Learn About Biliary Atresia

What is the definition of Biliary Atresia?

Biliary atresia is a blockage in the tubes (ducts) that carry a liquid called bile from the liver to the gallbladder.

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What are the alternative names for Biliary Atresia?

Jaundice newborns - biliary atresia; Newborn jaundice - biliary atresia; Extrahepatic ductopenia; Progressive obliterative cholangiopathy

What are the causes of Biliary Atresia?

Biliary atresia occurs when the bile ducts inside or outside the liver are abnormally narrow, blocked, or absent. The bile ducts carry a digestive fluid from liver to small bowel to break down fats and to filter out waste from the body.

The cause of the disease is not clear. It may be due to:

  • Viral infection after birth
  • Exposure to toxic substances
  • Multiple genetic factors
  • Perinatal injury
  • Some medicines such as carbamazepine

It more commonly affects people of East Asian and African-American descent.

The bile ducts help remove waste from the liver and carry salts that help the small intestine break down (digest) fat.

In babies with biliary atresia, bile flow from the liver to the gallbladder is blocked. This can lead to liver damage and cirrhosis of the liver, which can be deadly.

What are the symptoms of Biliary Atresia?

Symptoms usually start to occur between 2 to 8 weeks. Jaundice (a yellow color to the skin and mucus membranes) develops slowly 2 to 3 weeks after birth. The infant may gain weight normally for the first month. After that point, the baby will lose weight and become irritable, and will have worsening jaundice.

Other symptoms may include:

  • Dark urine
  • Swollen belly
  • Foul-smelling and floating stools
  • Pale or clay-colored stools
  • Slow growth
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What are the current treatments for Biliary Atresia?

An operation called the Kasai procedure is done to connect the liver to the small intestine. The abnormal ducts are bypassed. The surgery is more successful if done before the baby is 8 weeks old.

Liver transplant may still be needed before 20 years of age in most of the cases.

Who are the top Biliary Atresia Local Doctors?
Elite
Highly rated in
10
conditions
Pediatric Gastroenterology
Pediatrics
Gastroenterology

Cincinnati Childrens Hospital Medical Center

Burnet Campus

3333 Burnet Ave 
Cincinnati, OH 45229

Jorge Bezerra is a Pediatric Gastroenterologist and a Pediatrics doctor in Cincinnati, Ohio. Dr. Bezerra has been practicing medicine for over 38 years and is rated as an Elite doctor by MediFind in the treatment of Biliary Atresia. He is also highly rated in 10 other conditions, according to our data. His top areas of expertise are Biliary Atresia, Cholestasis, Bile Duct Obstruction, and High Blood Pressure in Infants. He is licensed to treat patients in Ohio and Kentucky. Dr. Bezerra is currently accepting new patients.

Elite
Highly rated in
9
conditions
General Surgery
Transplant Surgery

Michigan Medicine

U-M Kidney Transplant Clinic

4401 Campus Ridge Dr 
Midland, MI 48640

John Magee is a General Surgeon and a Transplant Surgeon in Midland, Michigan. Dr. Magee has been practicing medicine for over 34 years and is rated as an Elite doctor by MediFind in the treatment of Biliary Atresia. He is also highly rated in 9 other conditions, according to our data. His top areas of expertise are Biliary Atresia, Alagille Syndrome, Cholestasis, and Liver Transplant. He is licensed to treat patients in Michigan. Dr. Magee is currently accepting new patients.

 
 
 
 
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Elite
Highly rated in
3
conditions
Pediatrics
Pediatric Surgery

4650 Sunset Boulevard Office

4650 Sunset Blvd 
Los Angeles, CA 90027

Kasper Wang is a Pediatrics specialist and a Pediatric Surgeon in Los Angeles, California. Dr. Wang has been practicing medicine for over 28 years and is rated as an Elite doctor by MediFind in the treatment of Biliary Atresia. He is also highly rated in 3 other conditions, according to our data. His top areas of expertise are Biliary Atresia, High Blood Pressure in Infants, Atresia of Small Intestine, and Liver Failure. He is licensed to treat patients in California. Dr. Wang is currently accepting new patients.

What is the outlook (prognosis) for Biliary Atresia?

Early surgery will improve the survival of more than one third of babies with this condition. The long-term benefit of a liver transplant is not yet known, but it is expected to improve survival.

What are the possible complications of Biliary Atresia?

Complications may include:

  • Infection
  • Irreversible cirrhosis
  • Liver failure
  • Surgical complications, including failure of the Kasai procedure
When should I contact a medical professional for Biliary Atresia?

Call your provider if your child appears jaundiced, or if other symptoms of biliary atresia develop.

Bile produced in the liver
What are the latest Biliary Atresia Clinical Trials?
Evaluation Safety and Efficacy of Umbilical Cord Derived Mesenchymal Stem Cell (UC -MSC) Transplantation for Children Suffering From Liver Cirrhosis Due to Biliary Atresia: A Matched Control Prospective Study
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Peri Kasai Portoenterostomy Anchoring of the Jejunal Loop
What are the Latest Advances for Biliary Atresia?
Outcomes of Split Liver Transplantation vs Living Donor Liver Transplantation in Pediatric Patients: A 5-Year Follow-Up Study in Korea.
Autologous bone marrow mononuclear cell infusion for liver cirrhosis after the Kasai operation in children with biliary atresia.
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Biliary atresia combined Wilson disease identified by whole exome sequencing in Vietnamese patient with severe liver failure.
Who are the sources who wrote this article ?

Published Date : February 24, 2022
Published By : Neil K. Kaneshiro, MD, MHA, Clinical Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Berlin SC. Diagnostic imaging of the neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 38.

Cazares J, Ure B, Yamataka A. Biliary atresia. In: Holcomb GW, Murphy JP, St. Peter SD, eds. Holcomb and Ashcraft's Pediatric Surgery. 7th ed. Philadelphia, PA: Elsevier; 2020:chap 43.

Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC. Cholestasis. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 383.

O'Hara SM. The pediatric liver and spleen. In: Rumack CM, Levine D, eds. Diagnostic Ultrasound. 5th ed. Philadelphia, PA: Elsevier; 2018:chap 51.