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Liver disease in adults with severe alpha-1-antitrypsin deficiency

Tanash, Hanan A. LU and Piitulainen, Eeva LU (2019) In Journal of Gastroenterology 54(6). p.541-548
Abstract

Background: The proportion of adults with liver disease due to severe alpha-1-antitrypsin deficiency (AATD), with PiZZ phenotype, is not clear. The markers of the AATD liver disease, how it progresses, and measures for its prevention have not been established. The aim of this study was to analyze the risk of liver disease in individuals with severe AAT deficiency (PiZZ). Methods: Longitudinal clinical and laboratory data were obtained from the Swedish National registers, by cross-linkage between the Swedish national AATD register, the Swedish National Patient Register, the National Cancer Register and the National Causes of Death Register. Results: A total of 1595 PiZZ individuals were included in the analyses. The mean follow-up time... (More)

Background: The proportion of adults with liver disease due to severe alpha-1-antitrypsin deficiency (AATD), with PiZZ phenotype, is not clear. The markers of the AATD liver disease, how it progresses, and measures for its prevention have not been established. The aim of this study was to analyze the risk of liver disease in individuals with severe AAT deficiency (PiZZ). Methods: Longitudinal clinical and laboratory data were obtained from the Swedish National registers, by cross-linkage between the Swedish national AATD register, the Swedish National Patient Register, the National Cancer Register and the National Causes of Death Register. Results: A total of 1595 PiZZ individuals were included in the analyses. The mean follow-up time was 12 years (range 0.3–24). The mean number of follow-ups was 5 (range 2–15). Two or more liver function tests (LFTs) were available in 1123 individuals, and 26% of them (n = 290) had repeated elevated LFTs during the follow-up. The prevalence of any liver disease was 10% (n = 155). Liver cirrhosis was found in 7% of the individuals (n = 116) and hepatocellular carcinoma in 2% (n = 29). The mean age at the onset of liver disease was 61 (SD 15) years. In multivariate analyses, the male gender, age over 50 years, repeated elevated LFTs, hepatitis virus infection, and a diagnosis of diabetes were associated with increased risk of developing liver disease in adulthood (p < 0.01). Conclusion: The prevalence of liver disease in adult PiZZ individuals is 10%. Age over 50 years, the male gender, repeated elevated liver enzymes, hepatitis, and the presence of diabetes mellitus are risk factors for developing liver disease.

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author
and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Alpha-1-antitrypsin deficiency, Liver disease, Liver function tests
in
Journal of Gastroenterology
volume
54
issue
6
pages
541 - 548
publisher
Springer
external identifiers
  • pmid:30680526
  • scopus:85060595701
ISSN
0944-1174
DOI
10.1007/s00535-019-01548-y
language
English
LU publication?
yes
id
eab9db12-b488-45a2-8aa5-6e93b9181ca5
date added to LUP
2019-02-06 08:04:59
date last changed
2024-10-15 18:07:53
@article{eab9db12-b488-45a2-8aa5-6e93b9181ca5,
  abstract     = {{<p>Background: The proportion of adults with liver disease due to severe alpha-1-antitrypsin deficiency (AATD), with PiZZ phenotype, is not clear. The markers of the AATD liver disease, how it progresses, and measures for its prevention have not been established. The aim of this study was to analyze the risk of liver disease in individuals with severe AAT deficiency (PiZZ). Methods: Longitudinal clinical and laboratory data were obtained from the Swedish National registers, by cross-linkage between the Swedish national AATD register, the Swedish National Patient Register, the National Cancer Register and the National Causes of Death Register. Results: A total of 1595 PiZZ individuals were included in the analyses. The mean follow-up time was 12 years (range 0.3–24). The mean number of follow-ups was 5 (range 2–15). Two or more liver function tests (LFTs) were available in 1123 individuals, and 26% of them (n = 290) had repeated elevated LFTs during the follow-up. The prevalence of any liver disease was 10% (n = 155). Liver cirrhosis was found in 7% of the individuals (n = 116) and hepatocellular carcinoma in 2% (n = 29). The mean age at the onset of liver disease was 61 (SD 15) years. In multivariate analyses, the male gender, age over 50 years, repeated elevated LFTs, hepatitis virus infection, and a diagnosis of diabetes were associated with increased risk of developing liver disease in adulthood (p &lt; 0.01). Conclusion: The prevalence of liver disease in adult PiZZ individuals is 10%. Age over 50 years, the male gender, repeated elevated liver enzymes, hepatitis, and the presence of diabetes mellitus are risk factors for developing liver disease.</p>}},
  author       = {{Tanash, Hanan A. and Piitulainen, Eeva}},
  issn         = {{0944-1174}},
  keywords     = {{Alpha-1-antitrypsin deficiency; Liver disease; Liver function tests}},
  language     = {{eng}},
  month        = {{01}},
  number       = {{6}},
  pages        = {{541--548}},
  publisher    = {{Springer}},
  series       = {{Journal of Gastroenterology}},
  title        = {{Liver disease in adults with severe alpha-1-antitrypsin deficiency}},
  url          = {{http://dx.doi.org/10.1007/s00535-019-01548-y}},
  doi          = {{10.1007/s00535-019-01548-y}},
  volume       = {{54}},
  year         = {{2019}},
}