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[1型神经纤维瘤病患者小肠反复出血]

[Recurrent hemorrhage from the small intestine in a patient with type 1 neurofibromatosis].

作者信息

Folwaczny C, Win J, Fabritius J, Loeschke K

机构信息

Medizinische Klinik, Klinikum Innenstadt der Ludwig-Maximilians Universität München.

出版信息

Z Gastroenterol. 1995 Nov;33(11):669-72.

PMID:8600664
Abstract

Patients with NF-1 may exhibit visceral neurofibromas, Schwannomas and other tumors of ectodermal origin beside the classical cutaneous and neuronal manifestations of neurofibromatosis. In a 46-year-old woman with a previous diagnosis of NF-1 and a history of recurrent intestinal bleeding of obscure origin a well vascularized tumor in a branch of the A. messenterica superior was found by use of scintigraphy and angiography. Intraoperatively two inhomogeneons tumors were found in the proximal jejunum. Histologically these tumors were classified as neurofibromas. After partial resection of the jejunum no further intestinal bleeding occurred in this patient. Therefore the intestinal neurofibromas were presumably the source of the recurrent bleeding.

摘要

患有神经纤维瘤病1型(NF-1)的患者除了有神经纤维瘤病典型的皮肤和神经表现外,还可能出现内脏神经纤维瘤、施万细胞瘤和其他外胚层起源的肿瘤。在一名先前诊断为NF-1且有不明原因反复肠道出血病史的46岁女性患者中,通过闪烁扫描和血管造影在肠系膜上动脉的一个分支中发现了一个血供丰富的肿瘤。术中在空肠近端发现了两个不均质的肿瘤。组织学上,这些肿瘤被分类为神经纤维瘤。空肠部分切除术后,该患者未再发生肠道出血。因此,肠道神经纤维瘤可能是反复出血的来源。

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