摘要
遗传性血管性水肿(hereditary angioedema,HAE)是一种常染色体显性遗传病,以反复发作的皮肤和黏膜水肿为特征。水肿可出现在任何部位,最致命的为喉水肿,引发窒息,危及生命。若水肿累及消化道黏膜,可引起腹痛、呕吐等症状,易误诊为急腹症。颜面、躯干及四肢等皮肤水肿也严重影响患者生活质量。为提高急诊科医生的识别及处理能力,本专业组特编写此诊疗路径。路径除对HAE的发病机制、临床表现等进行介绍外,还汇总了既往在中国已经发表的急诊科病例,以便广大医生更好的理解疾病,并绘制诊疗路径图,为临床实践提供参考依据。
Hereditary angioedema(HAE)is an autosomal dominant disease characterized by recurrent skin and mucosal edema.Edema can occur anywhere,but laryngeal edema is the most deadly and can lead to asphyxia and be life-threatening.It can be easily misdiagnosed as acute abdominal disease if edema affects the mucous membranes of the gastrointestinal tract,with the symptoms of abdominal pain and vomiting.Edema of the face,trunk,and extremities also significantly affects patients′quality of life.In order to improve the recognition of emergency department physicians,the panel of expert formulated the diagnosis and treatment path.In addition to introducing the pathogenesis and clinical manifestations of HAE,the pathway also summarizes HAE cases from emergency department which were previously published in Chinese journal,so that physicians could understand the disease better.The pathway also provides a flowchart of HAE diagnosis in the emergency department as a reference for clinical practice.
作者
周宁
韩小彤
陈松
孙鹏
刘斌
杜俊凯
张春阳
郭庚
窦清理
姜伟
吕传柱
朱华栋
张茂
Zhou Ning;Han Xiaotong;Chen Song;Sun Peng;Liu Bin;Du Junkai;Zhang Chunyang;Guo Geng;Dou Qingli;Jiang Wei(Zhanjiang Central People′s Hospital,China;不详)
出处
《中国急救医学》
CAS
CSCD
2024年第2期99-105,共7页
Chinese Journal of Critical Care Medicine
基金
中国医学科学院医学与健康科技创新工程项目(2019-I2M-5-023)
四川省科技基金(2022YFS0602)
海南省重点科技专项(ZDKJ202004,ZDKJ2021038)。
