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.

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.

Emricasan (IDN‐6556) Lowers Portal Pressure in Patients With Compensated Cirrhosis and Severe Portal Hypertension

Guadalupe Garcia‐Tsao, Michael Fuchs, Mitchell Shiffman, Brian B. Borg, Nikolaos Pyrsopoulos, Kirti Shetty, Juan F. Gallegos‐Orozco, K. Rajender Reddy, Eyob Feyssa, Jean L. Chan, Mason Yamashita, James M. Robinson, Alfred P. Spada, David T. Hagerty, Jaime Bosch – 31 July 2018 – Caspases play a central role in apoptosis, inflammation, and fibrosis. They produce hemodynamically active, proinflammatory microparticles that cause intrahepatic inflammation, vasoconstriction, and extrahepatic splanchnic vasodilation.

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.

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

Pierre‐Yves Boëlle, Dominique Debray, Loic Guillot, Annick Clement, Harriet Corvol, on behalf of the French CF Modifier Gene Study Investigators – 30 July 2018 – Cystic fibrosis (CF)–related liver disease (CFLD) is a common symptom in patients with CF. However, its prevalence, risk factors, and evolution are unclear. We analyzed a large database of patients with CF to investigate the incidence of CFLD, its related risk factors, and the use and effect of ursodeoxycholic acid (UDCA) treatment.

Combined Hypothermic and Normothermic Machine Perfusion Improves Functional Recovery of Extended Criteria Donor Livers

Yuri L. Boteon, Richard W. Laing, Andrea Schlegel, Lorraine Wallace, Amanda Smith, Joseph Attard, Ricky H. Bhogal, Desley A. H. Neil, Stefan Hübscher, M. Thamara P. R. Perera, Darius F. Mirza, Simon C. Afford, Hynek Mergental – 30 July 2018 – Hypothermic oxygenated perfusion (HOPE) and normothermic perfusion are seen as distinct techniques of ex situ machine perfusion of the liver. We aimed to demonstrate the feasibility of combining both techniques and whether it would improve functional parameters of donor livers into transplant standards.

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

Pierre‐Yves Boëlle, Dominique Debray, Loic Guillot, Annick Clement, Harriet Corvol, on behalf of the French CF Modifier Gene Study Investigators – 30 July 2018 – Cystic fibrosis (CF)–related liver disease (CFLD) is a common symptom in patients with CF. However, its prevalence, risk factors, and evolution are unclear. We analyzed a large database of patients with CF to investigate the incidence of CFLD, its related risk factors, and the use and effect of ursodeoxycholic acid (UDCA) treatment.

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