Zobrazeno 1 - 10
of 2 070
pro vyhledávání: '"J., SOKOL"'
Publikováno v:
Scientific Reports, Vol 13, Iss 1, Pp 1-17 (2023)
Abstract Prolonged parenteral nutrition (PN) can lead to PN associated cholestasis (PNAC). Intestinally derived lipopolysaccharides and infused PN phytosterols lead to activation of NFκB, a key factor in PNAC. Our objective was to determine if inhib
Externí odkaz:
https://doaj.org/article/bcbfa0e51eea4a5a91206133ecae5ba0
Publikováno v:
Scientific Reports, Vol 13, Iss 1, Pp 1-3 (2023)
Externí odkaz:
https://doaj.org/article/34ab8561f9914a09b8f9f35c4a46009d
Autor:
Alexander Chaidez, Zhaoxing Pan, Shikha S. Sundaram, Julia Boster, Mark Lovell, Ronald J. Sokol, Cara L. Mack
Publikováno v:
Hepatology Communications, Vol 6, Iss 11, Pp 3015-3023 (2022)
Abstract Vibration controlled transient elastography (FibroScan) is used to predict the severity of liver fibrosis and steatosis. In pediatrics, few studies have been performed directly comparing liver histologic features with FibroScan liver stiffne
Externí odkaz:
https://doaj.org/article/9a1ecbd957be484a99d2f02a0c744548
Autor:
Richard J. Thompson, Reha Artan, Ulrich Baumann, Pier Luigi Calvo, Piotr Czubkowski, Buket Dalgic, Lorenzo D’Antiga, Angelo Di Giorgio, Özlem Durmaz, Emmanuel Gonzalès, Tassos Grammatikopoulos, Girish Gupte, Winita Hardikar, Roderick H.J. Houwen, Binita M. Kamath, Saul J. Karpen, Florence Lacaille, Alain Lachaux, Elke Lainka, Kathleen M. Loomes, Cara L. Mack, Jan P. Mattsson, Patrick McKiernan, Quanhong Ni, Hasan Özen, Sanjay R. Rajwal, Bertrand Roquelaure, Eyal Shteyer, Etienne Sokal, Ronald J. Sokol, Nisreen Soufi, Ekkehard Sturm, Mary Elizabeth Tessier, Wendy L. van der Woerd, Henkjan J. Verkade, Jennifer M. Vittorio, Terese Wallefors, Natalie Warholic, Qifeng Yu, Patrick Horn, Lise Kjems
Publikováno v:
JHEP Reports, Vol 5, Iss 8, Pp 100782- (2023)
Background & Aims: PEDFIC 2, an ongoing, open-label, 72-week study, evaluates odevixibat, an ileal bile acid transporter inhibitor, in patients with progressive familial intrahepatic cholestasis. Methods: PEDFIC 2 enrolled and dosed 69 patients acros
Externí odkaz:
https://doaj.org/article/943aa785601b46a586443dc2851475ff
Autor:
Benjamin L. Shneider, Binita M. Kamath, John C. Magee, Nathan P. Goodrich, Kathleen M. Loomes, Wen Ye, Cathie Spino, Estella M. Alonso, Jean P. Molleston, Jorge A. Bezerra, Kasper S. Wang, Saul J. Karpen, Simon P. Horslen, Stephen L. Guthery, Philip Rosenthal, Robert H. Squires, Ronald J. Sokol, for the Childhood Liver Disease Research Network (ChiLDReN)
Publikováno v:
Hepatology Communications, Vol 6, Iss 8, Pp 1910-1921 (2022)
Abstract The conduct of long‐term conventional randomized clinical trials in rare diseases is very difficult, making evidenced‐based drug development problematic. As a result, real‐world data/evidence are being used more frequently to assess ne
Externí odkaz:
https://doaj.org/article/4943757ba56340778b553a64b61dfc1f
Autor:
Benjamin L. Shneider, Catherine A. Spino, Binita M. Kamath, John C. Magee, Rosalinda V. Ignacio, Suiyuan Huang, Simon P. Horslen, Jean P. Molleston, Alexander G. Miethke, Rohit Kohli, Daniel H. Leung, M. Kyle Jensen, Kathleen M. Loomes, Saul J. Karpen, Cara Mack, Philip Rosenthal, Robert H. Squires, Alastair Baker, Sanjay Rajwal, Deirdre Kelly, Ronald J. Sokol, Richard J. Thompson, for ChiLDReN and UK IMAGO/IMAGINE Investigators
Publikováno v:
Hepatology Communications, Vol 6, Iss 8, Pp 1922-1933 (2022)
Abstract There is growing interest in, but limited data about, intestinal bile acid transport inhibitors as treatment for cholestatic liver disease. The current analyses combine two similar randomized placebo‐controlled trials with subsequent exten
Externí odkaz:
https://doaj.org/article/ff927fac8c8f49b3872a5335a74a6819
Publikováno v:
Hepatology Communications, Vol 7, Iss 3, Pp e0056-e0056 (2023)
Background and Aims:. Parenteral nutrition (PN) in patients with intestinal failure can lead to cholestasis (PNAC). In a PNAC mouse model, farnesoid X receptor (FXR) agonist (GW4064) treatment alleviated IL-1β-dependent cholestatic liver injury. The
Externí odkaz:
https://doaj.org/article/ab816f859480444d84c350d691e3dd1a
Autor:
Antonia Felzen, Daan B.E. van Wessel, Emmanuel Gonzales, Richard J. Thompson, Irena Jankowska, Benjamin L. Shneider, Etienne Sokal, Tassos Grammatikopoulos, Agustina Kadaristiana, Emmanuel Jacquemin, Anne Spraul, Patryk Lipiński, Piotr Czubkowski, Nathalie Rock, Mohammad Shagrani, Dieter Broering, Emanuele Nicastro, Deirdre Kelly, Gabriella Nebbia, Henrik Arnell, Björn Fischler, Jan B.F. Hulscher, Daniele Serranti, Cigdem Arikan, Esra Polat, Dominique Debray, Florence Lacaille, Cristina Goncalves, Loreto Hierro, Gema Muñoz Bartolo, Yael Mozer-Glassberg, Amer Azaz, Jernej Brecelj, Antal Dezsőfi, Pier Luigi Calvo, Enke Grabhorn, Steffen Hartleif, Wendy J. van der Woerd, Binita M. Kamath, Jian-She Wang, Liting Li, Özlem Durmaz, Nanda Kerkar, Marianne Hørby Jørgensen, Ryan Fischer, Carolina Jimenez-Rivera, Seema Alam, Mara Cananzi, Noemie Laverdure, Cristina Targa Ferreira, Felipe Ordoñez Guerrero, Heng Wang, Valerie Sency, Kyung Mo Kim, Huey-Ling Chen, Elisa de Carvalho, Alexandre Fabre, Jesus Quintero Bernabeu, Aglaia Zellos, Estella M. Alonso, Ronald J. Sokol, Frederick J. Suchy, Kathleen M. Loomes, Patrick J. McKiernan, Philip Rosenthal, Yumirle Turmelle, Simon Horslen, Kathleen Schwarz, Jorge A. Bezerra, Kasper Wang, Bettina E. Hansen, Henkjan J. Verkade
Publikováno v:
JHEP Reports, Vol 5, Iss 2, Pp 100626- (2023)
Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with rela
Externí odkaz:
https://doaj.org/article/bbb97439dbff47198444dd0e83e7f0cb
Autor:
Colin T Shearn, Aimee L Anderson, Michael W Devereaux, Karim C El Kasmi, David J Orlicky, Ronald J Sokol
Publikováno v:
PLoS ONE, Vol 18, Iss 8, p e0290385 (2023)
BackgroundWe have developed a mouse model of Parenteral Nutrition Associated Cholestasis (PNAC) in which combining intestinal inflammation and PN infusion results in cholestasis, hepatic macrophage activation, and transcriptional suppression of bile
Externí odkaz:
https://doaj.org/article/5602d9fb629c4595ba73c8d57a6b453a
Autor:
Colin T. Shearn, Aimee L. Anderson, Colin G. Miller, Reed C. Noyd, Michael W. Devereaux, Nata Balasubramaniyan, David J. Orlicky, Edward E. Schmidt, Ronald J. Sokol
Publikováno v:
Hepatology Communications, Vol 7, Iss 1, Pp e0020-e0020 (2023)
Background and Aims:. Cholestatic liver diseases, including primary sclerosing cholangitis, are characterized by periportal inflammation with progression to hepatic fibrosis and ultimately cirrhosis. We recently reported that the thioredoxin antioxid
Externí odkaz:
https://doaj.org/article/65bdde1608a44f779026293984db4f20