Hereditary angioneurotic edema. Report of a large kindred with defect in C’1 esterase inhibitor and review of the literature.


Of 39 individuals in a family with hereditary angioneurotic edema, 11 are clinically affected in 4 generations, with 2 deaths from laryngeal edema; those still living have abnormally low levels of C′1 esterase inhibitor. Five children have the biochemical defect but as yet no symptoms. Four members of the family have symptoms suggestive of the disease but have normal sera; they may have other illnesses. Two patients have shown marked improvement with testosterone treatment. The known biologic reactions involved are discussed, the recent literature is reviewed, and a previously unnoticed historical reference is added.

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