scholarly journals Risk factors for lung disease progression in children with cystic fibrosis

2018 ◽  
Vol 52 (4) ◽  
pp. 1801492
Author(s):  
Hafez Al Momani ◽  
Audrey Perry ◽  
Rhys Jones ◽  
Melissa McDonnell ◽  
Amaran Krishnan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Lung Disease ◽  
Disease Progression

scholarly journals Risk factors for lung disease progression in children with cystic fibrosis

2018 ◽  
Vol 51 (6) ◽  
pp. 1702509 ◽  
Author(s):  
Marieke van Horck ◽  
Kim van de Kant ◽  
Bjorn Winkens ◽  
Geertjan Wesseling ◽  
Vincent Gulmans ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Lung Disease ◽  
Disease Progression ◽  
Potential Risk ◽  
Allergic Bronchopulmonary Aspergillosis ◽  
Pulmonary Exacerbation ◽  
Exacerbation Rate ◽  
Potential Risk Factors ◽  
Pulmonary Exacerbations

To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009–2014).Lung disease progression was expressed as a decline in lung function (forced expiratory volume in 1 s (FEV1) % pred) and pulmonary exacerbation rate. Potential risk factors at baseline included sex, age, best FEV1 % pred, best forced vital capacity % pred, genotype, body mass index z-score, pancreatic insufficiency, medication use (proton pump inhibitors (PPIs), prophylactic antibiotics and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis and colonisation with Pseudomonas aeruginosa.The data of 545 children were analysed. PPI use was associated with both annual decline of FEV1 % pred (p=0.017) and future pulmonary exacerbation rate (p=0.006). Moreover, lower FEV1 % pred at baseline (p=0.007), prophylactic inhaled antibiotic use (p=0.006) and pulmonary exacerbations in the baseline year (p=0.002) were related to pulmonary exacerbations in subsequent years.In a cohort of Dutch children with CF followed for 5 years, we were able to identify several risk factors for future exacerbations. In particular, the association between PPI use and lung disease progression definitely requires further investigation.

Risk factors for lung disease progression in children with cystic fibrosis; a longitudinal cohort study

2017 ◽  
Author(s):  
Marieke van Horck ◽  
Kim D. G. van de Kant ◽  
Bjorn Winkens ◽  
Geertjan Wesseling ◽  
Vincent Gulmans ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Cohort Study ◽  
Lung Disease ◽  
Disease Progression ◽  
Longitudinal Cohort Study ◽  
Longitudinal Cohort

scholarly journals Risk Factors for the Progression of Cystic Fibrosis Lung Disease throughout Childhood

2014 ◽  
Vol 11 (1) ◽  
pp. 63-72 ◽  
Author(s):  
Don B. Sanders ◽  
Zhanhai Li ◽  
Anita Laxova ◽  
Michael J. Rock ◽  
Hara Levy ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Lung Disease ◽  
Cystic Fibrosis Lung ◽  
Cystic Fibrosis Lung Disease

scholarly journals Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis

2020 ◽  
Vol 24 (04) ◽  
pp. e434-e437
Author(s):  
Luciane Mazzini Steffen ◽  
Luise Sgarabotto Pezzin ◽  
Natassia Sulis ◽  
Nedio Steffen ◽  
Leonardo Araujo Pinto
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Disease Progression ◽  
Nasal Polyps ◽  
Nasal Swab ◽  
Statistical Significance ◽  
Upper Airway ◽  
Forced Expiratory Volume ◽  
Upper Respiratory Tract ◽  
Upper Airways

Abstract Introduction Cystic fibrosis (CF) is a genetic disease that limits the quality of life mainly due to respiratory symptoms. The relationship between findings of the upper airways and CF lung disease is not yet completely understood. Objective The aim of the present study is to describe the most frequent nasal findings and pathogens in patients with CF and investigate the association between the findings of the upper respiratory tract and markers of lung disease progression. Methods Retrospective study in patients with CF from the Pediatric Pulmonology Department who underwent otorhinolaryngological evaluation between 2015 and 2017. Nasal endoscopy and nasal swab collection were part of the evaluation. The severity markers used were: percentage of predicted forced expiratory volume in the first second (FEV1%), body mass index (BMI) and the Shwachman-Kulczycki (SK) clinical score. Results A total of 48 patients with CF were included. The mean of the predicted percentage of FEV1% was 83.36 ± 30.04. The average 14 and SK score 89.11 ± 10.50. The bacteriology of the nasal swab was positive in 27 (54.1%) patients. Staphylococcus aureus was positive in 18 patients, Pseudomonas aeruginosa in 5, Pseudomonas cepacea in 3 and Stenotrophomonas maltophila in 1 patient. Nasal polyps were found in nine participants. Nasal polyps were found in nine participants and were associated with lower SK score. Conclusion The pathogens found in the upper airway were, in order: S. aureus, P. aeruginosa, P. cepacea e S. maltophila. The presence of polyps in the nasal cavity showed statistical significance and appears to have association with the prognostic factor measured by the SK score.

scholarly journals Early-Life Pseudomonas aeruginosa Infection in Cystic Fibrosis and Lung Disease Progression

2017 ◽  
Vol 4 ◽  
pp. 2333794X1773846
Author(s):  
Argyri Petrocheilou ◽  
Maria Papagrigoriou-Theodoridou ◽  
Athanasios Michos ◽  
Stavros-Eleftherios Doudounakis ◽  
Ioanna Loukou ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Disease ◽  
Disease Progression ◽  
Early Life ◽  
Pseudomonas Aeruginosa Infection

scholarly journals Coinfection with Pseudomonas aeruginosa and Aspergillus fumigatus in cystic fibrosis

2020 ◽  
Vol 29 (158) ◽  
pp. 200011
Author(s):  
Karen Keown ◽  
Alastair Reid ◽  
John E. Moore ◽  
Clifford C. Taggart ◽  
Damian G. Downey
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Aspergillus Fumigatus ◽  
Lung Disease ◽  
Disease Progression ◽  
Expert Knowledge ◽  
Fungal Species ◽  
Interspecies Interactions ◽  
Cross Sectional ◽  
Infection And Inflammation

ObjectivesCystic fibrosis (CF) lung disease is characterised by mucus stasis, chronic infection and inflammation, causing progressive structural lung disease and eventual respiratory failure. CF airways are inhabited by an ecologically diverse polymicrobial environment with vast potential for interspecies interactions, which may be a contributing factor to disease progression. Pseudomonas aeruginosa and Aspergillus fumigatus are the most common bacterial and fungal species present in CF airways respectively and coinfection results in a worse disease phenotype.MethodsIn this review we examine existing expert knowledge of chronic co-infection with P. aeruginosa and A. fumigatus in CF patients. We summarise the mechanisms of interaction and evaluate the clinical and inflammatory impacts of this co-infection.ResultsP. aeruginosa inhibits A. fumigatus through multiple mechanisms: phenazine secretion, iron competition, quorum sensing and through diffusible small molecules. A. fumigatus reciprocates inhibition through gliotoxin release and phenotypic adaptations enabling evasion of P. aeruginosa inhibition. Volatile organic compounds secreted by P. aeruginosa stimulate A. fumigatus growth, while A. fumigatus stimulates P. aeruginosa production of cytotoxic elastase.ConclusionA complex bi-directional relationship exists between P. aeruginosa and A. fumigatus, exhibiting both mutually antagonistic and cooperative facets. Cross-sectional data indicate a worsened disease state in coinfected patients; however, robust longitudinal studies are required to derive causality and to determine whether interspecies interaction contributes to disease progression.

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