PMC4578060_01
Pedigree of the family. Proband is indicated by an arrow. The percentages represent the functional activities of C1 esterase inhibitor (Open symbol: unaffected, hatched symbol: affected, /: not alive, *: suspected case due to unexplained sudden death).
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HAE, the C4 level and functional activity of C1-INH of his family members were measured (Figure 1). His monozygotic twin daughters showed low C4 level and decreased functional activity of CHNH although they had never experienced clinical symptoms of HAE.