Giant cell transformation is a non-specific reaction by the liver. It can be idiopathic as in this case. It is frequently seen in response to infection such as congenital syphilis, CMV or HIV infections. It may be the only change seen in the biopsy for extrahepatic biliary atresia. Inborn errors of metabolism such as alpha 1-antitrypsin deficiency, galactosemia, hereditary fructose intolerance, tyrosinemia, and neonatal hemochromatosis. All of these must be ruled our prior to designating the disorder as idiopathic.
The outcome in non familial neonatal hepatitis is 60% recovery, 10% persistent fibrosis or inflammation, 2% develop cirrhosis and 30% die. Familial cases have a much worse outcome with only 30% recovery, 10% chronic liver disease and cirrhosis and 60% die.
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