Patient‐Reported Outcomes and the Economic Effects of Nonalcoholic Fatty Liver Disease and Nonalcoholic Steatohepatitis: The Value Proposition

Zobair M. Younossi – 2 August 2018 – Nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH) can progress to cirrhosis, hepatocellular carcinoma (HCC), death, or liver transplantation. NASH has also been associated with impaired health‐related quality of life and poses a significant economic burden.

Plasma From Patients Undergoing Liver Transplantation Is Resistant to Anticoagulant Activity of Soluble Thrombomodulin

Laura C. Burlage, Sarah Bos, Jelle Adelmeijer, Takumi Sakai, Robert J. Porte, Ton Lisman – 1 August 2018 – Recombinant human soluble thrombomodulin (ART‐123) is an anticoagulant and anti‐inflammatory agent clinically used for treatment of disseminated intravascular coagulation. Preclinical studies have shown that ART‐123 reduces hepatic ischemia/reperfusion. Although ART‐123 may therefore have clinical benefit in orthotopic liver transplantation, the substantial alterations in the hemostatic system may complicate its use in this setting.

Increasing Liver Transplantation Wait‐List Dropout for Hepatocellular Carcinoma With Widening Geographical Disparities: Implications for Organ Allocation

Neil Mehta, Jennifer L. Dodge, Ryutaro Hirose, John P. Roberts, Francis Y. Yao – 1 August 2018 – Given the increasing incidence of hepatocellular carcinoma (HCC) and regional variation in liver transplantation (LT) rates for HCC, we investigated temporal and geographic disparities in LT and wait‐list dropout. LT candidates receiving Model for End‐Stage Liver Disease (MELD) exception from 2005 to 2014 were identified from the United Network for Organ Sharing database (n = 14,320). Temporal differences were compared across 2 eras (2005‐2009 and 2010‐2014).

HSP90 Inhibition Drives Degradation of FGFR2 Fusion Proteins: Implications for Treatment of Cholangiocarcinoma

Dante Lamberti, Giulia Cristinziano, Manuela Porru, Carlo Leonetti, Jan B. Egan, Chang‐Xin Shi, Simonetta Buglioni, Carla A. Amoreo, Loriana Castellani, Mitesh J. Borad, Stefano Alemà, Sergio Anastasi, Oreste Segatto – 1 August 2018 – About 15% of intrahepatic cholangiocarcinomas (ICCs) express constitutively active fibroblast growth factor receptor 2 (FGFR2) fusion proteins (FFs) generated by chromosomal translocations.

Idiopathic Portal Hypertension

Virginia Hernández‐Gea, Anna Baiges, Fanny Turon, Juan Carlos Garcia‐Pagán – 31 July 2018 – Idiopathic portal hypertension (IPH) is a rare disorder characterized by clinical portal hypertension in the absence of a recognizable cause such as cirrhosis. Laboratory tests often reveal a preserved liver function with anemia, leukopenia, and thrombocytopenia due to splenomegaly. Imaging studies reveal signs of portal hypertension, whereas liver stiffness and portal pressure values are usually normal or slightly elevated.

Dual‐Specificity Phosphatase 9 Protects Against Nonalcoholic Fatty Liver Disease in Mice Through ASK1 Suppression

Ping Ye, Mei Xiang, Hua Liao, Jijun Liu, Hongbo Luo, Yayun Wang, Ling Huang, Manhua Chen, Jiahong Xia – 31 July 2018 – Nonalcoholic fatty liver disease (NAFLD), ranging from nonalcoholic fatty liver to nonalcoholic steatohepatitis (NASH), is the leading cause of chronic liver diseases. Until now, no medications for NAFLD have been approved by relevant governmental agencies. Dual‐specificity phosphatase 9 (Dusp9) is a member of the DUSP protein family. Dusp9 is expressed in insulin‐sensitive tissues, and its expression may be modified with the development of insulin resistance (IR).

Bone Density in Children With Chronic Liver Disease Correlates With Growth and Cholestasis

Kathleen M. Loomes, Cathie Spino, Nathan P. Goodrich, Thomas N. Hangartner, Amanda E. Marker, James E. Heubi, Binita M. Kamath, Benjamin L. Shneider, Philip Rosenthal, Paula M. Hertel, Saul J. Karpen, Jean P. Molleston, Karen F. Murray, Kathleen B. Schwarz, Robert H. Squires, Jeffrey Teckman, Yumirle P. Turmelle, Estella M. Alonso, Averell H. Sherker, John C. Magee, Ronald J. Sokol, the Childhood Liver Disease Research Network – 31 July 2018 – Osteopenia and bone fractures are significant causes of morbidity in children with cholestatic liver disease.

Treatment Barriers in Portopulmonary Hypertension

Batool AbuHalimeh, Michael J. Krowka, Adriano R. Tonelli – 31 July 2018 – Portopulmonary hypertension (PoPH) is a form of pulmonary arterial hypertension (PAH) that can develop as a complication of portal hypertension. Treatment of PoPH includes PAH‐specific therapies, and in certain cases, such therapies are necessary to facilitate a successful liver transplantation. A significant number of barriers may limit the adequate treatment of patients with PoPH and explain the poorer survival of these patients when compared to patients with other types of PAH.

Hepatopulmonary Syndrome in Children: A 20‐Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome

Suz Warner, Patrick J. McKiernan, Jane Hartley, Evelyn Ong, Indra D. Mourik, Girish Gupte, Mona Abdel‐Hady, Paolo Muiesan, Thamera Perera, Darius Mirza, Khalid Sharif, Deirdre A. Kelly, Susan V. Beath – 31 July 2018 – Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be overlooked. We report on the presenting symptoms, disease progression, and outcomes after liver transplantation (LT) in children with HPS. Twenty patients were diagnosed with HPS between 1996 and 2016.

DDX24 Mutations Associated With Malformations of Major Vessels to the Viscera

Pengfei Pang, Xiaojun Hu, Bin Zhou, Junjie Mao, Yu Liang, Zaibo Jiang, Mingsheng Huang, Ruihong Liu, Youyong Zhang, Jiesheng Qian, Jinsong Liu, Jinxin Xu, Yaqin Zhang, Maoheng Zu, Yiming Wang, Huanhuan He, Hong Shan – 31 July 2018 – Vascular malformations present diagnostic and treatment challenges. In particular, malformations of vessels to the viscera are often diagnosed late or incorrectly due to the insidious onset and deep location of the disease. Therefore, a better knowledge of the genetic mutations underlying such diseases is needed.

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