Lahey Transplant - Liver Diseases

Diseases Requiring Transplantation

Liver disease has varying degrees of severity and most of them do not require transplantation. However, when the liver function deteriorates to the point that life is endangered, liver transplantation should be considered. Liver disease this severe is called end stage liver disease (ESLD) and there are many types of diseases that can lead to ESLD. Some of these liver diseases are listed below:

Diseases

Chronic Viral Hepatitis (B, C, D)

Hepatitis is a general term that means inflammation of the liver. Many forms of hepatitis are the result of viral infections. The most common viral infections leading to liver transplantation are Hepatitis B and C. In the United States, Hepatitis C is the most common cause of ESLD while Hepatitis B is the most common cause of ESLD worldwide. There are several other viruses that may lead to ESLD but more often these other viruses cause a transient illness from which patients fully recover. One example of this type of virus is Hepatitis A, which usually causes acute hepatitis but may occasionally cause a sudden and severe disease resulting in rapid destruction of the liver.

Alcoholic Liver Disease (Laennec's Cirrhosis)

It is very common for individuals to suffer from liver damage when drinking large amounts of alcohol over a prolonged period of time. This damage may develop into fatty liver, alcoholic hepatitis, or cirrhosis. Fatty liver is reversible if alcohol consumption stops. Hepatitis or cirrhosis, however, is more serious, and may require a new liver transplant in order to fully recover.

Acute Liver Failure

Acute liver failure occurs when the liver stops working without warning. There are multiple causes for acute liver failure. Viral causes have been described above. Other common causes include ingestion of substances that are toxic to the liver such as large amounts of Tylenol or certain types of wild mushrooms. Some people lack the ability to break down certain drugs. If these individuals are exposed to such drugs, severe liver damage can result.

Autoimmune Hepatitis

This condition occurs when the immune system malfunctions and destroys liver tissue. It also has varying degrees of severity. It is a disease characterized by "flares," or periods of intense disease activity followed by periods of quiescence in which minimal or no disease activity is noted.

Nonalcoholic Fatty Liver Disease (NAFLD) and Nonalcoholic Steatohepatitis (NASH)

These are related diseases in which fat deposits accumulate in the liver. NAFLD is associated with lower amounts of fatty deposits and inflammation, does not show cirrhosis (irreversible scarring of the liver), and does not require liver transplantation. It may progress to NASH, a condition in which there are large deposits of fat in the liver as well as significant inflammation and impaired liver function. Cirrhosis frequently develops in this condition and leads to the need for liver transplantation.

Primary Biliary Cirrhosis (PBC)

This is a progressive liver disease (becomes worse over time) that occurs more commonly in women. The main characteristic of this disease is the destruction of small bile ducts within the liver. Bile ducts normally collect the bile produced in the liver and transport it to other parts of the digestive system. When bile is allowed to accumulate in the liver, toxic substances within that bile kill the liver cells they touch. The disease is thought to have some autoimmune features and may initially be treated with medications that suppress the immune system.

Primary Sclerosing Cholangitis (PSC)

This is a progressive liver disease that occurs more commonly in men. It eventually affects all the bile ducts both inside and outside of the liver and results in cirrhosis, portal hypertension (increased pressure in many of the liver's blood vessels) and liver failure. It frequently occurs in patients who also have inflammatory bowel disease. The cause is not known.

Hepatic Tumors

There are many types of liver tumors. The most common are listed below. Some patients with liver tumors may benefit from liver transplantation, but very strict rules must be satisfied regarding size and number of tumors, as well as absence of widespread (metastatic) disease.

Neuroendocrine Tumors

These tumors arise from cells that produce various types of hormones. The symptoms experienced by a patient will depend on the specific type of hormone produced.

Hepatocellular Carcinoma (HCC)

This is the most common tumor that develops directly from liver tissue. It is strongly associated with the presence of cirrhosis and chronic viral hepatitis; exposure to certain toxic chemicals may also increase the chances of developing HCC. Patients with early stage HCC are often good candidates for liver transplantation.

Cholangiocarcinoma

This is a relatively rare tumor that arises from cells lining the bile ducts. It occurs more often in patients who also have PSC.

Metabolic and Genetic Disorders

This refers to a group of diseases in which individuals are born lacking the ability to correctly process a specific compound. In general, this will lead to a toxic concentration of the compound in the liver and will result in different degrees of liver damage. As in the case of liver tumors, strict criteria must be met in order for liver transplantation to be a successful treatment. Some of the conditions in this category are listed below.

Alpha 1 Antitrypsin Deficiency

This is a common inherited disorder. A1AT is a protein that helps with the elasticity of the lungs, and it’s predominantly produced in the liver. Having a deficiency of A1AT means that the liver isn’t producing enough of this protein, and it may lead to emphysema, COPD, or other respiratory complications. About 10 to 15% of people with A1AT deficiency also develop liver disease, and a smaller percentage of those will need a liver transplant. Liver transplantation usually returns A1AT production to normal.

Hemochromatosis

People with this disorder absorb an abnormally high amount of iron from their intestines. The liver is a major site of iron storage; in individuals with hemochromatosis, excess iron accumulates in liver cells and often causes the liver to enlarge. However, not all affected people will develop significant liver disease. Additional factors, such as bacterial or viral infections or alcohol consumption are thought to be involved in the progression from iron overload to need for transplantation.

Wilson's Disease

This is an accumulation of copper in the liver, brain, and central nervous system, arising from an inability to properly metabolize the element. It is usually diagnosed in younger individuals (< 30 years) and first treatment is generally with medications that cause the excess copper to be excreted. If drug treatment does not work, liver transplantation will be considered.

Glycogen Storage Disease(s)

This actually refers to several conditions that have in common an inherited inability to correctly process glycogen resulting in abnormal amounts of glycogen in the liver. Normal liver cells produce enzymes needed to deal with some forms of glycogen; therefore, transplantation may be a good therapeutic alternative for selected patients with these deficiencies.

Polycystic Disease

This is an inherited disorder characterized by formation of cysts in kidneys and livers. The cysts in the kidney are usually diagnosed first, although some patients first develop complaints related to liver cysts. Symptoms develop more frequently in female adults and increase with age; the degree of severity varies widely. It is believed that pregnancy or use of hormonal therapy contribute to higher frequency in women. Liver transplantation or combined liver and kidney transplantation in patients who also have significant renal disease are common treatments for those patients who fail to respond to medical management of their disease.

Budd Chiari Syndrome

This syndrome occurs when blood clots form in the large veins that take blood from the liver into general circulation. If the clots are small and do not completely block the flow of blood from the liver, there may be few symptoms and medications to dissolve the clot may be all that is necessary. If the clot is large and completely blocks the blood vessels, blood will back up into the liver and, if not fixed, will cause severe liver damage. In some cases, it may be possible to "re-route" the blood flow by performing an operation. In many cases, liver transplantation will be necessary.