Studies of four Japanese families with hereditary angioneurotic edema: simultaneous activation of plasma protease systems and exogenous triggering stimuli
| Autor: | Osamu Takamiya, Sachiko Oh-ishi, Yasuhiro Uchida, Shinya Inai, Sanae Yoshimura, Hideto Kushiro, Kazuyoshi Nagaki, Yoshiaki Katayama, Shuji Funahashi, Chikao Yutani, Yoshiki Ando, Makoto Katori, Takenori Hashimoto, Yoko Matsumoto, Junzo Kodama, Kagehiro Uchida |
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| Rok vydání: | 1984 |
| Předmět: |
Male
medicine.medical_specialty Hereditary angioneurotic edema High-molecular-weight kininogen medicine.medical_treatment Kinins Complement C1 Inactivator Proteins Fibrinolytic Agents Japan Stress Physiological Internal medicine Edema Fibrinolysis medicine Humans Angioedema Protease business.industry Coagulants Danazol Hematology General Medicine Complement System Proteins Neutrophilia Complement system Pedigree Cold Temperature Endocrinology Tranexamic Acid Immunology Female Kallikreins medicine.symptom business Plasminogen activator Peptide Hydrolases |
| Zdroj: | Blut. 49(5) |
| ISSN: | 0006-5242 |
| Popis: | 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 $$\bar l$$ -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. |
| Databáze: | OpenAIRE |
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