Diagnostic Workup General		The clinical dilemma in detecting the infant with cholestasis is the disparity between the frequency of jaundice in general and that of direct jaundice. It is estimated that 5-15% of 2-week-olds are visibly jaundiced; however only about 1% of those jaundiced will have cholestasis. Although no screening test can predict cholestasis, there are definite clues in the history and physical. Dark urine, pale stools, a family history of prolonged jaundice or hepatomegaly should prompt a total and direct bilirubin. This is especially so in bottle-fed babies, in whom jaundice at 2-4 weeks of age is significantly less common (< 1% compared to 10% of breast-fed babies). Once cholestasis - an elevated direct bilirubin - is established, a staged approach to diagnosis is recommended. First, treatable disorders are searched for, especially in the acutely ill child. Next, biliary obstruction is looked for because of the benefit of early surgery. Finally, complications of cholestasis itself are addressed to guide further medical management. Treatable Causes of Neonatal Cholestasis Galactosemia Tyrosinemia Hereditary fructose intolerance Sepsis, sepsis, sepsis Hypopituitarism Hypothyroidism Disorders of bile acid synthesis Return to top of page