Cystic fibrosis

• Published in: Current opinion in infectious diseases Vol. 15; no. 2; p. 175
• Main Authors: Brennan, Amanda L, Geddes, Duncan M
• Format: Journal Article
• Language: English
• Published: United States 01.04.2002
• Subjects: Anti-Bacterial Agents - administration & dosage; Anti-Bacterial Agents - therapeutic use; Anti-Inflammatory Agents - administration & dosage; Anti-Inflammatory Agents - therapeutic use; Cystic Fibrosis - genetics; Cystic Fibrosis - microbiology; Cystic Fibrosis - physiopathology; Cystic Fibrosis - therapy; Drainage, Postural; Gram-Negative Bacterial Infections - complications; Gram-Negative Bacterial Infections - drug therapy; Humans; Mutation - genetics; Staphylococcal Infections - complications; Staphylococcal Infections - drug therapy
• ISSN: 0951-7375
• DOI: 10.1097/00001432-200204000-00013

Cystic fibrosis is the most common lethal inherited disorder with autosomal recessive inheritance. Major progress has been made in understanding the molecular mechanisms leading to increased susceptibility to Pseudomonas aeruginosa colonization. Persistent respiratory infection with P. aeruginosa leads to progressive pulmonary inflammation and is the major cause of morbidity and mortality. Treatment and prophylaxis of respiratory infection has improved the median survival and quality of life of cystic fibrosis patients. In the future, treatment of the underlying genetic defect may be possible.