Hereditary angioedema

被引：0
|
作者：
Aygoeren-Puersuen, Emel [1 ]
Bork, Konrad [2 ]
机构：
[1] Goethe Univ, Univ Klinikum Frankfurt, Klin Kinder & Jugendmed, Angioodem Ambulanz & Interdisziplinares Comprehen, Theodor Stern Kai 7, D-60590 Frankfurt, Germany
[2] Univ Med Mainz, Hautklin, Mainz, Germany
来源：
PADIATRIE UND PADOLOGIE | 2020年 / 55卷 / 01期
关键词：
Complement C1 inhibitor protein; Factor XII; Bradykinin; Hereditary angioedema; Prophylaxis; angioedema; ATTACKS; INHIBITOR; PROPHYLAXIS; PREVENTION; MUTATIONS; C1-INH; GENE;
D O I：
10.1007/s00608-019-00738-4
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
Hereditary angioedema (HAE) comprises a heterogeneous group of diseases with similar phenotypes but different underlying genotypes. Specific clinical signs may point to HAE as opposed to histaminergic angioedema: the typical prolonged development of angioedema over time, positive family history, a lack of response to antihistamines and steroids and response to bradykinin antagonists are typical signs of HAE. The different types of HAE may be associated with a severe clinical course. They are life-long conditions and are still potentially life-threatening. The quality of life of patients with HAE may be considerably impaired. Management plans should be individualized, which is facilitated by the variety of specific medicastions available.
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收藏
页码：12 / 20
页数：9
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