Cystic fibrosis (CF) is the most common lethal genetic disorder amongst Caucasians with a current life expectancy of about 40 years. A dysfunctional CF transmembrane conductance regulator chloride channel is the single primary cause of this complex systemic disease that negatively affects every secretory...
Cystic fibrosis. In: Loughlin GM, Eigen H (eds), Respiratory disease in children: Diagnosis and management. Baltimore: Williams & Wilkins, 1994: 263–269. Google Scholar Lewis PA. The epidemiology of cystic fibrosis. In: Hodson ME, Geddes DM (eds), Cystic fibrosis. London: Chapman & ...
Finally, the role of different viral and non-viral nanocarriers in the delivery of therapeutics to the lungs has been evaluated. 2. Pathogenesis of Cystic Fibrosis (CF) 2.1. Mutation in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) The leading cause of CF is a genetic alteration in...
of thecystic fibrosis transmembrane conductance regulator(CFTR), a Cl−channel expressed by epithelial cells, and by an increase in active Na+absorption. One promising treatment is the use of inhaled UTP andamiloride(a K+-sparing diuretic)19(Table 1; seeRefs 1, 4, 19–31). This treatment...
Cystic fibrosis (CF) or mucoviscidosis is the most frequent autosomal recessive genetic disorder in people with European ancestry. The classic form of CF results from loss of function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The gene is localized to the long...
Microbiologic data overview of Italian cystic fibrosis patients 来自 Semantic Scholar 喜欢 0 阅读量: 28 作者: TS Campana 摘要: The aim of our study was to determine the frequency of microbiological culturing and prevalence of colonization by principal pathogens of the respiratory tract of ...
CYSTIC FIBROSIS Cystic fibrosis is an autosomal recessive disease characterized by exocrine gland dysfunction that results in pulmonary infections and progressive pancreatic insufficiency in 85% of affected children.381 Precipitation of secretions in the small pancreatic ducts result in fatty and fibrous dege...
CCN4 (cellular communication network factor 4), a highly conserved, secreted cysteine-rich matricellular protein is emerging as a key player in the development and progression of numerous disease pathologies, including cancer, fibrosis, metabolic and inflammatory disorders. Over the past two decades, ...
Cystic fibrosis (CF) or mucoviscidosis is the most frequent autosomal recessive genetic disorder in people with European ancestry. The classic form of CF results from loss of function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The gene is localized to the ...
Sweat test and cystic fibrosis: overview of test performance at public and private centers in the state of So Paulo, Brazil The sweat test (ST) measures chloride levels in sweat and is considered the gold standard for the diagnosis of cystic fibrosis (CF). However, the reliabili... SM Fá...