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- Title
An unusual presentation of Wilson's disease in childhood: nodular fatty infiltration in liver.
- Authors
KuIoğlu, Zarife; Kansu, Aydan; Demirçeken, Fulya; Erden, Esra; Fitoz, Suat; Girgin, Nurten
- Abstract
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.
- Subjects
HEPATOLENTICULAR degeneration; BRAIN diseases; CIRRHOSIS of the liver; GENETIC disorders; HEPATIC manifestations of general diseases; FATTY degeneration; FATTY heart; FATTY liver; CHILDREN; PEDIATRICS
- Publication
Turkish Journal of Pediatrics, 2008, Vol 50, Issue 2, p167
- ISSN
0041-4301
- Publication type
Article