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Primary biliary cirrhosis, PBC, Hanot-Chauffard syndrome, or cholangiolitic biliary cirrhosis
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- The liver produces a substance called bile (needed for digestion of fats in the food), which flows through a series of channels called bile ducts.
- In PBC, the body's immune system (natural defense) attacks the bile ducts (for reasons currently not known) and damages them. When this happens, they leak bile into the surrounding liver tissue, causing damage and scarring. Over time, this ongoing scarring of the connective fibrous tissue of the liver will lead to a condition known as Cirrhosis (i.e., scarred, useless liver).
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- Itching (pruritus) of the skin
- Jaundice -- yellowish tinge to the skin and whites of the eyes
- Abdominal pain
- Fatty, greasy Diarrhea or loose stool
- With Cirrhosis:
- Weakness
- Weight loss
- Poor appetite
- Vomiting blood
- Bloating or feeling of excess gas in the abdomen
- Decreased urination
- Bleeding tendencies (e.g., nose bleeds, gum bleed, etc.)
- Abnormal menstruation
- Impotence (sexual problems)
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- Autoimmune -- immune system's white blood cells and Proteins known as antibodies attack the cells of the bile ducts. Over time, this leads to Cirrhosis and liver failure.
- Not all patients develop Cirrhosis
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- Symptoms
- Illnesses
- Medications
- Surgeries
- Habits
- Family history
- Allergies
- Medical exam may reveal signs and symptoms mentioned.
- Jaundice
- Edema -- swelling (due to
fluid) all over
- Spider veins -- tiny red veins
under the skin
- Fatty deposits under the skin
- Enlarged breast in males
(Gynecomastia)
- Yellow spots on the eyelids
- Enlarged liver
- Enlarged spleen
- Full blood panels -- liver and kidney functions, Electrolytes (Potassium, Sodium, etc.),
blood count (white and red cells) is done.
- Bleeding parameters (how easily
one bleeds) are done if there is bleeding.
- Blood is tested for an antibody
known as anti-mitochondrial antibody (present in 90% of
PCB cases.)
- Ultrasound (uses
sound) or CAT scan of the abdomen may be ordered to get
a better view of the structures inside (liver,
gallbladder, pancreas) to better assess the problem and
rule out other causes of liver failure (e.g., cancer,
stones, etc.)
- An ERCP (Endoscopic retrograde cholangiopancreatography) may be performed by the GI doctor (in the hospital and under sedation) by
inserting a flexible tube into
the stomach (through the mouth), advancing a catheter
(plastic tube) into the ducts, and injecting the ducts
with a dye that will highlight them when X- rayed. - ERCP will show any blockage or
narrowing of the biliary tree due to scarring.
- A biopsy is done in the hospital and requires a needle to be inserted into the liver to obtain a sample for study under a microscope (will confirm the diagnosis.)
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- 90% female
- Age -- most common in 40-60 year olds
- Infections
- Alcohol and drugs may add significantly to liver injury.
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- Symptomatic -- Cholestyramine for itching, antacids for Indigestion
- Vitamins K, A, and D supplements
(lost in fatty stool)
- Calcium supplement
- Cholchicine, Corticosteroids,
Azathioprine, and other medications may slow the disease by
suppressing the immune system attack on the ducts.
- Liver transplant from another
person who died may be needed if all else fails.
- With liver failure, avoid drugs and habits that can further damage the liver (i.e., alcohol use; needle sharing and unprotected sex can result in Hepatitis B or C).
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Contact
your doctor. Ask about the latest research and treatments. You can also contact the American Gastroenterological Association by calling 1-301-654-2055.
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