RESUMEN
Parenteral omega-3 fatty acid lipid emulsions have been evaluated for their potential role in reversing intestinal failure-associated liver disease. We report our experience using Omegaven in 2 patients with irreversible intestinal failure and intestinal failure-associated liver disease. Despite biochemical and histologic improvement in cholestasis, both patients had persisting, significant portal fibrosis on liver biopsy.
Asunto(s)
Emulsiones Grasas Intravenosas/uso terapéutico , Ácidos Grasos Omega-3/uso terapéutico , Enfermedad de Hirschsprung/terapia , Enfermedades Intestinales/terapia , Cirrosis Hepática Biliar/terapia , Nutrición Parenteral/métodos , Femenino , Humanos , Recién Nacido , Cirrosis Hepática Biliar/patología , Masculino , Nutrición Parenteral/efectos adversos , Insuficiencia del TratamientoRESUMEN
Hepatobiliary disease has been described in Bruton's x-linked agammaglobulinemia; however, veno-occlusive disease has not been reported in this setting. We report a case of end-stage liver disease in a patient with x-linked agammaglobulinemia who was found to have VOD and evidence of human herpes virus 7 infection in the explanted liver after transplantation.