[Alagille syndrome and isolated persistent ductus arteriosus]

The clinical case of a 21-year-old patient diagnosed with a variant of intrahepatic biliary ductopenia or Alagille syndrome, a dominant inherited disorder associated with a chronic cholestatic syndrome and abnormalities in different organs and systems. The present article discusses the main clinical abnormalities found in this syndrome and we describe a persistent arterial ductus as an isolated cardiovascular manifestation not previously reported in association to this syndrome.