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多内分泌腺瘤病中的进行性佐林格-埃利森综合征

The progressive Zollinger-Ellison syndrome in multiple endocrine neoplasia.

作者信息

Luttwak E M, Saltz N J

机构信息

Department of Surgery A, Hadassah University Hospital, Hebrew University, Hadassah Medical School, Jerusalem, Israel.

出版信息

Surg Gynecol Obstet. 1988 Nov;167(5):415-9.

PMID:2902696
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.

摘要

自从引入抗分泌的H2拮抗剂用于治疗卓艾综合征(ZES)以来,多发性内分泌腺瘤病(MEN 1型)患者的高胃泌素血症问题变得越来越有争议。ZES治疗中的一个问题是胃泌素瘤的恶性潜能或溃疡的复发性并发症是否会成为最终的死亡原因。另一个问题是MEN的关联对ZES患者的预后是有利还是不利因素。高钙血症会导致胃泌素分泌过多,从而在存在MEN的情况下对ZES的诊断产生怀疑。与ZES患者相比,MEN患者在探查性剖腹手术时更有可能没有胃泌素瘤。现有的争议涉及与MEN相关的进行性ZES病例或探查时未能检测到原发性胃泌素瘤时的治疗选择。为了突出这场辩论,分别介绍了在二十年和三十年期间观察到的两名患者的临床病程。这些患者接受了高剂量的西咪替丁治疗;此外,还对溃疡进行了常规手术治疗,并对胃泌素瘤进行了反复活检。在进行全胃切除术后(作为挽救生命的手术),两名患者情况良好。

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