Biliary Atresia

Common Name(s)

Biliary Atresia

Biliary Atresia is a digestive disorder characterized by the incomplete development of the bile duct system. The bile duct system is responsible for storing and transporting bile from the liver, where it is made, to the intestines, where it helps to digest food. Incomplete development of the ducts inhibits the flow of bile. Individuals with this defect have digestion problems as well as a yellow discoloration of the eye and skin (jaundice). Doctors diagnose this defect early in life, as the resulting symptoms are quickly apparent. Treatment is developed on a case-by-case basis and depends on the specific needs of the patient. Digestion specialists may suggest individuals eat a lower-fat diet, or undergo surgery to relieve the blockage or create a tube to allow bile to reach the digestive system.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Biliary Atresia" for support, advocacy or research.

Association of Gastrointestinal Motility Disorders, Inc. (AGMD)

AGMD is a nonprofit international organization which serves as an integral educational resource concerning digestive motility diseases and disorders. It also functions as an important information base for members of the medical and scientific communities. In addition, it provides a forum for patients suffering from digestive motility diseases and disorders as well as their families and members of the medical community.

Last Updated: 28 Feb 2015

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Biliary Atresia" for support, advocacy or research.

Association of Gastrointestinal Motility Disorders, Inc. (AGMD)

AGMD is a nonprofit international organization which serves as an integral educational resource concerning digestive motility diseases and disorders. It also functions as an important information base for members of the medical and scientific communities. In addition, it provides a forum for patients suffering from digestive motility diseases and disorders as well as their families and members of the medical community.

http://www.agmd-gimotility.org

Last Updated: 28 Feb 2015

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Biliary Atresia" returned 237 free, full-text research articles on human participants. First 3 results:

Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial.
 

Author(s): Jorge A Bezerra, Cathie Spino, John C Magee, Benjamin L Shneider, Philip Rosenthal, Kasper S Wang, Jessi Erlichman, Barbara Haber, Paula M Hertel, Saul J Karpen, Nanda Kerkar, Kathleen M Loomes, Jean P Molleston, Karen F Murray, Rene Romero, Kathleen B Schwarz, Ross Shepherd, Frederick J Suchy, Yumirle P Turmelle, Peter F Whitington, Jeffrey Moore, Averell H Sherker, Patricia R Robuck, Ronald J Sokol,

Journal: JAMA. 2014 May;311(17):1750-9.

 

Biliary atresia is the most common cause of end-stage liver disease in children. Controversy exists as to whether use of steroids after hepatoportoenterostomy improves clinical outcome.

Last Updated: 5 May 2014

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Biliary atresia: evaluation on two distinct periods at a reference pediatric service.
 

Author(s): Thais Costa Nascentes Queiroz, Alexandre Rodrigues Ferreira, Eleonora Druve Tavares Fagundes, Mariza Leitão Valadares Roquete, Francisco José Penna

Journal: Arq Gastroenterol. ;51(1):53-8.

 

Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period.

Last Updated: 24 Apr 2014

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Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia.
 

Author(s): Tae Jun Park, Keun Soo Ahn, Yong Hoon Kim, Hyungseop Kim, Ui Jun Park, Hyoung Tae Kim, Won Hyun Cho, Woo-Hyun Park, Koo Jeong Kang

Journal: Clin Mol Hepatol. 2014 Mar;20(1):76-80.

 

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity ...

Last Updated: 23 Apr 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Biliary Atresia" returned 26 free, full-text review articles on human participants. First 3 results:

Maternal microchimerism in biliary atresia: are maternal cells effector cells, targets, or just bystanders?
 

Author(s): Toshihiro Muraji

Journal: Chimerism. ;5(1):1-5.

 

The etiology of biliary atresia (BA) is unknown; however, the liver histology is similar to that observed in immune-mediated hepatic disorders. Liver fibrosis in BA progresses even after bile drainage has been achieved by the Kasai operation. Maternal microchimerism has been purported ...

Last Updated: 18 Apr 2014

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Aetiology of biliary atresia: what is actually known?
 

Author(s): Claus Petersen, Mark Davenport

Journal:

 

Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic ...

Last Updated: 15 Nov 2013

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The long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review.
 

Author(s): E J Bijl, K D Bharwani, R H J Houwen, R A de Man

Journal: Neth J Med. 2013 May;71(4):170-3.

 

Biliary atresia (BA) is a progressive inflammatory destructive process of the bile ducts occurring in about one of every 20,000 live births. If left untreated, biliary atresia can lead to liver failure. The only effective treatments for BA at the moment are the Kasai operation and ...

Last Updated: 31 May 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Biliary Atresia Study in Infants and Children
 

Status: Recruiting

Condition Summary: Biliary Atresia

 

Last Updated: 15 Sep 2014

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Pentoxifylline Therapy in Biliary Atresia
 

Status: Recruiting

Condition Summary: Biliary Atresia

 

Last Updated: 5 Feb 2015

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Evaluation of Immune Function in Biliary Atresia Children With Prolonged Jaundice
 

Status: Recruiting

Condition Summary: Biliary Atresia

 

Last Updated: 9 Sep 2005

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