scholarly journals Respiratory infection rates differ between geographically distant paediatric cystic fibrosis cohorts

2016 ◽  
Vol 2 (3) ◽  
pp. 00014-2016 ◽  
Author(s):  
Kathryn A. Ramsey ◽  
Emily Hart ◽  
Lidija Turkovic ◽  
Marc Padros-Goossens ◽  
Stephen M. Stick ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Respiratory Infections ◽  
Age Groups ◽  
Similar Rate ◽  
Lower Airway ◽  
Annual Review ◽  
Respiratory Pathogens ◽  
Geographical Differences ◽  
Old Patients

Respiratory infections are a major cause of pulmonary decline in children with cystic fibrosis (CF). We compared the prevalence of infection in early life at geographically distant CF treatment centres participating in the same surveillance programme in Australia.Lower airway microbiology, inflammation and structural lung disease at annual review were evaluated for 260 children 0–8 years old with CF at 1032 visits to CF treatment centres in Melbourne or Perth.Melbourne patients were more likely to be culture-positive for common respiratory pathogens at all age groups (odds ratio (OR) 1.85, 95% CI 1.33–2.58). Subjects <2 years old in Melbourne were also more likely to have neutrophil elastase present (OR 3.11, 95% CI 1.62–5.95). Bronchiectasis (OR 2.02, 95% CI 1.21–3.38) and air trapping (OR 2.53, 95% CI 1.42–4.51) in subjects 2–5 years old was more common in Melbourne subjects. The severity of structural lung disease was also worse in Melbourne patients >5 years old. Patients at both centres had a similar rate of hospitalisations and prescribed antibiotics.No procedural differences were identified that could explain the disparity between pathogen prevalence. Geographical differences in early acquisition of infection may contribute to variability in outcomes between CF centres.

scholarly journals The lower airway microbiota in early cystic fibrosis lung disease: a longitudinal analysis

Thorax ◽  
2017 ◽  
Vol 72 (12) ◽  
pp. 1104-1112 ◽  
Author(s):  
Katherine B Frayman ◽  
David S Armstrong ◽  
Rosemary Carzino ◽  
Thomas W Ferkol ◽  
Keith Grimwood ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Longitudinal Analysis ◽  
Cystic Fibrosis Lung ◽  
Lower Airway ◽  
Cystic Fibrosis Lung Disease

scholarly journals Genomic Diversity and Antimicrobial Resistance of Haemophilus Colonizing the Airways of Young Children with Cystic Fibrosis

mSystems ◽  
2021 ◽  
Vol 6 (4) ◽  
Author(s):  
Stephen C. Watts ◽  
Louise M. Judd ◽  
Rosemary Carzino ◽  
Sarath Ranganathan ◽  
Kathryn E. Holt
Keyword(s):  
Cystic Fibrosis ◽  
Antimicrobial Resistance ◽  
Young Children ◽  
Lung Disease ◽  
Respiratory Infections ◽  
Genomic Diversity ◽  
Disease Development ◽  
Acute Respiratory Infections ◽  
Early Disease ◽  
Advanced Stages

Cystic fibrosis (CF) lung disease begins during infancy, and acute respiratory infections increase the risk of early disease development and progression. Microbes involved in advanced stages of CF are well characterized, but less is known about early respiratory colonizers.

scholarly journals Defective Ion Channel in Cystic Fibrosis: Current Development in Treatment of Cystic Fibrosis

2020 ◽  
pp. 28-34
Author(s):  
Ngoga Godfrey ◽  
M. M. Ganyam ◽  
G.O. Ibiang ◽  
C. A. Difa ◽  
Nelson Christian
Keyword(s):  
Cystic Fibrosis ◽  
Ion Transport ◽  
Ion Channel ◽  
Lung Disease ◽  
Defense Mechanisms ◽  
Respiratory Infections ◽  
The Body ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Airway Defense

Cystic fibrosis is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body. Cystic fibrosis transmembrane conductance regulator (CFTR) is involved in the production of mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with cystic fibrosis, a defective gene in CFTR causes the secretions to become sticky and thick. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passage ways, especially in the lungs and pancreas. This mucus leads to the formation of bacterial microenvironments known as biofilms (a niche that harbors bacteria; Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa ) that are difficult for immune cells and antibiotics to penetrate. Viscous secretions and persistent respiratory infections repeatedly damage the lung by gradually remodeling the airways, which makes infection even more difficult to eradicate. CFTR, a Cl– selective ion channel, is a prototypic member of the ATP-binding cassette transporter super family that is expressed in several organs. Understanding how these complexes regulate the intracellular trafficking and activity of CFTR provides a unique insight into the aetiology of cystic fibrosis and other diseases associated to it. Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense mechanisms. The link between abnormal ion transport, disease initiation and progression is not fully understood, but airway mucus dehydration seems paramount in the initiation of CF lung disease. New therapies are currently in development that target the ion transport defects in CF with the intention of rehydrating airway surfaces.

scholarly journals Untargeted Metagenomic Investigation of the Airway Microbiome of Cystic Fibrosis Patients with Moderate-Severe Lung Disease

2020 ◽  
Vol 8 (7) ◽  
pp. 1003 ◽  
Author(s):  
Giovanni Bacci ◽  
Giovanni Taccetti ◽  
Daniela Dolce ◽  
Federica Armanini ◽  
Nicola Segata ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Temporal Dynamics ◽  
Clinical Status ◽  
Antibiotic Resistance Genes ◽  
Lower Airway ◽  
Metagenomic Sequencing ◽  
Taxonomic Profiling ◽  
Shotgun Metagenomic Sequencing ◽  
Airway Microbiome

Although the cystic fibrosis (CF) lung microbiota has been characterized in several studies, little is still known about the temporal changes occurring at the whole microbiome level using untargeted metagenomic analysis. The aim of this study was to investigate the taxonomic and functional temporal dynamics of the lower airway microbiome in a cohort of CF patients. Multiple sputum samples were collected over 15 months from 22 patients with advanced lung disease regularly attending three Italian CF Centers, given a total of 79 samples. DNA extracted from samples was subjected to shotgun metagenomic sequencing allowing both strain-level taxonomic profiling and assessment of the functional metagenomic repertoire. High inter-patient taxonomic heterogeneity was found with short-term compositional changes across clinical status. Each patient exhibited distinct sputum microbial communities at the taxonomic level, and strain-specific colonization of both traditional and atypical CF pathogens. A large core set of genes, including antibiotic resistance genes, were shared across patients despite observed differences in clinical status, and consistently detected in the lung microbiome of all subjects independently from known antibiotic exposure. In conclusion, an overall stability in the microbiome-associated genes was found despite taxonomic fluctuations of the communities.

scholarly journals Fungal Infection and Inflammation in Cystic Fibrosis

Pathogens ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 618
Author(s):  
T. Spencer Poore ◽  
Gina Hong ◽  
Edith T. Zemanick
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Clinical Outcomes ◽  
Fungal Infections ◽  
Allergic Bronchopulmonary Aspergillosis ◽  
Lower Airway ◽  
Future Research ◽  
Isolation And Identification ◽  
Helper Cell Type

Fungi are frequently recovered from lower airway samples from people with cystic fibrosis (CF), yet the role of fungi in the progression of lung disease is debated. Recent studies suggest worsening clinical outcomes associated with airway fungal detection, although most studies to date are retrospective or observational. The presence of fungi can elicit a T helper cell type 2 (Th-2) mediated inflammatory reaction known as allergic bronchopulmonary aspergillosis (ABPA), particularly in those with a genetic atopic predisposition. In this review, we discuss the epidemiology of fungal infections in people with CF, risk factors associated with development of fungal infections, and microbiologic approaches for isolation and identification of fungi. We review the spectrum of fungal disease presentations, clinical outcomes after isolation of fungi from airway samples, and the importance of considering airway co-infections. Finally, we discuss the association between fungi and airway inflammation highlighting gaps in knowledge and future research questions that may further elucidate the role of fungus in lung disease progression.

scholarly journals Respiratory Syncytial Virus: New Challenges for Molecular Epidemiology Surveillance and Vaccination Strategy in Patients with ILI/SARI

Vaccines ◽  
2021 ◽  
Vol 9 (11) ◽  
pp. 1334
Author(s):  
Fabio Tramuto ◽  
Carmelo Massimo Maida ◽  
Daniela Di Naro ◽  
Giulia Randazzo ◽  
Francesco Vitale ◽  
...  
Keyword(s):  
Respiratory Syncytial Virus ◽  
Respiratory Infections ◽  
Age Groups ◽  
Hospital Setting ◽  
Community Setting ◽  
Vaccination Strategy ◽  
Influenza Surveillance ◽  
Valuable Insight ◽  
Respiratory Pathogens ◽  
Syncytial Virus

Several respiratory pathogens are responsible for influenza-like illness (ILI) and severe respiratory infections (SARI), among which human respiratory syncytial virus (hRSV) represents one of the most common aetiologies. We analysed the hRSV prevalence among subjects with ILI or SARI during the five influenza seasons before the emergence of SARS-CoV-2 epidemic in Sicily (Italy). Respiratory specimens from ILI outpatients and SARI inpatients were collected in the framework of the Italian Network for the Influenza Surveillance and molecularly tested for hRSV-A and hRSV-B. Overall, 8.1% of patients resulted positive for hRSV. Prevalence peaked in the age-groups <5 years old (range: 17.6–19.1%) and ≥50 years old (range: 4.8–5.1%). While the two subgroups co-circulated throughout the study period, hRSV-B was slightly predominant over hRSV-A, except for the season 2019–2020 when hRSV-A strongly prevailed (82.9%). In the community setting, the distribution of hRSV subgroups was balanced (47.8% vs. 49.7% for hRSV-A and hRSV-B, respectively), while most infections identified in the hospital setting were caused by hRSV-B (69.5%); also, this latter one was more represented among hRSV cases with underlying diseases, as well as among those who developed a respiratory complication. The molecular surveillance of hRSV infections may provide a valuable insight into the epidemiological features of ILI/SARI. Our findings add new evidence to the existing knowledge on viral aetiology of ILI and SARI in support of public health strategies and may help to define high-risk categories that could benefit from currently available and future vaccines.

scholarly journals Allergic Bronchopulmonary Aspergillosis in Children with Cystic Fibrosis: An Update on the Newest Diagnostic Tools and Therapeutic Approaches

Pathogens ◽  
2020 ◽  
Vol 9 (9) ◽  
pp. 716
Author(s):  
Claudia Lattanzi ◽  
Giulia Messina ◽  
Valentina Fainardi ◽  
Maria Candida Tripodi ◽  
Giovanna Pisi ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Diagnostic Criteria ◽  
Respiratory Infections ◽  
Fungal Infections ◽  
Early Recognition ◽  
Allergic Bronchopulmonary Aspergillosis ◽  
Clinical Manifestations ◽  
Diagnostic Tools ◽  
The Impact

Cystic fibrosis (CF), the most common autosomal-recessive genetic disease in the Caucasian population, is characterized by frequent respiratory infections and progressive lung disease. Fungal species are commonly found in patients with CF, and among them, Aspergillus fumigatus is the most frequently isolated. While bacteria, particularly Pseudomonas aeruginosa, have a well-established negative effect on CF lung disease, the impact of fungal infections remains unclear. In patients with CF, inhalation of Aspergillus conidia can cause allergic bronchopulmonary aspergillosis (ABPA), a Th2-mediated lung disease that can contribute to disease progression. Clinical features, diagnostic criteria and treatment of ABPA are still a matter of debate. Given the consequences of a late ABPA diagnosis or the risk of ABPA overdiagnosis, it is imperative that the diagnostic criteria guidelines are reviewed and standardized. Along with traditional criteria, radiological features are emerging as tools for further classification as well as novel immunological tests. Corticosteroids, itraconazole and voriconazole continue to be the bedrock of ABPA therapy, but other molecules, such as posaconazole, vitamin D, recombinant INF-γ and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators, have been showing positive results. However, few studies have been conducted recruiting CF patients, and more research is needed to improve the prevention and the classification of clinical manifestations as well as to personalize treatment. Early recognition and early treatment of fungal infections may be fundamental to prevent progression of CF disease. The aim of this narrative review is to give an update on ABPA in children with CF.

scholarly journals Novel Neutrophil-Derived Proteins in Bronchoalveolar Lavage Fluid Indicate an Exaggerated Inflammatory Response in Pediatric Cystic Fibrosis Patients

2007 ◽  
Vol 53 (10) ◽  
pp. 1782-1791 ◽  
Author(s):  
Brendan J McMorran ◽  
Severine A Ouvry Patat ◽  
John B Carlin ◽  
Keith Grimwood ◽  
Alun Jones ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Inflammatory Response ◽  
Bronchoalveolar Lavage ◽  
Lung Disease ◽  
Airway Inflammation ◽  
Bronchoalveolar Lavage Fluid ◽  
Lavage Fluid ◽  
Respiratory Pathogens ◽  
Tissue Destruction ◽  
Tof Ms

Abstract Background: Airway inflammation in cystic fibrosis (CF) is exaggerated and characterized by neutrophil-mediated tissue destruction, but its genesis and mechanisms remain poorly understood. To further define the pulmonary inflammatory response, we conducted a proteome-based screen of bronchoalveolar lavage fluid (BALF) collected from young children with and without CF experiencing endobronchial infection. Methods: We collected BALF samples from 45 children younger than 5 years and grouped them according to the presence of respiratory pathogens: ≥1 × 105 colony-forming units (CFU)/mL BALF (18 and 12 samples with and without CF, respectively) and &lt;1 × 105 CFU/mL (23 and 15 samples). BALF proteins were analyzed with SELDI-TOF mass spectrometry (MS) and H4 ProteinChips®. Proteins were identified and characterized using trypsin digestion, tandem MS, Fourier transform ion cyclotron resonance MS, immunoblotting, and ELISA. Results: The SELDI-TOF MS BALF profiles contained 53 unique, reliably detected proteins. Peak intensities of 24 proteins differed significantly between the CF and non-CF samples. They included the neutrophil proteins, α-defensin 1 and 2, S100A8, S100A9, and S100A12, as well as novel forms of S100A8 and S100A12 with equivalent C-terminal deletions. Peak intensities of these neutrophil proteins and immunoreactive concentrations of selected examples were significantly higher in CF than non-CF samples. Conclusions: Small neutrophil-derived BALF proteins, including novel C-terminal truncated forms of S100A proteins, are easily detected with SELDI-TOF MS. Concentrations of these molecules are abnormally high in early CF lung disease. The data provide new insights into CF lung disease and identify novel proteins strongly associated with CF airway inflammation.

scholarly journals The clinical significance of oropharyngeal cultures in young children with cystic fibrosis

2018 ◽  
Vol 51 (5) ◽  
pp. 1800238 ◽  
Author(s):  
Oded Breuer ◽  
Daan Caudri ◽  
Lauren Akesson ◽  
Sarath Ranganathan ◽  
Stephen M. Stick ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Cystic Fibrosis ◽  
Lung Disease ◽  
Neutrophil Elastase ◽  
Lung Inflammation ◽  
Interleukin 8 ◽  
Lower Airway ◽  
Increased Risk ◽  
Post Test ◽  
Respiratory Exacerbations

In children with cystic fibrosis (CF) the associations between oropharyngeal swabs (OPSs) for detection of Pseudomonas and lung disease have not been evaluated.OPS and bronchoalveolar lavage (BAL) samples were obtained annually in children with CF from 2005 to 2017. OPS test characteristics were calculated using BAL as “gold standard”. Results were related to lung inflammation (BAL neutrophil elastase and interleukin-8), structural lung disease (chest computed tomography PRAGMA-CF (Perth–Rotterdam Annotated Grid Morphometric Analysis for CF) scores), respiratory exacerbations and future detection of Pseudomonas on BAL.From 181 patients, 690 paired OPS–BAL cultures were obtained. Prevalence of Pseudomonas in BAL was 7.4%. OPS sensitivity was 23.0% and specificity was 91.4%, reducing the post-test probability for a positive BAL following a negative OPS to 6.3%. Pseudomonas on OPS was not associated with lung inflammation or respiratory exacerbations, but was weakly associated with current PRAGMA-CF %Disease score (p=0.043). Pseudomonas on BAL was associated with positive neutrophil elastase (OR 4.17, 95% CI 2.04–8.53; p<0.001), increased interleukin-8 (p<0.001), increased all baseline PRAGMA computed tomography scores (p<0.001), progression of PRAGMA computed tomography scores (p<0.05) and increased risk of respiratory exacerbations (incidence rate ratio 2.11, 95% CI 1.15–3.87; p=0.017).In children with CF OPSs only marginally change the probability of detecting lower airway Pseudomonas and are not associated with lung disease indices nor exacerbations risk.

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