Techniques

How is Wilson’s disease treated?

Wilson’s disease is also known medically as hepatolenticular degeneration which is an autosomal recessive genetic disorder. In this disorder copper accumulates in tissues.

Overview
- This manifests as neurological or psychiatric symptoms and liver disease.
- It is treated with medication that reduces copper absorption
- Medication that removes the excess copper from the body also can be used.
- Occasionally a liver transplant is required for this disorder.
- The condition is due to mutations in the Wilson disease protein (ATP7B) gene.
- A single abnormal copy of the gene is present in 1 in 100 people.
- If a child inherits the gene from both parents, the child may develop Wilson’s disease.
- Symptoms usually appear between the ages of 6 and 20 years.
- Wilson’s disease occurs in 1 to 4 per 100,000 people.
- Wilson’s disease is named after Samuel Alexander Kinnier Wilson (1878–1937) who was the British neurologist who first described this condition in 1912.
- This condition is most common in eastern Europeans, Sicilians, and southern Italians.
- A small amount of copper obtained from food is needed to stay healthy, but excess copper is poisonous.
- High copper levels can cause life-threatening organ damage.

In Wilson disease, copper builds up in:
- the liver
- brain
- kidneys
- eyes
- other organs

Treatment of Wilson Disease
- The goal of treatment is to reduce the amount of copper in the tissues.
- This is done by a procedure called chelation.
- This process is where certain medications can bind to copper and help remove it through the kidneys or gut.
- Treatment must be done for lifelong.

The following medications may be used:
- Penicillamine (Cuprimine, Depen) binds to copper and leads to increased release of copper in the urine.
- Trientine (Syprine) binds (chelates) the copper and increases its release through the urine.
- Zinc acetate (Galzin) blocks copper from being absorbed in the intestinal tract.
Vitamin E supplements may also be used.

Risks
- Medications that chelate copper (especially penicillamine) can affect the function of the brain and nervous system (neurological function).
- Other medications under investigation may bind copper without affecting neurological function.
- Lifelong treatment is needed to control Wilson’s disease.
- The disorder may cause fatal effects.
- Loss of liver function and toxic effects of copper on the nervous system are some of the side effects.
- In cases where the disorder is not fatal, symptoms may be disabling.

Dietary Recommendations
A low-copper diet may also be recommended.
Foods to avoid include:
- Chocolate
- Dried fruit
- Liver
- Mushrooms
- Nuts
- Shellfish

Other things to care about:
- You may want to drink distilled water.
- Most tap water flows through copper pipes.
- Avoid using copper cooking utensils.
- Symptoms may be treated with exercise or physical therapy.
- People who are confused or unable to care for themselves may need special protective measures.
- A liver transplant may be considered in cases.
- This is done where the liver is severely damaged by the disease.

Possible Complications:
- Anemia (hemolytic anemia is rare)
- Central nervous system complications
- Cirrhosis
- Death of liver tissues
- Fatty liver
- Hepatitis
- Increased number of bone fractures
- Increased number of infections
- Injury caused by falls
- Jaundice
- Joint contractures or other deformity
- Loss of ability to care for self
- Loss of ability to function at work and home
- Loss of ability to interact with other people
- Loss of muscle mass (muscle atrophy)
- Psychological complications
- Side effects of penicillamine and other medications used to treat the disorder.
- Spleen problems

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What are different symptoms of liver cancer?

Liver cancer is also known as hepatocellular carcinoma, HCC.
- It is a cancer that originates in liver.
- Liver cancers are malignant tumors that grow on the surface or inside the liver.
- It is also known as primary liver cancer or hepatoma.
- The liver is made up of different cell types.
- They are cancer of, for example, bile ducts, blood vessels, and fat-storing cells.
- Liver cells (hepatocytes) make up 80% of the liver tissue.
- The majority of primary liver cancers (over 90%-95%) arise from liver cells.
- Liver cancer is often referred to cancer that has spread to the liver that has originated in other organs.
- This type of liver cancer is called metastatic liver disease (cancer) or secondary liver cancer.
- The term liver cancer actually can refer to either metastatic liver cancer or hepato cellular cancer.

Rarer forms of liver cancer include:
- Mesenchymal tissue
- Sarcoma
- Hepatoblastoma
- Cholangiocarcinoma (bile duct cancers)
- Angiosarcoma and hemangiosarcoma
- Lymphoma of liver

Signs and Symptoms of Liver Cancer
- Abdominal mass
- Abdominal pain
- Jaundice
- Nausea
- Liver dysfunction

What are liver cancer symptoms and signs?
- The initial symptoms are not known as these cancers are mostly recognized only at advanced stages.
- Abdominal pain is uncommon with liver cancer
- It usually signifies a very large tumor or widespread involvement of the liver.
- Unexplained weight loss.
- Unexplained fevers.
- The sudden appearance of ascites (abdominal fluid and swelling).
- Jaundice (yellow color of the skin)
- Muscle wasting without causative (precipitating) factors (for example, alcohol consumption).
- Block in the portal vein (a large vein that brings blood to the liver from the intestine and spleen).
- The blood will travel paths of less resistance, such as through esophageal veins which leads to increased pressure in these veins.
- Dilated (widened) veins called esophageal varices occur.
- The patient then is at risk for hemorrhage from the rupture of the varices into the gastrointestinal tract.
- Turbulent blood flow in the artery.
- The turbulence results in a distinct sound in the liver (hepatic bruit).
- Muscle wasting.
- Very swollen liver and massive formation of ascites.
- In some patients, as previously mentioned, the tumor can invade the portal vein and lead to the rupture of esophageal varices.

Symptoms of Cholangiocarcinoma
- Sweating
- Jaundice
- Abdominal pain
- Weight loss
- Hepatomegaly

Symptoms of Hepatocellular carcinoma
- Abdominal mass
- Abdominal pain
- Emesis
- Anemia
- Back pain
- Jaundice
- Itching
- Weight loss
- Fever