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Abstract Cholestatic jaundice occurs in young infants due to a variety of causes, its pathophysiological consequences on liver cell function are the same. Early recognition and treatment may prevent permanent liver damage in some of these patients. Secondary cirrhotic changes occur early if the diagnosis is delayed. Delayed presentation results in the poor prognosis of cholestatic syndrome of infancy. Biliary atresia and neonatal hepatitis are the two most common causes of cholestasis in the neonatal period. Treatment modalities differ among various condition; therefore, it is obvious that an early and correct diagnosis has a crucial role in the proper management of these children. Various diagnostic tools including liver function tests, enzyme assays, or imaging techniques are available for the evaluation of liver disorders. Although liver biopsy is an invasive method, it is the corner stone for a precise diagnosis to differentiate between biliary atresia and other non obstructive forms of neonatal cholestasis before any surgical procedure. If left untreated cholestatic liver injury results in the development of hepatic fibrosis and progress to cirrhosis. Paucity of bile ducts are grouped into syndromic and non-syndromic forms. Alagille syndrome is the second most common heritable cause of chronic liver disease, the first being alpha 1-antitrypsin deficiency. |