Hereditary Angioedema with Normal C1 Inhibitor: Update on Evaluation and Treatment.

Abstract:

:A new form of hereditary angioedema (HAE) was identified in the year 2000. Its clinical appearance resembles HAE types I and II, which are caused by mutations that result in a deficiency of C1 inhibitor (C1-INH). In patients with the new form of HAE, C1-INH plasma levels and function values are normal, so it's termed HAE with normal C1-INH (HAE-nC1). HAE-nC1, in a subgroup of patients, is thought to be caused by mutations that affect the F12 gene. The diagnosis of HAE-nC1 is based on history and clinical criteria. There are no licensed drugs with proven treatment effects for HAE-nC1.

authors

Magerl M,Germenis AE,Maas C,Maurer M

doi

10.1016/j.iac.2017.04.004

subject

Has Abstract

pub_date

2017-08-01 00:00:00

pages

571-584

issue

3

eissn

0889-8561

issn

1557-8607

pii

S0889-8561(17)30045-0

journal_volume

37

pub_type

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