primarybiliarycirrhosis

What is primary biliary cirrhosis?


The exact cause of primary biliary cirrhosis (PBC) is unknown. Most experts believe it is an autoimmune condition. In autoimmune conditions, something goes wrong with the immune system, causing it to attack healthy tissue.

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primary biliary cirrhosis information from trusted sources:

Primary biliary cirrhosis

Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile. This obstruction damages liver cells.

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Primary biliary cirrhosis

Primary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly destroyed. Bile, a fluid produced in your liver, is essential for the proper digestion of fats. It also helps rid your body of worn-out red blood cells, cholesterol and toxins. In primary biliary cirrhosis, the destruction of your bile ducts can cause harmful substances to build up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).

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Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a type of chronic liver disease that is poorly understood. It can cause:

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Cirrhosis

Cirrhosis (si-RO-sis) is a chronic (long term) condition where normal, healthy liver cells are damaged. The liver cells harden and die causing the liver to shrink and scar, not work well. Cirrhosis often starts slowly and you may not even know that you have the condition. You may end up with the liver totally failing if cirrhosis is not diagnosed and treated early. This may lead to coma and even death.

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Questran

QUESTRAN (Cholestyramine for Oral Suspension USP), the chloride salt of a basic anion exchange resin, a cholesterol lowering agent, is intended for oral administration. Cholestyramine resin is quite hydrophilic, but insoluble in water. The cholestyramine resin in QUESTRAN is not absorbed from the digestive tract. Four grams of anhydrous cholestyramine resin is contained in 9 grams of QUESTRAN powder. Four grams of anhydrous cholestyramine resin is contained in 5 grams of QUESTRAN LIGHT. It is represented by the following structural formula:Cholesterol is probably the sole precursor of bile acids. During normal digestion, bile acids are secreted into the intestines. A major portion of the bile acids is absorbed from the intestinal tract and returned to the liver via the enterohepatic circulation. Only very small amounts of bile acids are found in normal serum. 1) QUESTRAN (Cholestyramine for Oral Suspension USP), is indicated as adjunctive therapy to diet for the reduction of elevated serum cholesterol in patients with primary hypercholesterolemia (elevated low density lipoprotein [LDL] cholesterol) who do not respond adequately to diet. QUESTRAN may be useful to lower LDL cholesterol in patients who also have hypertriglyceridemia, but it is not indicated where hypertriglyceridemia is the abnormality of most concern.

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Primary Biliary Cirrhosis

Explains the possible causes of primary biliary cirrhosis. Describes symptoms, complications, diagnosis, and treatment.

Primary biliary cirrhosis - Wikipedia, the free encyclopedia

Primary biliary cirrhosis, often abbreviated PBC, is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts ...

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Primary Biliary Cirrhosis: eMedicine Gastroenterology

Dec 23, 2009 ... Overview: Primary biliary cirrhosis (PBC) is a chronic and progressive cholestatic disease of the liver. The etiology is unknown, ...

Read more on emedicine.medscape.com

Primary Biliary Cirrhosis – Complete medical information regarding ...

Mar 19, 2011 ... Primary biliary cirrhosis (PBC) is a chronic (long duration) disease characterized by progressive inflammation and destruction of the small ...

Read more on www.medicinenet.com

Primary Biliary Cirrhosis — Diagnosis and Treatment Options at ...

Primary Biliary Cirrhosis Learn about diagnosis and treatment options at Mayo Clinic.

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Contents

Complications
As liver damage progresses, people with primary biliary cirrhosis may develop a number of serious problems, including: Cirrhosis. The term "primary biliary cirrhosis" isn't entirely accurate because cirrhosis develops only in the later stages of the disease often many years after diagnosis. Yet when it does occur, cirrhosis can be life-threatening because it interferes with your liver's ability to carry out essential functions. Cases of primary biliary cirrhosis are divided into four stages. The first stage inflammation of the bile ducts is the least serious, and stage 4 cirrhosis the most serious. Ongoing cirrhosis can lead to liver failure, which occurs when your liver is no longer able...

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Coping and support
Although the prognosis for people with primary biliary cirrhosis is far better than it was in the past, it can still be difficult and frustrating to live with a chronic liver disease. When symptoms of the disease develop, such as fatigue and itching, they can seriously affect your quality of life.

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Diagnosis
In many cases, a suspected diagnosis of primary biliary cirrhosis (PBC) is made when carrying out a blood test for another, unrelated condition.

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Exams and Tests
Tests for the disease:Elevated immunoglobulin M level in the blood; Liver biopsy ; Mitochondrial antibodies (positive in about 95% of cases); Liver function tests ; Serum cholesterol and lipoproteins

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Lifestyle and home remedies
Taking good care of your overall health may help you feel better and improve some of the symptoms of primary biliary cirrhosis: Eat a reduced-sodium diet, because sodium increases your risk of tissue swelling and of accumulating fluid in your abdominal cavity (ascites).; Exercise and take calcium and vitamin D supplements to help reduce your risk of developing osteoporosis.; Avoid alcohol consumption, because it speeds the progression of liver disease.; Practice good skin care to reduce itching. Avoid scratching. Use cool, wet compresses. Try cool baking soda or oatmeal baths. Avoid irritating clothes, soaps and detergents.; Use artificial tears to help reduce dry eyes.; If you're a smoker,...

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Medical advice
Call your health care provider if you have:Abdominal swelling; Blood in the stools ; Confusion; Jaundice; Itching of the skin that does not go away and is not related to other causes; Vomiting blood

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Outlook (Prognosis)
The outcome can vary. If the condition is not treated, most patients will need a liver transplant after about 7 years. Doctors can now use statistics to predict the best time to do the transplant.

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Possible Complications
Progressive cirrhosis can lead to liver failure. Complications can include:Bleeding; Damage to the brain (encephalopathy); Fluid and electrolyte imbalance; Kidney failure; Malabsorption ; Malnutrition ; Soft bones (osteodystrophy)

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Preparing for your appointment
You're likely to start by first seeing your family doctor or a general practitioner. However, you may then be referred to a doctor who specializes in disorders of the digestive system (gastroenterologist).

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Risk factors
The following factors may increase your risk of primary biliary cirrhosis: Your sex. More than 90 percent of people with primary biliary cirrhosis are women.; Your age. Most people diagnosed with primary biliary cirrhosis are 35 to 60 years old. Although older adults can develop the disease, it's rare in children.; Family history. Having a family history of primary biliary cirrhosis increases your risk of developing the disease.

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Symptoms
Early stage Although some people with primary biliary cirrhosis remain symptom-free for years after they're diagnosed, others experience a number of symptoms early in the disease: Fatigue. A common symptom of primary biliary cirrhosis is fatigue, but doctors haven't found any correlation between the degree of exhaustion and the severity of the illness. This means that people with mild primary biliary cirrhosis and those with more serious disease may be equally fatigued.; Itching. Another common symptom, itching (pruritus), is often most bothersome over your legs, arms and back. The severity of itching may change, often becoming worse at night and improving during the day. Nighttime itching...

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Treatment
There is currently no cure for primary biliary cirrhosis (PBC), so the aim of treatment is...

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