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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Cabana, Michael D., editor. "Biliary Atresia." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617698/all/Biliary_Atresia.
Biliary Atresia. In: Cabana MDM, ed. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617698/all/Biliary_Atresia. Accessed June 3, 2023.
Biliary Atresia. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult (8th ed.). Wolters Kluwer. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617698/all/Biliary_Atresia
Biliary Atresia [Internet]. In: Cabana MDM, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2023 June 03]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617698/all/Biliary_Atresia.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Biliary Atresia ID - 617698 ED - Cabana,Michael D, BT - 5-Minute Pediatric Consult UR - https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617698/all/Biliary_Atresia PB - Wolters Kluwer ET - 8 DB - Pediatrics Central DP - Unbound Medicine ER -