Medical Literature - 1976 |
Hereditary angioedema: modification of clinical manifestations with androgens
Davis PJ, Davis FB, Charache P. 12/1976 Birth Defects: Original Article Series
12(6):283-287.
HAE is an autosomal dominant trait of decreased levels or function of circulating and tissue C’1 esterase inhibitor. The clinical illness is characterized by disabling episodes of peripheral, oropharyngeal and gut-wall edema. Long-term fluoxymesterone treatment of 5 affected males (393 patient-months) and oxymetholone treatment of 6 affected females (204 patient-months) has significantly decreased the frequency of attacks of edema without substantive side effects.
Not available online.
Hereditary angioedema: the clinical syndrome and its management
Frank MM, Gelfand JA, Atkinson JP. 5/1976 Annals of Internal Medicine
May;84(5):580-593
Hereditary angioedema is manifested by attacks of swelling of the extremities, face, trunk, airway, or abdominal viscera, occurring spontaneously or secondary to trauma. It is inherited as an autosomal dominant trait and is due to deficient activity of the inhibitor of the activated first component of complement. The clinical diagnosis can be confirmed by the findings of low levels of C4 or C1 esterase inhibitor activity, or both. Therapy may be divided into three phases: long-term prophylaxis of attacks, short-term prophylaxis of attacks, and treatment of acute attacks. Long-term prophylaxis may be achieved with antifibrinolytic agents and androgens. Short-term prophylaxis with these agents and plasma transfusions has been successful. Specific therapy for acute attacks is not available, but good supportive care, together with a knowledge of the course of the disease, can prevent asphyxiation from airway obstruction. Before the advent of therapy, mortality was reported as high as 30%.
Available online at: annals.org/article.aspx?articleid=690279 (small fee)
Letter: Hereditary angioedema and heparin therapy
Colman RW. 9/1976 Annals of Internal Medicine
Sep;85(3):399-400.
Available online at: annals.org/article.aspx?articleid=690529 (small fee)
The effect of synthetic androgens in hereditary angioneurotic edema: alteration of C1 inhibitor and C4 levels
Rosse WF, Logue GL, Silberman HR, Frank MM. 1/1976 Trans.Assoc.Am.Physicians
1976;89:122-132.
Not available online.
Treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities.
Gelfand JA, Sherins RJ, Alling DW, Frank MM. 12/1976 The New England Journal of Medicine
Dec 23;295(26):1444-1448
Danazol, an androgen derivative, was evaluated for its effectiveness in preventing attacks of hereditary angioedema in a double-blind study with nine patients. Of 47 placebo courses, 44 ended with attacks, but during 46 danazol courses only one attack occurred. Side effects were minimal, and virilization was not observed in the women studied. C1 esterase inhibitor levels increased three to four times, and levels of the fourth component of complement (C4) increased 15 times. These changes began during the first day of therapy and were maximal by one to two weeks. After therapy was stopped, C1 esterase inhibitor and C4 levels rapidly decreased. Danazol effectively prevents attacks in hereditary angioedema and acts to correct the underlying biochemical abnormality.
Available online at: nejm.org/doi/full/10.1056/NEJM197612232952602 (small fee)
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