Hereditary Angioedema: Diagnosis, Management, Current State of Art and Advances

نویسندگان

چکیده

Abstract
 Hereditary angioedema (HAE) is a rare, mostly inherited disorder however 25% of patients have de novo mutations. Although it’s rarity, it can be life threatening due to laryngeal involvement. Along with understanding the basis swelling, several new treatment options aside from C1-inhibitory protein (C1-INH) replacement been developed and are avaible on markets. However avaibility approved drugs for attacks HAE varies world wide. Treatment management requires treatment, pre-procedural long term prophylaxis (LTP). C1-INH which was firstly on-demand LTP by iv route, nowadays LTP, other used orally sc route. Despite developing medications, permanent such as gene therapy needed.

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ژورنال

عنوان ژورنال: The journal of pediatric academy

سال: 2022

ISSN: ['2718-0875']

DOI: https://doi.org/10.51271/jpea-2022-164