Spontaneously Resolved Neonatal Hemochromatosis.
- Author:
Sin Ae PARK
1
;
Joseph CHO
;
Jeong Kee SEO
;
Oh Kyung LEE
Author Information
1. Department of Pediatrics, Presbyterian Medical Center, Chonju, Korea. okleepmc@netian.com
- Publication Type:Case Report
- Keywords:
Hemochromatosis;
Neonate
- MeSH:
Biopsy;
Female;
Ferritins;
Hemochromatosis*;
Hemolysis;
Hemosiderin;
Hepatocytes;
Humans;
Hyperbilirubinemia;
Infant, Newborn;
Iron;
Kupffer Cells;
Liver;
Magnetic Resonance Imaging;
Spleen
- From:Journal of the Korean Society of Neonatology
2005;12(2):194-199
- CountryRepublic of Korea
- Language:Korean
-
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.
