Zobrazeno 1 - 10
of 312
pro vyhledávání: '"Saul J Karpen"'
Autor:
Koichi Sudo, Amber Delmas-Eliason, Shannon Soucy, Kaitlyn E. Barrack, Jiabao Liu, Akshaya Balasubramanian, Chengyi Jenny Shu, Michael J. James, Courtney L. Hegner, Henry D. Dionne, Alex Rodriguez-Palacios, Henry M. Krause, George A. O’Toole, Saul J. Karpen, Paul A. Dawson, Daniel Schultz, Mark S. Sundrud
Publikováno v:
Cellular and Molecular Gastroenterology and Hepatology, Vol 18, Iss 6, Pp 101392- (2024)
Backgrounds & Aims: Bile acids (BAs) are core gastrointestinal metabolites with dual functions in lipid absorption and cell signaling. BAs circulate between the liver and distal small intestine (i.e., ileum), yet the dynamics through which complex BA
Externí odkaz:
https://doaj.org/article/4c920e38c9f24de48d0c7062be6a9281
Autor:
Rouknuddin Q. Ali, Anne Meyer-Miner, Marie David-Rachel, Fiona J. H. Lee, Benjamin J. Wilkins, Saul J. Karpen, Brian Ciruna, Anand Ghanekar, Binita M. Kamath
Publikováno v:
Disease Models & Mechanisms, Vol 16, Iss 10 (2023)
Externí odkaz:
https://doaj.org/article/d1572e7bda6548719bb88957e5320cf4
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 6, Iss 6, Pp 1413-1424 (2022)
Cirrhotic cardiomyopathy (CCM), detected during two‐dimensional echocardiography (2DE), is prevalent in patients with biliary atresia (BA) awaiting transplant. Whether CCM occurs early in the lives of infants with BA is unknown. The aim of this stu
Externí odkaz:
https://doaj.org/article/e6fd5cc779764fda82f67ba4e863514f
Autor:
Jennifer K. Truong, Jianing Li, Qin Li, Kimberly Pachura, Anuradha Rao, Sanjeev Gumber, Claudia Daniela Fuchs, Andrew P. Feranchak, Saul J. Karpen, Michael Trauner, Paul A. Dawson
Publikováno v:
JCI Insight, Vol 8, Iss 6 (2023)
The pronounced choleretic properties of 24-norUrsodeoxycholic acid (norUDCA) to induce bicarbonate-rich bile secretion have been attributed to its ability to undergo cholehepatic shunting. The goal of this study was to identify the mechanisms underly
Externí odkaz:
https://doaj.org/article/e4cbe8bcbe354f94a29a2c7ee0acf8e0
Autor:
Jennifer K. Truong, Ashley L. Bennett, Caroline Klindt, Ajay C. Donepudi, Sudarshan R. Malla, Kimberly J. Pachura, Alex Zaufel, Tarek Moustafa, Paul A. Dawson, Saul J. Karpen
Publikováno v:
Journal of Lipid Research, Vol 63, Iss 9, Pp 100261- (2022)
Cyp2c70 is the liver enzyme in rodents responsible for synthesis of the primary 6-hydroxylated muricholate bile acid (BA) species. Cyp2c70 KO mice are devoid of protective, hydrophilic muricholic acids, leading to a more human-like BA composition and
Externí odkaz:
https://doaj.org/article/c8b1893ec9fd4770aafc4f38f85d3bae
Autor:
Benjamin L. Shneider, Nathan P. Goodrich, Wen Ye, Cindy Sawyers, Jean P. Molleston, Robert M. Merion, Daniel H. Leung, Saul J. Karpen, Binita M. Kamath, Laurel Cavallo, Kasper Wang, Jeffrey H. Teckman, James E. Squires, Shikha S. Sundaram, Philip Rosenthal, Rene Romero, Karen F. Murray, Kathleen M. Loomes, M. Kyle Jensen, Jorge A. Bezerra, Lee M. Bass, Ronald J. Sokol, John C. Magee, For the Childhood Liver Disease Research Network (ChiLDReN)
Publikováno v:
Hepatology Communications, Vol 4, Iss 11, Pp 1694-1707 (2020)
Elastographic measurement of liver stiffness is of growing importance in the assessment of liver disease. Pediatric experiences with this technique are primarily single center and limited in scope. The Childhood Liver Disease Research Network provide
Externí odkaz:
https://doaj.org/article/647571020799419cbe6477fa8ad3ebe4
Autor:
Binita M. Kamath, Cathie Spino, Richard McLain, John C. Magee, Emily M. Fredericks, Kenneth D. Setchell, Alexander Miethke, Jean P. Molleston, Cara L. Mack, Robert H. Squires, Estella M. Alonso, Karen F. Murray, Kathleen M. Loomes, M. Kyle Jensen, Saul J. Karpen, Philip Rosenthal, Danny Thomas, Ronald J. Sokol, Benjamin L. Shneider, for the Childhood Liver Disease Research Network (ChiLDReN)
Publikováno v:
Hepatology Communications, Vol 4, Iss 7, Pp 1012-1018 (2020)
Pruritus is a debilitating symptom for patients with Alagille syndrome (ALGS). In a previously reported trial of maralixibat, an investigational antipruritic agent, itching was assessed using a digital diary based on twice‐daily caregiver observati
Externí odkaz:
https://doaj.org/article/bb0995bcee5c43aa950f9efac0f78e7d