Encyclopedia of Health and Medicine

84 The Gastrointestinal System
hypertension requires surgical intervention.
Shunts can help redirect the flow of blood into the
liver and lower portal vein pressure. Sometimes
removing the SPLEEN (SPLENECTOMY) and blood vessels
surrounding the esophagus is necessary to
control esophageal varices. The only curative
treatment is LIVER TRANSPLANTATION, which, because
donor organs are so limited, is a treatment of final
resort when other therapies fail and LIVER FAILURE
becomes life-threatening.
Medications and intermediary surgical procedures
such as shunts can successfully manage portal
hypertension in many people, allowing good
QUALITY OF LIFE.
Risk Factors and Preventive Measures
Chronic liver disease and heart disease are the primary
risk factors for portal hypertension. Lifestyle
measures to minimize these conditions, and
appropriate treatments to manage them when
they do occur, significantly reduce the likelihood
that portal hypertension will develop.
See also CARDIOVASCULAR DISEASE PREVENTION;
HEMOCHROMATOSIS; HEPATITIS PREVENTION; LIFESTYLE
AND HEALTH; WILSON’S DISEASE.
primary biliary cirrhosis An autoimmune disorder
in which chronic and progressive INFLAMMA-
TION destroys the intrahepatic BILE DUCTS (bile ducts
within the LIVER), blocking the flow of BILE. Primary
biliary CIRRHOSIS appears to run in families,
suggesting a hereditary component. Early symptoms
include fatigue, tenderness or PAIN in the
upper right abdomen, and itching (PRURITIS). Over
time, signs of liver damage, such as JAUNDICE and
HEPATOMEGALY (enlarged liver), emerge. Primary
biliary cirrhosis is most common in women
between the ages of 40 and 60.
The diagnostic path includes
• LIVER FUNCTION TESTS, which typically show elevations
of the enzymes alanine aminotransferase
(ALT), aspartate aminotransferase (AST),
alkaline phosphatase (ALP), and gamma-glutamyl
transpeptidase (GGTP).
• BLOOD tests to measure the level of immunoglobin
(elevated) and detect the presence of
antimitochondrial antibodies (positive)
• imaging procedures such as abdominal ULTRA-
SOUND, COMPUTED TOMOGRAPHY (CT) SCAN, or MAG-
NETIC RESONANCE IMAGING (MRI)
• PERCUTANEOUS LIVER BIOPSY
The progressive destruction of the bile ducts
results in CHOLESTASIS and cirrhosis, leading ultimately
to LIVER FAILURE. Complications include
OSTEOPOROSIS (arising from the body’s inability to
metabolize vitamin D and calcium), PORTAL HYPER-
TENSION, ESOPHAGEAL VARICES, and primary LIVER
CANCER.
There are currently few medical treatment
options. The medication ursodiol (Actigall), sometimes
taken to help dissolve gallstones, slows the
progression of the inflammatory process in some
people. ANTIHISTAMINE MEDICATIONS can help relieve
the itching in the early stages; in later stages some
people experience relief from itching with bile
sequestrant medications such as cholestyramine
and colestipol, which bind with bile in the gastrointestinal
tract. The only curative treatment,
however, is LIVER TRANSPLANTATION.
See also AUTOIMMUNE DISORDERS; BILIARY ATRESIA;
CANCER RISK FACTORS; GALLBLADDER DISEASE; PRIMARY
SCLEROSING CHOLANGITIS.
primary sclerosing cholangitis A progressive
and chronic condition in which segments of the
BILE DUCTS become inflamed, causing SCAR tissue
(sclerosis) that narrows and stiffens them. The
scarring reduces and eventually destroys the ability
of the ducts to carry BILE. About 75 percent of
people who have primary sclerosing cholangitis
also have INFLAMMATORY BOWEL DISEASE (IBD), suggesting
a related autoimmune process. Primary
sclerosing cholangitis is most common in men
between the ages of 20 and 40.
In the early stages of the disease symptoms are
mild and tend to wax and wane. Early symptoms
may include fatigue, tenderness or PAIN in the
upper right abdomen, and mild JAUNDICE (yellowish
discoloration of the skin). Often the discovery
of primary sclerosing cholangitis comes with elevated
enzyme levels on LIVER FUNCTION TESTS done
for other reasons, with confirmation by imaging
procedures, such as ULTRASOUND or COMPUTED
TOMOGRAPHY (CT) SCAN, and PERCUTANEOUS LIVER