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 ves-
sels 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 proce-
dures such as shunts can successfully manage por-
tal hypertension in many people, allowing good
QUALITY OF LIFE.
Risk Factors and Preventive MeasuresChronic liver disease and heart disease are the pri-
mary 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 disor-
der in which chronic and progressive INFLAMMA-
TIONdestroys the intrahepatic BILE DUCTS(bile ducts
within the LIVER), blocking the flow of BILE. Pri-
mary biliary CIRRHOSISappears to run in families,
suggesting a hereditary component. Early symp-
toms include fatigue, tenderness or PAIN in the
upper right abdomen, and itching (PRURITIS). Over
time, signs of liver damage, such as JAUNDICEand
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 ele-
vations of the enzymes alanine aminotrans-
ferase (ALT), aspartate aminotransferase (AST),
alkaline phosphatase (ALP), and gamma-glu-
tamyl transpeptidase (GGTP). - BLOODtests to measure the level of immunoglo-
bin (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
- imaging procedures such as abdominal ULTRA-
The progressive destruction of the bile ducts
results in CHOLESTASISand cirrhosis, leading ulti-
mately 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), some-
times taken to help dissolve gallstones, slows the
progression of the inflammatory process in some
people. ANTIHISTAMINE MEDICATIONScan 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 gas-
trointestinal tract. The only curative treatment,
however, is LIVER TRANSPLANTATION.
See alsoAUTOIMMUNE 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 DUCTSbecome inflamed, causing SCARtissue
(sclerosis) that narrows and stiffens them. The
scarring reduces and eventually destroys the abil-
ity of the ducts to carry BILE. About 75 percent of
people who have primary sclerosing cholangitis
also have INFLAMMATORY BOWEL DISEASE(IBD), sug-
gesting 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(yellow-
ish discoloration of the skin). Often the discovery
of primary sclerosing cholangitis comes with ele-
vated enzyme levels on LIVER FUNCTION TESTSdone
for other reasons, with confirmation by imaging
procedures, such as ULTRASOUND or COMPUTED
TOMOGRAPHY (CT) SCAN, and PERCUTANEOUS LIVER84 The Gastrointestinal System