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2007, Número 4

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Rev Mex Pediatr 2007; 74 (4)


Un caso del síndrome de Alagille

Jiménez JJR, Castellanos RK, Huerta AR, Justiniani CNE, Yáñez LMP, Sierra TA

Idioma: Español
Referencias bibliográficas: 30
Paginas: 152-157
Archivo PDF: 234.88 Kb.


RESUMEN

El síndrome de Alagille es una enfermedad congénita, poco frecuente, se caracteriza por colestasis, anormalidades vertebrales, defectos cardiacos, anormalidades oculares y rasgos faciales particulares. Hay alteraciones en la diferenciación celular y organogénesis, asociadas al gen Jagged 1 (JAG 1) localizado en el cromosoma 20; el hígado tiene menor número de conductos biliares ocasionando colestasis. En esta comunicación se informa de un caso clínico y se revisa la información disponible acerca de esta enfermedad.


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