Management of hepatic encephalopathy in children

Abstract

Hepatic encephalopathy is a common metabolic condition in children, having a significantly different aetiopathogenesis from that in adults. The present paper reviews the medical interventions of proven efficacy and also discusses recent advances from various fields as applicable to management of children with this disorder, focusing on measures other than liver transplant. The most important component of managing a child with hepatic encephalopathy is basic intensive care with regulation of fluid status, glucose and electrolyte homeostasis. Specific management includes measures to reduce serum ammonia concentrations, and the prevention and prompt treatment of complications. Methods to reduce ammonia target various steps in its metabolism. This includes reducing its production in and absorption from the intestine and promoting its metabolism in the liver. Significant secondary complications occurring in fulminant hepatic failure which require urgent recognition and management include coagulopathy, cerebral oedema and renal dysfunction. Children with hepatic encephalopathy also have several other paediatric care issues such as fever, requirement for sedation, etc, where the choice of drug is not straightforward and is often different from other settings. This is reviewed here along with an attempt to provide rational choices based on available evidence. Certain controversial and experimental approaches to treatment of fulminant hepatic failure are also discussed, but clearly delineated from the established management protocol. Finally, the role of artificial liver support devices is discussed, with the realisation that they can provide an effective bridge during the time when a patient is waiting for a suitable donor for liver transplant.