Fig. 2

QUOROM flow-chart shows the number of publications screened and included in the systematic review for qualitative evidence. HAE-ANGPT1 = hereditary angioedema with a specific mutations in the angiopoietin-1 gene, HAE-FXII = hereditary angioedema with specific mutations in the factor XII gene, HAE-KNG-1 = hereditary angioedema with a specific mutation in the kininogen-1 gene, HAE-PLG = hereditary angioedema with a specific mutation in the plasminogen gene, HMWK = high molecular weight kininogen, HAEnCI = hereditary angioedema with normal C1 esterase inhibitor, MeSH = medical subject headings, N = number of records. *("complement c1 inhibitor protein"[MeSH Term] AND ("angioedema, hereditary" [MeSH Term]) search for a period from 1 January 2006 to 1 March 2020