A microbiological analysis of 102 patients suffering from cystic fibrosis was conducted over a 22 month period. 20 microbial species with the following incidence were identified: Pseudomonas aeruginosa: 83.4%; Candida albicans: 29.4%; Staphylococcus aureus: 24.5%; Staphylococcus epidermidis: 11.8%; Haemophilus influenzae: 11.8%; Streptococcus pneumoniae; 6.9%; Pseudomonas maltophilia: 6.8%; Aspergillus fumigatus: 5.9%. Other species were present in less than 5% of the patients. In the majority of specimens with P. aeruginosa, more than one type (up to six) was detectable. These strains were identical in colony appearance, O-serotype and pyocin-type. Quantitative analysis revealed concentrations of colony-forming units of 10(7) to 10(9) for P. aeruginosa, 10(6) to 10(8) for P. maltophilia, 10(4) to 10(7) for S. aureus, 10(4) to 10(6) for S. epidermidis and 10(4) to 10(7) for C. albicans in the majority of specimens. Significant differences were observed in the time periods during which the pathogens persisted in the patients. Maximum persistence was observed for P. aeruginosa. P. maltophilia and A. fumigatus had about similar persistence rates, which were lower than those for P. aeruginosa but above those for S. aureus and H. influenzae. S. epidermidis was eliminated within shorter periods than S. aureus. C. albicans, although the second most frequent microorganism identified, showed a very low persistence rate. The microbiological analysis confirms results from other research centers (high incidence of P. aeruginosa), but reveals significant regional differences as well (Pseudomonas cepacia not detectable, higher incidence of P. maltophilia and C. albicans). This underlines the necessity for detailed qualitative and quantitative microbiological analysis of sputa from cystic fibrosis patients as a prerequisite for rational analysis of etiological, epidemiological and therapeutical aspects of cystic fibrosis.

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Clinical trials using replication-deficient adenovirus as vectors for gene transfer into the airways of cystic fibrosis (CF) patients are in progress. However, little is known about the prevalence of wild-type adenovirus infections in patients with cystic fibrosis and their effect on lung function. To answer these questions, serum IgG and IgM antibody titers against adenovirus type 5 were prospectively measured by an indirect immunofluorescence assay in 199 CF outpatients and in a control group of 45 healthy children and young adults. In addition, we performed pulmonary function tests when the patients were in stable clinical condition. IgM antibodies against adenovirus were present in 104 of the 199 cystic fibrosis patients (52.3%). IgG antibodies against adenovirus were detected in 192 of the 199 cystic fibrosis patients (96.5%), and were significantly higher in cystic fibrosis patients older than 7 years than in younger patients and in age matched controls. IgG antibody titers measured a second time 11.8 months later in 143 of the 199 patients had increased in 48 (33.6%) patients. In 27 of these 48 patients, who had at least a 2-fold increase in antibody titer, FVC and FEV1 decreased by 9.8% (p < 0.05) and 8.3% (p = 0.05), respectively, over 45 months. In a comparison group matched for age, sex, and chronic Pseudomonas aeruginosa infection but no increase in antibody titers, FVC and FEV1 were unchanged. The results indicate that wild-type adenovirus infections are prevalent in cystic fibrosis patients and that wild-type adenovirus infections in cystic fibrosis patients seem to be associated with deterioration in lung function. These observations may have important implications for efficacy and safety considerations when using adenoviral vectors for gene therapy.

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