Cystic fibrosis (CF) is characterized by the presence of a viscoelastic mucus layer in the upper airways and bronchi. The underlying problem is a mutation in the gene encoding the cystic fibrosis transmembrane conductance regulator protein. Clinical studies of gene transfer for CF are ongoing. For...
It is formed by a compartment housing the capsule, inside the cover or the body of the inhaler, and a cutting element, inside the... IE Pera 被引量: 0发表: 2002年 Systemic deficiency of glutathione in cystic fibrosis Cystic fibrosis (CF), a disorder characterized by mutations of the CF...
Cystic fibrosisis characterized by abnormal fluid transport across many epithelia including airways, pancreas, sweat glands and small intestine. One therapeutic goal is to improve mucociliary clearance, and thereby prevent progressive lung damage caused by infections.Cystic fibrosisis associated with decreas...
A high proportion of patients with cystic fibrosis (CF) develop diabetes mellitus. In common with type II diabetes mellitus, diabetes mellitus in CF is characterized by a progressive decline in beta-cell function and an approximately 50% decline in beta-cell mass. It is not known whether islet...
Abnormal regulation of ion channels in cystic fibrosis epithelia Michael J. Welsh Jul 1990 Cystic fibrosis (CF), the most common lethal genetic disease in Caucasians, is characterized by defective electrolyte transport in several epithelia. In sweat duct, pancreatic, intestinal, and airway epithelia,...
Since cystic fibrosis is recessive, both copies of the gene have to be CF genes to cause the symptoms that occur in about 1 in every 2500 children. The high incidence of this lethal gene can be explained by the fact that CF carriers, who don't show any symptoms, enjoy some protection ...
Cystic fibrosis is a single-gene autosomal recessive disorder characterized by chronic airway infection, pancreatic insufficiency, gastrointestinal dysfunction, and male infertility. Cystic fibrosis is caused by a mutation in the cystic fibrosis membrane conductance regulator (CFTR) protein. Mutations in the...
BackgroundCystic fibrosis (CF) is characterized by recurrent respiratory infections and progressive lung disease. Although environmental factors account for 50% of the variation in CF lung function, few specific exposures have been identified. Studies using small study samples focusing on environmental all...
cystic fibrosis n.Abbr.CF A genetic disease that involves dysfunction of the exocrine glands and affects many organs and organ systems, especially the respiratory system, the pancreas, the intestines, the sweat glands, and, in males, the reproductive system. It is characterized by the chronic acc...
Study of currently approved drugs and exploration of future clinical development pipeline therapeutics for cystic fibrosis, and possible limitations in their use. Extensive literature search using individual and a combination of key words related to cyst