Article
Clinical features of genetically characterized types of hereditary angioedema with normal C1 inhibitor: a systematic review of qualitative evidence.
Orphanet journal of rare diseases - 15 Oct 2020
Bork Konrad, Machnig Thomas, Wulff Karin, Witzke Guenther, Prusty Subhransu, Hardt Jochen
Abstract excerpt
BACKGROUND: Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH) (HAEnCI) is associated with skin swellings, abdominal attacks, and the risk of asphyxia due to upper airway obstruction. Several different gene mutations linked to the HAE phenotype have been identified. Our aim was to qualitatively assess and describe the clinical differentiators of these genetically identified HAEnCI types. To achieve...
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