RESUMO
Acute graft-versus-host disease (GVHD) following orthotopic liver transplantation is a rare but severe disease with a 75% death rate in adults. Various therapeutic strategies have been proposed for steroid-refractory GVHD, but there is still no consensus. Tumor necrosis factor-alpha is a key inflammatory cytokine involved in acute GVHD physiopathology, and infliximab has shown encouraging results for the treatment of acute GVHD following hematopoietic stem cell transplantation. We report the first case of acute GVHD following liver transplantation that was refractory to steroids and anti-lymphocyte globulin but was successfully treated with infliximab.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Doença Enxerto-Hospedeiro/tratamento farmacológico , Transplante de Fígado/métodos , Esteroides/farmacologia , Idoso , Anti-Inflamatórios/uso terapêutico , Doença Enxerto-Hospedeiro/etiologia , Humanos , Infliximab , Masculino , Indução de Remissão , Fator de Necrose Tumoral alfa/metabolismoRESUMO
The ABCB4 gene codes for a protein involved in the transport of phosphatidylcholine across the canalicular membrane of the hepatocyte. ABCB4 gene defects have been associated with progressive familial intrahepatic cholestasis type 3, intrahepatic cholestasis of pregnancy, adult biliary cirrhosis and the more recently described low phospholipid associated cholelithiasis syndrome. The present paper describes 2 probands with a long history of recurrent pancreatitis and cholelithiasis and the same heterozygous, as yet undescribed del 3683>3688 within exon 28 of the ABCB4 gene resulting in a loss of function. This report shows that ABCB4 mutations may cause acute recurrent biliary pancreatitis.
Assuntos
Subfamília B de Transportador de Cassetes de Ligação de ATP/genética , Transportadores de Cassetes de Ligação de ATP/genética , Colelitíase/genética , Mutação , Pancreatite/genética , Doença Aguda , Adulto , Colelitíase/complicações , Colelitíase/diagnóstico , Colelitíase/terapia , Feminino , Deleção de Genes , Humanos , Masculino , Pessoa de Meia-Idade , Pancreatite/diagnóstico , Pancreatite/etiologia , Pancreatite/terapia , Linhagem , Reação em Cadeia da Polimerase , Resultado do TratamentoRESUMO
Pegylated interferon and ribavirin combination therapy represent the standard-of-care treatment for chronic hepatitis C, that allows to cure more than half of the patients. However, the success of this bitherapy is in balance with numerous side effects, especially hematologic and psychiatric. This review is focused on complementary treatments (erythropoietin, G-CSF, vitamin E, glutathion, ursodeoxycholic acid and antidepressants) likely to bring a benefit in maintaining adequate interferon and ribavirin dosages and in improving quality of life. This analysis has been performed by using the Medline(R) data base and with data from laboratories which commercialized these molecules. Erythropoietin, G-CSF and antidepressants are the best tools to optimize the bitherapy in its dose and its duration while privileging the quality of life of HCV-infected patients.
Assuntos
Antivirais/efeitos adversos , Hepatite C Crônica/tratamento farmacológico , Interferon-alfa/efeitos adversos , Polietilenoglicóis/efeitos adversos , Ribavirina/efeitos adversos , Anemia/induzido quimicamente , Anemia/prevenção & controle , Antidepressivos/uso terapêutico , Antioxidantes/uso terapêutico , Antivirais/administração & dosagem , Eritropoetina/uso terapêutico , Humanos , Interferon alfa-2 , Interferon-alfa/administração & dosagem , Neutropenia/induzido quimicamente , Neutropenia/prevenção & controle , Polietilenoglicóis/administração & dosagem , Proteínas Recombinantes , Ribavirina/administração & dosagem , Trombocitopenia/induzido quimicamente , Trombocitopenia/prevenção & controleRESUMO
Liver involvement in hereditary hemorrhagic telangiectasia may lead to high-output cardiac failure. Few data have been reported on orthotopic liver transplantation (OLT) for these patients. In this paper, we describe two patients treated by OLT as a salvage procedure for cardiac failure, and we review literature on this subject. Our two patients resumed normal cardiac function after OLT. This procedure appears to be a promising therapy with good long-term results despite dissection difficulties encountered due to the collateral arterial network reorganization.