Benjamin L Shneider

Summary

Affiliation: Mount Sinai School of Medicine
Country: USA

Publications

  1. ncbi Progressive intrahepatic cholestasis: mechanisms, diagnosis and therapy
    Benjamin L Shneider
    Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York, NY 10029, USA
    Pediatr Transplant 8:609-12. 2004
  2. ncbi National and regional analysis of exceptions to the Pediatric End-Stage Liver Disease scoring system (2003-2004)
    Benjamin L Shneider
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Liver Transpl 12:40-5. 2006
  3. 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
  4. ncbi Liver disease in autosomal recessive polycystic kidney disease
    Benjamin L Shneider
    Recanati Miller Transplantation Institute, New York, USA
    Pediatr Transplant 9:634-9. 2005
  5. ncbi Expert pediatric opinion on the Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension
    Benjamin Shneider
    Division of Pediatric Hepatology, Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Pediatr Transplant 10:893-907. 2006
  6. ncbi Critical analysis of the pediatric end-stage liver disease scoring system: a single center experience
    Benjamin L Shneider
    Department of Pediatrics, Mount Sinai School of Medicine, OneGustave L Levy Place, New York, NY 10029, USA
    Liver Transpl 11:788-95. 2005
  7. ncbi Pediatric liver transplantation in metabolic disease: clinical decision making
    Benjamin L Shneider
    Department of Pediatric Liver Transplant Medicine, Mount Sinai School of Medicine, New York, New York, USA
    Pediatr Transplant 6:25-9. 2002
  8. ncbi Abnormal concentrations of esterified carnitine in bile: a feature of pediatric acute liver failure with poor prognosis
    Benjamin L Shneider
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Hepatology 41:717-21. 2005
  9. ncbi Rapamycin successfully treats post-transplant autoimmune hepatitis
    Nanda Kerkar
    Recanati Miller Transplant Institute, Department of Pediatrics, Division of Pediatric Hepatology, The Mount Sinai School of Medicine, New York, NY, USA
    Am J Transplant 5:1085-9. 2005
  10. ncbi FTF and LRH-1, two related but different transcription factors in human Caco-2 cells: their different roles in the regulation of bile acid transport
    Debra H Pan
    Department of Pediatrics, Division of Pediatric Hepatology, Mount Sinai School of Medicine, One Gustave L Levy Place, Box 1656, New York, NY 10029, USA
    Biochim Biophys Acta 1732:31-7. 2005

Detail Information

Publications66

  1. ncbi Progressive intrahepatic cholestasis: mechanisms, diagnosis and therapy
    Benjamin L Shneider
    Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York, NY 10029, USA
    Pediatr Transplant 8:609-12. 2004
    ..End-stage liver disease and all of its attendant problems may develop in affected individuals by young adulthood. Optimal therapeutic approaches to PIHC are not well established and disease-specific approaches may be required...
  2. ncbi National and regional analysis of exceptions to the Pediatric End-Stage Liver Disease scoring system (2003-2004)
    Benjamin L Shneider
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Liver Transpl 12:40-5. 2006
    ..Therefore exceptions to the scoring system are required when children compete with adults for donor organs. In conclusion, urgent reassessment of the PELD scoring system is needed to avoid morbidity and mortality in children...
  3. 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...
  4. ncbi Liver disease in autosomal recessive polycystic kidney disease
    Benjamin L Shneider
    Recanati Miller Transplantation Institute, New York, USA
    Pediatr Transplant 9:634-9. 2005
    ..The implications of hepatic disease need to be considered in the decision-making regarding renal transplantation in ARPKD...
  5. ncbi Expert pediatric opinion on the Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension
    Benjamin Shneider
    Division of Pediatric Hepatology, Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Pediatr Transplant 10:893-907. 2006
    ....
  6. ncbi Critical analysis of the pediatric end-stage liver disease scoring system: a single center experience
    Benjamin L Shneider
    Department of Pediatrics, Mount Sinai School of Medicine, OneGustave L Levy Place, New York, NY 10029, USA
    Liver Transpl 11:788-95. 2005
    ..In conclusion the United Network for Organ Sharing policy that permits granting of exceptions has circumvented these problems with the PELD scoring system...
  7. ncbi Pediatric liver transplantation in metabolic disease: clinical decision making
    Benjamin L Shneider
    Department of Pediatric Liver Transplant Medicine, Mount Sinai School of Medicine, New York, New York, USA
    Pediatr Transplant 6:25-9. 2002
    ..The natural history of these disorders are considered in the context of the risks of liver transplantation. Indications, contraindications, and both current and future alternatives to transplantation, are considered...
  8. ncbi Abnormal concentrations of esterified carnitine in bile: a feature of pediatric acute liver failure with poor prognosis
    Benjamin L Shneider
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Hepatology 41:717-21. 2005
    ..In conclusion, the findings raise the hypothesis that abnormalities in fatty acid oxidation may predispose to a worse outcome in acute liver failure...
  9. ncbi Rapamycin successfully treats post-transplant autoimmune hepatitis
    Nanda Kerkar
    Recanati Miller Transplant Institute, Department of Pediatrics, Division of Pediatric Hepatology, The Mount Sinai School of Medicine, New York, NY, USA
    Am J Transplant 5:1085-9. 2005
    ..All six responded to treatment but drug was withdrawn in one patient. Adverse events were minimal. Rapa may prove to be an important addition in the control of autoimmune liver disease...
  10. ncbi FTF and LRH-1, two related but different transcription factors in human Caco-2 cells: their different roles in the regulation of bile acid transport
    Debra H Pan
    Department of Pediatrics, Division of Pediatric Hepatology, Mount Sinai School of Medicine, One Gustave L Levy Place, Box 1656, New York, NY 10029, USA
    Biochim Biophys Acta 1732:31-7. 2005
    ..FTF is not involved directly in bile acid mediated negative feedback regulation of the ASBT...
  11. pmc The membrane protein ATPase class I type 8B member 1 signals through protein kinase C zeta to activate the farnesoid X receptor
    Tamara Frankenberg
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, USA
    Hepatology 48:1896-905. 2008
    ..Inhibition of PKC zeta in Caco-2 cells resulted in activation of the human apical sodium-dependent bile acid transporter promoter...
  12. ncbi c-Fos is a critical mediator of inflammatory-mediated repression of the apical sodium-dependent bile acid transporter
    Ezequiel Neimark
    Division of Pediatric Hepatology, Department of Pediatrics, Mount Sinai School of Medicine, New York, New York 10029, USA
    Gastroenterology 131:554-67. 2006
    ..Ileal bile acid malabsorption is present in Crohn's ileitis. The molecular mechanisms of regulation of the apical sodium-dependent bile acid transporter (ASBT) by inflammatory cytokines in vitro and in vivo are investigated...
  13. ncbi Transitioning health care responsibility from caregivers to patient: a pilot study aiming to facilitate medication adherence during this process
    Rachel A Annunziato
    Department of Psychiatry, Mount Sinai School of Medicine, New York, NY 10029, USA
    Pediatr Transplant 12:309-15. 2008
    ..A randomized, controlled study with a substantial number of enrolled patients is needed to establish the efficacy of this or other approaches...
  14. ncbi Bile acid-induced negative feedback regulation of the human ileal bile acid transporter
    Ezequiel Neimark
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Hepatology 40:149-56. 2004
    ..In conclusion, the human ASBT is positively regulated by retinoic acid. Bile acids induce a negative feedback regulation of human ASBT via an FXR-mediated, SHP-dependent effect upon RAR/RXR activation of ASBT...
  15. ncbi Hepatitis C in children: a quaternary referral center perspective
    Carolina Rumbo
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    J Pediatr Gastroenterol Nutr 43:209-16. 2006
    ..3% of children in the United States, and the general impression is that it has a benign course in childhood. We analyzed a pediatric population with chronic HCV in a quaternary referral center...
  16. pmc beta-Klotho and FGF-15/19 inhibit the apical sodium-dependent bile acid transporter in enterocytes and cholangiocytes
    Jyoti Sinha
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, USA
    Am J Physiol Gastrointest Liver Physiol 295:G996-G1003. 2008
    ..These results indicate that both beta-Klotho and FGF-15/19 repress ASBT in enterocytes and cholangiocytes. These novel signaling pathways need to be considered in analyzing bile acid homeostasis...
  17. ncbi Childhood abuse, nonadherence, and medical outcome in pediatric liver transplant recipients
    Eyal Shemesh
    Department of Psychiatry, Recanati Miller Transplant Institute at Mount Sinai Medical Center, New York, NY, USA
    J Am Acad Child Adolesc Psychiatry 46:1280-9. 2007
    ..The study assessed the relationship between a history of child abuse, nonadherence to medications, and medical outcome in children who had a liver transplant...
  18. ncbi Assessment of depression in medically ill children presenting to pediatric specialty clinics
    Eyal Shemesh
    Department of Psychiatry, Division of Child and Adolescent Psychiatry, NY 10029, USA
    J Am Acad Child Adolesc Psychiatry 44:1249-57. 2005
    ..This approach has not been validated. We evaluated the diagnostic accuracy of the Children's Depression Inventory (CDI) in medically ill children who were referred for evaluation by their pediatricians...
  19. ncbi Parents and clinicians underestimate distress and depression in children who had a transplant
    Eyal Shemesh
    Department of Psychiatry, Mount Sinai Medical Center, New York, NY 10029, USA
    Pediatr Transplant 9:673-9. 2005
    ..The child's report of his or her emotional symptoms should be directly sought post-transplant...
  20. doi Liver transplantation for children with biliary atresia in the pediatric end-stage liver disease era: the role of insurance status
    Ronen Arnon
    Recanati Miller Transplantation Institute, Mount Sinai Medical Center, New York, NY, USA
    Liver Transpl 19:543-50. 2013
    ..Further studies are needed to unearth the reasons for these important differences in outcomes...
  21. pmc Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia
    Benjamin L Shneider
    Department of Pediatrics, Children s Hospital Pittsburgh of UPMC, Pittsburgh, PA 15224, USA
    Pediatrics 130:e607-14. 2012
    ..In this prospective multicenter study, we assessed the prevalence of FSV deficiency in infants with BA who received this FSV/TPGS preparation...
  22. ncbi Utility of azathioprine metabolite measurements in post-transplant recurrent autoimmune and immune-mediated hepatitis
    Carolina Rumbo
    Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York, NY, USA
    Pediatr Transplant 8:571-5. 2004
    ..In younger children the AZA dose requirements were two to four times higher than previously reported standard doses...
  23. ncbi Predominant extrahepatic biliary disease in autosomal recessive polycystic kidney disease: a new association
    Beatrice Goilav
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Pediatr Transplant 10:294-8. 2006
    ..Clinical management of patients with ARPKD should include consideration of potential problems related to extrahepatic bile duct disease...
  24. ncbi Medication adherence in pediatric and adolescent liver transplant recipients
    Eyal Shemesh
    Department of Pediatrics, Mount Sinai Medical Center, New York, New York 10029, USA
    Pediatrics 113:825-32. 2004
    ..There are also no published data about the age of transition at which a child assumes responsibility over taking the medications. This information is important if interventions to improve adherence are contemplated...
  25. ncbi Symptoms of posttraumatic stress disorder in patients who have had a myocardial infarction
    Eyal Shemesh
    Department of Psychiatry, Mount Sinai Medical Center, Box 1230, 1 Gustave L Levy Place, New York, NY 10029, USA
    Psychosomatics 47:231-9. 2006
    ..PTSD may be a treatable risk factor for poor post-MI outcome. Further research is needed to evaluate treatment options...
  26. pmc Hu antigen R and tristetraprolin: counter-regulators of rat apical sodium-dependent bile acid transporter by way of effects on messenger RNA stability
    Frank Chen
    Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
    Hepatology 54:1371-8. 2011
    ..Developmental changes in HuR and TTP protein abundance correlated with previously characterized ontogenic changes in rat ileal and renal ASBT expression...
  27. pmc Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome
    Jeffrey M Saland
    Department of Pediatrics, The Mount Sinai Medical Center, One Gustave L Levy Place, New York, NY 10029, USA
    Clin J Am Soc Nephrol 4:201-6. 2009
    ..A male infant with a family history of thrombotic microangiopathy developed atypical hemolytic uremic syndrome (aHUS)...
  28. ncbi Regulation of the mouse organic solute transporter alpha-beta, Ostalpha-Ostbeta, by bile acids
    Tamara Frankenberg
    Department of Pediatrics, Mt Sinai School of Medicine, New York, NY, USA
    Am J Physiol Gastrointest Liver Physiol 290:G912-22. 2006
    ..Although the positive regulatory pathway appears to be dominant, this arrangement provides a mechanism to finely titrate Ostalpha-Ostbeta expression to the bile acid flux...
  29. doi A hybrid strategy for the prevention of cytomegalovirus-related complications in pediatric liver transplantation recipients
    Rebecca P Madan
    Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY 10461, USA
    Transplantation 87:1318-24. 2009
    ..This single center, retrospective study describes experience with a hybrid prevention strategy combining short-course antiviral prophylaxis and preemptive cytomegalovirus (CMV) polymerase chain reaction (PCR) monitoring...
  30. ncbi Recent advances in pediatric liver transplantation
    Debora Kogan-Liberman
    Department of Pediatrics, Recanati Miller Transplantation Institute, Mount Sinai School of Medicine, Box 1656, One Gustave L Levy Place, New York, NY 10029, USA
    Curr Gastroenterol Rep 4:84-97. 2002
    ..This report highlights many recent published findings representing advances that have led to current successful approaches...
  31. ncbi Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report
    Sukru Emre
    Department of Pediatrics, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA
    Liver Transpl 13:1662-8. 2007
    ..APOLT is a feasible and successful surgical procedure for patients with Abernethy malformation and HPS...
  32. ncbi Cystic fibrosis in an adolescent being evaluated for primary sclerosing cholangitis
    Jyoti Sinha
    Department of Pediatrics, Mount Sinai School of Medicine, New York, New York 10029, USA
    Semin Liver Dis 26:80-4. 2006
  33. ncbi Wilson disease in children: serum aminotransferases and urinary copper on triethylene tetramine dihydrochloride (trientine) treatment
    Ronen Arnon
    Department of Pediatrics, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, New York, USA
    J Pediatr Gastroenterol Nutr 44:596-602. 2007
    ..To evaluate the efficacy of and adherence to trientine and/or zinc therapy in children with Wilson disease (WD)...
  34. ncbi Disorders of bile formation and biliary transport
    Gitit Tomer
    Division of Pediatric Gastroenetrology, One Gustave L Levy Place, Box 1656, Mount Sinai Medical Center, New York, NY 10029, USA
    Gastroenterol Clin North Am 32:839-55, vi. 2003
    ..Defects in bile acid biosynthesis and aminophospholipid transport may be responsive to medical or non-transplant surgical approaches...
  35. ncbi Progressive familial intrahepatic cholestasis, type 1, is associated with decreased farnesoid X receptor activity
    Frank Chen
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Gastroenterology 126:756-64. 2004
    ..The mechanisms by which mutations in the familial intrahepatic cholestasis-1 gene cause Byler's disease (progressive familial intrahepatic cholestasis type 1) are unknown...
  36. ncbi Controversies in the management of pediatric liver disease: Hepatitis B, C and NAFLD: Summary of a single topic conference
    Benjamin L Shneider
    Division of Pediatric Hepatology, Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, USA
    Hepatology 44:1344-54. 2006
  37. pmc Phospholipase D2 mediates signaling by ATPase class I type 8B membrane 1
    Frank Chen
    Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
    J Lipid Res 54:379-85. 2013
    ..These studies show that FIC1 signals to FXR via a signaling pathway including PLD2 and PKCĪ¶...
  38. pmc King's College Hospital Criteria for non-acetaminophen induced acute liver failure in an international cohort of children
    Vinay Sundaram
    Division of Gastroenterology, Hepatology, and Nutrition, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
    J Pediatr 162:319-23.e1. 2013
    ..To validate King's College Hospital criteria (KCHC) in children with non-acetaminophen induced pediatric acute liver failure (PALF) and to determine whether re-optimizing the KCHC would improve predictive accuracy...
  39. doi Portal hypertension in children: expert pediatric opinion on the report of the Baveno v Consensus Workshop on Methodology of Diagnosis and Therapy in Portal Hypertension
    Benjamin L Shneider
    Children s Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA
    Pediatr Transplant 16:426-37. 2012
    ..The opinions of that expert panel are reported...
  40. ncbi Biliary atresia: a transplant perspective
    Benjamin L Shneider
    Department of Pediatrics, Thomas E Starzl Transplantation Institute, Children s Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
    Liver Transpl 13:1482-95. 2007
  41. ncbi Hepatocellular carcinoma and congenital absence of the portal vein in a child receiving growth hormone therapy for turner syndrome
    Raffaella A Morotti
    Lillian and Henry M Stratton Hans Popper Department of Pathology, Mount Sinai School of Medicine, New York, New York 10029, USA
    Semin Liver Dis 27:427-31. 2007
    ....
  42. ncbi Comparison of parent and child reports of emotional trauma symptoms in pediatric outpatient settings
    Eyal Shemesh
    Department of Psychiatry, Mount Sinai Medical Center, 1 Gustave L Levy Pl, Box 1230, New York, NY 10029, USA
    Pediatrics 115:e582-9. 2005
    ..This study evaluated discrepancies in parental versus child reports of the child's emotional trauma symptoms in pediatric medical care settings...
  43. ncbi MR cholangiography in the evaluation of neonatal cholestasis: initial results
    Karen I Norton
    Department of Radiology, Mount Sinai School of Medicine, 1 Gustave L Levy Pl, Box 1234, New York, NY 10029, USA
    Radiology 222:687-91. 2002
    ....
  44. ncbi Wilson's disease and hemochromatosis
    Ezequiel Neimark
    Department of Pediatrics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Adolesc Med Clin 15:175-94, xi. 2004
    ..End-organ disease from iron accumulation is protean and includes progressive damage of the liver, pancreas, skin, heart, and pituitary. It is important to permit therapeutic phlebotomy to commence before the onset of complications...
  45. ncbi Azathioprine metabolite measurements in the treatment of autoimmune hepatitis in pediatric patients: a preliminary report
    Carolina Rumbo
    Division of Pediatric Hepatology, Mount Sanai School of Medicine, New York, New York 10029, USA
    J Pediatr Gastroenterol Nutr 35:391-8. 2002
    ..Potential adverse effects of azathioprine (AZA), such as neutropenia and hepatotoxicity, make its use in autoimmune hepatitis (AIH) problematic...
  46. ncbi Jaundice accompanied by giant cell hepatitis and eosinophilia in childhood
    Nanda Kerkar
    Mount Sinai Medical Center, New York, New York 10029 6574, USA
    Semin Liver Dis 24:107-11. 2004
  47. ncbi Minor salivary gland biopsy in neonatal hemochromatosis
    Shane R Smith
    Department of Otolaryngology, Mount Sinai School of Medicine, New York, NY, USA
    Arch Otolaryngol Head Neck Surg 130:760-3. 2004
    ..However, ready diagnosis remains a problem. A liver biopsy specimen showing siderosis is not specific for hemochromatosis and may be risky in patients with coagulopathy...
  48. ncbi Autoimmune hyperlipidemia in a child with autoimmune hepatitis
    Carolina Rumbo
    Division of Pediatric Hepatology, The Mount Sinai School of Medicine, New York, New York 10029, USA
    J Clin Gastroenterol 35:196-9. 2002
    ..All of her clinical manifestations and biochemical abnormalities dramatically improved with immunosuppression. The overlapping syndrome of systemic lupus erythematosus, autoimmune hepatitis, and autoimmune hyperlipidemia is discussed...
  49. ncbi Rapid evolution of congenital hepatic fibrosis after liver transplantation for acute liver failure: the potential role of extrahepatic factors
    Judith Flores
    Department of Gastroenterology, Hospital de Pediatria, Centro Medico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mexico City, Mexico
    Liver Transpl 14:660-4. 2008
    ..Examination of the explanted liver revealed remarkable progression of the congenital hepatic fibrosis, suggesting that the phenotype of this disease may be dependent in part on extrahepatic factors...
  50. pmc Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conference
    Meral Gunay-Aygun
    National Human Genome Research Institute, the Molecular Imaging Program, National Cancer Institute, The National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892 1851, USA
    J Pediatr 149:159-64. 2006
  51. pmc Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group
    Robert H Squires
    University of Pittsburgh, Children s Hospital of Pittsburgh, PA 15213, USA
    J Pediatr 148:652-658. 2006
    ..To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors...
  52. pmc Growth failure and outcomes in infants with biliary atresia: a report from the Biliary Atresia Research Consortium
    Patricia A DeRusso
    Johns Hopkins School of Medicine and The Johns Hopkins Children s Center, Baltimore, Maryland, USA
    Hepatology 46:1632-8. 2007
    ..The combination of intermediate bilirubin concentrations and poor mean weight z-scores 3 months after HPE was also associated with poor clinical outcome...
  53. ncbi Pediatric liver transplantation: past, present, and future
    Benjamin L Shneider
    Liver Transpl 12:511-3. 2006
  54. ncbi Primary sclerosing cholangitis: summary of a workshop
    Nicholas F Larusso
    Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA
    Hepatology 44:746-64. 2006
    ..Thus, significant opportunities remain for basic and clinical research into the cause, natural history, and therapy of PSC...
  55. ncbi FXR-mediated down-regulation of CYP7A1 dominates LXRalpha in long-term cholesterol-fed NZW rabbits
    Guorong Xu
    Medical Service, Veteran s Administration Medical Center, East Orange, NJ 07018, USA
    J Lipid Res 44:1956-62. 2003
    ..Thus, in rabbits, when FXR and LXRalpha are activated simultaneously, the inhibitory effect of FXR overrides the stimulatory effect of LXRalpha to suppress CYP7A1 mRNA expression...
  56. ncbi Complex inheritance of familial hypercholanemia with associated mutations in TJP2 and BAAT
    Victoria E H Carlton
    Liver Center Laboratory and Department of Medicine, San Francisco General Hospital, University of California San Francisco, California 94110, USA
    Nat Genet 34:91-6. 2003
    ..Mutations in both TJP2 and BAAT may disrupt bile acid transport and circulation. Inheritance seems to be oligogenic, with genotype at BAAT modifying penetrance in individuals homozygous with respect to the mutation in TJP2...
  57. ncbi Intrahepatic cholestasis: summary of an American Association for the Study of Liver Diseases single-topic conference
    William F Balistreri
    Cincinnati Children s Hospital Medical Center and the University of Cincinnati, OH, USA
    Hepatology 42:222-35. 2005
  58. ncbi Inhibition of human m-epoxide hydrolase gene expression in a case of hypercholanemia
    Qin Shi Zhu
    Department of Biochemistry and Molecular Biology, Keck School of Medicine, University of Southern California, 2011 Zonal Avenue, Los Angeles, CA 90033, USA
    Biochim Biophys Acta 1638:208-16. 2003
    ....
  59. ncbi Coordinate expression of regulatory genes differentiates embryonic and perinatal forms of biliary atresia
    Dong Yi Zhang
    Division of Pediatric Gastroenterology, Hepatology, and Nutrition of the Cincinnati Children s Hospital Medical Center and University of Cincinnati, Cincinnati, OH, USA
    Hepatology 39:954-62. 2004
    ..Further studies exploring these biological processes are required to determine the significance of these findings...
  60. ncbi Sex steroid hormones, growth and puberty in children with portal hypertension
    Cynthia M Yuse-Gabriel
    Division of Pediatric Endocrinology, Schneider Children s Hospital of North Shore Long Island Jewish Health System, New Hyde Park, NY, USA
    J Pediatr Endocrinol Metab 17:607-13. 2004
    ..We conclude from this cross-sectional study that children with portal hypertension do not have clinical evidence of growth failure or abnormal puberty, despite subtle changes in the growth axis...
  61. ncbi Novel fibrinogen gamma375 Arg-->Trp mutation (fibrinogen aguadilla) causes hepatic endoplasmic reticulum storage and hypofibrinogenemia
    Stephen O Brennan
    Molecular Pathology Laboratory, Canterbury Health Laboratories, Christchurch, New Zealand
    Hepatology 36:652-8. 2002
    ....
  62. ncbi Genetic modifiers of cholestatic liver disease: an evolving field
    Jorge Bezerra
    J Pediatr Gastroenterol Nutr 42:7-8. 2006
  63. ncbi Hepatic overexpression of murine Abcb11 increases hepatobiliary lipid secretion and reduces hepatic steatosis
    Anne Figge
    Department of Medicine III, University Hospital Aachen, Aachen University, 52074 Aachen, Germany
    J Biol Chem 279:2790-9. 2004
    ..Because these mice are resistant to hepatic lipid accumulation, regulation of Abcb11 may be important for the pathogenesis and treatment of steatohepatitis...
  64. doi Update on progressive familial intrahepatic cholestasis
    Feras T Alissa
    Departments of Pediatrics, University of Pittsburgh School of Medicine and Children s Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania 15213, USA
    J Pediatr Gastroenterol Nutr 46:241-52. 2008
    ..Disease- and genotype-specific prognoses and therapeutic approaches may exist, although much more information needs to be ascertained before clinicians can confidently make decisions based on genetic information...
  65. doi Screening for biliary atresia: A ray of hope
    Benjamin L Shneider
    Hepatology 47:1105-7. 2008
  66. doi Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families
    Sandra S Strautnieks
    Institute of Liver Studies, King s College London School of Medicine at King s College Hospital, London, England
    Gastroenterology 134:1203-14. 2008
    ..We characterized mutations of ABCB11 (encoding BSEP) in such patients and correlated genotypes with residual protein detection and risk of malignancy...