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Alagille syndrome is a multi-systemc genetic disorder with variable phenotypic penetrance that was first described in 1969 by Daniel Alagille.It is characterized by anomalies of the intrahepatic bile ducts, heart, eye and skeleton, which are associated with facial features . The prognosis depends on the severity of the liver and heart diseases. The authors reported two cases characterized by the variability of clinical expression and evolution. The study concerned two girls aged of 2 and 4 months with no family history, who developed cholestatic jaundice evolving from the first month of life. The aim of this work is to remind the different clinical expressivity and the differentmodalities to manage the patients in order to ensure a best quality of life.
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