Atypical cystic fibrosis of the pancreas with normal levels of sweat chloride and minimal pancreatic lesions.

This paper presents additional evidence of the variability and independence of the 
involvement of the sweat glands in cystic fibrosis. A case report 
of an infant girl, who died at age 9 years of 
progressive pulmonary disease, is presented. The case indicates that fatal cystic 
fibrosis, or a variant thereof, can exist with normal levels of 
sweat chloride and that the pulmonary disease develops independently of the 
functional abnormality of the sweat glands. 