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

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

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)

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

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

“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

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

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

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

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!

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

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

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