佐林-艾利森综合征:病例报告和专题综述

Rosaura J. Serrano, Kevin Avilés, Yanetzy E. Corona, Gaby A. Alarcón
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摘要

祖林格-艾利森综合征(Zollinger-Ellison syndrome,ZES)的特征是十二指肠或胰腺中分泌胃泌素的神经内分泌肿瘤(胃泌素瘤)。这是一种罕见病,大多数患者在 20-50 岁之间确诊,男性发病率较高。胃泌素瘤的恶性风险很高,需要通过泌素刺激试验和奥曲肽闪烁成像等影像学检查才能确诊。我们介绍的病例是一名 24 岁的男性,除了有消化性溃疡病史外,还伴有腹泻、气喘、腺痛和腹痛。实验室检查显示血红蛋白水平偏低,胃泌素水平升高。内镜检查发现了巨大溃疡,随后的手术发现了胃空肠粘连、胃部病变和梅克尔憩室。影像学检查证实胰腺中存在神经内分泌肿瘤活动。ZES 会导致持续的高胃泌素血症,引起消化性溃疡和其他消化道并发症。胃泌素瘤可发生于不同部位,可导致消化性溃疡、吸收不良和腹泻。诊断需要空腹血清胃泌素水平升高和胃酸分泌过多。治疗包括在诊断检测前停用质子泵抑制剂(PPI),并对合适的患者进行肿瘤手术切除。由于检测方法不可靠以及需要进行胰泌素检测,ZES 的诊断可能比较复杂。对于没有转移的散发性胃泌素瘤,建议进行手术切除,而对于手术后残留的胃酸过多,则可能需要药物治疗。患者应接受影像学检查以确定肿瘤位置,并定期监测并发症和复发情况。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Zollinger-Ellison syndrome: case report and topic review
Zollinger-Ellison syndrome (ZES) is characterized by gastrin-secreting neuroendocrine tumors (gastrinomas) in the duodenum or pancreas. It is a rare condition, most patients are diagnosed between the ages of 20 and 50, and a higher incidence in men. Gastrinomas are associated with a high risk of malignancy and the diagnosis is confirmed by the secretin stimulation test and imaging studies such as octreotide scintigraphy. We present the case of a 24-year-old man who presented with melena, asthenia, adynamia and abdominal pain, in addition to a history of peptic ulcer. Laboratory tests revealed low levels of hemoglobin and elevated levels of gastrin. Endoscopy showed a giant ulcer and subsequent surgery revealed stomach-jejunum adhesions, gastric lesions and Meckel's diverticulum. Imaging studies confirmed neuroendocrine tumor activity in the pancreas. ZES leads to sustained hypergastrinemia, causing peptic ulcers and other digestive tract complications. Gastrinomas can arise from a variety of locations and can cause peptic ulcers, malabsorption, and diarrhea. Diagnosis requires elevated fasting serum gastrin levels and hypersecretion of gastric acid. Treatment involves discontinuation of proton pump inhibitors (PPIs) before diagnostic testing and surgical resection of tumors in suitable candidates. The diagnosis of ZES can be complicated due to the unreliability of the assays and the need for secretin testing. Surgical resection is recommended for sporadic gastrinomas without metastasis, while medical treatment may be necessary for postsurgical residual hyperacidity. Patients should undergo imaging studies for tumor localization and regular monitoring for complications and recurrences.
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