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Zhang‐Xu Liu, Neil Kaplowitz – 30 May 2007
Zhang‐Xu Liu, Neil Kaplowitz – 30 May 2007
Hisamitsu Hayashi, Yuichi Sugiyama – 30 May 2007 – Progressive familial intrahepatic cholestasis type 2 (PFIC2) is caused by a mutation in the bile salt export pump (BSEP/ABCB11) gene. We previously reported that E297G and D482G BSEP, which are frequently found mutations in European patients, result in impaired membrane trafficking, whereas both mutants retain their transport function. The dysfunctional localization is probably attributable to the retention of BSEP in endoplasmic reticulum (ER) followed by proteasomal degradation.
Stephen P. Hunt, Hiroshi Kiyama, Andrew J. H. Smith, Christa Laurence – 30 May 2007
Minoru Nakamura – 30 May 2007
Way S. Lee, Ronald J. Sokol – 30 May 2007 – Hepatic involvement is a common feature in childhood mitochondrial hepatopathies, particularly in the neonatal period. Respiratory chain disorders may present as neonatal acute liver failure, hepatic steatohepatitis, cholestasis, or cirrhosis with chronic liver failure of insidious onset. In recent years, specific molecular defects (mutations in nuclear genes such as SCO1, BCS1L, POLG, DGUOK, and MPV17 and the deletion or rearrangement of mitochondrial DNA) have been identified, with the promise of genetic and prenatal diagnosis.
Rebecca J. Roberts, Antonia C. Wells, Esther Unitt, Meryl Griffiths, Angela D. Tasker, Michael E.D. Allison, J. Andrew Bradley, Christopher J.E. Watson – 30 May 2007 – Sirolimus‐induced pneumonitis has emerged as a potentially serious complication in renal transplantation but only single case reports of this condition have been described after liver transplantation (LT), where experience with sirolimus is relatively limited. We report our experience, the largest to date, of sirolimus‐induced pneumonitis following LT.
Adrian M. Di Bisceglie – 30 May 2007
Craig J. McClain, Shirish Barve, Ion Deaciuc – 30 May 2007
James D. Perkins – 30 May 2007 – Hepatic artery thrombosis is an uncommon complication of liver transplantation. However, it is a major indication for re‐transplantation. The use of transcatheter thrombolysis and subsequent surgical revascularization as a graft salvage procedure is discussed. Four of 5 cases (80%) were successful in re‐establishing flow and uncovering underlying arterial anatomic defects. None were treated definitively by endoluminal measures due to an inability to resolve the underlying anatomic defects.
Neeraj Saraf, M. Isabel Fiel, Graciela DeBoccardo, Sukru Emre, Thomas D. Schiano – 30 May 2007 – Early histological recurrence of hepatitis C after liver transplantation (LT) has a negative impact on patient and graft survival. We report a case of histological recurrence of HCV occurring in the second week after LT. A 75‐year‐old woman with chronic HCV and hepatocellular carcinoma underwent LT with an organ from a 75‐year‐old HCV‐negative deceased donor.