C1 inhibitor deficiency: consensus document
نویسندگان
چکیده
منابع مشابه
C1 inhibitor deficiency: 2014 United Kingdom consensus document
C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient patients, representing a joint venture between the United Kingdom Primary Immunodeficiency Network and Hereditary Angioedema UK. To develop the consen...
متن کاملCLINICAL REVIEW Acquired C1 Esterase Inhibitor Deficiency
Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. I...
متن کاملLymphoproliferative disease and acquired C1 inhibitor deficiency.
Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal gammopathies of unknown significance and 9 have non-Hodgkin's lymphoma. Our series suggest that different forms of B cell disorders coexist and/or ...
متن کامل[Practical management of C1 inhibitor deficiency].
C1 inhibitor deficiency is a rare syndrome clinically characterized by recurrent episodes of swelling of subcutaneous tissue or angioedema. It can involve the skin, upper respiratory airways and abdomen. There are two main types: hereditary and acquired. Angioedema can involve practically any part of the cutaneous surface, it can cause lethal laryngeal edema and can present as gastrointestinal ...
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This UIP document provides an update on venous symptoms in CO and C1 patients. The correlation between venous symptoms and the presence of telangiectases and/or reticular veins is one of the most controversial topics in chronic venous disorders. As symptoms may be non-specific of chronic venous disease, it is important to differentiate venous symptoms from symptoms of other causes. Some data fr...
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ژورنال
عنوان ژورنال: Clinical & Experimental Immunology
سال: 2005
ISSN: 0009-9104,1365-2249
DOI: 10.1111/j.1365-2249.2005.02726.x