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多发性内分泌腺瘤病 1 型伴上消化道出血和穿孔:病例报告并文献复习。

Multiple endocrine neoplasia type 1 with upper gastrointestinal hemorrhage and perforation: a case report and review.

机构信息

Department of Gastroenterology, Shanghai First People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200080, China.

出版信息

World J Gastroenterol. 2013 Feb 28;19(8):1322-6. doi: 10.3748/wjg.v19.i8.1322.

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary syndrome known to predispose subjects to endocrine neoplasms in a variety of tissues such as the parathyroid glands, pituitary gland, pancreas and gastrointestinal tract. We herein report a patient with a past history of pituitary adenoma, presenting with symptoms of chronic diarrhea for nearly one year and a sudden upper gastrointestinal hemorrhage as well as perforation without signs. Nodules in the duodenum and in the uncinate process and tail of pancreas and enlargement of the parathyroid glands were detected on preoperative imaging. Gastroscopy revealed significant ulceration and esophageal reflux diseases. The patient underwent subtotal parathyroidectomy and autotransplantation, pylorus-preserving pancreaticoduodenectomy and pancreatic tail resection and recovered well. The results observed in our patient suggest that perforation and bleeding of intestine might be symptoms of Zollinger-Ellison Syndrome in patients with MEN1.

摘要

多发性内分泌腺瘤病 1 型(MEN1)是一种罕见的遗传性综合征,已知易使患者发生各种组织的内分泌肿瘤,如甲状旁腺、垂体、胰腺和胃肠道。我们在此报告一例既往有垂体腺瘤病史的患者,表现为慢性腹泻近一年,突然出现无明显迹象的上消化道出血和穿孔。术前影像学检查发现十二指肠和钩突及胰尾有结节,甲状旁腺肿大。胃镜检查显示明显的溃疡和食管反流病。患者行甲状旁腺次全切除术及自体移植、保留幽门的胰十二指肠切除术和胰尾切除术,术后恢复良好。我们的患者观察到的结果提示,MEN1 患者的穿孔和肠道出血可能是 Zollinger-Ellison 综合征的症状。

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