https://pubmed.ncbi.nlm.nih.gov/34990749/

J Hepatol. 2022 Jan 3;S0168-8278(21)02304-7.
doi: 10.1016/j.jhep.2021.12.027.Online ahead of print.

Emergencies in Pediatric hepatology

Barath Jagadisan 1, Anil Dhawan 2

Abstract

Etiology of several liver diseases in children are age specific and many of these conditions if not diagnosed early and managed timely have significant repercussions towards their long term outcome. We address this subject in the following five clinical scenarios that cover most of the diagnostic and therapeutic emergencies in children. The wide spectrum of conditions causing liver disease in infants may present as conjugated jaundice and that could be the only symptom of time-sensitive disorders such as biliary atresia, metabolic disorders, infections, hematological and alloimmune disorders where an algorithmic multistage testing is required to establish the correct diagnosis. Early administration of vitamin K in cholestatic infants, use of specific milk formulae and disease specific medications are essential to avoid mortality and life-long morbidity. Management of pediatric acute liver failure requires co-ordination with a liver transplant centre, safe transport and detailed age-specific etiological work-up. Central to survival is supportive care with clinical stabilization until transplantation if indicated . Gastrointestinal bleeding due to varices may present as the initial manifestation or during follow up in patients with portal vein thrombosis or chronic liver disease. We discuss the initial stabilisation and management options including pharmacological therapy followed by endoscopic and radiological interventions. Liver-based metabolic disorders may present as hyperammonaemia or hypoglycaemia with minimal or no abnormality of liver enzymes. Early recognition and specific therapies can be lifesaving or avoid neurological sequalae. Liver tumors and liver trauma are both rare occurrences in children and are best managed by a multidisciplinary team in a specialist centre.