MyMedExpert Health Articles

Hemochromatosis (HE-mo-kro-ma-TOE-sis) is a disease in which too much iron builds up in your body. This extra iron is toxic to the body and can damage the organs. If hemochromatosis is not treated, it can lead to illness or even death.

Most people with hemochromatosis inherit the condition from their parents. If you inherit two hemochromatosis genes, one from each parent, you will have the condition. These two abnormal genes cause your body to absorb more iron than usual from the diet.

Hemochromatosis is one of the most common inherited (genetic) diseases in the United States. Approximately 1 million people in the United States have the pair of hemochromatosis genes. But not everyone with the pair of hemochromatosis genes develops signs and symptoms of the disease. Estimates of how many people actually develop signs and symptoms of iron overload vary greatly. The estimates range as high as half of all people with the two hemochromatosis genes.
Effects on the Body

In hemochromatosis, iron can build up in most of your body’s organs, but especially in the liver, heart, and pancreas. When this happens, the iron can poison the organs and lead to organ failure.

* Liver. Hemochromatosis can lead to enlargement, cirrhosis (sir-RO-sis; scarring of the liver so it does not work properly), failure, or cancer of the liver.
* Heart. Hemochromatosis can cause irregular heart rate or rhythm (arrhythmia) and lead to heart failure.
* Pancreas. Hemochromatosis can lead to diabetes mellitus.

Characteristics of Hemochromatosis

How serious the disease is varies from person to person. Some people never have any symptoms or complications, even with high iron levels. Others have serious side effects or die from the disease. Certain factors can affect how serious the disease is. For example, other genes, besides the hemochromatosis genes, may change or lessen the severity of the disease. Vitamin C in the diet can increase the amount of iron the body absorbs from food and make hemochromatosis worse. Alcohol use can increase liver damage and cirrhosis. Certain conditions, such as hepatitis, can damage or weaken the liver.
Outlook

The outlook for people with hemochromatosis depends on how much organ damage has already occurred at the time of diagnosis. Early diagnosis and treatment are important. Treatment may be able to prevent, delay, or sometimes reverse complications of the disease. Treatment may lead to higher energy levels and better quality of life. For people who are diagnosed and treated early, normal lifespans are possible. If left untreated, hemochromatosis can lead to severe organ damage and even death.

If you have any specific question, you can ask the doctors at www.mymedexpert.com

Primary biliary cirrhosis is a disease that slowly destroys the liver’s bile ducts. Bile is a substance produced in the liver that helps digest fat in the small intestine and remove toxins from the body. When the ducts are damaged, bile builds up in the liver and damages liver tissue. Biliary cirrhosis can develop over time and may cause the liver to stop working.

The cause of primary biliary cirrhosis is unknown. The disease affects women more often than men and usually occurs between the ages of 30 and 60 years. Some research suggests that the disease may be an autoimmune disorder.
What are the symptoms of primary biliary cirrhosis?

The first and most common symptoms of primary biliary cirrhosis are itchy skin and fatigue. Other symptoms may eventually develop, including

* jaundice, which leads to a yellowing of the eyes and skin
* fatty deposits under the skin
* fluid retention
* dry eyes and mouth

In the later stages of the disease, some people develop osteoporosis, arthritis, and thyroid problems.

How is primary biliary cirrhosis diagnosed?

Primary biliary cirrhosis is diagnosed through laboratory tests, x rays, and in some cases, a liver biopsy to help to determine the extent of disease progression. A liver biopsy uses a thin needle to remove a small sample of liver tissue. The sample is then examined with a microscope.

How is primary biliary cirrhosis treated?

Initial treatment is usually aimed at relieving symptoms. Vitamin replacement therapy, calcium supplements, and drugs to treat itching are usually prescribed.

Some patients have also benefitted from ursodeoxycholic acid (Urso 250 and URSO Forte), which is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis. Ursodiol (Actigall) has also helped some patients by increasing bile flow. Neither of these drugs cure the disease, but they can help delay its progression. If the liver becomes severely damaged, a transplant may be necessary.

Liver biopsy is considered minor surgery, so it is done at the hospital. For the biopsy, you will lie on a hospital bed on your back with your right hand above your head. After marking the outline of your liver and injecting a local anesthetic to numb the area, the physician will make a small incision in your right side near your rib cage, then insert the biopsy needle and retrieve a sample of liver tissue. In some cases, the physician may use an ultrasound image of the liver to help guide the needle to a specific spot.

You will need to hold very still so that the physician does not nick the lung or gallbladder, which are close to the liver. The physician will ask you to hold your breath for 5 to 10 seconds while he or she puts the needle in your liver. You may feel pressure and a dull pain. The entire procedure takes about 20 minutes.

Two other methods of liver biopsy are also available. For a laparoscopic biopsy, the physician inserts a special tube called a laparoscope through an incision in the abdomen. The laparoscope sends images of the liver to a monitor. The physician watches the monitor and uses instruments in the laparoscope to remove tissue samples from one or more parts of the liver. Physicians use this type of biopsy when they need tissue samples from specific parts of the liver.

Transvenous biopsy involves inserting a tube called a catheter into a vein in the neck and guiding it to the liver. The physician puts a biopsy needle into the catheter and then into the liver. Physicians use this procedure when patients have blood-clotting problems or fluid in the abdomen.

If you have any specific question, you can ask the doctors at www.mymedexpert.com

Cirrhosis has many causes. In the United States, chronic alcoholism and hepatitis C are the most common ones.

Alcoholic liver disease. To many people, cirrhosis of the liver is synonymous with chronic alcoholism, but in fact, alcoholism is only one of the causes. Alcoholic cirrhosis usually develops after more than a decade of heavy drinking. The amount of alcohol that can injure the liver varies greatly from person to person. In women, as few as two to three drinks per day have been linked with cirrhosis and in men, as few as three to four drinks per day. Alcohol seems to injure the liver by blocking the normal metabolism of protein, fats, and carbohydrates.

Chronic hepatitis C. The hepatitis C virus ranks with alcohol as a major cause of chronic liver disease and cirrhosis in the United States. Infection with this virus causes inflammation of and low grade damage to the liver that over several decades can lead to cirrhosis.

Chronic hepatitis B and D. The hepatitis B virus is probably the most common cause of cirrhosis worldwide, but it is less common in the United States and the Western world. Hepatitis B, like hepatitis C, causes liver inflammation and injury that over several decades can lead to cirrhosis. Hepatitis D is another virus that infects the liver, but only in people who already have hepatitis B.

Autoimmune hepatitis. This disease appears to be caused by the immune system attacking the liver and causing inflammation, damage, and eventually scarring and cirrhosis.

Inherited diseases. Alpha-1 antitrypsin deficiency, hemochromatosis, Wilson disease, galactosemia, and glycogen storage diseases are among the inherited diseases that interfere with the way the liver produces, processes, and stores enzymes, proteins, metals, and other substances the body needs to function properly.

Nonalcoholic steatohepatitis (NASH). In NASH, fat builds up in the liver and eventually causes scar tissue. This type of hepatitis appears to be associated with diabetes, protein malnutrition, obesity, coronary artery disease, and treatment with corticosteroid medications.

Blocked bile ducts. When the ducts that carry bile out of the liver are blocked, bile backs up and damages liver tissue. In babies, blocked bile ducts are most commonly caused by biliary atresia, a disease in which the bile ducts are absent or injured. In adults, the most common cause is primary biliary cirrhosis, a disease in which the ducts become inflamed, blocked, and scarred. Secondary biliary cirrhosis can happen after gallbladder surgery if the ducts are inadvertently tied off or injured.

Drugs, toxins, and infections. Severe reactions to prescription drugs, prolonged exposure to environmental toxins, the parasitic infection schistosomiasis, and repeated bouts of heart failure with liver congestion can all lead to cirrhosis.

If you have any specific question, you can ask the doctors at www.mymedexpert.com