Medical Literature - 1978

Hepatic function and fibrinolysis in patients with hereditary angioedema undergoing long-term treatment with tranexamic acid

Agostoni A, Marasini B, Cicardi M, Martignoni G, Uziel L, Pietrogrande M. 8/1978 Allergy

33(4):216-221

Prophylactic treatment with antifibrinolytic agents, epsilon-aminocaproic and tranexamic acid, reduces the incidence and severity of attacks in patients with hereditary angioedema. Long-term effectiveness or risk of antifibrinolytic agents has not been established. Sixteen patients needing continuous prophylaxis because of frequency and severity of attacks were treated with tranexamic acid. In four patients this treatment was ineffective and the drug was withdrawn after 2 months. A remission or reduction in the frequency or severity of attacks was observed in 12 patients treated for a period ranging from 8 to 34 months. Hepatic tests and blood fibrinolytic activity were not influenced by long-term oral treatment with tranexamic acid.

Available online at: onlinelibrary.wiley.com/doi/10.1111/j.1398-9995.1978.tb01537.x/abstract (small fee)

Plasma kinin activation in tranexamic acid treated patients with hereditary angioneurotic edema

Laurberg G. 7/1978 Archives of Dermatological Research

Jul 28;262(2):153-156

Plasma kinin formation after in vitro activation of factor XII in the coagulation system was examined in 4 patients with hereditary angioneurotic edema. Compared to controls activationcurves showed a steeper increase and higher maximum levels. Administration of tranexamic acid to the patients resulted in partial normalisation of plasma kinin activation.

Available online at: link.springer.com/article/10.1007%2FBF00455384 (small fee)

Remissions induced in hereditary angioneurotic edema with an attenuated androgen (danazol): correlation between concentrations of C1-inhibitor and the forth and second components of complement

Pitts JS, Donaldson VH, Forristal J, Wyatt RJ. 10/1978 Journal of Laboratory & Clinical Medicine

Oct;92(4):501-507

Serum concentrations of antigenic and functional C1-INH increased in patients in whom remissions from attacks of HANE were induced with danazol. The levels of C4 were directly related to serum concentrations of C1-INH antigens up to a concentration of about half the C1-INH found in normal plasma; at this point, the C4 concentrations were in the normal range and no longer correlated well with C1-INH concentration. Serum C2 levels correlated less well with C1-INH concentration. In normal serum there was a poor correlation between C1-INH, C4, and C2 levels, suggesting that C1-INH is normally present in excess of the amount needed for normal C4 levels. The increments in serum C1-INH were related to the dose of danazol.

Not available online.

Specific functional and immunologic assay of plasma plasminogen in hereditary angioedema, in hereditary angioedema treated with tranexamic acid, and in normal subjects

Spragg J. 2/1978 Journal of Immunology

Feb;120(2):592-596

Plasma plasminogen levels were determined by a specific esterolytic assay and a radial immunodiffusion assay, both of which were standardized on a weight basis by using highly purified plasminogen diluted in plasminogen-free plasma. In thirteen normal individuals the functional and antigenic levels were 275 +/- 61 microgram/ml and 285 +/- 61 microgram/ml, respectively (r = 0.95), whereas in 58 plasmas from individuals with hereditary angioedema the levels were 272 +/- 58 microgram/ml and 265 +/- 64 microgram/ml, respectively (r = 0.89). In two patients treated with tranexamic acid either acutely or chronically, both the functional and antigenic plasminogen levels were diminished.

Available online at: jimmunol.org/content/120/2/592.full.pdf+html (small fee)

The second component of complement (C2) as an index of hereditary angioneurotic edema

Sobel AT, Moisy M, Belghiti D, Gabay Y, Lagrue G. 11/1978 J.Clin.Lab.Immunol.

Nov;1(3):179-181

Measurements of C2 hemolytic activity were performed in the sera of 13 patients with Hereditary Angioneurotic Edema. Prior to treatment, C2 values correlated with the severity of the disease in each patient. During androgen therapy with Danazol, C2 measurements reflected the clinical benefit of the drug more accurately than C4 levels, thus explaining the effectiveness of low drug doses. This study also suggests that breakdown products of C2 may play an essential role in the pathogenesis of the edema.

Not available online.

Treatment of hereditary angioedema

Marasini B, Cicardi M, Martignoni GC, Agostoni A. 8/1978 Klinische Wochenschrift

Aug 15;56(16):819-823

The purpose of this study was to report the results of different treatments in 20 patients with hereditary angioedema. Effectiveness of tranexamic acid in preventing swellings was evaluated in 15 patients: in all but 3 subjects tranexamic acid was effective without serious side effects. 15 severe attacks of edema were managed with intravenous infusions of either kallikrein inhibitor (8 cases) or concentrate of C1 esterase inhibitor (7 cases). In only 1 case was the kallikrein inhibitor unsuccessful. C 1 esterase inhibitor concentrate proved highly effective in the treatment of acute attacks (the result was lacking in one patient because of too low dosage of the drug). No side effects were observed with both treatments, but improvement was more rapidly achieved with infusion of C1 esterase inhibitor. The serum levels of C4 and C1 esterase inhibitor and the activity of C 1 esterase inhibitor before and after long-term prophylaxis and acute attacks treatment were investigated.

Available online at: link.springer.com/article/10.1007%2FBF01489716 (small fee)

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