Glossary

A diagnostic and therapeutic procedure through which extra fluid from the abdomen (ascites) is removed through a catheter (small, flexible tube).

The build-up of fluid in the space between the lining of the abdomen and abdominal organs. The most common causes of ascites are cirrhosis, heart failure, and cancer. Ascites results from high pressure in the blood vessels of the liver (portal hypertension).

Devices used for those afflicted by a disability or injury. These are tools, products, or types of equipment that assist in performing tasks and activities. They may help one move around, see, communicate, eat, or get dressed.

Liver inflammation that occurs when the body's infection-fighting system (immune system) attacks liver cells. This causes swelling, inflammation and liver damage. It is a long-term or chronic inflammatory liver disease.

Also known as overlap syndrome, refers to an autoimmune condition with immunological, clinical and histological factors of both autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC).

Immune system overactivity, where the body attacks and damages its own tissues (autoimmune disease). This is currently being studied as a factor contributing to the development of PSC, along with genetic and environmental factors.

The process of stretching a stricture (narrowed area of a bile duct) using a small, balloon-like device, thereby improving bile flow.

One way of controlling bleeding of varices. Varices are large, dilated veins that develop in the esophagus (swallowing tube) when there is elevated pressure in the portal vein, the large vein that enters the liver. Sometimes these varices can bleed or be at high risk of bleeding. Placing a “band” on the vein allows for a clot to form and stop the bleeding. The bands are placed using an endoscope.

A liquid made by the liver and contains water, cholesterol, bile salts, electrolytes, and waste products such as bilirubin. Bile is stored in the gallbladder and passes through the bile ducts into the intestine where it helps digest fat.

Tube-like structures that carry bile from the liver to the intestines.

Also called biliary tree or biliary tract, refers to the liver, gallbladder and bile ducts which create, move, store, and release bile into the small intestine.

An orange-yellow substance made during the normal process when red blood cells break down. Bilirubin goes through the liver, mixing with bile, and then is removed from the body through the stool.

Any biological-based measurement that provides useful information regarding a person’s health. For example, blood test results showing increased glucose levels can be used as a biomarker for diabetes. MRI, CT, and x-ray scans are all examples of imaging biomarkers. Biomarkers are used to diagnose a disease or predict prognosis; track disease progression; determine whether a drug prevents, improves, or slows disease progression within clinical trials.

Samples of urine, blood, tissue, cells, DNA, RNA, or protein, that are stored in a biorepository for future research.

Within the context of the PSC Partners Patient Registry, refers to a person who is involved in the participant’s care and enters the participant’s information into the Registry on the participant’s behalf.

A rare cancerous (malignant) growth in one of the bile ducts.

The imaging of the bile ducts by x-rays aided by the injection of a dye.

The inflammation of one or more bile ducts. Cholangitis may be caused by a bacterial infection.

The reduction or stoppage of bile flow. Disorders of the liver, bile duct, or pancreas can cause cholestasis, as can certain medications.

Something that continues over a long period of time or often recurs. A chronic condition usually does not go away easily or quickly. Chronic is the opposite of acute.

The late stage of scarring (fibrosis) of the liver caused by many forms of liver diseases.

A research study that tests a medical, surgical, or behavioral intervention in people. These trials are the primary way that researchers determine if a new form of treatment or prevention, such as a new drug, is safe and effective in people.

Two health conditions that are present at the same time, such as PSC and IBD.

A type of inflammatory bowel disease, Crohn’s most commonly affects the end of the small bowel (the ileum) and the beginning of the colon, but it may affect any part of the gastrointestinal (GI) tract.

A bone densitometry scan using a small dose of radiation to produce pictures of the inside of the body (usually the lower (or lumbar) spine and hips) to measure bone loss.

A procedure in which an organ is removed from one transplant candidate and immediately transplanted into a second patient, with the first patient receiving a new organ from a deceased donor.

The swelling or puffiness caused by excess fluid trapped in your body's tissues, often seen in the hands, ankles and feet.

A procedure using an endoscope to see and diagnose diseases of the gallbladder, biliary system, pancreas, and liver. The test looks "upstream" where digestive fluid comes from -- the liver, gallbladder, and pancreas -- to where it enters the small intestines. If there is a blockage, a stent (tube) may be placed.

Also called esophagogastroduodenoscopy, is a nonsurgical procedure used to examine a person's digestive tract. Using an endoscope, a flexible tube with a light and camera attached to it, the doctor can view the digestive tract and perform certain procedures, such as a biopsy or stent (tube). Endoscopic ultrasound (EUS) uses ultrasound during endoscopy.

A blood test that provides a score reflecting the severity of liver fibrosis. ELF may be used as a diagnostic test, a prognostic marker, and for disease monitoring.

Reddish, painful, and tender lumps most commonly located in the front of the legs below the knees.

Abnormal, enlarged veins in the lower part of the esophagus.

Situated or originating outside the liver.

An acronym for Federal Information Security Management Act is legislation meant to ensure the security of data in the federal government. Annual reviews are conducted to assess the security controls in information systems. NIH requires that every registry under its umbrella be FISMA compliant. The PSC Partners Patient Registry is FISMA compliant.

The procedure through which stool from a healthy donor is placed into the gut of a patient in order to treat some conditions. This procedure is typically done via colonoscopy.

A type of liver elastography, a special ultrasound technology that measures liver stiffness (hardness) and fatty changes in the liver.

The scarring of the liver tissues when the liver tries to repair and replace damaged or inflamed cells. Scar tissue does not work as well as healthy liver tissue.

An acronym for General Educational Development tests, refers to a group of five subject tests, which, when passed, certifies that the taker has American or Canadian high school level academic skills.

The acronym for Health Insurance Portability and Accountability Act (1996) which was created for the protection of identifiable health information. HIPAA requires the protection and confidential handling of protected health information. The PSC Partners Patient Registry is HIPAA compliant.

The deterioration of brain function that occurs in people with severe liver disease, also called “brain fog.” HE occurs when toxins that are normally cleared from the body by the liver, build up in the blood, and eventually affect the brain.

Inflammation of the liver, commonly caused by a viral infection, but also may be from autoimmune disease, or as a result of other infections, medications, or toxic substances (alcohol or certain drugs).

Biliary strictures that occur due to elevated IgG4-positive plasma cells in tissue. Serum IgG4 elevation is frequently associated with pancreatic involvement.

A term used to describe cases of IBD in which a clear diagnosis of ulcerative colitis (UC) or Crohn’s disease cannot be made. Most are eventually declared as ulcerative colitis or Crohn’s disease.

An umbrella term used to describe disorders that involve chronic inflammation of the digestive tract, such as ulcerative colitis and Crohn’s disease. Symptoms of ulcerative colitis and Crohn’s can include severe diarrhea, bloody stools, abdominal pain, fatigue, and weight loss.

An independent board that approves, monitors and reviews research involving humans. Its number one priority is to protect human subjects from harm. The PSC Partners patient registry is IRB approved and will be IRB monitored annually.

A condition in which the skin, whites of the eyes and mucous membranes turn yellow because of a high level of bilirubin, a yellow-orange bile pigment. Jaundice has many causes, including liver disease (hepatitis), gallstones, blockage of the bile ducts, blood disorders, and tumors, as well as a disease called Gilbert’s syndrome, or medication side effects.

When a small sample of liver tissue is removed by inserting a special needle through the abdominal wall and into the liver. Ultrasound guidance is often used and numbing medicine is applied before the needle is placed. The tissue can then be examined under a microscope for signs of damage or disease, to help decide the best treatment, or to find out how a treatment for liver disease is working.

A method to identify who most needs a liver transplant for patients with chronic liver disease. The MELD score includes the laboratory values of bilirubin, creatinine, and international normalized ratio (INR), and sodium. For children under 12, the PELD (Pediatric End-Stage Liver Disease) score is used to indicate disease severity.

A non-invasive exam with a special type of magnetic resonance imaging (MRI) to show detailed images of the hepatobiliary and pancreatic systems, including the liver, gallbladder, bile ducts, pancreas, and pancreatic duct.

A medical imaging technique that uses a magnetic field and computer-generated radio waves to create detailed images of the organs and tissues in the body

In humans, this consists of the 10-100 trillion symbiotic (involving beneficial or harmful interaction among cells) microbial cells harbored by each person, primarily bacteria in the gut; the human microbiome consists of the genes these cells harbor.

A unique patient ID that can be created without transmitting or storing a patient’s identifying information. This allows datasets to be linked together at the patient level while staying compliant with HIPAA, GDPR, and most regional privacy laws.

Within the context of the PSC Partners Patient Registry patient surveys, this means the patient.

An outcome directly reported by the patient and pertains to the patient’s health, quality of life, or functional status associated with health care or treatment. This places patients at the center of healthcare research and clinical care evaluation. Patient outcomes may include general health, ability to complete various activities, mood, level of fatigue, or pain.

An X-ray of the bile ducts inside and outside the liver; taken after contrast (dye) is injected directly into the bile ducts.

Lymphoid and/or plasmacytic proliferations that can occur in patients receiving chronic immunosuppression for solid organ transplantation.

Foods, typically high fiber, that promote the growth of beneficial microorganisms in the intestines.

A chronic autoimmune disease in which the small bile ducts in the liver become injured and inflamed and are eventually destroyed.
● PBC affects the bile ducts within the liver (intrahepatic), while PSC can affect the bile ducts inside or outside of the liver (extrahepatic).
● PBC is more predominant in people assigned female at birth, by a ratio of 10:1. PSC is more predominant in people assigned male at birth, by a ratio of 2:1.
● PSC is highly associated with preexisting IBD (80%), while PBC is not.
● PSC is associated with an increased risk of developing bile duct cancer, while PBC is not.

Live microorganisms that when administered in adequate amounts, can confer a health benefit on the person by improving or restoring the gut flora.

Localized or generalized itching, a prevalent and distressing symptom of cholestasis.

PSC recurs post-transplant in 10%–37% of transplant recipients. The diagnostic criteria of rPSC include a confirmed diagnosis of PSC before transplant, a cholestatic pattern of liver enzyme elevations, cholangiography demonstrating biliary strictures, and other criteria.

A standardized database of medical data that can include information such as diagnosis, family history, medications, surgeries, quality of life, and test results. Often registries are disease-specific and data can be either patient reported or physician reported. The PSC Partners Patient Registry is a patient-driven, patient-reported registry that serves to facilitate PSC research and clinical trials.

Within the context of the PSC Partners Patient Registry, this is the person who assists the Registry participants, and those who are interested in joining, by answering questions. The coordinator reviews survey responses to ensure consistency.

A procedure to bypass the bile duct to allow digestive juices to drain from the liver directly into the small intestine.

Stiffening or abnormal hardening of a body tissue.

A less common variant of PSC that is characterized by typical cholestatic and histological features of PSC but with normal bile ducts on cholangiography.

A procedure in which a single deceased donor liver is divided into right and left portions that are implanted into two recipients simultaneously. Typically, an adult patient receives about 60 percent of the liver (the right lobe), while a pediatric patient receives the other about 40 percent of the liver (the left lobe).

A thin, hollow tube that is placed in the bile duct to hold the duct open after the duct has been blocked or partly blocked. The stent allows bile to flow through the bile duct into the intestine to help digestion.

An abnormal narrowing of a body passage, especially a tube or canal like the bile duct, which can slow or obstruct the flow of body fluids. Inflammation, cancer, or scar tissue may cause a stricture.

A non-invasive test using a specialized ultrasound machine and low-frequency elastic waves to assess the hardness (or stiffness) of the liver and estimate the degree of liver fibrosis or cirrhosis.

A procedure that involves inserting a stent (tube) to connect the portal veins to adjacent blood vessels that have lower pressure.

A type of inflammatory bowel disease (IBD) that causes long-lasting inflammation and sores (ulcers) in the innermost lining of the large intestine (colon) and rectum. PSC is often associated with ulcerative colitis.

A radiation-free imaging method that uses high-frequency sound waves which reflect off body structures to create images (pictures) of the inside of your body.

Inflammation of the uvea, the middle layer of the eye.