Article
Medicine, General & Internal
Anna Engell Holm, Hans Henrik Lawaetz Schultz, Helle Krogh Johansen, Tania Pressler, Thomas Kromann Lund, Martin Iversen, Michael Perch
Summary: This study monitored the time of bacterial re-colonization in lung transplant patients with cystic fibrosis and found that 70% of patients were re-colonized by bacteria after 2 years, with no observed impact on survival rates from re-colonization or different bacterial species.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Otorhinolaryngology
Joseph R. Johnson, Peter H. Hwang, Jayakar Nayak, Zara M. Patel
Summary: This study examined the effects of endoscopic sinus surgery (ESS) timing on pulmonary health in lung transplant patients with cystic fibrosis (CF). The results showed that early ESS interventions following lung transplantation can improve pulmonary function, attenuate pulmonary exacerbations, and reduce the need for antibiotic use.
INTERNATIONAL FORUM OF ALLERGY & RHINOLOGY
(2022)
Review
Pharmacology & Pharmacy
Rosy Ghanem, Veronique Laurent, Philippe Roquefort, Tanguy Haute, Sophie Ramel, Tony Le Gall, Thierry Aubry, Tristan Montier
Summary: The respiratory epithelium is susceptible to diseases like cystic fibrosis, which impact airway function. Artificial mucus models play a crucial role in evaluating gene transfer systems' effectiveness in reaching the epithelium.
Article
Cell Biology
Juliette L. Simonin, Alexandre Luscher, Davide Losa, Mehdi Badaoui, Christian van Delden, Thilo Kohler, Marc Chanson
Summary: Defective hydration of airway surface mucosa is associated with recurrent lung infection in cystic fibrosis. The presence of a healthy airway surface liquid can prevent infection.
Article
Medicine, Research & Experimental
Giandomenico Maggiore, Michele Pietragalla, Christian De Amicis, Cosimo Nardi, Chiara Bruno, Oreste Gallo, Luigi Bonasera, Anna Perrone, Annalisa Cavallo, Stefano Colagrande, Giovanni Taccetti, Luca Giovanni Locatello
Summary: Adult cystic fibrosis (CF) patients are increasingly undergoing endoscopic sinus surgery (ESS) for chronic rhinosinusitis (CRS) symptoms. Anatomical differences were found in CF patients compared to non-CF patients, but no increased risk of postoperative complications was seen in the CF group despite more aggressive surgical procedures.
Article
Biochemistry & Molecular Biology
Jaehi Chung, Felix Wuennemann, Johanna Salomon, Sebastien Boutin, Dario L. Frey, Tobias Albrecht, Cornelia Joachim, Monika Eichinger, Marcus A. Mall, Mark O. Wielpuetz, Olaf Sommerburg
Summary: This study found a positive correlation between inflammation and structural abnormalities in MRI in cystic fibrosis patients, with pro-inflammatory markers NE and NE/A1AT complex levels significantly correlating with CRS-MRI sum score. S. aureus infection was associated with increased levels of IL-6 and IL-8, indicating these biomarkers as signs of CRS pathology in CF patients.
Article
Otorhinolaryngology
Andrew V. Thamboo, Melissa Lee, Mohit Bhutani, Charles Chan, Yvonne Chan, Ken R. Chapman, Christopher J. Chin, Lori Connors, Del Dorscheid, Anne K. Ellis, Richard M. Gall, Krystelle Godbout, Arif Janjua, Amin Javer, Shaun Kilty, Harold Kim, Gordon Kirkpatrick, John M. Lee, Richard Leigh, Catherine Lemiere, Eric Monteiro, Helen Neighbour, Paul K. Keith, George Philteos, Jaclyn Quirt, Brian Rotenberg, Juan C. Ruiz, John R. Scott, Doron D. Sommer, Leigh Sowerby, Marc Tewfik, Susan Waserman, Ian Witterick, Erin D. Wright, Cory Yamashita, Martin Desrosiers
Summary: This study focuses on the use of biologic therapy for the management of upper airway disease, and provides consensus recommendations for Canadian physicians.
JOURNAL OF OTOLARYNGOLOGY-HEAD & NECK SURGERY
(2023)
Article
Medicine, Research & Experimental
Saartje Uyttebroek, Lieven Dupont, Mark Jorissen, Laura Van Gerven
Summary: The study aimed to evaluate the burden of CF-related CRS in patients with CF. The results showed a high prevalence of CF-CRS in the cohort, with only a minority of patients having controlled disease. Patients receiving CFTR modulators had significantly lower CT scores and less bacterial colonization.
Article
Chemistry, Medicinal
Giuseppe Migliorisi, Mirella Collura, Francesca Ficili, Tiziana Pensabene, Dafne Bongiorno, Antonina Collura, Francesca Di Bernardo, Stefania Stefani
Summary: This study reports on a one-year case-control study involving 26 CF patients with at least one F508de1 mutation. The triple combination therapy targeting CFTR provided significant clinical benefits and improved patients' quality of life. Further investigations are needed to fully understand the microbiological respiratory implications and long-term advantages of elexacaftor-tezacaftor-ivacaftor treatment.
Review
Medicine, Research & Experimental
Mei Yin, Xiaoping Gao, Lingling Di, Pei Yang, Jing Liu, Xia Li, Xiaohui Yan, Wei Wang, Hua Cui
Summary: The study aimed to quantify the impact of endoscopic sinus surgery on pulmonary function in patients with cystic fibrosis and chronic rhinosinusitis. Analysis of 12 studies with 570 patients showed that the surgery did not improve the pulmonary function in these patients. Further prospective studies and meta-analyses on this topic are needed for future research.
Review
Microbiology
Christina S. Thornton, Michael G. Surette
Summary: Cystic fibrosis is the most common and lethal genetic disease among the Caucasian population, leading to chronic airway inflammation and declining pulmonary function. Studies have shown a diverse community of anaerobic bacteria in CF patients' airways, potentially impacting disease progression through synergistic interaction with principal pathogens. Despite the understanding of this complex bacterial milieu, the specific roles of anaerobes in disease progression remain unclear.
JOURNAL OF CLINICAL MICROBIOLOGY
(2021)
Article
Microbiology
Catherine R. Armbruster, Kelvin Li, Megan R. Kiedrowski, Anna C. Zemke, Jeffrey A. Melvin, John Moore, Samar Atteih, Adam C. Fitch, Matthew DuPont, Christopher D. Manko, Madison L. Weaver, Jordon R. Gaston, John F. Alcorn, Alison Morris, Barbara A. Methe, Stella E. Lee, Jennifer M. Bomberger
Summary: Chronic rhinosinusitis (CRS) is a common manifestation of upper respiratory disease in people with cystic fibrosis (CF). This study found that changes in the sinus microbial community are associated with increased respiratory disease and inflammation.
MICROBIOLOGY SPECTRUM
(2022)
Article
Biochemistry & Molecular Biology
Andrew L. Thurman, Xiaopeng Li, Raul Villacreses, Wenjie Yu, Huiyu Gong, Steven E. Mather, Guillermo S. Romano-Ibarra, David K. Meyerholz, David A. Stoltz, Michael J. Welsh, Ian M. Thornell, Joseph Zabner, Alejandro A. Pezzulo
Summary: Lack of CFTR affects the transcriptome, composition, and function of airway epithelia in people with advanced cystic fibrosis. The study shows that the effects of CFTR deficiency on the airway are mainly driven by infection and inflammation rather than cell-intrinsic abnormalities present at birth. The research highlights the distinct gene expression and function of different types of airway epithelial cells.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
(2022)
Article
Pharmacology & Pharmacy
Raphaelle Youf, Adeel Nasir, Mareike Mueller, Franck Thetiot, Tanguy Haute, Rosy Ghanem, Ulrich Jonas, Holger Schoenherr, Gilles Lemercier, Tristan Montier, Tony Le Gall
Summary: Antimicrobial photodynamic therapy (aPDT) is a treatment method that relies on various parameters. In this study, the application of Ruthenium(II) polypyridyl ([Ru(II)]) complexes in the airways of Cystic Fibrosis (CF) patients was investigated, and the influence of different parameters on their properties was studied. The results provide significant evidence for the clinical relevance of [Ru(II)]-based aPDT in CF lung airways.
Article
Microbiology
Efthymia Iliana Matthaiou, Wayland Chiu, Carol Conrad, Joe Hsu
Summary: Cystic fibrosis lung transplant recipients are at a higher risk of life-threatening invasive aspergillosis. Alkalization of macrophages lacking the CFTR gene is associated with this increased risk, and the presence of iron in the post-transplant microenvironment exacerbates this alkalization. The alkalization impairs the ability of macrophages to clear Aspergillus fumigatus conidia. pH modulation after transplantation may be a therapeutic strategy to reduce the risk of invasive aspergillosis.
Article
Infectious Diseases
Nikolaus Kneidinger, Matthias Hecker, Vasiliki Bessa, Ina Hettich, Alexandra Wald, Sabine Wege, Anna-Barbara Nolde, Maike Oldigs, Zulfiya Syunyaeva, Heinrike Wilkens, Jens Gottlieb
Summary: This study aims to assess the outcome and associated factors in lung transplant recipients during the Omicron era of COVID-19, and found that age and glomerular filtration rate < 30 ml/min/1.73m(2) were independent risk factors for severe or critical COVID-19.
Article
Pediatrics
Emilia da Silva Goncalves, Jose Dirceu Ribeiro, Fernando Augusto Lima Marson, Ciro Garcia Montes, Antonio Fernando Ribeiro, Jochen Georg Mainz, Adyleia Aparecida Dalbo Contrera Toro, Juliana Helena Zamariola, Daniela Souza Paiva Borgli, Elizete Aparecida Lomazi, Maria de Fatima Correa Pimenta Servidoni
Summary: The prevalence of GERD in children with CF was assessed using multichannel intraluminal impedanciometry associated with pH monitoring (MIIpH) and compared with respiratory symptoms. It was found that MIIpH detected 30% more GERD patients than pHmetry.
JORNAL DE PEDIATRIA
(2023)
Article
Multidisciplinary Sciences
Valentino Bezzerri, Valentina Gentili, Martina Api, Alessia Finotti, Chiara Papi, Anna Tamanini, Christian Boni, Elena Baldisseri, Debora Olioso, Martina Duca, Erika Tedesco, Sara Leo, Monica Borgatti, Sonia Volpi, Paolo Pinton, Giulio Cabrini, Roberto Gambari, Francesco Blasi, Giuseppe Lippi, Alessandro Rimessi, Roberta Rizzo, Marco Cipolli
Summary: As an inherited disorder characterized by severe pulmonary disease, cystic fibrosis could be considered a comorbidity for coronavirus disease 2019. However, current clinical evidence suggests that cystic fibrosis may actually protect against SARS-CoV-2 infection. The expression of SARS-CoV-2 receptors in primary airway epithelial cells is regulated by the CFTR channel, and dysfunctional CFTR channels in cystic fibrosis cells result in reduced viral entry and replication.
NATURE COMMUNICATIONS
(2023)
Article
Multidisciplinary Sciences
Jens Gottlieb, Bettina Fischer, Jonas Schupp, Heiko Golpon
Summary: This study investigated the effectiveness of CNI-free immunosuppression using mTOR inhibitors in lung transplant recipients. The results showed that the 1-year survival rate was 36% in patients with malignancy who were converted to CNI-free immunosuppression, while the 1-year survival rate was 100% in other patients. Additionally, patients who received this immunosuppression showed improvement in glomerular filtration rate.
Article
Medicine, General & Internal
Silvia Accordino, Ciro Canetta, Greta Bettini, Federica Corsico, Gabriele Ghigliazza, Laura Barbetta, Christian Folli, Valeria Savojardo, Francesco Blasi
Summary: The aim of this study was to compare the characteristics of COVID-19 patients admitted to a Medical Intermediate Care Unit for acute respiratory failure due to SARS-CoV-2 pneumonia throughout four pandemic waves. The results showed that COVID-19 patients have become progressively older and with more comorbidities, and non-survivors were older and more comorbid compared to patients transferred to the ICU. There was no statistical difference in in-hospital mortality, but the rate of ICU-transfers significantly decreased.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Respiratory System
Geert M. Verleden, Jens Gottlieb
EUROPEAN RESPIRATORY REVIEW
(2023)
Review
Infectious Diseases
Susanna Esposito, Francesco Blasi, Nigel Curtis, Sheldon Kaplan, Tiziana Lazzarotto, Marianna Meschiari, Cristina Mussini, Maddalena Peghin, Carlos Rodrigo, Antonio Vena, Nicola Principi, Matteo Bassetti
Summary: This paper discusses the main characteristics and clinical use of new licenced anti-staphylococcal drugs. In vitro studies have shown that some new anti-staphylococcal antibiotics have better antimicrobial activity and more favorable pharmacokinetic properties and safety compared to currently available drugs. However, further research is needed before the problem of S. aureus resistance to antibiotics can be completely solved.
Article
Biology
Giorgia Grassi, Elisa Cairoli, Lucrezia Maria Silvana Gentile, Iacopo Chiodini, Marta Zampogna, Alberto Ghielmetti, Letizia Corinna Morlacchi, Valeria Rossetti, Lorenzo Rosso, Ilaria Righi, Mario Nosotti, Maura Arosio, Francesco Blasi, Cristina Eller Vainicher
Summary: Based on our study, the skeletal outcomes in long-term survivors (>5 years) after lung transplantation were similar between patients with cystic fibrosis (CF) and those with non-cystic fibrosis (nCF), with a lower incidence of fractures compared to the first two years post-transplant. Bone mineral density and trabecular bone score remained stable after the second year post-transplant.
Article
Cardiac & Cardiovascular Systems
Jens Gottlieb, Fernando Torres, Tarik Haddad, Gundeep Dhillon, Daniel F. Dilling, Christiane Knoop, Reinaldo Rampolla, Rajat Walia, Vivek Ahya, Romain Kessler, Marie Budev, Claus Neurohr, Allan R. Glanville, Robert Jordan, Danielle Porter, Matt McKevitt, Polina German, Ying Guo, Jason W. Chien, Timothy R. Watkins, Martin R. Zamora
Summary: This study evaluated the efficacy of the RSV fusion inhibitor presatovir in RSV-infected lung transplant recipients. The results showed that presatovir did not significantly improve nasal RSV load, symptoms, or lung function in lung transplant recipients.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Editorial Material
Respiratory System
Mario Cazzola, Paola Rogliani, Francesco Blasi
Summary: The complexity of COPD calls for the identification of patient groups with similar characteristics and needs. Different clinical phenotypes and endotypes have been studied, but a new taxonomic classification based on causes has been proposed. The tractable traits approach is a simpler method, but lacks validation through large randomized controlled trials. Despite its application following the GOLD strategy, there are challenges in identifying and prioritizing treatable traits.
INTERNATIONAL JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE
(2023)
Article
Respiratory System
Jens Gottlieb, Thomas Fuehner, Patrick Zardo
Summary: This study investigated the management and outcome of obstructive airway complications (OACs) after lung transplantation. The results showed that bilateral OACs impose a high burden on patients and are associated with early and late graft loss. Patients with bilateral OACs had reduced graft function and impaired quality of life.
THERAPEUTIC ADVANCES IN RESPIRATORY DISEASE
(2023)
Review
Respiratory System
Francesco Amati, Francesco Bindo, Anna Stainer, Andrea Gramegna, Marco Mantero, Mattia Nigro, Linda Bussini, Michele Bartoletti, Francesco Blasi, Stefano Aliberti
Summary: There has been a significant increase in the use of broad-spectrum antibiotics as empirical therapy for patients with community-acquired pneumonia (CAP) in the past 15 years. This is driven by evidence of an increased incidence of drug-resistant pathogens (DRP) in CAP patients, including MRSA and Pseudomonas aeruginosa. However, recent studies have shown that the incidence of DRP in CAP varies greatly depending on the local ecology, healthcare systems, and countries where the studies were conducted. Some studies have questioned the effectiveness of broad-spectrum antibiotic coverage in improving outcomes in CAP, citing increased costs, longer hospital stays, drug adverse events, and antibiotic resistance as potential drawbacks.
ADVANCES IN RESPIRATORY MEDICINE
(2023)
Article
Respiratory System
Andrea Gramegna, Fabio Majo, Gianfranco Alicandro, Gloria Leonardi, Luca Cristiani, Francesco Amati, Martina Contarini, Stefano Aliberti, Alessandro Giovanni Fiocchi, Francesco Blasi
Summary: This study aims to identify potential determinants of heterogeneity in weight gain following 6-month ETI therapy. The baseline BMI and the presence of at least one CFTR residual function mutation were found to be the major determinants of weight gain heterogeneity. The results indicate that ETI is highly effective in improving weight gain in underweight CF patients.
RESPIRATORY RESEARCH
(2023)
Meeting Abstract
Cardiac & Cardiovascular Systems
L. Morlacchi, V. Rossetti, S. Uceda Renteria, I. Righi, F. Ceriotti, L. Rosso, F. Blasi
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Meeting Abstract
Cardiac & Cardiovascular Systems
V. Vaira, A. Storaci, S. Franzi, M. Pitasi, F. Gentile, V. Musso, L. Morlacchl, V. Rossetti, F. Blasi, M. Nosotti, S. Ferrero, A. Palleschi
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)