C1-inhibitor deficiency can be inherited or acquired; both conditions lead to recurrent angioedema that can be life threatening when the larynx is involved (hereditary angioedema, HAE; acquired angioedema, AAE). The genetic defect is due to the heterozygous deficiency of C1-Inh that is transmitted...
C1-inhibitor deficiency (hereditary angioedema): case report and review. JJ Burge - 《Virginia Medical》 被引量: 1发表: 1983年 Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Two hundred and twenty-six patients with inherited C1 inhibitor (...
The disease is transmitted as an autosomal dominant trait and two variants have been described: type I characterized by functional and antigenic C1-inhibitor (C1-INH) deficiency and type II with normal or upper normal C1-INH antigenic levels but no functional activity. The clinical picture is ...
Acquired C1 esterase inhibitor deficiency is a clinical syndrome closely resembling hereditary angioedema in which most patients have an associated malignancy of B cell lineage. Sera from 33 patients with B cell neoplasms were assayed for C1 esterase inhibitor level by rocket immunoelectrophoresis. The ...
Acquired C1-inhibitor (C1 INH) deficiency is usually found in association with an underlying disease that is believed to be responsible for increased C1 INH catabolism, ultimately leading to the development of C1 INH deficiency. We report a remarkable patient with acquired C1 INH deficiency in whom...
Autoimmune C1‐inhibitor deficiency C1-inhibitor (C1-inh) is a plasma glycoprotein which belongs to the superfamily of serine protease inhibitors (serpins) and inactivates proteases of the co... K Whaley,RB Sim,S He - 《Clinical & Experimental Immunology》 被引量: 0发表: 1996年 Autoantibody ...
C1 inhibitorecallantideicatibanttranexamic acidINTRODUCTION: is a localized and self-limiting edema of the subcutaneous and submucosal tissue. with (C1-INH-) is the best characterized form of . In C1-INH-, the reduced plasma levels of C1-INH cause instability of the contact system with release...
In two patients with angioedema and attacks of abdominal pain due to C1 inhibitor deficiency, long-term treatment with EACA (for more than 2 decades in one of them) was associated with a substantial decrease in the frequency and severity of episodes. One patient had definite hereditary ...
Acquired C1 Inhibitor Deficiency with Angioedema Symptoms in a Patient Infected with Echinococcus granulosus A patient with echinococcosis, acquired deficiency of the inhibitor of the activated first component of complement and angioedema symptoms has been studied... M Cicardi,D Frangi,L Bergamaschini,....