Liver diseases in
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Biliary atresia is a cause of neonatal
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
Liver biopsy shows striking proliferation of small bile ducts at the margins of
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
Histologically there is hepatocyte damage, parenchymal inflammation and, in many
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
• 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
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
It is seen in children and may be triggered by preceding viral infection or
Histologically the liver shows microvesicular fatty change.
The condition is fatal in about 50% of cases.
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