Cystic fibrosis in adults: an autopsy study.

An annotated catalog of clinical and pathologic observations in an adult population 
with CF has been presented. The spectrum of disease is broad, 
and the diagnosis usually requires the demonstration of abnormalities in multiple 
systems. Tissues of each germ layer have been found involved in 
CF. Correlation of clinical and pathologic observations suggests that manifestations are 
essentially episodic and focal. In this population, the most characteristic abnormalities 
recognized by the pathologist are focal biliary cirrhosis, distinctive obstructive lesions 
of the male genital tract, prolonged staphylococcal or pseudomonas colonization of 
respiratory secretions, and obstructive bronchopulmonary disease. Some form of pancreatic atrophy 
is usually present. 