Neonatal cholestasis. New approaches to diagnostic evaluation and therapy.

Neonatal cholestasis remains a major diagnostic challenge despite increasing knowledge regarding its pathogenesis. The time constraint and urgency in the investigational process is underscored by the age-dependent success rate of the surgical corrective procedures for EHBA. Appropriate interpretation of imaging and pathologic studies requires a pediatric center familiar with the entities causing neonatal cholestasis. When liver failure or progressive hepatic dysfunction is likely to occur, early referral to a liver transplant center is recommended. Despite the increasing experience and excellent results of pediatric liver transplantation, at this point, surgical corrective procedures such as the Kasai procedure remain the first line of treatment for most patients with EHBA.