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孤立性胰腺胃泌素细胞腺瘤和β细胞腺瘤:1型多发性内分泌腺瘤病1例报告

Separate pancreatic gastrin cell and beta-cell adenomas: report of a patient with multiple endocrine adenomatosis type 1.

作者信息

Peurifoy J T, Gomez L G, Thompson J C

出版信息

Arch Surg. 1979 Aug;114(8):956-8. doi: 10.1001/archsurg.1979.01370320088018.

Abstract

A patient initially showed symptoms of peptic ulcer disease in 1953 and was later found to have hypercalcemia and hyperparathyroidism. Peptic ulcer symptoms persisted after parathyroidectomy, and results of studies provided evidence of the Zollinger-Ellison syndrome. Evaluation of the patient's family showed a classic pattern of multiple endocrine adenomatosis type 1. The patient underwent total gastrectomy and excision of a gastrin cell adenoma in 1971 with relief of symptoms, but with persistent hypergastrinemia. He remained in good health until January 1976, when symptoms of hypoglycemia developed. Results of laboratory studies were compatible with the diagnosis of a pancreatic beta-cell adenoma. At the time of operation, an adenoma of the head of the pancreas was found. The tumor was excised; no other metastatic tumors were found. The tumor was compatible with a beta-cell adenoma and was found to contain high concentrations of insulin; there was no important amount of gastrin. Symptoms of hypoglycemia have entirely disappeared.

摘要

一名患者于1953年最初出现消化性溃疡病症状,后来被发现患有高钙血症和甲状旁腺功能亢进。甲状旁腺切除术后消化性溃疡症状持续存在,研究结果为卓-艾综合征提供了证据。对该患者家族的评估显示出1型多发性内分泌腺瘤病的典型模式。该患者于1971年接受了全胃切除术和胃泌素细胞腺瘤切除术,症状缓解,但仍有持续性高胃泌素血症。他一直健康状况良好,直到1976年1月出现低血糖症状。实验室研究结果与胰腺β细胞腺瘤的诊断相符。手术时,发现胰腺头部有一个腺瘤。肿瘤被切除;未发现其他转移性肿瘤。该肿瘤与β细胞腺瘤相符,且发现含有高浓度胰岛素;没有大量胃泌素。低血糖症状已完全消失。

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