TY - JOUR
T1 - Advances in medical management of acute liver failure in children
T2 - promoting native liver survival
AU - Deep, Akash
AU - Alexander, Emma C.
AU - Bulut, Yonca
AU - Fitzpatrick, Emer
AU - Grazioli, Serge
AU - Heaton, Nigel
AU - Dhawan, Anil
N1 - Funding Information:
AkD is Chair of Scientific Affairs of the European Society of Pediatric and Neonatal Intensive Care (ESPNIC) and Chair of the Working Group for Liver Failure in ESPNIC. AnD is a recipient of a National Institute for Health Research i4i grant to develop treatment for ALF with alginate microbead embedded hepatocytes and a Medical Research Council (MRC) grant for a first in human clinical trial of alginate embedded human hepatocytes as treatment for ALF (MRC MR/V038583/1); has been paid by Ambys and Johnson & Johnson for consultation; has a patent pending approval for alginate microbead manufacturing, and is part of the European Society for Paediatric Gastroenterology Hepatology and Nutrition. ALF guideline group. EF is in receipt of an MRC grant (MRC MR/V038583/1) for the research of hepatocyte transplantation; and has received payment for organising a Falk Symposium (unconnected). All other authors declare no competing interests.
Publisher Copyright:
© 2022 Elsevier Ltd
PY - 2022/9/14
Y1 - 2022/9/14
N2 - Paediatric acute liver failure (PALF) is defined as a biochemical evidence of acute liver injury in a child with no previous history of chronic liver disease characterised by an international normalised ratio (INR) of 1·5 or more unresponsive to vitamin K with encephalopathy, or INR of 2·0 or more with or without encephalopathy. PALF can rapidly progress to multiorgan dysfunction or failure. Although the transplant era has substantially changed the outlook for these patients, transplantation itself is not without risks, including those associated with life-long immunosuppression. Consequently, there has been an increased focus on improving medical management to prioritise bridging of patients to native liver survival, which is possible due to improved understanding of the underlying pathophysiology of multiorgan involvement in PALF. In this Review, we discuss recent advances in the medical management of PALF with an aim of reducing the need for liver transplantation. The Review will focus on the non-specific immune-mediated inflammatory response, extracorporeal support devices, neuromonitoring and neuroprotection, and emerging cellular and novel future therapeutic options.
AB - Paediatric acute liver failure (PALF) is defined as a biochemical evidence of acute liver injury in a child with no previous history of chronic liver disease characterised by an international normalised ratio (INR) of 1·5 or more unresponsive to vitamin K with encephalopathy, or INR of 2·0 or more with or without encephalopathy. PALF can rapidly progress to multiorgan dysfunction or failure. Although the transplant era has substantially changed the outlook for these patients, transplantation itself is not without risks, including those associated with life-long immunosuppression. Consequently, there has been an increased focus on improving medical management to prioritise bridging of patients to native liver survival, which is possible due to improved understanding of the underlying pathophysiology of multiorgan involvement in PALF. In this Review, we discuss recent advances in the medical management of PALF with an aim of reducing the need for liver transplantation. The Review will focus on the non-specific immune-mediated inflammatory response, extracorporeal support devices, neuromonitoring and neuroprotection, and emerging cellular and novel future therapeutic options.
UR - http://www.scopus.com/inward/record.url?scp=85138044772&partnerID=8YFLogxK
U2 - 10.1016/S2352-4642(22)00190-0
DO - 10.1016/S2352-4642(22)00190-0
M3 - Review article
C2 - 35931098
AN - SCOPUS:85138044772
SN - 2352-4650
VL - 6
SP - 725
EP - 737
JO - The Lancet Child and Adolescent Health
JF - The Lancet Child and Adolescent Health
IS - 10
ER -