Approaches to acute liver failure in children

Abstract

Acute liver failure (ALF) is a rare but often fatal disorder in childhood. Its aetiology includes infections, toxins, metabolic disorders, infiltrative diseases, autoimmune hepatitis, ischaemia, irradiation damage, but in a high proportion of cases it remains unknown. In contrast to adults, in children with ALF hepatic encephalopathy can be a late event, and may not develop at all, despite a lethal outcome, particularly in infants. Children with ALF should be managed in experienced centres with facilities for liver transplantation. Transplantation should be offered only if the underlying disease is treatable by liver replacement and if the prognosis of transplant is better than that of the underlying disease, as in many cases of ALF the liver has the potential to recover with supportive treatment, if the child is kept alive and stable long enough. Universally accepted criteria for listing for transplantation have not been defined as yet. In our centre, maximum INR, bilirubin level, and white cell count, together with age have proven to be reliable predictors of outcome. Future efforts in the management of ALF should concentrate on designing efficient supportive therapy and specific treatments to provide effective non-transplant therapeutic options.

Safety and efficacy of N-acetylcysteine in children with non-acetaminophen-induced acute liver failure

“…1,3,21,27 This includes immediate transfer to a unit experienced in the management of this condition and with facilities of LT because it is the only absolute treatment for many causes of this devastating condition. 28,29 As shown in early studies in adults' with acetaminophen-induced ALF 18,[30][31][32][33][34][35] NAC is a safe drug with minor side effects, most of them self-limited or resolved with either the use of antihistamine drugs or by lowering the infusion rate.…”

“…1,21 This involved continuous intravenous dextrose infusion to prevent hypoglycemia; broadspectrum prophylactic antimicrobials to prevent bacterial and fungal infections, [22][23][24][25] and ranitidine and/or sucralfate to prevent acute gastrointestinal bleeding. 26 Children were admitted to the pediatric intensive care unit if they developed encephalopathy grade Ͼ2, became hypoxic, or needed sedation.…”

“…[1][2][3] Multiorgan dysfunction in ALF is believed to derive from oxidative stress due to reactive oxygen species and reactive nitrogen species 4 and from immunological injury mediated by cytokines. [5][6][7][8] N-acetylcysteine (NAC) is a thiol-containing agent that scavenges free oxygen radicals and replenishes cellular mitochondrial and cytosolic glutathione stores.…”

Neonatal liver failure: aetiologies and management—state of the art

“…There are no well-defined criteria for listing children for liver transplantation. We have developed a prognostic model of ALF in children in which INR≥4, bilirubin≥ 235 mmol/l, age<2 years, and WBC>9×10 9 /l are associated with poor outcome without LT [10]. Over the recent years, split liver grafts and reduced sized grafts have increased the chances of a successful liver transplantation even in small infants.…”

Bridging to transplantation in acute liver failure in a 7-month-old infant

“…Acute liver failure (ALF) in infancy is a rare but often fatal event that has a mortality of 44-87% depending on the underlying disease [1]. At present, transplantation seems to be the only lifesaving treatment for most cases.…”