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: Mann, Jake P. (VerfasserIn) , Lenz, Dominic (VerfasserIn) , Stamataki, Zania (VerfasserIn) , Kelly, Deirdre (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 15 February 2023
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
Volltext
Verfasserangaben:Jake P. Mann, Dominic Lenz, Zania Stamataki, and Deirdre Kelly

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520 |a 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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