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冯雷克林霍增氏病中的肠道神经纤维瘤病:表现为因反复肠道出血导致的慢性贫血。

Intestinal neurofibromatosis in von Recklinghausen's disease: presenting as chronic anemia due to recurrent intestinal hemorrhage.

作者信息

Hahn J S, Chung J B, Han S H, Lee S W, Noh S H, Lee J T, Chun S I, Kim G H

机构信息

Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

出版信息

Korean J Intern Med. 1992 Jul;7(2):137-42. doi: 10.3904/kjim.1992.7.2.137.

Abstract

Neurofibromatosis (von Recklinghausen's disease) is a neuroectodermal disorder characterized by pigmentary changes of the skin (café-au-lait spots), cutaneous and visceral tumors (neurofibromas) and systemic abnormalities. The involvement of gastrointestinal tract in neurofibromatosis is not common. The most common symptoms, refer able to lesions in the gut, are hematemesis, melena and abdominal pain. We experienced a case of intestinal neurofibroma in von Recklinghausen's disease. The patient was a 39 year-old female who had suffered from chronic iron deficiency anemia and recurrent gastrointestinal hemorrhage due to two neurofibromas of jejunum for 3 years, which was diagnosed by superior mesenteric and ileal arteriogram and 99mTc pertechnetate-labelled RBC scan, and treated by segmental resection of jejunum with end to end anastomosis.

摘要

神经纤维瘤病(冯·雷克林豪森病)是一种神经外胚层疾病,其特征为皮肤色素沉着改变(咖啡牛奶斑)、皮肤和内脏肿瘤(神经纤维瘤)以及全身异常。神经纤维瘤病累及胃肠道并不常见。肠道病变最常见的症状是呕血、黑便和腹痛。我们遇到一例冯·雷克林豪森病合并肠道神经纤维瘤的病例。患者为一名39岁女性,因空肠的两个神经纤维瘤导致慢性缺铁性贫血和反复胃肠道出血3年,经肠系膜上动脉和回肠动脉造影以及99m锝高锝酸盐标记红细胞扫描确诊,并行空肠节段切除端端吻合术治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/72d1/4532108/4d46732912ef/kjim-7-2-137-10f1.jpg

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