Abstract | OBJECTIVES: METHODS: Four male patients with Wilson's disease were enrolled in this study of pre- and post-treatment iron metabolism. RESULTS: Pretreatment copper contents of the liver were high in all four male patients studied as diagnostic of Wilson's disease. Genetic analysis supported their clinical diagnosis of Wilson's disease without a background of hemochromatosis. Pretreatment serum ceruloplasmin levels were <20 mg/dl in all four patients. A standard penicillamine treatment for 3-8.5 yr further decreased their serum ceruloplasmin levels. Post-treatment serum ferroxidase activity was low as was the serum ceruloplasmin protein. Copper contents in the liver decreased after treatment in all subjects. In contrast, nonheme iron in the liver increased during treatment. Pretreatment liver specimens were positive for histochemical iron in two patients, and post-treatment specimens were positive in all four patients. In two patients, serum aminotransferase levels rebounded with elevation of serum ferritin concentration during the treatment period. Subsequent iron reduction by phlebotomy ameliorated their biochemical liver damage. CONCLUSION:
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Authors | Y Shiono, S Wakusawa, H Hayashi, T Takikawa, M Yano, T Okada, H Mabuchi, S Kono, H Miyajima |
Journal | The American journal of gastroenterology
(Am J Gastroenterol)
Vol. 96
Issue 11
Pg. 3147-51
(Nov 2001)
ISSN: 0002-9270 [Print] United States |
PMID | 11721763
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Chelating Agents
- Iron
- Penicillamine
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Topics |
- Adolescent
- Adult
- Chelating Agents
(therapeutic use)
- Hepatolenticular Degeneration
(drug therapy, metabolism)
- Humans
- Iron
(metabolism)
- Liver
(metabolism)
- Male
- Penicillamine
(therapeutic use)
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