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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
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Chronic hepatitis in childhoodModes 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 Also Gall 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 of liver 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