[A case report of hereditary angioedema and studies on the serum components of complement, C1-inactivator and proteinase inhibitors during edema attack].
Sixteen years old girl was admitted because of for the past ten years' frequent edema attack and abdominal pain. Laboratory examination revealed hypocomplementemia, marked depletion of the fourth component of complement and low level of C1-inactivator. Familial studies revealed that her mother was also hypocomplementemic and in low level of C1-inactivator. Serial studies performed on the alterlation of components of complement, C1-inactivator, alpha 1-antitrypsin, antithrombin III, and alpha 2-macroglobulin during edema attack. The fourth component of complement and C1-inactivator were markedly depleted in remission and attack. Remarkable depletion was found in antithrombin III and esterase inhibition activity of C1-inactivator during attack. In contrast, alpha 1-antitrypsin and alpha 2-macroglobulin did not change. The present study may explain that Hageman factor fragments, activated by C1s, promotes kinin generation via kalikrein activation. And the condition that complete functional deficiency of C1-inactivator was main role in this circuit. Fibrynolysis and late components of complement was less influence on edema attack.
D000293:Adolescent; D000799:Angioedema / Q000209:etiology / Q000235:genetics* / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000097:blood*; D003165:Complement System Proteins / Q000032:analysis*; D005260:Female; D006801:Humans; D011480:Protease Inhibitors / Q000097:blood*
D003174:Complement C1 Inactivator Proteins; D011480:Protease Inhibitors; D003165:Complement System Proteins