scholarly journals Glycoprotein A as a biomarker of pulmonary infection and inflammation in children with cystic fibrosis

2019 ◽  
Vol 55 (2) ◽  
pp. 401-406
Author(s):  
Ajay C. Kevat ◽  
Rosemary Carzino ◽  
Suzanna Vidmar ◽  
Sarath Ranganathan
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Infection ◽  
Infection And Inflammation

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 Targeted Drug Delivery Technologies Potentiate the Overall Therapeutic Efficacy of an Indole Derivative in a Mouse Cystic Fibrosis Setting

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1601
Author(s):  
Matteo Puccetti ◽  
Marilena Pariano ◽  
Giorgia Renga ◽  
Ilaria Santarelli ◽  
Fiorella D’Onofrio ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Targeted Drug Delivery ◽  
Fungal Infections ◽  
Murine Models ◽  
Multisystem Disease ◽  
Aryl Hydrocarbon ◽  
Target Organs ◽  
Infection And Inflammation ◽  
Anti Inflammatory ◽  
Inflammatory Toxicity

Inflammation plays a major role in the pathophysiology of cystic fibrosis (CF), a multisystem disease. Anti-inflammatory therapies are, therefore, of interest in CF, provided that the inhibition of inflammation does not compromise the ability to fight pathogens. Here, we assess whether indole-3-aldehyde (3-IAld), a ligand of the aryl hydrocarbon receptor (AhR), may encompass such an activity. We resorted to biopharmaceutical technologies in order to deliver 3-IAld directly into the lung, via dry powder inhalation, or into the gut, via enteric microparticles, in murine models of CF infection and inflammation. We found the site-specific delivery of 3-IAld to be an efficient strategy to restore immune and microbial homeostasis in CF organs, and mitigate lung and gut inflammatory pathology in response to fungal infections, in the relative absence of local and systemic inflammatory toxicity. Thus, enhanced delivery to target organs of AhR agonists, such as 3-IAld, may pave the way for the development of safe and effective anti-inflammatory agents in CF.

Pulmonary infection secondary to Blastobotrys raffinosifermentans in a cystic fibrosis patient: Review of the literature

Mycoses ◽  
2021 ◽  
Author(s):  
Mohanad Al‐Obaidi ◽  
Hamid Badali ◽  
Connie Cañete‐Gibas ◽  
Hoja P. Patterson ◽  
Nathan P. Wiederhold
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Pulmonary Infection ◽  
Review Of The Literature

scholarly journals Differential Persistence among Genomovars of the Burkholderia cepacia Complex in a Murine Model of Pulmonary Infection

2002 ◽  
Vol 70 (5) ◽  
pp. 2715-2720 ◽  
Author(s):  
Karen K. Chu ◽  
Donald J. Davidson ◽  
T. Keith Halsey ◽  
Jacqueline W. Chung ◽  
David P. Speert
Keyword(s):  
Cystic Fibrosis ◽  
Clinical Outcomes ◽  
Murine Model ◽  
Burkholderia Cepacia ◽  
Pulmonary Infection ◽  
Burkholderia Cepacia Complex ◽  
Clinical Infection ◽  
Minimal Toxicity

ABSTRACT Cystic fibrosis patients infected with strains from different genomovars of the Burkholderia cepacia complex can experience diverse clinical outcomes. To identify genomovar-specific determinants that might be responsible for these differences, we developed a pulmonary model of infection in BALB/c mice. Mice were rendered leukopenic by administration of cyclophosphamide prior to intranasal challenge with 1.6 × 104 bacteria. Five of six genomovar II strains persisted at stable numbers in the lungs until day 16 with minimal toxicity, whereas zero of seven genomovar III strains persisted but resulted in variable toxicity. We have developed a chronic pulmonary model of B. cepacia infection which reveals differences among genomovars in terms of clinical infection outcome.

scholarly journals EPS4.02 Porphyromonas, a candidate biomarker for detection of Pseudomonas aeruginosa pulmonary infection in cystic fibrosis

2018 ◽  
Vol 17 ◽  
pp. S43 ◽  
Author(s):  
C.-A. Guilloux ◽  
G. Marenne ◽  
S. Mondot ◽  
C. Lamoureux ◽  
L. Billard ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Pulmonary Infection

Pulmonary Infection and its Management in Cystic Fibrosis Patients in the Czech Republic

1996 ◽  
pp. 251-257 ◽  
Author(s):  
Věra Vávrová ◽  
Zdenka Jedličková ◽  
Otto Lochmann ◽  
Dana Zemková ◽  
Hana Krásničanová ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Czech Republic ◽  
Pulmonary Infection ◽  
The Czech Republic

The General Approach to Cystic Fibrosis Pulmonary Infection in Russia

1996 ◽  
pp. 167-173
Author(s):  
Tatiana E. Guembitskaia ◽  
Ludmila A. Vishnyakova ◽  
Ludmila A. Zhelenina ◽  
Nikolai J. Kapranov
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Infection

scholarly journals Impact of Airway Inflammation on the Efficacy of CFTR Modulators

Cells ◽  
2021 ◽  
Vol 10 (11) ◽  
pp. 3260
Author(s):  
Carla M. P. Ribeiro ◽  
Martina Gentzsch
Keyword(s):  
Cystic Fibrosis ◽  
Airway Inflammation ◽  
Primary Cultures ◽  
Lavage Fluid ◽  
Improved Outcomes ◽  
Inflammatory Stimuli ◽  
Infection And Inflammation ◽  
The Impact ◽  
Cftr Modulators

Defective CFTR biogenesis and activity in cystic fibrosis airways leads to airway dehydration and impaired mucociliary clearance, resulting in chronic airway infection and inflammation. Most cystic fibrosis patients have at least one copy of the F508del CFTR mutation, which results in a protein retained in the endoplasmic reticulum and degraded by the proteosomal pathway. CFTR modulators, e.g., correctors, promote the transfer of F508del to the apical membrane, while potentiators increase CFTR activity. Corrector and potentiator double therapies modestly improve lung function, whereas triple therapies with two correctors and one potentiator indicate improved outcomes. Enhanced F508del rescue by CFTR modulators is achieved by exposing F508del/F508del primary cultures of human bronchial epithelia to relevant inflammatory stimuli, i.e., supernatant from mucopurulent material or bronchoalveolar lavage fluid from human cystic fibrosis airways. Inflammation enhances the biochemical and functional rescue of F508del by double or triple CFTR modulator therapy and overcomes abrogation of CFTR correction by chronic VX-770 treatment in vitro. Furthermore, the impact of inflammation on clinical outcomes linked to CFTR rescue has been recently suggested. This review discusses these data and possible mechanisms for airway inflammation-enhanced F508del rescue. Expanding the understanding of how airway inflammation improves CFTR rescue may benefit CF patients.

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