Article
Biochemistry & Molecular Biology
Francesco Amati, Andrea Gramegna, Martina Contarini, Anna Stainer, Cristina Curcio, Stefano Aliberti, Angelo Guido Corsico, Francesco Blasi
Summary: This study investigated the prevalence of AAT deficiency in CF adults and found that AAT deficiency is not common among adults with CF.
Review
Gastroenterology & Hepatology
Esther Caparros, Reiner Wiest, Michael Scharl, Gerhard Rogler, Ana Gutierrez Casbas, Bahtiyar Yilmaz, Marcin Wawrzyniak, Ruben Frances
Summary: This review delves into the modification of the gut microbiota cluster network during CD progression and discusses how this shift compromises the gut barrier integrity, allowing the translocation of microbes and their products. It also retraces gut microbiota dysbiosis interactions with the main pathophysiologic factors of CD, providing insights on genetic background, immune inflammatory processes, and potential benefits of targeting a specific gut microbiota.
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)
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
Biochemistry & Molecular Biology
Kiera H. Harwood, Rachel M. McQuade, Andrew Jarnicki, Elena K. Schneider-Futschik
Summary: Cystic fibrosis is caused by a defect in the CFTR protein, resulting in respiratory complications and a highly inflammatory environment in the lungs. Neutrophil infiltration plays a critical role in driving inflammation through various pathways. Understanding these mechanisms can lead to the development of new therapeutic targets for CF patients.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Endocrinology & Metabolism
Lise Coderre, Lyna Debieche, Joelle Plourde, Remi Rabasa-Lhoret, Sylvie Lesage
Summary: Cystic fibrosis-related diabetes (CFRD) is a common comorbidity in cystic fibrosis patients, with a prevalence of over 50% in adults. The etiology of CFRD is not fully understood, but research suggests it may result from beta-cell extrinsic factors. Inflammation and ductal cells near beta-cells could play a role in CFRD development.
FRONTIERS IN ENDOCRINOLOGY
(2021)
Article
Chemistry, Medicinal
Sara Consalvi, Giovanna Poce, Carla Ghelardini, Lorenzo Di Cesare Mannelli, Paola Patrignani, Annalisa Bruno, Maurizio Anzini, Vincenzo Calderone, Alma Martelli, Lara Testai, Antonio Giordani, Mariangela Biava
Summary: This review discusses the rational for further studies of COX-2 inhibitors-NO releaser hybrids (NO-Coxibs) in the pharmacological treatment of airway inflammation in Cystic Fibrosis (CF), indicating that previous research results may provide suitable candidates for the treatment of CF.
EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY
(2021)
Review
Cell Biology
Monica Averna, Paola Melotti, Claudio Sorio
Summary: Cystic fibrosis is characterized by pulmonary bacterial colonization and hyperinflammation. White blood cells in CF patients are directly impacted by altered CFTR expression/function, impairing their ability to resolve infections and inflammation. Specific CFTR modulators have been introduced clinically, providing a tool for disease management and investigation into CF pathophysiology. These drugs improve lung function, quality of life, and may correct the dysregulated immune function that characterizes CF. Analysis of CFTR expression and function in leukocytes can provide insights into CF pathogenesis and potential clinical applications.
Review
Biochemistry & Molecular Biology
Anca Daniela Pinzaru, Cristina Maria Mihai, Tatiana Chisnoiu, Alexandru Cosmin Pantazi, Vasile Valeriu Lupu, Mustafa Ali Kassim Kassim, Ancuta Lupu, Elena Grosan, Ahmed Zaki Naji Al Jumaili, Irina Ion, Gabriela Stoleriu, Ileana Ion
Summary: Cystic fibrosis (CF) is the most common inherited condition that leads to death, especially in those of Caucasian heritage. Around 20% of young adults diagnosed with CF will develop hyperglycemia as a complication, which is later classified as a disease associated with CF. Impaired insulin secretion and glucose intolerance are the primary mechanisms associated with diabetes (type 1 or type 2) and CF. Oxidative stress plays a crucial role in triggering other chronic diseases, including CF-related diabetes. Understanding the connection between CF-related diabetes and oxidative stress is essential for individualized therapies.
Article
Pharmacology & Pharmacy
Hongwei Han, Guangda Peng, Maureen Meister, Hongwei Yao, Jenny J. Yang, Ming-Hui Zou, Zhi-Ren Liu, Xiangming Ji
Summary: Exposure to electronic nicotine delivery systems (ENDS) exacerbates airspace enlargement, mucus secretion, and fibrogenesis in COPD mice, along with alterations in inflammatory responses and M2 macrophage phenotype.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Gastroenterology & Hepatology
Alexandre Coimbra, Jordi Rimola, Miriam Cuatrecasas, Gert De Hertogh, Gert Van Assche, Ragna Vanslembrouck, Henning Glerup, Agnete Hedemann Nielsen, Rikke Hagemann-Madsen, Yoram Bouhnik, Magaly Zappa, Dominique Cazals-Hatem, Geert D'Haens, Jaap Stoker, Sybren Meijer, Gerhard Rogler, Andreas Boss, Achim Weber, Rui Zhao, Mary E. Keir, Alexis Scherl, Alex de Crespigny, Timothy T. Lu, Julian Panes
Summary: MRE-based scores are correlated with histologic fibrosis and inflammation in Crohn's disease, and may assist in the characterization and treatment of stenotic patients.
CLINICAL AND TRANSLATIONAL GASTROENTEROLOGY
(2022)
Article
Gastroenterology & Hepatology
Nazli Khodayari, Regina Oshins, Alek M. Aranyos, Sergio Duarte, Sayedamin Mostofizadeh, Yuanqing Lu, Mark Brantly
Summary: We characterized a mouse model of human alpha-1 antitrypsin deficiency with a strong manifestation of liver disease, which can be used as an in vivo tool for testing preventive and therapeutic reagents. Our data explored the altered immunophenotype of alpha-1 antitrypsin-deficient liver macrophages and suggested a relationship between acute inflammation, immune response, and fibrosis.
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Pali Shah, Erin Lowery, Cecilia Chaparro, Gary Visner, Sarah E. Hempstead, James Abraham, Zubin Bhakta, Maggie Carroll, Lillian Christon, Lara Danziger-Isakov, Joshua M. Diamond, Erika Lease, Jessica Leonard, Marina Litvin, Ray Poole, Fanny Vlahos, Chelsey Werchan, Michelle A. Murray, Erin Tallarico, Albert Faro, Joseph M. Pilewski, Ramsey R. Hachem
Summary: Cystic Fibrosis Lung Transplant Recipients have good long-term outcomes but require specialized care. This document provides recommendations for the management of perioperative and underlying comorbidities of CFLTRs.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2021)
Article
Immunology
Ruchi Yadav, Quan-Zhen Li, Hanwen Huang, S. Louis Bridges, J. Michelle Kahlenberg, Arlene A. Stecenko, Balazs Rada
Summary: This study identified several new autoantibodies in the blood of CF patients and found associations with neutrophil components, diabetes, lung disease severity, and P. aeruginosa infection.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Pediatrics
Evi Jaspers, Ine Van Dijck, Ilse Hoffman, Noel Knops, Xavier Stephenne, Peter Witters, Marijke Proesmans
Summary: This case report describes a child born with both cystic fibrosis (CF) and alpha-1 antitrypsin deficiency (A1ATD), two rare inherited diseases that mainly affect the lungs and liver. The diagnosis of CF was confirmed by sweat test and genetic analysis, while the diagnosis of A1ATD was based on clinical suspicion and genetic analysis. The child developed severe liver disease and required an infant liver transplantation.