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

Hereditary Angioedema: diagnosis, management, current State of art and advances


Abstract views: 64

Authors

DOI:

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

Keywords:

Hereditary angioedema, C1-inhibitory protein, C1-INH replacement, bradykinin, plasma kallikrein, lanadelumab

Abstract

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 of swelling, several new treatment options aside from C1-inhibitory protein (C1-INH) replacement have been developed and are avaible on the markets. However the avaibility of approved drugs for attacks of HAE varies world wide. Treatment management requires angioedema attacks treatment, pre-procedural treatment and long term prophylaxis (LTP). C1-INH which was firstly developed and approved for on-demand treatment, pre-procedural treatment and LTP by iv route, nowadays for LTP, other developed and approved options are used by orally and sc route. Despite the new developing medications, permanent treatment such as gene therapy is needed.

Classification of angioedema without wheals

Published

2022-08-30

How to Cite

Karabiber, E., & Karakoç Aydıner, E. (2022). Hereditary Angioedema: Diagnosis, Management, Current State of Art and Advances: Hereditary Angioedema: diagnosis, management, current State of art and advances. The Journal of Pediatric Academy, 3(2), 37–46. https://doi.org/10.51271/jpea-2022-164