RESUMO
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.
Assuntos
Agamaglobulinemia/complicações , Hepatopatia Veno-Oclusiva/complicações , Falência Hepática/etiologia , Infecções por Roseolovirus/diagnóstico , Adolescente , Agamaglobulinemia/tratamento farmacológico , DNA Viral/sangue , Hepatopatia Veno-Oclusiva/cirurgia , Herpesvirus Humano 7/genética , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fígado/patologia , Transplante de Fígado , Masculino , Infecções por Roseolovirus/complicaçõesRESUMO
Liver transplantation (LT) was achieved for factor V Leiden-induced thrombophilia in a neonate with hepatic veno-occlusive disease. Initial LT was performed with a liver segment removed from a child with primary oxalosis. Four months later, a second, definitive LT was performed. The child remains well without recurrent thrombosis.
Assuntos
Fator V/genética , Hepatopatia Veno-Oclusiva/cirurgia , Transplante de Fígado , Mutação Puntual , Trombofilia/cirurgia , Hepatopatia Veno-Oclusiva/genética , Humanos , Recém-Nascido , Falência Hepática/genética , Falência Hepática/cirurgia , Masculino , Reação em Cadeia da Polimerase , Análise de Sequência de DNA/métodos , Trombofilia/genéticaRESUMO
Veno-occlusive disease of the liver is a recently established disease entity. Its etiology, pathology, and symptomatology, and natural history are discussed on the basis of 99 cases of this disease so far authenticated at the University College of the West Indies. In the surgical literature the disease is added to the list of causes of intra-hepatic portal hypertension, and the cases of 4 patients are presented in detail in whom portacaval anastomoses were performed for the decompression of esophageal varices. The surgical implications of this disease have been stated and some of the difficulties enountered in the treatment of portal hypertension in infants and children outlined (Summary)