Medical Literature - 1983

Cicardi M, Bergamaschini L, Tucci A, Agostoni A, Tornaghi G, Coggi G, et al. 9/1983 Journal of Allergy & Clinical Immunology

Sep;72(3):294-298

17 alpha-Alkylated androgens are highly effective in preventing attacks in HAE patients. These drugs, however, seem to be implicated in the development of cholestatic jaundice, peliosis hepatis, and liver tumors. In order to assess the risk-benefit balance of the long-term therapy with androgen derivatives, a follow-up investigation was performed in 13 HAE patients. The results of this study indicate that long-term treatment (15 to 47 mo) with low doses of danazol or stanozolol does not induce significant hepatic damage detectable by laboratory tests or liver biopsy. However, the limited number of patients, although in a rather long period of observation, still suggests a careful control and the use of minimal effective doses.

Available online at: jacionline.org/article/0091-6749%2883%2990034-9/abstract

Bergamaschini L, Cicardi M, Tucci A, Gardinali M, Frangi D, Valle C, et al. 2/1983 Allergy

Feb;38(2):81-84.

Ten acute attacks were managed in nine patients with hereditary angioedema by means of the infusion of a C1 INH concentrate produced on large scale. No side effects were observed.

Available online at: onlinelibrary.wiley.com/doi/10.1111/j.1398-9995.1983.tb01590.x/abstract (small fee)

Koo E, Feher KG, Feher T, Fust G. 7/1983 Klinische Wochenschrift

Jul 15;61(14):715-717

Hereditary angioneurotic edema (HAE) is a complement-related clinical disorder with a deficiency of the C1 esterase inhibitor protein. Eight patients with severe attacks of the disease were treated with the adrenal “androgen” dehydroepiandrosterone sulphate (DS). Steroid therapy for 3-28 months resulted in dramatic improvement in their clinical state and a moderate increase in the serum concentration of C1 inhibitor. There was a significant increase in the serum level of either unconjugated dehydroepiandrosterone (D) or of DS during treatment.

Available online at: link.springer.com/article/10.1007%2FBF01487618 (small fee)

Fernandez M, Dieguez I, Neffen H, Oehling A. 1/1983 The Journal of Clinical Investigation

Jan-Feb;11(1):19-23

This clinical entity described for the first time by Osler in 1888 presented a great therapeutic problem during many decades because of its severity. Landerman and later on Donaldson and Evans established the pathogenic mechanisms of this disease finding a deficiency in the inhibitor of the first activated component of complement, an alpha 2 aminoglycoprotein, to be the mechanism responsible of the same. More concretely, alterations in the plasmin, kinin and kallikrein systems are those that will lead to a change in vascular permeability with resultant tissue alterations. Four cases of hereditary angioneurotic edema are studied in female patients aged between 15 and 50 years and with family history consistent with angioneurotic familiar edema in which there were six cases of death due to edema of the glottis. Once the diagnosis had been made the patients were subjected to treatment with EACA at doses of 2.5 gm every 6 hours. The determinations of complement were similar in the four cases, with marked decreases in C4 and C1 inhibitor with a decrease in total complement in three cases. Regarding secondary effects, vomiting was found only in one cases, which as the dose was reduced did not necessitate termination of treatment. In summary, considering the results obtained in the cases above, we believe that due to its good tolerance and moderate cost, epsilon-amino-caproic acid at the abovementioned dosage is an excellent pharmacological agent in the treatment of Osler’s hereditary angioneurotic edema.

Available online at: ncbi.nlm.nih.gov/pmc/articles/PMC423402/

Berman BA, Ross RN. 2/1983 Cutis

Feb;31(2):124.

Not Available online.

Brickman CM, Hosea SW. 4/1983 International Journal of Dermatology

Apr;22(3):141-147.

Available online at: onlinelibrary.wiley.com/doi/10.1111/j.1365-4362.1983.tb03351.x/abstract (small fee)

Yelvington M, Prograis LJ Jr, Pizzo CJ, Curd JG 9/1983 American Journal of Clinical Pathology

An immunodiffusion assay for detecting C1 inhibitor function in human serum was described recently by Ziccardi and Cooper. In our present study, the applicability of this assay for C1 inhibitor deficiency or C1 inhibitor dysfunction was evaluated. Of the 39 patients evaluated, all eight patients with the common (C1 inhibitor deficiency) form of hereditary angioedema and all three patients with the variant (dysfunctional C1 inhibitor) form of hereditary angioedema were identified correctly. Treatment of patients with hereditary angioedema with stanozolol or danocrine increased their serum C1 inhibitor concentrations and normalized the immunodiffusion assay for C1 inhibitor function. In addition, the assay allowed the correct identification of three patients with the acquired form of C1 inhibitor deficiency, because the sera of these patients exhibited a distinctive pattern. The 25 samples from patients (chronic angioedema, chronic urticaria, or hypocomplementemic vasculitis) without C1 inhibitor deficiency had normal assays.

Sep;80(3):309-313

Not available online.