Síndrome de Allagile. Presentación de un caso

  • Lidia María Ortiz-C
  • Gloria Celeste Samudio-D
Palabras clave: Síndrome de Allagile, pediatría, criterios diagnósticos.

Resumen

El Síndrome de Allagile, conocido también como síndrome de Alagille-Watson y displasia arterio hepática se caracteriza por una colestasis crónica producida por una hipoplasia de las vías biliares intrahepáticas. Se asocia a malformaciones congénitas cardiacas, renales y esqueléticas en pacientes con un fenotipo peculiar. Estas características constituyen los cinco criterios clásicos de diagnóstico. La biopsia hepática ya no es imprescindible para el diagnóstico, siempre y cuando exista ictericia colestasica. El tratamiento es sintomático. El trasplante hepático debe plantearse solo en caso de falla hepática porque la sobrevida no es buena. Se presenta el caso de una niña con diagnóstico de Síndrome de Allagile, que presentó cuatro de los cinco criterios clásicos.

Citas

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Publicado
2017-10-22
Sección
Casos Clínicos