Fibrocystic disease of the pancreas and diabetes in an adult with unusual pulmonary manifestations.

CYSTIC FIBROSIS OF THE PANCREAS is predominantly a disease of infancy. Although the cause is still unknown, much progress has been made toward a better understanding of the disease since the first complete description in this country by Anderson1 in 1938. Several years later, when the widespread involvement of all the mucus-producing exocrine glands in the disease was described, the term mucoviscidosis was suggested because of the thickening of the mucus which occurs. More recently di Sant 'Agnese5 pointed out that both names are improper because the pancreas is usually but not always involved and the disease is not confined to the mucus-producing exocrine glands. In the light of present knowledge, 'he suggested the term generalized exocrinopathy as being more accurate from a descriptive point of view. In the American and English literature there are few references to cystic fibrosis of the pancreas in adults. In 1955 di Sant 'Agnese5 reported his findings in 325 patients he observed over a 15-year period in Babies Hospital, Columbia University. The oldest patient in the series was 19 years of age. In 1946, Hellerstein8 reported a case in a 35-year-old Negro male who died 12 hours after admission to a hospital with Friedlander's pneumonia; there was no antecedent history and the diagnosis of cystic fibrosis of the pancreas was based upon the incidental observation at necropsy of numerous cysts and diffuse fibrosis in the pancreas. Hellerstein9 recently called attention to the fact that many cases of cystic fibrosis of the pancreas in adults were reported in the German literature and cited a reference to the adult cases in The System of Pathology by Henke-Lubaesch. In 1954 Baxter2 reported experience with the disease at Ohio State University and stated that before antibiotics the life span of patients was measured in months or a very few years, perhaps only one or two. He indicated that with proper management some patients may live until they are 18 to 22 years of age. This obvious disparity in survival ages reported by previous German and present-day observers leads one to wonder whether the German adult cases reported might not be attributable to cystic fibrosis following pancreatitis. Di Sant 'Agnese5 has proposed the following four criteria for present-day clinical diagnosis of cystic fibrosis of the pancreas: 1. Pancreatic deficiency. 2. Pulmonary pathologic changes 3. Excess chlorides in sweat. 4. Family history (assists only). These criteria are based on his observations that there is complete absence of pancreatic exocrine function in 90 per cent of the patients; pulmonary pathologic change occurs in all patients at some time in the disease; and finally, abnormal sweat with increased chloride and/or sodium content occurs in 99 per cent of patients. Absence of the disease in siblings does not rule out the disease in the patient. In reviewing the literature, we were unable to find a single reference to cystic fibrosis of the pancreas and associated or co-existing disease since 1938. In 1950 Molnar" reviewed the literature up to that time in a case report and made no reference to associated diabetes. In 1955 di Sant 'Agnese6 presented an excellent and comprehensive review of the pulmonary manifestations of fibrocystic disease of the pancreas, one of the unusual aspects of the case herein presented. He said that respiratory involvement has a variable onset from the age of a few weeks to several years, occurring between six months and two years of age in the great majority of cases. It must be recognized, he pointed out, that this is in reality a generalized disease of which the pancreatic and pulmonary lesions are only one expression. The 292 patients reported upon had, at some time during their illness, pulmonary disease of variable degree -severe in most instances and accounting for 90 per cent of the deaths. He made the observation that if patients reached late childhood without irreversible damage being done to their lungs, they seemed to improve and the degree of clearing in the roentgen picture was surprising. With regard to prognosis in this group, he suspected that at least many would go on to chronic pulmonary disease as adults. The oldest in the reported group was 18 years of age. The following unusual case of cystic fibrosis of the pancreas is presented because the patient is an adult who has associated diabetes and the pulmonFrom the Department of Pulmonary Diseases, Santa Barbara General Hospital, Santa Barbara. Submitted April 21, 1958.