Severe Paediatric Liver Conditions

1. Severe Paediatric Liver Conditions • Acute liver failure • Reye syndrome • “Impila poisoning” • Ascites • Chronic liver disease Chronic hepatitis Hepatic schistosomiasis Veno-occlusive disease Storage disorders

2. Acute liver failure • Infective Hepatitis A is commonest • Toxic liver necrosis Herbal toxins : Impila Drugs: analgesics, antibiotic (anti TB) cytotoxic, anticonvulsant, anaesthetic, antiretroviral • Metabolic : Inherited disorders

3. Acute liver failure Critical impairment of liver function: synthesis, detoxification, excretion • Liver disease: anorexia, vomiting, jaundice, ascites, enlarged or shrinking liver, foetor. • Secondary complications: encephalopathy, bleeding tendency, renal failure, sepsis, metabolic disturbance (hypoglycaemia, acidosis)

4. Stage 1 Stage 2 Behaviour changes,shortened attention span, incoordination and tremor Drowsiness, disorientation, asterixis, ataxia, hypertonia Hepatic encephalopathy

5. Stage 3 Stage 4 Delirium, hallucinations, stupor, seizures, rigidity, hyperreflexia Coma, decorticate and decerebrate posturing, opisthotonus, ocular palsies, cardiac arrest Hepatic encephalopathy

6. “Impila” poisoning • Callilepsis laureola in herbal medicines • Causes hypoglycaemia, renal damage, centrilobular necrosis of the liver • Sudden onset coma, convulsions, vomiting,diarrhoea • Acidotic, not jaundiced, floppy • Biochemical evidence of liver failure, hypoglycaemia, renal damage • High mortality and residual morbidity

7. Reye syndrome • Preceding viral infection treated with salicylates • Rapidly progressive anicteric hepatic encephalopathy with microvesicular fatty infiltration of the viscera • High mortality and morbidity in survivors

8. Reye syndrome: biochemical findings • Hypoglycaemia • Metabolic acidosis • Transaminases elevated • Hyperammonaemia • Prolonged prothrombin time • Decreased serum albumin • Leucocytosis • Raised acute phase reactants

9. Management of liver failure • Limit further toxic accumulation: enema, gastric lavage, oral neomycin • Maintain energy and reduce protein intake • Maintain metabolic and hydration state: IV fluids, glucose, vitamin K • Identify cause if possible to ascertain possibility of treatment • Poor prognostic factors: Progressive shrinkage of liver, failure to respond to vitamin K, progressive rise in bilirubin level

10. Veno-occlusive disease of the liver • Obstruction to hepatic venous outflow Central vein of lobule and branches Endothelial oedema, fibroblastic proliferation, obstruction by fibrosis and thrombosis • Caused by drugs/ plant alkaloids: Senecio • Massive congestive hepatomegaly and ascites • Not usually jaundiced • Centrilobular necrosis leads to cirrhosis • Symptomatic treatment only • Potential danger of herbal medicines!

11. Hepatic Schistosomiasis • Schistosoma mansoni eggs in portal tract • Portal fibrosis, not cirrhosis • Presinusoidal portal hypertension • Liver may be of normal size or firm to hard hepatomegaly, often left lobe • Large firm spleen often an incidental finding • Features of portal hypertension • Liver decompensation is rare, most improve with time

12. Storage diseases involving the liver Metabolic defects resulting in liver accumulation and damage including progression to cirrhosis • Glycogen storage disease Inability to mobilise glycogen Stunting Spleen not involved Fasting hypoglycaemia and lactic acidosis • Lipid storage disorders Gaucher’s, Niemann Pick, Tay-Sachs Usually CNS involvement Spleen enlarged

13. Wilsons disease Defect in copper metabolism results in copper accumulation in liver, brain and other tissues Presents with • Hepatic damage: Acute hepatitis Chronic active hepatitis Cirrhosis Fulminant hepatic failure • CNS: extrapyramidal and basal ganglia signs • Ophthalmological: Kayser-Fleischer ring • Other: cardiac, renal, skeletal, endocrine