The Turkish Journal of Pediatrics 2008 , Vol 50 , Num 2
An unusual presentation of Wilson’s disease in childhood: nodular fatty infiltration in liver
1Departments of Pediatric Gastroenterology, Ankara University Faculty of Medicine, Ankara, Turkey
2Departments of Pathology, Ankara University Faculty of Medicine, Ankara, Turkey
3Departments of Radiology, Ankara University Faculty of Medicine, Ankara, Turkey
Kuloğlu Z, Kansu A, Demirçeken F, Erden E, Fitoz S, Girgin N. An unusual presentation of Wilson’s disease in childhood: nodular fatty infiltration in liver. Turk J Pediatr 2008; 50: 167-170.

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. Keywords : Wilson’s disease, nodular fatty infiltration, liver, children

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