In vivo antisense oligonucleotide reduction of NPC1 expression as a novel mouse model for Niemann Pick type C– associated liver disease

Victoria M. Rimkunas, Mark J. Graham, Rosanne M. Crooke, Laura Liscum – 25 April 2008 – Niemann‐Pick type C (NPC) is a fatal autosomal recessive lipidosis that is characterized by lysosomal storage of cholesterol and glycosphingolipids. Patients exhibit prolonged neonatal jaundice, hepatosplenomegaly, and progressive neurodegeneration that generally result in death by the teen years. Most clinical cases are caused by mutations in the NPC1 gene. Current mouse models of NPC are not well suited for studying the liver disease due to the rapidly progressing neurological disease.

Metabolic liver disease in children

Keli Hansen, Simon Horslen – 23 April 2008 – The aim of this article is to provide essential information for hepatologists, who primarily care for adults, regarding liver‐based inborn errors of metabolism with particular reference to those that may be treatable with liver transplantation and to provide adequate references for more in‐depth study should one of these disease states be encountered. Liver Transpl 14:713–733, 2008. © 2008 AASLD.

Ischemic cholangiopathy following liver transplantation from donation after cardiac death donors

Edie Y. Chan, Les C. Olson, James A. Kisthard, James D. Perkins, Ramasamy Bakthavatsalam, Jeffrey B. Halldorson, Jorge D. Reyes, Anne M. Larson, Adam E. Levy – 23 April 2008 – The use of donation after cardiac death (DCD) donor hepatic allografts is becoming more widespread; however, there have been published reports of increased graft failure from specific complications associated with this type of allograft. The complication of ischemic cholangiopathy (IC) has been reported to occur more frequently after the use of DCD hepatic allografts.

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