Search results for " FIBROSIS"

Development and electronic validation of the revised Cystic Fibrosis Questionnaire (CFQ-R Teen/Adult)

2018

Abstract Background The Cystic Fibrosis Questionnaire-Revised (CFQ-R+14) is a disease-specific, health-related quality of life instrument for cystic fibrosis (CF) patients ≥14years. We have developed a Spanish electronic version of the CFQ-R (e-CFQ-R+14 Spain). Our aim was to compare the paper and electronic versions and to validate the electronic version. Methods Fifty CF patients completed the study. All answered the paper and electronic versions on day 1 and repeated the e-CFQR version 15days later. Results Concordance between the electronic and paper copy versions was high, with correlations above 0.9 in all domains. Test-retest reliability of the e-CFQ-R results was strong, with coeffi…

Telomerase and Telomere Length in Pulmonary Fibrosis

2013

In addition to its expression in stem cells and many cancers, telomerase activity is transiently induced in murine bleomycin (BLM)-induced pulmonary fibrosis with increased levels of telomerase transcriptase (TERT) expression, which is essential for fibrosis. To extend these observations to human chronic fibrotic lung disease, we investigated the expression of telomerase activity in lung fibroblasts from patients with interstitial lung diseases (ILDs), including idiopathic pulmonary fibrosis (IPF). The results showed that telomerase activity was induced in more than 66% of IPF lung fibroblast samples, in comparison with less than 29% from control samples, some of which were obtained from lu…

IMPROVEMENT OF MOLECULAR TECHNOLOGIES AND PRENATAL DIAGNOSIS OF CYSTIC FIBROSIS

2008

Pleuroparenchymal fibroelastosis: one more walk on the wild side of drugs?

2014

In this issue of the European Respiratory Journal , Beynat-Mouterde et al. [1] report on six young adults (three of whom were female) who developed a clinical imaging pattern of predominant upper lobe fibrosis with apical pneumothoraces (fig. 1). Presentation in all six patients was similar with cough, dyspnoea, occasional chest pain and weight loss. Imaging was distinctive and showed a cephalad, irregular, pleural-based thickening that encroached on the lung bilaterally. Five patients presented with “platythorax” (fig. 2), a preferential reduction in the anterio-posterior diameter of the chest wall. In all patients, severe restrictive or restrictive-obstructive lung dysfunction progressed …

Molecular analysis of genes encoding CFTR interactors of SLC26 family in CF patients: preliminary results

2008

Aclidinium inhibits human lung fibroblast to myofibroblast transition

2011

Background Fibroblast to myofibroblast transition is believed to contribute to airway remodelling in lung diseases such as asthma and chronic obstructive pulmonary disease. This study examines the role of aclidinium, a new long-acting muscarinic antagonist, on human fibroblast to myofibroblast transition. Methods Human bronchial fibroblasts were stimulated with carbachol (10 −8 to 10 −5  M) or transforming growth factor-β1 (TGF-β1; 2 ng/ml) in the presence or absence of aclidinium (10 −9 to 10 −7  M) or different drug modulators for 48 h. Characterisation of myofibroblasts was performed by analysis of collagen type I and α-smooth muscle actin (α-SMA) mRNA and protein expression as well as α…

Bronchial to subclavian shunt in a CF patient. A potential pitfall for embolization

2003

Bronchial artery embolization is a well accepted and widely used technique in the management of massive haemoptysis in cystic fibrosis (CF). It can be a complex procedure requiring a deep knowledge of the bronchial artery anatomy including the possible bronchial anastomoses. We report a case of complex vascular anatomy of the left bronchial artery with multiple anastomoses with the ipsilateral subclavian artery as cause of non-attempted embolization. © 2003 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Psychological Profile of Patients Diagnosed With Idiopathic Pulmonary Fibrosis

2022

Functional Analysis in Single-Lung Transplant Recipients

2004

Objective To develop and evaluate a postprocessing tool to quantify ventilated split-lung volumes on the basis of 3 He-MRI and to apply it in patients after single-lung transplantation (SLTX). High-resolution CT (HRCT) was employed as a reference modality providing split air-filled lung volumes. Lung volumes derived from pulmonary function test results served as clinical parameters and were used as the “gold standard.” Material and methods Eight patients (mean age, 54 years) with emphysema and six patients (mean age, 58 years) with idiopathic pulmonary fibrosis. All patients were evaluated following SLTX. HRCT was performed during inspiration (slice thickness, 1 mm; increment, 10 mm). For c…

The impact of SARS-COV2 pandemic on the management oF IPF patients: Our narrative experience

2021

Abstract Background The SARS-CoV-2 pandemic has changed the health-care systems around the world in a remarkable way. We describe the strategies adopted to cope with the limitations imposed by the pandemic to the access to health care by patients diagnosed with idiopathic Pulmonary Fibrosis (IPF). Material and methods We conducted a retrospective observational analysis including IPF patients under antifibrotic drugs (nintedanib and pirfenidone) that accessed to the Outpatient clinic of the University of Palermo, Italy. Patients received a phone number and an email address in case of any urgency and a virtual meeting was settled up monthly. Results 40 patients (M/F: 30/10) were followed up, …