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炎症性肠病:先天性纤溶酶原缺乏症的胃肠道表现

Inflammatory Bowel Disease: A Gastrointestinal Presentation of Congenital Plasminogen Deficiency.

作者信息

Balram Bhairavi, Thiesen Aducio, Kroeker Karen I

机构信息

Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada.

Department of Laboratory Medicine and Pathology, University of Alberta Hospital, University of Alberta, Edmonton, Alberta, Canada.

出版信息

ACG Case Rep J. 2021 Jun 2;8(5):e00613. doi: 10.14309/crj.0000000000000613. eCollection 2021 May.

Abstract

Plasminogen deficiency (PD) is a rare autosomal recessive disease that results in the formation of fibrin-rich pseudomembranes, which impair wound-healing capacity. We report a 21-year-old man with congenital PD-associated inflammatory bowel disease. After an episode of infection, he developed chronic diarrhea. Colonoscopy revealed moderate friability and erythema of the colon. Histology showed fibrin deposits in the lamina propria of the colonic mucosa with surrounding inflammation and focal ulceration. He was treated with infliximab and achieved clinical remission. To our knowledge, this is the first reported case of colonic involvement of PD.

摘要

纤溶酶原缺乏症(PD)是一种罕见的常染色体隐性疾病,可导致富含纤维蛋白的假膜形成,从而损害伤口愈合能力。我们报告了一名患有先天性PD相关炎症性肠病的21岁男性。一次感染发作后,他出现了慢性腹泻。结肠镜检查显示结肠中度脆弱和红斑。组织学检查显示结肠黏膜固有层有纤维蛋白沉积,周围有炎症和局灶性溃疡。他接受了英夫利昔单抗治疗并实现了临床缓解。据我们所知,这是首例报道的PD累及结肠的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c269/8174548/98a713bc0255/ac9-8-e00613-g001.jpg

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