[Histological features of paediatric acute liver failure : Experiences from a tertiary referral centre for paediatric liver disease in the UK].

Abstract

Paediatric acute liver failure (PALF; also fulminant hepatic failure, fulminant hepatitis) is a critical clinical syndrome that is characterised by a sudden, rapid deterioration and disease progression in a usually previously healthy child. The pathogenesis is an advanced degree of hepatocellular necrosis that exceeds the rate of hepatocyte regeneration. The diagnostic criteria of PALF (modelled on adult criteria) were developed by the "Pediatric Acute Liver Failure (PALF) Study Group" (NIH). The rule of the liver biopsy in PALF is controversial and in some cases contraindicated (coagulopathic state). In addition, extensive necrosis is a common finding in PALFs but may not be predictive of the overall outcome (transplantation versus continuous treatment) due to sampling issues. There are, however, some histological patterns that offer a degree of specificity that can be carefully considered in the overall clinical picture. The histopathologists will be part of a multidisciplinary team and can contribute to the diagnostic and prognostic pathway.The aetiologies of PALF are numerous and depend on age and geographical region. For all age groups the main causes can be divided into infectious, immunological, metabolic and toxin drug related. Rarer causes include circulatory disorders and malignancies. In the paediatric group, up to 30-50% of causes leading to PALF remain unknown.