Chronic hepatitis in childhood Modes of presentation Acute onset jaundice and persisting Gradual development of signs of liver disease Asymptomatic finding of enlarged liver

The child with acute onset jaundice but not otherwise ill Unconjugated hyperbilirubinaemia: Haemolysis Conjugated hyperbilirubinaemia: Mostly Hepatitis A, B, C AlsoGall stones, Extrahepatic obstruction including worms in bile duct

Acute onset jaundice and seriously ill Consider the following an emergency: Bleeding tendency Confused or abnormal level of consciousness Toxic or high fevers

Chronic Liver Disease History of prior acute liver disease : jaundice symptoms suggesting liver dysfunction Examination findings Poor growth Signs ofliver dysfunction hepato/ + splenomegaly portal hypertension

Chronic Liver Disease Tests of liver dysfunction Synthesis Detoxification Excretion Maintenance of fasting glucose Cell Integrity : enzymes Tests for aetiology Hepatitis viruses Auto-immunity Wilsons disease Tests for evidence and complications of portal hypertension Histology Inflammation, Necrosis, Fibrosis, Regeneration Storage and Infiltration

Hepatitis Inflammation of the liver is caused by : Infection Viral Hep A,B,C,D,E,F,G; Others Parasitic Bacterial Toxins/drugs Immune/auto-immune Metabolic conditions eg Wilsons disease

Chronic hepatitis Chronic persistent hepatitis Persisting inflammation Gradual improvement Chronic active hepatitis Auto-immune Hepatitis B, C Often progress to cirrhosis

Chronic active hepatitis Acute onset or non-specific malaise Jaundice Hepatosplenomegaly Cutaneous features of chronic liver disease spider angiomata, striae, acne Extrahepatic manifestations of auto-immunity arthritis, haemolytic anaemia,IBD etc

Chronic active hepatitis Transaminases and bilirubin elevated Decreased serum albumen Elevated serum globulin Auto-antibodies often present esp in HBsAg negative patients Smooth muscle antibodies Liver Kidney Antibody Antinuclear antibody Rheumatoid factor Liver biopsy confirms

Management Chronic hepatitis B : Alpha Interferon Auto-immune hepatitis: steroids, azathioprine Dietary support: Energy, vitamins Avoid hepatotoxic medications Monitor for development of cirrhosis

Cirrhosis Normal liver architecture replaced by nodules of regenerating tissue surrounded by fibrous tissue. End stage of many different conditions. Progressive disease resulting in liver failure. Rate of progression variable. Clinical features of underlying condition portal hypertension chronic liver disease

Cirrhosis Biliary cirrhosis Biliary atresia or obstruction Cystic fibrosis Post-necrotic cirrhosis Post hepatitis Venous congestion Genetic diseases Wilson’s disease Galactosaemia

Portal hypertension Pre-hepatic Portal or splenic vein obstruction Intrahepatic pre-sinusoidal Schistosomiasis Intrahepatic pre-and post-sinusoidal Chronic hepatitis Cirrhosis Congenital hepatic fibrosis Veno-occlusive disease Post-hepatic Budd-Chiari syndrome IVC obstruction Pericarditis or heart failure

Clinical effects of portal hypertension Splenic enlargement and hypersplenism Anaemia Thrombocytopaenia Leukopaenia Pressure-mediated effects in portal vessels Collateral vessel enlargement and bleeding Oesophageal varices Hydrostatic effects Ascites and abdominal distension Clinical features of the underlying liver disease