Article
Respiratory System
Michelle Casey, Claudie Gabillard-Lefort, Oisin F. McElvaney, Oliver J. McElvaney, Tomas Carroll, Ronan C. Heeney, Cedric Gunaratnam, Emer P. Reeves, Mark P. Murphy, Noel G. McElvaney
Summary: Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to improve lung function in people with cystic fibrosis (PWCF). This study investigates the biological effects of ETI on pulmonary and systemic inflammation in PWCF. The results demonstrate that ETI reduces inflammatory markers and normalizes protein levels, suggesting its immunomodulatory effects and potential as a disease modifier.
Article
Critical Care Medicine
Daniel P. Cook, Christopher M. Thomas, Ashley Y. Wu, Mark Rusznak, Jian Zhang, Weisong Zhou, Jacqueline-Yvonne Cephus, Katherine N. Gibson-Corley, Vasiliy V. Polosukhin, Allison E. Norlander, Dawn C. Newcomb, David A. Stoltz, R. Stokes Peebles
Summary: Type 2 inflammation is observed in cystic fibrosis patients. This study reveals that CFTR deficiency leads to increased expression and release of IL-33, and deletion of IL-33 reduces allergen-induced inflammation in the CF lung.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Immunology
Thomas Plante-Bordeneuve, Charles Pilette, Antoine Froidure
Summary: The interaction between lung epithelium and the immune system plays a crucial role in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Pathways such as DAMPs-PAMPs are highlighted as potential mechanisms involved in disease progression.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Pharmacology & Pharmacy
Li Chen, Desong Kong, Siwei Xia, Feixia Wang, Zhanghao Li, Feng Zhang, Shizhong Zheng
Summary: Liver fibrosis is a repair process of chronic liver injuries, and its progression relies on the activation of hepatic stellate cells and remodeling of the extracellular matrix. Liver fibrosis can lead to liver dysfunction and hepatocellular carcinoma. Innate immune cells and autophagy play complex roles in liver fibrosis. Further research on these roles is crucial for the prevention and treatment of liver fibrosis.
FRONTIERS IN PHARMACOLOGY
(2022)
Review
Multidisciplinary Sciences
Anand Kumar Purushothaman, Everette Jacob Remington Nelson
Summary: Pathophysiological manifestations of cystic fibrosis (CF) arise from a functional defect in the cystic fibrosis transmembrane conductance regulator (CFTR), leading to mucus obstruction and pathogen colonization. The role of CFTR in modulating immune cell function and vascular integrity, regardless of mucus thickening, in determining the host's response to pathogens/allergens and causing systemic inflammation is often overlooked. This review highlights the significance of innate immunity and systemic inflammatory response in the development of CF and other CFTR-associated pathologies.
Editorial Material
Immunology
Xinxin Wang, Yuping Lai
Summary: In this study, the authors investigate the mechanism by which pattern recognition receptors NOD1 and NOD2 sense bacterial muropeptides to induce inflammation in keratinocytes. They demonstrate that solute carrier 46A2 (SLC46A2) transports DAP-muropeptides into the cytosol, leading to NOD1 activation and subsequent skin inflammation during psoriasis.
Review
Biochemistry & Molecular Biology
Genta Ishikawa, Angela Liu, Erica L. Herzog
Summary: This review synthesizes available data regarding the complex role of innate immunity in IPF, discussing substances present in the fibrotic microenvironment, such as PAMPs and DAMPs, and the proposed contribution of innate immune populations. The relationships to environmental and genetic factors, disease outcomes, and potential therapies are considered, along with unanswered questions and future study opportunities.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2021)
Article
Respiratory System
Andrew Achaiah, Emily Fraser, Peter Saunders, Rachel K. Hoyles, Rachel Benamore, Ling-Pei Ho
Summary: This study shows that neutrophil levels, rather than monocyte levels, are correlated with a quantifiable increase in fibrosis on lung imaging in patients with IPF, indicating their greater contribution to the progression of fibrosis in IPF.
BMJ OPEN RESPIRATORY RESEARCH
(2023)
Review
Cell Biology
Sophie Gohy, Alexandra Moeremans, Charles Pilette, Amandine Collin
Summary: The respiratory epithelium serves as the first line of defense against inhaled pathogens in cystic fibrosis patients, with mucosal alterations and local immunity linked to immunoglobulin A playing crucial roles in inflammation and bacterial overgrowth within the airways.
Article
Oncology
Wenyong Huang, Dongmei Ye, Wenjing He, Xiaoshun He, Xiaomin Shi, Yifang Gao
Summary: The study found that MAIT cells in patients with HCC had lower levels in both peripheral blood and liver compared to healthy controls, with reduced IFN-γ production. While peripheral MAIT cells showed upregulation of HLA-DR and PD-1, intrasinusoidal MAIT cells did not exhibit significant differences. Additionally, MAIT cells were significantly enriched in the livers of HCC patients, showing high levels of activation and exhaustion markers.
JOURNAL FOR IMMUNOTHERAPY OF CANCER
(2021)
Review
Immunology
Daniela E. Kirwan, Deborah L. W. Chong, Jon S. Friedland
Summary: In 2019, 10 million people developed symptomatic tuberculosis (TB) disease, resulting in 1.2 million deaths. The tissue destruction in TB is mainly mediated by enzymes, particularly matrix metalloproteinases (MMPs) secreted by leukocytes and stromal cells. Platelets are implicated in TB immunopathology, with high platelet counts in TB patients correlating with disease severity and a hypercoagulable profile. Platelets likely drive TB immunopathology by influencing other immune cells, such as monocytes, leading to increased activation markers, MMP secretion, and enhanced phagocytosis. Targeted anti-platelet agents are being considered for the treatment of TB as part of host-directed therapies.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Luca Occhigrossi, Federica Rossin, Valeria Rachela Villella, Speranza Esposito, Carlo Abbate, Manuela D'Eletto, Maria Grazia Farrace, Antonella Tosco, Roberta Nardacci, Gian Maria Fimia, Valeria Raia, Mauro Piacentini
Summary: Cystic fibrosis (CF) is a rare genetic disease caused by mutations in the CFTR gene, leading to chronic lung infections. This study reveals the link between recurrent infections in CF patients and impaired innate immune response. The use of STING pathway agonists can restore the immune response and potentially be utilized as a new treatment strategy.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Luca Fabris, Chiara Milani, Romina Fiorotto, Valeria Mariotti, Eleanna Kaffe, Barbara Seller, Aurelio Sonzogni, Mario Strazzabosco, Massimiliano Cadamuro
Summary: In this study, the role of Scribble, Yes-associated protein (YAP), and beta-catenin in regulating the fibroinflammatory phenotype of FPC-defective cholangiocytes was investigated. The results showed that defective anchor of Scribble to the membrane facilitates the nuclear translocation of YAP and beta-catenin, leading to the development of a fibroinflammatory phenotype in the cholangiocytes.
Review
Immunology
Sarah Cormican, Matthew D. Griffin
Summary: Innate immune cells play a key role in kidney inflammation and fibrosis, with the CX3CL1-CX3CR1 signaling pathway being closely linked to acute and chronic kidney diseases. Targeting this pathway may offer potential benefits for patients with CKD.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Dilan Khalili, Christina Kalcher, Stefan Baumgartner, Ulrich Theopold
Summary: The study reveals that expressing a dominant-active form of the Ras oncogene in Drosophila salivary glands leads to fibrotic lesions, which can be alleviated by the antimicrobial peptide drosomycin. This fibrotic lesion is characterized by disturbed basement membrane attracting clot components and a loss of cell polarity.
JOURNAL OF INNATE IMMUNITY
(2021)
Letter
Immunology
Harriet Corvol, Aurelia Alimi, Blandine Prevost, Aurelie Schnuriger, Hubert Ducou Le Pointe, Jerome Rambaud, Marie-Dominique Tabone
CLINICAL INFECTIOUS DISEASES
(2022)
Article
Respiratory System
Julie Mesinele, Manon Ruffin, Astrid Kemgang, Loic Guillot, Pierre-Yves Boelle, Harriet Corvol
Summary: This study investigated the epidemiology of Pseudomonas aeruginosa infection and its impact on lung function in cystic fibrosis patients. The results showed that CF-related diabetes and liver disease were risk factors for infection, and lung function declined significantly after infection.
JOURNAL OF CYSTIC FIBROSIS
(2022)
Article
Respiratory System
A. Deschildre, R. Abou Taam, D. Drummond, L. Giovannini-Chami, G. Labouret, S. Lejeune, G. Lezmi, M. T. Lecam, C. Marguet, H. Petat, C. Taille, S. Wanin, H. Corvol, R. Epaud
REVUE DES MALADIES RESPIRATOIRES
(2022)
Article
Respiratory System
A. Deschildre, R. Abou-Taam, D. Drummond, L. Giovannini-Chami, G. Labouret, S. Lejeune, G. Lezmi, M. T. Lecam, C. Marguet, H. Petat, C. Taille, S. Wanin, H. Corvol, R. Epaud
REVUE DES MALADIES RESPIRATOIRES
(2022)
Letter
Immunology
Guillaume Thouvenin, Blandine Prevost, Harriet Corvol
JOURNAL OF INFECTIOUS DISEASES
(2023)
Article
Clinical Neurology
Marie Cheminelle, Marie-Christine Nougues, Arnaud Isapof, Guillaume Aubertin, Harriet Corvol, Nicole Beydon, Jessica Taytard
Summary: This study investigates the sleep and respiratory disorders in children with DM1 disease, finding that the majority of the children exhibit symptoms of sleep disordered breathing, and some require non-invasive ventilation treatment. The study provides useful insights for respiratory follow-up and management in children with DM1 disease, and suggests the need for further research.
NEUROMUSCULAR DISORDERS
(2023)
Review
Pediatrics
Harriet Corvol, Blandine Prevost, Guillaume Aubertin, Guillaume Thouvenin, Jessica Taytard, Nadia Nathan
Summary: This manuscript describes the experience of a pediatric respiratory unit during the first year of the COVID-19 pandemic, focusing on children and young people with underlying conditions such as immunosuppression, sickle cell disease, and cystic fibrosis. It discusses the observation of severe pediatric and young adult cases, post-infectious inflammatory syndromes, and long-COVID.
PEDIATRIC PULMONOLOGY
(2023)
Article
Microbiology
Jeanne Bigot, Manon Ruffin, Juliette Guitard, Sandra Vellaissamy, Sophie Thorez, Harriet Corvol, Loic Guillot, Viviane Balloy, Christophe Hennequin
Summary: Bronchial epithelial cells (BEC) play a crucial role in innate immunity against inhaled fungi by synthesizing proinflammatory cytokines and exerting antifungal activity. The innate immune memory of BEC allows them to enhance their inflammatory response against fungal pathogens, potentially offering new therapeutic options for chronic pulmonary diseases.
Article
Critical Care Medicine
Yi-Hui Zhou, Paul J. Gallins, Rhonda G. Pace, Hong Dang, Melis A. Aksit, Elizabeth E. Blue, Kati J. Buckingham, Joseph M. Collaco, Anna V. Faino, William W. Gordon, Kurt N. Hetrick, Hua Ling, Weifang Liu, Frankline M. Onchiri, Kymberleigh Pagel, Elizabeth W. Pugh, Karen S. Raraigh, Margaret Rosenfeld, Quan Sun, Jia Wen, Yun Li, Harriet Corvol, Lisa J. Strug, Michael J. Bamshad, Scott M. Blackman, Garry R. Cutting, Ronald L. Gibson, Wanda K. O'Neal, Fred A. Wright, Michael R. Knowles
Summary: This study aims to identify genetic modifier loci associated with pulmonary disease severity in patients with cystic fibrosis. The research used whole-genome sequencing data and genetic genotypes to discover two loci associated with lung disease. These findings support mechanistic studies and the development of novel therapeutics for CF lung disease.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Health Care Sciences & Services
Nicole Beydon, Camille Taille, Harriet Corvol, Judith Valcke, Jean-Jacques Portal, Laurent Plantier, Gilles Mangiapan, Caroline Perisson, Guillaume Aubertin, Alice Hadchouel, Guillaume Briend, Laurent Guilleminault, Catherine Neukirch, Pierrick Cros, Corinne Appere de Vecchi, Bruno Mahut, Eric Vicaut, Christophe Delclaux
Summary: This study aimed to compare the effect of a smartphone web app and a paper action plan on unscheduled medical contacts for children and adults with asthma. The results showed that the smartphone web app did not decrease the number of medical contacts compared to the paper action plan.
JOURNAL OF MEDICAL INTERNET RESEARCH
(2023)
Letter
Pediatrics
Sebastien Heritier, Jean Donadieu, Pierre-Louis Leger, Marie De Tersant, Valeria Della Vallee, Mohamed-Aziz Barkaoui, Solenne Le Louet, Abdellatif Tazi, Jessica Taytard, Harriet Corvol, Zofia Helias-Rodzevicz, Jean-Francois Emile, Elie Fadel, Dominique Fabre, Severine Feuillet, Nadia Nathan
PEDIATRIC PULMONOLOGY
(2023)
Article
Pediatrics
Elora Peulier-Maitre, Erik Hervieux, Jeanne Bigot, Chiara Sileo, Sophie Denamur, Harriet Corvol
Summary: This case report presents a 13-year-old boy with pulmonary cystic echinococcosis, who presented with low-volume hemoptysis. Lung imaging revealed a large cystic mass and smaller pseudo-nodular lesions, indicating a large intrathoracic hydatid cyst and ruptured cysts. The diagnosis was confirmed by a positive echinococcosis Western Blot assay. Treatment involved surgical removal of the large cyst, followed by a two-week course of albendazole and praziquantel, and then two years of albendazole. The patient had a successful recovery.
PEDIATRIC PULMONOLOGY
(2023)
Article
Pediatrics
Julie Cassibba, Ralph Epaud, Laureline Berteloot, Sabrina Aberbache, Lauren Bitton, Camille Fletcher, Manon Fleury, Celine Delestrain, Harriet Corvol, Alix de Becdelievre, Raphael Borie, Marie Legendre, Aurore Coulomb l'Hermine, Camille Louvrier, Celine Lustremant, Meryem Sari Hassoun, Chiara Sileo, Alice Hadchouel, Nadia Nathan
Summary: The study found that multidisciplinary team meetings (MDTm) for childhood interstitial lung disease (chILD) can improve the diagnosis and management of the disease, increasing physician confidence in handling these cases.
PEDIATRIC PULMONOLOGY
(2023)
Meeting Abstract
Respiratory System
L. Guillot, J. Oliva, M. Ruffin, C. Calmel, H. Corvol, O. Terrier
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Genetics & Heredity
Quan Sun, Weifang Liu, Jonathan D. Rosen, Le Huang, Rhonda G. Pace, Hong Dang, Paul J. Gallins, Elizabeth E. Blue, Hua Ling, Harriet Corvol, Lisa J. Strug, Michael J. Bamshad, Ronald L. Gibson, Elizabeth W. Pugh, Scott M. Blackman, Garry R. Cutting, Wanda K. O'Neal, Yi-Hui Zhou, Fred A. Wright, Michael R. Knowles, Jia Wen, Yun Li
Summary: In this study, the researchers conducted genome-wide association studies (GWAS) and whole genome sequencing (WGS) on cystic fibrosis (CF) cases using genotype imputation. The results showed that imputation increased the sample size and improved the accuracy of polygenic risk score (PRS) prediction. They also built a CF-specific reference panel that can benefit other investigators studying CF.
HUMAN GENETICS AND GENOMICS ADVANCES
(2022)
