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KMID : 0390019930030010121
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Pediatric Allergy and Respiratory Disease
1993 Volume.3 No. 1 p.121 ~ p.127
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Two cases of angioedema due to CI inactivator deficiency
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Abstract
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C1 inactivator belongs to a group of serum protease inhibitors that includes antithrombin III and alpha-1 proteasel inhibitor. C1 inactivator has a modulating effect on the activation of various pathways including the complement, fibrinolytic,
and
kinin
pathways. A deficiency of C1 inactivator allows for non-physiologic and inappropriate activation of these pathways which results in angioedema.
We experienced 2 cases of angioedema due to C1 inactivator deficiency in a 14 year-old boy and a 7 year-old boy who had suffered from recurrent dermographism with pruritus since 5 years ago and 6 months ago. After admission, they were diagnosed
angioedema due to C1 inactivator deficiency by the clinical manifestations and laboratory tests including allergic skin test, complement assay, C1 inactivator functional assay. We started treatment with Azeptin(Azelastine HC1) and shall observe
following clinical course. The cases were prensented with brief review of literature.
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KEYWORD
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