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Expert Consensus on the Diagnosis and Treatment of Hereditary Angioedema in China (2024 Edition)

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机构: [1]Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Allergy, Beijing, Peoples R China [2]Beijing Key Lab Precis Med Diag & Treatment Allerg, Beijing, Peoples R China [3]Natl Clin Res Ctr Dermatol & Immunol Dis, Beijing, Peoples R China [4]Xingan League Peoples Hosp, Dept Allergy, Xingan League, Inner Mongolia, Peoples R China [5]Nanjing Med Univ, Affiliated Hosp 1, Dept Allergol, Nanjing, Peoples R China [6]Nanjing Med Univ, Affiliated Hosp 1, Dept Otorhinolaryngol, Nanjing, Peoples R China [7]Shanghai Jiao Tong Univ, Renji Hosp, Sch Med, Dept Allergy, Shanghai, Peoples R China [8]Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Gastroenterol, Beijing, Peoples R China [9]Capital Med Univ, Beijing Chaoyang Hosp, Dept Dermatol, Beijing, Peoples R China [10]Tianjin Med Univ, Dept Allergy, Gen Hosp, Tianjin, Peoples R China [11]Sichuan Univ, West China Hosp, Allergy Ctr, Chengdu, Peoples R China [12]Henan Prov Peoples Hosp, Dept Allergy, Zhengzhou, Peoples R China [13]China Med Univ, Shengjing Hosp, Dept Allergy, Shenyang, Peoples R China [14]Kunming Med Univ, Affiliated Hosp 1, Dept Otorhinolaryngol, Kunming, Peoples R China [15]Shanxi Med Univ, Tongji Shanxi Hosp, Hosp 3, Shanxi Acad Med Sci,Shanxi Bethune Hosp,Dept Aller, Taiyuan, Peoples R China [16]Tianjin Acad Tradit Chinese Med, Affiliated Hosp, Dept Dermatol, Tianjin, Peoples R China [17]Tianjin Inst Integrat Dermatol, Tianjin, Peoples R China [18]Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Emergency, Beijing, Peoples R China [19]Huazhong Univ Sci & Technol, Tongji Hosp, Tongji Med Coll, Dept Allergy, Wuhan, Peoples R China
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关键词: Hereditary angioedema C1 inhibitor Complement Diagnosis and treatment Expert consensus

摘要:
Background: Hereditary angioedema (HAE) is a rare, life-threatening autosomal dominant disorder characterized by recurrent episodes of subcutaneous and submucosal edema. Recent years have witnessed significant advancements in HAE management globally as well as in China, including improved understanding of its pathophysiology and the development of targeted therapies. In China, since the publication of the first national consensus in 2019, accumulating clinical experience and the availability of novel therapeutic agents have created an urgent need to update diagnostic and treatment guidelines to reflect current best practices. Summary: This updated 2024 consensus was developed through collaboration among multidisciplinary experts in allergy, otorhinolaryngology, gastroenterology, dermatology, and emergency medicine across China. It provides comprehensive, evidence-based recommendations for HAE-C1-INH management. This consensus refined diagnostic algorithms incorporating clinical presentation, quantitative/functional C1-INH assays, and complement C4 testing, with genetic sequencing reserved for cases with strong clinical suspicion but normal C1-INH levels/function. It stratified treatment approaches reflecting China's current therapeutic landscape: (1) on-demand therapy with icatibant, which is the only currently approved bradykinin B2 receptor antagonist in China; (2) short-term prophylaxis using androgens or fresh frozen plasma for procedural triggers; (3) long-term prophylaxis with lanadelumab which is the first-line monoclonal anti-kallikrein antibody available in China. Special considerations for pediatric, pregnant, and breast-feeding patients are also addressed. Key Message: As the first updated consensus since 2019, this guideline standardizes HAE management across China while addressing regional disparities in diagnostic capabilities and treatment accessibility. It emphasizes early diagnosis to prevent life-threatening laryngeal edema and promotes individualized treatment strategies tailored to China's therapeutic landscape. Future directions include emerging targeted therapies and the development of biomarkers for disease severity prediction. Implementation of these recommendations is expected to significantly reduce diagnostic delays, improve patient outcomes, and enhance quality of life for individuals with HAE in China.

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大类 | 4 区 医学
小类 | 4 区 过敏 4 区 免疫学
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最新[2024]版:
Q3 ALLERGY Q4 IMMUNOLOGY

影响因子: 最新[2024版] 最新五年平均 出版当年[2025版] 出版当年五年平均 出版前一年[2024版]

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第一作者机构: [1]Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Allergy, Beijing, Peoples R China [2]Beijing Key Lab Precis Med Diag & Treatment Allerg, Beijing, Peoples R China [3]Natl Clin Res Ctr Dermatol & Immunol Dis, Beijing, Peoples R China
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通讯机构: [1]Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Allergy, Beijing, Peoples R China [2]Beijing Key Lab Precis Med Diag & Treatment Allerg, Beijing, Peoples R China [3]Natl Clin Res Ctr Dermatol & Immunol Dis, Beijing, Peoples R China
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