Pathology of cystic fibrosis review of the literature and comparison with 146 autopsied cases.

This review of pathologic findings in cystic fibrosis includes a survey of 
morphologic changes from the original descriptions of CF and the numerous 
subsequent reports, and an analysis of 146 cases autopsied at the 
Johns Hopkins Hospital and Billings Hospital. Achylia occurs in 85 to 
90% of patients. Approximately 1 of 7 patients with CF presents 
with meconium ileus. In 1962 we described gallbladder abnormalities in 24 
of 72 cases of CF (33%). In Anderson's original description of 
CF, hemosiderosis was present in nearly every liver, and fatty infiltration 
was a frequent finding. The respiratory tract is invariably involved in 
CF. The heart has never been considered a primary target organ 
in CF. Bodian described a higher incidence of nephrocalcinosis in CF 
than in routine pediatric autopsies (31% compared to 11%). With the 
increased longevity of patients with CF male sterility has become a 
recognized complication. Increased electrolyte concentrations in sweat are the most diagnostic 
manifestation of CF, although an occasional patient has escaped this defect. 
Nasal polyps are frequent and often recurrent problems in patients with 
CF. Malabsorption due to pancreatic exocrine deficiency was originally described as 
the main manifestation of CF. The autopsy diagnosis of CF is 
usually based on a combination of lesions rather than on the 
changes in a single organ. 