Table 8.2.

Aetiological evaluation in paediatric liver

Specific therapies for ALF
Autoimmune hepatitis: steroids (methylprednisolone 2 mg/kg/day, Max 60 mg/day)
Acetaminophen toxicity: N-acetylcysteine (100 mg/kg/day) until INR is normal
Mushroom poisoning: benzylpenicillin (1,000,000 U/kg/day) or thioctic acid (300 mg/kg/day)
Galactosaemia/hereditary fructose intolerance: elimination diet
Hereditary tyrosinaemia: NTBC (0.5 mg/kg bd) + elimination diet
Haemophagocytic lymphohistiocytosis:
Chemotherapy ± haematopoietic stem cell transplantation (familial/genetically verified and persistent/reactivation of secondary HLH)
Neonatal haemochromatosis:
Double volume exchange transfusion and intravenous immunoglobulin (1 g/kg)

(Reproduced with permission from Shanmugam et al., Acute liver failure in children: Management protocol, manual of paediatric liver intensive care) (Springer publication in print)