The 2nd Manchester Cystic Fibrosis Conference: Facing the Challenges of Today
The 2nd Manchester Cystic Fibrosis Conference: Facing the Challenges of TodayDate: Tuesday, February 6, 2018Year: 2018Location: Stoller Hall - Chetham's School of Music, Hunts Bank, Manchester, M31DAContent-type: current_conferences
H élène Guegan, Sylviane Chevrier, Chantal Belleguic, Eric Deneuville, Florence Robert-Gangneux, Jean-Pierre Gangneux
Aspergillus fumigatus is frequently encountered in sputum samples of patients with cystic fibrosis (CF), which traditionally has been interpreted as saprophytic airway colonization. However, this mere bystander r...
CONCLUSIONS: There is lack of evidence for the efficacy and safety of anti-IgE (omalizumab) therapy in people with cystic fibrosis and allergic bronchopulmonary aspergillosis. There is a need for large prospective randomized controlled studies of anti-IgE therapy in people with cystic fibrosis and allergic bronchopulmonary aspergillosis with both clinical and laboratory outcome measures such as steroid requirement, allergic bronchopulmonary aspergillosis exacerbations and lung function. PMID: 29551015 [PubMed - as supplied by publisher]
Necrotizing bronchial dehiscence following lung transplantation is a rare and deadly complication with no strict management consensus. Here, we report the case of a patient who underwent bilateral sequential lung transplantation for cystic fibrosis through a clamshell incision and developed, on postoperative day 17, complete bilateral anastomotic dehiscence. The latter was attributed to a Pseudomonas aeruginosa and Aspergillus necrotizing infection. The patient was managed surgically using a bilateral posterolateral thoracotomy approach with resection of the bronchial margins, end-to-end re-anastomosis and intercostal muscle coverage.
ConclusionsBronchiectasis is not one disease. It has a significant impact on healthcare utilization and survival. Differences between populations are likely to relate to differing aetiologies and understanding the drivers of bronchiectasis in disadvantaged populations will be key.
Abstract Scedosporium species are opportunistic pathogens causing a great variety of infections in both immunocompetent and immunocompromised individuals. The Scedosporium genus ranks the second among the filamentous fungi colonizing the airways of patients with cystic fibrosis (CF), after Aspergillus fumigatus, and most species are capable to chronically colonize the respiratory tract of these patients. Nevertheless, few data are available regarding evasion of the inhaled conidia to the host immune response. Upon microbial infection, macrophages and neutrophils release reactive oxygen species (ROS). To colonize t...
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Mutations in this chloride channel lead to mucus accumulation, subsequent recurrent pulmonary infections, and inflammation, which, in turn, cause chronic lung disease and respiratory failure. Recently, rates of nontuberculous mycobacterial (NTM) infections in CF patients have been increasing. Of particular relevance is infection with Mycobacterium abscessus, which causes a serious, life-threatening disease and constitutes one of the most antibiotic-resistant NTM species. Interestingly, an in...
Thursday, February 15, 2018 - 10:48Slide presentation
Airflow obstruction infection and inflammation are key components of respiratory morbidity in patients with cystic fibrosis (CF). Chronic respiratory fungal infections, most commonly with moulds of the genera Aspergillus and Exophiala, are common in this patient cohort. An increased susceptibility to fungal infections may be observed in individuals with defects in IL-17RA signalling pathways, a situation characterised by excessive mucus production, increased Th2 cytokine, IL-17F, IL-33 and reduced IFN- γ secretion in patients without CF .
Pulmonary and systemic antifungal immunity influences quality of life and survival of people with cystic fibrosis. Aspergillus fumigatus (Af) induces specific IgG and IgE. Mast cells respond to IgE, IgG and direct interactions with Af. Mast cells are the source of the protease tryptase. We aimed at evaluating serum baseline tryptase as a potential biomarker of the Af-host interaction in cystic fibrosis patients. Serum baseline tryptase, IgE and IgG directed to Af extract and Af molecular allergens were measured in 76 cystic fibrosis patients.