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Heterozygous, a1-antitrypsin deficiency may be associated with cold urticaria.

Lindmark, Bertil and Wallengren, Joanna LU orcid (1992) In Allergy 47(5). p.456-458
Abstract
Proteins of the serpin family (serine protease inhibitor) control key steps in the inflammatory, coagulation and complement systems. C1‐inhibitor deficiency predisposes to hereditary angioneurotic oedema, and other serpins control proteolytic enzymes that may cause complement activation or the forming of oedema. We investigated whether deficiency of proteins of the serpin family may predispose to cold urticaria and therefore screened 7 male patients with severe cold urticaria for the presence of deficieney alleles of some of the members of the serpin antiprotease family. There were no findings of C1‐inhibitor, α1‐antitrypsin, α2‐antiplasmin, antithrombin III, tissue plasminogen activator inhibitor or thyroxine binding protein deficiency.... (More)
Proteins of the serpin family (serine protease inhibitor) control key steps in the inflammatory, coagulation and complement systems. C1‐inhibitor deficiency predisposes to hereditary angioneurotic oedema, and other serpins control proteolytic enzymes that may cause complement activation or the forming of oedema. We investigated whether deficiency of proteins of the serpin family may predispose to cold urticaria and therefore screened 7 male patients with severe cold urticaria for the presence of deficieney alleles of some of the members of the serpin antiprotease family. There were no findings of C1‐inhibitor, α1‐antitrypsin, α2‐antiplasmin, antithrombin III, tissue plasminogen activator inhibitor or thyroxine binding protein deficiency. The prevalence of heterozygous α1‐antichymotrypsin deficieney was significantly higher than expected (prevalence ratio 25.8 (95% confidence interval 6.0‐112), p< 0.0001). This finding is in concert with previous studies that have shown lower mean levels of α1‐antichymotrypsin among patients with cold urticaria and suggests that heterozygous deficiency of this antiprotease, which controls neutrophil eathepsin G and mast cell chymase may predispose to cold urticaria. The present series is, however, small and the results need confirmation in larger materials. (Less)
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author
and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Allergy
volume
47
issue
5
pages
456 - 458
publisher
Wiley-Blackwell
external identifiers
  • scopus:0026496199
ISSN
1398-9995
DOI
10.1111/j.1398-9995.1992.tb00663.x
language
English
LU publication?
yes
id
c7bf6ba5-0ba9-4715-a009-cc6b2079a19c
date added to LUP
2019-07-17 16:06:49
date last changed
2021-04-25 03:55:45
@article{c7bf6ba5-0ba9-4715-a009-cc6b2079a19c,
  abstract     = {{Proteins of the serpin family (serine protease inhibitor) control key steps in the inflammatory, coagulation and complement systems. C1‐inhibitor deficiency predisposes to hereditary angioneurotic oedema, and other serpins control proteolytic enzymes that may cause complement activation or the forming of oedema. We investigated whether deficiency of proteins of the serpin family may predispose to cold urticaria and therefore screened 7 male patients with severe cold urticaria for the presence of deficieney alleles of some of the members of the serpin antiprotease family. There were no findings of C1‐inhibitor, α1‐antitrypsin, α2‐antiplasmin, antithrombin III, tissue plasminogen activator inhibitor or thyroxine binding protein deficiency. The prevalence of heterozygous α1‐antichymotrypsin deficieney was significantly higher than expected (prevalence ratio 25.8 (95% confidence interval 6.0‐112), p&lt; 0.0001). This finding is in concert with previous studies that have shown lower mean levels of α1‐antichymotrypsin among patients with cold urticaria and suggests that heterozygous deficiency of this antiprotease, which controls neutrophil eathepsin G and mast cell chymase may predispose to cold urticaria. The present series is, however, small and the results need confirmation in larger materials.}},
  author       = {{Lindmark, Bertil and Wallengren, Joanna}},
  issn         = {{1398-9995}},
  language     = {{eng}},
  number       = {{5}},
  pages        = {{456--458}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Allergy}},
  title        = {{Heterozygous, a1-antitrypsin deficiency may be associated with cold urticaria.}},
  url          = {{http://dx.doi.org/10.1111/j.1398-9995.1992.tb00663.x}},
  doi          = {{10.1111/j.1398-9995.1992.tb00663.x}},
  volume       = {{47}},
  year         = {{1992}},
}