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Boletín Médico del Hospital Infantil de México

2009, Número 2

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Bol Med Hosp Infant Mex 2009; 66 (2)


Síndrome de Alagille: una causa de colestasis intrahepática persistente

Vázquez-Frias R, Worona-Dibner L, Cabrera-Muñoz ML, Sadowinski-Pine S

Idioma: Español
Referencias bibliográficas: 24
Paginas: 178-188
Archivo PDF: 1200.91 Kb.


FRAGMENTO

Se trató de un escolar masculino de seis años de edad, sin antecedentes heredofamiliares o perinatales de importancia. Presentó ictericia desde el nacimiento que se manejó con fototerapia, agregándose a los cuatro meses coluria y acolia. Fue visto en un hospital pediátrico de tercer nivel por soplo sistólico grado III/VI en foco pulmonar, hepatomegalia, colestasis (bilirrubina total [BT] 16.4 mg/dL, bilirrubina directa [BD] 9.6 mg/dL, gamma-glutamiltransferasa [GGT] 867 U/L) y elevación de aminotransferasas (aspartatoaminotransferasa [AST] 220 U/L y alaninoaminotransferasa [ALT] 125 U/L). La serología viral y el TORCH fueron negativos, tamiz metabólico normal, el ultrasonido hepático mostró ausencia de vesícula biliar, y en el gammagrama de excreción biliar no hubo paso del trazador al intestino.


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