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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.

DOI:10.1001/archsurg.1979.01370320088018
PMID:37817
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。

相似文献

1
Separate pancreatic gastrin cell and beta-cell adenomas: report of a patient with multiple endocrine adenomatosis type 1.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
Arch Surg. 1979 Aug;114(8):956-8. doi: 10.1001/archsurg.1979.01370320088018.
2
Hypersecretion of insulin, glucagon and gastrin in a kindred with multiple adenomatosis.一个患有多发性腺瘤病的家族中胰岛素、胰高血糖素和胃泌素分泌过多。
Diabetes. 1968;17:299.
3
Insulin, proinsulin, glucagon and gastrin in pancreatic tumors and in plasma of patients with organic hyperinsulinism.胰腺肿瘤及器质性高胰岛素血症患者血浆中的胰岛素、胰岛素原、胰高血糖素和胃泌素。
J Clin Endocrinol Metab. 1977 Apr;44(4):681-94. doi: 10.1210/jcem-44-4-681.
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Familial nesidioblastosis as the predominant manifestation of multiple endocrine adenomatosis.
Am J Med. 1972 Feb;52(2):211-27. doi: 10.1016/0002-9343(72)90071-x.
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Islet cell tumors of the pancreas and the alimentary tract.
Am J Surg. 1975 Feb;129(2):102-10. doi: 10.1016/0002-9610(75)90284-6.
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Islet cell functional pathology.胰岛细胞功能病理学
Pathol Annu. 1977;12 Pt 1:1-15.
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Common origin for all neuroendocrine tumors.所有神经内分泌肿瘤的共同起源。
Acta Radiol Ther Phys Biol. 1973 Aug;12(4):321-6. doi: 10.3109/02841867309131097.
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Somatostatin inhibition of insulin and gastrin hypersecretion in pancreatic islet-cell carcinoma.生长抑素对胰岛细胞癌中胰岛素和胃泌素分泌过多的抑制作用。
N Engl J Med. 1975 Jun 26;292(26):1385-6. doi: 10.1056/NEJM197506262922606.
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[Pathologicoanatomical aspects of tumors with endocrine activity].[具有内分泌活性肿瘤的病理解剖学方面]
Langenbecks Arch Chir. 1971 Dec;329:426-37. doi: 10.1007/BF01770552.
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[Associated pancreatic insular infiltrations. Insulinoma, gastrinoma, glucagonoma. Clinical, biological, and anatomo-pathological data].
Actual Endocrinol (Paris). 1973;13(0):15-27.

引用本文的文献

1
Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features.佐林格-埃利森综合征中的血清胃泌素:II. 对美国国立卫生研究院293例患者进行胃泌素激发试验的前瞻性研究,并与文献中的537例病例进行比较。评估诊断标准,提出新标准,并与临床和肿瘤特征进行相关性分析。
Medicine (Baltimore). 2006 Nov;85(6):331-364. doi: 10.1097/MD.0b013e31802b518c.
2
Serum gastrin in Zollinger-Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature.佐林格-埃利森综合征中的血清胃泌素:I. 对美国国立卫生研究院309例患者空腹血清胃泌素的前瞻性研究,并与文献中的2229例病例进行比较。
Medicine (Baltimore). 2006 Nov;85(6):295-330. doi: 10.1097/01.md.0000236956.74128.76.
3
Genetic screening to identify the gene carrier in Italian and German kindreds affected by multiple endocrine neoplasia type 1 (MEN 1) syndrome.
J Endocrinol Invest. 1995 May;18(5):329-35. doi: 10.1007/BF03347833.
4
Multiple endocrine neoplasia type I: general features and new insights into etiology.多发性内分泌腺瘤1型:一般特征及病因学新见解
J Endocrinol Invest. 1991 Jan;14(1):61-72. doi: 10.1007/BF03350266.