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, Volume 49, Issue 5, pp 405–418 | Cite as

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

  • Junzo Kodama
  • Kagehiro Uchida
  • Sanae Yoshimura
  • Yoshiaki Katayama
  • Hideto Kushiro
  • Chikao Yutani
  • Shuji Funahashi
  • Osamu Takamiya
  • Yoko Matsumoto
  • Yoshiki Ando
  • Takenori Hashimoto
  • Kazuyoshi Nagaki
  • Makoto Katori
  • Yasuhiro Uchida
  • Sachiko Ohishi
  • Shinya Inai
Original Articles

Summary

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.

Key words

Hereditary angioneurotic edema Complement Fibrinolysis Coagulation Kallikrein-kinin Exogenous trigger 

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Copyright information

© Springer-Verlag 1984

Authors and Affiliations

  • Junzo Kodama
    • 1
  • Kagehiro Uchida
    • 1
  • Sanae Yoshimura
    • 1
  • Yoshiaki Katayama
    • 1
  • Hideto Kushiro
    • 1
  • Chikao Yutani
    • 1
  • Shuji Funahashi
    • 2
  • Osamu Takamiya
    • 2
  • Yoko Matsumoto
    • 2
  • Yoshiki Ando
    • 2
  • Takenori Hashimoto
    • 2
  • Kazuyoshi Nagaki
    • 3
  • Makoto Katori
    • 4
  • Yasuhiro Uchida
    • 4
  • Sachiko Ohishi
    • 4
  • Shinya Inai
    • 5
  1. 1.National Cardiovascular CenterSuita/OsakaJapan
  2. 2.National Osaka HospitalOsakaJapan
  3. 3.Center for Adult Diseases of OsakaOsakaJapan
  4. 4.School of MedicineKitasato UniversityKanagawaJapan
  5. 5.Osaka Medical CollegeTakatsuki/OsakaJapan

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