IDIOPATHIC NEONATAL HEPATITIS PRESENTING AS NEONATAL HEPATIC SIDEROSIS AND STEATOSIS

Idiopathic neonatal hepatitis (INH) is a heterogenous disease of undet ermined cause, We report a retrospective histologic reevaluation of IN H. Sixty patients with INH were reviewed along with 32 biliary atresia (BA) patients, Histologic findings, iron and fat deposits, giant cell transformation, portal fibrosis, and bile duct proliferation were sem iquantitatively graded from 0 to 4+, Significant histologic findings w ere defined as greater than or equal to 2+. Frequencies of patients wi th significant histologic findings in the INH group were compared with those of the BA group. Among the patients with significant histologic findings, those in the INH group had significantly less iron deposits (P < 0.01), portal fibrosis (P < 0.01), and bile duct proliferation ( P < 0.01) than those of the BA group, A combination of significant hep atic macrovesicular steatosis and siderosis was observed in 10 INH pat ients but not in any BA patient (10/60 vs 0/32, P < 0.05). Without ext ensive treatment, the 10 INH patients all recovered, and hepatic abnor malities normalized by the age of 12 months. In conclusion, the presen t study showed that the recognition of hepatic siderosis is helpful to distinguish BA from INH and that in a subset of INH patients hepatic macrovesicular steatosis and siderosis occurs.