| Popis: |
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 these serum specimens, for normal serum lost no inhibitor during its incubation with that from a patient. In addition, lack of this inhibitor did not reflect a generalized absence of antiproteolytic activity, for antiplasmin and antitrypsin were present. This evidence suggests that absence of serum inhibitor of C′1-esterase is an inherited abnormality in those with the familial type of angioneurotic edema. This does not explain the sporadic occurrence of localized swellings; tissue mechanisms are implicated. This determination may permit identification of young family members who will have attacks of swelling later in life. |
