This glossary is for general informational purposes. It is not intended to be used for self-diagnosing or treatment. Always consult a health care professional.

A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

A

acute - sudden and severe onset (ie., acute liver failure).

Alagille’s Syndrome - a rare inherited liver disorder in which bile ducts are destroyed in infancy.

albumin - an abundant protein in the body produced in the liver from amino acids.

Alpha-1 Antitrypsin Deficiency - an inherited metabolic disorder which affects the lungs and/or liver.

ammonia intoxication - when the liver is severely compromised, it cannot convert ammonia to urea, thus producing high ammonia levels.

angiography - radiographic visualization of blood vessels.

ascites - fluid which develops in the abdomen when the liver is not functioning properly. Ascites often develops with cirrhosis of the liver.

B

bile - a by-product of the liver, the fluid flows to the intestines to aid in digestion and remove waste.

bile duct - the passageway which takes bile from the liver and empties it into the intestines.

bile lakes - dilatations of bile ducts in the liver.

biliary - pertaining to the liver, bile ducts or gallbladder.

Biliary Atresia - a disorder in newborns which destroys the bile ducts that carry bile from the liver to the intestines. This disease is a progressive destruction of the bile ducts.

bilirubin - the breakdown product of old blood cells in the body that are converted by the liver into a form with can be utilized and excreted by the body.

Biliary Tree - pertaining to the biliary system. Also the newsletter of the Children’s Liver Alliance.

biopsy - the removal of a small piece of tissue (ie., from the liver) using a thin needle for examination under a microscope.

Bowel - term used for both the large and small intestines.

Byler’s Disease - an autosomal recessive disorder in which cholestasis leads to hepatic fibrosis in infancy.

C

cholangitis - an inflammation of the bile ducts caused by bacteria from the bowel.

cholestasis - stoppage or suppression of bile flow.

cirrhosis - permanent scarring of the liver.

Crigler-Najjar - an inherited disorder of bilirubin metabolism resulting in chronic, severe unconjugated hyperbilirubinemia and jaundice.

crossmatch - test to determine whether antibodies recipient that could cause early rejection of the graft.

D

distention - a visible increase in the abdomen.

E

edema - an accumulation of fluid in the tissue which causes swelling.

encephalopathy - occurs when blood bypasses the liver. Can cause drowsiness or coma.

F

fibrosis - early scarring of the liver which may be caused by a healing response to injury, infection or inflammation.

Fulminant Hepatic Failure - a syndrome that occurs in a previously healthy patient who develops massive acute liver injury and rapid clinical deterioration with coagulopathy and hepatic encephalopathy within 2 weeks (or 8 weeks by an alternative criteria) of the onset of symptoms or jaundice.

G

gallbladder - a small pocket which stores bile.

Galactosemia - a rare hereditary disease leading not only to cirrhosis in infants, but more seriously, to early devastating illness if not diagnosed quickly.

gastroenterologist - doctor specializing in diseases of the stomach and
gastrointestinal track.

graft - an organ for transplant.

H

hepatic - relating to the liver.

hepatitis - an inflammation of the liver due to a virus or other insult to the liver.

hepatologist - doctor specializing in diseases of the liver.

I

immunosuppression - suppression of the body’s immune system using medications such as FK506, Neoral, Cyclosporine, CellCept and Imuran to prevent organ rejection.

J

jaundice - a yellow appearance of the skin and sclera (whites of eyes) caused by the buildup of bilirubin.

K

Kasai Procedure - a surgical method performed to reestablish bile flow from the liver to the intestine in infants diagnosed with biliary atresia.

L

liver - an internal organ which stores and filters blood, excretes toxic substances from other parts of the body, secretes bile and aids in metabolism. It weighs about 3 pounds and is about the size of a football.

living-related liver transplant - also known as “living donor”, a parent (or other family member) donates part of their own liver to be transplanted into their child.

N

Neonatal Hemochromatosis - a very rare disease in newborns which causes large amounts of iron to accumulate in the liver, thus destroying it.

nuclear scan - in liver disease, it determines how much, if any, bile is flowing through the liver.

O

OPO - also known as organ procurement organization, responsible for overseeing the procurement and donation of donor organs.

P

portal hypertension - increased blood pressure within the portal vein.

portal vein - the vein which drains the intestine supplying the liver.

R

rejection - when the transplanted organ is attacked by the immune system and antibodies are produced to fight them.

rickets - A weakening of the bones due to Vitamin D deficiency.

S

sclerotherapy - a method for treating distended veins by injecting a

solution to spleen - a large vascular organ which may increase in size with liver disease.

T

thrombosis - clotting within a blood vessel

U

ultrasound - A device used to identify any obvious abnormalities in the body.

UNOS - United Network for Organ Sharing. UNOS is responsible for overseeing the fair distribution of organs for transplantation.

V

varcies - high blood pressure in the veins within the esophagus and stomach. When these veins rupture, the term, bleeding varcies, is used.

W

Wilson’s Disease - an inherited disorder where large amounts of copper accumulate in the liver.

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