遗传性血管性水肿诊治的“院内多学科会诊协作网联动线上线下全程管理”模式的初探  

The management mode of hereditary angioedema: intra-hospital multi-diciplinary treatment, linkage of cooperation network, online and offline whole process management

作　　者：姚我[1] 吴定钱[2] 沈颖[3] 沈磊[4] 李燕[5] 葛柳雅[6] 张松照[7] 陈炯[8] 汪慧英[1] Yao Wo;Wu Dingqian;Shen Ying;Shen Lei;Li Yan;Ge Liuya;Zhang Songzhao;Chen Jiong;Wang Huiying(Department of Allergy and Clinical Immunology,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310009,China;Department of Emergency Medicine,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310009,China;Department of Dermatology,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310009,China;Department of Otolaryngology,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310009,China;Department of Gastroenterology,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310009,China;Department of Outpatient Nursing,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310009,China;Department of Clinical Laboratory Medicine,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310009,China;State Key Laboratory for Managing Biotic and Chemical Threats to the Quality and Safety of Agro-Products,Ningbo University,Ningbo 315211,China)

机构地区：[1]浙江大学医学院附属第二医院变态反应科,杭州310009 [2]浙江大学医学院附属第二医院急诊科,杭州310009 [3]浙江大学医学院附属第二医院皮肤科,杭州310009 [4]浙江大学医学院附属第二医院耳鼻咽喉科,杭州310009 [5]浙江大学医学院附属第二医院消化内科,杭州310009 [6]浙江大学医学院附属第二医院门诊护理部,杭州310009 [7]浙江大学医学院附属第二医院检验科,杭州310009 [8]宁波大学农产品质量安全危害因子与风险防控国家重点实验室,宁波315211

出　　处：《中华预防医学杂志》2024年第3期406-413,共8页Chinese Journal of Preventive Medicine

基　　金：国家中医药管理局科技司-浙江省中医药管理局共建科技计划项目(GZY-ZJ-KJ-23027)。

摘　　要：遗传性血管性水肿(hereditary angioedema,HAE)是一种常染色体显性遗传的罕见病,患病率约为50 000分之一,以反复发作、不可预测的皮肤或黏膜下水肿为临床特征,累及咽喉部可导致潜在的致命风险。其疾病的罕见性、临床表现的多样性,造成医务人员认识不足,导致临床的误诊误治。因此,提高医务人员对HAE的认识非常重要。本院建立以院内多学科会诊(multi-disciplinary treatment,MDT)团队、省内协作网联动、线上线下患者管理和媒体宣教交互的管理模式(“院内MDT协作网联动线上线下全程管理”),通过这种模式的推广,显著提高了浙江省HAE患者的精准诊断,缩短了诊断时间,显著降低了患者的严重喉头水肿发作急救,同时为HAE的临床诊治提供参考。Hereditary angioedema(HAE)is an autosomal dominant inherited disease characterized by recurrent and unpredictable episodes of subcutaneous or submucosal edema.These attacks could induce fatal risk when larynx is involved.The estimated prevalence of HAE is about 1 in 50000.Due to its rarity and the diversity of clinical manifestations,HAE is known little by related physicians and misdiagnosis and mistreatment happens very often.Therefore,it is crucial to improve physicians′understanding of HAE.To address this,a comprehensive management approach for the diagnosis and treatment of HAE have developed in our hospital.This approach includes intra-hospital multi-disciplinary treatment(MDT),collaboration with provincial network hospitals,patient education online and offline interacting with media propaganda.By implementing this approach,the diagnostic precision was significantly improved,the diagnostic time was significantly shortened,and the frequency of emergency interventions for severe laryngeal edema was significantly reduced.Additionally,the collection of data from HAE patients has provided valuable clinical insights for the diagnosis and treatment of HAE in China.

关 键 词：遗传性血管性水肿 管理模式 临床路径 C1酯酶抑制物 

分 类 号：R596.1[医药卫生—内科学]