We have previously reported that urinary copper excretion of more than 25 p.mo1124 hr after penicillamine challenge is the most accurate single diagnostic test for Wilson's disease (WD) in children with liver disease (1). In a study of 75 children, 15 of 17 with WD had urinary copper levels more than 25 p,mo1/24 hr, whereas this feature was observed in only 1 of 58 children with other liver disorders. Other parameters of copper metabolism (ceruloplasmin, serum and liver copper content and baseline 24-hr urinary copper excretion) were less sensitive in differentiating WD from other liver disorders.
We would like to report our recent experience in three children (two girls; age 8, 10 and 15 yr) with acute persistent hepatitis A virus (HAV) infection, a group that was not included in the original study. All patients were positive for HAV IgM and had been jaundiced for at least 12 wk, with pruritus, fatigue, anorexia and weight loss before referral to our unit. Their histories and family histories were unremarkable. All were jaundiced, with firm hepatomegaly. The spleen was impalpable, and there were no signs of chronic liver disease.
The persistence of symptoms prompted us to investigate for other, underlying pathological liver conditions. Serological markers for other hepatotrophic viruses (HBV, hepatitis C virus, cytomegalovirus, Epstein-Barr virus, adenovirus, herpes and rubella) were negative. All three patients were of the a,-antitrypsin phenotype MM on isoelectric focusing. Patient 3 had increased IgG levels (23 gm/L), with a smooth muscle antibody titer of 1/:80.
We performed investigations for WD (Table 1).