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Epsilon-amino-caproic acid in the treatment of Osler's hereditary angioneurotic edema.

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.

Adolescent↗
PubMed1978

Hereditary angioneurotic edema and Charcot-Marie-Tooth disease in the same family.

In one family two genetic diseases were transmitted as autosomal dominant traits; hereditary angioneurotic edema was inherited from the paternal side and Charcot-Marie Tooth disease from the maternal side of the family. The conditions occurred separately in 8 and 11 members respectively and together (an exceedingly rare occurrence) in 3. Of six siblings, two girls and four boys, all had Charcot-Marie-Tooth disease, and three, the two girls and one of the boys, also had hereditary angioneurotic edema.

Adolescent↗
PubMed1980

Acquired angioedema and hypocompleentemia in a patient with myelofibrosis. Effect of danazol treatment.

This paper reports the findings of an "acquired" hereditary angioedema-like syndrome in a patient with myelofibrosis. No previous personal or family history of angioedema was present. The serum complement pattern showed a marked reduction of Cl esterase inhibitor, Clq and C4. All family members had a normal complement profile. Because of frequent attacks of laryngeal angioedema, prophylactic treatment with danazol was started. A striking clinical response was observed as well as a normalizing effect on the underlying biochemical abnormality.

Aged↗
PubMed1980

[The dynamics and its clinical significance of complement system, especially alternative pathway related to normal human pregnancy, delivery, puerperium and fetus (author's transl)].

The author studied human complement system, especially alternative pathway as a primitive biological defense system, in maternal serum during normal pregnancy, delivery and puerperium. The results obtained were as follows. 1. The mean value of hemolytic activity of alternative pathway (AlCH50) in healthy women was 18.3 +/- 1.1. This value was increased gradually in progress of gestational stages up to 26.6 +/- 2.3 of the average in the last trimester. Stochastically, significant differences about this value was noted between in the first trimester and in the last. This AlCH50 is decreased temporarily at delivery, and it was increased again rapidly, then went down to normal range about one month after delivery. 2. The average of immunochemical C3PA by SRID method during pregnancy was 22.5 +/- 0.4 mg/dl. It was significantly higher than the average 18.1 +/- 0.4 mg/dl of 59 healthy women. C3PA was noted to remain high level constantly during pregnancy. This C3PA level after delivery was observed to take a similar pattern as AlCH50. 3. beta 1C/1A globulin (C3) in 19 healthy women was 60.7 +/- 2.1 mg/dl. This C3 was also increased gradually during pregnancy up to maximum level of 87.5 +/- 2.8 mg/dl at the 3rd trimester. 4. Classical pathway (CH50, C4 and Cl INA) and fetal complement level were investigated at the same time and discussed here.

Adolescent↗
PubMed1982

A shared effect of abscesses and nonviable tissue influencing the development of sepsis and acute respiratory distress syndrome.

The influence of extensive accumulation of pus (n=20) or nonviable tissue (n=50) on complement has been studied. Plasma concentrations of C1 esterase inhibitor, C4, C3 and C5 were determined by electroimmunodiffusion technique. A good correlation between the extent of complement depletion and the amount of nonviable tissue or pus present was found. A prolonged state with reduced plasma concentrations of complement was in high frequency followed by the development of bacteremia. Patients with ongoing extensive complement activation were prone to develop ARDS. The relation between a depleted complement system and the development of bacteremia has been analysed and so has a relation between impaired oxygenation of arterial blood and products formed when complement is activated.

Abscess↗
PubMed1980

[Immunological study of alopecia areata].

Sixteen patients with varying degrees of alopecia areata, without an associated disease were examined before and during a local DNCB therapy for evidence of immunological abnormalities in both cellular and humoral immune parameters. The mean values of IgG, IgA, IgM, IgE, complement factors C3 and C4 and of alpha 1-antitrypsin were found within the norm, of the C1-Inhibitor slight diminished. Antinuclear antibodies in low titer were observed in three, antithyroglobulin antibodies in two young patients. Immuncomplexes were always negative. The mean value for T cells (67 +/- 7%) was normal as well as the lymphocyte transformation to phytohemagglutin, pokweed-mitogen and candidin, the PPD stimulation was slight reduced. No changes in the immunprofile were observed during the DNCB therapy, with exception of a slight, but significant increase of the T cell number from 63 +/- 8% to 77 +/- 5%. Our findings exclude a common systemic immune defect in alopecia areata.

Adolescent↗
PubMed1981

[Hereditary angioedema: description of a sibship and review].

Report on a newly recognized sibship with hereditary angioedema (HAE). The 27 persons investigated include 8 with decreased concentrations of C-1INH and C4 in the Serum. Three of these are children without symptoms of HAE. Five patients have characteristic attacks of HAE, some of which are predominantly abdominal. The effects of treatment with danazol in 4 patients are described. HAE is briefly reviewed with special reference to pathogenesis and treatment.

Adolescent↗
PubMed1981

[The complement system in familial Mediterranean fever: studies in 41 families (author's transl)].

A total increase of blood complement components, particularly C4, is found in subjects with familial Mediterranean fever both before and after colchicine therapy. This effect differs from the serum haptoglobin and orosomucoïd concentration decreases detected after identical therapy, confering diagnostic value to this inflammatory syndrome. This could be of both hepatic and extrahepatic origin. For the latter, it is possible that up take of circulating monocytes, macrophage precursors, by the connective tissue of the serum sub-mesothelial layer is responsible for the lesion.

Complement C1 Inactivator Proteins↗
PubMed1982

Effect of plicatic acid on human serum complement includes interference with C1 inhibitor function.

In an earlier study, we presented evidence that plicatic acid (PA) activated C in normal human serum (NHS) by an immunoglobulin-independent mechanism that did not involve factor B of the alternative pathway and that required Ca++ for initiation. The present paper further verifies that PA acts through the classical pathway. In PA-treated NHS, titers of C1, C4, C3, and C5 decreased after incubation at 37 degrees C, but in C2-deficient serum, only the titers of C1 and C4 were decreased after identical PA treatment. Activation of purified precursor C1 did not occur when it was incubated with PA at concentrations that would have produced C1 consumption in serum. Thus, PA added to serum initiates activation of the classical pathway, but is incapable of activating C1 directly. PA added to a mixture of native C4 and activated C1s did not alter the kinetics or the extent of C4 inactivation by the C1s. However, when mixtures of C4, C1s, and C1-In were incubated with PA, the ability of the inhibitor to inactivate the C1s was markedly reduced. These data indicate that the mechanism by which PA activates C in serum may involve interference with the normal regulatory mechanism that controls C1 activity.

Angioedema↗
PubMed1982

Familial angioedema associated with C1 esterase-inhibitor deficiency. A new genetic variant in hereditary angioedema?

In the absence of a positive family history for hereditary angioedema (HAE), both siblings in a family were found to have clinical and laboratory findings similar to those in HAE. Both siblings became symptom free after receiving danazol treatment, accompanied by a subnormal rise in C1 esterase inhibitor and C4 levels. As both siblings and their father share HLA haplotype A2, B7, the influence of other HLA loci on the clinical expression of this disorder should be considered. Although the disorder found in these siblings may be inherited as a recessive disorder, which to our knowledge has never been described before, or may have developed as the result of spontaneous genetic mutation, it is more appropriate to classify these two cases as familial angioedema until proved otherwise.

Adolescent↗
PubMed1982

[Studies on relationship between complement and blood coagulation system in toxemia of pregnancy (author's transl)].

Presence of chronic DIC (disseminated intravascular coagulation) eliciting an impeded blood coagulation has been postulated of late as one of the etiology causing toxemia of pregnancy, for which studies have been immunologically made. These theories remain unestablished. In this regard, the role of complement in blood coagulation has been noted, and their correlation is being elucidated. The author introduced a concept of complement to etiological theory of an impeded blood coagulation origin, by which toxemia of pregnancy was studied with emphasis placed on their correlation. The results obtained are as follow: 1) Thrombin and thromboplastin allowed in vitro to decreases the potency of complement, and the lowering also was seen even in the case of simultaneous supplement of urokinase and plasminogen. 2) The decrease also was periodically seen in rabbit's DIC experimentally made. 3) An increase in CH50, C3, C4, and factor B of normal pregnancy were of significance when compared with those of the control (p less than 0.001), while C1 inactivator decreased significantly (p less than 0.001). 4) CH50 was 52.2 +/- 2.4U/ml in severe toxemia, a decrease being of significance (p less than 0.01) as compared with that in third trimester of normal pregnancy. Those other parameters which tended to decrease included hemolytic activity of alternative pathway (AP-CH50), C4, and factor B except C1 inactivator with a trend being high.

Adolescent↗
PubMed1982

Action of a new non-steroid anti-inflammatory drug, carprofen (Ro 20-5720) on activation of the complement system: an in vitro research.

Carprofen (Ro 20-5720), DL-6-chloro-alpha-methyl-carbazole-2-acetic acid, is a new non-steroid anti-inflammatory drug. We have studied the possible in vitro interference of the drug with the activation of human complement system. Carprofen exerts a constant inhibitory action of immunohaemolysis at a concentration of 4.5 X 10(-4)M. At the same concentration the drug is able to prevent the activation of the alternate pathway induced by insulin. Its specific site of action is on C1 and C3 components activation, according to the inhibiting activity on both classic and alternate pathway. However at a lower dosage (7.5 X 10(-6)M) carprofen is able to display an inducing action on the alternate pathway, thus confirming the complex in vitro interferences of the drug with the complement system.

Anti-Inflammatory Agents↗
PubMed1978

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.

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.

Angioedema↗
PubMed1982

Complement, coagulation and fibrinolytic parameters in hereditary angioedema (HAE).

The intrinsic clotting, the kinin generating and the fibrinolytic systems were investigated in 10 patients with hereditary angioedema (HAE), 10 patients with chronic urticaria and 18 healthy volunteers. In spite of the fact that patients suffering from HAE severely lack C1 INH, neither the intrinsic coagulation nor the fibrinolytic systems are impaired. There was a slight decrease of plasma kallikrein--as already known--and moreover a greater decrease in HMW-kininogen, and increase in Factor XII levels. Furthermore, activation of pre-kallikrein was delayed in these patients. These findings make it apparent that lowered HMW-kininogen levels compensate the lack of C1 INH, thus preventing an enhanced activation of the intrinsic clotting and the fibrinolytic systems.

Angioedema↗