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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:
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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页码:12 / 20
页数:9
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