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隐源性多灶性溃疡性狭窄性肠炎(CMUSE):三十年的故事

Cryptogenic Multifocal Ulcerous Stenosing Enteritis (CMUSE): A Tale of Three Decades.

作者信息

Singh Ayaskanta, Sahu Manoj Kumar, Panigrahi Manas Kumar, Misra Debasis

机构信息

Department of Gastroenterology and Hepatobiliary Sciences, Siksha O. Anusandhan University Institute of Medical Sciences and SUM Hospital, Bhubaneswar, India.

Department of Gastroenterology, All India Institute of Medical Sciences, Bhubaneswar, India.

出版信息

ACG Case Rep J. 2017 Mar 15;4:e44. doi: 10.14309/crj.2017.44. eCollection 2017.

Abstract

We present a patient who, over the course of 35 years, presented with repeated episodes of ileus and melena and was misdiagnosed several times, leading to several surgeries. Macroscopic and microscopic features of his resected intestine were compatible with cryptogenic multifocal ulcerous stenosis enteritis (CMUSE), but additional angioectatic lesions were also noted. The patient responded dramatically to hormonal therapy consisting of ethinylestradiol and norethisterone. To our knowledge, this is the first reported case of CMUSE that has shown response to hormonal therapy. We suggest that this is a variant of CMUSE in which vasculopathy plays a role in the pathogenesis.

摘要

我们报告一例患者,在35年的病程中反复出现肠梗阻和黑便,曾多次被误诊,接受了多次手术。其切除肠道的宏观和微观特征与隐源性多灶性溃疡性狭窄性肠炎(CMUSE)相符,但也发现了额外的血管扩张性病变。该患者对由炔雌醇和炔诺酮组成的激素治疗反应显著。据我们所知,这是首例报道的对激素治疗有反应的CMUSE病例。我们认为这是CMUSE的一种变体,其中血管病变在发病机制中起作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8176/5356201/3bc0ae2f8c4a/CG-CGCR170010F001.jpg

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