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弥漫性胰腺纤维化:多灶性特发性纤维化的一种罕见特征。

Diffuse pancreatic fibrosis: an uncommon feature of multifocal idiopathic fibrosclerosis.

作者信息

Levey J M, Mathai J

机构信息

Department of Medicine, University of Massachusetts Medical School, Worcester, USA.

出版信息

Am J Gastroenterol. 1998 Apr;93(4):640-2. doi: 10.1111/j.1572-0241.1998.181_b.x.

Abstract

Multifocal idiopathic fibrosclerosis (MIF) is a rare syndrome characterized by exuberant fibrosis involving diverse organ systems. MIF is manifest by varying combinations of the following conditions: mediastinal fibrosis, retroperitoneal fibrosis, orbital pseudotumor, Riedel's thyroiditis, and sclerosing cholangitis. Less common features of MIF include Dupuytren's contractures, lymphoid hyperplasia, Peyronie's disease, vasculitis, testicular fibrosis, and pachymeningitis. Fibrosis arising from the pancreas has been previously described in two patients with MIF. We report a 58-yr-old white man with MIF manifest as orbital pseudotumor, sclerosing cholangitis, lymph node hyperplasia, and diffuse pancreatic fibrosis.

摘要

多灶性特发性纤维硬化症(MIF)是一种罕见综合征,其特征为累及多个器官系统的过度纤维化。MIF表现为以下情况的不同组合:纵隔纤维化、腹膜后纤维化、眼眶假瘤、Riedel甲状腺炎和硬化性胆管炎。MIF较少见的特征包括杜普伊特伦挛缩、淋巴增生、佩罗尼氏病、血管炎、睾丸纤维化和硬脊膜炎。先前曾在两名MIF患者中描述过胰腺纤维化。我们报告一名58岁白人男性,患有表现为眼眶假瘤、硬化性胆管炎、淋巴结增生和弥漫性胰腺纤维化的MIF。

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