Glossary

Alanine aminotransferase (ALT): an enzyme primarily located in the liver that helps the body get rid of nitrogen; ALT levels can get higher when the liver is injured

Albumin: a protein made in the liver; when albumin levels drop, it can be a sign of inflammation from liver illness

Alkaline phosphatase (ALP): an enzyme that can be traced in the blood and can be used to track how well a person is responding to their primary biliary cholangitis (PBC) treatment

Alpha1-antitrypsin: a protein that is primarily produced in the liver and can be used to warn doctors that certain diseases may be developing

Antimitochondrial antibodies (AMAs): antibodies are proteins that the body releases to identify and neutralize harmful molecules in the body such as bacteria and viruses; AMAs are antibodies that attack the membranes of certain cells and are seen in most, but not all, people who have primary biliary cirrhosis (PBC)

Ascites: an accumulation of fluid in the abdominal area of the body that is a symptom of later-phase primary biliary cirrhosis (PBC)

Aspartate aminotransferase (AST): an enzyme that helps rid the body of excess nitrogen; AST can be elevated as a result of damage to tissues of the body, including the liver

Autoimmune disease: a disorder where the body’s natural defense mechanisms attack the body’s own cells as though a foreign body such as a virus or bacteria

Autoimmune thyroid disease (Hashimoto’s disease): A disorder causing chronic inflammation of the thyroid gland, which may affect its function

Bile: a fluid made in the liver and stored in the gallbladder; when released by the liver, bile helps with digestion

Bile ducts: tubes in the body that carry bile from the liver to the small intestine; in PBC, bile ducts are damaged which can cause bile to build up in the liver; bile buildup can become toxic and can further damage the bile ducts

Bilirubin: a yellow bile pigment that is usually processed by the liver and excreted as bile; when the liver is not working properly, bile can accumulate in the blood and tissues of the body and can make the skin, and sometimes the eyes, appear yellow (jaundice)

Biomarker: a biological substance that indicates that a medical condition exists

Celiac disease: A disorder in which individuals are not able to tolerate gluten, a protein found in wheat, leaving individuals unable to absorb nutrients

Cholangitis: inflammation of the bile ducts

Cholestasis: slowing down or stopping the normal flow of bile

Cirrhosis: a serious condition of the liver where the organ loses its shape and stops working because of an accumulation of scar tissue; cirrhosis can cause chronic liver failure and may require a liver transplant

Contagious: capable of being transmitted from one person to another

CREST syndrome: a disorder that results in hardening of the skin and consists of calcinosis, Raynaud’s disease, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome

Depression: a mental state characterized by feelings of sadness, despair, and discouragement; depression can range from normal feelings of “the blues” to a serious disorder that interferes with daily life

Enzyme: a protein molecule that helps encourage chemical reactions in the body

Fibrosis: the formation of excessive fibrous tissue in response to tissue damage

Fluid retention: when the body holds on to liquids that otherwise would be removed; the liquids accumulate and can cause swelling

Gamma-glutamyl transpeptidase (GGT): an enzyme that generally occurs in the cells of the kidney

Hepatocellular carcinoma: cancer of the liver

Inflammation: the increase of immune cells to a particular group of cells or tissues as a response to cellular injury; typically involves pain, heat, redness, and swelling

Inherited: passed from parent to child through the genes

Jaundice: a yellow appearance of the skin and/or eyes caused by having too much bile in the body

Liver biopsy: a procedure that removes a small amount of tissue from the liver; the sample can then be closely looked at to make a diagnosis or to see how damaged the liver may be

Osteoporosis: a condition where bones become weaker and more fragile

Portal hypertension: abnormally high blood pressure in a major vein of the liver that is a complication of cirrhosis

Progressive: becoming worse over time

Proteins: an important group of compounds in the body that are essential components of cells and tissues and help with chemical reactions important to life

Prothrombin time: a measure of the clotting ability of the blood

Pruritus: itching of the skin that can affect a specific spot on the skin or can be felt all over

Rare disease: in the United States, a disease is considered rare (or orphan) if it affects fewer than 200,000 people

Raynaud’s disease: A disorder that causes a restriction of oxygen flow to the fingers and toes resulting in discoloration of the skin in response to stress or cold

Rheumatoid arthritis: A disorder in which the joints are chronically inflamed

Sclerosis: a hardening of tissue caused by inflammation and the buildup of scar tissue

Sjögren’s syndrome: A disorder in which the body’s mucus-secreting glands, such as the tear ducts of the eyes and saliva glands of the mouth, stop working properly over time

Varices: enlarged and abnormally windy veins

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