Zollinger-Ellison syndrome. Current concepts in diagnosis and management.
Zollinger-Ellison syndrome. Current concepts in diagnosis and management.
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佐林格-埃里森综合症。
DOI：
10.1056/nejm198711053171907
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发表时间：
1987
期刊：
影响因子：
--
通讯作者：
R. Jensen
中科院分区：
文献类型：
--
作者：
M. Michael Wolfe;R. Jensen
Zollinger-Ellison综合征虽然不常见，但并不罕见，大多数患者的临床表现与普通消化性溃疡患者相似。早期的研究强调大量胃酸分泌过多的并发症会导致死亡。然而，随着有效的抗分泌剂来控制胃酸分泌的可用性，死亡现在更频繁地与缓慢生长但恶性的胃泌素瘤的转移潜力相关。因此，医生在评估慢性消化性溃疡或不明原因分泌性腹泻患者时应高度怀疑Zollinger-Ellison综合征。对这类患者应进行旨在早期诊断Zollinger-Ellison综合征的评估，并应从测定空腹血清胃泌素水平开始开始。至少有50%的胃泌素瘤患者血清胃泌素浓度不能诊断，因此需要激发性检测以确定正确的诊断。在确定存在综合征后，患者应接受有效的抗分泌剂治疗，剂量足以在下一次给药前1小时内将基础酸排出量降至10 mmol以下。虽然有些病人可以满意地维持这种方式延长时间，一种方法，旨在肿瘤定位和切除，建议在大多数患者。术前评估应开始与静脉造影剂的CT扫描。如果肿瘤的位置和范围仍有疑问，应进行选择性血管造影，偶尔门静脉采血测定胃泌素。如果转移性疾病被证明，或如果MEN-I存在，手术旨在肿瘤切除，虽然它偶尔是有效的，可能会失败。由于胰头切除术的发病率和死亡率相当高，不能切除的胰头肿瘤不宜行胰头切除术。在其他Zollinger-Ellison综合征患者中，应进行探查性手术;这应包括仔细搜索和切除所有胰腺和胰腺外胃泌素瘤。通过这种方法，至少有20%的Zollinger-Ellison综合征患者可以治愈。
The Zollinger-Ellison syndrome, although uncommon, is not rare, and most patients with the disorder present with clinical manifestations similar to those of patients with common peptic ulcer. Early studies emphasized death due to complications of massive gastric acid hypersecretion. However, with the availability of potent antisecretory agents to control acid secretion, death is now more frequently associated with the metastatic potential of slowly growing but malignant gastrinomas. Therefore, physicians should maintain a high degree of suspicion of the Zollinger-Ellison syndrome in assessing patients with either chronic peptic ulcer or unexplained secretory diarrhea. An evaluation aimed at early diagnosis of the Zollinger-Ellison syndrome should be instituted in such patients and should begin with a determination of the fasting serum gastrin level. At least 50 percent of patients with gastrinoma will have nondiagnostic serum gastrin concentrations and will therefore require provocative testing to establish the correct diagnosis. After the presence of the syndrome is established, patients should be treated with a potent antisecretory agent in doses sufficient to reduce basal acid output to less than 10 mmol in the hour preceding administration of the next dose. Although some patients may be maintained satisfactorily in this manner for extended periods, an approach aimed at tumor localization and extirpation is recommended in most patients. Preoperative evaluation should begin with CT scanning with intravenous contrast material. Selective angiography, and occasionally, portal venous sampling for gastrin, should be performed if the location and extent of tumor remain in question. If metastatic disease is demonstrated, or if MEN-I is present, surgery aimed at tumor resection, although it is occasionally effective, will probably be unsuccessful. Because of the considerable morbidity and mortality associated with pancreatoduodenectomy, it should not be performed for unresectable tumor in the head of the pancreas. In other patients with the Zollinger-Ellison syndrome, exploratory surgery should be performed; this should include a careful search for, and resection of, all pancreatic and extrapancreatic gastrinomas. With this approach, it is likely that at least 20 percent of all patients with the Zollinger-Ellison syndrome can be cured.
胃酸缺乏相关的高胃泌素血症患者静脉内促胰液素试验呈阳性。
影响因子：
29.4
作者：
Feldman,M;Schiller,LR;Walsh,JH;Fordtran,JS;Richardson,CT
通讯作者：
Richardson,CT
胃泌素瘤综合征中 NH2 末端胃泌素的评估。
DOI：
10.1210/jcem-62-5-970
发表时间：
1986
期刊：
The Journal of clinical endocrinology and metabolism
影响因子：
--
作者：
Kothary,PC;Fabri,PJ;Gower,W;O'Dorisio,TM;Ellis,J;Vinik,AI
通讯作者：
Vinik,AI
采用剖腹探查术、近端胃迷走神经切断术和 H2 受体拮抗剂治疗 Zollinger-Ellison 综合征。
影响因子：
29.4
作者：
Richardson,CT;Peters,MN;Feldman,M;McClelland,RN;Walsh,JH;Cooper,KA;Willeford,G;Dickerman,RM;Fordtran,JS
通讯作者：
Fordtran,JS