Liver diseases in childhood

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Biliary atresia is a cause of neonatal jaundice.
In biliary atresia there is destruction of bile ducts that leads to severe jaundice in neonates.
Two patterns are seen:
Intrahepatic atresia, in which ducts within the liver are absent.
Extrahepatic atresia, in which the main extrahepatic ducts are not patent.
These syndromes are believed to be due to intrauterine inflammatory diseases, which can also cause neonatal hepatitis.
Liver biopsy shows striking proliferation of small bile ducts at the margins of portal tracts.
Affected children develop severe jaundice.
Extrahepatic atresia may be treated by surgical bypass operations. Children with severe disease develop
unremitting jaundice and secondary biliary cirrhosis.

Neonatal hepatitis is a syndrome with many causes

Neonatal hepatitis is a clinical condition with many causes, presenting as neonatal jaundice.
Histologically there is hepatocyte damage, parenchymal inflammation and, in many cases, giant-cell
transformation of hepatocytes (so-called giant cell hepatitis).
The main causes are:
Idiopathic (no cause found), accounting for 50% of cases.
alpha1-antitrypsin deficiency, responsible in about 30% of cases.
Viral hepatitis.
Hepatitis due to Toxoplasma, rubella, cytomegalovirus or herpes simplex (TORCH group).
Metabolic causes (galactosaemia, hereditary fructose intolerance).

Jaundice in neonates can cause brain damage termed kernicterus
Unconjugated hyperbilirubinaemia in neonates may lead to kernicterus. Unconjugated bilirubin, which
is lipid-soluble, enters the brain to cause damage to neurons, particularly in the basal ganglia.

Affected children develop spasticity, choreo-athetosis and mental deficiency. The most common causes are
haemolysis due to rhesus incompatibility, or functional immaturity of hepatic conjugating enzyme systems.
Cystic fibrosis leads to cirrhosis in adult life in about 10% of cases.

In cystic fibrosis affected neonates may develop obstructive jaundice due to blockage of
bile ducts by abnormally viscous bile. Of those who survive to adult life,
10-15% develop chronic liver disease. This is a form of secondary biliary cirrhosis due to chronic obstruction to bile ducts by viscid bile.

Reye's syndrome is associated with microvesicular fat in liver cells
Reye's syndrome results in acute liver failure associated with cerebral oedema and encephalopathy.
It is seen in children and may be triggered by preceding viral infection or salicylate administration.
Histologically the liver shows microvesicular fatty change.
The condition is fatal in about 50% of cases.
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Revised: 02-11-2014.