Effect of an oral iron chelator or iron‐deficient diets on uroporphyria in a murine model of porphyria cutanea tarda

Nadia Gorman, Adrian Zaharia, Heidi S. Trask, Juliana G. Szakacs, Nicholas J. Jacobs, Judith M. Jacobs, Dominic Balestra, Jacqueline F. Sinclair, Peter R. Sinclair – 28 November 2007 – Porphyria cutanea tarda is a liver disease characterized by elevated hepatic iron and excessive production of uroporphyrin (URO). Phlebotomy is an effective treatment that probably acts by reducing hepatic iron. Here we used Hfe(−/−) mice to compare the effects on hepatic URO accumulation of two different methods of hepatic iron depletion: iron chelation using deferiprone (L1) versus iron‐deficient diets.

Quality of life and everyday activities in patients with primary biliary cirrhosis

Carlo Selmi, M. Eric Gershwin, Keith D. Lindor, Howard J. Worman, Ellen B. Gold, Mitchell Watnik, Jessica Utts, Pietro Invernizzi, Marshall M. Kaplan, John M. Vierling, Christopher L. Bowlus, Marina G. Silveira, Ilaria Bossi, USA PBC Epidemiology Group – 28 November 2007 – Primary biliary cirrhosis (PBC) is generally a slowly progressive disease that may lead to cirrhosis and liver failure.

Dendritic cells, the liver, and transplantation

Tina L. Sumpter, Masanori Abe, Daisuke Tokita, Angus W. Thomson – 28 November 2007 – Interstitial liver dendritic cells (DCs) exhibit phenotypic diversity and functional plasticity. They play important roles in both innate and adaptive immunity. Their comparatively low inherent T cell stimulatory ability and the outcome of their interactions with CD4+ and CD8+ T cells, as well as with natural killer (NK) T cells and NK cells within the liver, may contribute to regulation of hepatic inflammatory responses and liver allograft outcome.

Scavenger receptor class B type I is a key host factor for hepatitis C virus infection required for an entry step closely linked to CD81

Mirjam B. Zeisel, George Koutsoudakis, Eva K. Schnober, Anita Haberstroh, Hubert E. Blum, François‐Loïc Cosset, Takaji Wakita, Daniel Jaeck, Michel Doffoel, Cathy Royer, Eric Soulier, Evelyne Schvoerer, Catherine Schuster, Françoise Stoll‐Keller, Ralf Bartenschlager, Thomas Pietschmann, Heidi Barth, Thomas F. Baumert – 28 November 2007 – Hepatitis C virus (HCV) is a major cause of chronic hepatitis worldwide. Scavenger receptor class B type I (SR‐BI) has been shown to bind HCV envelope glycoprotein E2, participate in entry of HCV pseudotype particles, and modulate HCV infection.

Presentation of an acquired urea cycle disorder post liver transplantation

Marwan Ghabril, Justin Nguyen, David Kramer, Trina Genco, Martin Mai, Barry G. Rosser – 28 November 2007 – The liver's role as the largest organ of metabolism and the unique and often critical function of liver‐specific enzyme pathways imply a greater risk to the recipient of acquiring a donor metabolic disease with liver transplants versus other solid organ transplants. With clinical consequences rarely reported, the frequency of solid organ transplant transfer of metabolic disease is not known.

Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in abernethy malformation. A case report

Sukru Emre, Ronen Arnon, Emil Cohen, Raffaella A. Morotti, Dmitriy Vaysman, Benjamin L. Shneider – 28 November 2007 – Congenital absence of portal vein and extrahepatic portocaval shunt, also referred to as an Abernethy type 1, is a rare malformation of the mesenteric vasculature. A 9‐year‐old girl presented with dyspnea on exertion and orthodeoxia. A diagnosis of an Abernethy malformation type 1b and hepatopulmonary syndrome (HPS) was made, and she underwent auxiliary partial orthotopic liver transplantation (APOLT). Symptoms and signs of HPS resolved 2 months after the operation.

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