Also known as: BA, extrahepatic ductopenia, progressive obliterative cholangiopathy
What is biliary atresia?
The biliary system refers to the ducts and channels that allow the fluid produced in the liver ( bile ) to drain into the intestines. Bile helps with the digestion of food ( and carries waste products from the liver to the intestines for removal ). In biliary atresia the bile duct system is blocked. When the bile can't leave the liver, it causes liver damage which can affect many other parts of the body.
What causes biliary atresia?
Biliary atresia is quite rare and seems to affect girls more often than boys. The causes of biliary atresia are not exactly clear. In some children the ducts don't form normally while the baby is in the womb.In others it may be related to a viral infection that occurs soon after the baby is born. Some infants with biliary atresia are also more likely to have other birth defects, such as problems with the heart, spleen, intestines or blood vessels.
What are the symptoms of biliary atresia?
Most babies with biliary atresia appear normal after birth ( some may be jaundiced- yellow eyes and skin- as newborns ), with symptoms of jaundice developing 2-8 weeks later. In addition, infants with biliary atresia may have dark urine, clay-colored stools, irritability, and weight loss.
What are biliary atresia care options?
A surgical procedure known as a Kasai procedure is often effective at treating biliary atresia. It involves redirecting the flow of bile around the blocked portions of the ducts. In cases where the procedure fails, a liver transplant may be necessary. Nutritional support to improve nutrition is important.
Reviewed by: Jack Wolfsdorf
This page was last updated on: 4/17/2017 3:42:17 PM
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Dr. Muñiz-Crim is employed by Pediatric Specialists of America (PSA), the physician-led group practice of Miami Children's Health System, and is the PSA Section Chief for Pediatric Gastroenterology.
Dr. Koyfman is employed by Pediatric Specialists of America (PSA), the physician-led group practice of Miami Children's Health System, and sees patients at Nicklaus Children's Hospital in Miami and the Nicklaus Children's West Kendall Outpatient Center.