
doaj   +1 more source

Continuous glucose monitoring detects spontaneous reactive and fasting hypoglycaemia in cystic fibrosis adults without diabetes

Internal Medicine Journal, EarlyView.
Abstract People with cystic fibrosis (pwCF) experience a spectrum of glucose disorders. Spontaneous hypoglycaemia is a unique entity affecting pwCF and can be an incidental finding on biochemistry. Our prospective, single‐arm pilot study of eight pwCF without confirmed diabetes and incidental biochemical hypoglycaemia found 62.5% demonstrated fasting ...
Shanal Kumar, Karen Hay, Felicity Loel, Angela Matson   +3 more
wiley   +1 more source

Impact of CFTR modulators on chronic and intermittent bronchial infection

Background: Sputum microbiology influences the prognosis of lung disease in people with CF. As CFTR modulators change the bronchial microenvironment, we studied the effect of the introduction of CFTR modulators on intermittent and chronic bacterial ...
Bugli, Céline, Berardis, Silvia, 2025 ERS Congress, Goubau, Christophe, Gohy, Sophie, Sluijters, Alice, Stavart, Corentin   +6 more
core   +1 more source

Adaptation to extreme environments: A review of Antarctic fish physiology

Journal of Fish Biology, EarlyView.
Abstract Having evolved in the chronically cold and thermally stable waters of the Southern Ocean (SO) for millions of years, Antarctic fish possess a suite of physiological specializations that enable them to survive at temperatures near the freezing point of seawater. Adaptations include the evolution of antifreeze proteins, modifications of membrane
Seungyeon Lee, Sachithra Amarin Hettiarachchi, Il‐Chan Kim, Jae‐Seong Lee, Thomas Desvignes, Julia M. York, Laura Ghigliotti, Jilda Alicia Caccavo, Jin‐Hyoung Kim   +8 more
wiley   +1 more source