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与血管性血友病相关的小肠血管发育异常:病例报告

Small bowel angiodysplasia associated with von Willebrand's disease: report of a case.

作者信息

Patti Rosalia, Almasio Piero L, Buscemi Salvatore, Tripodo Claudio, Di Vita Gaetano

机构信息

Department of Surgical and Oncologic Science, Division of General Surgery, University of Palermo, Via Autonomia Siciliana 70, Palermo, Italy.

出版信息

Surg Today. 2006;36(7):659-62. doi: 10.1007/s00595-006-3202-0.

Abstract

The association between angiodysplasia (AD) and von Willebrand's disease (vWD) was first described by Quick in 1967. The clinical course of patients with vWD and AD is characterized by multiple admissions to hospital for gastrointestinal bleeding, necessitating transfusions of packed red cells, factor VIII, and von Willebrand factor concentrates. The therapeutic management of these patients is difficult. Both medical and endoscopic techniques have been tried, but no treatment modality has been completely successful. We describe the clinical course of a patient affected by vWD, who suffered repeated massive gastrointestinal bleeding caused by small bowel AD. Intestinal resection was the only effective treatment, resulting in complete remission of the gastrointestinal bleeding.

摘要

血管发育异常(AD)与血管性血友病(vWD)之间的关联最早由奎克于1967年描述。患有vWD和AD的患者的临床病程特点是多次因胃肠道出血入院,需要输注浓缩红细胞、凝血因子VIII和血管性血友病因子。这些患者的治疗管理很困难。药物和内镜技术都已尝试过,但没有一种治疗方式是完全成功的。我们描述了一名受vWD影响的患者的临床病程,该患者因小肠AD反复发生大量胃肠道出血。肠切除术是唯一有效的治疗方法,使胃肠道出血完全缓解。

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