Outcomes in children who underwent transplantation for autoimmune hepatitis

Steven R. Martin, Fernando Alvarez, Ravinder Anand, Changhong Song, Wanrong Yin – 6 January 2011 – The outcomes of 113 children with autoimmune hepatitis (AIH), registered with Studies of Pediatric Liver Transplantation and who underwent transplantation between 1995 and 2006, were compared with those who underwent transplantation for other diagnoses (non‐AIH). A total of 4.9% of liver transplants were for AIH; 81% of these patients had AIH type 1 and most underwent transplantation for complications of chronic disease (60%), the majority in females (72%).

The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: A viable mouse model for mitochondrial hepatopathy

Per Levéen, Heike Kotarsky, Matthias Mörgelin, Riitta Karikoski, Eskil Elmér, Vineta Fellman – 28 December 2010 – Mitochondrial dysfunction is an important cause for neonatal liver disease. Disruption of genes encoding oxidative phosphorylation (OXPHOS) components usually causes embryonic lethality, and thus few disease models are available. We developed a mouse model for GRACILE syndrome, a neonatal mitochondrial disease with liver and kidney involvement, caused by a homozygous BCS1L mutation (232A>G).

Early predictors of corticosteroid treatment failure in icteric presentations of autoimmune hepatitis

Andrew D. Yeoman, Rachel H. Westbrook, Yoh Zen, Paola Maninchedda, Bernard C. Portmann, John Devlin, John G. O'Grady, Phillip M. Harrison, Michael A. Heneghan – 22 December 2010 – Autoimmune hepatitis (AIH) typically responds to treatment in 90% of patients. Early prediction of treatment outcome would be advantageous in clinical practice. We evaluated whether parameters at initiation of therapy or changes in these parameters at day 3 and day 7 following corticosteroid initiation predicted treatment failure.

Molecular therapy for obesity and diabetes based on a long‐term increase in hepatic fatty‐acid oxidation

Josep M. Orellana‐Gavaldà, Laura Herrero, Maria Ida Malandrino, Astrid Pañeda, Maria Sol Rodríguez‐Peña, Harald Petry, Guillermina Asins, Sander Van Deventer, Fausto G. Hegardt, Dolors Serra – 22 December 2010 – Obesity‐induced insulin resistance is associated with both ectopic lipid deposition and chronic, low‐grade adipose tissue inflammation. Despite their excess fat, obese individuals show lower fatty‐acid oxidation (FAO) rates. This has raised the question of whether burning off the excess fat could improve the obese metabolic phenotype.

HepaRG cells: A human model to study mechanisms of acetaminophen hepatotoxicity

Mitchell R. McGill, Hui‐Min Yan, Anup Ramachandran, Gordon J. Murray, Douglas E. Rollins, Hartmut Jaeschke – 17 December 2010 – Acetaminophen (APAP) overdose is the leading cause of acute liver failure in Western countries. In the last four decades much progress has been made in our understanding of APAP‐induced liver injury through rodent studies. However, some differences exist in the time course of injury between rodents and humans. To study the mechanism of APAP hepatotoxicity in humans, a human‐relevant in vitro system is needed.

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