| If asked to name the
most prevalent inherited diseases, most people would probably suggest cystic
fibrosis, sickle cell anemia or hemophilia. Few would even have heard of
hereditary hemochromatosis. But this disease, characterized by the uptake
of too much iron into a sufferer's body, affects one in every three hundred
Caucasians, and one in nine is a carrier. Iron is crucial for the working
of hemoglobin, the red pigment in blood that carries oxygen from the lungs
to all other parts of the body. But too much iron can be as disabling as
too little.
In hereditary hemochromatosis deposits of iron appear
in practically every major organ, particularly the liver, pancreas and
heart, resulting in complete and widespread organ failure. It was first
described in 1865. The majority of patients have a Celtic ancestor who
lived 60 or 70 generations ago.
Patients with hemochromatosis regularly absorb two
to three times as much dietary iron as normal perons.
Nature 403, 46 (2000)
New England Journal
of Medicine December 23, 1999 341:1986-1995
| 