A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C′ 1-esterase
VH Donaldson, RR Evans - The American journal of medicine, 1963 - Elsevier
Affected persons in three families with hereditary angioneurotic edema completely lack
naturally occurring serum inhibitor of C′ 1esterase. This inhibitor is also absent from some
younger relatives who have not yet had typical attacks of swelling. Its absence is
characteristic of the hereditary form of angioneurotic edema, for those with nonhereditary
types have normal or increased amounts of the inhibitor. Of 500 persons tested, no others
completely lacked this inhibitor. This deficiency did not result from a destructive property in …
naturally occurring serum inhibitor of C′ 1esterase. This inhibitor is also absent from some
younger relatives who have not yet had typical attacks of swelling. Its absence is
characteristic of the hereditary form of angioneurotic edema, for those with nonhereditary
types have normal or increased amounts of the inhibitor. Of 500 persons tested, no others
completely lacked this inhibitor. This deficiency did not result from a destructive property in …