John J. Dennehy, M.D.
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Of 39 individuals in a family with hereditary angioneurotic edema 15 are clinically affected in 4 generations, with 2 deaths from laryngeal edema. Acute attacks of nonallergic edema of the skin, intestines, and larynx have been recognized as an hereditary entity since Osler's description in 1888. In 1963 a defect in the inhibitor of the first component of complement was reported in this disease. Low or absent levels of C′1-esterase inhibitor have been demonstrated in members of this family, including some not as yet clinically affected. The complement system and pertinent literature are reviewed.
John J. Dennehy. Hereditary Angioneurotic Edema: Report of a Large Kindred with Defect in C′1 Esterase Inhibitor.. Ann Intern Med. 1969;70:1088. doi: 10.7326/0003-4819-70-5-1088_2
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Published: Ann Intern Med. 1969;70(5):1088.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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