Severe alpha-1 antitrypsin deficiency is associated with a higher risk of complications after first decompensation than other aetiologies of cirrhosis
Alpha-1 antitrypsin deficiency is an inherited disease that affects the lung and the liver. Carrying two severely dysfunctional copies of the alpha-1 antitrypsin gene may cause advanced chronic liver disease/cirrhosis. Affected individuals with a first complication of cirrhosis are more prone to developing further liver-related events (including multiorgan dysfunction) and requiring liver transplantation (which cures the inherited liver disease) compared to patients who have similarly advanced liver disease. These findings should prompt the development of disease-modifying treatments and early listing for liver transplantation.
Authors
- Mattias Mandorfer (ORCID: https://orcid.org/0000-0003-2330-0017)
- Malin Fromme (ORCID: https://orcid.org/0000-0003-0382-5705)
- Martin Wagner (ORCID: https://orcid.org/0000-0001-9206-1326)
- Pavel Strnad (ORCID: https://orcid.org/0000-0003-3164-9614)
- Benjamin Maasoumy (ORCID: https://orcid.org/0000-0002-3250-3177)
- Mònica Pons (ORCID: https://orcid.org/0000-0002-0985-3320)
- Jef Verbeek (ORCID: https://orcid.org/0000-0002-1549-8003)
- Jan Stolk (ORCID: https://orcid.org/0000-0002-7887-386X)
- Lukas Van Melkebeke (ORCID: https://orcid.org/0000-0003-0361-1176)
- Tammo Lambert Tergast (ORCID: https://orcid.org/0000-0003-3908-6297)
- Georg Semmler (ORCID: https://orcid.org/0000-0002-0411-166X)
- Markus Peck‐Radosavljevic (ORCID: https://orcid.org/0000-0002-0597-2728)
- Thomas Reiberger (ORCID: https://orcid.org/0000-0002-4590-3583)
- Benedikt Silvester Hofer (ORCID: https://orcid.org/0000-0003-3403-3372)
- Jan Šperl (ORCID: https://orcid.org/0000-0001-8619-2610)
- Mathias Jachs (ORCID: https://orcid.org/0000-0003-2871-4147)
- Michael A Trauner (ORCID: https://orcid.org/0000-0002-1275-6425)
- Bart van Hoek (ORCID: https://orcid.org/0000-0001-6527-764X)
- Soňa Fraňková (ORCID: https://orcid.org/0000-0002-1462-5920)
- Naomi Kappe (ORCID: https://orcid.org/0000-0003-4614-1477)
- Lorenz Balcar (ORCID: https://orcid.org/0000-0002-6708-3061)
- Benedikt Schaefer (ORCID: https://orcid.org/0000-0001-8690-2774)
- Harald Hofer
- Anna Sophie Karl
- Heinz Zoller
- Hannah Rieland
Institutions
- Universitat Autònoma de Barcelona (ES)
- Innsbruck Medical University (AT)
- Universität Innsbruck (AT)
- Medical University of Graz (AT)
- Leiden University Medical Center (NL)
- Medizinische Hochschule Hannover (DE)
- Klinikum Klagenfurt (AT)
- Vall d'Hebron Institut de Recerca (ES)
- Klinikum Wels-Grieskirchen (AT)
- Vall d'Hebron Hospital Universitari (ES)
- ERN GUARD-Heart (NL)
- Medical University of Vienna (AT)
- RWTH Aachen University (DE)
- KU Leuven (BE)
Publication Details
- Journal
- JHEP Reports
- Published
- 2025-03-20
- DOI
- https://doi.org/10.1016/j.jhepr.2025.101398
- Citations
- 2
- Primary Topic
- Liver Disease and Transplantation
- Type
- article
- Field-Weighted Citation Impact
- 2.64
Funders
- CSL Behring
- Deutsche Forschungsgemeinschaft
- RWTH Aachen University
- Interdisziplinäres Zentrum für Klinische Forschung, Universitätsklinikum Würzburg