Acute Liver Failure in Infancy

Etiology, Outcome and Prognostic Indicators of Childhood Fulminant Hepatic Failure in the United Kingdom

“…There are difficulties in applying this definition to the pediatric population, particularly to very young infants, in whom features of HE may be difficult to ascertain and in whom presentation may occur late in the course of illness (1,8). Most investigators have not regarded the presence of HE as a prerequisite for the diagnosis of FHF in neonates and young infants (8,9,12). The second difficulty with this definition is the requirement that pre-existing liver disease be absent.…”

“…The second difficulty with this definition is the requirement that pre-existing liver disease be absent. Many authors include liver failure that occurs with an underlying primary systemic disease, such as hemophagocytosis, acute leukemia and inborn errors of metabolism as part of FHF (9,12). In this study, children with pre-existing systemic illness and liver failure were excluded.…”

“…In infants younger than 12 months, the main cause of FHF is metabolic disease (9,12). Durand et al noted neonatal hemochromatosis and mitochondrial cytopathy as important causes of neonatal FHF (12), whereas Mieli-Vergani et al noted hemophagocytic lymphohistiocytosis as the most important cause (9).…”

“…In developed countries the cause of FHF differs according to age and geographical location (1,8). In English infants younger than 2 years, hemophagocytic lymphohistiocytosis, cryptogenic hepatitis and metabolic diseases account for 75% of cases of FHF (9). Infectious causes were rare, accounting for only two of the 45 cases studied (9).…”

“…In English infants younger than 2 years, hemophagocytic lymphohistiocytosis, cryptogenic hepatitis and metabolic diseases account for 75% of cases of FHF (9). Infectious causes were rare, accounting for only two of the 45 cases studied (9). In contrast, in France, viral hepatitis and druginduced liver injury are important causes (10,11).…”