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01194.txt
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1994-01-17
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$Unique_ID{BRK01194}
$Pretitle{}
$Title{What Causes Hemochromatosis?}
$Subject{iron tissues ill defined symptoms rare diseases hemochromatosis
disease cirrhosis liver bronze pigmentation inherited recessive trait
chromosomes gene phlebotomy genetic chromosome blood irons tissue symptom
inherit}
$Volume{N-7,V-7}
$Log{
Diseases of the Liver*0016101.scf}
Copyright (c) 1991-92,1993 Tribune Media Services, Inc.
What Causes Hemochromatosis?
------------------------------------------------------------------------------
QUESTION: I have hemochromatosis. Please describe this disease, the cause
and treatment, the side effects and prognosis for cure. Can this disease be
transmitted to my children? Are there any organizations that could provide
further information or inform me of medical centers that specialize in the
treatment of this disease?
------------------------------------------------------------------------------
ANSWER: A pretty tall order for just one day's column; it really deserves a
medical book chapter. But since I can provide you with some useful
information that may help, here goes. Hemochromatosis is a disease in which
large amounts of iron are deposited in body tissues, causing local damage and
tissue injury. It is rarely seen before middle age, and is most common in men
(about 10 to 1 over women) between the ages of 40 and 60. Typically it
produces cirrhosis of the liver, a bronze pigmentation to the skin, and
diabetes in from 50 percent to 60 percent of the patients. It can also affect
the heart, causing enlargement, conduction disturbances leading to irregular
beating and congestive heart failure. It is inherited as a recessive trait,
and both chromosomes must carry the affected gene for it to produce clinical
symptoms. Blood tests do exist that can help identify the presence of the
affected gene in first degree relatives. Blood tests also can reveal the
presence of abnormally large quantities of iron in the blood serum. The
treatment of this disease is phlebotomy, or the regular withdrawal of blood.
For every pint of blood removed, about 250 mg of iron is removed from the
patient. This may be carried out as frequently as is necessary to reduce the
amount of iron deposited in the tissues, and may be carried out weekly at the
beginning of treatment. Once the condition has been brought under control, as
demonstrated by normal blood serum iron levels, the removal of a pint of blood
every two to three months generally suffices to keep things under control.
The complications of diabetes, cirrhosis and heart abnormalities are treated
with medications that are indicated and usually prescribed in those
circumstances. Your best bet for more information about this complex disease
is to direct your inquiry to the Hereditary Hemochromatosis Research
Foundation, P.O. Box 8569, Albany, NY 12208. They might be able to provide
the names of medical centers with special expertise in the disease as well.
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The material contained here is "FOR INFORMATION ONLY" and should not replace
the counsel and advice of your personal physician. Promptly consulting your
doctor is the best path to a quick and successful resolution of any medical
problem.