Specific diseases Idiopathic neon hepatitis		As mentioned before, the relative percentage of this diagnosis has steadily declined over the years as newer genetic and metabolic diseases are described. Nowadays the term "idiopathic neonatal hepatitis" should be reserved for cases of prolonged neonatal cholestasis when known causes have been ruled out and classic liver biopsy findings are present. Clinically, jaundice appears earlier than with BA, usually within the first week, overlapping physiologic jaundice. Most patients appear well and are normally grown, but up to a third may have failure to thrive. Acholic stools are unusual, but may be seen if the cholestasis is severe. The liver is enlarged and firm. Direct bilirubin, ALT and AST are 2-10 times normal. Prognosis is uncertain due to the "default" nature of the diagnosis. Management often becomes that of chronic liver disease, in addition to serially following liver function tests (LFTs) until normalization. If new symptoms develop or cholestasis does not resolve, repeat liver biopsy and further workup is indicated. A subset of patients has been described with early onset of cholestasis, clearly identifiable perinatal distress (either acute or chronic, mean gestational age at birth 37 weeks, often SGA) and complete resolution of jaundice with normalization of LFTs by 3-4 months. Liver biopsy was consistent with "neonatal hepatitis". Interestingly, there was discordance among LFTs, with the GGTP reaching its highest level as the direct bilirubin approached normal. Return to top of page