Relationship between clinical conditions, chest radiographic findings and respiratory function tests in cystic fibrosis.

We explored 44 children suffering from cystic fibrosis with predominant pulmonary involvement 
and followed in particular the course of illness of 30 of 
these patients during an average period of 4 years. We were 
impressed by the enormous variability of the pulmonary symptomatology in these 
children. The question arose whether this clinical impression is supported by 
the finding of the thorax radiograph and the results of the 
lung functions tests and the blood gas measures at rest and 
on exercise. From the clinical point of view, there is a 
possibility to make a classification of severity in pulmonary aspects of 
this disease. Because of the large ranges of the score values 
a clear distinction of the severity could only be possible if 
large groups of patients were to be compared. For the interindividual 
comparison the cumulative evaluation of a clinical score, the chest radiograph 
and the lung function tests are indispensable. Only the continuous follow-up 
of these examinations allows one to notice, in time, a progressive 
lung involvement with its degenerative changes. 