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.
Dennehy JJ. 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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