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doi: 10.3892/etm.2021.10972 224 FrisoliMLEssienKHarrisJE

Evidence of abnormal mitochondrial ultrastructure in hepatocytes of patients with Wilsons disease supports the theory that mitochondria are one of the affected organelles in copper toxicity.17 There is also evidence to suggest that the mechanism of hepatocyte death in Wilsons disease is via apoptosis that may explain the typical mild elevation in the transaminases associated with hepatitis in Wilsons disease.18 Clinical Presentation One of the challenges of diagnosing Wilsons disease is the wide spectrum of clinical presentations