Cystic fibrosis is recognized as a generalized exocrinopathy involving the pancreas, lung, duodenum and salivary glands as well as the eccrine sweat glands. An understanding of the pathogenesis of the exocrine glands is important to identify early changes in this genetic disorder. Based on biochemical evaluations, approximately 85% of patients with cystic fibrosis have severe pancreatic disease (1, 2). Although the remaining subjects lack evidence of steatorrhoea, it is not clear whether the pancreas is morphologically or functionally normal.

The characteristic pathologic changes recognized at autopsy in the cystic fibrosis pancreas include achylia, inspissation of secretions in the ducts and acini, and increase in fibrous connective tissue. The pancreatic lesions have been reported to vary in severity, particularly in the neonatal period. Investigation of the exocrine pancreatic changes, in utero and in the neonatal period are important to understanding the pathogenesis of cystic fibrosis.