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Arch Pediatr ; 7(1): 40-4, 2000 Jan.
Article in French | MEDLINE | ID: mdl-10668084

ABSTRACT

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.


Subject(s)
Hemochromatosis/pathology , Infant, Newborn, Diseases/pathology , Liver Transplantation , Diagnosis, Differential , Female , Hemochromatosis/diagnosis , Hemochromatosis/therapy , Humans , Infant , Infant, Newborn , Infant, Newborn, Diseases/diagnosis , Infant, Newborn, Diseases/therapy , Iron/blood , Liver Failure/etiology , Magnetic Resonance Imaging , Male
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