Document Detail

Iron accumulation in the liver of male patients with Wilson's disease.
MedLine Citation:
PMID:  11721763     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: There is accumulating evidence that ceruloplasmin, a copper protein with ferroxidase activity, plays an important role in iron metabolism. The genetic disorder, aceruloplasminemia, can lead to tissue storage of iron as in hemochromatosis. Because most patients with Wilson's disease, a genetic copper toxicosis, have hypoceruloplasminemia, some could be affected by iron overload. 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: Iron overload related to hypoceruloplasminemia may be clinically important, particularly in male patients with Wilson's disease.
Y Shiono; S Wakusawa; H Hayashi; T Takikawa; M Yano; T Okada; H Mabuchi; S Kono; H Miyajima
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The American journal of gastroenterology     Volume:  96     ISSN:  0002-9270     ISO Abbreviation:  Am. J. Gastroenterol.     Publication Date:  2001 Nov 
Date Detail:
Created Date:  2001-11-27     Completed Date:  2002-01-24     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0421030     Medline TA:  Am J Gastroenterol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  3147-51     Citation Subset:  IM    
Department of Medicine, Faculty of Pharmaceutical Sciences, Hokuriku University, Kanazawa, Japan.
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MeSH Terms
Chelating Agents / therapeutic use*
Hepatolenticular Degeneration / drug therapy*,  metabolism*
Iron / metabolism*
Liver / metabolism*
Penicillamine / therapeutic use*
Reg. No./Substance:
0/Chelating Agents; 52-67-5/Penicillamine; 7439-89-6/Iron
Comment In:
Am J Gastroenterol. 2001 Nov;96(11):3055-7   [PMID:  11721750 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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