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Arch Pediatr ; 7(1): 40-4, 2000 Jan.
Artigo em Francês | MEDLINE | ID: mdl-10668084

RESUMO

UNLABELLED: Neonatal hemochromatosis is characterized by abnormal hepatic and extrahepatic iron overload, which spares the reticuloendothelial system. In neonates, hemochromatosis results in an acute and frequently lethal liver failure. CASE REPORTS: We report five cases of neonatal hemochromatosis which demonstrate various aspects of this disorder and underline the complexity of both the diagnosis and treatment. Case 3 had an extremely low arterial pressure, a presentation not yet described. CONCLUSION: Neonatal hemochromatosis should be suspected in the presence of cholestasis with liver failure of perinatal onset and with high blood level ferritin. Abdominal nuclear magnetic resonance and/or liver biopsy can confirm neonatal hemochromatosis. For one of our patients, a medical treatment allowed us to perform a liver transplantation.


Assuntos
Hemocromatose/patologia , Doenças do Recém-Nascido/patologia , Transplante de Fígado , Diagnóstico Diferencial , Feminino , Hemocromatose/diagnóstico , Hemocromatose/terapia , Humanos , Lactente , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/terapia , Ferro/sangue , Falência Hepática/etiologia , Imageamento por Ressonância Magnética , Masculino
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