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| {{Infobox_Disease |
| | __NOTOC__ |
| Name = {{PAGENAME}} |
| | {{Biliary atresia}} |
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| | '''For patient information, click [[Biliary atresia (patient information)|here]]''' |
| Caption = |
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| DiseasesDB = 1400 |
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| ICD10 = {{ICD10|Q|44|2|q|38}} |
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| ICD9 = {{ICD9|751.61}} |
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| ICDO = |
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| OMIM = 210500 |
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| MedlinePlus = |
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| eMedicineSubj = ped |
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| eMedicineTopic = 237 |
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| MeshName = Biliary+Atresia |
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| MeshNumber = C06.130.120.123 |
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| }}
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| {{SI}}
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| {{EH}} | | {{CMG}} '''Assosciate Editor(s)-In-Chief:''' {{skhan}} |
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| ==Overview==
| | {{SK}} Extrahepatic ductopenia; progressive obliterative cholangiopathy; atresia of bile ducts |
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| '''Biliary atresia''' is a [[rare disease|rare condition]] in [[neonate|newborn infants]] in which the common [[bile duct]] between the [[liver]] and the [[small intestine]] is blocked or absent. If unrecognised, the condition leads to [[liver failure]] but not (as one might think) to [[kernicterus]]. This is because the liver is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood-brain barrier. The cause of the condition is unknown. The only effective treatments are certain surgeries, or liver transplantation.
| | ==[[Biliary atresia overview|Overview]]== |
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| ==Symptoms and diagnosis== | | ==[[Biliary atresia classification|Classification]]== |
| Initially, the symptoms are indistinguishable from [[Jaundice#Neonatal jaundice|neonatal jaundice]], a common phenomenon. Symptoms are usually evident between two and six weeks after birth. Besides jaundice, other symptoms include pale stools, dark urine, swollen abdominal region and large hardened liver (which may or may not be observable by the naked eye). Prolonged jaundice that is resistant to phototherapy and/or exchange [[blood transfusion|transfusions]] should prompt a search for secondary causes. By this time, [[liver enzyme]]s are generally measured, and these tend to be grossly deranged, [[hyperbilirubinaemia]] is conjugated and therefore does not lead to [[kernicterus]]. [[Medical ultrasonography|Ultrasound]] investigation or other forms of imaging can confirm the diagnosis. Further testing include radioactive scans of the liver and a liver biopsy.
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| Biliary atresia is a very rare disorder. About one in 10,000 to 20,000 babies in the U.S are affected every year. Biliary atresia seems to affect girls slightly more often than boys. Within the same family, it is common for only one child in a pair of twins or only one child within the same family to have it. Asians and African-Americans are affected more frequently than Caucasians. There does not appear to be any link to medications or immunizations given immediately before or during pregnancy. | | ==[[Biliary atresia pathophysiology|Pathophysiology]]== |
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| ==Pathophysiology== | | ==[[Biliary atresia causes|Causes]]== |
| There is no known cause of biliary atresia.
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| As the biliary tract cannot transport bile to the intestine, [[bile]] is retained in the liver (known as stasis) and results in cirrhosis of the liver.
| | ==[[Biliary atresia differential diagnosis|Differentiating Biliary Atresia from other Diseases]]== |
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| ==Treatment== | | ==[[Biliary atresia epidemiology and demographics|Epidemiology and Demographics]]== |
| If the intrahepatic biliary tree is unaffected, surgical reconstruction of the extrahepatic biliary tract is possible. This surgery is called a Kasai procedure (after the Japanese surgeon who developed the surgery, Dr. Morio Kasai) or [[hepatoportoenterostomy]].
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| If the atresia is complete, [[liver transplantation]] is the only option.
| | ==[[Biliary atresia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==External links== | | ==Diagnosis== |
| *[http://www.classkids.org/library/bloodtest.htm Intro. to pediatric blood tests for liver function]
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| *[http://www.cincinnatichildrens.org/svc/alpha/l/liver/diseases/biliary.htm Biliary Atresia, Liver Diseases and Treatments, Cincinnati Children's Hospital Medical Center]
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| *[http://www.barcnetwork.org/index.html Biliary Atresia Research Consortium (U.S.)]
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| *[http://www.childliverdisease.org/ Children's Liver Disease Foundation (U.K.)]
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| *[http://www.biliary-atresia.com/ European Biliary Atresia Registry]
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| *[http://www.biliaryatresia.blogspot.com/ Imagine Bright Futures - Musings and research on biliary atresia (the leading cause of pediatric liver transplants).]
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| *[http://www.liverfoundation.org/cgi-bin/dbs/pressrelease.cgi?db=pressrelease&uid=default&ID=15&view_records=1 Fund for the Cure - Biliary Atresia] from the Biliary Atresia Research Consortium and the American Liver Foundation
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| ==Research links==
| | [[Biliary atresia history and symptoms|History and Symptoms]] | [[Biliary atresia physical examination|Physical Examination]] | [[Biliary atresia laboratory findings|Laboratory Findings]] | [[Biliary atresia CT|CT]] | [[Biliary atresia MRI|MRI]] | [[Biliary atresia echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Biliary atresia other imaging findings|Other Imaging Findings]] | [[Biliary atresia other diagnostic studies|Other Diagnostic Studies]] |
| *[http://www.jiaps.com/article.asp?issn=0971-9261;year=2005;volume=10;issue=1;spage=48;epage=49;aulast=Kumar Choledochal cyst associated with extrahepatic bile duct atresia]
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| *[http://www.cincinnatichildrens.org/research/about/ann-report/2003/profile2003/gastro.htm Understanding How Inflammation Causes Biliary Atresia, Jorge Bezerra, MD, Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center]
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| ==Support groups== | | ==Treatment== |
| *[http://www.liverfamilies.org/ LIVER FAMILIES: An international online support group for families whose lives have been touched by pediatric liver disease and transplant.]
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| *[http://www.childliverdisease.org/ Children's Liver Disease Foundation: An organisation dedicated to fighting childhood liver disease by supporting affected families, funding research and helping to educate healthcare professionals and the public.]
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| *[http://www.classkids.org Children's Liver Association for Support Services, C.L.A.S.S: An all-volunteer, nonprofit organization dedicated to serving the emotional, educational and financial needs of families coping with pediatric liver disease and transplantation. Their goal is "to be both a service to families and a valuable resource for the medical community."]
| | [[Biliary atresia medical therapy|Medical Therapy]] | [[Biliary atresia surgery|Surgery]] | [[Biliary atresia prevention|Prevention]] | [[Biliary atresia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Biliary atresia future or investigational therapies|Future or Investigational Therapies]] |
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| {{SIB}}
| | ==Case Studies== |
| | [[Biliary atresia case study one|Case #1]] |
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| [[Category:Gastroenterology]] | | [[Category:Gastroenterology]] |
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| [[Category:Congenital disorders]] | | [[Category:Congenital disorders]] |
| [[Category:Rare diseases]] | | [[Category:Rare diseases]] |
| | [[Category:Neonatology]] |
| | [[Category:Disease]] |
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| [[de:Gallengangatresie]]
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| [[fr:Atrésie biliaire]] | | [[fr:Atrésie biliaire]] |
| [[pl:Atrezja dróg żółciowych]] | | [[pl:Atrezja dróg żółciowych]] |