Spontaneously Resolved Neonatal Hemochromatosis.

Park,  Sin Ae; Cho,  Joseph; Seo,  Jeong Kee; Lee,  Oh Kyung

Case Report

Journal of the Korean Society of Neonatology 2005;12(2):194-199.
Published online November 1, 2005.

Spontaneously Resolved Neonatal Hemochromatosis.

Sin Ae Park, Joseph Cho, Jeong Kee Seo, Oh Kyung Lee

¹Department of Pediatrics, Presbyterian Medical Center, Chonju, Korea. okleepmc@netian.com
²Department of Pediatrics, College of Medicine, Seoul National University, Korea.

보존적 치료만으로 호전된 Neonatal Hemochromatosis 1례

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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.

Key Words: Hemochromatosis; Neonate