Cystic Fibrosis Patients Age Research Articles

Anaerobic metabolism and quorum sensing by Pseudomonas aeruginosa biofilms in chronically infected cystic fibrosis airways: rethinking antibiotic treatment strategies and drug targets

Recent evidence indicates that Pseudomonas aeruginosa residing as biofilms in airway mucus of cystic fibrosis (CF) patients is undergoing anaerobic metabolism, a form of growth requiring gene products that are not utilized during aerobic growth. The outer membrane protein, OprF, and the rhl quorum sensing circuit are two previously unrecognized cellular factors that are required for optimal anaerobic biofilm viability. Without OprF, bacteria grow extremely poorly because they lack nitrite reductase activity while lacking rhlR or rhlI forces bacteria to undergo metabolic suicide by overproduction of nitric oxide. Furthermore, anaerobic growth favors maintenance of the mucoid, alginate-overproducing phenotype. Thus, with increasing age of CF patients, mucoid populations predominate, indicating that anaerobic bacteria reside in the inspissated airway mucus. Because many frontline antibiotics used in the treatment of CF airway disease are either ineffective or show reduced efficacy during anaerobic conditions, we propose development of new drugs to combat anaerobic metabolism by P. aeruginosa for more effective treatment of chronic CF lung infections.

PROGNOSIS FOR SURVIVAL WITH CYSTIC FIBROSIS: THE EFFECTS OF EARLY DIAGNOSIS AND CYSTIC FIBROSIS CENTER CARE

Warwick, W.J. (Department of Pediatrics, University of Minnesota Hospitals, Minneapolis, Minnesota, 55455, U.S.A.) Prognosis for survival with cystic fibrosis: the effects of early diagnosis and cystic fibrosis center care. Acta Paediatr Scand 1982; suppl 301: 27‐31. — Improved diagnosis, i.e. more cystic fibrosis (CF) patients diagnosed at an early age, is possible but will require a great increase in the sensitivity of physicians, providing care for infants and young children, to the possibility that early symptoms could be CF at a time a baby is “too well to have CF” coupled with a simple fast screening type of sweat test. Prognosis for survival for CF patients treated at CF Centers is improved with early diagnosis but the data concerning newborn screening are still too few. The general care of CF patients around the world in the absence of a CF Center shows expected survival of 50 percent to 4 or 5 years of age; addition of a CF Center and a CF Care Team increases that 50 percent survival to 20 years. Improvement in early diagnosis or newborn screening is needed to reduce the number of CF patients who die undiagnosed. CF Center and CF team care increases the survival age of CF patients by 4 to 6 times. Early diagnosis before pulmonary complications have begun more than double the effectiveness of CF Center care. In Denmark, the Paediatric Department TG provides the leadership and training and clinical care and research needed to maintain progress and to improve upon the prognosis for CF. Data from Denmark obtained through the central Danish CF Registry confirm in Denmark these observations from around the world of the benefits of early diagnosis and center treatment.Improvement in early diagnosis or discovery of and general adoption of a newborn screening program is what is needed to reduce the number of CF patients who die undiagnosed. CF Center and CF Team care increases the survival age of CF patients by 4 to 6 times. Early diagnosis before pulmonary complications have begun more than double the effectiveness of CF Center care. In Denmark, the Paediatric Department TG provides the leadership and training and clinical care and research needed to maintain progress and to improve upon the prognosis for CF.