Day 56: Patients will be given intravenous sedation with midazolam and will have repeat 
measurements of nasal PD, nasal brushings and biopsy, tracheal PD, and bronchoscopy for 
BAL, brushings, and biopsy. Following the examination, subjects will recieve the AvlCF2 
vector at the same dose level of their previous dose (10 10 , 10 11 , or 10 12 ) in the bronchus (the 
same site previously treated on day 0) in a total of 20 ml of sterile fluid. At the end of the 
procedure, the trachea will be "painted" with a small amount of the suspension, using small 
volumes and working from peripheral to proximal location. The equivalent lobe on the other 
side will be used as a control. 
Day 59: History and physical examination will be repeated. Chest x-ray, lung scans, oxygen 
saturation and pulmonary function tests will be repeated. Patients will be sedated for 
measurements of tracheal PD. Bronchoscopy will be performed for BAL, brushings, and 
biopsy. Adenovirus culture will be performed on BAL and pharyngeal swabs. Blood will be 
drawn for adenovirus serology, CBC and differential, PT and PTT, and serum chemistry. 
Urinalysis will be repeated. 
Day 63, 70, and 77: . Patients will be kept under observation at weekly intervals. They will 
have weekly history and physical, oxygen saturations, pulmonary function tests, cultures, and 
blood work. Chest x-rays and lung scans will be repeated according to Table III. If the 
culture for the recombinant adenovirus using 293 cells is negative on day 70, patients will be 
discharged following the same protective precautions as were followed after the first 
administration of the vector. 
Day 84: Patients will be readmitted to the clinical research center. They will have a history 
and physical, chest x-ray, oxygen saturation and pulmonary function tests. Following sedation 
they will have measurements of nasal potential difference, nasal brushings and biopsy, tracheal 
potential difference, and bronchoscopy for BAL, brushings and biopsy. BAL, pharyngeal 
cultures, and stool will be sent for adenovirus culture using the permissive cell line. PCR will 
be repeated for wild adenovirus infection. Blood will be obtained for adenovirus serology. 
CBC and differential, PT and PTT, serum chemistry, and urinalysis will be obtained. 
Days 91, 98, and 105: Patients will be seen at weekly intervals for history and physical, 
oxygen saturation and pulmonary function tests, cultures, PCR for wild adenovirus, and blood 
tests as shown in Table III. 
Day 112: This is the final day of the study. Patients will be seen again in the clinical research 
center for history and physical, chest x-ray, lung scans, oxygen saturation, and pulmonary 
function tests. Following intravenous sedation they will have measurements of nasal PD, nasal 
brushing and biopsy, and tracheal PD. Bronchoscopy will then be performed for BAL, 
brushings, and biopsy. Adenovirus culture will be performed with BAL, pharyngeal swabs, 
and stool. PCR for wild adenovirus will be performed. Blood will be obtained for adenovirus 
serology, CBC and differential, PT and PTT, and serum chemistry. Urinalysis will be 
obtained. Sputum culture will be repeated. 
Throughout the experimental protocol we will maintain routine CF care; namely, chest 
physical therapy 3 times per day, pancreatic enzymes, multivitamins, and routine aerosol 
therapy. 
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Recombinant DNA Research, Volume 17 
