Disorders of the Biliary Tract
Although disorders of the biliary tract don't garner as much attention as other conditions, they are common. Today's endoscopy team faces more and more of these disorders every day.
In terms of biliary endoscopy, we've gone from an era a decade ago where endoscopic approaches to the biliary tree were primarily diagnostic - Is there or is there not a stone? Is there or is there not a stricture? - to an era in which the radiology is so good that we can do all of that with either CT (computed tomography) scanning or MRI (magnetic resonance imaging), Over 90 percent of these procedures are now therapeutic, with an overall excellent efficacy and very low risk to the patient with real advances in terms of morbidity and mortality.
Primary Sclerosing Cholangitis
Although primary sclerosing cholangitis (PSC) is primarily a disease of the bile ducts of the liver, ducts of the gallbladder and pancreas may also be involved.
Inflamed bile ducts become scarred and hardened, eventually causing liver failure. The disease may be present for several years before symptoms develop, such as fatigue, loss of appetite, weight loss and jaundice.PSC is diagnosed through cholangiography, which involves injecting dye into the bile ducts and taking an X-ray.
About 70 percent of patients who have PSC usually have underlying inflammatory bowel disease, mainly ulcerative colitis. PSC is more common among men than women. It usually begins between ages 30 and 60, but the disease can also arise during childhood.Due to the difference in clinical presentation of PSC in children, a high degree of suspicion is often required to make the diagnosis of childhood PSC. Most children present with non-specific symptoms, including fatigue and weight loss and, less commonly, itching.
When you think of the cholestatic diseases in children or adults, technicians and people that may see them for endoscopic reasons should be aware that itch is a very important system that may be related to the disease.
For PSC, there isn't a lot of effective treatment, You can try endoscopic therapy, which could be attempted to make the patient feel better. Unfortunately, it doesn't change the natural history of the disease. Most of the patients will undergo cirrhosis and permanent liver damage.
Primary Biliary Cirrhosis
Primary biliary cirrhosis (PBC), a chronic liver disease, is more prevalent than was previously suspected. The disease affects females more often than males, with onset between the ages of 30 and 60 years. PBC, up to the time of a patient developing actual cirrhosis, is a misnomer. It is only the fourth and final stage of the disease that is truly a biliary cirrhosis.
PBC is characterized by progressive destruction of the bile ducts in the liver, with inflammation leading to scarring and cirrhosis. Symptoms can include itching, jaundice, cholesterol deposits on the skin, fluid retention and dry eyes or mouth. Asymptomatic patients usually survive more than 10 years, while patients with symptomatic PBC and jaundice survive approximately seven years.
Diagnosis is based on physical examination, biochemical tests and liver biopsy. The new advances in this disease relate to a recognition of its primary auto antibody marker, which is referred to as antimitochondrial antibody, or AMA. AMAs are found in 96 percent of patients with PBC.The presence of AMA is almost diagnostic of the disorder.
Identification of PBC is important because effective treatment with ursodeoxycholic acid has been shown to halt disease progression and improve survival without need for liver transplantation.
A discussion about diseases of the biliary tract would not be complete without mentioning gallstones. Approximately 16 to 22 million people have or have had gallstones in the United States. The management of gallstone disease accounts for 1 percent of annual healthcare expenditures in the United States, approximately $6 billion.
There are two main types of stones. Cholesterol stones arise exclusively in the gallbladder and consist of 50 percent to 100 percent cholesterol.Pigment stones may arise anywhere in the biliary tree and are classified as black or brown. Symptoms often include fever, jaundice and persistent pain. Ultrasonography provides a simple, quick method of diagnosis. It has a 95-percent sensitivity and specificity for stones more than 4 millimeter in diameter.
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