Common virus linked to faster disease progression in cystic fibrosis

(European Lung Foundation) A new study has found that cystic fibrosis patients who have a common virus may experience faster disease progression than patients who do not have the virus. Signs of faster cystic fibrosis disease progression included earlier times to lung transplant referral and reaching the final stages of the disease.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news

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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 intr...
Source: Am J Physiol Lung Ce... - Category: Respiratory Medicine Authors: Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research
Despite improvements in the control of respiratory infections and life expectancy in cystic fibrosis (CF), highly resistant pathogens such as nontuberculous mycobacteria have emerged as an increasing challenge [1]. Disease due to M. abscessus is of particular concern as it is associated with increased healthcare utilisation, accelerated lung function decline, impaired quality of life (QoL) [2], adverse impacts on post-lung transplant outcomes [3], and increased mortality [2]. Good quality evidence to support current treatment regimens is lacking, and access to effective antimicrobial treatment options is limited.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Editorial Source Type: research
Abstract RATIONALE: Cystic fibrosis (CF) pulmonary disease is characterized by chronic infection with Pseudomonas aeruginosa and sustained neutrophil-dominant inflammation. The lack of effective anti-inflammatory therapies for people with CF (PWCF) represents a significant challenge. OBJECTIVES: To identify altered immunometabolism in the CF neutrophil, and investigate the feasibility of specific inhibition of the NLR family, pyrin domain-containing protein 3 (NLRP3) inflammasome as a CF anti-inflammatory strategy in vivo. METHODS: Key markers of increased aerobic glycolysis, known as a Warburg effect, i...
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research
Lung transplantation is an established therapeutic option for patients with irreversible end-stage pulmonary disease limiting life expectancy and quality of life. Common indications for lung transplantation include chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis, pulmonary arterial hypertension, and alpha-1 antitrypsin deficiency. Complications of lung transplantation can be broadly divided etiologically into surgical, infectious, immunologic, or neoplastic. Moreover, specific complications often occur within a certain time interval following surgery, which can be broadly classified as...
Source: Journal of Thoracic Imaging - Category: Radiology Tags: Review Articles Source Type: research
Authors: Nieborak-Raczkowska M, Kubisa B, Piotrowska M, Grodzki T, Kubisa A, Pieróg J, Wojtyś M, Bielewicz M, Czarnecka M, Wójcik J, Wójcik N, Sielicki W, Brykczyński M PMID: 31410098 [PubMed]
Source: Polish Journal of Cardio-Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Tags: Kardiochir Torakochirurgia Pol Source Type: research
Abstract Sickle cell disease afflicts millions of people worldwide and approximately 100,000 Americans. Complications are myriad and arise as a result of complex pathological pathways 'downstream' to a point mutation in DNA, and include red blood cell membrane damage, inflammation, chronic hemolytic anemia with episodic vaso-occlusion, ischemia and pain, and ultimately risk of cumulative organ damage with reduced patient lifespan. The National Heart, Lung, and Blood Institute's 2014 evidence-based guideline for sickle cell disease -management states additional research is needed before investigational curative the...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
A 30-year-old cystic fibrosis (CF) male with recurrent multi-drug resistant Burkholderia multivorans infections developed persistent bacteremia, despite high-dose antibiotics for>10 days, and ventilator as well as venovenous (VV) extracorporeal membrane oxygenation (ECMO) dependence limiting his transplant candidacy.1,2 No non-pulmonary sources of infection were identified; his chest radiograph demonstrated significant pulmonary disease. We performed bilateral pneumonectomies to clear his infection.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Case Anecdotes, Comments and Opinions Source Type: research
ConclusionsTen ‐year survivors were significantly younger, weighed less, and had significantly shorter lengths of hospitalization after transplantation. Bilateral lung transplantation was a significant factor in prolonged survival. Survival also improved with institutional experience
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: ORIGINAL ARTICLE Source Type: research
The increasing life expectancy of individuals with Cystic Fibrosis (CF) is likely to be associated with new age-related challenges, colorectal cancer (CRC) most notably; recent consensus recommendations for CRC s...
Source: BMC Gastroenterology - Category: Gastroenterology Authors: Tags: Case report Source Type: research
PMID: 31356757 [PubMed - as supplied by publisher]
Source: Am J Respir Crit Car... - Category: Intensive Care Authors: Tags: Am J Respir Crit Care Med Source Type: research
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