Abstract

ORTIZ-C, Lidia María  and  SAMUDIO-D, Gloria Celeste. Alagille Syndrome: a Case Report. Pediatr. (Asunción) [online]. 2014, vol.41, n.1, pp.51-56. ISSN 1683-9803.

Alagille syndrome, also known as Alagille-Watson syndrome or arteriohepatic dysplasia, is characterized by chronic cholestasis produced by hypoplasia of the intrahepatic bile ducts. It is associated with congenital heart, kidneys, and skeletal malformations in patients with a particular phenotype. These characteristics constitute the five classical diagnostic criteria. Hepatic biopsy is no longer essential for diagnosis when obstructive jaundice is present. Treatment is palliative. Liver transplant should be considered only in case of liver failure, due to unsatisfactory survival rates. We present the case of a female child diagnosed with Alagille syndrome who presented four of the five classic criteria

Keywords : Alagille syndrome; children; diagnosis; diagnostic techniques and procedures.

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