Characterization of Cystic Fibrosis Airway Smooth Muscle Cell Proliferative and Contractile Activities.

In this study, the contractile and proliferative properties of ASM cells isolated from healthy donor and CF transplant lungs were compared. Proliferation assays showed CF ASM proliferate at a higher rate than healthy cells. Through calcium analysis, no differences in contractile activation was found, however, CF ASM lagged in their reuptake of calcium. The combination CFTR corrector and potentiator, VX-809/770, was used to restore proper CFTR function in CF ASM which resulted in a reduction in proliferation and in a normalization of calcium reuptake kinetics. These results show that improper CFTR function in ASM cause intrinsic changes in their proliferative and contractile properties. PMID: 31508974 [PubMed - as supplied by publisher]
Source: Am J Physiol Lung Ce... - Category: Respiratory Medicine Authors: Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research

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AbstractThe contribution of T-cells after lung transplant (LTx) remains controversial with no current consensus of their role concerning chronic lung allograft dysfunction. Using flow cytometry to assess T-cell subsets of bronchoalveolar lavage fluid (BALF) in 16 cystic fibrosis (CF) LTx recipients, we identified a decline in CD4+ T-cell frequency and an increase in CD8+ T-cell frequency in patients who developed severe bronchiolitis obliterans syndrome (BOS) (N = 10) when comparing baseline (6 months post-LTx) and follow-up (most recent bronchoscopy—clinical or surveillance per protocol). Comparin...
Source: Lung - Category: Respiratory Medicine Source Type: research
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Source: OnMedica Latest News - Category: UK Health Source Type: news
Conclusion: BE accounts for 11,2% of all LTx done in our center, a distribution that is superior to ISHLT registry. Comparing to all LTx pts we recognize a good BE post-transplant outcome, with a similar survival rate at 1-year and even superior at 5-years. These results reinforce the need to referral pts with severe and end stage BE and supports the role of LTx as valuable treatment for this disease.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Transplantation Source Type: research
MUC5B polymorphism has been associated with an increased risk of IPF. Compared to the 9% found in controls, the minor allele was found in 34% of patients with familial IPF, in 38% of sporadic IPF. Recently, MUC5B has also been associated with chronic hypersensitivity pneumonitis (24.4-and 32.2 vs. 10.7%) and rheumatoid arthritis with interstitial lung disease (32.6 vs 10.9%).We retrospectively assessed MUC5B SNP (rs35705950) in all patients that underwent lung transplant between 1991 and 2015 in our hospital (n=746). We associated the incidence of the MUC5B minor allele with the underlying lung disease and post transplant ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Idiopathic interstitial pneumonias Source Type: research
Conclusions: Of those patients deemed suitable for therapy, TLI was well tolerated with the majority of complications being minor and not leading to cessation of treatment. Therapy is associated with a significant slowing in the rate of decline in lung function impacting overall survival.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Transplantation Source Type: research
Introduction: Identification of risk factors that predict poor survival can aid clinical decision making and allow optimization of any potentially modifiable factors (Stephenson, A.L. et al. J Heart Lung Transplant 2015;34:1139–1145). This may facilitate improved patient selection and ultimately improve overall outcomes.Aims: To describe the Irish experience of co-morbidity post-transplant for cystic fibrosis and to investigate potential non-pulmonary pre-transplant risk factors that could impact on long term survival.Methods: We performed a retrospective review of all 61 patients who received a lung transplant for c...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Transplantation Source Type: research
Conclusions: The survival rate of CF patients after LTx in Norway is similar to internationally published data. LTx increases survival significantly compared to the observed survival on the waitlist and patients of intermediary risk seem to have the greatest survival benefit.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Transplantation Source Type: research
Conclusion: Not performing quality control of clinical MBW data may result in overestimation of LCI. We recommend prospective MBW quality control in the clinical setting.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric respiratory physiology and sleep Source Type: research
We report our 10-year experience in the use of NIV with regards to indications and outcomes.Methods: A retrospective analysis of data captured prospectively was performed. All patients started on NIV between 2008 and 2018 were included; lung transplant (LTX) recipients and those who received NIV for
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Cystic fibrosis Source Type: research
We report outcomes at 6 months after NIV initiation in adult patients attending a large CF centre.Methods: A retrospective analysis of data captured prospectively was performed. All episodes of NIV between 2008 and 2018 were included. Exclusion criteria were: use in lung transplant (LTx) recipients, use for
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Cystic fibrosis Source Type: research
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