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Você conhece esta síndrome?DOI: 10.1590/S0365-05962008000300012 Keywords: alagille syndrome, cholestasis intrahepatic, pruritus, xanthomatosis. Abstract: alagille syndrome, an inherited autosomal disorder of dominant trait, is one of the most common causes of chronic liver disease in childhood. its hallmark is the paucity of intrahepatic bile ducts, with variable degrees of liver impairment. cutaneous manifestations include intense pruritus, often refractory to treatment, and xanthomata.
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