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...
Cystic Fibrosis Cystic fibrosis(CF) is a genetic disorder characterized by multisystem organ involvement including the pancreas, lungs, andgastrointestinal tract.Pancreatic enzymereplacement is necessary to promote adequate digestion of nutrients. Nutritional status has a strong impact on the survival of pat...
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...
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...
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 (CF) is an autosomal recessive disorder that is characterized by an abnormality of exocrine gland function. Adult patients represent a rapidly growing percentage of the CF population. Pulmonary changes are seen in nearly every case and are the most serious complication of CF. In ad...
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...
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 The FASEB Journal 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...
Cystic fibrosis (CF) lung disease is characterized by an exaggerated, and extended inflammation, with a massive influx of neutrophils and high concentrations of interleukin (IL)-8 in bronchoalveolar lavage fluid (BALF). Furthermore, patients with CF have chronic bacterial airway infection, particularly...