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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Balcar, L; Fromme, M; Kappe, N; Schaefer, B; Frankova, S; van Melkebeke, L; Stolk, J; Jachs, M; Semmler, G; Hofer, BS; Tergast, TL; Rieland, H; Karl, AS; Sperl, J; Wagner, M; Pons, M; Hofer, H; Peck-Radosavljevic, M; Trauner, M; Reiberger, T; Maasoumy, B; Zoller, H; van Hoek, B; Verbeek, J; Strnad, P; Mandorfer, M.
Severe alpha-1 antitrypsin deficiency is associated with a higher risk of complications after first decompensation than other aetiologies of cirrhosis
JHEP REP. 2025; 7(6): 101398 Doi: 10.1016/j.jhepr.2025.101398
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Co-Autor*innen der Med Uni Graz
Trauner Michael
Wagner Martin
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Abstract:
Background & Aims: Alpha-1 antitrypsin deficiency (AATD) causes/predisposes to advanced chronic liver disease. However, the role of the SERPINA1 Pi & lowast;ZZ genotype in patients with decompensated cirrhosis is unclear. Thus, we evaluated the impact of the Pi & lowast;ZZ genotype on the disease course after the first hepatic decompensation event. Methods: We retrospectively included 59 adults with decompensated cirrhosis and severe AATD (Pi & lowast;ZZ) from 12 European tertiary care centres. First decompensation was considered as baseline. To compare the course of AATD to other cirrhosis aetiologies, we applied propensity score matching for Child-Turcotte-Pugh (CTP) score as well as age/sex. Patients were followed until further decompensation, liver transplantation or liver-related death. Results: Most patients were male (74.6%), with a mean age of 55 years. The most common type of first decompensation was ascites (n = 40; 67.8%), followed by variceal bleeding (n = 13; 22.0%) and overt hepatic encephalopathy (n = 6; 10.2%). Median CTP and MELD (model for end-stage liver disease) scores at first decompensation were 8 and 14, respectively. Median MELD scores were 16 and 20 points at listing and liver transplantation (median time on list: 2.9 [IQR 1.1-7.2] months), respectively. Patients with other aetiologies (subdistribution hazard ratio: steatotic liver disease: 0.62, 95% CI 0.44-0.88, p = 0.007; abstinent alcohol-associated liver disease: 0.50, 95% CI 0.35-0.71, p <0.001; hepatitis C virus-associated cirrhosis: 0.56, 95% CI 0.37-0.83, p = 0.004) had a significantly lower risk of further hepatic decompensation, liver transplantation, or liver-related death, compared to those with Pi & lowast;ZZ. Exchanging further decompensation with acute-on-chronic liver failure yielded similar results. Conclusion: Our study defines the course of decompensated cirrhosis in patients with severe AATD (Pi & lowast;ZZ), who are particularly prone to complications of cirrhosis and exhibit a more progressive disease course than those with cirrhosis of other aetiologies. (c) 2025 The Authors. Published by Elsevier B.V. on behalf of European Association for the Study of the Liver (EASL). This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Find related publications in this database (Keywords)
AATD
advanced chronic liver disease
portal hypertension
ascites
variceal bleeding
hepatic encephalopathy
acute-on-chronic liver failure
liver transplantation
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