[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.
Sánchez-Angulo
J I
JI
Seccion de Aparato Digestivo, Hospital del Servicio Andaluz de Salud, Jerez de la Frontera, Cádiz.
Benítez-Roldán
A
A
Castro-Fernández
A
A
Ruiz-Campos
J
J
D000328:Adult; D016738:Alagille Syndrome / Q000150:complications* / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D001706:Biopsy; D004374:Ductus Arteriosus, Patent / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D004452:Echocardiography; D005260:Female; D006801:Humans; D013902:Radiography, Thoracic