Biliary atresia is a rare disease of the liver and bile ducts that occurs in infants. Symptoms of the disease appear or develop about two to eight weeks after birth. Cells within the liver produce liquid called bile. Bile helps to digest fat. It also carries waste products from the liver to the intestines for excretion.

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Biliary atresia BA is a congenital biliary disorder, which is characterized by an absence or severe deficiency of the extrahepatic biliary tree. It is one of the most common causes of neonatal cholestasis, often causing cirrhosis immediately and leading to death and accounts for over half of children who undergo liver transplantation.

It is thought to affect 1 in 10,, newborn infants. There is a recognized male predilection. It precipitates within the first three months of life. Infants with biliary atresia may appear normal and healthy at birth. Most often, symptoms develop between two weeks to two months of life, and may include :. Although typically thought to result from an idiopathic destructive inflammatory process which leads to fibrotic remnants at porta hepatis, the disease may be secondary to viral infections or autoimmune induced injury in some cases.

Cases of biliary atresia typically demonstrate relatively good hepatic uptake with no evidence of excretion into the bowel at 24 hours. Its important to diagnose it early since Kasai portoenterostomy done within initial 2 months of age has very good prognosis. Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Updating… Please wait. Unable to process the form. Check for errors and try again. Thank you for updating your details. Log In. Sign Up.

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Biliary atresia

NCBI Bookshelf. Asma I. Siddiqui ; Tahani Ahmad. Authors Asma I. Siddiqui 1 ; Tahani Ahmad.


Biliary Atresia

Biliary atresia BA is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. For these infants the aetiology lies within the first trimester of gestation. For others affected with BA, aetiology is more obscure and perinatal destruction of fully-formed ducts perhaps by the action of hepatotropic viruses has been suggested. Whatever the cause, the lumen of the extrahepatic duct is obliterated at a variable level and this forms the basis for the commonest classification Types I, II, III. All patients with BA present with varying degree of conjugated jaundice, pale non-pigmented stools and dark urine.


Biliary atresia , also known as extrahepatic ductopenia and progressive obliterative cholangiopathy , is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. It has an incidence of one in 10,—15, live births in the United States , [2] and a prevalence of one in 16, in the British Isles. The cause of biliary atresia in Egyptian infants has been proven to be as a result of aflatoxin induced cholangiopathy acquired prenatally in infants who have glutathione S transferase M1 deficiency. The biliary atresia phenotype caused by congenital aflatoxicosis in GST M1 deficient neonates is named Kotb disease.

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