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Encyclopedia > Primary Biliary Cirrhosis
Primary biliary cirrhosis
Classifications and external resources
ICD-10 K74.3
ICD-9 571.6

Primary biliary cirrhosis is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts (bile canaliculi) within the liver. When these ducts are damaged bile builds up in the liver (cholestasis) and over time damages the tissue. This can lead to scarring, fibrosis, cirrhosis, and ultimately liver failure. It is a rare disease, about 200 out of a million; 10 to 1 women to men, although different references vary widely on these numbers. The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... The liver is an organ in some animals, including mammals (and therefore humans), birds, and reptiles. ... Bile canaliculus (plural:Bile canaliculi) is a thin tube that collects bile secreted by hepatocytes. ... Bile (or gall) is a bitter, greenish-yellow alkaline fluid secreted by hepatocytes from the liver of most vertebrates. ... In medicine, cholestasis is a condition where bile cannot flow from the liver to the duodenum. ... Fibrosis is the formation or development of excess fibrous connective tissue in an organ or tissue as a reparative or reactive process, as opposed to formation of fibrous tissue as a normal constituent of an organ or tissue. ... Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrotic scar tissue as well as regenerative nodules, leading to progressive loss of liver function. ... Liver failure is the final stage of liver disease. ...

Contents

Signs and symptoms

The following signs may be present in PBC:

The word fatigue is used in everyday living to describe a range of afflictions, varying from a general state of lethargy to a specific work induced burning sensation within muscle. ... An itch (Latin: pruritus) is a sensation felt on an area of skin that makes a person or animal want to scratch it. ... Jaundice, also known as icterus (attributive adjective: icteric), is a yellowing of the skin, conjuctiva (clear covering over the sclera, or whites of the eyes) and mucous membranes caused by increased levels of bilirubin in the human body (or the body of another red blooded animal). ... Bilirubin is a yellow breakdown product of heme catabolism. ... Xanthelasma (or xanthelasma palpebrarum) are sharply demarcated yellowish collections of cholesterol underneath the skin, usually around the eyes. ... Cholesterol is a sterol (a combination steroid and alcohol) and a lipid found in the cell membranes of all body tissues, and transported in the blood plasma of all animals. ... Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrotic scar tissue as well as regenerative nodules, leading to progressive loss of liver function. ... In medicine, portal hypertension is hypertension (high blood pressure) in the portal vein and its branches. ... In medicine (gastroenterology), esophageal varices are extreme dilations of sub mucosal veins in the mucosa of the esophagus in diseases featuring portal hypertension, secondary to cirrhosis primarily. ... Hepatic encephalopathy is a condition (usually caused by liver cirrhosis and its resultant portal hypertension) where brain cells are damaged by a build-up of toxic substances in the blood. ...

Diagnosis

To diagnose PBC, distinctions should be established from other conditions with similar symptoms, such as autoimmune hepatitis or primary sclerosing cholangitis (PSC). In medicine (gastroenterology), hepatitis is any disease featuring inflammation of the liver. ... Primary sclerosing cholangitis (PSC) is an inflammatory disease of the bile duct, which leads to cholestasis (blockage of bile transport to the gut). ...


Diagnostic blood tests include: Blood tests are laboratory tests done on blood to gain an appreciation of disease states and the function of organs. ...

Abdominal ultrasound or a CT scan is usually performed to rule out blockage to the bile ducts. Previously most suspected sufferers underwent a liver biopsy, and - if uncertainty remained - endoscopic retrograde cholangiopancreatography (ERCP, an endoscopic investigation of the bile duct). Now most patients are diagnosed without invasive investigation since the combination of anti-mitochondrial antibodies (see below) and typical (cholestatic) liver function tests are considered diagnostic. However, a liver biopsy is necessary to determine the stage of disease. Liver function tests (LFTs or LFs), are groups of clinical biochemistry laboratory blood assays designed to give a doctor or other health professional information about the state of a patients liver. ... Alkaline phosphatase, drawn from PDB 1ANI. Alkaline phosphatase (ALP) (EC 3. ... Wikipedia does not yet have an article with this exact name. ... Antinuclear antibodies (ANAs, also known as antinuclear factor or ANF) are detected in a large group of autoimmune disorders. ... Schematic of antibody binding to an antigen An antibody is a protein complex used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ... Medical ultrasonography (sonography) is an ultrasound-based diagnostic imaging technique used to visualize muscles and internal organs, their size, structure and any pathological lesions, making them useful for scanning the organs. ... CAT apparatus in a hospital Computed axial tomography (CAT), computer-assisted tomography, computed tomography, CT, or body section roentgenography is the process of using digital processing to generate a three-dimensional image of the internals of an object from a large series of two-dimensional X-ray images taken around... A biopsy (in Greek: bios = life and opsy = look/appearance) is a medical test involving the removal of cells or tissues for examination. ... Endoscopic retrograde cholangiopancreatography (ERCP) is endoscopy of the biliary tree and the pancreatic duct. ... Endoscopic images of a duodenal ulcer Endoscopy means looking inside and refers to looking inside the human body for medical reasons. ... X-Ray of the bile duct during a laprascopic cholecystectomy A bile duct is any of a number of long tube-like structures that carry bile. ...


Etiology

The cause of the disease is unknown at this time, but research indicates that there is an immunological basis for the disease, making it an autoimmune disorder. Most of the patients (>90%) seem to have auto-mitochondrial antibodies (AMAs) against pyruvate dehydrogenase complex (PDC-E2), an enzyme complex that is found in the mitochondria. In addition, a more specific test to confirm this disease from a bone disorder such as Paget's (disease of bone) which also has increases in Alkaline phosphatase is the Gamma-glutamyl trans peptidase test (GGTP). An increase in GGTP could indicate presence of Primary Biliary Cirrhosis. Immunology is a broad branch of biomedical science that covers study of all aspects of the immune system in all organisms. ... A disease or medical condition is an abnormality of the body or mind that causes discomfort, dysfunction, distress, or death to the person afflicted or those in contact with the person. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Pyruvate dehydrogenase complex (PDC) is a complex of three enzymes that transform pyruvate into acetyl-CoA by a process called pyruvate decarboxylation. ... In cell biology, a mitochondrion is an organelle found in the cells of most eukaryotes. ... Gamma glutamyl transferase (GGT or GGTP, or Gamma-GT) (EC 2. ...


Therapy

There is no known cure, but medication may slow the progression so that a normal lifespan and quality of life may be attainable for many patients. However, specific treatment for fatigue, which may be invalidating in some patients, is unavailable. Ursodeoxycholic acid (Ursodiol) is one, which helps reduce the cholestasis. To relieve itching caused by bile acids in circulation, which would normally be removed by the liver, cholestyramine (a bile acid sequestrant) may be prescribed to absorb bile acids in the gut and be eliminated, rather than re-enter the blood stream. As in all liver diseases, alcoholic beverages are contraindicated. In advanced cases, a liver transplant, if successful, results in a favourable prognosis. Wikipedia does not have an article with this exact name. ... Ursodiol (trade names Actigall, Ursofalk, Urso Forte) is a bile acid found in large quantities in bear bile; it also occurs naturally in human bile in smaller quantities. ... Cholestyramine (Questran®, Questran Light®, Cholybar®) is a bile acid sequestrant, which binds bile in the gastrointestinal tract to prevent its reabsorption. ... In pharmacology, bile acid sequestrants a group of medication used for binding bile in the gastrointestinal tract. ... Bottles of cachaça, a Brazilian alcoholic beverage. ...


References

Medical

  • Mendelian Inheritance in Man (OMIM) 109720
  • M. Eric Gershwin, John M. Vierling, Michael P. Manns, eds. Liver Immunology. Philadelphia, Pa.: Hanley and Belfus, 2003. ISBN 1-56053-499-0. (State of the art; technical.)
  • Marshall M. Kaplan, and M. Eric Gershwin, "Primary Biliary Cirrhosis", New Engl. J. of Medicine, 353:1261-1273 September 22, 2005 Number 12 . Review article
  • Carlo Selmi, Ross L. Coppel, and M. Eric Gershwin, "Primary Biliary Cirrhosis", in Noel R. Rose, Ian R. Mackey, eds, The Autoimmune Diseases, 4th edition, Academic Press, 2006

The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ...

General

  • Sanjiv Chopra. The Liver Book: A Comprehensive Guide to Diagnosis, Treatment, and Recovery, Atria, 2002, ISBN 0-7434-0585-4
  • Melissa Palmer. Dr. Melissa Palmer's Guide to Hepatitis and Liver Disease: What You Need to Know, Avery Publishing Group; Revised edition May 24, 2004, ISBN 1-58333-188-3. her webpage.
  • Howard J. Worman. The Liver Disorders Sourcebook, McGraw-Hill, 1999, ISBN 0-7373-0090-6.

See also

Bile canaliculus (plural:bile canaliculi; also called bile capillaries) is a thin tube that collects bile secreted by hepatocytes. ...

External links


  Results from FactBites:
 
Primary Biliary Cirrhosis (408 words)
Primary biliary cirrhosis is a liver disease that slowly destroys the bile ducts in the liver.
The cause of primary biliary cirrhosis is unknown.
Primary biliary cirrhosis is diagnosed through laboratory tests, x rays, and in some cases, a liver biopsy (a simple operation to remove a small piece of liver tissue).
Primary biliary cirrhosis - Wikipedia, the free encyclopedia (575 words)
Primary biliary cirrhosis is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts within the liver.
To diagnose PBC, distinctions should be established from other conditions with similar symptoms, such as autoimmune hepatitis or primary sclerosing cholangitis (PSC).
An increase in GGTP indicates the presence of Primary Biliary Cirrhosis.
  More results at FactBites »

 
 

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