J Korean Soc Neonatol.
2005 Nov;12(2):194-199.
Spontaneously Resolved Neonatal Hemochromatosis
- Affiliations
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- 1Department of Pediatrics, Presbyterian Medical Center, Chonju, Korea. okleepmc@netian.com
- 2Department of Pediatrics, College of Medicine, Seoul National University, Korea.
Abstract
- Neonatal hemochromatosis is a very rare disorder with an unclear etiology with an extremely poor outcome. We report a case of female newborn who presented with direct hyperbilirubinemia without any evidence of congenital infection, immune related hemolysis or exogenous iron uptake. Diagnostic studies revealed highly elevated ferritin level. T2 weighted image of abdominal Magnetic Resonance Imaging (MRI) showed decreased signal intensity of entire left lobe and a part of right lobe of liver with normal spleen. Liver biopsy showed a large amount of hemosiderin in the hepatocytes and Kupffer cells. All the biochemical and excretory liver functions normalized with conservative managements.
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