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糖皮质激素治疗联合使用纤维蛋白原(凝血因子 XIII 浓缩剂)治疗疑似 IgA 血管炎相关小肠结肠炎所致严重腹泻和低白蛋白血症的缓解:一例报告

Relief of Suspected IgA Vasculitis-Associated Enterocolitis Manifested as Severe Diarrhea and Hypoalbuminemia Treated With Glucocorticoid Therapy and Administration of Fibrogammin® (Purified Coagulation Factor XIII Concentrate): A Case Report.

作者信息

Aikawa Takashi, Negishi Takashi, Iida Tomohiro

机构信息

Internal Medicine, Kiryu Kosei General Hospital, Kiryu, JPN.

出版信息

Cureus. 2025 Aug 4;17(8):e89316. doi: 10.7759/cureus.89316. eCollection 2025 Aug.

Abstract

A 62-year-old female was admitted to our hospital with abdominal pain, diarrhea, and bloody stool. She suffered from severe diarrhea 30 times per day and consequently got hypoalbuminemia and hyponatremia. Esophagogastroduodenoscopy and total colonoscopy showed diffuse erosion of the duodenum, terminal ileum, and colorectum. Her endoscopic findings were similar to the former case reports of immunoglobulin A (IgA) vasculitis-associated enterocolitis. Further investigation of biopsy demonstrated that IgA-positive lymphocytes invaded the perivascular of the submucosal layer. However, we did not find IgA deposition in the capillary wall, and no granuloma, basal plasma cytosis, or crypt abscess was detected. Although a definite diagnosis could not be established, we considered the possibility of refractory IgA vasculitis-associated enterocolitis and initiated glucocorticoid therapy and administration of Fibrogammin® (purified coagulation factor XIII concentrate). The patient's diarrhea improved within one and a half months after admission.

摘要

一名62岁女性因腹痛、腹泻和便血入院。她每天腹泻严重达30次,结果出现了低白蛋白血症和低钠血症。食管胃十二指肠镜检查和全结肠镜检查显示十二指肠、回肠末端和结直肠弥漫性糜烂。她的内镜检查结果与先前免疫球蛋白A(IgA)血管炎相关小肠结肠炎的病例报告相似。活检的进一步检查表明,IgA阳性淋巴细胞侵入黏膜下层血管周围。然而,我们未在毛细血管壁发现IgA沉积,也未检测到肉芽肿、基底浆细胞增多或隐窝脓肿。尽管无法确诊,但我们考虑了难治性IgA血管炎相关小肠结肠炎的可能性,并开始使用糖皮质激素治疗以及给予纤维蛋白原(纯化的凝血因子XIII浓缩物)。患者入院后一个半月内腹泻情况有所改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91a3/12406167/0eaa3d4eb254/cureus-0017-00000089316-i01.jpg

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