Primary Biliary Cirrhosis (cont.)
In this Article

Hepatorenal syndrome
Patients with advanced liver disease and portal hypertension can sometimes
develop the hepatorenal syndrome. This syndrome is a serious problem with the
functioning of the kidneys without actual physical damage to the kidneys
themselves. The hepatorenal syndrome is defined by progressive failure of the
kidneys to clear substances from the blood and produce adequate volumes of urine
even though some other kidney functions, such as retention of salt, are
maintained. If liver function improves or a healthy liver is transplanted into a
patient with hepatorenal syndrome, the kidneys often begin to work normally.
This restoration of kidney function indicates that liver failure is associated
with an inability of the liver to either produce or detoxify substances that
affect kidney function.
Hepatopulmonary syndrome
Rarely, some patients with advanced cirrhosis can develop the hepatopulmonary
syndrome. These patients can experience difficulty with breathing because
certain hormones released in advanced cirrhosis cause abnormal functioning of
the lungs. The basic lung problem in the hepatopulmonary syndrome is that the
blood flowing through the small vessels in the lungs does not come in sufficient
contact with the alveoli (air pockets) of the lungs. Therefore, the blood cannot
pick up enough oxygen from the air that is breathed and the patient experiences
difficulty breathing.
Liver cancer (hepatocellular carcinoma)
Patients with PBC that develop cirrhosis have an increased risk of developing
a primary cancer of the liver cells (hepatocytes) called liver cancer
(hepatocellular carcinoma). Primary refers to the fact that the tumor originates
in the liver. A secondary tumor originates elsewhere in the body and can spread
(metastasize) to the liver.
Cirrhosis due to any cause increases the risk of liver cancer. Therefore, the
development of a primary liver cancer in an individual with PBC is not
unexpected. However, the risk of hepatocellular carcinoma in PBC appears to be
lower than the risk in cirrhosis caused by some other liver diseases, such as
chronic viral hepatitis. A recent report indicated that hepatocellular carcinoma
might be more common in men than women with PBC. Indeed, this one series of 273
patients with advanced PBC found hepatocellular carcinoma in 20% of the men
compared to only 4.1% of the women. The way hepatocellular cancer develops in
PBC, however, is not understood.
The most common symptoms and signs of primary liver cancer are abdominal pain
and swelling, an enlarged liver, weight loss, and fever. In addition, these
liver tumors can produce and release a number of substances, including ones that
cause an increase in red blood cells (erythrocytosis), low blood sugar
(hypoglycemia), and high blood calcium (hypercalcemia).
The most useful diagnostic tests for hepatocellular carcinoma are a blood
test called an alpha-fetoprotein and an imaging study of the liver (either a CT
Scan or an MRI with intravenous dye/contrast). The best screening tests for
early detection of hepatocellular carcinoma in patients with cirrhosis are
serial alpha-fetoprotein levels and ultrasound examinations of the liver every 6
to 12 months. It is important to note that about 40% of hepatocellular cancers
do not produce elevated levels of alpha-fetoprotein.
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