PMID- 30680526
OWN - NLM
STAT- MEDLINE
DCOM- 20200619
LR  - 20200619
IS  - 1435-5922 (Electronic)
IS  - 0944-1174 (Print)
IS  - 0944-1174 (Linking)
VI  - 54
IP  - 6
DP  - 2019 Jun
TI  - Liver disease in adults with severe alpha-1-antitrypsin deficiency.
PG  - 541-548
LID - 10.1007/s00535-019-01548-y [doi]
AB  - 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.
FAU - Tanash, Hanan A
AU  - Tanash HA
AD  - Department of Respiratory Medicine and Allergology Malmo, Skane University 
      Hospital, Lund University, Jan Waldenstroms gata 24, plan 4, 205 02, Malmo, 
      Sweden. Hanan.Tanash@med.lu.se.
FAU - Piitulainen, Eeva
AU  - Piitulainen E
AD  - Department of Respiratory Medicine and Allergology Malmo, Skane University 
      Hospital, Lund University, Jan Waldenstroms gata 24, plan 4, 205 02, Malmo, 
      Sweden.
LA  - eng
PT  - Journal Article
DEP - 20190124
PL  - Japan
TA  - J Gastroenterol
JT  - Journal of gastroenterology
JID - 9430794
SB  - IM
MH  - Adult
MH  - Age Factors
MH  - Diabetes Mellitus/*epidemiology
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Liver Diseases/*epidemiology/etiology/physiopathology
MH  - Liver Function Tests
MH  - Longitudinal Studies
MH  - Male
MH  - Middle Aged
MH  - Registries
MH  - Risk Factors
MH  - Sex Factors
MH  - Sweden/epidemiology
MH  - alpha 1-Antitrypsin Deficiency/*complications/physiopathology
PMC - PMC6536463
OTO - NOTNLM
OT  - Alpha-1-antitrypsin deficiency
OT  - Liver disease
OT  - Liver function tests
COIS- The authors report no conflicts of interest. The authors alone are responsible 
      for the content and writing of the paper.
EDAT- 2019/01/27 06:00
MHDA- 2020/06/20 06:00
PMCR- 2019/01/24
CRDT- 2019/01/26 06:00
PHST- 2018/08/06 00:00 [received]
PHST- 2019/01/14 00:00 [accepted]
PHST- 2019/01/27 06:00 [pubmed]
PHST- 2020/06/20 06:00 [medline]
PHST- 2019/01/26 06:00 [entrez]
PHST- 2019/01/24 00:00 [pmc-release]
AID - 10.1007/s00535-019-01548-y [pii]
AID - 1548 [pii]
AID - 10.1007/s00535-019-01548-y [doi]
PST - ppublish
SO  - J Gastroenterol. 2019 Jun;54(6):541-548. doi: 10.1007/s00535-019-01548-y. Epub 
      2019 Jan 24.