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Mashup Score: 0Implementation of standardized cystic fibrosis care algorithm to improve the center data-quality improvement project international collaboration - 1 year(s) ago
Better lung function in people with cystic fibrosis (pwCF) is strongly associated with improved quality of life and survival. The most often used pulmonary function measurement parameter is forced expiratory volume in one second percent predicted (FEV1pp), and this is also one of the indicators of cystic fibrosis (CF) care quality [1,2]. Patient registries allow for comparison of different CF…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 0
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, leading to reductions in the quantity and/or function of CFTR protein, an anion channel present in various epithelial cells [1–4]. The F508del-CFTR mutation is the most common CFTR mutation, with nearly 90% of people with CF in some regions of the world having ≥1…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 0Early life growth trajectories in cystic fibrosis are associated with lung function at age six - 1 year(s) ago
Decline in pulmonary status is the hallmark of cystic fibrosis (CF); however, poor growth is one of its earliest manifestations. The combined effects of decreased nutrient intake, malabsorption, and increased metabolic demands contribute to poor nutritional status and growth, which may be present in infancy and persist through adolescence and adulthood. Poor nutritional status is associated with…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 0EFFECT OF ELEXACAFTOR/TEZACAFTOR/IVACAFTOR ON ANNUAL RATE OF LUNG FUNCTION DECLINE IN PEOPLE WITH CYSTIC FIBROSIS - 1 year(s) ago
Progressive lung function decline is a hallmark of cystic fibrosis (CF), with most patients experiencing a decline in percent predicted forced expiratory volume in 1 second (ppFEV1) of 1 to 3 percentage points annually [1]. More rapid decline is associated with more severe CF lung disease and earlier mortality; therefore, preserving lung function is a primary goal of CF clinical care [2,3].
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 0
Pulmonary exacerbations (PEx) are clinically important events for people living with cystic fibrosis (CF) [1] and are characterized by worsening respiratory symptoms typically due to augmented infection and inflammation [2]. Despite the lack of a consensus definition of PEx, increased respiratory symptoms and decreased lung function are used in clinical practice to diagnose PEx [3]. Both…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 1
Maintenance treatment with inhaled antibiotic agents has been found to reduce the burden of bacteria in the airways [1–3], as well as the frequency of exacerbations and hospitalizations in people with cystic fibrosis (pwCF) who are chronically infected with Pseudomonas aeruginosa (PA) [1,3–8]. Accordingly, CF practice guidelines recommend chronic treatment with inhaled antibiotics for pwCF who…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 1
Maintenance treatment with inhaled antibiotic agents has been found to reduce the burden of bacteria in the airways [1–3], as well as the frequency of exacerbations and hospitalizations in people with cystic fibrosis (pwCF) who are chronically infected with Pseudomonas aeruginosa (PA) [1,3–8]. Accordingly, CF practice guidelines recommend chronic treatment with inhaled antibiotics for pwCF who…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 0Impact of antibiotic eradication therapy of Pseudomonas aeruginosa on long term lung function in cystic fibrosis - 2 year(s) ago
Cystic fibrosis is a life-limiting autosomal recessive disorder with highest prevalence in Europe, North America and Australia [1]. Around 30,000 individuals are affected in the United States and 45,000 in Europe [2]. Over the last few decades, the median age of survival has increased substantially due to optimised treatment of secondary effects of CFTR dysfunction such as airway infection [3].
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 2Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway disease - 2 year(s) ago
Resident macrophages play a key role in tissue homeostasis and responses to infection and injury [1]. In the airways, macrophages quickly adapt to variations in oxygen pressure, inflammation, and the presence of pathogens [2]. Exposure of airway macrophages at birth and thereafter to host- and microbe-derived factors shapes long-term responses and the likelihood to mount an aberrant response to…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
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Mashup Score: 1Aerosolized lancovutide in adolescents (≥12 years) and adults with cystic fibrosis – a randomized trial - 4 year(s) ago
Mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene lead to absence or dysfunction of the CFTR anion channel in epithelial membranes and cause the clinical manifestations of cystic fibrosis (CF). Over the last decade, highly effective modulators of CFTR function have been developed that may prove transformational for approximately 90% of people who have specific CFTR…
Source: Journal of Cystic FibrosisCategories: Latest Headlines, PulmonologyTweet
Implementation of #standardized #cysticfibrosis #care #algorithm to improve the center #data-quality #improvement project international collaboration https://t.co/qPURxdr66C https://t.co/4CwQS40gxO