Abstract:
:Hereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The most widespread underlying genetic deficiency is a heterozygous deficiency of the serine protease inhibitor C1 esterase inhibitor (C1-Inh). In addition to low C4 levels, the most important laboratory parameter for correct diagnosis of HAE or angioedema due to acquired C1-Inh deficiency is reduced C1-Inh function (fC1-Inh). No direct recommendations about the assays for fC1-Inh or sample handling conditions are available, although this would prove especially useful when a laboratory first starts to offer assays on fC1-Inh for HAE diagnosis. In the present study we evaluated the performance of fC1-Inh assays in the 15 different laboratories that are specialised in HAE diagnostics and assessed inter-laboratory variation with each laboratory using their own assays and standards. A double-blind survey was conducted using plasma/serum samples from healthy donors and HAE patients and the uniformity of HAE diagnosis was evaluated. It can be concluded that the diagnosis of fC1-Inh deficiency was made correctly in most cases in this survey. We can recommend the chromogenic assay for the determination of fC1-Inh, while the complex ELISA needs further investigation.
journal_name
J Immunol Methodsjournal_title
Journal of immunological methodsauthors
Wagenaar-Bos IG,Drouet C,Aygören-Pursun E,Bork K,Bucher C,Bygum A,Farkas H,Fust G,Gregorek H,Hack CE,Hickey A,Joller-Jemelka HI,Kapusta M,Kreuz W,Longhurst H,Lopez-Trascasa M,Madalinski K,Naskalski J,Nieuwenhuys E,Pdoi
10.1016/j.jim.2008.06.004subject
Has Abstractpub_date
2008-09-30 00:00:00pages
14-20issue
1-2eissn
0022-1759issn
1872-7905pii
S0022-1759(08)00209-3journal_volume
338pub_type
杂志文章abstract::A procedure is described for the routine detection of RNA transcripts in small numbers of hematopoietic cells growing in semi-solid agar. It is suggested that hybridization depends upon RNA expression and that as few as 2500 mRNA molecules per colony are easily detected. Applications of this technique are described in...
journal_title:Journal of immunological methods
pub_type: 杂志文章
doi:10.1016/0022-1759(86)90430-8
更新日期:1986-10-23 00:00:00
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journal_title:Journal of immunological methods
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journal_title:Journal of immunological methods
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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journal_title:Journal of immunological methods
pub_type: 杂志文章
doi:10.1016/0022-1759(75)90036-8
更新日期:1975-11-01 00:00:00
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journal_title:Journal of immunological methods
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journal_title:Journal of immunological methods
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journal_title:Journal of immunological methods
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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journal_title:Journal of immunological methods
pub_type: 杂志文章
doi:10.1016/0022-1759(84)90451-4
更新日期:1984-08-03 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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更新日期:2002-05-01 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
doi:10.1016/0022-1759(94)00255-u
更新日期:1995-01-27 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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更新日期:1996-05-27 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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更新日期:2017-03-01 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
doi:10.1016/0022-1759(84)90394-6
更新日期:1984-05-11 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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更新日期:1980-01-01 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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journal_title:Journal of immunological methods
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journal_title:Journal of immunological methods
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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更新日期:1995-11-16 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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pub_type: 杂志文章
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更新日期:1996-09-09 00:00:00
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journal_title:Journal of immunological methods
pub_type: 杂志文章
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更新日期:2008-02-29 00:00:00
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pub_type: 杂志文章
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