Cirrhosis
Frequently Asked Questions

What is Cirrhosis?
    Cirrhosis is a condition that affects the liver. The liver weighs about 3 pounds and is the largest organ in the body. It is located in the upper right side of the abdomen, below the ribs. When specific diseases cause the liver to become permanently injured and scarred, the condition is called cirrhosis.
    The scar tissue that forms in cirrhosis harms the structure of the liver, blocking the flow of blood through the organ. The loss of normal liver tissue slows the processing of nutrients, hormones, drugs, and toxins by the liver. Also slowed is production of proteins and other substances made by the liver.
    Cirrhosis is the seventh leading cause of death by disease in the U.S. Nationally, about 25,000 people die from cirrhosis each year. There also is a great toll in terms of human suffering, hospital costs, and the loss of work by people with cirrhosis.

What Are the Major Causes of Cirrhosis?
    Cirrhosis has many causes. In the United States, chronic alcoholism is the most common cause. Cirrhosis also may result from chronic viral hepatitis (types B, C, or D).
    Liver injury that results in cirrhosis also may be caused by a number of inherited diseases such as cystic fibrosis, alpha-1 antitrypsin deficiency, hemochromatosis, Wilson's disease, galactosemia, and glycogen storage diseases.
    Two inherited disorders result in the abnormal storage of metals in the liver leading to tissue damage and cirrhosis. People with Wilson's disease store too much copper in their livers, brains, kidneys, and in the corneas of their eyes.
    In another disorder, known as hemochromatosis, too much iron is absorbed, and the excess iron is deposited in the liver and in other organs, such as the pancreas, skin, intestinal lining, heart, and endocrine glands.
    If a person's bile duct becomes blocked, this also may cause cirrhosis. The bile ducts carry bile formed in the liver to the intestines, where the bile helps in the digestion of fat.
    In babies, the most common cause of cirrhosis due to blocked bile ducts is a disease called biliary atresia. In this case, the bile ducts are absent or injured, causing the bile to back up in the liver.
    These babies are jaundiced (their skin is yellowed) after their first month in life. Sometimes they can be helped by surgery in which a new duct is formed to allow bile to drain again from the liver.
    In adults, the bile ducts may become inflamed, blocked, and scarred due to another liver disease, primary biliary cirrhosis. Another type of biliary cirrhosis also may occur after a patient has gallbladder surgery in which the bile ducts are injured or tied off.
    Other, less common, causes of cirrhosis are severe reactions to prescribed drugs, prolonged exposure to environmental toxins, and repeated bouts of heart failure with liver congestion.

What Are The Symptoms and Complications of Cirrhosis?
    People with cirrhosis often have few symptoms at first. The two major problems that eventually cause symptoms are loss of functioning liver cells and distortion of the liver caused by scarring. The person may experience fatigue, weakness, and exhaustion. Loss of appetite is usual, often with nausea and weight loss.
    As liver function declines, less protein is made by the organ. For example, less of the protein albumin is made, which results in fluid accumulating in the legs (edema) or abdomen (ascites). A decrease in proteins needed for blood clotting makes it easy for the person to bruise or to bleed easily.
    In the later stages of cirrhosis, jaundice (yellow skin) may occur, caused by the buildup of bile pigment that is passed by the liver into the intestines.
    Some people with cirrhosis experience intense itching due to bile products that are deposited in the skin. Gallstones often form in persons with cirrhosis because not enough bile reaches the gallbladder.
    The liver of a person with cirrhosis also has trouble removing toxins, which may build up in the blood. These toxins can dull mental function and lead to personality changes and even coma (encephalopathy).
    Early signs of toxin accumulation in the brain may include neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, or changes in sleeping habits.
    Drugs taken usually are filtered out by the liver, and this cleansing process also is slowed down by cirrhosis. The liver does not remove the drugs from the blood at the usual rate, so the drugs act longer than expected, building up in the body. People with cirrhosis often are very sensitive to medications and their side effects.
    A serious problem for people with cirrhosis is pressure on blood vessels that flow through the liver. Normally, blood from the intestines and spleen is pumped to the liver through the portal vein. But in cirrhosis, this normal flow of blood is slowed, building pressure in the portal vein (portal hypertension). This blocks the normal flow of blood, causing the spleen to enlarge. So blood from the intestines tries to find a way around the liver through new vessels.
    Some of these new blood vessels become quite large and are called "varices." These vessels may form in the stomach and esophagus (the tube that connects the mouth with the stomach). They have thin walls and carry high pressure.
    There is great danger that they may break, causing a serious bleeding problem in the upper stomach or esophagus. If this happens, the individual's life is in danger, and action must be taken quickly to stop the bleeding.

How Is Cirrhosis Diagnosed?
    The doctor often can diagnosis cirrhosis from the individual's symptoms and from laboratory tests. During a physical exam, for instance, the doctor could notice a change in how your liver feels or how large it is. If the doctor suspects cirrhosis, he or she will recommend that you have blood tests.
    The purpose of these tests is to find out if liver disease is present. In some cases, other tests that take pictures of the liver (such as the computerized axial tomography scan, ultrasound, and the radioisotope liver/spleen scan) may be recommended.
    The doctor may suggest that the diagnosis be confirmed by putting a needle through the skin (biopsy) to take a sample of tissue from the liver.
    In some cases, cirrhosis is diagnosed during surgery when the doctor is able to see the entire liver. The liver also can be inspected through a laparoscope, a viewing device that is inserted through a tiny incision in the abdomen.

What Are the Treatment Options for Cirrhosis?
    Treatment of cirrhosis is aimed at stopping or delaying its progress, minimizing the damage to liver cells, and reducing complications.
    In alcoholic cirrhosis, for instance, the person must stop drinking alcohol to halt progression of the disease. If a person has hepatitis, the doctor may administer steroids or antiviral drugs to reduce liver cell injury.
    Medications may be recommended to control the symptoms of cirrhosis, such as itching. Edema and ascites (fluid retention) are treated by reducing salt in the diet. Medications called "diuretics" are sometimes used to remove excess fluid and to prevent edema from recurring.
    Diet and drug therapies can help to improve the altered mental function that cirrhosis can cause. For instance, decreasing dietary protein results in less toxin formation in the digestive tract. Laxatives such as lactulose may be given to help absorb toxins and speed their removal from the intestines.
    The two main problems in cirrhosis are liver failure, when liver cells just stop working, and the bleeding caused by portal hypertension. The doctor may prescribe blood pressure medication, such as a beta blocker, to treat the portal hypertension.
    If the individual bleeds from the varices of the stomach or esophagus, the doctor can inject these veins with a sclerosing agent administered through a flexible tube (endoscope) that is inserted through the mouth
and esophagus.
    In critical cases, a liver transplant or another surgery (such as a portacaval shunt) that is sometimes used to relieve the pressure in the portal vein and varices may be an option.
    Individuals with cirrhosis often live healthy lives for many years. Even when complications develop, they usually can be treated. A few individuals with cirrhosis have undergone successful liver transplantation.
    It's important to remember, however, that all tests, procedures and medications carry risks.  To make an informed decision about your health, be sure to ask your physician about the benefits, risks and costs of all procedures and medications.

CHILD-PUGH SCORING OF CIRRHOSIS

 Indications

Evaluating prognosis in Cirrhosis

Criteria

1. Total Serum Bilirubin

Bilirubin <2 mg/dl: 1 point
Bilirubin 2-3 mg/dl: 2 points
Bilirubin >3 mg/dl: 3 points

2. Serum Albumin Indications

Albumin >3.5 g/dl: 1 point
Albumin 2.8 to 3.5 g/dl: 2 point
Albumin <2.8 g/dl: 3 point

3. INR

INR <1.70: 1 point
INR 1.71 to 2.20: 2 point
INR >2.20: 3 point

4. Ascites

No Ascites: 1 point
Ascites controlled medically: 2 point
Ascites poorly controlled: 3 point

5. Encephalopathy

No Encephalopathy: 1 point
Encephalopathy controlled medically: 2 point
Encephalopathy poorly controlled: 3 point
 

Interpretation

Child Class A: 5 to 6 points
Life expectancy: 15 to 20 years
Abdominal surgery peri-operative mortality: 10%

Child Class B: 7 to 9 points
Indicated for liver transplantation evaluation
Abdominal surgery peri-operative mortality: 30%

Child Class C: 10 to 15 points
Life expectancy: 1 to 3 years
Abdominal surgery peri-operative mortality: 82%
 

References
 Mansour (1997) Surgery 122:730-5
 Propst (1995) Dig Dis Sci 40:1805-15
 Riley (2001) Am Fam Physician 64(10):1735-40

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