Abstract

The discovery of the cystic fibrosis gene has provided new approaches to gene therapy and drug therapy. At the present time, however, we are still faced with the morbidity and mortality due to lung infections caused by Pseudomonas aeruginosa and Pseudomonas cepacia. The present survey of some of the recently published articles shows that applied science related to the clinical care of cystic fibrosis (CF) patients is also progressing. Cross-infection in CF centers and during social contacts between CF patients out of the hospital environment has been described in some countries. Prophylactically, cohort isolation procedures are effective. The individual clinical course of the chronic pulmonary infection is adversely influenced during prediabetic mellitus periods and by development of high titers of specific IgG2 and IgG3 antibodies against P. aeruginosa. A vaccine against P. aeruginosa is currently being tested in CF patients. The induced antibodies have much higher affinity and opsonophagocytic activity than infection-induced antibodies. Ciprofloxacin, which is widely used in CF, easily gives rise to resistance in P. aeruginosa and Staphylococcus aureus. Restricting the use of this drug in CF is therefore necessary. Aerosolized recombinant DNase is a promising and efficient new drug for management of airflow limitation due to purulent airway secretion in CF. For end-stage CF, double-lung and heart-lung transplantation show encouraging intermediate-term results, the two main obstacles being donor organ shortage and obliterative bronchiolitis.

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