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.

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.

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.

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.

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.

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.

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.

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.

Lucy Golden‐Mason, Hugo R. Rosen – 28 July 2018

Ernesto Cortes, Dariusz Lachowski, Alistair Rice, Antonios Chronopoulos, Benjamin Robinson, Stephen Thorpe, David A Lee, Lucia A Possamai, Haiyun Wang, David J Pinato, Armando E. del Río Hernández – 28 July 2018 – Hepatic stellate cells (HSCs) are essential perisinusoidal cells in both healthy and diseased liver. HSCs modulate extracellular matrix (ECM) homeostasis when quiescent, but in liver fibrosis, HSCs become activated and promote excess deposition of ECM molecules and tissue stiffening via force generation and mechanosensing.