遗传性血管性水肿患者诊疗流程与健康管理模式

Diagnosis and treatment procedures and health management for patients with hereditary angioedema

遗传性血管性水肿(HAE)属致死性高的可治性罕见病,容易漏诊和误诊,以累及头面部、四肢、消化道、生殖器、喉等部位的反复发作的水肿为特征。HAE诊断并不困难,但是由于其发病率低,临床症状缺乏特异性,导致接诊医务人员难以及时诊断和进行正确干预,因此,提高对HAE的认知程度和预防其反复发作对HAE患者至关重要。鉴于目前医护人员和广大群众对HAE的疾病认知程度较低,患者一旦出现喉水肿急性发作时难以及时得到专科救治,使不少HAE患者随之发生窒息而致死致残。为此,本文基于互联网移动终端平台,通过设置“一键求助”,为HAE患者提供全天候快速应急响应系统,实现HAE患者规范化管理和紧急救治流程的全程智慧化随访管理。

As a recognized rare and highly fatal disease,hereditary angioedema(HAE)is difficult to diagnose and characterized by recurrent edema involving the head,limbs,genitals and larynx,etc.Diagnosis of HAE is not difficult.However,low incidence and lack of clinical characteristics lead to difficulty of doctors on timely diagnosis and correct intervention for HAE patients.Therefore,it is crucial to improve the awareness of this disease and prevent its recurrence.for HAE patients.In view of absent cognition of doctors and the general public on HAE,patients often suffer from sudden death or become disabled due to laryngeal edema which cannot be treated in time.Thus,based on the Internet mobile terminal platform,the team set up an all-day rapid emergency response system which is provided for HAE patients by setting up"one-click help".The aim is to offer optimization on overall management of HAE and designed the intelligent follow-up management to provide timely assistance and specialized suggestion for patients with acute attacks.