biliary atresia

Summary

Summary: Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE.

Top Publications

  1. Mohanty S, Donnelly B, Bondoc A, Jafri M, Walther A, Coots A, et al. Rotavirus replication in the cholangiocyte mediates the temporal dependence of murine biliary atresia. PLoS ONE. 2013;8:e69069 pubmed publisher
    b>Biliary atresia (BA) is a neonatal disease that results in obliteration of the biliary tree...
  2. Shimadera S, Iwai N, Deguchi E, Kimura O, Ono S, Furukawa T, et al. Predicting factors on the occurrence of cystic dilatation of intrahepatic biliary system in biliary atresia. Pediatr Surg Int. 2010;26:611-4 pubmed publisher
    Cystic dilatation of intrahepatic biliary system (CDIB) is an intractable complication of biliary atresia (BA)...
  3. Grizelj R, Vukovic J, Novak M, Batinica S. Biliary atresia: the Croatian experience 1992-2006. Eur J Pediatr. 2010;169:1529-34 pubmed publisher
    The objective of this study was to determine the outcomes of Croatian children with biliary atresia. Health records of infants born in Croatia between January 1, 1992 and December 31, 2006 who were diagnosed with biliary atresia and ..
  4. Chitsaz E, Schreiber R, Collet J, Kaczorowski J. Biliary atresia: the timing needs a changin'. Can J Public Health. 2009;100:475-7 pubmed
    b>Biliary atresia (BA), a uniquely pediatric liver disease, is the leading cause of liver-related death in children and the most frequent indication for liver transplantation in the pediatric population...
  5. Hadzic N, Quaglia A, Portmann B, Paramalingam S, Heaton N, Rela M, et al. Hepatocellular carcinoma in biliary atresia: King's College Hospital experience. J Pediatr. 2011;159:617-22.e1 pubmed publisher
    To establish risks for development of hepatocellular carcinoma (HCC) in children with biliary atresia (BA), the most common chronic liver disease of childhood.
  6. Brindley S, Lanham A, Karrer F, Tucker R, Fontenot A, Mack C. Cytomegalovirus-specific T-cell reactivity in biliary atresia at the time of diagnosis is associated with deficits in regulatory T cells. Hepatology. 2012;55:1130-8 pubmed publisher
    b>Biliary atresia (BA) is a progressive, inflammatory cholangiopathy that culminates in fibrosis of extrahepatic and intrahepatic bile ducts...
  7. Harpavat S, Finegold M, Karpen S. Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics. 2011;128:e1428-33 pubmed publisher
    Healthy infants are thought to acquire biliary atresia (BA) in the first weeks of life. Because those diagnosed earlier have better outcomes, we were interested in determining the earliest time BA could be detected...
  8. Song Z, Dong R, Fan Y, Zheng S. Identification of serum protein biomarkers in biliary atresia by mass spectrometry and enzyme-linked immunosorbent assay. J Pediatr Gastroenterol Nutr. 2012;55:370-5 pubmed
    There is an urgent need to identify noninvasive and convenient biomarkers for early diagnosis of biliary atresia (BA). The aim of the present study was to identify potential protein biomarkers for BA.
  9. Yang H, Plosch T, Lisman T, Gouw A, Porte R, Verkade H, et al. Inflammation mediated down-regulation of hepatobiliary transporters contributes to intrahepatic cholestasis and liver damage in murine biliary atresia. Pediatr Res. 2009;66:380-5 pubmed publisher
    To investigate the hypothesis that during the development of biliary atresia, early changes in hepatobiliary transport are mainly related to the inflammatory process and lead to intrahepatic cholestasis and subsequent liver injury, ..

More Information

Publications62

  1. Jimenez Rivera C, Jolin Dahel K, Fortinsky K, Gozdyra P, Benchimol E. International incidence and outcomes of biliary atresia. J Pediatr Gastroenterol Nutr. 2013;56:344-54 pubmed publisher
    International trends in incidence and outcomes of biliary atresia (BA) are controversial and a wide range of estimates have been reported worldwide...
  2. Mack C, Feldman A, Sokol R. Clues to the etiology of bile duct injury in biliary atresia. Semin Liver Dis. 2012;32:307-16 pubmed publisher
    b>Biliary atresia (BA) is an infantile obstructive cholangiopathy of unknown etiology with suboptimal therapy, which is responsible for 40 to 50% of all pediatric liver transplants...
  3. Shinkai M, Ohhama Y, Take H, Kitagawa N, Kudo H, Mochizuki K, et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital. J Pediatr Gastroenterol Nutr. 2009;48:443-50 pubmed
    ..and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA)...
  4. Davenport M, Ong E, Sharif K, Alizai N, McClean P, Hadzic N, et al. Biliary atresia in England and Wales: results of centralization and new benchmark. J Pediatr Surg. 2011;46:1689-94 pubmed publisher
    b>Biliary atresia (BA) is a rare, potentially life-threatening condition of the newborn presenting with conjugated jaundice...
  5. Chongsrisawat V, Vejapipat P, Siripon N, Poovorawan Y. Transient elastography for predicting esophageal/gastric varices in children with biliary atresia. BMC Gastroenterol. 2011;11:41 pubmed publisher
    ..The purpose of this study has been to explore the accuracy of TE and clinical parameters in predicting the presence of esophageal/gastric varices in children with biliary atresia (BA) following portoenterostomy.
  6. Tucker R, Feldman A, Fenner E, Mack C. Regulatory T cells inhibit Th1 cell-mediated bile duct injury in murine biliary atresia. J Hepatol. 2013;59:790-6 pubmed publisher
    b>Biliary atresia (BA) is a pediatric inflammatory disease of the biliary system which leads to cirrhosis and the need for liver transplantation...
  7. Omenetti A, Bass L, Anders R, Clemente M, Francis H, Guy C, et al. Hedgehog activity, epithelial-mesenchymal transitions, and biliary dysmorphogenesis in biliary atresia. Hepatology. 2011;53:1246-58 pubmed publisher
    b>Biliary atresia (BA) is notable for marked ductular reaction and rapid development of fibrosis...
  8. Huang L, Si X, Feng J. NF-kappaB related abnormal hyper-expression of iNOS and NO correlates with the inflammation procedure in biliary atresia livers. Pediatr Surg Int. 2010;26:899-905 pubmed publisher
    To study the expression of inducible nitric oxide synthase (iNOS) and its related regulators in biliary atresia (BA) livers and exlpore their relationships with the inflammation pathway in BA livers.
  9. Duché M, Ducot B, Ackermann O, Baujard C, Chevret L, Frank Soltysiak M, et al. Experience with endoscopic management of high-risk gastroesophageal varices, with and without bleeding, in children with biliary atresia. Gastroenterology. 2013;145:801-7 pubmed publisher
    b>Biliary atresia, the most common cause of childhood cirrhosis, increases the risks for portal hypertension and gastrointestinal bleeding...
  10. Duché M, Ducot B, Tournay E, Fabre M, Cohen J, Jacquemin E, et al. Prognostic value of endoscopy in children with biliary atresia at risk for early development of varices and bleeding. Gastroenterology. 2010;139:1952-60 pubmed publisher
    b>Biliary atresia is the most common cause of childhood cirrhosis...
  11. Honsawek S, Chaiwatanarat T, Vejchapipat P, Chongsrisawat V, Thawornsuk N, Poovorawan Y. Relationships between OPG, RANKL, bone metabolism, and bone mineral density in biliary atresia. Pediatr Surg Int. 2009;25:261-7 pubmed publisher
    ..However, the relationship between the OPG-RANKL system and bone status in biliary atresia (BA) has not, as yet, been clarified...
  12. Harada K, Nakanuma Y. Biliary innate immunity in the pathogenesis of biliary diseases. Inflamm Allergy Drug Targets. 2010;9:83-90 pubmed
    ..thought to be involved in the pathogenesis of cholangiopathies in case of primary biliary cirrhosis (PBC) and biliary atresia. Biliary epithelial cells possess the Toll-like receptor (TLR) family which recognizes pathogen-associated ..
  13. Serinet M, Wildhaber B, Broue P, Lachaux A, Sarles J, Jacquemin E, et al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics. 2009;123:1280-6 pubmed publisher
    Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists and if this effect persists with extended follow-up...
  14. Shanmugam N, Harrison P, Devlin J, Peddu P, Knisely A, Davenport M, et al. Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 days. J Pediatr Gastroenterol Nutr. 2009;49:435-41 pubmed publisher
    We investigated the role and safety of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing biliary atresia (BA) in prolonged neonatal cholestasis, when standard workup was inconclusive.
  15. Livesey E, Cortina Borja M, Sharif K, Alizai N, McClean P, Kelly D, et al. Epidemiology of biliary atresia in England and Wales (1999-2006). Arch Dis Child Fetal Neonatal Ed. 2009;94:F451-5 pubmed publisher
    To identify the epidemiological characteristics of infants with biliary atresia in England and Wales, since centralisation of its management in 1999.
  16. Shima H, Igarashi G, Wakisaka M, Hamano S, Nagae H, Koyama M, et al. Noninvasive acoustic radiation force impulse (ARFI) elastography for assessing the severity of fibrosis in the post-operative patients with biliary atresia. Pediatr Surg Int. 2012;28:869-72 pubmed publisher
    ..The aim of this study was to determine whether ARFI elastography is a reliable method for predicting the severity of fibrosis in the post-operative patients with biliary atresia.
  17. Honsawek S, Chayanupatkul M, Chongsrisawat V, Theamboonlers A, Praianantathavorn K, Udomsinprasert W, et al. Serum adiponectin and transient elastography as non-invasive markers for postoperative biliary atresia. BMC Gastroenterol. 2011;11:16 pubmed publisher
    b>Biliary atresia (BA) is a progressive inflammatory disorder of the extrahepatic bile ducts leading to the obliteration of bile flow...
  18. Feldman A, Mack C. Biliary atresia: cellular dynamics and immune dysregulation. Semin Pediatr Surg. 2012;21:192-200 pubmed publisher
    The cause of biliary atresia is unknown; in the past few decades, the majority of investigations related to its pathogenesis have centered on viral infections and immunity...
  19. Zahm A, Hand N, Boateng L, Friedman J. Circulating microRNA is a biomarker of biliary atresia. J Pediatr Gastroenterol Nutr. 2012;55:366-9 pubmed
    The lack of reliable noninvasive diagnostic biomarkers of biliary atresia (BA) results in delayed diagnosis and worsened patient outcome...
  20. Hand N, Horner A, Master Z, Boateng L, Leguen C, Uvaydova M, et al. MicroRNA profiling identifies miR-29 as a regulator of disease-associated pathways in experimental biliary atresia. J Pediatr Gastroenterol Nutr. 2012;54:186-92 pubmed publisher
    b>Biliary atresia (BA) is a pediatric liver disease of unknown underlying etiology, in which fibroinflammatory destruction of the extrahepatic biliary system leads to obstructive cholestasis...
  21. Lee H, Chang T, Yeung C, Chan W, Jiang C, Chen W, et al. Association of interferon-gamma gene polymorphisms in Taiwanese children with biliary atresia. J Clin Immunol. 2010;30:68-73 pubmed publisher
    b>Biliary atresia (BA) is a devastating neonatal hepatobiliary disease characterized by bile duct inflammation and fibrosis...
  22. Feldman A, Tucker R, Fenner E, Pelanda R, Mack C. B cell deficient mice are protected from biliary obstruction in the rotavirus-induced mouse model of biliary atresia. PLoS ONE. 2013;8:e73644 pubmed publisher
    A leading theory regarding the pathogenesis of biliary atresia (BA) is that bile duct injury is initiated by a virus infection, followed by an autoimmune response targeting bile ducts...
  23. Uemura M, Ozawa A, Nagata T, Kurasawa K, Tsunekawa N, Nobuhisa I, et al. Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background mice. Development. 2013;140:639-48 pubmed publisher
    Congenital biliary atresia is an incurable disease of newborn infants, of unknown genetic causes, that results in congenital deformation of the gallbladder and biliary duct system...
  24. Zhou K, Lin N, Xiao Y, Wang Y, Wen J, Zou G, et al. Elevated bile acids in newborns with Biliary Atresia (BA). PLoS ONE. 2012;7:e49270 pubmed publisher
    b>Biliary Atresia (BA), a result from inflammatory destruction of the intrahepatic and extrahepatic bile ducts, is a severe hepatobiliary disorder unique to infancy...
  25. Udomsinprasert W, Tencomnao T, Honsawek S, Anomasiri W, Vejchapipat P, Chongsrisawat V, et al. +276 G/T single nucleotide polymorphism of the adiponectin gene is associated with the susceptibility to biliary atresia. World J Pediatr. 2012;8:328-34 pubmed publisher
    b>Biliary atresia (BA) is an intractable neonatal inflammatory and obliterative cholangiopathy, leading to progressive fibrosis and cirrhosis. Adiponectin, an anti-inflammatory adipokine, is known to play a possible role in liver diseases...
  26. Lages C, Simmons J, Chougnet C, Miethke A. Regulatory T cells control the CD8 adaptive immune response at the time of ductal obstruction in experimental biliary atresia. Hepatology. 2012;56:219-27 pubmed publisher
    ..are effector cells of cholangiocyte injury in human and in rhesus rotavirus (RRV)-induced experimental biliary atresia (BA)...
  27. Shivakumar P, Sabla G, Whitington P, Chougnet C, Bezerra J. Neonatal NK cells target the mouse duct epithelium via Nkg2d and drive tissue-specific injury in experimental biliary atresia. J Clin Invest. 2009;119:2281-90 pubmed
    b>Biliary atresia is a neonatal obstructive cholangiopathy that progresses to end-stage liver disease...
  28. Suzuki M, Muraji T, Obatake M, Nio M, Ito K, Suzuki K, et al. Urinary sulfated bile acid analysis for the early detection of biliary atresia in infants. Pediatr Int. 2011;53:497-500 pubmed publisher
    Measurement of urinary sulfated bile acid (USBA) is a non-invasive method to detect bile congestion. Our aim was to evaluate the feasibility of USBA analysis for the early detection of biliary atresia (BA).
  29. Jafri M, Donnelly B, Bondoc A, Allen S, Tiao G. Cholangiocyte secretion of chemokines in experimental biliary atresia. J Pediatr Surg. 2009;44:500-7 pubmed publisher
    b>Biliary atresia (BA) is a disease of the newborn that results in obstruction of the biliary tree...
  30. Chou M, Chuang J, Eng H, Chen C, Wang C, Chen C, et al. Endotoxin and CD14 in the progression of biliary atresia. J Transl Med. 2010;8:138 pubmed publisher
    b>Biliary atresia (BA) is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/or extra-hepatic bile ducts. However, the mechanisms contributing to the pathogenesis of BA remain uncertain...
  31. Khalil B, Perera M, Mirza D. Clinical practice: management of biliary atresia. Eur J Pediatr. 2010;169:395-402 pubmed publisher
    b>Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown...
  32. Guo C, Zhu J, Pu C, Deng Y, Zhang M. Combinatory effects of hepatic CD8+ and NK lymphocytes in bile duct injury from biliary atresia. Pediatr Res. 2012;71:638-44 pubmed publisher
    ..We investigated the role of T lymphocytes and NK lymphocytes in the destruction of extrahepatic bile ducts of patients with biliary atresia.
  33. Harada K, Sato Y, Ikeda H, Isse K, Ozaki S, Enomae M, et al. Epithelial-mesenchymal transition induced by biliary innate immunity contributes to the sclerosing cholangiopathy of biliary atresia. J Pathol. 2009;217:654-64 pubmed publisher
    ..RNA (dsRNA) genome and the biliary innate immune response to dsRNA are implicated in the aetiopathogenesis of biliary atresia (BA)...
  34. Sanada Y, Aida J, Kawano Y, Nakamura K, Shimomura N, Ishikawa N, et al. Hepatocellular telomere length in biliary atresia measured by Q-FISH. World J Surg. 2012;36:908-16 pubmed publisher
    Liver transplantation for biliary atresia is indicated whenever a Kasai portoenterostomy is considered unfeasible. However, the timing of liver transplantation in biliary atresia has not been precisely defined...
  35. Hertel P, Crawford S, Finegold M, Estes M. Osteopontin upregulation in rotavirus-induced murine biliary atresia requires replicating virus but is not necessary for development of biliary atresia. Virology. 2011;417:281-92 pubmed publisher
    b>Biliary atresia (BA) is a progressive fibro-inflammatory pediatric liver disease in which osteopontin (OPN), a glycoprotein with inflammatory and fibrogenic activity, may play a pathogenic role...
  36. Saito T, Hishiki T, Terui K, Mitsunaga T, Terui E, Nakata M, et al. Toll-like receptor mRNA expression in liver tissue from patients with biliary atresia. J Pediatr Gastroenterol Nutr. 2011;53:620-6 pubmed publisher
    ..Toll-like receptor (TLR) cascades may trigger progressive inflammatory biliary destruction that manifests as biliary atresia (BA) in newborns or infants...
  37. Kumagi T, Drenth J, Guttman O, Ng V, Lilly L, Therapondos G, et al. Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review. Liver Int. 2012;32:510-8 pubmed publisher
    b>Biliary atresia is a progressive biliary injury which occurs only in infants.
  38. Coots A, Donnelly B, Mohanty S, McNeal M, Sestak K, Tiao G. Rotavirus infection of human cholangiocytes parallels the murine model of biliary atresia. J Surg Res. 2012;177:275-81 pubmed publisher
    b>Biliary atresia (BA) is the leading indication for liver transplantation in the pediatric population...
  39. Ure B, Kuebler J, Schukfeh N, Engelmann C, Dingemann J, Petersen C. Survival with the native liver after laparoscopic versus conventional kasai portoenterostomy in infants with biliary atresia: a prospective trial. Ann Surg. 2011;253:826-30 pubmed publisher
    A prospective study to compare survival with own liver in laparoscopic versus conventional Kasai portoenterostomy in patients with biliary atresia.
  40. Schukfeh N, Al Gamrah A, Petersen C, Kuebler J. Detection of hepatotropic viruses has no impact on the prognosis after Kasai procedure. J Pediatr Surg. 2012;47:1828-32 pubmed publisher
    A viral origin of biliary atresia (BA) is discussed, and several studies have demonstrated different viral strains in liver biopsies of patients undergoing Kasai portoenterostomy...
  41. Nio M, Sasaki H, Wada M, Kazama T, Nishi K, Tanaka H. Impact of age at Kasai operation on short- and long-term outcomes of type III biliary atresia at a single institution. J Pediatr Surg. 2010;45:2361-3 pubmed publisher
    We reevaluated the impact of age at Kasai operation on the short- and long-term outcomes of type III biliary atresia (BA).
  42. Lin Y, Chang M, Liao S, Wu J, Ni Y, Tiao M, et al. Decreasing rate of biliary atresia in Taiwan: a survey, 2004-2009. Pediatrics. 2011;128:e530-6 pubmed publisher
    The pathogenesis of biliary atresia (BA) is unclear, but epidemiological studies may help to elucidate possible causes...
  43. Tiao M, Liou C, Huang L, Wang P, Lin T, Chen J, et al. Associations of mitochondrial haplogroups b4 and e with biliary atresia and differential susceptibility to hydrophobic bile Acid. PLoS Genet. 2013;9:e1003696 pubmed publisher
    Mitochondrial dysfunction has been implicated in the pathogenesis of biliary atresia (BA). This study aimed to determine whether a specific mitochondrial DNA haplogroup is implicated in the pathogenesis and prognosis of BA...
  44. Lu B, Brindley S, Tucker R, Lambert C, Mack C. ?-enolase autoantibodies cross-reactive to viral proteins in a mouse model of biliary atresia. Gastroenterology. 2010;139:1753-61 pubmed publisher
    b>Biliary atresia (BA) is a neonatal cholangiopathy of unknown etiology...
  45. Turowski C, Leonhardt J, Teichmann B, Heim A, Baumann U, Kuebler J, et al. Preconceptional oral vaccination prevents experimental biliary atresia in newborn mice. Eur J Pediatr Surg. 2010;20:158-63 pubmed publisher
    b>Biliary atresia (BA) in humans resembles BA induced in Balb/c-mice by Rhesus Rotavirus (RRV). In mice, susceptibility to BA is ascribed to the lack of maternally derived immune protection...
  46. Mohanty S, Ivantes C, Mourya R, Pacheco C, Bezerra J. Macrophages are targeted by rotavirus in experimental biliary atresia and induce neutrophil chemotaxis by Mip2/Cxcl2. Pediatr Res. 2010;67:345-51 pubmed publisher
    b>Biliary atresia is an obstructive cholangiopathy of unknown etiology...
  47. Davenport M. Biliary atresia: clinical aspects. Semin Pediatr Surg. 2012;21:175-84 pubmed publisher
    b>Biliary atresia (BA) remains an enigmatic disease with a degree of etiologic heterogeneity...
  48. Hartley J, Davenport M, Kelly D. Biliary atresia. Lancet. 2009;374:1704-13 pubmed publisher
    b>Biliary atresia is a rare disease of infancy, which has changed within 30 years from being fatal to being a disorder for which effective palliative surgery or curative liver transplantation, or both, are available...
  49. Yang J, Ma D, Peng Y, Song L, Li C. Comparison of different diagnostic methods for differentiating biliary atresia from idiopathic neonatal hepatitis. Clin Imaging. 2009;33:439-46 pubmed publisher
    To retrospectively analyze different methods in differentiating biliary atresia from idiopathic neonatal hepatitis.
  50. Superina R, Magee J, Brandt M, Healey P, Tiao G, Ryckman F, et al. The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg. 2011;254:577-85 pubmed publisher
    The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes.
  51. Lee M, Kim M, Lee M, Yoon C, Han S, Oh J, et al. Biliary atresia: color doppler US findings in neonates and infants. Radiology. 2009;252:282-9 pubmed publisher
    To describe color Doppler ultrasonographic (US) findings in livers of neonates with biliary atresia (BA) and to compare them with US findings in livers of neonates with non-BA and control subjects.
  52. Matthews R, Eauclaire S, Mugnier M, Lorent K, Cui S, Ross M, et al. DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of infantile biliary atresia. Hepatology. 2011;53:905-14 pubmed publisher
    Infantile cholestatic disorders arise in the context of progressively developing intrahepatic bile ducts. Biliary atresia (BA), a progressive fibroinflammatory disorder of extra- and intrahepatic bile ducts, is the most common ..
  53. Takahashi Y, Nishimoto Y, Matsuura T, Hayashida M, Tajiri T, Soejima Y, et al. Surgical complications after living donor liver transplantation in patients with biliary atresia: a relatively high incidence of portal vein complications. Pediatr Surg Int. 2009;25:745-51 pubmed publisher
    The aim of this study is to present the surgical complications in living donor liver transplantation (LDLT) for biliary atresia (BA) as a treatment for end stage liver disease.