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[与卓-艾综合征和甲状旁腺功能亢进相关的多发性内分泌肿瘤]

[Multiple endocrine neoplasms associated with Zollinger-Ellison syndrome and hyperparathyroidism].

作者信息

Tellaroli J C, Charleux H, Semin A M, Parrot B, Fasano J J, Cain P

出版信息

Ann Gastroenterol Hepatol (Paris). 1984 Jul-Sep;20(4):199-201.

PMID:6152141
Abstract

We report the case of a 43 year old man with multiple endocrine neoplasia (Zollinger-Ellison syndrome-hyperparathyroidism) diagnosed during investigations for a severe ulcer with diarrhoea. The history suggested Zollinger-Ellison syndrome, especially as basal acid production and blood gastrin levels were high. Hypercalcaemia and low blood phosphate levels suggested hyperparathyroidism, which was confirmed by ultra-sound of the neck, showing two parathyroid adenomas. The aim of treatment was two-fold: suppress the hyperparathyroidism and treat the Zollinger-Ellison syndrome. The approach is surgical in most cases, but the tumour is often impossible to remove and total gastrectomy has become rare with the progress in anti-secretory therapy. When the tumour behind a case of Zollinger-Ellison syndrome cannot be found, complementary chemotherapy is indicated. Other endocrine lesions must always be sought and the family investigated to exclude Wermer syndrome.

摘要

我们报告了一例43岁男性患者,在因严重溃疡伴腹泻进行检查时被诊断为多发性内分泌肿瘤(卓-艾综合征-甲状旁腺功能亢进症)。病史提示卓-艾综合征,尤其是基础胃酸分泌和血胃泌素水平升高。高钙血症和低血磷水平提示甲状旁腺功能亢进症,颈部超声证实了这一点,显示有两个甲状旁腺腺瘤。治疗目的有两个:抑制甲状旁腺功能亢进症并治疗卓-艾综合征。大多数情况下采用手术治疗,但肿瘤往往无法切除,随着抗分泌治疗的进展,全胃切除术已很少见。当找不到卓-艾综合征病例背后的肿瘤时,需进行辅助化疗。必须始终寻找其他内分泌病变,并对其家族进行调查以排除韦默综合征。

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