Hereditary angioneurotic edema and thromboembolic diseases: I: How symptoms of acute attacks change with aging.

Localized edema of the larynx and pharynx leading to death from asphyxia has long been recognized as a characteristic symptom of hereditary angioneurotic edema (HANE). Long-term follow-up of younger HANE patients has revealed that transient localized acute attacks of edema affect tissues where the microcirculation maintains the blood supply. However, with aging, HANE attacks precipitate disseminated intravascular coagulation (DIC) or multiple organ failure (MOF). Substitution with a C1-inhibitor (C1-INH) has resulted in a fulminant lethal end with a rapid and profound decrease in antithrombin-III (AT-III) activity. A possible mechanism is as follows: Exogenous stimuli activate plasma proteinase systems with the generation of plasma kallikrein that activates the tissue factor pathway (TF) and liberates bradykinin (BK). In younger patients, BK enhances vascular permeability. In the elderly, activated TF is controlled by tissue factor pathway inhibitor (TFPI) and generates thrombin, which is the target enzyme of AT-III and precipitates DIC or MOF. In elderly patients, the characteristic symptom of HANE is hypercoagulation by age-related changes in the biosynthesis of AT-III or TFPI.

[A surgical case of giant mediastinal teratoma].

A 35-year-old female was admitted with a large abnormal shadow in the left lung field on a chest X-ray. She was examined by chest tomogram, chest CT, MRI and bronchofiberscope. The CT and MRI showed a large tumor mass pressing the left lung and calcified deposits in the central part of the tumor. Bronchofiberscopic findings showed a stenosis of the left main bronchus. The clinical diagnosis was mature teratoma arising from anterior medistinum. She underwent a median sternotomy and the tumor was removed. The size of the resected specimen, filled with sebaceous material, hair and teeth, etc., was 14 x 12 x 10 cm (1,045 g). Histopathological examination of the specimen revealed a mediastinal mature teratoma. She has been well for 16 months postoperatively.

Studies of four Japanese families with hereditary angioneurotic edema: simultaneous activation of plasma protease systems and exogenous triggering stimuli.

Forty-five relatives of 4 families with hereditary angioneurotic edema (HANE) were studied. Twenty-five, including 11 asymptomatic kindreds with the disposition, showed typical changes in complement system compatible with HANE. Follow-up study of HANE patients showed that, even in remission period, complement, coagulation and fibrinolytic systems can be activated. During edema attacks, moderate haemoconcentration and neutrophilia were encountered and kallikrein-kinin system was found to be also activated. Replacement therapy with C 1-inhibitor preparation for an edema attack revealed that clinical improvement paralleled the increase in blood levels of high molecular weight kininogen. Thus, HANE attack is considered to be elicited in kindreds with the hereditary disposition by activation of plasma protease systems, particularly by that of kallikrein-kinin system. On the other hand, exogenous triggers that can initiate activation of the protease systems can be classified into 2, neuro-humoral (sympathetic nerve response) and physico-chemical, categories. Hence, the edema attack of kindreds with the hereditary disposition can at least be modified by the biosynthesis of plasma factors and the individual susceptibility to the liberated catecholamines. These two different reaction processes are considered to be linked by the release of plasminogen activator and/or Hageman factor activating enzyme.

Rapid emergency testing using blood plasma.

A method of blood analysis, using plasma instead of serum, is very valuable in shortening the time for reporting an emergency test by about 50-60%, by eliminating the serum separation step. Good correlation between plasma and serum was obtained for common chemical analyses.

Serological differentiation of strains of group B streptococci by the soft-agar technique.

Soft-agar technique was applied for serological determination of strains of group B streptococci. With representative type strains, their colonial morphology was converted from diffuse- to compact-type growth only by the addition of homologous rabbit anti-serum in the medium and no conversion of colonial morphology was observed by heterologous rabbit antisera. Twenty seven out of 30 fresh isolates obtained from human clinical specimens showed diffuse-type growth in soft-agar medium and were subjected to this examination. Twenty-one strains reacted with a single antiserum, 5 strains showed reactivities to two different antisera although reaction to an antiserum were significantly higher than those of the other antiserum and no reaction was observed with a strain. Twenty single and 5 major serotypes determined by this technique were coincided with those differentiated by Lancefield's precipitin method. From these experimental results, soft-agar technique was regarded being available for serological typing of strains of group B streptococci.