Biliary atresia Health Dictionary

Biliary Atresia: From 2 Different Sources


A rare disorder, present from birth, in which some or all of the bile ducts fail to develop or have developed abnormally.

As a result, bile is unable to drain from the liver (see cholestasis).

Unless the atresia can be treated, secondary biliary cirrhosis will develop and may prove fatal.

Symptoms include deepening jaundice, usually beginning a week after birth, and the passing of dark urine and pale faeces.

Treatment is by surgery to bypass the ducts.

If this fails, or if the jaundice recurs, a liver transplant is the only possible treatment.

Health Source: BMA Medical Dictionary
Author: The British Medical Association
a congenital or acquired condition characterized by obstructed bile flow secondary to destruction or absence of extrahepatic bile ducts. Babies usually present within the first few weeks of life with jaundice that does not improve with time. Some forms of biliary atresia can be corrected surgically, but if the diagnosis has been delayed the condition may lead to irreversible liver damage requiring liver transplantation.
Health Source: Oxford | Concise Colour Medical Dictionary
Author: Jonathan Law, Elizabeth Martin

Biliary Colic

See CHOLECYSTITIS, CHOLECYSTALGIA, etc.... biliary colic

Atresia

The absence of a natural opening, or closure of it by a membrane. Thus atresia may be found in newborn infants, preventing the bowels from moving. In young girls after puberty, absence of the menstrual ?ow may be due to such a malformation at the entrance to the VAGINA.... atresia

Biliary Cirrhosis

An uncommon form of liver cirrhosis that results from problems with the bile ducts, either due to an autoimmune disorder known as primary biliary cirrhosis, or a longstanding blockage. Primary biliary cirrhosis affects mainly middle-aged women and seems to be linked with a malfunction of the immune system. Secondary biliary cirrhosis results from prolonged bile duct obstruction or biliary atresia. In both types, liver function is impaired due to cholestasis (accumulation of bile in the liver). In primary biliary cirrhosis, the bile ducts within the liver become inflamed and are destroyed. Symptoms include itching, jaundice, an enlarged liver, and sometimes abdominal pain, fatty diarrhoea, and xanthomatosis. Osteoporosis may develop. Symptoms of liver cirrhosis and liver failure may occur after several years. Drugs can minimize complications and relieve symptoms such as itching. A liver transplant is the only long-term cure.

The symptoms and signs of secondary biliary cirrhosis include abdominal pain and tenderness, liver enlargement, fevers and chills, and sometimes blood abnormalities. Treatment is the same as for bile duct obstruction.... biliary cirrhosis

Biliary System

The organs and ducts by which bile is formed, concentrated, and carried from the liver to the duodenum (the first part of the small intestine). Bile is secreted by the liver cells and collected by a network of bile ducts that carry the bile out of the liver by way of the hepatic duct. A channel called the cystic duct branches off the hepatic duct and leads to the gallbladder where bile is concentrated and stored. Beyond this junction, the hepatic duct becomes the common bile duct and opens into the duodenum at a controlled orifice called the ampulla of Vater. The presence of fat in the duodenum after a meal causes secretion of a hormone, which opens the ampulla of Vater and makes the gallbladder contract, squeezing stored bile into the duodenum.

The main disorders affecting the biliary system are gallstones, congenital biliary atresia and bile duct obstruction.

(See also gallbladder, disorders of.)... biliary system

Choanal Atresia

A congenital defect of the nose in which 1 or both of the nasal cavities are not fully developed.... choanal atresia

Oesophageal Atresia

A rare birth defect in which the oesophagus forms into 2 separate, blind-ended sections during development. There is usually an abnormal channel (tracheoesophageal fistula) between one of the sections and the trachea. The condition may be suspected before birth if the mother had polyhydramnios. The infant cannot swallow, and drools and regurgitates milk continually. If there is an upper tracheoesophageal fistula, milk may be sucked into the lungs, provoking attacks of coughing and cyanosis. Immediate surgery is needed to join the blind ends of the oesophagus and close the fistula. If the operation is successful, the baby should develop normally. Some babies, however, do not survive.... oesophageal atresia

Biliary

adj. relating to or affecting the bile duct or bile. See also fistula.... biliary

Duodenal Atresia

a condition in which there is congenital narrowing of the duodenum causing complete obstruction. It presents at birth with vomiting, which is usually bile-stained, and is associated with other congenital abnormalities, particularly *Down’s syndrome. Treatment is by restoration of any fluid and electrolyte loss followed by surgical repair.... duodenal atresia

Tricuspid Atresia

a rare form of congenital heart disease in which there is no communication between the right atrium and the right ventricle. Affected babies present with *cyanosis, breathlessness, particularly on feeding, and *failure to thrive. Diagnosis is by *echocardiography. Treatment involves surgical intervention, but the prognosis is often poor.... tricuspid atresia



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