Cholestasis in Infancy

Abstract

The natural history of cholestatic syndromes in infancy remains largely unclarified for lack of sufficient data. Newborn and premature infants are particularly vulnerable to cholestasis because of immaturities in bile-forming mechanisms. Until recently, two broad categories of etiologic factors have been thought to be associated with cholestasis in early infancy: mechanical obstruction (almost always extrahepatic), and hepatocellular damage (the "neonatal hepatitis" group). Although in both groups specific etiologic factors have been identified, the majority of cases are currently of unknown etiology. Problems in differential diagnosis are reviewed. In the neonatal period, laboratory screening procedures usually do not uncover cholestatic liver disease until the infants become icteric. It is important to note that patients with liver dysfunction may remain anicteric or become anicteric while cholestasis persists. It is, therefore, important that biochemical markers of cholestasis other than conjugated bilirubin be found.