Summary
A detailed study of the altered gastrointestinal physiology and findings at autopsy in a patient with Zollinger-Ellison syndrome is presented. The results warrant certain considerations with regard to mechanisms involved in the altered physiology.
The importance of the discovery by Gregoryet al. of a gastrinlike substance in the pancreatic tumor of such a patient is obvious in relation to the excessive gastric secretion which was first emphasized in this syndrome. Our results indicate that undue vagal activity may also play a role, in some cases at least.
Data are presented which indicate that the parietal cell mass in some of these patients may be maximally or nearly maximally stimulated at all times.
A rather remarkable effect of adequate anticholinergic and ulcer therapy on the control of the gastric secretion, diarrhea, and ulcer healing is recorded.
It is our belief that the characteristic features of this disease, the fulminant peptic ulcer diathesis, the watery diarrhea with or without excessive potassium loss, and steatorrhea can all be related to the inordinate increase of gastric secretion; that the non-B cell tumor or islet hyperplasia must be very important in the gastric hypersecretion; and that vagal activity can also exert an important influence on this secretion (demonstrated in the case reported above).
Finally, the nonfunctioning parathyroid adenoma found microscopically was of no importance in the patients clinical picture.
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References
Zollincer, R. M., andEllison, E. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas.Ann. Surg. 142: 709, 1955.
Sun, David, C. H., andShay, H. Optimal effective dose of anticholinergic drug in peptic ulcer therapy.A.M.A. Arch. Int. Med. 97:442, 1956.
Marks, I. N., andShay, H. Augmented histamine test, Ewald test meal and Diagnex test.Am. J. Dig. Dis. (New Series) 5:1, 1960.
Shay, H., andSun, David C. H. Stress and Gastric secretion in man. I. A study of the mechanism involved in insulin hypoglycemia.Am. J. Med. Sci. 228:6, 1954.
Kay, A. W. Effect of large doses of histamine on gastric secretion of HCl.Brit. Med. J. 2:77, 1953.
Shay, H., Sun, David C. H., Chey, W. Y., andO'Leary, D. K. Practical pancreatic function profile for the diagnosis of chronic pancreatic disease.Am. J. Dig. Dis. (New Series) 6:142, 1961.
Zollinger, R. M., andCraig, T. V. Ulcerogenic tumor of the pancreas.Am. J. Surg. 99:424, 1960.
Maximov, A. A., andBloom, W.A Textbook of Histology (ed. 5), p. 449, Philadelphia, Saunders, 1948.
Gregory, R. A., Tracy, H. J., French, I. M., andSircus, W. Extraction of a gastrin-like substance from a pancreatic tumor in a case of Zollinger-Ellison syndrome.Lancet 1:644, 1956.
Gregory, R. A., andTracy, H. J. The preparation and properties of gastrin.J. Physiol. 149:70, 1959.
Hallenbeck, G. A., Code, C. F., andKennedy, J. C. “Gastrin” of Pancreatic Origin. Sixty-second Annual Meeting of the American Gastroenterological Association. Chicago, May 27, 1961.
Zollinger, R. M., andMcPherson, R. C. Ulcerogenic tumors of the pancreas.Am. J. Surg. 95:359, 1958.
Summerskill, W. H. Malabsorption and jejunal ulceration due to gastric hyper-secretion with pancreatic islet cell hyperplasia.Lancet 1:120, 1959.
Elman, R., andHartmann, A. F. Spontaneous peptic ulcers of duodenum after continued loss of total pancreatic juice.A. M. A. Arch. Surg. 23: 1030, 1930.
Dragstedt, L. R., Clark, D. F., andVermeulen, C. Significance of lipocaic in surgery.Ann. Surg. 110:907, 1939.
Cox, A. J., andBarnes, V. R. Experimental hyperplasia of the stomach mucosa.Proc. Soc. Exp. Biol. & Med. 60:118, 1945.
Meyers, W. C. A study of gastric mucosa in various diseases affecting the upper part of the gastro-intestinal tract.Gastroenterology 10:923, 1948.
Cox, A. J. Stomach size and its relation to chronic peptic ulcer.A. M. A. Arch. Path. 54: 407, 1952.
Card, W. I., andMarks, I. N. The relationship between the acid output of the stomach following “maximal” histamine stimulation and the parietal cell mass.Clin. Sci. 19:147, 1960.
Illingworth, C. F. W. Inborn and extraneous factors in the etiology of peptic ulcer.J. Roy. Coll. Surg. Edinburgh 2:14, 1956.
Rudolf, L. E., Dammin, G. F., andMoore, F. D. Intractable peptic ulcer and endocrine adenomas with pituitary amphophilic hyperplasia.Surgery 48:170, 1960.
Verner, J. V., andMorrison, A. B. Islet cell tumor and a syndrome of refractory diarrhoea and hypokalemia.Am. J. Med. 25:374, 1958.
Donaldson, R. M., Jr., Vom Eigen, P. R., Dwight, R. M. Gastric hypersecretion, peptic ulceration and islet-cell tumor of the pancreas (the Zollinger-Ellison Syndrome). Report of case and review of literature.New Engl. J. Med. 257:965, 1957.
Telling, M., andSmiddy, F. G. Islet tumours of the pancreas with intractable diarrhoea.Gut, 2:12, 1961.
Shay, H. Etiology of peptic ulcer.Am. J. Dig. Dis. (New Series 6:29, 1961.
Shay, H. The pathologic physiology of gastric and duodenal ulcer.Bull. N. Y. Acad. Med. 20: 264, 1944 (2nd Series).
Brown, C. H., Neville, W. E., andHazard, J. B. Islet-cell adenoma, without hypoglycemia, causing duodenal obstruction.Surgery 27:616, 1950.
Maynard, E. P., andPoint, W. W. Steatorrhea associated wi 3th ulcerogenic tumor of the pancreas.Am. J. Med. 25:456, 1958.
Borgström, B., Dahlqvist, G. L., andSjövall, J. Studies of intestinal digestion and absorption in the huma.J. Clin. Invest. 6:1521, 1957.
Isselbacher, Kurt J. Discussion Panel—Newer Concepts of gastrointestinal absorption. Fats. Sixty-second Annual Meeting American Gastrointestinal Association. May 26, 1961, Chicago, Illinois.
Chey, W. Y., Shay, H., andO'Luary, D. K. Role of pancreas in the intestinal absorption of fat in man. To be reported.
Rawson, A. B., England, M. T., Gillam, G. G., French, J. M., andStammers, F. A. R. Zollinger-Ellison Syndrome with diarrhea and malabsorption.Lancet 1:131, 1960.
Friedell, H. V., Dickson, D. R., andRydell, J. R. Ulcerogenic tumor of the pancreas.Am. J. Gastroent. 31:58, 1959.
Warren, S., andJoslin, F. P. Pathology of Diabetes Mellitus. p. 25. Philadelphia, Lea and Febigar, 1938.
Bensley, R. R. Structure and relationships of the Islets of Langerhans.Harvey Lect. 10:250, 1914–15.
Barbosa, J. J., Dockerty, M. B., andWaugh, J. M. Pancreatic heterotopia.Surg. Gynec. & Obstet. 82:527, 1946.
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Shay, H., Chey, W.Y., Koide, S.M. et al. Mechanism of the disordered physiology involved in the Zollinger-Ellison syndrome. Digest Dis Sci 7, 401–419 (1962). https://doi.org/10.1007/BF02232360
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DOI: https://doi.org/10.1007/BF02232360