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31 - Management of hemochromatosis
- from Part VI - Therapy of hemochromatosis and iron overload
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- By James C. Barton, Southern Iron Disorders Center, Birmingham, Alabama, Sharon M. McDonnell, Centers for Disease Control and Prevention, Atlanta. Georgia, Paul C. Adams, London Health Sciences Centre, University of Western Ontario, London, Ontario, Canada, Pierre Brissot, Hôpital Universitaire Pontchaillou, Rennes, France, Lawrie W. Powell, University of Queensland, Brisbane, Australia, Corwin Q. Edwards, University of Utah College of Medicine and LDS Hospital, Salt Lake City, Utah, James D. Cook, University of Kansas Medical Center, Kansas City, Kansas, Kris V. Kowdley, University of Washington, Seattle, Washington, USA
- Edited by James C. Barton, Southern Iron Disorders Center, Alabama, Corwin Q. Edwards, University of Utah
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- Book:
- Hemochromatosis
- Published online:
- 05 August 2011
- Print publication:
- 13 January 2000, pp 329-338
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- Chapter
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Summary
Introduction
The complications of iron overload in the hemochromatosis can be avoided by early diagnosis and appropriate management. Therapeutic phlebotomy is used to remove excess iron and maintain low normal body iron stores, and it should be initiated in men with serum ferritin levels of 300 g/l or more and in women with serum ferritin levels of 200 μg/l or more, regardless of the presence or absence of symptoms. Typically, therapeutic phlebotomy consist of (i) removal of 1 unit (450 to 500 ml) of blood weekly untilthe serum ferritin level is 10 to 20 μg/l and (ii) maintenance of the serum ferritin level at 50 μg/l or less the reafter by periodic removal of blood. Hyperferritinemia attributable to iron overload is resolved by therapeutic phlebotomy. When applied before iron overload becomes severe, this treatment also prevents complications of iron overload, including hepatic cirrhosis, primary liver cancer, diabetes mellitus, hypogonadotrophic hypogonadism, joint disease, and cardiomyopathy. In patients with established iron overload disease, weakness, fatigue, increased hepaticenzyme concentrations, right upper quadrant pain, and hyperpigmentation are often substantially alleviated by therapeutic phlebotomy. Patients with liver disease, joint disease, diabetes mellitus and other endocrinopathic abnormalities, and cardiac abnormalities of ten require additional, specific management. Dietary management of hemochromatosis includes avoidance of medicinal iron, mineral supplements, excess vitamin C, and uncooked sea foods. This can reduce the rate of iron reaccumulation; reduce retention of non-ferrous metals; and help reduce complications of liver disease, diabetes mellitus, and Vibrio infection. This comprehensive approach to the management of hemochromatosis can decrease the frequency and severity of iron overload, improve quality of life, and increase longevity.
37 - Chronic viral hepatitis and hemochromatosis
- from Part VII - Infections and immunity in hemochromatosis
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- By Kris V. Kowdley, University of Washington School of Medicine, Seattle, USA
- Edited by James C. Barton, Southern Iron Disorders Center, Alabama, Corwin Q. Edwards, University of Utah
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- Book:
- Hemochromatosis
- Published online:
- 05 August 2011
- Print publication:
- 13 January 2000, pp 387-395
-
- Chapter
- Export citation
-
Summary
Introduction
The liver synthesizes transferrin and ferritin, and is the major tissue storage site of iron. Consequently, iron metabolism may be altered in chronic hepatitis. Stored ferritin may be released into the circulation due to lysis of damaged hepatocytes, leading to elevated serum ferritin concentrations. Transferrin receptor expression by hepatocytes may be up-regulated as a consequence of chronic inflammation, and lead to increased iron absorption and hepatic iron. Chronic viral hepatitis may up-regulate hepatic production of transferrin receptor or ferritin directly and effect increased hepatic iron deposition. Thus, chronic inflammatory processes in the liver such as viral hepatitis may cause increased plasma and hepatic iron concentrations by a variety of mechanisms. Ferrous iron promotes free radical formation and lipid peroxidation that mediates acute and chronic tissue damage. Because iron status influences the immune response, primary iron overload disorders and hepatic iron overload have been linked to an increased risk of bacterial infections due to Vibrio, Neisseria, and Yersinia. Iron may act as a substrate for viral replication, and therefore patients with chronic viral hepatitis and iron overload may have increased viral replication. The iron-binding proteins lactoferrin and transferrin play an important role in the immune response to infection. Although the effect of lactoferrin on hepatitis C virus replication is unknown, lactoferrin inhibits replication of herpes simplex virus, cytomegalovirus, and human immunodeficiency virus in vitro. Therefore, iron status in patients with chronic viral hepatitis may be important clinically due to its effects on hepatic inflammation and viral replication, and reducing iron stores may decrease hepatic injury in patients with chronic hepatitis.
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