Liver Failure in Early Infancy

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To the Editor: Our thanks to Dr Ganju et al for their comments on our recent publication in the Journal of Pediatric Gastroenterology and Nutrition(1). The aim of our article was to increase awareness of liver failure in this young age group. We included a group of infants we labelled as having hypoxic ischaemic injury to the liver. This was a mixed group and included infants with severe chronic placental insufficiency who presented immediately after birth, infants with an acute birth asphyxia, and others whose hypoxic/ischaemic insult occurred later (eg, those associated with congenital heart disease or postcardiac surgery). Thus the majority were not true acute birth asphyxia. This type of insult is recognized in our practice as hepatologists and may not be included in other series because of referral patterns (2–4). All the infants in this group had an international normalized ratio of >2 and we agree that the finding of encephalopathy in this group was more likely to be a feature of global hypoxic ischaemic insult rather than hepatic encephalopathy.
The definition of acute liver failure in children and particularly infants remains an area of debate. We applied the definition of the paediatric acute liver failure study group so that our data could be compared to that study (2,3). The infants with mitochondrial disorders, tyrosinaemia, galactosaemia, and neonatal haemochromatosis all presented in liver failure. We, however, excluded infants whose liver disease was diagnosed before they developed liver failure. We agree that the terminology is less than perfect but we took the same pragmatic approach adopted by the paediatric acute liver failure study.

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