Results 31 to 40 of about 3,820 (178)

Forsaken Foregut: Case Report of Simultaneous Black Esophagus and Ischemic Cholangiopathy

Case Reports in Medicine, 2017
Black esophagus or acute esophageal necrosis rarely occurs after severe hemodynamic compromise or low-flow states. Other contributing factors may include corrosive injury from gastric contents and diminished mucosal repair mechanisms.
Paul A. Cameron, Franzjosef Schweiger
doaj   +1 more source

Recapitulating Cholangiopathy-Associated Necroptotic Cell Death In Vitro Using Human Cholangiocyte OrganoidsSummary

Cellular and Molecular Gastroenterology and Hepatology, 2022
Background & Aims: Liver and bile duct diseases often are associated with extensive cell death of cholangiocytes. Necroptosis represents a common mode of programmed cell death in cholangiopathy, however, detailed mechanistic knowledge is limited ...
Shaojun Shi, Monique M.A. Verstegen, Henk P. Roest, Arif I. Ardisasmita, Wanlu Cao, Floris J.M. Roos, Petra E. de Ruiter, Marije Niemeijer, Qiuwei Pan, Jan N.M. IJzermans, Luc J.W. van der Laan   +10 more
doaj   +1 more source

Expanded Hepatic Progenitor Cells Featured with Aggregation of α‐Synuclein Contribute to Pathologic Bile Duct Regeneration in Biliary Atresia

Advanced Science, EarlyView.
Expanded NCAM1+EpCAM+ hepatic progenitor cells in biliary atresia are characterized by aggregation of α‐synuclein. This pathological protein potentiates cellular susceptibility to GSH‐dependent redox dyshomeostasis, induces unstable biliary cell fate specification, and subsequently drives aberrant biliary regeneration.
Hua Xie, Mengyan Zhu, Zhongxian Zhu, Jiaqi Tang, Ruyi Zhang, Zequan Ding, Junzhi Li, Zhongluan Wu, Paul Kwong Hang Tam, Qianghu Wang, Vincent Chi Hang Lui, Yankai Xia, Weibing Tang   +12 more
wiley   +1 more source

Genotype–Phenotype Correlations of Monoallelic PFIC Variants in Pediatric Liver Disease: A Multicenter Retrospective Cohort Study

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Progressive familial intrahepatic cholestasis (PFIC) is classically caused by biallelic pathogenic variants, yet monoallelic variants of uncertain significance (VUS) in PFIC‐associated genes are increasingly identified in children with cholestasis, creating diagnostic uncertainty.
Brett J. Hoskins, Tiziano Pramparo, Chaowapong Jarasvaraparn, Michael J. Wilsey, Voytek Slowik, Lakshmi Kunam, Janis M. Stoll, Ruben E. Quiros‐Tejeira, Simon Lam, Wikrom Karnsakul   +9 more
wiley   +1 more source

Recent discoveries in microbiota dysbiosis, cholangiocytic factors, and models for studying the pathogenesis of primary sclerosing cholangitis

Open Medicine, 2022
Primary sclerosing cholangitis (PSC) is a cholangiopathy caused by genetic and microenvironmental changes, such as bile homeostasis disorders and microbiota dysbiosis.
Huang Yu, Zhang Shuai, Weng Jie-Feng, Huang Di, Gu Wei-Li   +4 more
doaj   +1 more source

European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) position paper on screening, diagnosis and investigation of paediatric metabolic dysfunction‐associated steatotic liver disease

Journal of Pediatric Gastroenterology and Nutrition, EarlyView.
Abstract Metabolic dysfunction‐associated steatotic liver disease (MASLD) is the most common reason for elevated liver enzymes in children in Europe, affecting more than 5% of all children. Since the last iteration of this position paper, there have been substantial advances in our understanding of the disease.
Jake P. Mann, Sander Lefere, Maarten Buytaert, Anna Alisi, Cigdem Arikan, Jernej Brecelj, Rachel M. Brown, Irene Degrassi, Giulia Fiore, Emer Fitzpatrick, Sven Francque, Christian A. Hudert, Wojciech Janczyk, Lauren Johansen, Bart G. Koot, Anastasia Konidari, Eberhard Lurz, Claudia Mandato, Maria Mercadal‐Hally, Hadar Moran‐Lev, Antonella Mosca, Giusy Ranucci, Maria Rogalidou, Penelope C. Rose, Piotr Socha, Pietro Vajro, Elvira Verduci, Anita C. E. Vreugdenhil, Natalia Zavhorodnia, Aglaia Zellos, Ruth De Bruyne   +30 more
wiley   +1 more source

Inter‐tissue relationships of gene expression in liver, muscle and adipose tissue of children with end‐stage chronic liver disease

Journal of Pediatric Gastroenterology and Nutrition, EarlyView.
Abstract Objectives End‐stage chronic liver disease in children is associated with sarcopenia and aberrant adipose tissue mass. We investigated correlations between liver pathology‐associated gene pathways (fibrosis, inflammation and steatosis) and metabolic genes in muscle and adipose tissue.
Eirini Kyrana, Cheng Zhang, Han Jin, Siamak Salehi, Jonathan C. K. Wells, Maesha Deheragoda, Ragai R. Mitry, Anil Dhawan   +7 more
wiley   +1 more source

Histological Changes in Portal Cavernoma Cholangiopathy

Diagnostics, 2023
Introduction: Portal cavernoma cholangiopathy (PCC)’ refers to abnormalities of the extrahepatic and intrahepatic bile ducts in patients with portal hypertension.
Archana Rastogi, Chhagan Bihari, Shalini L. Thapar, Vikram Bhatia   +3 more
doaj   +1 more source

A hepatic enigma: Pediatric presentation of primary biliary cholangitis

JPGN Reports, EarlyView.
Abstract Primary biliary cholangitis (PBC) is a chronic autoimmune condition characterized by destruction of intrahepatic bile ducts, leading to fibrosis and cirrhosis of the liver. It is an extremely rare pediatric disease with very few pediatric cases reported to date. Here, we report the case of a 14‐year‐old female who presented with elevated liver
Sindhura Kasturi, Rachel Schenker, Nick Shillingford, George Yanni   +3 more
wiley   +1 more source

Biliary fibrosis is an important but neglected pathological feature in hepatobiliary disorders: from molecular mechanisms to clinical implications

Medical Review
Fibrosis resulting from pathological repair secondary to recurrent or persistent tissue damage often leads to organ failure and mortality. Biliary fibrosis is a crucial but easily neglected pathological feature in hepatobiliary disorders, which may ...
Zhao Jinyu, Yue Ping, Mi Ningning, Li Matu, Fu Wenkang, Zhang Xianzhuo, Gao Long, Bai Mingzhen, Tian Liang, Jiang Ningzu, Lu Yawen, Ma Haidong, Dong Chunlu, Zhang Yong, Zhang Hengwei, Zhang Jinduo, Ren Yanxian, Suzuki Azumi, Wong Peng F., Tanaka Kiyohito, Rerknimitr Rungsun, Junger Henrik H., Cheung Tan T., Melloul Emmanuel, Demartines Nicolas, Leung Joseph W., Yao Jia, Yuan Jinqiu, Lin Yanyan, Schlitt Hans J., Meng Wenbo   +30 more
doaj   +1 more source