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Wilson disease is a disease of the liver associated with increased levels of copper.  In Wilson disease, the liver does not properly release copper into bile the way that it normally should.  Remember that bile is a liquid produced by the liver that helps with the digestion of fats.

Similar to hereditary hemochromatosis, which is a disease of increased iron absorption, Wilson disease is a disorder associated with increased absorption of copper from food.  Copper then builds up in the liver and other tissue resulting in organ injury.  Eventually, the excess of copper that builds up in the liver is released directly into the blood stream carrying it throughout the blood to other organs.  Excess of copper will lead to damage to the eyes, brain, and kidneys.  In untreated cases, severe brain injury, liver failure, and death can occur.

Symptoms of Wilson disease can occur between age 5 and 25 years old though can be seen in patients over 40.  Careful examination of a patient’s eye will reveal Kayser Fleischer rings, which is a brown ring around the cornea of the eye that can only be seen during a detailed eye exam.  The diagnosis of Wilson disease is made through blood tests that measure the amount of copper in the blood, urine, and liver.  An eye exam, as noted above, can detect the Kayser-Fleischer ring.  A protein in the blood called ceruloplasmin is decreased in Wilson Disease and in the right clinical setting, this abnormal test can confirm the diagnosis.

The treatment for Wilson disease is removal of copper from the body.  One of the most common medications is D-penicillamine or trientine hydrochloride.  Additionally, zinc acetate, which stops the intestine from absorbing copper, will also promote copper excretion.

In patients that have Wilson disease, lifelong treatment is required.  Wilson disease is a condition that when more serious conditions developed may result in the need for liver transplantation.

Obesity and Fatty Liver Disease

Obesity, and all of its related complications, is more serious than most adults in America believe. More than one-third (34.9% or 78.6 million) of U.S. adults are obese. Obesity-related conditions include heart disease, stroke, type 2 diabetes, fatty liver disease, and certain types of cancer, are some of the leading causes of preventable death. We are seeing an increase in the number of young children and adolescents developing obesity, and all of the related complications.

The cost of obesity is staggering, with annual medical cost of obesity exceeding $147 billion in 2008 U.S. dollars. The medical costs for people who are obese were $1,429 higher than those of normal weight.

Dr. Galati and the Liver Specialists of Texas team are dedicated to evaluate, treat, and manage all aspects of obesity and non-alcoholic fatty liver disease (NAFLD and NASH), including the complication of cirrhosis and liver failure. Developing a customized plan of care for each patient they see is their objective.

Liver Transplant Resources

Dr. Galati has been involved in Liver Transplantation since 1989. As Medical Director for the Center for Liver Disease and Transplantation at Houston Methodist Hospital, Dr. Galati has cared for thousands of patients with advanced liver disease. In those with the most severe form of advanced liver disease and cirrhosis, liver transplantation is a life-saving surgery. For more information on liver transplantation, click here.

Indications for liver transplant include:

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