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The progressive Zollinger-Ellison syndrome in multiple endocrine neoplasia.
Surg Gynecol Obstet. 1988 Nov; 167(5):415-9.SG

Abstract

The problem of hypergastrinemia in patients with the syndrome of multiple endocrine neoplasia (MEN type 1) has become increasingly controversial since the introduction of the antisecretory H2 antagonists for the treatment of the Zollinger-Ellison syndrome (ZES). One of the questions in the management of ZES is whether the malignant potential of the gastrinomas or the recurrent complications of ulcer will be the ultimate cause of death. Another problem is whether the association of MEN is a favorable or unfavorable factor to the prognosis of patients with ZES. Hypercalcemia contributes to excessive gastrin secretion, thereby casting doubt on the diagnosis of ZES in the presence of MEN. Patients with MEN are also more likely than patients with ZES not to have gastrinoma on an exploratory laparotomy. The existing controversy concerns the choices of therapy in instances of progressive ZES associated with MEN or when there is a failure to detect a primary gastrinoma when exploration is performed. To highlight the debate, the clinical courses of two patients, observed during two and three decades, respectively, are presented. These patients were receiving high doses of cimetidine; in addition, conventional surgical treatment for ulcer and repeated biopsies for gastrinoma were performed. After total gastrectomies (as life saving procedures), both patients are well.

Authors+Show Affiliations

Department of Surgery A, Hadassah University Hospital, Hebrew University, Hadassah Medical School, Jerusalem, Israel.No affiliation info available

Pub Type(s)

Case Reports
Comparative Study
Journal Article

Language

eng

PubMed ID

2902696

Citation

Luttwak, E M., and N J. Saltz. "The Progressive Zollinger-Ellison Syndrome in Multiple Endocrine Neoplasia." Surgery, Gynecology & Obstetrics, vol. 167, no. 5, 1988, pp. 415-9.
Luttwak EM, Saltz NJ. The progressive Zollinger-Ellison syndrome in multiple endocrine neoplasia. Surg Gynecol Obstet. 1988;167(5):415-9.
Luttwak, E. M., & Saltz, N. J. (1988). The progressive Zollinger-Ellison syndrome in multiple endocrine neoplasia. Surgery, Gynecology & Obstetrics, 167(5), 415-9.
Luttwak EM, Saltz NJ. The Progressive Zollinger-Ellison Syndrome in Multiple Endocrine Neoplasia. Surg Gynecol Obstet. 1988;167(5):415-9. PubMed PMID: 2902696.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The progressive Zollinger-Ellison syndrome in multiple endocrine neoplasia. AU - Luttwak,E M, AU - Saltz,N J, PY - 1988/11/1/pubmed PY - 1988/11/1/medline PY - 1988/11/1/entrez SP - 415 EP - 9 JF - Surgery, gynecology & obstetrics JO - Surg Gynecol Obstet VL - 167 IS - 5 N2 - The problem of hypergastrinemia in patients with the syndrome of multiple endocrine neoplasia (MEN type 1) has become increasingly controversial since the introduction of the antisecretory H2 antagonists for the treatment of the Zollinger-Ellison syndrome (ZES). One of the questions in the management of ZES is whether the malignant potential of the gastrinomas or the recurrent complications of ulcer will be the ultimate cause of death. Another problem is whether the association of MEN is a favorable or unfavorable factor to the prognosis of patients with ZES. Hypercalcemia contributes to excessive gastrin secretion, thereby casting doubt on the diagnosis of ZES in the presence of MEN. Patients with MEN are also more likely than patients with ZES not to have gastrinoma on an exploratory laparotomy. The existing controversy concerns the choices of therapy in instances of progressive ZES associated with MEN or when there is a failure to detect a primary gastrinoma when exploration is performed. To highlight the debate, the clinical courses of two patients, observed during two and three decades, respectively, are presented. These patients were receiving high doses of cimetidine; in addition, conventional surgical treatment for ulcer and repeated biopsies for gastrinoma were performed. After total gastrectomies (as life saving procedures), both patients are well. SN - 0039-6087 UR - https://www.unboundmedicine.com/medline/citation/2902696/The_progressive_Zollinger_Ellison_syndrome_in_multiple_endocrine_neoplasia_ L2 - http://www.diseaseinfosearch.org/result/7600 DB - PRIME DP - Unbound Medicine ER -