Question
A 2½ years old boy presented in Jan 2009 with incidentally detected hepatomegaly. There was no jaundice, fever or vomiting. On examination, weight was 12 kg, height was 85.8 cm and there was hepatomegaly {9 cm span}. Other systems were normal. Liver function tests are depicted in Table 1. His Hepatitis A IgM, HBsAg, Hepatitis E IgM, Hepatitis C ELISA were negative. His liver enzymes continued to be elevated for next 6 months. Autoimmune markers are depicted in Table 1. His 24 hrs urine copper are also depicted in Table 1. Her serum alpha fetoprotein and alpha 1 antitrypsin levels were normal. Liver biopsy showed mild early fibrosis with liver copper content of 21.2 µg, gm. Hepatocytes were PAS positive. In Nov 2009 in view of positive anti smooth muscle antibody, he was started on oral steroids to which his liver enzymes decreased in April 2010. His anti smooth antibody became negative in Feb 2010. In June 2011, he again had elevated liver enzymes which increased markedly in Nov 2011. His serum IgG was 8.78 gm, dl and liver biopsy showed same amount fibrosis without interface hepatitis. His antibodies to ANA, smooth muscle, LKM were negative.