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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?
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-
- 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?
-
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-
- 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.
-
- ----------------
-
- 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.
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-