scholarly journals Respiratory Exacerbations in Indigenous Children From Two Countries With Non-Cystic Fibrosis Chronic Suppurative Lung Disease/Bronchiectasis

CHEST Journal ◽  
2014 ◽  
Vol 146 (3) ◽  
pp. 762-774 ◽  
Author(s):  
Gregory J. Redding ◽  
Rosalyn J. Singleton ◽  
Patricia C. Valery ◽  
Hayley Williams ◽  
Keith Grimwood ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Indigenous Children ◽  
Suppurative Lung Disease ◽  
Respiratory Exacerbations

Indigenous children from three countries with non-cystic fibrosis chronic suppurative lung disease/bronchiectasis

2013 ◽  
Vol 49 (2) ◽  
pp. 189-200 ◽  
Author(s):  
Rosalyn J. Singleton ◽  
Patricia C. Valery ◽  
Peter Morris ◽  
Catherine A. Byrnes ◽  
Keith Grimwood ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Indigenous Children ◽  
Suppurative Lung Disease

scholarly journals Management of bronchiectasis and chronic suppurative lung disease in Indigenous children and adults from rural and remote Australian communities

2008 ◽  
Vol 189 (7) ◽  
pp. 386-393 ◽  
Author(s):  
Anne B Chang ◽  
Keith Grimwood ◽  
Graeme Maguire ◽  
Paul T King ◽  
Peter S Morris ◽  
...  
Keyword(s):  
Lung Disease ◽  
Rural And Remote ◽  
Indigenous Children ◽  
Suppurative Lung Disease

scholarly journals Chronic suppurative lung disease in sisters mimicking cystic fibrosis

1974 ◽  
Vol 49 (7) ◽  
pp. 520-524 ◽  
Author(s):  
J. J. Cogswell ◽  
R. A. Risdon ◽  
B. Taylor
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Suppurative Lung Disease

scholarly journals Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations

Cells ◽  
2020 ◽  
Vol 9 (9) ◽  
pp. 1952 ◽  
Author(s):  
Garrett Pehote ◽  
Neeraj Vij
Keyword(s):  
Cystic Fibrosis ◽  
Immune Response ◽  
Lung Disease ◽  
Lung Diseases ◽  
Therapeutic Potential ◽  
Ubiquitin Proteasome System ◽  
Chronic Obstructive ◽  
Cellular Homeostasis ◽  
Cellular Debris ◽  
Respiratory Exacerbations

The preservation of cellular homeostasis requires the synthesis of new proteins (proteostasis) and organelles, and the effective removal of misfolded or impaired proteins and cellular debris. This cellular homeostasis involves two key proteostasis mechanisms, the ubiquitin proteasome system and the autophagy–lysosome pathway. These catabolic pathways have been known to be involved in respiratory exacerbations and the pathogenesis of various lung diseases, such as chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), idiopathic pulmonary fibrosis (IPF), acute lung injury (ALI), acute respiratory distress syndrome (ARDS), and coronavirus disease-2019 (COVID-19). Briefly, proteostasis and autophagy processes are known to decline over time with age, cigarette or biomass smoke exposure, and/or influenced by underlying genetic factors, resulting in the accumulation of misfolded proteins and cellular debris, elevating apoptosis and cellular senescence, and initiating the pathogenesis of acute or chronic lung disease. Moreover, autophagic dysfunction results in an impaired microbial clearance, post-bacterial and/or viral infection(s) which contribute to the initiation of acute and recurrent respiratory exacerbations as well as the progression of chronic obstructive and restrictive lung diseases. In addition, the autophagic dysfunction-mediated cystic fibrosis transmembrane conductance regulator (CFTR) immune response impairment further exacerbates the lung disease. Recent studies demonstrate the therapeutic potential of novel autophagy augmentation strategies, in alleviating the pathogenesis of chronic obstructive or restrictive lung diseases and exacerbations such as those commonly seen in COPD, CF, ALI/ARDS and COVID-19.

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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