Common mechanisms in pediatric acute liver failure
Acute liver failure (ALF) is a rare but potentially fatal disease in children. The etiology is multifactorial, including infection, autoimmune, and genetic disorders, as well as indeterminate hepatitis, which has a higher requirement for liver transplantation. Activation of the innate and adaptive i...
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| Hauptverfasser: | , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
15 February 2023
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| In: |
Trends in molecular medicine
Year: 2023, Jahrgang: 29, Heft: 3, Pages: 228-240 |
| ISSN: | 1471-499X |
| DOI: | 10.1016/j.molmed.2022.11.006 |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.molmed.2022.11.006 Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1471491422002994 
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| Verfasserangaben: | Jake P. Mann, Dominic Lenz, Zania Stamataki, and Deirdre Kelly |
| Zusammenfassung: | Acute liver failure (ALF) is a rare but potentially fatal disease in children. The etiology is multifactorial, including infection, autoimmune, and genetic disorders, as well as indeterminate hepatitis, which has a higher requirement for liver transplantation. Activation of the innate and adaptive immune systems leads to hepatocyte-specific injury which is mitigated by T regulatory cell activation. Recovery of the native liver depends on activation of apoptotic and regenerative pathways, including the integrated stress response (ISR; e.g., PERK), p53, and HNF4α. Loss-of-function mutations in these pathways cause recurrent ALF in response to non-hepatotropic viruses. Deeper understanding of these mechanisms will lead to improved diagnosis, management, and outcomes for pediatric ALF. |
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| Beschreibung: | Online verfügbar 7 Dezember 2022, Artikelversion 15 Februar 2023 Gesehen am 28.03.2023 |
| Beschreibung: | Online Resource |
| ISSN: | 1471-499X |
| DOI: | 10.1016/j.molmed.2022.11.006 |