遗传性血管性水肿发病诱因和防治的研究进展
| 作 者：徐青, 张红杰 |
| 单 位：The First Affiliated Hospital with Nanjing Medical University |
| 基金项目：国家自然科学基金 |
| 摘 要： |
| 遗传性血管性水肿（HAE）是一种以缓激肽介导的血管通透性增加为主要机制的罕见遗传性疾病，临床表现为反复发作的皮肤、胃肠道和喉头水肿。本文系统综述了HAE的主要发病诱因及其分子机制，重点总结胃肠道受累的临床特点、鉴别诊断要点和诱因管理策略，并结合近3年的研究进展，概述新型靶向治疗药物在HAE防治中的应用现状，旨在提高消化科医师对HAE的认识，减少误诊和不必要的侵入性干预，促进规范化诊疗。 |
| 关键词：血管性水肿，遗传性；腹痛；胃肠道水肿；诱发因素；缓激肽；诊断，鉴别 |
Progress of Research on Triggers and Management of Hereditary Angioedema
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| Institution: |
| Abstract: |
| Hereditary angioedema (HAE) is a rare genetic disorder characterized by increased vascular permeability mediated by bradykinin, clinically manifesting as recurrent episodes of cutaneous, gastrointestinal, and laryngeal edema. This article systematically reviewed the major triggers and molecular mechanisms of HAE, with a focus on the clinical features of gastrointestinal involvement, key points of differential diagnosis, and trigger management strategies. Combined with the research progress in the past three years, the current application of novel targeted therapeutic agents in the prevention and treatment of HAE was also summarized. This review aimed to improve the awareness of HAE among gastroenterologists, reduce misdiagnosis and unnecessary invasive interventions, and standardize the diagnosis and treatment approaches. |
| Keywords: Angioedema, Hereditary; Abdominal Pain; Gastrointestinal Edema; Triggering Factors; Bradykinin; Diagnosis, Differential |
| 投稿时间：2026-01-16 |
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