Article
Hereditary Angioedema with Normal C1 Inhibitor: Update on Evaluation and Treatment.
Immunology and allergy clinics of North America - 1 Aug 2017
Magerl Markus, Germenis Anastasios E, Maas Coen, Maurer Marcus
Abstract excerpt
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...
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