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Complement profile in a Cr inhibitor deficient family

Identifieur interne : 002E48 ( Istex/Checkpoint ); précédent : 002E47; suivant : 002E49

Complement profile in a Cr inhibitor deficient family

Auteurs : K. Sayama [Japon] ; S. Shiraishi [Japon] ; Y. Miki [Japon]

Source :

RBID : ISTEX:6CE538992D4E5C8087CBF3697F6618247A0BB8C4

Abstract

Complement components and anaphylatoxins in a Cr inhibitor (CrINH) deficient family were studied. C4a was increased when CrINH was decreased, and C3a was increased in subjects with systemic lupus erythematosus (SLE)‐like symptoms and with angioedema attacks. Danazol was effective in controlling the clinical as well as complement abnormalities including low CH50, CrINH and C4, which increased within 10 days after danazol treatment was started. Two‐dimensional immunoelectrophoresis of CrINH showed that there was no functionally or electrophoretically abnormal CrINH present before or after danazol treatment. C3a and C4a were considered to play important roles in the pathogenesis of angioedema and associated SLE‐like symptoms.

Url:
DOI: 10.1111/j.1365-2133.1985.tb02410.x


Affiliations:


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ISTEX:6CE538992D4E5C8087CBF3697F6618247A0BB8C4

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<div type="abstract" xml:lang="en">Complement components and anaphylatoxins in a Cr inhibitor (CrINH) deficient family were studied. C4a was increased when CrINH was decreased, and C3a was increased in subjects with systemic lupus erythematosus (SLE)‐like symptoms and with angioedema attacks. Danazol was effective in controlling the clinical as well as complement abnormalities including low CH50, CrINH and C4, which increased within 10 days after danazol treatment was started. Two‐dimensional immunoelectrophoresis of CrINH showed that there was no functionally or electrophoretically abnormal CrINH present before or after danazol treatment. C3a and C4a were considered to play important roles in the pathogenesis of angioedema and associated SLE‐like symptoms.</div>
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