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. ncbi Evidence for the epithelial to mesenchymal transition in biliary atresia fibrosis
    Rosalyn Diaz
    Department of Medicine Gastroenterology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Hum Pathol 39:102-15. 2008
  2. ncbi Immunological gap in the infectious animal model for biliary atresia
    G Czech-Schmidt
    Department of Pediatric Surgery, Medical School Hannover, Carl-Neuberg-Strasse 1, Hannover, 30625, Germany
    J Surg Res 101:62-7. 2001
  3. pmc Neonatal NK cells target the mouse duct epithelium via Nkg2d and drive tissue-specific injury in experimental biliary atresia
    Pranavkumar Shivakumar
    Cincinnati Children s Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229 3039, USA
    J Clin Invest 119:2281-90. 2009
  4. pmc Cholangiocytes as immune modulators in rotavirus-induced murine biliary atresia
    Barrett H Barnes
    Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Children s Hospital, University of Colorado Denver School of Medicine, Aurora, CO 80045, USA
    Liver Int 29:1253-61. 2009
  5. pmc Genome-wide association study identifies a susceptibility locus for biliary atresia on 10q24.2
    Maria Mercè Garcia-Barceló
    Department of Surgery, The University of Hong Kong, Hong Kong, China
    Hum Mol Genet 19:2917-25. 2010
  6. ncbi Innate immune response to double-stranded RNA in biliary epithelial cells is associated with the pathogenesis of biliary atresia
    Kenichi Harada
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    Hepatology 46:1146-54. 2007
  7. pmc Macrophages are targeted by rotavirus in experimental biliary atresia and induce neutrophil chemotaxis by Mip2/Cxcl2
    Sujit K Mohanty
    Cincinnati Children s Hospital Medical Center and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
    Pediatr Res 67:345-51. 2010
  8. pmc Cholangiocyte expression of alpha2beta1-integrin confers susceptibility to rotavirus-induced experimental biliary atresia
    Mubeen Jafri
    Department of Pediatric and Thoracic Surgery, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Am J Physiol Gastrointest Liver Physiol 295:G16-G26. 2008
  9. pmc Hedgehog activity, epithelial-mesenchymal transitions, and biliary dysmorphogenesis in biliary atresia
    Alessia Omenetti
    Division of Gastroenterology, Duke University Medical Center, Durham, NC, USA
    Hepatology 53:1246-58. 2011
  10. pmc Regulatory T cells control the CD8 adaptive immune response at the time of ductal obstruction in experimental biliary atresia
    Celine S Lages
    Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Hepatology 56:219-27. 2012

Detail Information

Publications319 found, 100 shown here

  1. ncbi Evidence for the epithelial to mesenchymal transition in biliary atresia fibrosis
    Rosalyn Diaz
    Department of Medicine Gastroenterology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Hum Pathol 39:102-15. 2008
    ..role in biliary fibrosis and might be found in patients with especially rapid forms, such as is seen in biliary atresia. We therefore obtained liver tissue from patients with biliary atresia as well as a variety of other ..
  2. ncbi Immunological gap in the infectious animal model for biliary atresia
    G Czech-Schmidt
    Department of Pediatric Surgery, Medical School Hannover, Carl-Neuberg-Strasse 1, Hannover, 30625, Germany
    J Surg Res 101:62-7. 2001
    BACKGROUND: Extrahepatic biliary atresia (EHBA), the etiology of which still remains unclear, occurs exclusively in newborns and has recently been simulated in an animal model...
  3. pmc Neonatal NK cells target the mouse duct epithelium via Nkg2d and drive tissue-specific injury in experimental biliary atresia
    Pranavkumar Shivakumar
    Cincinnati Children s Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229 3039, USA
    J Clin Invest 119:2281-90. 2009
    b>Biliary atresia is a neonatal obstructive cholangiopathy that progresses to end-stage liver disease...
  4. pmc Cholangiocytes as immune modulators in rotavirus-induced murine biliary atresia
    Barrett H Barnes
    Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Children s Hospital, University of Colorado Denver School of Medicine, Aurora, CO 80045, USA
    Liver Int 29:1253-61. 2009
    b>Biliary atresia (BA) is a progressive disease characterized by bile duct inflammation and fibrosis. The aetiology is unknown and may be due to a virus-induced, autoimmune-mediated injury of cholangiocytes...
  5. pmc Genome-wide association study identifies a susceptibility locus for biliary atresia on 10q24.2
    Maria Mercè Garcia-Barceló
    Department of Surgery, The University of Hong Kong, Hong Kong, China
    Hum Mol Genet 19:2917-25. 2010
    b>Biliary atresia (BA) is characterized by the progressive fibrosclerosing obliteration of the extrahepatic biliary system during the first few weeks of life...
  6. ncbi Innate immune response to double-stranded RNA in biliary epithelial cells is associated with the pathogenesis of biliary atresia
    Kenichi Harada
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    Hepatology 46:1146-54. 2007
    Infections of Reoviridae consisting of a double-stranded RNA (dsRNA) genome are a possible cause of biliary atresia (BA)...
  7. pmc Macrophages are targeted by rotavirus in experimental biliary atresia and induce neutrophil chemotaxis by Mip2/Cxcl2
    Sujit K Mohanty
    Cincinnati Children s Hospital Medical Center and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
    Pediatr Res 67:345-51. 2010
    b>Biliary atresia is an obstructive cholangiopathy of unknown etiology...
  8. pmc Cholangiocyte expression of alpha2beta1-integrin confers susceptibility to rotavirus-induced experimental biliary atresia
    Mubeen Jafri
    Department of Pediatric and Thoracic Surgery, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Am J Physiol Gastrointest Liver Physiol 295:G16-G26. 2008
    ..in the newborn period results in biliary epithelial cell (cholangiocyte) infection and the murine model of biliary atresia. Rotavirus infection of a cell requires attachment, which is governed in part by cell-surface expression of ..
  9. pmc Hedgehog activity, epithelial-mesenchymal transitions, and biliary dysmorphogenesis in biliary atresia
    Alessia Omenetti
    Division of Gastroenterology, Duke University Medical Center, Durham, NC, USA
    Hepatology 53:1246-58. 2011
    b>Biliary atresia (BA) is notable for marked ductular reaction and rapid development of fibrosis...
  10. pmc Regulatory T cells control the CD8 adaptive immune response at the time of ductal obstruction in experimental biliary atresia
    Celine S Lages
    Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Hepatology 56:219-27. 2012
    ..are effector cells of cholangiocyte injury in human and in rhesus rotavirus (RRV)-induced experimental biliary atresia (BA)...
  11. pmc Transient elastography for predicting esophageal/gastric varices in children with biliary atresia
    Voranush Chongsrisawat
    Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
    BMC Gastroenterol 11:41. 2011
    ..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.
  12. pmc MicroRNA profiling identifies miR-29 as a regulator of disease-associated pathways in experimental biliary atresia
    Nicholas J Hand
    Department of Pediatrics, University of Pennsylvania School of Medicine, Children s Hospital of Philadelphia Research Institute, ARC 902G, 3615 Civic Center Blvd, Philadelphia, PA 19104 4318, USA
    J Pediatr Gastroenterol Nutr 54:186-92. 2012
    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...
  13. ncbi Assessment of liver fibrosis and cirrhosis by aspartate aminotransferase-to-platelet ratio index in children with biliary atresia
    Sang Yong Kim
    Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
    J Pediatr Gastroenterol Nutr 51:198-202. 2010
    In patients with biliary atresia (BA), liver fibrosis and cirrhosis commonly occur even after Kasai hepatoportoenterostomy...
  14. ncbi Epithelial-mesenchymal transition induced by biliary innate immunity contributes to the sclerosing cholangiopathy of biliary atresia
    Kenichi Harada
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    J Pathol 217:654-64. 2009
    ..RNA (dsRNA) genome and the biliary innate immune response to dsRNA are implicated in the aetiopathogenesis of biliary atresia (BA)...
  15. ncbi Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?
    C Chardot
    Service de Chirurgie Pediatrique, Universite Catholique de Louvain, Brussels, Belgium
    J Pediatr 138:224-8. 2001
    The prognosis of hepatoportoenterostomy (the Kasai operation) for biliary atresia worsens when the age at surgery increases. This study examines whether the Kasai operation remains justified after 3 months of life.
  16. ncbi Universal screening for biliary atresia using an infant stool color card in Taiwan
    Cheng Hui Hsiao
    Department of Pediatrics, Cardinal Tien Hospital Yung Ho Branch, Taipei, Taiwan
    Hepatology 47:1233-40. 2008
    b>Biliary atresia is the most common cause of death from liver disease in children...
  17. pmc Effector role of neonatal hepatic CD8+ lymphocytes in epithelial injury and autoimmunity in experimental biliary atresia
    Pranavkumar Shivakumar
    Cincinnati Children s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229 3039, USA
    Gastroenterology 133:268-77. 2007
    Lymphocytes populate the livers of infants with biliary atresia, but it is unknown whether neonatal lymphocytes regulate pathogenesis of disease...
  18. pmc Obstruction of extrahepatic bile ducts by lymphocytes is regulated by IFN-gamma in experimental biliary atresia
    Pranavkumar Shivakumar
    Department of Pediatrics, Cincinnati Children s Hospital Medical Center, Ohio 45229 3039, USA
    J Clin Invest 114:322-9. 2004
    The etiology and pathogenesis of bile duct obstruction in children with biliary atresia are largely unknown...
  19. ncbi Biliary atresia
    Jane L Hartley
    Liver Unit, Birmingham Children s Hospital NHS Trust, Birmingham, UK
    Lancet 374:1704-13. 2009
    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...
  20. pmc Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop
    Ronald J Sokol
    Department of Pediatrics, University of Colorado School of Medicine, The Children s Hospital, Denver, CO, USA
    Hepatology 46:566-81. 2007
    b>Biliary atresia is the most common cause of end-stage liver disease in the infant and is the leading pediatric indication for liver transplantation in the United States...
  21. pmc The pathogenesis of biliary atresia: evidence for a virus-induced autoimmune disease
    Cara L Mack
    Pediatric Liver Center and Liver Transplant Program, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, The Children s Hospital, University of Colorado at Denver Health Sciences Center, Denver, Colorado 80218, USA
    Semin Liver Dis 27:233-42. 2007
    b>Biliary atresia is a mystifying cause of neonatal cholestasis, manifested by progressive inflammation and fibrosis of both the extrahepatic and intrahepatic bile ducts...
  22. ncbi Elevation of serum galectin-3 and liver stiffness measured by transient elastography in biliary atresia
    S Honsawek
    Faculty of Medicine Chulalongkorn University, Department of Biochemistry, Bangkok, Thailand
    Eur J Pediatr Surg 21:250-4. 2011
    b>Biliary atresia (BA) is an intractable neonatal liver disease characterized by progressive fibrosclerotic obliteration of the extrahepatic biliary tree...
  23. ncbi Temporal-spatial activation of apoptosis and epithelial injury in murine experimental biliary atresia
    Nissa Erickson
    Cincinnati Children s Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229 3039, USA
    Hepatology 47:1567-77. 2008
    b>Biliary atresia is a fibro-inflammatory cholangiopathy that obstructs the extrahepatic bile ducts in young infants...
  24. ncbi The evidence of maternal microchimerism in biliary atresia using fluorescent in situ hybridization
    Makoto Hayashida
    Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812 8582, Japan
    J Pediatr Surg 42:2097-101. 2007
    b>Biliary atresia (BA) is a cholestatic disease of unknown etiology. It has recently been suggested that graft-vs-host disease caused by microchimerism is an etiology in the development of autoimmune disease...
  25. ncbi The role of reovirus type 3 infection in an established murine model for biliary atresia
    P O Szavay
    Department of Paediatric Surgery, Hannover Medical School, Hannover, Germany
    Eur J Pediatr Surg 12:248-50. 2002
    Infection of newborn Balb/c-mice with Rhesus rotavirus (RRV) leads to cholestasis and biliary atresia. In this current model, Reovirus Type 3 was investigated to ascertain whether Reovirus Type 3 causes the same or similar hepatobiliary ..
  26. ncbi Early stage of biliary atresia is associated with significant changes in 8-hydroxydeoxyguanosine and mitochondrial copy number
    Mao Meng Tiao
    Department of Pediatrics, Chang Gung Memorial Hospital Kaohsiung Medical Center, Chang Gung University College of Medicine, Taiwan
    J Pediatr Gastroenterol Nutr 45:329-34. 2007
    Oxidative stress is known to be involved in the pathogenesis of biliary atresia (BA), but the mechanism has yet to be elucidated...
  27. pmc Effect of rotavirus strain on the murine model of biliary atresia
    Steven R Allen
    Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, MLC 2923, Cincinnati, OH 45229, USA
    J Virol 81:1671-9. 2007
    b>Biliary atresia is a devastating disorder of the newborn in which afflicted infants develop inflammation and fibrosis of the extrahepatic biliary tract, resulting in cirrhosis and end-stage liver disease...
  28. ncbi Serial circulating markers of inflammation in biliary atresia--evolution of the post-operative inflammatory process
    Bommayya Narayanaswamy
    Departments of Paediatric Surgery, King s College Hospital, London SE5 9RS
    Hepatology 46:180-7. 2007
    b>Biliary atresia (BA) may be characterized as an occlusive cholangiopathy affecting both intra- and extra-hepatic parts of the biliary tree, together with a pronounced inflammatory response consisting of hepatic infiltration of (..
  29. pmc Analysis of liver repair mechanisms in Alagille syndrome and biliary atresia reveals a role for notch signaling
    Luca Fabris
    CeLiveR, Gastroenterology and Liver Transplant Unit, Ospedali Riuniti di Bergamo, Bergamo, Italy
    Am J Pathol 171:641-53. 2007
    ..Instead, in biliary atresia (BA) severe cholestasis is associated with a pronounced "ductular reaction" and rapid progression ..
  30. ncbi Epidemiological features of biliary atresia in Taiwan, a national study 1996-2003
    Mao Meng Tiao
    Department of Pediatrics, Chang Gung Memorial Hospital Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan
    J Gastroenterol Hepatol 23:62-6. 2008
    The incidence of biliary atresia (BA) varies among different countries. It is supposed to be higher in Asian countries than in Western countries; however, the incidence of BA in Taiwan has not been well investigated...
  31. ncbi The inv mouse as an experimental model of biliary atresia
    Shinichi Shimadera
    Division of Surgery, Children s Research Hospital, Kyoto Prefectural University of Medicine, Kyoto 602 8566, Japan
    J Pediatr Surg 42:1555-60. 2007
    The causation of biliary atresia (BA) remains unclear. However, ductal plate malformation (DPM), maldevelopment of the intrahepatic bile ducts, is 1 of the preferred theories...
  32. ncbi Integrin alphavbeta6 and mediators of extracellular matrix deposition are up-regulated in experimental biliary atresia
    Evan P Nadler
    Division of Pediatric Surgery, Department of Surgery, New York University School of Medicine, New York, New York 10016, USA
    J Surg Res 154:21-9. 2009
    b>Biliary atresia (BA) is a progressive obliteration of the extrahepatic bile ducts resulting in hepatic fibrosis. The underlying mechanisms have not been defined...
  33. pmc Maternal microchimerism in the livers of patients with biliary atresia
    David L Suskind
    Department of Pediatrics, University of California, San Francisco, USA
    BMC Gastroenterol 4:14. 2004
    b>Biliary atresia (BA) is a neonatal cholestatic disease of unknown etiology. It is the leading cause of liver transplantation in children...
  34. pmc Th2 signals induce epithelial injury in mice and are compatible with the biliary atresia phenotype
    Jun Li
    Cincinnati Children s Hospital Medical Center and the Department of Pediatrics of the University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
    J Clin Invest 121:4244-56. 2011
    b>Biliary atresia (BA) is a destructive cholangiopathy of childhood in which Th1 immunity has been mechanistically linked to the bile duct inflammation and obstruction that culminate in liver injury...
  35. pmc Clues to the etiology of bile duct injury in biliary atresia
    Cara L Mack
    Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, and Digestive Health Institute, Children s Hospital Colorado, Aurora, Colorado 80045, USA
    Semin Liver Dis 32:307-16. 2012
    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...
  36. pmc Biliary atresia: cellular dynamics and immune dysregulation
    Amy G Feldman
    Section of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Children s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO 80045, USA
    Semin Pediatr Surg 21:192-200. 2012
    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...
  37. ncbi Hepatocellular telomere length in biliary atresia measured by Q-FISH
    Yukihiro Sanada
    Department of Transplant Surgery, Jichi Medical University, 3311 1 Yakushiji, Shimotsuke City, Tochigi, 329 0498, Japan
    World J Surg 36:908-16. 2012
    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...
  38. ncbi NF-kappaB related abnormal hyper-expression of iNOS and NO correlates with the inflammation procedure in biliary atresia livers
    Lei Huang
    Department of Pediatric Surgery, Nanjing Children s Hospital Affiliated to Nanjing Medical University, Nanjing, China
    Pediatr Surg Int 26:899-905. 2010
    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.
  39. pmc α-enolase autoantibodies cross-reactive to viral proteins in a mouse model of biliary atresia
    Brandy R Lu
    Section of Pediatric Gastroenterology, Hepatology, and Nutrition, The Children s Hospital, University of Colorado Denver School of Medicine, Aurora, Colorado 80045, USA
    Gastroenterology 139:1753-61. 2010
    b>Biliary atresia (BA) is a neonatal cholangiopathy of unknown etiology...
  40. pmc Differential expression of hepatic fibrosis mediators in sick and spontaneously recovered mice with experimental biliary atresia
    Evan P Nadler
    Department of Surgery, Division of Pediatric Surgery, New York University School of Medicine, New York, New York
    J Surg Res 159:611-7. 2010
    Hepatic fibrosis leading to cirrhosis is the major morbidity in patients with biliary atresia (BA). This fibrosis is due to an imbalance in extracellular matrix (ECM) breakdown and deposition...
  41. pmc Intestinal synthesis and secretion of bile salts as an adaptation to developmental biliary atresia in the sea lamprey
    Chu Yin Yeh
    Department of Physiology, Michigan State University, East Lansing, MI 48824, USA
    Proc Natl Acad Sci U S A 109:11419-24. 2012
    ..Patients with biliary atresia (i.e., obliteration of the biliary tree) suffer liver fibrosis and cirrhosis...
  42. ncbi Biliary atresia
    Richard A Schreiber
    BC Children s Hospital, Vancouver, British Columbia, Canada
    J Pediatr Gastroenterol Nutr 35:S11-6. 2002
  43. ncbi Optimum prednisolone usage in patients with biliary atresia postportoenterostomy
    Hiroyuki Kobayashi
    Department of Pediatric Surgery, Juntendo University School of Medicine, Tokyo 113 8421, Japan
    J Pediatr Surg 40:327-30. 2005
    Prednisolone is used routinely after portoenterostomy (PE) in patients with biliary atresia (BA). The authors reviewed their patients with BA post-PE to assess prednisolone protocols.
  44. pmc Endotoxin and CD14 in the progression of biliary atresia
    Ming Huei Chou
    Institute of Basic Medical Sciences, National Chang Kung University, Tainan, Taiwan
    J Transl Med 8:138. 2010
    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...
  45. ncbi Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan
    Tien Hau Lien
    Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
    Hepatology 53:202-8. 2011
    In Taiwan, a screening system using an infant stool color card to promote the early diagnosis of biliary atresia (BA) was established in 2002...
  46. pmc Cholangiocyte secretion of chemokines in experimental biliary atresia
    Mubeen Jafri
    Department of Pediatric and Thoracic Surgery, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    J Pediatr Surg 44:500-7. 2009
    b>Biliary atresia (BA) is a disease of the newborn that results in obstruction of the biliary tree...
  47. ncbi Prognosis of biliary atresia: what can be further improved?
    Christophe Chardot
    J Pediatr 148:432-5. 2006
  48. ncbi Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver
    Panayotis Lykavieris
    Service d hépatologie pédiatrique, Hopital de Bicetre, AP HP, Le Kremlin Bicetre, France
    Hepatology 41:366-71. 2005
    To define the long-term prognosis of children undergoing the Kasai operation for biliary atresia, a retrospective study was undertaken comprising 271 patients operated between 1968 and 1983...
  49. ncbi Laparoscopic Kasai portoenterostomy for biliary atresia
    Edward Esteves
    Pediatric Surgery Division, University of Goias, Goiania, Brazil
    Pediatr Surg Int 18:737-40. 2002
    ..It is concluded that LARP for EHBDA can be done safely in infants using an extracorporeal transumbilical enteric anastomosis, with several advantages compared with open surgery. The role of LARP in facilitating LT is yet to be defined...
  50. ncbi Image and diagnosis. Intracranial haemorrhage due to vitamin K deficiency, as the first symptom of extrahepatic biliary atresia
    V Ertekin
    Faculty of Medicine, Department of Pediatric Gastroenterology, Ataturk University, Erzurum, Turkey
    West Indian Med J 54:392, 397. 2005
  51. ncbi Intrauterine growth retardation, duodenal and extrahepatic biliary atresia, hypoplastic pancreas and other intestinal anomalies: further evidence of the Martínez-Frías syndrome
    Enrique Galán-Gómez
    Unidad de Genetica, Departamento de Pediatria, Hospital Materno Infantil, Servicio Extremeño de Salud, Universidad de Extremadura, Badajoz, Spain
    Eur J Med Genet 50:144-8. 2007
    ..parents, who presented with an MCA pattern characterized by low birth weight, duodenal atresia, extrahepatic biliary atresia, hypoplastic pancreas and intestinal malrotation. The infant died 60 days after birth...
  52. ncbi Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening
    Marie Odile Serinet
    French Observatory of Biliary Atresia, Le Kremlin, Bicĉtre, Paris, France
    Pediatrics 123:1280-6. 2009
    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...
  53. ncbi Matrilysin (MMP-7) is a major matrix metalloproteinase upregulated in biliary atresia-associated liver fibrosis
    Chao Cheng Huang
    Graduate Institute of Clinical Medical Sciences, Chang Gung University, Lin Ko, Taiwan
    Mod Pathol 18:941-50. 2005
    ..the proteases responsible for tissue remodeling during liver fibrosis caused by various disorders including biliary atresia. However, information regarding the relative contribution of these proteases to liver fibrosis is still ..
  54. ncbi Analysis of biliary epithelial-mesenchymal transition in portal tract fibrogenesis in biliary atresia
    Yu Hua Deng
    Department of Hepatobiliary Surgery, Children s Hospital of Chongqing Medical University, 136 Zhongshan 2nd Rd, Chongqing, 400014, People s Republic of China
    Dig Dis Sci 56:731-40. 2011
    ..This study was designed to investigate whether biliary epithelial cells (BECs) undergoing epithelial-mesenchymal transition (EMT) might be found in patients with biliary atresia, thereby serving as a source of fibrotic myofibroblasts.
  55. ncbi Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting
    Ashish Singhal
    Department of Surgical Gastroenterolgy and Liver Transplant, Indraprastha Apollo Hospital, New Delhi, India
    Pediatr Transplant 13:1041-7. 2009
    ..This is a report of a rare case of LDLT in a four-yr old male child suffering with biliary atresia (post-failed Kasai procedure) associated with (i) a large congenital CEPSh from the spleno-mesentric ..
  56. ncbi The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients
    Barbara E Wildhaber
    Department of Surgery, Section of Pediatric Surgery, C S Mott Children s Hospital, University of Michigan, Ann Arbor, MI, USA
    J Pediatr Surg 38:1480-5. 2003
    ..The aim of this study was to utilize clinical outcome methodology through multivariable analysis of perioperative factors to predict a successful Kasai-portoenterostomy (PE)...
  57. ncbi Anomalous development of the hepatobiliary system in the Inv mouse
    M V Mazziotti
    Department of Surgery, Washington University School of Medicine, Washington School of Medicine, St Louis, MO, USA
    Hepatology 30:372-8. 1999
    Extrahepatic biliary atresia (BA) is a devastating disease of the neonate in which the hepatic and/or common bile duct is obliterated or interrupted...
  58. ncbi Survival with the native liver after laparoscopic versus conventional kasai portoenterostomy in infants with biliary atresia: a prospective trial
    Benno M Ure
    Department of Pediatric Surgery, Hannover Medical School, Germany
    Ann Surg 253:826-30. 2011
    A prospective study to compare survival with own liver in laparoscopic versus conventional Kasai portoenterostomy in patients with biliary atresia.
  59. ncbi Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction
    Kimio Kanegawa
    Department of Radiology, Kobe Children s Hospital, 1 1 1 Takakuradai Suma ku, Kobe 654 0081, Japan
    AJR Am J Roentgenol 181:1387-90. 2003
    A retrospective review was performed to evaluate the importance of the "triangular cord" sign in comparison with gallbladder length and contraction for the diagnosis of biliary atresia in pediatric patients.
  60. ncbi Fractures in biliary atresia misinterpreted as child abuse
    Patricia A DeRusso
    Department of Pediatrics, Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA
    Pediatrics 112:185-8. 2003
    Bone fractures in children without a history of injury are highly suspicious for child abuse. Biliary atresia is a disorder associated with metabolic bone disease, and there are numerous reports of osteopenia, rickets, and/or fractures ..
  61. ncbi Pathogenesis and outcome of biliary atresia: current concepts
    Ronald J Sokol
    Pediatric Liver Center and Liver Transplantation Program, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado Health Sciences Center, Denver, Colorado, U S A
    J Pediatr Gastroenterol Nutr 37:4-21. 2003
  62. ncbi Biliary atresia: US diagnosis
    Terry M Humphrey
    Department of Radiology and Children s Liver and GI Unit, St James s University Hospital, Beckett Street, Leeds LS9 7TF, England
    Radiology 244:845-51. 2007
    To evaluate prospectively the sensitivity of ultrasonography (US) in the diagnosis of biliary atresia (BA), with surgery as the reference standard.
  63. ncbi Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia
    Yasuni Nakanuma
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    Histol Histopathol 25:223-35. 2010
    ..of pediatric biliary diseases, particularly Caroli's disease with congenital hepatic fibrosis (CHF) and biliary atresia (BA), is reviewed...
  64. ncbi Expression of the interferon-induced Mx proteins in biliary atresia
    Abdul Nasser Al-Masri
    Department of Pediatric Surgery, Medical School Hannover, 30625 Hannover, Germany
    J Pediatr Surg 41:1139-43. 2006
    b>Biliary atresia (BA) is a rare disease of the newborn for which the Kasai procedure is curative only for a few of the patients...
  65. ncbi Induction of innate immune response and absence of subsequent tolerance to dsRNA in biliary epithelial cells relate to the pathogenesis of biliary atresia
    Kenichi Harada
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    Liver Int 28:614-21. 2008
    ..g. endotoxin tolerance). Viral infections of the Reoviridae genus with a dsRNA genome are suspected to be part of the aetiology of biliary atresia (BA), but the negative biliary mechanisms remain unexplored.
  66. ncbi Expression of toll-like receptors and type 1 interferon specific protein MxA in biliary atresia
    Ying Hsien Huang
    Department of Pediatrics, Chang Gung Memorial Hospital Kaohsiung Medical Center and the Graduate Institute of Clinical Medical Sciences, Chang Gung University College of Medicine, Kaohsiung, Taiwan, ROC
    Lab Invest 87:66-74. 2007
    Viral infection and type I interferon have been implicated in the pathogenesis of biliary atresia (BA), but the expression of toll-like receptors (TLRs) that recognize viruses, as well as of type 1 interferon specific signaling molecules ..
  67. pmc Post-natal paucity of regulatory T cells and control of NK cell activation in experimental biliary atresia
    Alexander G Miethke
    Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children s Hospital Medical Center, MLC 2010, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
    J Hepatol 52:718-26. 2010
    Although recent studies have identified important roles for T and NK cells in the pathogenesis of biliary atresia (BA), the mechanisms by which susceptibility to bile duct injury is restricted to the neonatal period are unknown.
  68. pmc Armed CD4+ Th1 effector cells and activated macrophages participate in bile duct injury in murine biliary atresia
    Cara L Mack
    Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology and Nutrition, The Children s Hospital, 1056 East 19th Avenue B290, Denver, CO 80218, USA
    Clin Immunol 115:200-9. 2005
    b>Biliary atresia (BA) is an inflammatory cholangiopathy of infancy. A proposed mechanism regarding the pathogenesis of BA is that of a virus-induced, immune-mediated injury to bile ducts...
  69. pmc Utilization of sialic acid as a coreceptor is required for reovirus-induced biliary disease
    Erik S Barton
    Department of Microbiology and Immunology and Lamb Center for Pediatric Research, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA
    J Clin Invest 111:1823-33. 2003
    Infection of neonatal mice with some reovirus strains produces a disease similar to infantile biliary atresia, but previous attempts to correlate reovirus infection with this disease have yielded conflicting results...
  70. ncbi Prolonged neonatal jaundice and the diagnosis of biliary atresia: a single-center analysis of trends in age at diagnosis and outcomes
    Sharad I Wadhwani
    Division of Gastroenterology, St Louis Children s Hospital, Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Pediatrics 121:e1438-40. 2008
    Age at diagnosis is a modifiable risk factor in outcomes after hepatoportoenterostomy in biliary atresia; however, distinguishing biliary atresia from other more common causes of prolonged neonatal jaundice can be difficult...
  71. ncbi A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000
    Benjamin L Shneider
    Department of Pediatrics, Mount Sinai Medical Center, New York, NY 10029, Department of Biostatistics, University of Michigan, Ann Arbor, MI, and Department of Pediatrics, Children s Hospital of Philadelphia, PA, USA
    J Pediatr 148:467-474. 2006
    To determine the prognostic factors and optimal approaches to the diagnosis and management of biliary atresia, the leading indication for liver transplantation in children.
  72. ncbi Long-term prognosis of patients with biliary atresia: a 25 year summary
    Pei Yin Hung
    Department of Pediatrics, National Taiwan University Hospital, No 7 Chung Shan South Road, Taipei, Taiwan
    J Pediatr Gastroenterol Nutr 42:190-5. 2006
    The purpose of this study was to delineate the long-term prognosis of biliary atresia (BA) in Taiwan.
  73. ncbi Hepatic fibrosis scan for liver stiffness score measurement: a useful preendoscopic screening test for the detection of varices in postoperative patients with biliary atresia
    Hye Kyung Chang
    Department of Pediatric Surgery, Severance Children s Hospital, Yonsei University College of Medicine, Seoul, Korea
    J Pediatr Gastroenterol Nutr 49:323-8. 2009
    Even after successful Kasai portoenterostomy, progressive hepatic fibrosis in postoperative patients with biliary atresia (BA) can be associated with portal hypertension and esophageal or gastric varices...
  74. ncbi Comparison of different diagnostic methods for differentiating biliary atresia from idiopathic neonatal hepatitis
    Ji Gang Yang
    Nuclear Medicine Department of Beijing Friendship Hospital Affiliated to Capital Medical University, 95 Yong An Road Xuanwu District, Beijing 100050, People s Republic of China
    Clin Imaging 33:439-46. 2009
    To retrospectively analyze different methods in differentiating biliary atresia from idiopathic neonatal hepatitis.
  75. ncbi Neonatal cholestasis
    Frederick J Suchy
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, USA
    Pediatr Rev 25:388-96. 2004
  76. ncbi Abnormal activation of OPN inflammation pathway in livers of children with biliary atresia and relationship to hepatic fibrosis
    L Huang
    Department of Pediatric Surgery, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, China
    Eur J Pediatr Surg 18:224-9. 2008
    ..Aim of the study was to investigate the expression of OPN (osteopontin) and its upper-downstream regulating factors in the biliary atretic liver and explore the relationship to progressive intrahepatic fibro-inflammation...
  77. ncbi Prophylactic endoscopic sclerotherapy of large esophagogastric varices in infants with biliary atresia
    Mathieu Duché
    Division of Radiologie, Hopital Bicetre, Le Kremlin Bicetre, France
    Gastrointest Endosc 67:732-7. 2008
    Esophageal varices-related GI bleeding occurs frequently and early in life in children with biliary atresia and it may be life threatening.
  78. pmc Current management of biliary atresia
    Deirdre A Kelly
    The Liver Unit, Birmingham Children s Hospital NHS Trust, Birmingham, UK
    Arch Dis Child 92:1132-5. 2007
    Extra-hepatic biliary atresia occurs in approximately 1:15,000 live births leading to about 50 new cases/year in the UK. Presentation is with prolonged jaundice, usually in a term baby who develops signs of obstructive jaundice...
  79. ncbi Current concept about postoperative cholangitis in biliary atresia
    Yi Luo
    Department of Pediatric Surgery, Children s Hospital of Fudan University, Shanghai 200032, China
    World J Pediatr 4:14-9. 2008
    ..acholic stool and positive blood culture is a common and serious complication following Kasai's operation for biliary atresia. The aim of this review was to describe the pathogenesis, clinical manifestations, medical treatment and ..
  80. ncbi Etiopathogenesis of biliary atresia
    R J Sokol
    Pediatric Liver Center and Liver Transplantation Program, Department of Pediatrics, University of Colorado School of Medicine and The Children s Hospital, Denver, Colorado 80218, USA
    Semin Liver Dis 21:517-24. 2001
    b>Biliary atresia, a progressive sclerosis of the extrahepatic biliary tree that occurs only within the first 3 months of life, is one of the most common causes of neonatal cholestasis and accounts for over half of children who undergo ..
  81. ncbi Management of patients with biliary atresia in France: results of a decentralized policy 1986-2002
    Marie Odile Serinet
    From the French Observatory of Biliary Atresia, University Hospital of Bicetre, Le Kremlin, Bicetre, France
    Hepatology 44:75-84. 2006
    This study analyzed the results of the decentralized management of biliary atresia (BA) in France, where an improved collaboration between centers has been promoted since 1997...
  82. ncbi Relationships between OPG, RANKL, bone metabolism, and bone mineral density in biliary atresia
    Sittisak Honsawek
    Department of Biochemistry, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
    Pediatr Surg Int 25:261-7. 2009
    ..However, the relationship between the OPG-RANKL system and bone status in biliary atresia (BA) has not, as yet, been clarified...
  83. ncbi Congenital bile duct anomalies (biliary atresia) and chromosome 22 aneuploidy
    Jacqueline Allotey
    Department of Pediatric Surgery, King s College Hospital, SE5 9RS London, United Kingdom
    J Pediatr Surg 43:1736-40. 2008
    b>Biliary atresia is a disease of unknown etiology but not usually thought to have a significant genetic predisposition...
  84. ncbi Cystic biliary atresia: an etiologic and prognostic subgroup
    Enrica Caponcelli
    Department of Paediatric Surgery, King s College Hospital, SE5 9RS London, UK
    J Pediatr Surg 43:1619-24. 2008
    Cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA) in which prognosis may be relatively favorable but liable to misdiagnosis as choledochal cyst, and potentially offers insights into the etiology of BA...
  85. ncbi Antenatal presentation of biliary atresia
    Rupert Hinds
    Departments of Child Health and Paediatric Surgery, King s College Hospital, Denmark Hill, London, United Kingdom
    J Pediatr 144:43-6. 2004
    ..the incidence, presence of associated anomalies, type of malformation, and natural history of children with biliary atresia (BA) who presented antenatally...
  86. ncbi Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center
    Virginie Fouquet
    Department of Pediatric Surgery, Hopital Bicetre, Le Kremlin Bicetre, France
    Liver Transpl 11:152-60. 2005
    The aim of this study was to review our experience in orthotopic liver transplantation (OLT) for biliary atresia (BA) in children and analyze the survival and prognostic factors, and long-term outcome...
  87. pmc Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background mice
    Mami Uemura
    Department of Veterinary Anatomy, The University of Tokyo, Yayoi 1 1 1, Bunkyo ku, Tokyo 113 8657, Japan
    Development 140:639-48. 2013
    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...
  88. pmc Progressive biliary destruction is independent of a functional tumor necrosis factor-alpha pathway in a rhesus rotavirus-induced murine model of biliary atresia
    Rebecca M Tucker
    Division of Allergy and Clinical Immunology, Department of Medicine, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    Viral Immunol 20:34-43. 2007
    ..In summary, although RRV-inoculated BALB/c mice have highly elevated expression of TNF-alpha, this cytokine does not play an obligate role in disease progression...
  89. ncbi Role of immunologic costimulatory factors in the pathogenesis of biliary atresia
    Hiroyuki Kobayashi
    Department of Pediatric Surgery, Juntendo University School of Medicine, Tokyo, Japan
    J Pediatr Surg 38:892-6. 2003
    The authors studied the patterns of expression of immunologic costimulatory molecules (B7-1, B7-2, and CD40) in biliary atresia (BA) patients to confirm any correlation with clinical course/outcome.
  90. pmc Characterization of erythrovirus B19 genomes isolated in liver tissues from patients with fulminant hepatitis and biliary atresia who underwent liver transplantation
    Kenji Abe
    Department of Pathology, National Institute of Infectious Diseases, Shinjuku ku, Tokyo, Japan
    Int J Med Sci 4:105-9. 2007
    Fulminant hepatitis and biliary atresia are serious problems and their causes have not been explained well...
  91. ncbi Elevated expression of IL2 is associated with increased infiltration of CD8+ T cells in biliary atresia
    Masato Shinkai
    Children s Research Center, Our Lady s Hospital for Sick Children, University College Dublin, 12 Dublin, Ireland
    J Pediatr Surg 41:300-5. 2006
    ..within the liver and bile ducts has been proposed as a potential mechanism in the pathogenesis of biliary atresia (BA)...
  92. ncbi Experience with endoscopic management of high-risk gastroesophageal varices, with and without bleeding, in children with biliary atresia
    Mathieu Duché
    Hépatologie Pédiatrique and Centre de Référence National de l Atrésie des Voies Biliaires, Hopital Bicetre, AP HP, Le Kremlin Bicetre, France Université Paris Sud 11, Le Kremlin Bicetre, France Radiologie Pédiatrique, Hopital Bicetre, AP HP, Le Kremlin Bicetre, France
    Gastroenterology 145:801-7. 2013
    b>Biliary atresia, the most common cause of childhood cirrhosis, increases the risks for portal hypertension and gastrointestinal bleeding...
  93. pmc Silencing of the rotavirus NSP4 protein decreases the incidence of biliary atresia in murine model
    Jiexiong Feng
    Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
    PLoS ONE 6:e23655. 2011
    b>Biliary atresia is a common disease in neonates which causes obstructive jaundice and progressive hepatic fibrosis...
  94. ncbi Changes in epigenetic regulation of CD4+ T lymphocytesin biliary atresia
    Rui Dong
    Department of Pediatric Surgery, Children s Hospital of Fudan University, Ministry of Health, Shanghai, 201102 People s Republic of China
    Pediatr Res 70:555-9. 2011
    b>Biliary atresia (BA) is a virus-induced autoimmune disease associated with abnormal DNA methylation patterns that contribute to disease presentation...
  95. ncbi Hepatocellular carcinoma in biliary atresia: King's College Hospital experience
    Nedim Hadzic
    Paediatric Liver Centre, King s College Hospital, Denmark Hill, London, United Kingdom
    J Pediatr 159:617-22.e1. 2011
    To establish risks for development of hepatocellular carcinoma (HCC) in children with biliary atresia (BA), the most common chronic liver disease of childhood.
  96. ncbi Biliary atresia: lessons learned from the voluntary German registry
    J Leonhardt
    St Bernward Hospital, Pediatric Surgery, Hildesheim, Germany
    Eur J Pediatr Surg 21:82-7. 2011
    Aim of the study was to carry out a 5-year survey of German patients with biliary atresia (BA) and to launch a discussion regarding the feasibility of voluntary registries in unregulated healthcare systems.
  97. ncbi Identification of transforming growth factors actively transcribed during the progress of liver fibrosis in biliary atresia
    Shin Ye Lee
    Department of Surgery Chang Gung Memorial Hospital, Taiwan, China
    J Pediatr Surg 39:702-8. 2004
    ..Their expression has not been completely elucidated in the progress of liver fibrosis associated with biliary atresia (BA).
  98. ncbi Immunoglobulin deposits in liver tissue from infants with biliary atresia and the correlation to cytomegalovirus infection
    Bjorn Fischler
    Department of Pediatrics, Huddinge University Hospital, Karolinska Institutet, SE 141 86 Stockholm, Sweden
    J Pediatr Surg 40:541-6. 2005
    The aim of this report was to study the amount and distribution of immunoglobulin deposits in liver biopsies from infants with biliary atresia (BA) and correlate the results to the cytomegalovirus (CMV) infection status.
  99. ncbi The prognostic value of ductal plate malformation and other histologic parameters in biliary atresia: an immunohistochemical study
    Y Low
    Department of Paediatric Surgery, KK Women's and Children's Hospital, Singapore
    J Pediatr 139:320-2. 2001
    ..a term given to intrahepatic bile ducts that retain the fetal configuration, is observed in some cases of biliary atresia. We examined 21 livers from patients with biliary atresia; ductal plate malformation occurred in 38% of cases,..
  100. pmc Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth
    Sanjiv Harpavat
    Department of Pediatrics, BaylorCollege of Medicine, Houston, TX, USA
    Pediatrics 128:e1428-33. 2011
    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...
  101. pmc Circulating microRNA is a biomarker of biliary atresia
    Adam M Zahm
    Division of Gastroenterology and Nutrition, Department of Pediatrics, University of Pennsylvania School of Medicine, Children s Hospital of Philadelphia Research Institute, Philadelphia, PA 19104, USA
    J Pediatr Gastroenterol Nutr 55:366-9. 2012
    The lack of reliable noninvasive diagnostic biomarkers of biliary atresia (BA) results in delayed diagnosis and worsened patient outcome...

Research Grants55

  1. Biliary Atresia, Cholestatic Liver Diseases, and Cystic Fibrosis: Indiana Univers
    Jean Pappas Molleston; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Pediatric liver disease has significant morbidity and mortality. Biliary atresia accounts for more than half of pediatric liver transplants...
  2. Colorado Center for Childhood Liver Disease Research and Education
    Ronald J Sokol; Fiscal Year: 2013
    ..A group of these disorders has been studied at our Center for the past 6 years within the Biliary Atresia Research Consortium (BARC) and the Cholestatic Liver Disease Consortium (CLiC), and include biliary atresia, ..
  3. The Pittsburgh Cholestatic Liver Disease Consortium
    Benjamin L Shneider; Fiscal Year: 2013
    ..liver diseases including Alagille syndrome, alpha-1 antitrypsin deficiency, bile acid synthesis defects, biliary atresia, cystic fibrosis, mitochondrial hepatopathies and progressive familial intrahepatic cholestasis, lead to ..
  4. The role of regulatory T cells in biliary atresia
    Alexander Miethke; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Biliary atresia (BA) is a progressive fibroinflammatory obstruction of the extrahepatic biliary tree and the most common indication for pediatric liver transplantation worldwide...
  5. Immunologic dysfunction in biliary atresia
    Jorge A Bezerra; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Biliary atresia is the most common cause of end-stage cirrhosis in children and the number one indication for pediatric liver transplantation...
  6. T Cell-Mediated Mechanisms of Autoimmunity in Murine and Human Biliary Atresia
    CARA LYNN MACK; Fiscal Year: 2011
    Project Summary. Biliary atresia (BA) is a progressive, inflammatory, sclerosing extrahepatic and intrahepatic cholangiopathy which presents in infancy and leads to bile duct obstruction and biliary cirrhosis...
  7. Establichment of CHLA's ChilDREN Clinical Center
    KASPER SAONUN WANG; Fiscal Year: 2013
    ..b>Biliary Atresia (BA) and idiopathic neonatal hepatitis, studied under the Biliary Atresia Research Consortium, along with ..
  8. Center for the Study of Pediatric Cholestasis
    Ronen Arnon; Fiscal Year: 2013
    The purpose of this competing renewal application is to continue, expand and merge the Biliary Atresia Research Consortium (BARC) and the Cholestatic Liver Consortium (CLiC) to form the Childhood Liver Disease Research and Education ..
  9. DNA methylation in biliary development and disease
    Randolph P Matthews; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Biliary atresia (BA) is the most common cause of obstructive jaundice in infants and is lethal if uncorrected...
  10. Circulating miRNA as a biomarker for biliary atresia
    Joshua R Friedman; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Biliary atresia (BA) is a disease of infants in which the bile ducts are progressively destroyed (reviewed in (7))...
  11. Childhood Liver Disease Research and Education Network (CHiLDREN) - UCSF (CC)
    Philip Rosenthal; Fiscal Year: 2013
    UCSF is currently one of the ten original sites in the Biliary Atresia Research Consortium (BARC) and the Cholestatic Liver Consortium (CLiC) which is being continued, expanded and merged to form the Childhood Liver Disease Research and ..
  12. MicroRNA in Liver Development and Disease
    Joshua R Friedman; Fiscal Year: 2012
    ..Many diseases are caused by aberrations in normal developmental pathways. Biliary atresia, the leading indication for pediatric liver transplantation, is associated with developmental anomalies...
  13. Hhex-a Homeobox Gene Necessary for Liver Development
    Clifford W Bogue; Fiscal Year: 2010
    ..Additionally, this research will shed important insight into the pathogenesis of diseases of the biliary tract, including biliary atresia and polycystic liver disease.
  14. Isolation, Identification and Characterization of a Toxin Causing Biliary Atresia
    Rebecca G Wells; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Biliary atresia (BA) is a fibro inflammatory disorder that is the leading cause of neonatal cholestasis and the most common indication for liver transplant in the pediatric population...
  15. Studies of Pediatric Liver Transplantation
    Anne S Lindblad; Fiscal Year: 2010
    ..predicting both short and long-term (5 years or more) outcomes overall and within primary disease subgroups (biliary atresia, metabolic disease, tumor, autoimmune hepatitis, etc.)...
  16. Midwest Children's Consortium
    Yumirle P Turmelle; Fiscal Year: 2013
    ..presentation, with the goal of defining, then reducing, the factors that hinder timely referral for biliary atresia. Aim 4 (Apply imaging methodologies to ChiLDREN diseases) amalgamates SLCH/WUSTL's advanced liver ..
  17. The Role of TGF-beta in the pathogenesis of Experimental Biliary Atresia
    Evan P Nadler; Fiscal Year: 2012
    ..of this project is to determine the role of transforming growth factor-beta (TGF-beta) in the pathogenesis of biliary atresia. Our initial experiments are designed to establish a durable and reproducible animal model of biliary atresia...
  18. Emory Clinical Center: Childhood Liver Disease Research and Education Network
    Rene Romero; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): In children, chronic liver disease is a rare but devastating condition. Biliary atresia is a disease of unknown etiology and is the primary indication for liver transplantation in children...
  19. The Molecular Determinants of Virus Induced Biliary Atresia
    Gregory M Tiao; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Biliary atresia is the most common cause of pediatric end stage liver disease and the number one indication for pediatric liver transplantation...
  20. Clinical Center: Childhood Liver Disease Research and Education Network (ChiLDREN
    PETER FRANK WHITINGTON; Fiscal Year: 2013
    ..Education Network (ChiLDREN), the major objective of which is to "combine the expertise and resources of the [Biliary Atresia Research Consortium] BARC and [Cholestatic Liver Consortium] CLiC clinical centers .....
  21. Childhood Liver Disease Research and Education Network Clinical Center in Seattle
    Karen F Murray; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Biliary atresia and the other cholestatic liver diseases of childhood are important causes of pediatric liver disease...
  22. Advancing our Understanding of Rare Pediatric Liver Diseases
    KATHLEEN MARY LOOMES; Fiscal Year: 2013
    The overall goal of this application is to ensure the continued success of the Biliary Atresia Research Consortium (BARC) and the Cholestatic Liver Consortium (CLiC)...
  23. Clinical Center for Cholestatic Liver Disease in Children
    Jorge A Bezerra; Fiscal Year: 2013
    We propose to transition our membership status from the Biliary Atresia Research Consortium (BARC) and the Cholestatic Liver Consortium (CLiC) to the new Childhood Liver Disease Research and Education Network (ChiLDREN)...
  24. Biological Basis of Phenotypes and Clinical Outcomes in Biliary Atresia
    Jorge A Bezerra; Fiscal Year: 2012
    PROJECT SUMMARY/ABSTRACT This application proposes ancillary studies to the NIDDK-Biliary Atresia Research Consortium (BARC)...
  25. The Johns Hopkins Pediatric Liver Center ChiLDREN Grant
    Kathleen Schwarz; Fiscal Year: 2013
    ..2) Expand our ongoing study of the Role of Rotavirus in "Human Biliary Atresia";this is a study in which we are investigating sera from BA and genetic cholestatic control infants (provided ..
  26. BILIARY ATRESIA IN THE INV MOUSE
    David Perlmutter; Fiscal Year: 2000
    Extrahepatic biliary atresia (BA) is a devastating disease of the neonate in which the hepatic and/or common bile duct is obliterated or interrupted...
  27. Genetic Susceptibility to Biliary Atresia
    Nancy Bettina Spinner; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): This is a proposal to identify genetic susceptibility factors for biliary atresia (BA)...
  28. Childhood Liver Disease Research and Education Network Data Coordinating Center
    John C Magee; Fiscal Year: 2013
    ..Specifically we will continue, expand, and merge the efforts of investigators from the Biliary Atresia Research Consortium (BARC) and the Cholestatic Liver Disease Consortium (CLiC) to form the Childhood Liver ..
  29. Mechanisms of fibrosis in pediatric liver diseases
    Rebecca G Wells; Fiscal Year: 2011
    Pediatric liver diseases, most notably biliary atresia, and biliary fibrosis in general differ significantly from other forms of liver fibrosis resulting from parenchymal damage, particularly at the cellular level...
  30. Pathogenic Mechanisms of Virus Induced Biliary Atresia
    GREGORY TIAO; Fiscal Year: 2009
    b>Biliary atresia is the most common cause of neonatal cholestasis and if untreated results in end-stage liver disease (ESLD) and death...
  31. Screening for Genetic Mechanisms for Biliary Atresia
    David Perlmutter; Fiscal Year: 2006
    Extrahepatic biliary atresia (BA) is a devastating condition that affects 1 in 8000 to 1 in 18000 live births with obliteration or discontinuity of the hepatic or common bile ducts at any point from the porta hepatitis to the duodenum...
  32. FETAL BILIARY MALFORMATIONS: INDUCTION AND PREVENTION
    M Thaler; Fiscal Year: 1980
    ..This injury resembles intrahepatic biliary atresia in human newborns...
  33. IMMUNE-MEDIATED BILE DUCT INJURY IN BILIARY ATRESIA
    SHERI KRAMS; Fiscal Year: 2001
    DESCRIPTION (adapted from the application) Biliary atresia is a very serious disease of the newborn in which there is an obliteration or discontinuity of the biliary system...
  34. Hex - a Homeobox Gene Essential for Liver Development
    CLIFFORD BOGUE; Fiscal Year: 2007
    ..Additionally, this research will shed important insight into the pathogenesis of diseases of the biliary tract, including biliary atresia and polycystic liver disease.
  35. Proteomics of Biliary Atresia
    Robert Heuckeroth; Fiscal Year: 2005
    b>Biliary Atresia (BA) is the most serious liver disease of childhood and the most common indication for pediatric liver transplantation. This progressive sclerosing cholangiopathy, however, remains difficult to diagnose and to manage...
  36. MicroRNA in Liver Development
    Joshua Friedman; Fiscal Year: 2009
    ..This class includes biliary atresia, of which approximately 1/3rd of cases are associated with a developmental defect of the bile ducts (1, 2)...
  37. Cytokine Regulation of Liver Growth Hormone Signaling
    Lee Denson; Fiscal Year: 2004
    Children with cholestatic liver diseases including extrahepatic biliary atresia (EHBA) exhibit an acquired growth hormone (GH) resistance, in which GH secretion is normal, but hepatic expression of the GH receptor (GHR) and synthesis of ..
  38. STRATEGIC TARGETING OF VIRAL GENOMES IN BILIARY ATRESIA
    Ross Shepherd; Fiscal Year: 2009
    DESCRIPTION (provided by applicant): We hypothesise that viral infection could play an etiologic role in biliary atresia (BA), one of the most serious digestive/liver diseases of infancy...
  39. Detection of HLA Predominance and Novel HLA Shared Epitopes in Biliary Atresia
    CARA LYNN MACK; Fiscal Year: 2010
    Project Summary/Abstract Biliary atresia (BA) is a progressive, inflammatory, sclerosing cholangiopathy which presents in infancy and leads to bile duct obstruction, biliary cirrhosis and the need for liver transplantation in the ..
  40. The Jaundice Chip: A diagnostic tool for cholestatic liver disease
    Jorge Bezerra; Fiscal Year: 2008
    ..unreadable] [unreadable]..
  41. The plasminogen system and liver repair
    Jorge A Bezerra; Fiscal Year: 2010
    ..These studies are relevant to human health by the identification of potential therapeutic targets to restore liver function in patients and by advancing the current understanding of liver stem cell biology. ..
  42. Molecular determinants of clinical phenotypes in biliary atresia
    Jorge Bezerra; Fiscal Year: 2007
    DESCRIPTION (provided by applicant): [unreadable] This application proposes ancillary studies to the NIDDK-Biliary Atresia Research Consortium (BARC)...
  43. Role of Cytokines and Autoimmunity in Murine BA
    Cara Mack; Fiscal Year: 2005
    b>Biliary atresia (BA) appears to result from a chronic, progressive inflammatory mediated destruction of extrahepatic and intrahepatic bile ducts, leading some to call it "infantile obliterative cholangiopathy"...
  44. Immunologic Mechanism/Destruction/Biliary Artesia
    Cara Mack; Fiscal Year: 2006
    ..I have been intrigued with biliary atresia (BA) and its possible mechanisms of pathogenesis since my exposure to this disease in pediatric residency...
  45. OXIDANT HEPATIC INJURY IN CHOLESTASIS
    Ronald Sokol; Fiscal Year: 2005
    ....
  46. Rare Liver Disease Network
    Ronald Sokol; Fiscal Year: 2005
    ..The RLDN will be a full partner in the Rare Diseases Clinical Research Network and will participate collaboratively with the other Clinical Research Centers and the Data and Technology Coordinating Center. ..
  47. Occult Hepatitis B
    Michael Torbenson; Fiscal Year: 2007
    ..abstract_text> ..
  48. Functional Outcomes in Pediatric Liver Transplantation
    Estella Alonso; Fiscal Year: 2009
    ....
  49. INTERLEUKIN 6 AND HUMAN BILIARY EPITHELIAL PROLIFERATION
    Anthony Demetris; Fiscal Year: 2006
    ..abstract_text> ..
  50. Alcohol and HIV protease inhibitors interactions
    Dennis Feierman; Fiscal Year: 2003
    ..Because of the importance of CYP3A4 with respect to HIV-PI drug metabolism, and its induction by ethanol, the interactions of HIV-PI and ethanol are of clinical importance and are the major focus of this proposal. ..
  51. ETIOLOGY OF GROWTH FAILURE IN BILIARY ATRESIA
    Barbara Haber; Fiscal Year: 2002
    ..from the application) The aim of this proposal is to determine the basis of poor growth for children with biliary atresia (BA) and to ascertain if these patients are likely to benefit from the anabolic and growth promoting effects ..
  52. Genetic Analysis of Zebrafish Biliary Mutants
    Randolph Matthews; Fiscal Year: 2008
    ..These studies in zebrafish may provide clues as to biliary development in mammals and to genetic contributions to cholestatic diseases in humans [unreadable] [unreadable]..
  53. NEWBORN GENETIC SCREENING:FOR WHOSE BENEFIT?
    Lainie Ross; Fiscal Year: 2005
    ..OUTCOMES: The major outcomes will be a series of peer-reviewed articles and a full-length book entitled Newborn Genetic Screening: In whose Interest? For whose Benefit? ..