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Diseases » Biliary conditions » Glossary
 

Glossary for Biliary conditions

  • Aagenaes syndrome: A rare inherited birth abnormality involving underdeveloped lymph vessels which results in swollen legs and liver problems.
  • Acute Cholecystitis: Acute inflammation of the gall bladder, usually due to obstruction by a gall stone
  • Addison-Gull syndrome: A condition where scarring of the liver and bile ducts which results in chronic jaundice, enlarged spleen and liver and yellow skin plaques due to abnormal lipid metabolism.
  • Alagille Syndrome: A genetic disorder affecting the liver and characterized by the absence of some or all of the liver bile ducts that transport bile within the liver.
  • Arthrogryposis -- renal dysfunction -- cholestasis syndrome: A very rare syndrome characterized by joint contractures, kidney dysfunction and liver problems.
  • Benign intrahepatic cholestasis type 1: A rare liver disorder characterized by intermittent episodes of cholestasis (impaired bile flow within the liver) that doesn't involve obstruction of bile ducts outside the liver. The jaundice tends to disappear in weeks or months without treatment and the condition does not lead to liver failure. Type 1 is caused by a defect on the ATP8B1 gene on chromosome 18q21.
  • Benign intrahepatic cholestasis type 2: A rare liver disorder characterized by intermittent episodes of cholestasis (impaired bile flow within the liver) that doesn't involve obstruction of bile ducts outside the liver. The jaundice tends to disappear in weeks or months without treatment and the condition does not lead to liver failure. Type 2 is caused by a defect on the ABCB11 gene on chromosome 2q24.
  • Bile Duct Conditions: An inflammatory bacterial infection that affects the meninges
  • Bile acid synthesis defect, congenital, 2: A defect which prevents the body from making bile acid which results in progressive liver disease. The defect is a deficiency of a particular enzyme (cholestasis with delta(4)-3-oxosteroid 5-beta-reductase) needed to make bile acid.
  • Bile acid synthesis defect, congenital, 4: A defect which prevents the body from making bile acid which results in progressive liver disease.
  • Bile acid synthesis defects: A defect which prevents the body from making bile acid which results in progressive liver disease.
  • Bile acid synthesis defects, congenital, 1: A defect which prevents the body from making bile acid which results in progressive liver disease. The defect is a deficiency of a particular enzyme (3-beta-hydroxy-delta-5-C27-steroid oxidoreductase) needed to make bile acid.
  • Bile acid synthesis defects, congenital, 2: A defect which prevents the body from making bile acid which results in progressive liver disease. The defect is a deficiency of a particular enzyme (cholestasis with delta(4)-3-oxosteroid 5-beta-reductase) needed to make bile acid.
  • Bile acid synthesis defects, congenital, 3: A defect which prevents the body from making bile acid which results in progressive liver disease. The defect involved a deficiency of 7-alpha-hydroxylase which is an enzyme needed to prevent the accumulation of 27-hydroxycholesterol which is toxic to the liver.
  • Bile acid synthesis defects, congenital, 4: A defect which prevents the body from making bile acid which results in progressive liver disease.
  • Bile duct cancer, extrahepatic: A rare cancer that develops in the part of the common bile duct that is outside the liver. The common bile duct channels bile from the gall bladder (which stores bile) and the liver (which makes bile) to the small intestine to assist digestion.
  • Bile duct cysts: The formation of a cyst (dilation or swelling) in the bile duct which can obstruct the flow of bile and result in jaundice.
  • Bile duct paucity in Graft-Versus-Host Disease: A reduced number of ducts that carry bile through and from the liver (bile duct) that results from graft-versus-host disease.
  • Bile duct paucity in Liver Allograft Rejection: A reduced number of ducts that carry bile through and from the liver (bile duct) that results from liver allograft rejection.
  • Bile duct paucity, non syndromic form: A reduced number of ducts that carry bile through and from the liver (bile duct) that is not associated with a syndrome. Paucity of bile ducts is associated with many liver conditions such as biliary cirrhosis and sclerosing cholangitis.
  • Bile ducts paucity, non syndromic form:
  • Bile plug syndrome: A rare condition where thick bile in infants causes blockage of the bile ducts which can result in jaundice, enlarged liver and anemia.
  • Biliary Atresia: A rare condition that can affect newborns where the bile duct that carries the bile from the liver to the small intestine is blocked or missing.
  • Biliary atresia, intrahepatic, non syndromic form: Congenital obstruction of the passages in the liver that carry bile. The nonsyndromic form is not associated with any other abnormalities.
  • Biliary atresia, intrahepatic, syndromic form: Congenital obstruction of the passages in the liver that carry bile. The syndromic form is associated with other congenital abnormalities such as heart and visceral defects.
  • Biliary cirrhosis: Biliary cirrhosis is a condition where the bile ducts are unable to transport bile effectively due to blockage, inflammation, scarring or some other damage to the bile ducts. The condition may result from such things as congenital defect of the bile ducts (e.g. biliary atresia), cystic fibrosis, gallstones or a variety of other secondary conditions. The cause of primary biliary cirrhosis is not fully understood.
  • Biliary colic: Colicky pain as a result of gallstones blocking the gallbladder.
  • Biliary disorder: Any condition affecting the bile ducts
  • Biliary hypoplasia: An underdeveloped biliary duct system which is involved in transporting bile. The bile ductules may be completely absent or be fewer in number than normal.
  • Biliary tract cancer: Cancer that develops in the gallbladder or bile ducts.
  • Byler Disease: A rare inherited conditions where bile is unable to drain from the liver where it builds up and causes progressive liver damage. The conditions has an early onset and usually leads to end-stage liver disease by the end of the second decade.
  • Cholangitis: bile duct inflammation (cholangitis)
  • Cholecystitis: Inflammation of the gallbladder which concentrates and stores bile. The condition may occur suddenly (acute) or persist over a longer period of time (chronic).
  • Choledochal cyst, hand malformation: A rare disorder characterized by the dilation of a portion of the biliary system and hand malformation.
  • Cholelithiasis: Is the presence of gallstones in the gallbladder
  • Cholestasis: A condition where the bile flow is impaired or completely halted.
  • Cholestasis -- lymphoedema, syndrome: A rare inherited birth abnormality involving underdeveloped lymph vessels which results in swollen legs and liver problems.
  • Cholestasis -- pigmentary retinopathy -- cleft palate: A rare syndrome characterized by degeneration of retinal pigments, cleft lip, cleft palate, kidney problems and cholestasis (bile flow obstruction).
  • Cholestasis disease of pregnancy: A liver condition that can occur usually in the last stage of pregnancy. The flow of bile in the gallbladder or liver is impaired by the high levels of pregnancy hormones. The bile acids build up in the organs and then enter the bloodstream. The main symptom is usually itching. Symptoms usually resolve after delivering the baby. Risk factors includes existing liver condition, multiple pregnancy and a family history of the condition.
  • Cholestasis, Intrahepatic: A liver disorder where the bile is unable to flow properly through the liver. The severity of the condition is variable depending on the cause of the condition.
  • Cholestasis, intrahepatic of pregnancy: A rare liver disorder (impaired flow of bile) that occurs during third trimester of pregnancy with symptoms usually disappearing after delivery.
  • Cholestasis, progressive familial intrahepatic 1: A rare inherited condition where bile is unable to drain from the liver where it builds up and causes progressive liver damage.
  • Cholestasis, progressive familial intrahepatic 2: A rare inherited condition where bile is unable to drain from the liver where it builds up and causes progressive liver damage. The condition has an early onset and usually leads to end-stage liver disease by the end of the second decade. The various types of this condition differ in the origin of the genetic defect (liver-specific ATP-binding cassette transporter on chromosome 2q24). Type 2 is also associated with an increased risk of liver cancer in the first few years of life.
  • Cholestasis, progressive familial intrahepatic 3: A rare inherited condition where bile is unable to drain from the liver where it builds up and causes progressive liver damage. The condition has an early onset and usually leads to end-stage liver disease by the end of the second decade. The various types of this condition differ in the origin of the genetic defect (liver-specific ATP-binding cassette transporter on chromosome 2q24). Type 2 is also associated with an increased risk of liver cancer in the first few years of life.
  • Cholestatic jaundice -renal tubular insufficiency: A very rare syndrome characterized by liver and kidney problems.
  • Congenital bronchobiliary fistula: A rare malformation where there is an abnormal opening between a bile duct and the bronchial (respiratory) system.
  • Fatigue: Excessive tiredness or weakness.
  • Flatulence: Passing wind through the anus
  • Gall Bladder Cancer: Cancer of the gall bladder.
  • Gall bladder conditions: Any condition that affects the gallbladder
  • Gallstones: Stone-like deposits in the gall bladder.
  • Habba syndrome: Chronic diarrhea caused by a dysfunctional gall bladder which produces an abnormal amount of bile.
  • Hanot-MacMahon-Thannhauser syndrome: A liver disorder where chronic inflammation of the small portal bile ducts leads to blockage of the smallest ducts. As a result, fatty deposits develop in the skin, the liver and kidneys become enlarged and jaundice occurs.
  • Hepatic amyloidosis with intrahepatic cholestasis: Amyloidosis is a rare group of metabolic disorders where a protein called amyloid accumulates in body organs and tissues where it can cause damage. In hepatic amyloidosis, the amyloid deposits occur primarily in the liver which affects the flow of bile through the organ and results in intrahepatic cholestasis.
  • Hypoplasia hepatic ductular: A rare liver disorder where there are fewer than normal bile ducts in the liver - sometimes all the bile ducts are absent. Alagille syndrome is characterized by this liver abnormalitiy as well as a distinctive face, abnormal vertebrae and retarded physical, mental and sexual development.
  • Ichthyosis congenital -- biliary atresia: A very rare syndrome characterized mainly by dry, rough, scaly skin from birth and an abnormal biliary tract.
  • Itching skin: Itching feeling of the skin.
  • Jaundice: Bile or liver problem causing yellowness.
  • Lambert syndrome: A rare syndrome characterized by a club foot, inguinal hernia, biliary atresia and branchial dysplasia.
  • Left-sided gallbladder: A very rare disorder where the gallbladder is located in the left side of the abdomen. Generally this causes no symptoms but may be important if abdominal surgery is being undertaken.
  • Lutz-Richner-Landolt syndrome: A rare disorder involving kidney and biliary abnormalities.
  • Malabsorption due to bile acid synthesis defects, idiopathic: Impaired synthesis of bile acids which impairs digestive system functioning and results in severely inhibited absorption of fats.
  • Malignant obstructive biliary disease: Obstruction or blockage in one of the bile ducts by a tumor. Bile ducts are vessels which carry bile from the liver to the digestive system or gallbladder. Jaundice is usually one of the main symptoms.
  • NISCH syndrome: A rare syndrome characterized by sparse scalp hair, scaly skin and inflammation and fibrosis of the bile ducts.
  • Neonatal Jaundice: Common skin yellowing jaundice in newborn babies.
  • Neuroma biliary tract: A benign nerve tumor that occurs in the biliary tract. Often occurs after certain surgeries such as gall bladder removal.
  • Obstructive biliary disease: A disease process that causes obstruction or blockage in one of the bile ducts which are vessels that carry bile from the liver to the digestive system or gallbladder. Diseases that can cause such obstructions includes tumors, gallstones, parasites, bile duct inflammation, trauma to the bile duct and biliary strictures.
  • PFIC: A rare inherited condition where bile is unable to drain from the liver where it builds up and causes progressive liver damage. End-stage liver disease usually occurs before adulthood. There are three different subtypes of the disorder, each with a different genetic origin of the defect.
  • Primary Biliary Cirrhosis 1: Primary biliary cirrhosis is a chronic condition involving progressive destruction of the bile ducts in the liver. This results in an abnormal buildup of bile in the liver which eventually progresses to liver failure. Primary biliary cirrhosis 1 is linked to a genetic defect on chromosome 3p12-q13.2.
  • Primary Biliary Cirrhosis 2: Primary biliary cirrhosis is a chronic condition involving progressive destruction of the bile ducts in the liver. This results in an abnormal buildup of bile in the liver which eventually progresses to liver failure. Primary biliary cirrhosis 2 is linked to a genetic defect on chromosome 6p21.3.
  • Primary Biliary Cirrhosis 3: Primary biliary cirrhosis is a chronic condition involving progressive destruction of the bile ducts in the liver. This results in an abnormal buildup of bile in the liver which eventually progresses to liver failure. Primary biliary cirrhosis 3 is linked to a genetic defect on chromosome 1p31.2.
  • Primary biliary cirrhosis: Primary biliary cirrhosis is a chronic and progressive cholestatic disease of the liver.
  • Primary sclerosing cholangitis: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology that is recognized increasingly in children.
  • Rokitansky-Aschoff sinuses of the gallbladder: An abnormality of the gallbladder wall where small pouches of the mucus layer develop abnormal sinuses or spaces within that gallbladder lining.
  • Sclerosing Cholangitis: Chronic hereditary disease causing inflammation and destruction of the bile ducts in and around the liver with subsequent blockage to bile flow
  • Secondary Biliary Cirrhosis: Secondary biliary cirrhosis is a condition where the bile ducts are unable to transport bile effectively due to a secondary cause which results in blockage, inflammation, scarring or some other damage to the bile ducts. The condition may result from such things as congenital defect of the bile ducts (e.g. biliary atresia), cystic fibrosis, gallstones or a variety of other secondary conditions.
  • Upper abdominal pain: The occurrence of abdominal pain which occurs in the upper abdoment
  • Weight loss: Loss of body weight.
  • Xanthogranulomatous cholecystitis: A lipid laden foam cell tumour of the gallbladder resulting in inflammation

 

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