Update on the etiologies and management of neonatal cholestasis.

The early detection of cholestatic liver disease is one of the major challenges facing pediatricians when evaluating the jaundiced infant. Early recognition of liver disease greatly facilitates the care and outcome of infants, because several serious life-threatening disorders may have cholestasis as a major presenting sign of underlying neonatal liver disease. A key component of the work-up is measurement of serum conjugated bilirubin levels, which if elevated should prompt the clinician to initiate a work-up to determine the cause of neonatal cholestasis. In general, if a patient is developing progressive jaundice soon after birth, is still jaundiced at 2 weeks of life, or develops jaundice within the first month of life, a work-up for neonatal cholestasis should begin. A number of previously undiagnosed causes of neonatal cholestasis are beginning to be assigned genetic and infectious etiologies, with significant implications for the work-up and management of cholestatic infants.