An unusual presentation of Wilson's disease in childhood: nodular fatty infiltration in liver
Wilson's disease is a rare inherited disorder characterized by progressive accumulation of copper in the body tissues. Liver and brain are the most commonly involved organs and the disease is presented predominantly by hepatic manifestations in childhood. Histopathological findings of he...
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| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Hacettepe University Institute of Child Health
2008-04-01
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| Series: | The Turkish Journal of Pediatrics |
| Online Access: | https://turkjpediatr.org/article/view/2404 |
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| Summary: | Wilson's disease is a rare inherited disorder characterized by progressive accumulation of copper in the body tissues. Liver and brain are the most commonly involved organs and the disease is presented predominantly by hepatic manifestations in childhood. Histopathological findings of hepatic involvement may vary from steatosis to end stage cirrhosis. Although diffuse fatty infiltration is a typical finding of Wilson's disease, it can very rarely present in nodular pattern. We report the first case with Wilson's disease who presented with nodular fatty infiltration in the liver in childhood.
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| ISSN: | 0041-4301 2791-6421 |