1. Pulmonary, pancreatic, hepatic, reproductive symptoms.
2. Death before reaching age 30 years.
1. All ethnic groups and geographic locations.
2. Most common in Caucasians (1 per 2500 newborns).
1. European folklore, clinical description, diagnosis.
1. Positional cloning, negative gene map, marker.
1. Glycoprotein chloride channel.
2. Missense, nonsense, frame shift, splicing mutations.
3. Specific ethnic variations and geographic trends.
4. Cystic fibrosis phenotypic subtypes.
1. Antibiotics, enzymes, transplantation.
2. Deoxyribonuclease, amiloride, nucleotides.
Cystic Fibrosis is a genetic disorder which affects children and young adults. Inheritance of the mutant gene may result in chronic pulmonary, pancreatic, and liver disease, as well as other disorders. Because the condition is fairly common, it has received considerable attention. Cellular physiologic and molecular genetic discoveries in recent years have provided considerable information regarding cystic fibrosis' various dimensions. Still, though, the disease's pathophysiology, diagnosis, and treatment, continue to be subjects of scrutiny.
A generalized multi organ system disease, cystic fibrosis (CF) is caused by a single biochemical abnormality (11:134). The disease is characterized by slowly accumulating mucous secretions in the affected organs (14:474). One of the major problems with CF is progressive respiratory failure. This pathology eventually results in chronic obstructive pulmonary disease. These patients tend to suffer from chronic respiratory infection the most common of which is caused by Pseudomonas aeruginosa (13:27). Indeed, more than 90 percent of CF patients die as a result of blockage of their airways (10:4). Other manifestations of cystic fibrosis can include pancreatic insufficiency with concomitant malabsorption of nutrients, chronic sinusitis, liv...