Back to Search
Start Over
Liver structures of a patient with idiopathic copper toxicosis.
- Source :
-
Medical molecular morphology [Med Mol Morphol] 2012 Jun; Vol. 45 (2), pp. 105-9. Date of Electronic Publication: 2012 Jun 21. - Publication Year :
- 2012
-
Abstract
- 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.
- Subjects :
- Ascites diagnosis
Ascites etiology
Chelating Agents therapeutic use
Child
Diagnosis, Differential
Hepatocytes pathology
Humans
Liver pathology
Liver Diseases etiology
Male
Mallory Bodies pathology
Treatment Outcome
Trientine therapeutic use
Copper poisoning
Liver drug effects
Liver Diseases diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1860-1499
- Volume :
- 45
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Medical molecular morphology
- Publication Type :
- Academic Journal
- Accession number :
- 22718296
- Full Text :
- https://doi.org/10.1007/s00795-011-0556-1