This is the first report describing the liver structures of a Japanese patient with
idiopathic copper toxicosis, which should be differentiated from
hepatolenticular degeneration of
Wilson disease. An 11-year-old Japanese boy presented with
ascites associated with biochemical liver damage. Involvement of hepatitis virus was ruled out by laboratory tests. Because urinary
copper excretion was increased,
Wilson disease was highly suspected, but the serum level of
ceruloplasmin was normal, and Kayser-Fleischer rings were not detected by
slit lamp examination. Brain images were within normal limits. ATP7B analysis was negative for mutations. Liver specimen showed
cirrhosis associated with
chronic active hepatitis. Almost all hepatocytes were positive for
orcein-stained granules. Mallory bodies were found in some hepatocytes. Fatty change was minimal, and there were no glycogenated nuclei in the parenchyma. Combined regimens of
trientine and
zinc for 6 months improved the decompensated state of liver function. After 2.5 years of treatment, a second liver biopsy was performed. The post-treatment liver showed complete disappearance of portal
inflammation and remarkable decrease in cuprothionein granules. Mallory bodies disappeared from the parenchyma. An abundance of hepatocellular Mallory bodies and heavy
copper loading limited to the liver may be specific to
idiopathic copper toxicosis.