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Iron Overload in
ACERULOPLASMINEMIA
In aceruloplasminemia, a deficiency in ceruloplasmin (also known as ferroxidase) prevents adequate oxidization of Fe2+ to Fe3+. As transferrin only binds iron in the ferric state, this inhibits the binding of iron by transferrin,(1,2) causing the absorption of NTBI from the diet, and exposing vital organs to oxidative stress. The movement of iron from intracellular stores into plasma is impaired, leading to iron overload.
Learn about transfusional iron overload
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Iron and
Skeletomuscular Damage
Chronic iron
overload may
lead over time to osteoporosis, arthropathy, muscle cramps, or myalgia.
References
- * (1)Miyajima H, Genetic disorders affecting proteins of iron and copper metabolism: clinical implications. Intern Med. 2002;41(10):762-9.
- * (2)Sheth S, Brittenham GM, Genetic disorders affecting proteins of iron metabolism: clinical implications. Annu Rev Med. 2000;51:443-64.