scholarly journals Eradication ofBurkholderia cepaciaUsing Inhaled Aztreonam Lysine in Two Patients with Bronchiectasis

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
A. Iglesias ◽  
I. Artiles ◽  
J. J. Cabanillas ◽  
R. Álvarez-Sala ◽  
C. Prados
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Clinical Data ◽  
Pulmonary Infection ◽  
Single Center ◽  
Bronchial Infection

There are not many articles about the chronic bronchial infection/colonization in patients with underlying lung disease other than cystic fibrosis (CF), especially with non-CF bronchiectasis (NCFBQ). The prevalence ofB. cepacia complexis not well known in NCFBQ. The vast majority of published clinical data onBurkholderiainfection in individuals with CF is comprised of uncontrolled, anecdotal, and/or single center experiences, and no consensus has emerged regarding treatment. We present two cases diagnosed with bronchiectasis (BQ) of different etiology, with early pulmonary infection byB. cepacia complex, which was eradicated with inhaled aztreonam lysine.

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 Factors associated with severe lung disease in an adult population with cystic fibrosis: a single-center experience

2020 ◽  
Vol 50 (4) ◽  
pp. 945-952
Author(s):  
Berrin ER ◽  
Ebru ÇELEBİOĞLU ◽  
Ebru YALÇIN ◽  
Deniz DOĞRU ◽  
Özlem ERDEN AKI ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Adult Population ◽  
Single Center ◽  
Factors Associated ◽  
Single Center Experience ◽  
Severe Lung Disease ◽  
Severe Lung

scholarly journals Ivacaftor Therapy in CF Patients: Single Center Experience

2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
Pritish Mondal ◽  
Amber Loyson ◽  
Jorge Lascano ◽  
Satyanarayan Hegde
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Lung Disease ◽  
Case Series ◽  
Forced Expiratory Volume ◽  
Third Party ◽  
Normal Lung ◽  
Single Center ◽  
Single Center Experience ◽  
Pediatric Age Group

Ivacaftor is the first novel cystic fibrosis pharmaceutical that acts at the molecular level to potentiate cystic fibrosis transmembrane conductance regulator (CFTR) function and was first approved for clinical use in 2012. We are sharing our single center experience of five patients: four from pediatric age group and one adult patient. All patients had both subjective and objective improvements in their health. Despite established lung disease, our patients had significant improvement in both their FEV1 (forced expiratory volume in 1 second) and FEF25–75and BMI (body mass index). Larger studies demonstrated only 6.7% improvement in mean FEV1 after starting Ivacaftor therapy but their patient population had normal lung function to begin with. In contrast our case series demonstrates that, in patients with established lung disease and diminished lung function, Ivacaftor can be expected to result in much higher recovery in lung function. Mean FEV1 improved by 35% in our case series. Ivacaftor is extremely expensive, costing $300,000 per patient per year requiring lifelong therapy, hence requiring prior authorizations from most third-party payers in the USA. The knowledge shared from our experience will be useful for other clinicians to petition healthcare policymakers on behalf of their patients.

Regional differences in infection and structural lung disease in infants and young children with cystic fibrosis

2020 ◽  
Vol 19 (6) ◽  
pp. 917-922 ◽  
Author(s):  
Rosemary Carzino ◽  
Katherine B. Frayman ◽  
Louise King ◽  
Suzanna Vidmar ◽  
Sarath Ranganathan
Keyword(s):  
Cystic Fibrosis ◽  
Young Children ◽  
Lung Disease ◽  
Regional Differences ◽  
Infants And Young Children

scholarly journals DNA Methylation at ATP11A cg11702988 Is a Biomarker of Lung Disease Severity in Cystic Fibrosis: A Longitudinal Study

Genes ◽  
2021 ◽  
Vol 12 (3) ◽  
pp. 441
Author(s):  
Fanny Pineau ◽  
Davide Caimmi ◽  
Sylvie Taviaux ◽  
Maurane Reveil ◽  
Laura Brosseau ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Dna Methylation ◽  
Clinical Trials ◽  
Lung Disease ◽  
Disease Severity ◽  
Genome Wide ◽  
A Genome ◽  
Lung Disease Severity ◽  
Epigenetic Biomarker

Cystic fibrosis (CF) is a chronic genetic disease that mainly affects the respiratory and gastrointestinal systems. No curative treatments are available, but the follow-up in specialized centers has greatly improved the patient life expectancy. Robust biomarkers are required to monitor the disease, guide treatments, stratify patients, and provide outcome measures in clinical trials. In the present study, we outline a strategy to select putative DNA methylation biomarkers of lung disease severity in cystic fibrosis patients. In the discovery step, we selected seven potential biomarkers using a genome-wide DNA methylation dataset that we generated in nasal epithelial samples from the MethylCF cohort. In the replication step, we assessed the same biomarkers using sputum cell samples from the MethylBiomark cohort. Of interest, DNA methylation at the cg11702988 site (ATP11A gene) positively correlated with lung function and BMI, and negatively correlated with lung disease severity, P. aeruginosa chronic infection, and the number of exacerbations. These results were replicated in prospective sputum samples collected at four time points within an 18-month period and longitudinally. To conclude, (i) we identified a DNA methylation biomarker that correlates with CF severity, (ii) we provided a method to easily assess this biomarker, and (iii) we carried out the first longitudinal analysis of DNA methylation in CF patients. This new epigenetic biomarker could be used to stratify CF patients in clinical trials.

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