Article
Critical Care Medicine
Heather D. Green, Andrew M. Jones
Summary: Cystic fibrosis is a disease characterized by chronic airway infection and progressive decline in respiratory function. Recent changes in the landscape of airway infection in CF have resulted in an increased prevalence of atypical gram-negative bacterial infections. The clinical relevance and treatment strategies for many of these lower prevalence organisms are still not well understood.
Article
Multidisciplinary Sciences
Satoshi Watanabe, Nikolay S. Markov, Ziyan Lu, Raul Piseaux Aillon, Saul Soberanes, Constance E. Runyan, Ziyou Ren, Rogan A. Grant, Mariana Maciel, Hiam Abdala-Valencia, Yuliya Politanska, Kiwon Nam, Lango Sichizya, Hermon G. Kihshen, Nikita Joshi, Alexandra C. McQuattie-Pimentel, Katherine A. Gruner, Manu Jain, Jacob I. Sznajder, Richard I. Morimoto, Paul A. Reyfman, Cara J. Gottardi, G. R. Scott Budinger, Alexander V. Misharin
Summary: This study revealed that promoting the differentiation of AT2 cells into AT1 cells with ISRIB can accelerate epithelial repair, reduce the recruitment of profibrotic alveolar macrophages, and ameliorate lung fibrosis in the pathogenesis of pulmonary fibrosis.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Review
Infectious Diseases
Lauren C. Magee, Mariam Louis, Vaneeza Khan, Lavender Micalo, Nauman Chaudary
Summary: Cystic Fibrosis is a genetic disease caused by a mutation in the CFTR protein, leading to respiratory issues. CF patients are often colonized or infected with fungi, but diagnosing and treating fungal colonization and active infections in adult CF patients remains challenging. Limited data is available for eradication and treatment.
INFECTION AND DRUG RESISTANCE
(2021)
Article
Immunology
Jonathan D. Cogen, Frankline M. Onchiri, Nicole Mayer Hamblett, Ronald L. Gibson, Wayne J. Morgan, Margaret Rosenfeld
Summary: The study showed that prolonged use of oral antibiotics in children with cystic fibrosis increased the risk of acquiring Achromobacter xylosoxidans, while treatment with intravenous antibiotics was associated with an increased risk of acquiring multidrug-resistant Pseudomonas aeruginosa and MRSA.
CLINICAL INFECTIOUS DISEASES
(2021)
Article
Critical Care Medicine
Yosafe Wakwaya, Deepa Ramdurai, Jeffrey J. Swigris
Summary: Chronic cough in patients with IPF may involve multiple mechanisms, including comorbid conditions and IPF itself. Diagnostic and management approaches need to be multi-faceted, but may lack robust data support.
Article
Gastroenterology & Hepatology
David A. Rudnick, Jiansheng Huang, Tunda Hidvegi, Andrew S. Chu, Pamela Hale, Admire Munanairi, Dennis J. Dietzen, Paul F. Cliften, Eric Tycksen, Andrew J. Lutkewitte, Brian N. Finck, Stephen C. Pak, Gary A. Silverman, David H. Perlmutter
Summary: Insulin signaling exacerbates hepatic proteotoxicity in PiZ mice with alpha 1-antitrypsin deficiency, and PGC1 alpha is identified as a novel therapeutic target.
Article
Pharmacology & Pharmacy
Zara Sheikh, Peta Bradbury, Tristan A. Reekie, Michele Pozzoli, Paul D. Robinson, Michael Kassiou, Paul M. Young, Hui Xin Ong, Daniela Traini
Summary: Current study investigated the potential interactions between the anti-inflammatory agent Ibuprofen and CF-approved inhaled antibiotics, revealing differential effects on the antibacterial and anti-inflammatory efficacy of the combinations. The study highlighted the importance of understanding drug-drug interactions and highlighted the inherent anti-inflammatory properties of the antibiotics used in CF treatment.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2021)
Review
Endocrinology & Metabolism
Sarah S. Malik, Diksha Padmanabhan, Rebecca L. Hull-Meichle
Summary: Cystic fibrosis related diabetes is a common complication in patients with cystic fibrosis, but the mechanisms behind its occurrence are not well understood. Cystic fibrosis affects the pancreatic/islet microenvironment, leading to the loss of beta cell function and pathological changes. Further research can help develop better treatment methods.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Review
Cell Biology
Marjolein M. Ensinck, Marianne S. Carlon
Summary: Cystic fibrosis (CF) is a common monogenic disorder caused by mutations in the CFTR gene. Significant progress has been made in understanding the molecular basis of CF and developing treatments that target the underlying defects. Currently, a highly effective CFTR modulator treatment is available for the majority of CF patients. This review provides an extensive overview of past and ongoing efforts in developing therapies targeting CF defects and discusses potential strategies for patients without causal therapies.
Review
Biochemistry & Molecular Biology
Jia-Huang Chen, Chia-Hsien Wu, Chih-Kang Chiang
Summary: ER stress is a trigger for the development of many kidney diseases, and unfolded protein responses (UPRs) play a role in restoring protein homeostasis under this stress. Modulating UPRs and ER proteostasis may have therapeutic potential for kidney diseases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Critical Care Medicine
Gemma E. Stanford, Kavita Dave, Nicholas J. Simmonds
Summary: Pulmonary exacerbations in cystic fibrosis patients are significant events associated with declining lung function, reduced quality of life, hospitalizations, and decreased survival. With the increasing adult CF population globally, the management of PExs needs to evolve to reflect changing demographics and improve outcomes. Studies are underway to address unmet needs and optimize treatment strategies for PExs in adults with CF.
Review
Pharmacology & Pharmacy
Yizi Wang, Bin Ma, Wenya Li, Peiwen Li
Summary: Triple combination therapy for cystic fibrosis patients achieves better clinical results and comparable adverse events compared to the control group.
FRONTIERS IN PHARMACOLOGY
(2022)
Review
Endocrinology & Metabolism
Amir Moheet, Antoinette Moran
Summary: Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis (CF) and is associated with poor health outcomes. The pathophysiology of CFRD is complex and not fully understood, but factors such as inflammation, alterations in the hormone axis, insulin resistance, and genetic factors may contribute to its development. Further research is needed to better understand the mechanisms underlying CFRD and the impact of diabetes on clinical outcomes in CF.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Article
Respiratory System
Nicola Jane Rowbotham, Sherie Smith, Zoe C. Elliott, Belinda Cupid, Lorna J. Allen, Katherine Cowan, Lucy Allen, Alan Robert Smyth
Summary: Through online surveys and workshops, patients and clinicians selected the revised top 10 priorities for cystic fibrosis clinical research from 971 new research questions and 15 questions from 2018, in order to determine the new focus of research in cystic fibrosis.
Article
Pediatrics
Raphael Enaud, Eric Frison, Sophie Missonnier, Aude Fischer, Victor de Ledinghen, Paul Perez, Stephanie Bui, Michael Fayon, Jean-Francois Chateil, Thierry Lamireau
Summary: This study evaluated the repeatability and reproducibility of TE and pSWE VTQ in children with CF, finding both techniques to be reliable for assessing liver fibrosis. The high ICC values obtained suggest that TE and pSWE VTQ can be used effectively in the follow-up of CF patients, depending on their availability in CF centers.
PEDIATRIC RESEARCH
(2022)
