Lenz, Dominic and Abdulaziz, Muhammad and Peters, Bianca and Wagner, Matias and Schlieben, Lea D. and Corman, Victor M. and Baumann, Ulrich and Bufler, Philip and Dattner, Tal and Ganschow, Rainer and Genzel, Kristin and Hammann, Nicole and Hartleif, Steffen and Hegen, Bianca and Henning, Stephan and Hoerning, Andre and Jankofsky, Martin and Junge, Norman and Kathemann, Simone and Knoppke, Birgit and Kohl-Sobania, Martina and Laass, Martin and Lainka, Elke and Lurz, Eberhard and Melter, Michael and Mueller, Hanna and Pilic, Denisa and Ries, Markus and Schiefele, Lisa and Schwerd, Tobias and Sturm, Ekkehard and Wegner, Mechtild and Urschitz, Michael S. and Garbade, Sven F. and Wenning, Daniel and Drosten, Christian and Fichtner, Alexander and Koelker, Stefan and Hoffmann, Georg F. and Prokisch, Holger and Staufner, Christian (2025) Paediatric acute liver failure: A prospective, nationwide, population-based surveillance study in Germany. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 81 (3). pp. 653-662. ISSN 0277-2116, 1536-4801
Full text not available from this repository. (Request a copy)Abstract
ObjectivesPaediatric acute liver failure (PALF) is a rare but life-threatening condition, yet comprehensive epidemiological data in Germany are lacking. Our study aimed to systematically analyse incidence, aetiology, and outcome of PALF in Germany.MethodsIn a nationwide, population-based surveillance study, cases of PALF (defined following the PALF study group inclusion criteria) were queried from 2016 to 2018 through the German Paediatric Surveillance Unit (ESPED). Demographic, clinical, laboratory, therapeutic, and outcome data were collected and analysed. In case of unexplained aetiology, whole exome and virus sequencing was offered as a complementary diagnostic.ResultsOver the 3-year period, 148 cases were reported, yielding an estimated incidence of 3.7 per 1 million children per year. Neonates and infants were predominantly affected (45% of the cases); median age at PALF was 1.2 years (range: 0-17.9 years). Metabolic/genetic diseases were the most common cause (23%), followed by infectious causes (17%). The overall diagnostic yield was 73%, diagnosis remained unknown in 40 cases. Clinical outcome was age-dependent: new-borns showed a significant higher lethality (42%), followed by infants (29%), toddlers (15%), and school children (12%). Liver transplantation was reported in 22% of cases.ConclusionsThis study provides comprehensive insights into PALF epidemiology in Germany. Metabolic/genetic causes and infectious diseases were most common. Advances in standardised diagnostic work-up and genetic analysis have enhanced diagnostic yield, yet mortality remains substantial, particularly among neonates. Further research is warranted to improve diagnostic accuracy, therapeutic outcomes, and overall management of PALF.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | EXPERIENCE; ETIOLOGY; DISEASE; aetiology; ESPED; incidence; outcome; PALF |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Kinder- und Jugendmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 26 Mar 2026 10:08 |
| Last Modified: | 26 Mar 2026 10:08 |
| URI: | https://pred.uni-regensburg.de/id/eprint/68005 |
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