Zollinger-Ellison syndrome. Diagnosis and therapy.
Zollinger-Ellison syndrome. Diagnosis and therapy.
复制标题
佐林格-埃里森综合症。
作者：
Davide Campana;L. Piscitelli;Elena Mazzotta;M. Bonora;Carla Serra;L. Salomone;Roberto Corinaldesi;Paola Tomassetti
佐林格-埃里森综合征（ZES）以难治性消化性溃疡疾病、严重腹泻和胃酸分泌过多为特征，并伴有胰腺胰岛细胞肿瘤（胃原质瘤）。62-80%的病例散发，20-38%的病例伴有多发性内分泌肿瘤1型（MEN 1）。当完整胃患者血浆胃泌素为> - 1000 pg/mL，基础酸输出为> - 15 mEq/h，胃切除术患者血浆胃泌素输出为> - 5 mEq/h，或当高胃泌素血症伴有pH <2时，诊断为ZES是确定的。治疗的基础是控制胃酸分泌过多和恶性肿瘤及其可能的转移。质子泵抑制剂是最有效的抗分泌药物，可以大剂量施用而没有药物相关的不良反应。如果可能，所有散发的、局部的胃泌素瘤都应切除。当肝转移也存在时，它们的减积可能改善症状和生存，并促进药物治疗。对于与MEN - 1相关的胃鞘瘤的手术方法存在一些争议。生长抑素类似物可用于减少胃酸高分泌、血清胃泌素和胃肠嗜铬细胞，从而有助于更有效地治疗该疾病。它们的抗增殖作用可用于治疗肝转移瘤。化疗和/或干扰素仅适用于恶性进展性疾病的患者。栓塞和化疗栓塞对控制临床症状有效；然而，它们似乎并不能提高生存率。
Zollinger-Ellison syndrome (ZES) is characterised by refractory peptic ulcer disease, severe diarrhoea and gastric acid hypersecretion associated with an islet-cell tumor of the pancreas (gastrinoma). ZES is sporadic in 62-80% of cases and in 20-38% of cases is associated with multiple endocrine neoplasia type 1 (MEN 1). The diagnosis of ZES is certain when the plasma gastrin is >1000 pg/mL and the basal acid output is >15 mEq/h in patients with an intact stomach, >5 mEq/h in gastrectomised patients, or when the hypergastrinemia is associated with a pH <2. Treatment is based on the control of gastric acid hypersecretion and of the malignant tumor and its possible metastases. Proton pump inhibitors are the most effective antisecretory drugs and can be administered at high dosages without drug-related adverse effects. All sporadic, localised gastrinomas should be excised if possible. When liver metastases are also present, their debulking may improve symptoms and survival, and facilitate medical treatment. There is some controversy as to the surgical approach for gastrinomas associated with MEN 1. Somatostatin analogues can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric enterochromaffin-like cells, and can thus contribute to treating the disease more effectively. Their antiproliferative effect can be used in treating liver metastases. Chemotherapy and/or interferon are indicated only in patients with malignant progressive disease. Embolisation and chemoembolisation are effective in controlling clinical symptoms; however, they do not seem to improve survival.