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Familial α1‐Antichymotrypsin Deficiency

ERIKSSON, STEN LU ; LINDMARK, BERTIL and LILJA, HANS LU orcid (1986) In Acta Medica Scandinavica 220(5). p.447-453
Abstract

ABSTRACT We studied patients and their relatives with partial deficiency, approximately 50% of normal plasma levels, of α1‐antichymotrypsin (ACT), an acute phase reactant with anti‐cathepsin G activity. Six of eight ACT deficient individuals, over 25 years of age, had liver and three of eight lung manifestations, varying from severe disease to subtle laboratory abnormalities. The ACT of deficient individuals (who are heterozygotes for a rare gene, q=0.003) had normal crossed immunoelectrophoretic properties. The abnormal gene is inherited in an autosomal, dominant way. The results suggest that deficiency of this antiprotease, which also has immune response modulating properties, may predispose to liver and lung disease. 1986... (More)

ABSTRACT We studied patients and their relatives with partial deficiency, approximately 50% of normal plasma levels, of α1‐antichymotrypsin (ACT), an acute phase reactant with anti‐cathepsin G activity. Six of eight ACT deficient individuals, over 25 years of age, had liver and three of eight lung manifestations, varying from severe disease to subtle laboratory abnormalities. The ACT of deficient individuals (who are heterozygotes for a rare gene, q=0.003) had normal crossed immunoelectrophoretic properties. The abnormal gene is inherited in an autosomal, dominant way. The results suggest that deficiency of this antiprotease, which also has immune response modulating properties, may predispose to liver and lung disease. 1986 Association for the Publication of the Journal of Internal Medicine

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Please use this url to cite or link to this publication:
author
; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
deficiency, heredity, liver disease, lung disease, α ‐antichymotrypsin
in
Acta Medica Scandinavica
volume
220
issue
5
pages
447 - 453
publisher
Wiley-Blackwell
external identifiers
  • scopus:0022838995
  • pmid:3492865
ISSN
0001-6101
DOI
10.1111/j.0954-6820.1986.tb02794.x
language
English
LU publication?
yes
id
e0c0c593-03f1-4bce-85d0-c3c40fc73dae
date added to LUP
2022-12-06 17:02:27
date last changed
2024-01-03 19:32:27
@article{e0c0c593-03f1-4bce-85d0-c3c40fc73dae,
  abstract     = {{<p>ABSTRACT We studied patients and their relatives with partial deficiency, approximately 50% of normal plasma levels, of α<sub>1</sub>‐antichymotrypsin (ACT), an acute phase reactant with anti‐cathepsin G activity. Six of eight ACT deficient individuals, over 25 years of age, had liver and three of eight lung manifestations, varying from severe disease to subtle laboratory abnormalities. The ACT of deficient individuals (who are heterozygotes for a rare gene, q=0.003) had normal crossed immunoelectrophoretic properties. The abnormal gene is inherited in an autosomal, dominant way. The results suggest that deficiency of this antiprotease, which also has immune response modulating properties, may predispose to liver and lung disease. 1986 Association for the Publication of the Journal of Internal Medicine</p>}},
  author       = {{ERIKSSON, STEN and LINDMARK, BERTIL and LILJA, HANS}},
  issn         = {{0001-6101}},
  keywords     = {{deficiency; heredity; liver disease; lung disease; α ‐antichymotrypsin}},
  language     = {{eng}},
  number       = {{5}},
  pages        = {{447--453}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Acta Medica Scandinavica}},
  title        = {{Familial α<sub>1</sub>‐Antichymotrypsin Deficiency}},
  url          = {{http://dx.doi.org/10.1111/j.0954-6820.1986.tb02794.x}},
  doi          = {{10.1111/j.0954-6820.1986.tb02794.x}},
  volume       = {{220}},
  year         = {{1986}},
}