2 results
31 - Management of hemochromatosis
- from Part VI - Therapy of hemochromatosis and iron overload
-
- 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
-
- Book:
- Hemochromatosis
- Published online:
- 05 August 2011
- Print publication:
- 13 January 2000, pp 329-338
-
- Chapter
- Export citation
-
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.
13 - Ferritin metabolism in hemochromatosis
- from Part III - Metal absorption and metabolism in hemochromatosis
-
- By Gregory J. Anderson, Joint Clinical Sciences Program, Queensland Intitute of Medical Research and the University of Queensland, PO Royal Brisbane Hospital, Brisbane, Queensland, Australia, Grant A. Ramm, Joint Clinical Sciences Program, Queensland Intitute of Medical Research and the University of Queensland, PO Royal Brisbane Hospital, Brisbane, Queensland, Australia, June W. Halliday, Joint Clinical Sciences Program, Queensland Intitute of Medical Research and the University of Queensland, PO Royal Brisbane Hospital, Brisbane, Queensland, Australia, Lawrie W. Powell, Joint Clinical Sciences Program, Queensland Intitute of Medical Research and the University of Queensland, PO Royal Brisbane Hospital, Brisbane, Queensland, Australia
- Edited by James C. Barton, Southern Iron Disorders Center, Alabama, Corwin Q. Edwards, University of Utah
-
- Book:
- Hemochromatosis
- Published online:
- 05 August 2011
- Print publication:
- 13 January 2000, pp 145-156
-
- Chapter
- Export citation
-
Summary
Introduction
In all mammalian cells, iron in excess of current metabolic requirements is incorporated into ferritin. Effective iron storage is an essential component of cellular iron homeostasis, because iron not sequestered within the cell can catalyze potentially cytotoxic free radical-generating reactions. Although all cells can store iron in ferritin, macrophages and hepatocytes are particularly adapted for this function and retain excess iron as a reserve for times of increased body iron needs. The hepatocyte can take up iron in a variety of different forms and act as a major site of available iron stores, and thus has a central ‘buffering’ role in internal iron exchange.
Because hemochromatosis is an iron storage disorder, ferritin, the principal iron storage protein, plays an important role in the disease. Ferritin sequesters the iron distributed throughout the body as a consequence of elevated intestinal iron absorption. The serum ferritin concentration accurately reflects the body iron load and provides a valuable diagnostic tool. The iron in ferritin is not biologically inert but can be utilized readily for various cellular functions. The ability of ferritin to release iron in times of demand is essential physiologically but also underlies the treatment of hemochromatosis by phlebotomy therapy.
Aspects of ferritin metabolism relevant to hemochromatosis will be discussed in this chapter. The areas covered include a brief overview of ferritin biochemistry, a discussion of ferritin synthesis and its regulation in the intestinal mucosa, the liver and the reticuloendothelial (RE) system, and the role played by the serum ferritin concentration in the diagnosis of hemochromatosis.