Abstract
Acute liver failure is due to necrosis of the liver leading to loss of liver function. The definition includes coagulopathy and encephalopathy. Other organs are often affected and there is a high mortality rate despite intensive care support. The initial clinical features are subtle with poor feeding, difficulty waking and poor temperature control, which can be difficult to detect in young infants. Initial investigations will show prolonged prothrombin time, hypoglycaemia, increased transaminases and bilirubin. A neonate with acute liver failure or liver dysfunction should be referred immediately to a tertiary paediatric liver centre for further advice, investigations and management. Many will require timely transfer for specialised care and consideration for transplantation. The most common causes are infection and metabolic disease (Tables 2.1 and 2.2) whilst liver vascular disease e.g. haemangioendotheliomas also present as an acutely ill baby with hepatomegaly, preserved liver biochemistry, heart failure and consumptive coagulopathy.
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Further Reading
Cantz T, Sharma AD, Ott M. Concise review: cell therapies for hereditary metabolic liver diseases-concepts, clinical results, and future developments. Stem Cells. 2015;33(4):1055–62.
Lee WS, Sokol RJ. Mitochondrial hepatopathies: advances in genetics, therapeutic approaches, and outcomes. J Pediatr. 2013;163(4):942–8.
McKiernan P. The acutely ill baby. In: Kelly DA, editor. Diseases of the liver and biliary system in children. 4th ed. Chichester: Wiley; 2017. p. 127–43.
McKiernan PJ. Nitisinone for the treatment of hereditary tyrosinemia type I. Expert Opin Orphan Drugs. 2013;1(6):491–7.
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Hartley, J. (2018). The Acutely Ill Baby. In: Kelly, D., Sharif, K., Hartley, J. (eds) Atlas of Pediatric Hepatology. Springer, Cham. https://doi.org/10.1007/978-3-319-69529-7_2
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DOI: https://doi.org/10.1007/978-3-319-69529-7_2
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