The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis

1995 ◽  
Vol 20 (2) ◽  
pp. 63-70 ◽  
Author(s):  
K. Balough ◽  
M. McCubbin ◽  
M. Weinberger ◽  
W. Smits ◽  
R. Ahrens ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Early Stages ◽  
Infection And Inflammation ◽  
The Relationship

scholarly journals NOVOSTI V OBRAVNAVI PLJUČNE BOLEZNI PRI OTROCIH IN MLADOSTNIKIH S CISTIČNO FIBROZO

2015 ◽  
Vol 84 (2) ◽  
Author(s):  
Marina Praprotnik ◽  
Ana Kotnik Pirš ◽  
Barbara Salobir ◽  
Majda Oštir ◽  
Matjaž Turel ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
Lung Disease ◽  
Respiratory Insufficiency ◽  
Clinical Skills ◽  
Holistic Approach ◽  
Patient Registries ◽  
Recent Developments ◽  
Chronic Respiratory Insufficiency ◽  
Infection And Inflammation

Cystic fibrosis (CF) is a multi-organ disease,  affecting mostly lungs and gastrointestinal tract. Data from patient registries show that the survival of patients with CF has progressively improved over the past several decades, as a result of advances in antibiotic treatment, supplementation of pancreatic enzymes, better nutrition and a holistic approach to treatment in CF centres.The purpose of this review is to survey recent developments in the treatment of lung disease  in children and adolescents with CF.We describe newborn screening for CF.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue.Lung disease is the most common cause of morbidity and mortality in CF patients. Emerging new therapies are targeted at all points in the pathogenesis of lung disease, from drugs that treat infection and inflammation in the airways to gene transfer studies  and to drugs that augment airway surface liquid height. A number of antibacterial agents formulated for inhalation are at various stages of study and there are several anti-inflammatory candidate drugs in  clinical trials.  The most important development  in the recent years is  modulation of the abnormal protein that causes CF, the cystic fibrosis transmembrane regulator (CFTR), where drugs are targeted at specific defects in the transcription, processing or functioning.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue. The role of the CF nurse, who has responsibilities in educating and teaching clinical skills to patients and families, is described.

scholarly journals The chemokine CCL18 characterises Pseudomonas infections in cystic fibrosis lung disease

2014 ◽  
Vol 44 (6) ◽  
pp. 1608-1615 ◽  
Author(s):  
Andreas Hector ◽  
Carolin Kröner ◽  
Melanie Carevic ◽  
Martina Bakele ◽  
Nikolaus Rieber ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Gene Expression Analysis ◽  
Ex Vivo ◽  
Healthy Controls ◽  
Protein Levels ◽  
Cystic Fibrosis Lung Disease ◽  
Potential Biomarker ◽  
Infection And Inflammation ◽  
Airway Cells

Cystic fibrosis (CF) lung disease is characterised by chronic Pseudomonas aeruginosa infection and leukocyte infiltration. Chemokines recruit leukocytes to sites of infection. Gene expression analysis identified the chemokine CCL18 as upregulated in CF leukocytes. We hypothesised that CCL18 characterises infection and inflammation in patients with CF lung disease.Therefore, we quantified CCL18 protein levels in the serum and airway fluids of CF patients and healthy controls, and studied CCL18 protein production by airway cells ex vivo.These studies demonstrated that CCL18 levels were increased in the serum and airway fluids from CF patients compared with healthy controls. Within CF patients, CCL18 levels were increased in P. aeruginosa-infected CF patients. CCL18 levels in the airways, but not in serum, correlated with severity of pulmonary obstruction in CF. Airway cells isolated from P. aeruginosa-infected CF patients produced significantly higher amounts of CCL18 protein compared with airway cells from CF patients without P. aeruginosa infection or healthy controls.Collectively, these studies show that CCL18 levels characterise chronic P. aeruginosa infection and pulmonary obstruction in patients with CF. CCL18 may, thus, serve as a potential biomarker and therapeutic target in CF lung disease.

Neutrophils in cystic fibrosis

2016 ◽  
Vol 397 (6) ◽  
pp. 485-496 ◽  
Author(s):  
Julie Laval ◽  
Anjali Ralhan ◽  
Dominik Hartl
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Structural Damage ◽  
Fungal Pathogens ◽  
Therapeutic Potential ◽  
Inflammatory Cells ◽  
Current Evidence ◽  
Infection And Inflammation ◽  
Shed Light

Abstract Cystic fibrosis (CF) lung disease is characterized by chronic infection and inflammation. Among inflammatory cells, neutrophils represent the major cell population accumulating in the airways of CF patients. While neutrophils provide the first defensive cellular shield against bacterial and fungal pathogens, in chronic disease conditions such as CF these short-lived immune cells release their toxic granule contents that cause tissue remodeling and irreversible structural damage to the host. A variety of human and murine studies have analyzed neutrophils and their products in the context of CF, yet their precise functional role and therapeutic potential remain controversial and incompletely understood. Here, we summarize the current evidence in this field to shed light on the complex and multi-faceted role of neutrophils in CF lung disease.

scholarly journals ASTHMA IN CYSTIC FIBROSIS

2015 ◽  
Vol 64 (2) ◽  
pp. 118-121
Author(s):  
Marcela Daniela Ionescu ◽  
◽  
Ioana-Alina Anca ◽  
Mihaela Balgradean ◽  
◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Airway Obstruction ◽  
Autosomal Recessive ◽  
Pulmonary Infection ◽  
Autosomal Recessive Disease ◽  
Personal History ◽  
Common Symptom ◽  
Infection And Inflammation ◽  
History Of

Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians. Lung disease is characterized by impaired mucocilliary clearance with airway obstruction and chronic pulmonary infection and inflammation. Wheeze is a common symptom in CF, but in some cases the wheeze is due to the presence of concomitant asthma. There is no consensus on how to define CF asthma, but the diagnosis is predominantly based on the patient’s strong family and personal history of atopy.

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.

scholarly journals Elastase activity on sputum neutrophils correlates with severity of lung disease in cystic fibrosis

2018 ◽  
Vol 51 (3) ◽  
pp. 1701910 ◽  
Author(s):  
A. Susanne Dittrich ◽  
Iris Kühbandner ◽  
Stefanie Gehrig ◽  
Verena Rickert-Zacharias ◽  
Matthew Twigg ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Resonance Energy Transfer ◽  
Resonance Energy ◽  
Forced Expiratory Volume ◽  
Healthy Controls ◽  
Elastase Activity ◽  
Residual Capacity ◽  
The Relationship

Neutrophil elastase (NE) is a key risk factor for severity of cystic fibrosis (CF) lung disease. Recent studies identified increased NE activity on the surface of airway neutrophils from CF-like mice and patients with CF. However, the role of surface-bound NE in CF lung disease remains unknown. We determined the relationship between surface-bound NE activity and severity of lung disease in CF.Surface-bound NE activity was measured on sputum neutrophils from 35 CF patients and eight healthy controls using novel lipidated Förster resonance energy transfer reporters and correlated with free NE activity, neutrophil counts, interleukin-8, myeloperoxidase and antiproteases in sputum supernatant, and with lung function parameters.Surface-bound NE activity was increased in CF compared to healthy controls (p<0.01) and correlated with free NE activity (p<0.05) and other inflammation markers (p<0.001). Surface-bound and free NE activity correlated with forced expiratory volume in 1 s % predicted (p<0.01 and p<0.05), but only surface-bound NE activity correlated with plethysmographic functional residual capacity % pred (p<0.01) in patients with CF.We demonstrate that surface-bound NE activity on airway neutrophils correlates with severity of lung disease in patients with CF. Our results suggest that surface-bound NE activity may play an important role in the pathogenesis and serve as novel biomarker in CF lung disease.

scholarly journals Cell based therapy aides in infection and inflammation resolution in the murine model of cystic fibrosis lung disease

2013 ◽  
Vol 03 (02) ◽  
pp. 139-153 ◽  
Author(s):  
Tracey L. Bonfield ◽  
Donald Lennon ◽  
Santosh K. Ghosh ◽  
Amy M. DiMarino ◽  
Aaron Weinberg ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Murine Model ◽  
Cystic Fibrosis Lung ◽  
Cell Based Therapy ◽  
Cystic Fibrosis Lung Disease ◽  
Infection And Inflammation ◽  
Inflammation Resolution

scholarly journals Transforming Growth Factorβ1 Genotypes in Relation to TGFβ1, Interleukin-8, and Tumor Necrosis Factor Alpha in Induced Sputum and Blood in Cystic Fibrosis

2013 ◽  
Vol 2013 ◽  
pp. 1-8 ◽  
Author(s):  
O. Eickmeier ◽  
L. v. d. Boom ◽  
F. Schreiner ◽  
M. J. Lentze ◽  
D. NGampolo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Induced Sputum ◽  
Necrosis Factor Alpha ◽  
Free Interval ◽  
Factor Alpha ◽  
The Relationship ◽  
Necrosis Factor ◽  
Severe Lung Disease ◽  
Severe Lung

Background. High-producer TGFβ1 genotypes are associated with severe lung disease in cystic fibrosis (CF), but studies combining IL-8, TNFα-, and TGFβ1(+genotype) levels and their impact on CF lung disease are scarce.Aim. Assessing the relationship between TGFβ1, IL-8, and TNF-αand lung disease in CF in an exacerbation-free interval.Methods. Twenty four patients delta F508 homozygous (median age 20.5 y, Shwachman score 75, FEV1(%) 83) and 8 controls (median age 27.5 y) were examined. TGFβ1 was assessed in serum and induced sputum (IS) by ELISA, for IL-8 and TNF-αby chemiluminescence in IS and whole blood. Genotyping was performed for TGFβ1 C−509T and T+869C utilizing RFLP.Results. TGFβ1 in IS (CF/controls median 76.5/59.1 pg/mL,P<0.074) was higher in CF. There was a negative correlation between TGFβ1 in serum and lung function (LF) (FEV1 (r=−0.488,P=0.025), MEF 25 (r=−0.425,P=0.055), and VC (r=−0.572,P=0.007)). Genotypes had no impact on TGFβ1 in IS, serum, and LF. In IS TGFβ1 correlated with IL-8 (r=0.593,P<0.007) and TNF-α(r=0.536,P<0.018) in patients colonized by bacteria with flagellin.Conclusion. TGFβ1 in serum not in IS correlates with LF. In patients colonized by bacteria with flagellin, TGFβ1 correlates with IL-8 and TNF-αin IS.

Sign in / Sign up

Export Citation Format

Share Document