24 hr 
- creatinine clearance, total protein 
Sterility evaluation 
- semen analysis (males) ; ultrasonographic 
evaluation of pelvis (woman) 
EKG 
- electrocardiogram 
Roentgenographic 
Chest X-ray 
- posterior-anterior, lateral (Brasfield 
semiquantitative scoring) (Brasfield et al . , 1980) 
Chest CT 
chest computed tomography (Bhalla semiquantitative 
scoring) (Bhalla et al . , 1991) 
Scintigraphic (V/Q) 
133 Xe ventilation scan " m Tc-rnacroaggregated albumin 
perfusion scan 
Lung function 
Spirometry 
- forced vital capacity, forced expiratory volume in 
1 sec, forced expiratory flow 25-75% 
He dilution, DLCO 
Helium dilution (total lung capacity, 
functional residual capacity, residual volume) , 
diffusing capacity (single breath) 
Body box 
- body plethysmography (total lung capacity, airway 
resistance) 
ABG 
- arterial blood gases (p0 2 , pC0 2 , pH, saturation) 
Sputum 
Volume 
- 17 hr, 7 am to 12 pm 
Culture 
- routine and quantitative bacterial culture 
Bronchoalveolar lavage 
- cell number, type, culture (routine and quanti- 
tative bacterial culture) , volume of epithelial 
lining fluid (urea method) , mediators (neutrophil 
elastase, interleukin 8, tumor necrosis factor) 
Nasal brush 
- cell number, type, culture (routine and quantita- 
tive bacterial culture, virus culture), biologic 
efficacy [expression of normal CFTR genotype at 
mRNA level (sequencing and PCR with specific 
probes); level of total CFTR mRNA (quantitative 
PCR. Northern, in situ): secretion of Cl" in 
response to intracellular elevations of cAMP <' 36 C1“ 
Recombinant DNA Research, Volume 16 
[745] 
