Abstract

The abdominal sweat content of chloride, sodium and potassium was found to be markedly-increased in fifty patients with fibrocystic disease of the pancreas as compared with sixty control cases representing a variety of other diseases. The rate of sweating was not significantly different in the two groups. The abnormal increase in sweat electrolyte levels in patients with cystic fibrosis of the pancreas was shown not to be secondary to pancreatic deficiency or to chronic pulmonary disease. Renal and adrenal function in respect to sodium chloride was found to be normal in fibrocystic patients studied under rigidly controlled metabolic conditions. The defect must therefore be in the sweat glands themselves. In hot weather the abnormally high electrolyte levels in the sweat lead at times to massive salt depletion followed by peripheral vascular collapse, hyperpyrexia, coma and death. This sequence of events can be avoided by timely intravenous administration of physiologic saline solution. Several family members of known patients with cystic fibrosis of the pancreas were found to have sweat electrolyte abnormalities similar to those described in the patients. At times these were associated with generalized obstructive emphysema, without demonstrable pancreatic involvement. This leads to speculation as to the existence of incomplete forms of the disease and their relation to certain types of chronic pulmonary disease. While the pancreatic lesions were the first to attract attention to cystic fibrosis of the pancreas, this disorder is in reality a generalized disease in which many and perhaps all exocrine glands are affected.

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