ABSTRACT
Neonatal cholestasis is caused by impaired excretion of biliary substances resulting in their accumulation in blood. Neonatal cholestasis should be ruled out in infants presenting with jaundice that persists after 2 weeks of life. It is important to check fractionated serum bilirubin levels in these patients and immediately refer the patients with conjugated hyperbilirubinemia to a pediatric gastroenterologist for further evaluation. Conjugated hyperbilirubinemia, pale stools and dark urine are the cardinal features of neonatal cholestasis. Early recognition and a stepwise diagnostic evaluation of the infant with cholestasis are essential in successfully treating or managing the complications of the metabolic and infectious liver diseases of the infant as well as surgically relieving obstruction in patients who have biliary atresia. Biliary atresia is the most common cause of neonatal cholestasis and the prognosis is directly related to the age at the time of surgery, with better prognosis if surgery is done before 60 days of age. Cholestasis in premature infants is multifactorial and should have a modified approach to the evaluation of cholestasis. Medical management of cholestasis is mostly supportive, consisting of management of complications of chronic cholestasis like pruritus and nutritional support for malabsorption and vitamin deficiency