HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Iron accumulation in the liver of male patients with Wilson's disease.

AbstractOBJECTIVES:
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.
AuthorsY Shiono, S Wakusawa, H Hayashi, T Takikawa, M Yano, T Okada, H Mabuchi, S Kono, H Miyajima
JournalThe American journal of gastroenterology (Am J Gastroenterol) Vol. 96 Issue 11 Pg. 3147-51 (Nov 2001) ISSN: 0002-9270 [Print] United States
PMID11721763 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Chelating Agents
  • Iron
  • Penicillamine
Topics
  • Adolescent
  • Adult
  • Chelating Agents (therapeutic use)
  • Hepatolenticular Degeneration (drug therapy, metabolism)
  • Humans
  • Iron (metabolism)
  • Liver (metabolism)
  • Male
  • Penicillamine (therapeutic use)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: