Clinical, chemical and pathologic data are presented on 9 patients with the generalized disease, "fibrocytic disease of the pancreas," but with normal or partial pancreatic exocrine function.Pancreatic function was evaluated not only by duodenal assay, but by studies of intestinal absorption of fat and nitrogen. Pancreatic function was entirely normal in 3 patients, partially present in 3 others, and in the remaining 3, normal at first, but decreasing to low levels at a later date. Pathologic findings in the pancreas at autopsy in 1 case are presented and the histologic appearance of a biopsy specimen of this organ is described in another.It is emphasized that the nutritional symptoms of the disease are secondary to pancreatic deficiency and may be absent if this gland is functioning normally. No evidence is found for a close relation between pancreatic function and pulmonary involvement.The data reported in this paper strongly support the view that partial or even normal pancreatic function may occur in "fibrocystic disease of the pancreas." The diagnostic implications are discussed. It is concluded that finding of tryptic activity in normal concentration in duodenal juice does not negate the diagnosis. Evidence is presented supporting the reliability of the "sweat test," since it may be positive at a time when other findings are not present.In the light of recent knowledge, criteria for the diagnosis of "fibrocystic disease of the pancreas" are restated. The diagnostic importance of the demonstration of an abnormality of sweat electrolytes in association with the chronic pulmonary pathology is stressed, especially if pancreatic function is normal.The use of the stool trypsin test, increased viscosity of duodenal contents and microscopic examination of stool for excess fat as screening tests is discussed. The latter is preferred. In patients having the disease without pancreatic deficiency, excess fecal fat is not present. Nevertheless if the diagnosis is suspected on the basis of the respiratory involvement, a "sweat test" should be done.The conclusion is reached that "fibrocystic disease of the pancreas," is not primarily a disease of the pancreas, but a generalized condition in which this organ is usually, but not necessarily affected. Varying degrees of involvement of the pancreas, lungs, liver and sweat glands are inherent in this disease. Complete and incomplete forms of the condition exist.The basic nature of the disturbance is discussed. There is an accumulating body of evidence that the secretory activity of many and perhaps all exocrine glands, mucus-producing and others, is affected in this disorder. Possible mechanisms of hereditary transmission of the basic defect are considered.A plea is made for retention of the name "cystic fibrosis of the pancreas" or "fibrocystic disease of the pancreas" until a better one is proposed, perhaps after the etiology and pathogenesis of the disease are further clarified. The term "mucoviscidosis," by implying that only mucus-secreting structures are affected, tends to prolong an erroneous concept and introduce another improper term to further confuse an already muddled field.
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