Liver structures of a patient with idiopathic copper toxicosis.
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.
Department of Medicine, Aichi Gakuin University School of Pharmacy, 1-100 Kusumoto-cho, Chikusa-ku, Nagoya, 464-8650, Japan. hhayashi@dpc.agu.ac.jp
Hisao
H
Hayashi
Tsutomu
T
Shinohara
Keisuke
K
Goto
Yoshikazu
Y
Fujita
Yu
Y
Murakami
Ai
A
Hattori
Yasuaki
Y
Tatsumi
Atsumi
A
Shimizu
Takashi
T
Ichiki
D001201:Ascites / Q000175:diagnosis / Q000209:etiology; D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D003300:Copper / Q000506:poisoning*; D003937:Diagnosis, Differential; D022781:Hepatocytes / Q000473:pathology; D006801:Humans; D008099:Liver / Q000187:drug effects* / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D059609:Mallory Bodies / Q000473:pathology; D016896:Treatment Outcome; D014266:Trientine / Q000627:therapeutic use
D002614:Chelating Agents; D003300:Copper; D014266:Trientine