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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
3
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pubmed:dateCreated |
1997-1-21
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pubmed:abstractText |
We looked for autoantibodies to C1 inhibitor (C1-INH) and evaluated the relationship of their presence to the associated lymphoproliferative diseases and to the cleaved form of C1-INH in 13 patients with acquired C1-INH deficiency (acquired angio-oedema (AAE)). At the time of manifestation of angio-oedema symptoms or within a few years the following diseases were diagnosed: liver angioma (n = 1), M-components (n = 7, one of whom also had echinococcal liver cysts), breast cancer (n = 1), chronic lymphocytic leukaemia (CLL; n = 1); three patients had no associated disease. Anti-C1-INH autoantibodies, measured both as immunoglobulin binding to C1-INH immobilized onto microtitre plates (ELISA) and as plasma inhibitory activity of C1-INH function, were found in 12 patients. Binding of C1-INH to paraproteins, transferred to Immobilon after agarose gel electrophoresis, was detectable in five of seven M-components associated with AAE. Immunoblotting analysis of SDS-PAGE-separated plasma demonstrated that C1-INH circulated in the cleaved 96-kD form in the 12 patients with autoantibodies, but not in the one without. In conclusion, the large majority of our patients have autoantibodies to C1-INH. Circulating autoantibodies are necessary for the generation of cleaved C1-INH. The paraproteins associated with AAE are frequently autoantibodies to C1-INH and thus account for its consumption.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Dec
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pubmed:issn |
0009-9104
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
106
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
475-80
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pubmed:dateRevised |
2008-11-20
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pubmed:meshHeading |
pubmed-meshheading:8973615-Adult,
pubmed-meshheading:8973615-Aged,
pubmed-meshheading:8973615-Angioedema,
pubmed-meshheading:8973615-Autoantibodies,
pubmed-meshheading:8973615-Complement C1 Inactivator Proteins,
pubmed-meshheading:8973615-Complement C4,
pubmed-meshheading:8973615-Female,
pubmed-meshheading:8973615-Humans,
pubmed-meshheading:8973615-Lymphoproliferative Disorders,
pubmed-meshheading:8973615-Male,
pubmed-meshheading:8973615-Middle Aged
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pubmed:year |
1996
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pubmed:articleTitle |
Relevance of lymphoproliferative disorders and of anti-C1 inhibitor autoantibodies in acquired angio-oedema.
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pubmed:affiliation |
Institute of Internal Medicine, University of Milan, Italy.
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pubmed:publicationType |
Journal Article
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