Cirrhosis of the Liver
The liver weighs about three pounds and is the largest organ in the body.
It is located in the upper right side of the abdomen, below the ribs. When
chronic 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.
What is the Impact of Cirrhosis?
Cirrhosis is the eleventh leading cause of death by disease in the United
States. Almost one-half of these are alcohol related. 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. It can result from direct injury to the liver
cells (i.e., hepatitis) or from indirect injury via inflammation or obstruction
to bile ducts which drain the liver cells of bile. Common causes of direct
liver injury include chronic alcoholism (most common cause in the United
States), chronic viral hepatitis (types B, C, and D) and auto immune hepatitis.
Common causes of indirect injury by way of bile duct damage include primary
biliary cirrhosis, primary sclerosing cholangitis and biliary atresia (common
cause of cirrhosis in infants).
Less common causes of cirrhosis include direct liver
injury from inherited disease such as cystic fibrosis, alpha-1-antitrypsin
deficiency, hemochromatosis,
Wilsons disease, galactosemia, and glycogen storage disease.
Two inherited disorders result in the abnormal storage
of metals in the liver leading to tissue damage and cirrhosis. People
with Wilsons
disease store too much copper in their liver, brain, 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 persons 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 of 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.
Very rare causes of cirrhosis include: reactions to drugs (e.g., vitamin
A, methotrexate, amiodarone) exposure to environmental toxins, and repeated
bouts of heart failure with liver congestion.
f after full evaluation of a patient with cirrhosis,
the etiology still is not clear, the disease is called "cystogenic cirrhosis." As
much as 10 percent of cirrhosis falls into this category.
What are the Symptoms 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. Some patients present with menstrual abnormalities
(amenorrhea), impotence, loss of sexual drive or painfully enlarged breasts
(in men).
As liver function declines, less protein is made by the organ. For example,
less of the protein albumin is made, which results in water 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.
In the later stages of cirrhosis, jaundice (yellow skin) may occur, caused
by the buildup of bile pigment that is normally 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 is also 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 are often 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 patientŐs life
is in danger, and the doctor must act quickly to stop the bleeding.
How is Cirrhosis Diagnosed?
The doctor often can diagnose cirrhosis from the patients
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, you will be given 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 are performed such as the computerized
axial tomography (CAT) scan, ultrasound, and the radioisotope liver/spleen
scan.
The doctor may decide to confirm the diagnosis 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 Treatments for Cirrhosis?
Treatment of cirrhosis is aimed to stop the development of scar tissue
in the liver and prevent complications. When cirrhosis is due to an identifiable
cause, treatment programs may be specific, such as for management of hepatitis
B and C, or steroids and immunosuppressive agents for auto-immune chronic
active hepatitis.
No matter what the cause of cirrhosis, every patient must avoid all substances,
habits, and drugs that may further damage the liver, precipitate complications,
or speed the progression to liver failure. Alcohol, in addition to causing
cirrhosis, may accelerate the progression of liver scarring due to other
causes, such as hepatitis C. All patients with liver disease should not
drink alcoholic beverages. Even some non-prescription drugs and vitamins,
acetaminophen, in relatively small doses (more than five doses a day) and
Vitamin A (more than 25,000 IU/day) may precipitate liver failure. Non-steroidal
anti-inflammatory drugs, such as ibuprofen, may precipitate severe bleeding
and/or kidney failure.
The cirrhotic patient is at increased risk of contracting other infections
that may be more severe than in healthy patients. Immunizations for hepatitis
A, B, influenza, and pneumococcal pneumonia are available and should be
administered. Raw seafood may contain bacteria that can cause life-threatening
infections and therefore should be avoided.
How are the Complications of Cirrhosis
Treated?
The abnormal accumulation of fluid may cause swelling
of the ankles (edema) and abdomen (ascites). Therefore, patients should
reduce the amount of fluid and salt in their diet or use drugs called "diuretics" that
mobilize and excrete the excess fluid through the kidneys. Occasionally,
the ascites may become infected, a condition known as Spontaneous Bacterial
Peritonitis, and require treatment with antibiotics.
When the liver does not efficiently function to cleanse the body of toxins
and drugs, the mental state of patients may change dramatically and lead
to coma, called Hepatic Encephalopathy. Treatment is directed at reducing
the protein in the diet, avoiding sedatives and pain medications, and using
laxatives and/or antibiotics to decrease the absorption of toxins from
the intestines.
Sometimes, bleeding from the esophagus or stomach caused by abnormal veins
(varices) may occur and is a life-threatening emergency requiring hospitalization.
Variceal bleeding can usually be controlled with the use of a flexible
tube (endoscope) that is inserted through the mouth into the esophagus
and stomach and used to inject clotting agents into the veins or to rubberband
ligate the varices.
Liver failure refers to the end stage of liver disease and cirrhosis when
the liver stops working and cannot support life. Liver failure is difficult
to treat and survival is limited. Therefore, patients with any complication
of cirrhosis are considered to be at risk of developing liver failure.
When complications develop, it may be possible to manage them. When it
is likely that liver failure will develop, some patients with cirrhosis
are able to undergo liver transplantation. The treating gastroenterologist
may recommend liver transplantation when complications of cirrhosis develop
in an attempt to avoid liver failure.
Additional Reading:
Biliary Atresia.. This
fact sheet presents information on biliary atresia and cirrhosis, including
discussions of diagnosis, treatment, and complications. Available from
the American Liver Foundation. 1428 Pompton Avenue, Cedar Grove, NJ 07009.
(201) 256-2550 or (800) 223-0179.
Cirrhosis: Many Causes.. This
fact sheet presents general information on cirrhosis of the liver, research,
and the work of the American Liver Foundation. Available from the foundation.
Clayman, CB, ed. The American Medical Association Encyclopedia
of Medicine.. New York:
Random House, 1989. Authoritative reference guide for patients, with
sections on cirrhosis, hepatitis, and other disorders affecting the
liver. Widely available in libraries and bookstores.
Primary Biliary Cirrhosis.
. This
fact sheet presents information on PBC and cirrhosis, including discussions
of diagnosis, treatment, and liver transplantation.
Available from the American Liver Foundation.
Rosenfeld, I. Second Opinion: Your Comprehensive Guide to Treatment. New
York: Bantam Books, 1988. General medical guide with sections on cirrhosis
and other disorders affecting the liver. Widely available in libraries
and bookstores.
Resources
United Network for Organ Sharing, 1100 Boulders Parkway, Suite 500, P.O.
Box 13770, Richmond, VA 23225-8770. (804) 330-8500.
return
to brochures
|