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題 名 | Neuronal Intestinal Dysplasia type B with Massive Small Intestinal Hemorrhage: Report of One Case=腸神經發育不良第二型併大量小腸出血:一病例報告 |
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作 者 | 朱育德; 黃高彬; 李一賢; 鄭永銘; | 書刊名 | 臺灣兒科醫學會雜誌 |
卷 期 | 44:5 民92.09-10 |
頁 次 | 頁303-306+322 |
分類號 | 417.5476 |
關鍵詞 | 腸神經發育不良; 下消化道出血; Neuronal intestinal dysplasia; Lower gastrointestinal bleeding; |
語 文 | 英文(English) |
中文摘要 | 腸神經發育不良第二型是在近三十年來新發表的疾病,它的特色是小腸活動力不佳而導致便秘。我們提出一位患有二次危及性命的腸胃道大量出血及發燒的三歲七個月男童,鎝-紅血球掃瞄及异位黏膜掃瞄未能表現出血點,胃鏡及大腸鏡均為陰性,開刀發現由Treitz韌帶以下100~170公分之空腸腸壁變厚并有黏膜殘骸、血塊、及黏液形成索狀物。病理報告中說明在黏膜下神經叢中有巨大神經節細胞,在固有層中有散佈的神經節細胞。道些現象與腸神經發育不良第二型相符合。根據所查資料顯示有發燒的大量血便是腸神經發育不良第二型少見的表現。我們提出此例以提醒腸神經發育不良可能與大量的血便症狀有相關性。 |
英文摘要 | Neuronal intestinal dysplasia type B is characterized by poor intestinal motility that usually results in constipation. Massive gastrointestinal hemorrhage is very unusual as the initial presentation in this disease entity. We report on a 3-year-and-7-month-old boy who had experienced two episodes of life- threatening lower gastrointestinal hemorrhage and high fever. Technetium-99m labeled red blood cell scan and the Meckel’s diverticulum scan failed to demonstrate the bleeding point. The results of panendoscopy and colonoscopy were negative. Laparotomy revealed a 70 cm-long thick-walled intestine (from 100 cm to 170 cm below the Treitz ligament) with cord like material formed by mucosa debris, blood clot and mucus. Pathology report revealed hyperplasic nerve bundles in submucosal plexus with giant ganglion formation, consistent with neuronal intestinal dysplasia type B. |
本系統中英文摘要資訊取自各篇刊載內容。