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| {{Infobox_Disease |
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| Name = {{PAGENAME}} |
| | {{Biliary atresia}} |
| Image = |
| | '''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 = |
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| eMedicineTopic = |
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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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| {{CMG}} '''Assosciate Editor(s)-In-Chief:''' [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]
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| {{SK}} Extrahepatic ductopenia, Progressive obliterative cholangiopathy | | {{CMG}} '''Assosciate Editor(s)-In-Chief:''' {{skhan}} |
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| '''For patient information, click [[Biliary atresia (patient informattion)|here]]'''
| | {{SK}} Extrahepatic ductopenia; progressive obliterative cholangiopathy; atresia of bile ducts |
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| ==Overview== | | ==[[Biliary atresia overview|Overview]]== |
| '''Biliary atresia''', is a congenital or acquired disease of the liver and one of the principal forms of chronic rejection of a transplanted liver allograft.
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| In the congenital form, the common [[bile duct]] between the [[liver]] and the [[small intestine]] is blocked or absent.
| | ==[[Biliary atresia classification|Classification]]== |
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| The acquired type most often occurs in the setting of autoimmune disease, and is one of the principal forms of chronic rejection of a transplanted liver allograft.
| | ==[[Biliary atresia pathophysiology|Pathophysiology]]== |
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| ==Classification== | | ==[[Biliary atresia causes|Causes]]== |
| There are three main types of extrahepatic biliary atresia:-
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| *Type I: atresia restricted to the common bile duct.
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| *Type II: atresia of the common hepatic duct.
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| *Type III: atresia of the right and left hepatic duct.
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| ==Pathophysiology==
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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.
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| There have been many theories about etiopathogenesis such as Reovirus 3 infection, congenital malformation, congenital CMV infection, autoimmune theory. This means that the etiology and pathogenesis of biliary atresia are largely unknown. However, there have been extensive studies about the pathogenesis and proper management of progressive liver fibrosis, which is arguably one of the most important aspects of biliary atresia patients. 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. Proliferation of the small bile ductules occur, and peribiliary fibroblasts become activated. These "reactive" biliary epithelial cells in cholestasis, unlike normal condition, produce and secrete various cytokines such as [[CCL2|CCL-2 or MCP-1]], [[Tumor necrosis factor| Tumor necrosis factor (TNF)]], [[Interleukin-6| Interleukin-6 (IL-6)]], [[Transforming growth factor-beta|TGF-beta]], [[Endothelin| Endothelin (ET)]], and [[Nitric oxide| nitric oxide (NO)]]. Among these, TGF-beta is the most important profibrogenic cytokine that can be seen in liver fibrosis in chronic cholestasis. During the chronic activation of biliary epithelium and progressive fibrosis, afflicted patients eventually show signs and symptoms of portal hypertension (esophagogastric varix bleeding, hypersplenism, hepatorenal syndrome(HRS), hepatopulmonary syndrome(HPS)). The latter two syndromes are essentially caused by systemic mediators that maintain the body within the hyperdynamic states.
| | ==[[Biliary atresia differential diagnosis|Differentiating Biliary Atresia from other Diseases]]== |
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| Associated anomalies include, in about 20% cases,
| | ==[[Biliary atresia epidemiology and demographics|Epidemiology and Demographics]]== |
| *Cardiac lesions
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| *[[Polysplenia]]
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| *[[Situs inversus]]
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| *Absent [[vena cava]]
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| *A preduodenal [[portal vein]]
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| ==Causes== | | ==[[Biliary atresia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| There is no known cause of biliary atresia.
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| ==Epidemiology and Demographics==
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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. | |
| ==Natural history, Complications and Prognosis==
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| ===Complications===
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| 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.
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| ===Prognosis===
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| Recent large volume studies from Davenport et al. (Ann Surg, 2008) show that age of the patient is not an absolute clinical factor affecting the prognosis. In the latter study, influence of age differs according to the disease etiology—i.e., whether isolated BA, BASM (BA with splenic malformation ), or CBA(cystic biliary atresia).
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| ==Diagnosis== | | ==Diagnosis== |
| Initially, the symptoms are indistinguishable from [[Jaundice#Neonatal jaundice|neonatal jaundice]], a common phenomenon.
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| Symptoms are usually evident between two and six weeks after birth.
| | [[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]] |
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| Infants and children with biliary atresia have progressive cholestasis with all the usual concomitant features:
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| *[[Jaundice]]
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| *Pale stools
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| *Dark urine
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| *[[Pruritus]]
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| *Malabsorption with growth retardation
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| *Fat-soluble vitamin deficiencies
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| *[[Hyperlipidemia]]
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| *Eventually [[cirrhosis]] with [[portal hypertension]].
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| *Swollen abdominal region[[(Ascites)]] and
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| *Large hardened liver (which may or may not be observable by the naked eye).
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| Prolonged jaundice that is resistant to phototherapy and/or exchange [[blood transfusion|transfusions]] should prompt a search for secondary causes.
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| 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]].
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| [[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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| ==Treatment== | | ==Treatment== |
| The only effective treatments are certain surgeries, or liver transplantation.
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| 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. Timely Kasai portoenterostomy (e.g. < 60 postnatal days) has shown better outcomes. Nevertheless, a considerable number of the patients, even if Kasai portoenterostomy has been successful, eventually undergo liver transplantation within a couple of years after Kasai portoenterostomy.
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| It is widely accepted that corticosteroid treatment after a Kasai operation, with or without choleretics and antibiotics, has a beneficial effect on the postoperative bile flow and can clear the jaundice; but the dosing and duration of the ideal steroid protocol have been controversial ("blast dose" vs. "high dose" vs. "low dose"). Furthermore, it has been observed in many retrospective longitudinal studies that steroid does not prolong survival of the native liver or transplant-free survival. Davenport at al. also showed (hepatology 2007) that short-term low-dose steroid therapy following a Kasai operation has no effect on the mid- and long-term prognosis of biliary atresia patients.
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| ==References==
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| {{Reflist|2}}
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| ==Research links==
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| *[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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| ==Support groups==
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| *[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:Rare diseases]] | | [[Category:Rare diseases]] |
| [[Category:Neonatology]] | | [[Category:Neonatology]] |
| [[Category:Overview complete]]
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| [[Category:Mature chapter]]
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| [[Category:Disease]] | | [[Category:Disease]] |
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