Acute Liver Failure in Neonates, Infants and Children

Denis Devictor; Pierre Tissieres; Philippe Durand; Laurent Chevret; Dominique Debray

Disclosures

Expert Rev Gastroenterol Hepatol. 2011;5(6):717-729. 

In This Article

Abstract and Introduction

Abstract

Acute liver failure (ALF) is a rare but devastating syndrome. ALF in children differs from that observed in adults in both the etiologic spectrum and the clinical picture. Specific therapy to promote liver recovery is often not available and the underlying cause of the liver failure is often not determined. Management requires a multidisciplinary approach and should focus on preventing or treating complications and arranging for early referral to a transplant center. Although liver transplantation has increased the chance of survival, children who have ALF still face an increased risk of death, both while on the waiting list and after emergency liver transplantation. This article will review the current knowledge of the epidemiology, pathobiology and treatment of ALF in neonates, infants and children, and discuss some recent controversies.

Introduction

Acute liver failure (ALF) is a rare but potentially devastating process that often leads to urgent liver transplantation when it is believed that liver regeneration is unlikely.[1–3] Its true incidence in the pediatric population is unknown, but ALF accounts for 10–15% of all pediatric liver transplantations.[1–3] Exhaustive and up-to-date reviews have been published in the pediatric literature.[4–10] In neonates and infants, metabolic diseases are the main cause of ALF, for which specific medical therapies may in some instances preclude the need for liver transplantation.[1,9] In older children, viruses (especially hepatitis A virus), drug-induced hepatotoxicity and autoimmune hepatitis are the most common identified causes of ALF, but the cause of ALF still remains undetermined in a large proportion of children.[3,8,11] Theses etiologies not only differ by age but also by geography. ALF affects children worldwide and the prevalence of hepatotropic viruses as a cause of pediatric ALF varies in different parts of the world. Virus-induced hepatitis is the main causes of ALF in developing countries where hepatotropic viruses are endemic, whereas the prevalence of these viruses, as a cause of ALF, is negligible in developed countries.

Consensual recommendations regarding management of ALF in adults have been published but these remain scarce in pediatrics.[6,7,9] This article examines the specificities of ALF in neonates and children; for example, recent advances in the definition, emerging causes and clinical presentation, and discusses controversies in its management. Specific medical therapies available for a few metabolic diseases presenting with ALF in infancy will be discussed.

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