New insights into the pathogenesis of cystic fibrosis.

摘要: Cystic fibrosis (CF) is the most frequent inheritable disease with a lethal course during childhood. The characteristic high viscosity of mucoid secretion products in lungs, pancreas, and gut cause plugging secondary damage these organs. In past 20 years effective treatment intestinal obstruction neonatal period infections lungs has improved prognosis significantly. Many patients will reach adulthood near future. 10 new insights into changed diagnostic procedures and, it to be hoped, soon also treatment. first development was estimation brush-border enzymes amniotic fluid. With this method prenatal diagnosis possible 17th—18th week pregnancy. recent discovery gene on chromosome 7 its structure important breakthrough. At same time process CI transport across mucosal membrane many types epithelium subject investigation by sever...