Management of patients with Zollinger-Ellison syn drome differs markedly from management of patients with idiopathic gastric acid hypersecretion or routine peptic ulcer disease. Because of the possible complica tions arising from gastric acid hypersecretion or from complications caused by the gastrinoma itself, patients with these disorders frequently present to critical care physicians. It is important that critical care physicians be familiar with the acute presentation of patients with Zollinger-Ellison syndrome as well as the appropriate treatment. We review the important points pertaining to the recognition and treatment of Zollinger-Ellison syn drome in the acute care setting.
Get full access to this article
View all access options for this article.
References
1.
Zollinger RM , Ellison EHPrimary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg1955;142:709-728
2.
Gregory RA, Tracy HJ, French JM, Sircus W.Extraction of gastrin-like substance from pancreatic tumour in case of Zollinger-Ellison syndrome. Lancet1960 ;1:1045—1049
3.
Wolfe MM, Jensen RTZollinger-Ellison syndrome. N Engl J Med1987 ;317:1200-1209
4.
Jensen RT, Gardner JD, Raufman JP, et al. Zollinger-Ellison syndrome: current concepts and management . Ann Intern Med1983;98:59-75
5.
Collen MJ, Howard JM, McArthur KE, et al. Comparison of ranitidine and cimetidine in the treatment of gastric hypersecretion. Ann Intern Med1984 ;100:52-58
6.
Jensen RT, Collen MJ, McArthur KE, et al. Comparison of the effectiveness of ranitidine and cimetidine in inhibiting acid secretion in patients with gastric hypersecretory states . Am j Med1984;77:90—105
7.
Howard JM, Chremos AN, Collen MJ, et al. Famotidine, a new, potent, long-acting histamine H2-receptor antagonist: comparison with cimetidine and ranitidine in the treatment of Zollinger-Ellison syndrome. Gastroenterology1985; 88:1026-1033
8.
Vinayek R., Howard JM, Maton PN, et al. Famotidine in the therapy of gastric hypersecretory states. Am J Med1986; 81:(suppl 4B):49-59
9.
Ellison EH, Wilson SDThe Zollinger-Ellison syndrome: reappraisal and evaluation of 260 registered cases. Ann Surg1964;160:512—530
10.
Stage JG, Stadil R.The clinical diagnosis of the Zollinger-Ellison syndrome. Scand J Gastroenterol1979;14:(suppl 53):79—91
11.
Texter EC, Navab F., Mantell G., Berman R Maintenance therapy of duodenal ulcer with famotidine. Am J Med1986;81:25—32
12.
Welch EC, Rodkey GV, Gryska PRA thousand operations for ulcer disease. Ann Surg1986;204:454—457
13.
Richter JE, Pandol SJ, Castell DO, McCarthy DMGastroesophageal reflux disease in Zollinger-Ellison syndrome. Ann Intern Med1981;95:37-43
14.
Jensen RT, Doppman JL, Gardner JDGastrinoma. In: Go Vlw, Brooks FP, DiMagno EP, et al, eds. The exocrine pancreas: biology, pathobiology and diseases . New York: Raven, 1986:727-745
15.
Miller LS, Frucht H., Saeed ZA, et al. Esophageal involvement in Zollinger-Ellison syndrome (ZES). Gastroenterology1988;94:A304
16.
Eberle F., Grun R Multiple endocrine neoplasia, Type I (MEN I). Ergeb Inn Med Kinderheilkd1981;46:75-150
17.
Norton JA, Doppman JL, Jensen RTCancer of the endocrine system. In: DeVita VT, Hellman S, Rosenberg SA, eds. Cancer: principles and practice of oncology , ed 3. Philadelphia: Lippincott, 1989:1269-1344
Shimoda SS, Saunders DR, Rubin CEThe Zollinger-Ellison syndrome with steatorrhea: mechanisms of fat and B 12 malabsorption. Gastroenterology1968 ;55:705-723
20.
Legerton CWDuodenal and gastric ulcer healing rates: a review. Am J Gastroenterol1981;76:500-504
21.
Brodgen RN, Carmine AA, Heel RC, et al. Ranitidine: a review of its pharmacology and therapeutic use in peptic ulcer disease and other allied diseases. Drugs1982;24:267-303
22.
Brodgen RN, Heel TC, Speight TM, Avery GSCimetidine: a review of its pharmacology and therapeutic efficacy in peptic ulcer disease. Drugs1982;15:93-131
23.
Campoli-Richards DM, Clissold SPFamotidine: pharmacodynamics and pharmacokinetics properties and a preliminary review of its therapeutic use in peptic ulcer disease and Zollinger-Ellison syndrome. Drugs1986;32:197—221
24.
Collen MJ, Stanezak CA, Ciarleglio CABasal acid output in patients with refractory duodenal ulcer disease . Dig Dis Sci1987;A92:1351
25.
Jensen RT, Gardner JD, Gastrinoma. In: Bayless TM, Brain MC, Cherniack RM, eds. Current therapy in internal medicine—2. Philadelphia: BC Decker, 1987:626-629
26.
Creutzfeldt W. , Arnold R., Creutzfeldt C., Track NSPathomorphological, biochemical and diagnostic aspects of gastrinomas (Zollinger-Ellison syndrome) . Hum Pathol1975;6:47-76
27.
Thompson JC, Reeder DD, Villar HV, Fender HA Natural history and experience with diagnosis and treatment of the Zollinger-Ellison syndrome. Surg Gynecol Obstet1975; 140:721-739
28.
Deveney CW, Deveney KS, Way LWThe Zollinger-Ellison syndrome: 23 years later. Ann Surg1978;188:384-393
29.
Deveney CW, Deveney KS, Stark D., et al. Resection of gastrinomas. Ann Surg1983;188:384-393
30.
Friesen SRTreatment of the Zollinger-Ellison syndrome. Am J Surg1982;143:331-338
31.
Norton JA, Collen MJ, Gardner JD, et al. Prospective study of gastrinoma localization and resection in patients with Zollinger-Ellison syndrome. Ann Surg1986;204:468-478
32.
Norton JAInvited commentary. World J Surg1984 ;8:572-574
33.
Norton JA, Cornelius MJ, Doppman JL, et al. Effect of parathyroidectomy in patients with hyperparathyroidism and Zollinger-Ellison syndrome and multiple endocrine neoplasia type I: a prospective study. Surgery1987; 102:958—966
34.
Thompson MW , Lloyd RU, Nishiyama RH, et al. MEN-I pancreas: a histological and immunohistochemical study. World J Surg1984 ;8:561-568
35.
von Schrenck T., Howard JM, Doppman JL, et al. Prospective study of chemotherapy in patients with metastatic gastrinoma. Gastroenterology1988;94:1326—1334
36.
Baron JHClinical tests of gastric acid secretion. New York : Oxford University Press, 1979 :13-18
Isenberg JI , Walsh JH, Grossman MIZollinger-Ellison syndrome . Gastroenterology1973;65:140-165
39.
Raufman J-P. , Collins SM, Pandol SJ, et al. Reliability of symptoms in assessing control of gastric acid secretion in patients with Zollinger-Ellison syndrome . Gastroenterology1983;84:108—113
40.
McCarthy DMReport on the United States experience with cimetidine in Zollinger-Ellison syndrome and other hypersecretory states. Gastroenterology1978;74:453-458
41.
Jensen RTBasis for failure of cimetidine in patients with Zollinger-Ellison syndrome . Dig Dis Sci1984;29:363-366
42.
McArthur KE , Collen MJ, Maton PN, et al. Omeprazole: effective, convenient therapy for Zollinger-Ellison syndrome. Gastroenterology1985;88:939-444
43.
Maton PN, Frucht H., Vinayek R., et al. Medical management of patients with Zollinger-Ellison syndrome who have had previous gastric surgery: a prospective study. Gastroenterology1988;94:294-299
44.
Saeed ZA, Norton JA, Frank WO, et al. Parenteral antisecretory drug therapy in patients with Zollinger-Ellison syndrome. Gastroenterology1989;96:1393-1402
45.
Fraker D., Norton JA, Saeed Z., et al. A prospective study of pre- and post-operative control of acid hypersecretion in patients with Zollinger-Ellison syndrome. Surgery1988; 104:1054-1063
46.
Ostro MJ, Russel JA, Soldin SJ, et al. Control of gastric pH with cimetidine: boluses versus primed infusions. Gastroenterology1985;89:532-537
47.
Maton PN, Gardner JD, Jensen RTDiagnosis and management of Zollinger-Ellison syndrome. Endocrine and Metabolic Clinics of NorthAmerica1989 ;18:519-543
48.
Miller LS, Garnder JD, Jensen RTZollinger-Ellison syndrome. In: Collen MJ , Benjamin S, eds. Handbook of experimental pharmacology1990 (in press)
49.
Olbe L., Haglund U., Leth R., et al. Effects of substituted benzimidazole (H 149/94) on gastric acid secretion in humans. Gastroenterology1982 ;83:193-198
Lamers Cbhw , Lind T., Moberg S., et al. Omeprazole in Zollinger-Ellison syndrome: effects of a single dose and of long-term treatment in patients resistant to histamine H2receptor antagonists. N Engl J Med1984;310:758-776
52.
Lloyd-Davies KA, Rutgersson K., Solvell L.Omeprazole in Zollinger-Ellison syndrome: four-year international study. Aliment Pharmacol Therap1988;2:13-37
53.
Maton PN, Vinayek R., Frucht H., et al. Long term efficacy and safety of omeprazole in patients with Zollinger-Ellison syndrome: a prospective study. Gastroenterology1989;97:827-836
54.
Delchier J-C. , Soule J-C., Mignon M., et al. Effectiveness of omeprazole in seven patients with Zollinger-Ellison syndrome resistant to histamine H2-receptor antagonists. Dig Dis Sci1986;31:693-699
55.
Corleto J., Paoti M., Annibale B., et al. Loss of efficacy of famotidine (FMT) in the control of gastric acid secretion in patients with Zollinger-Ellison syndrome (ZES) reversal with omeprazole (OMP). Gastroenterology1988 ;94A79
56.
Hirschowitz BI, Deren J., Raufman JP, et al. A multicenter US study of ompeprazole treatment of Zollinger-Ellison syndrome (ZES). Gastroenterology1988;94 A 188
57.
Lehy T., Mignon M., Cadiot G., et al. Gastric endocrine cell behavior in Zollinger-Ellison patients upon long-term potent antisecretory treatment. Gastroenterology1989; 96:1029-1040
58.
Frucht H., Maton PN, Jensen RTThe use of omeprazole in patients with Zollinger-Ellison syndrome . Dig Dis Sci1990 (in press)
59.
Ekman H., Lansson E., Havu N., et al. Toxicological studies on omeprazole. Scand J Gastroenterol1985;20(suppl 108):53—69
60.
Larson H., Carlsson E., Mattsson H., et al. Plasma gastrin and gastric enterochromaffin cell activation and proliferation— studies with omeprazole and ranitidine in intact and antrectomized rats. Gastroenterology1986 ;90:391-399
61.
Fox PS, Hofmann JW, Wilson SD, DeCosse JJSurgical management of Zollinger-Ellison syndrome. Surg Clin North Am1974;54:395-407
62.
Thompson JC , Lewis BG, Wiener I., Townsend CM Jr. The role of surgery in the Zollinger-Ellison syndrome. Ann Surg1983;197:594—600
63.
Malagelada JR, Edis AJ, Adson MA, et al. Medical and surgical options in the management of patients with gastrinoma. Gastroenterology1983;84:1524-1532
64.
Richardson CT, Peters MN, Feldman M., et al. Treatment of Zollinger-Ellison syndrome with exploratory laparotomy, proximal gastric vagotomy, and H2-receptor antagonists: a prospecive study. Gastroenterology1985;89:357-367
65.
Feldman M., Schiller LR, Walsh JH, et al. Positive intravenous secretin test in patients with achlorhydria-related hypergastrinemia. Gastroenterology1987;93:59-62
Friesen SR, Tomita T.Pseudo-Zollinger-Ellison syndrome: hypergastrinemia, hyperchlorhydria without tumor. Ann Surg1981;194:481-493
68.
Van Heerden JA, Bernatz PE, Rovelstad RAThe retained antrum—clinical considerations . Mayo Clin Proc1971; 46:25-27
69.
Cherner JA, Jensen RT, Dubois A., et al. Gastrointestinal dysfunction in systemic mastocytosis: a prospective study. Gastroenterology1988;95:657-667
70.
Wolfe MM, Jain DK, Edgerton JR Zollinger-Ellison syndrome associated with persistently normal fasting serum gastrin concentrations. Ann Intern Med1985;103:215-217
71.
Frucht H., Howard JM, Slaff JI, et al. Secretin and calcium provocative tests in Zollinger-Ellison syndrome. A prospective study. Ann Intern Med (in press)
72.
McGuigun JE , Wolfe MMSecretin injection test in the diagnosis of gastrinoma. Gastroenterology1980;79:1324-1331
73.
Saeed ZA, Doppman JL, Norton J., et al. Gastrinoma localization in Zollinger-Ellison syndrome. Internal Medicine for the Specialist1988;9:79-99
74.
Ruszniewski P., Mignon M., Rene E., Bonfils S.Localization of tumoral process in Zollinger-Ellison syndrome (ZES). A retrospective study in 76 patients. Gastroenterology1986; 90:1610
75.
Wank SA, Doppman JL, Miller DL, et al. Prospective study of the ability of computerized axial tomography to localize gastrinomas in patients with Zollinger-Ellison syndrome. Gastroenterology1987;92:905-912
76.
Damgaard-Petersen K., Stage JGCT scanning in patients with Zollinger-Ellison syndrome and carcinoid syndrome. Scand J Gastroenterol1979;53:117-122
Roche A., Raisonnier A., Gillon-Savouret MCPancreatic venous sampling and arteriography in localizing insulinomas and gastrinomas: procedure and results in 55 cases. Radiology1982;145:621-627
81.
Andersson T. , Eriksson B., Hemmingsson A., et al. Angiography, computed tomography, magnetic resonance imaging and ultrasonography in detection of liver metastasis from endocrine gastrointestinal tumors. Acta Radiol1987;28:535-539
82.
Frucht H., Doppman JL, Norton JA, et al. MRI imaging of gastrinomas: comparison with computed tomography, angiography and ultrasound. Radiology1989 ;171:713-717
83.
Glowniak JV , Shapiro B., Vinnik AI, et al. Percutaneous transhepatic venous sampling of gastrin. N Engl J Med1982;307:293—297
84.
Burcharth F. , Stage JG, Stadil F., et al. Localization of gastrinoma by transhepatic portal catheterization and gastrin assay. Gastroenterology1979;77:444—450
85.
Cherner JA, Doppman JL, Norton JA, et al. Prospective assessment of selective venous sampling for gastrin to localize gastrinomas. Ann Intern Med1986 ;105:841—847
86.
Charboneau WJ, James EM, Van Heerden JA, et al. Intraoperative real-time ultrasonographic localization of pancreatic insulinomas. J Ultrasound Med1983;2:251-255
87.
Cromack DT, Norton JA, Sigel B., et al. The use of high-resolution intraoperative ultrasound to localize gastrinomas: an initial report of a prospective study. World J Surg1987;11:648—653
88.
Norton JA, Cromack DT, Shawker TH, et al. Intraoperative ultrasonographic localization of islet cell tumors: a prospective comparison to palpation. Ann Surg1988;207:160—168
89.
Sigel B., Machi J., Ramos JR, et al. The role of imaging ultrasound during pancreatic surgery. Ann Surg1984;200: 486-493
90.
Maton PN, Miller DL, Doppman JLThe role of selective angiography in the management of patients with Zollinger-Ellison syndrome. Gastroenterology1987;92:913-918
91.
Norton JA, Sugarbaker PH, Doppman JL, et al. Aggressive resection of metastatic disease in selected patients with malignant gastrinoma. Ann Surg1986;203:352-359
92.
Sheppard BC , Norton JA, Doppmann JL, et al. Management of islet cell tumors in patients with multiple-endocrine-neoplasia: a prospective study. Surgery1989 (in press)
93.
Moertel CG, Hanley JA, Johnson LAStreptozotocin alone compared with streptozotocin plus fluorouracil in the treatment of advanced islet-cell carcinoma. N Engl J Med1980;303:1189—1192
94.
Shepherd JJ , Senator GBRegression of liver metastases in patients with gastrin-secreting tumor treated with SMS 201-995. Lancet1986;2:574
95.
Maton PN, Camilleri M., Griffin G., et al. Role of hepatic arterial embolization in the carcinoid syndrome . Br Med J1983;287:932—935
96.
Ericksson B. , Oberg K., Alm G., et al. Treatment of malignant endocrine pancreatic tumors with human leucocyte interferon. Lancet1986;2:1307-1308
97.
Carrasco CH , Chuang VP, Wallace S.Apudoma metastatic to the liver: treatmeant by hepatic artery embolization. Radiology1983;149:79-83
98.
Bonfils S., Ruszniewski P., Laucouret H., et al. Long-term management of Zollinger-Ellison syndrome with SMS 201-995, a long acting somatostatin analogue. Can J Physiol Pharmacol1986;64:63
99.
Maton PN, Gardner JD, Jensen RTUse of the long-acting somatostatin analogue, SMS 201-995, in patients with pancreatic islet tumors. Dig Dis Sci1989;34:28S-39S
