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Hemochromatosis
Iron overload
Why does hemochromatosis cause…
Bronze skin
Liver disease
DM
Arthropathy
Heart disorders
Hypogonadotropic hypogonadism
Due to iron deposits and stimulating melanocytes
Increased iron scars the liver → Cirrhosis
Iron does the same to the liver by becoming ROS → Pancreatitis → Less insulin → DM
Arthropathy → Iron can crystallize in joints
Iron even deposits in the heart
Same here, iron can deposit in the APG
Iron Chelation
Using drugs to help bind free iron/remove the amount of excess iron there is in the body
Why do patients who receive tranfusions often need iron chelating agents?
Because RBC transfusions come with a base amount of iron that can accumulate
Deferoxamine
Function
Administration
ADR(3)
Binds iron and gets excreted mostly by kidneys
Given parenterally
Retinal toxicity, auditory toxicity, bone abnormalities due to deferoxamine binding other metals and reducing iron which is needed in cartilage proliferation at growth plates
Deferiprone
Function
Administration
Quirk
ADR(1)
A smaller, lipophillic chelator that can get into cells to reach deposited iron
PO
Can work well with Deferoxamine and shuttle iron to cells for Hb synthesis
Neutropenia
Deferasirox
Function
Administration
ADRs(2)
Monitor how?
Lipophilic chelator that binds iron and secreted via bile into stool
PO
Renal/hepatic
Serum creatinine and LFTs