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Biliary atresia (BA) is a congenital disease characterized by fibrosis, obstruction, and obliteration of the biliary system that is universally fatal without intervention.
- BA accounts for approximately 30% of cases of neonatal cholestasis.
- BA is the most common cause of persistent cholestasis in infants and children and the most frequent indication for pediatric liver transplantation.
- The disease affects 1:8,000 to 1:18,000 live births.
- No single genetic mutation has been identified as the sole cause of BA, and there is no clear pattern of inheritance.
- Genes influencing morphogenesis may contribute to pathophysiology.
- Biliary obstruction begins at, or near, the time of birth and progresses throughout early infancy, leading to damage and ultimately scarring of liver parenchyma.
- Approximately 20% of biliary patients have at least one other major congenital anomaly (i.e., embryonal form) including splenic malformation, interrupted inferior vena cava, midline liver, situs inversus, preduodenal portal vein, and intestinal malrotation.
- More common form is the perinatal form that is not associated with malformations.
Etiology is not completely defined, but many different pathogenic mechanisms have been proposed, including the following:
- Perinatal infection of the liver and biliary tract with potential organisms including cytomegalovirus, rotavirus, and reovirus
- Immune dysregulation
- Defective morphogenesis
- Environmental toxin exposure
- Vascular insufficiency
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