以空腸破裂為表現之第一型多發性內分泌腫瘤：壹病例報告

JUJENUM PERFORATIONS AS THE PRESENTATION OF MULTIPLE ENDOCRINE NEOPLASIA TYPE 1: A CASE REPORT

第一型多發性內分泌腫瘤是一種體顯性遺傳的罕見內分泌疾病。其臨床特徵包括副甲狀腺機能亢進、腸胃道和胰臟內分泌腫瘤及腦垂腺腫瘤。大多數的病人都以副甲狀腺機能亢進為主要的表現。我們報告一例空腸破裂的病人，最後診斷為第一型多發性內分泌腫瘤。對於消化性潰瘍導致腸胃道破裂的病人，若其穿孔為多發性或位於不尋常的位置，則需高度懷疑是否有胃泌素瘤的可能性；若確定有胃泌素瘤，則需進一步篩檢第一型多發性內分泌腫瘤是否存在。

Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited syndrome. Hyperparathyroidism, enteropancreatic tumors and pituitary adenomas are the main components of MEN1 , with hyperparathyroidism being the most common and usually the earliest clinical manifestation. We report a case of MEN1 presented with jujenum perforations. In a case of multiple peptic ulcer perforations or perforations at unusual sites, gastrinoma should be highly suspected, and further MEN1 survey should be performed.