Frequency of the cholesteryl ester storage disease common LIPA E8SJM mutation (c.894G>A) in various racial and ethnic groups

Stuart A. Scott, Benny Liu, Irina Nazarenko, Suparna Martis, Julia Kozlitina, Yao Yang, Charina Ramirez, Yumi Kasai, Tommy Hyatt, Inga Peter, Robert J. Desnick – 19 February 2013 – Cholesteryl ester storage disease (CESD) and Wolman disease are autosomal recessive later‐onset and severe infantile disorders, respectively, which result from the deficient activity of lysosomal acid lipase (LAL). LAL is encoded by LIPA (10q23.31) and the most common mutation associated with CESD is an exon 8 splice junction mutation (c.894G>A; E8SJM), which expresses only ∼3%‐5% of normally spliced LAL.

Advances in endoscopic management of biliary tract complications after liver transplantation

Mustafa A. Arain, Rajeev Attam, Martin L. Freeman – 15 February 2013 – Biliary tract complications after liver transplantation (LT) most commonly include biliary leaks, strictures, and stone disease. Living donor recipients and donation after cardiac death recipients are at an increased risk of developing biliary complications. Biliary leaks usually occur early after transplantation, whereas strictures and stone disease occur later. The diagnosis of biliary complications relies on a combination of clinical presentation, laboratory abnormalities, and imaging modalities.

Deficiency of intestinal mucin‐2 ameliorates experimental alcoholic liver disease in mice

Phillipp Hartmann, Peng Chen, Hui J. Wang, Lirui Wang, Declan F. McCole, Katharina Brandl, Peter Stärkel, Clara Belzer, Claus Hellerbrand, Hidekazu Tsukamoto, Samuel B. Ho, Bernd Schnabl – 13 February 2013 – The intestinal mucus layer protects the epithelium from noxious agents, viruses, and pathogenic bacteria present in the gastrointestinal tract. It is composed of mucins, predominantly mucin (Muc) 2, secreted by goblet cells of the intestine.

Sorafenib delays recurrence and metastasis after liver transplantation in a rat model of hepatocellular carcinoma with high expression of phosphorylated extracellular signal‐regulated kinase

Jun Yan, Changjun Tan, Fangming Gu, Jiahao Jiang, Min Xu, Xiuzhen Huang, Zhi Dai, Zheng Wang, Jia Fan, Jian Zhou – 13 February 2013 – Liver transplantation (LT) is one of the curative treatments for hepatocellular carcinoma (HCC). However, cancer recurrence and metastasis after LT are common in some HCC patients with high‐risk factors (even in those within the Milan criteria). It remains unclear whether adjuvant therapy with sorafenib inhibits HCC recurrence and metastasis after LT. Therefore, we performed orthotopic LT in an August Irish Copenhagen (ACI) rat model of HCC.

Hemodynamic response to propranolol in patients with recurrent hepatitis C virus–related cirrhosis after liver transplantation: A case‐control study

Filippo Schepis, Ranka Vukotic, Annalisa Berzigotti, José A. Carrión, Xavier Forns, Juan G. Abraldes, Juan‐Carlos García‐Valdecasas, Miguel Navasa, Juan‐Carlos García‐Pagán, Jaime Bosch – 13 February 2013 – Cirrhosis recurrence is frequent after orthotopic liver transplantation for hepatitis C virus (HCV). Because transplantation causes liver denervation, we hypothesized that the response to propranolol might differ in transplant patients versus nontransplant patients with cirrhosis and portal hypertension.

The mTOR pathway in hepatic malignancies

Mamatha Bhat, Nahum Sonenberg, Gregory J. Gores – 13 February 2013 – The mechanistic/mammalian target of rapamycin (mTOR) pathway plays a critical role in cellular metabolism, growth, and proliferation and has been evaluated as a target for therapy in various malignancies. The mTOR pathway is a major tumor‐initiating pathway in hepatocellular carcinoma, with up‐regulation seen in up to 50% of tumors. Metformin, which represses mTOR signaling by activating adenosine monophosphate–activated protein kinase, has been shown to decrease liver carcinogenesis in population studies.

Early biochemical response to ursodeoxycholic acid and long‐term prognosis of primary biliary cirrhosis: Results of a 14‐year cohort study

Li‐Na Zhang, Tian‐Yan Shi, Xu‐Hua Shi, Li Wang, Yun‐Jiao Yang, Bin Liu, Li‐Xia Gao, Zong‐Wen Shuai, Fang Kong, Hua Chen, Wei Han, Shao‐Mei Han, Yun‐Yun Fei, Quan‐Cai Cui, Qian Wang, Min Shen, Dong Xu, Wen‐Jie Zheng, Yong‐Zhe Li, Wen Zhang, Xuan Zhang, Feng‐Chun Zhang – 13 February 2013 – The biochemical response to ursodeoxycholic acid (UDCA) in primary biliary cirrhosis is a strong predictor of long‐term outcome and thus facilitates the rapid identification of patients needing new therapeutic approaches.

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