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Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is an inflammatory disease of the bile duct, which leads to cholestasis (blockage of bile transport to the gut). Bile is necessary for the absorption of dietary fat. more...

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Blockage of the bile duct leads to accumulation, damages the liver (leading to jaundice) and eventually causes liver failure. PSC is considered an autoimmune disease.

Signs and symptoms

  • Severe jaundice with itching
  • Malabsorption (especially of fat) and steatorrhea, leading to decreased levels of the fat-soluble vitamins, A, D and K.
  • Signs of cirrhosis
  • Infection of the bile duct (as ascending bacteria are not cleared)

Diagnosis

The diagnosis is by imaging of the bile duct, usually in the setting of endoscopic retrograde cholangiopancreatography (ERCP, endoscopy of the bile duct and pancreas). Another option is magnetic resonance cholangiopacreaticography (MRCP), where magnetic resonance imaging is used to visualise the biliary tract.

Other tests often done are a full blood count, liver enzymes, bilirubin levels (usually grossly elevated), renal function, electrolytes. Fecal fat determination is occasionally ordered when the symptoms of malabsorption are prominent.

Differential diagnostics: Primary biliary cirrhosis.

Pathophysiology

The cause(s) for PSC are unknown, but it is considered an autoimmune disorder.

Bile ducts, both intra- and extrahepatically (inside the liver and outside), are inflamed and develop scarring, obstructing the flow of bile. As bile assists in the enteric breakdown and absorption of fat, the absence of bile leads to fat malabsorption. The bile accumulates in the duct, leading to liver cell damage and liver failure.

PSC is associated with ulcerative colitis. It is assumed that these diseases share a common cause.

Epidemiology

It happens more in men than in women. The disease normally starts from age 30 to 60. It can however also start with children. PSC progresses slowly, so the disease can be active for a long time before it is noticed or diagnosed.

Therapy

Standard treatment includes ursodiol, a bile acid naturally produced by the liver, which has been shown to lower elevated liver enzyme numbers in people with PSC, but has not yet been proven effective at prolonging the life of the liver. Treatment also includes medication to relieve itching (antipruritics), antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamin A, vitamin D, and vitamin K.

In some cases, surgery to open major blockages in the common bile duct is also necessary. Liver transplantation (including live transplants whereby a portion of a living donor is given to the recipient) is an option if the liver begins to fail.

Read more at Wikipedia.org


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Expert Advice on Health and Fitness - primary sclerosing cholangitis; chronic fatigue syndrome; bacterial vaginosis - Brief Article
From Ebony, 1/1/00

"How rare is `Walter Payton's disease' and would a new liver have saved his life?"

`WALTER PAYTON'S DISEASE'

Q With the recent death of football great Walter Payton, I heard for the first time about the rare disease that took his life. I can't pronounce it and simply call it "Walter Payton's disease." How rare is it, and would a new liver have been enough to save his life? T.R., Kansas City, Mo.

A Because it is a mouthful to say, primary sclerosing cholangitis might very well become commonly known as "Walter Payton's disease," the same way that amyotrophic lateral sclerosis (ALS) is primarily referred to as "Lou Gehrig's disease."

Doctors say primary sclerosing cholangitis is a rare liver disease that causes a narrowing of the bile ducts inside and outside the liver. Its cause is unknown, and when the inflammation spreads, the bile ducts are destroyed and scar tissue is created. In some cases, the tissue can become cancerous. Doctors say that's what happened in Payton's case, and he died as a result of bile-duct cancer.

Once the cancer was discovered, Payton was no longer considered a transplant candidate. At that stage, a new liver would not have saved his life, doctors say, because the cancer probably had spread beyond the liver or because the cancer likely would have recurred in a transplanted liver.

Primary sclerosing cholangitis, a disease that strikes only about three people out of every 100,000, usually affects males between the ages of 40 and 50, doctors say. The disease can go unnoticed for years because it causes vague symptoms that include fatigue and itching, and is not necessarily associated with a history of liver disease.

IDENTIFYING CHRONIC FATIGUE SYNDROME

Q I am a 33-year-old, single female who works a 40-hour week but have few outside activities. My problem is that I am tired all the time, no matter how much sleep I get. When I wake up in the morning, I'm tired. When I go to work, I'm tired. When I go to bed at night, I'm tired. Could I be a victim of what they call chronic fatigue syndrome? L.W., Baltimore

Since fatigue is a symptom of a variety of medical conditions (such as liver, thyroid or kidney problems), you should contact your physician as soon as possible to schedule a thorough physical examination, which can lead to the identification of the cause of your constant exhaustion.

A It's possible that you are a victim of chronic fatigue syndrome (sometimes called "yuppie flu"), a condition that can strike anyone at any age, doctors say, but it's three times more common in women for reasons that are unknown. In addition to feelings of being constantly worn out, many victims complain of flu-like symptoms and often have low blood pressure, difficulty with concentration, poor short-term memory, irritability, a recurring sore throat, a low-grade fever and swollen lymph nodes.

The exact cause of chronic fatigue syndrome is unknown, but some researchers suspect it may be caused by a virus, however, no distinct viral cause has been identified. Doctors say there is currently no treatment that has been proven to be effective in curing the illness, instead the symptoms are treated. In addition to some medication, patients with chronic fatigue syndrome are encouraged to maintain active social lives, and mild physical exercise may also be helpful.

BACTERIAL VAGINOSIS

Q My girlfriend, who has frequent yeast infections, went to her doctor to be treated for what she thought was a yeast infection. However, she was told that she had a condition called bacterial vaginosis. What is it? How common is it? And how serious is it? K.G., Denver

A Bacterial vaginosis is the most common kind of vaginal infection, physicians say, and most women will get it at some point in their childbearing years. It is caused by the rapid growth of certain types of bacteria that upset the balance of "good" and "bad" types of bacteria in the vagina. In most cases, it's not dangerous but could cause problems for women who are pregnant or those having gynecological surgery. Symptoms include a strong fishy smell, a white or gray discharge, or a watery or foamy discharge.

COPYRIGHT 2000 Johnson Publishing Co.
COPYRIGHT 2000 Gale Group

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