Sweat electrolytes in familial pulmonary disease.

The etiology of chronic obstructive pulmonary emphysema is frequently uncertain, and the known laboratory procedures leading to a specmc diagnosis are often unrevealing. In this paper, chronic obstructive pulmonary emphysema will be discussed with reference to sweat electrolytes and the disease entity, cystic fibrosis of the pancreas. Pediatric literature frequently associates chronic obstructive pulmonary emphysema with classic cystic fibrosis of the pancreas. di Sant' Agnese and -workers,' in 1953, revealed that abnormally high concentrations of sweat chloride and sodium were constantly associated with cystic fibrods of the pancreas. At that time, the classic syndrome with di Sant'Agnese's findings included a strong familial incidence, pancreatic insufficiency with intestinal rnalabsorption, obstructive pulmonary disease, increase of sweat electrolytes, stunted growth, and early death. Recently, however, several cases of chronic pulmonary disease with a familial incidence and increased sweat electrolytes, but without pancreatic dysfunction, have been recorded as "cystic fibrosis of the pancreas.'These authors have chosen to accept this diagnosis on the basis of abnormal values for sweat electrolytes and the familial incidence of pulmonary disease. Recently, we encountered an adult patient with severe chronic obstructive pulmonary emphysema of unknown cause. Investigation revealed several family members with chronic pulmonary disease. The findings are reported herein.