Pathology of cystic fibrosis. pp. 115-24.

The varied morphologic lesions in cystic fibrosis each result from obstruction of 
one type or another, documented or presumed. They are modified by 
the age of the individual, the severity and the conditions peculiar 
to each tissue. These lesions, plus the functional changes in sweat 
electrolytes, membrane transport and serum factor affecting syncrony of cilia, represent 
the diverse actions of a genetic defect, the intermediate stages of 
which are as yet unknown. Neither the diversity of abnormalities, their 
range in severity, nor differences in the age of onset argue 
convincingly for a trigger mechanism or acquire rather than inborn deficiency. 
