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
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.
The objective of this study was to identify risk factors for development of Aspergillus respiratory isolation in CF.
Allergy to thermotolerant filamentous fungi, particularly Aspergillus fumigatus, is closely associated with fixed airflow obstruction, bronchiectasis, and other radiologically defined abnormalities, such as mucus plugging.1 However, not all asthma patients who are immunoglobulin E (IgE) sensitized to A fumigatus develop these complications. To identify markers of poor outcomes in fungal allergy with asthma (and cystic fibrosis), the term allergic bronchopulmonary aspergillosis (ABPA) was coined.
Case 1: A 20-years old woman with cystic fibrosis (CF), deltaF508 homozygote, predicted 35% FEV1 was treated with inhalative antibiotics due to colonisation with Pseudomonas aeruginosa (PA). An allergic bronchopulmonary aspergillosis has previously been treated, with no evidence of reactivation. After severe exacerbations and a drop of FEV1 to 17%, she stabilized with steroids and IV-antibiotics. Despite a reduction of Pseudomonas, a low FEV1 persisted. Yeast grew in repeated sputum cultures, but could not be identified by standard methods. ITS-sequencing revealed the presence of Blastobotrys raffinosifermentans. Adequate ...
Conclusions: This is the first study that investigates the association of fungal finding in sinuses of CF patients, and its correlation with clinical and allergological findings. A Huge amount of fungal spores in the air is the threat for the development of FRS and early detection of fungal growth in the sinuses of these patients could decrease complications of CF and development of comorbidities.
Aspergillus bronchitis (AB) in the absence of allergic bronchopulmonary aspergillosis (ABPA) has recently been described as new entity in cystic fibrosis (CF) patients.We identified 16 CF individuals (age range 8 – 39 yrs, 69% females) with AB in 2016 (prevalence 2.3%). 8 subjects had no sensitization to Aspergillus fumigatus (AF), whereas 8 subjects showed mild sensitization (specific IgE
Conclusion: Our findings represent the first characterisation of the Asian pulmonary microbiome in bronchiectasis, including analysis of the bacterial and fungal microbiota. The direct and mechanistic implications of these findings on clinical disease, in comparison to Caucasian cohorts, require further investigation.Funding: This research is supported by the Singapore Ministry of Health’s National Medical Research Council under its Transition Award (NMRC/TA/0048/2016) (S.H.C).