Zobrazeno 1 - 10
of 130
pro vyhledávání: '"Cara L. Mack"'
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:
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
Publikováno v:
Gastro Hep Advances, Vol 1, Iss 3, Pp 461-470 (2022)
Background and Aims: Biliary atresia (BA) entails an inflammatory injury of the biliary tree, leading to fibrosis of the extrahepatic and intrahepatic bile ducts. The chronic inflammatory biliary injury may be due to lack of appropriate regulatory T
Externí odkaz:
https://doaj.org/article/06eaf4ff4f404e33a4639589faf7a709
Autor:
Cara L. Mack
Publikováno v:
Frontiers in Immunology, Vol 13 (2022)
The strongest genetic association with autoimmunity is within chromosome 6p21, where the human leukocyte antigen (HLA) complex resides. This review will focus on the HLA associations within pediatric autoimmune hepatitis, autoimmune sclerosing cholan
Externí odkaz:
https://doaj.org/article/2d40f577c35c48c096f1b179a080e127
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
Outcomes of Childhood Cholestasis in Alagille Syndrome: Results of a Multicenter Observational Study
Autor:
Binita M. Kamath, Wen Ye, Nathan P. Goodrich, Kathleen M. Loomes, Rene Romero, James E. Heubi, Daniel H. Leung, Nancy B. Spinner, David A. Piccoli, Estella M. Alonso, Stephen L. Guthery, Saul J. Karpen, Cara L. Mack, Jean P. Molleston, Karen F. Murray, Philip Rosenthal, James E. Squires, Jeffrey Teckman, Kasper S. Wang, Richard Thompson, John C. Magee, Ronald J. Sokol, for the Childhood Liver Disease Research Network (ChiLDReN)
Publikováno v:
Hepatology Communications, Vol 4, Iss 3, Pp 387-398 (2020)
Alagille syndrome (ALGS) is an autosomal dominant multisystem disorder with cholestasis as a defining clinical feature. We sought to characterize hepatic outcomes in a molecularly defined cohort of children with ALGS‐related cholestasis. Two hundre
Externí odkaz:
https://doaj.org/article/447c90d603d64b7688e87239ee217567
Autor:
Dor Yoeli, Yuhuan Luo, Alexander Chaidez, Zhaohui Wang, Megan A. Adams, Christene A. Huang, Cara L. Mack, Nalu Navarro-Alvarez
Publikováno v:
Journal of Clinical and Translational Science, Vol 6, Pp 84-84 (2022)
OBJECTIVES/GOALS: Biliary atresia (BA) is a progressive congenital disease that is characterized by periductular inflammation and fibrosis that leads to bile duct destruction and cholestasis in neonates. Galectin-3 (Gal3) plays a key role in inflamma
Externí odkaz:
https://doaj.org/article/69cc27cafea6499bb7a32de03aa17b7c
Autor:
Benjamin L. Shneider, Cathie Spino, Binita M. Kamath, John C. Magee, Lee M. Bass, Kenneth D. Setchell, Alexander Miethke, Jean P. Molleston, Cara L. Mack, Robert H. Squires, Karen F. Murray, Kathleen M. Loomes, Philip Rosenthal, Saul J. Karpen, Daniel H. Leung, Stephen L. Guthery, Danny Thomas, Averell H. Sherker, Ronald J. Sokol, for the Childhood Liver Disease Research Network
Publikováno v:
Hepatology Communications, Vol 2, Iss 10, Pp 1184-1198 (2018)
Abstract Medically refractory, severe, cholestasis‐induced pruritus in Alagille syndrome may be improved by surgical interruption of the enterohepatic circulation. This multicenter trial (NCT02057692) tested the hypothesis that the intestinal bile
Externí odkaz:
https://doaj.org/article/8ae3fd4f3e8a4abcab02fe41923d46b0
Autor:
Cara L. Mack
Publikováno v:
Cellular and Molecular Gastroenterology and Hepatology, Vol 1, Iss 3, Pp 267-274 (2015)
Biliary atresia (BA) is the most frequent identifiable cause of neonatal cholestasis, and the majority of patients will need liver transplantation for survival. Despite surgical intervention with the Kasai portoenterostomy, significant fibrosis and c
Externí odkaz:
https://doaj.org/article/de3c57017b1b4d91808350398bbe3c4b
Autor:
Richard J Thompson, Henrik Arnell, Reha Artan, Ulrich Baumann, Pier Luigi Calvo, Piotr Czubkowski, Buket Dalgic, Lorenzo D'Antiga, Özlem Durmaz, Björn Fischler, Emmanuel Gonzalès, Tassos Grammatikopoulos, Girish Gupte, Winita Hardikar, Roderick H J Houwen, Binita M Kamath, Saul J Karpen, Lise Kjems, Florence Lacaille, Alain Lachaux, Elke Lainka, Cara L Mack, Jan P Mattsson, Patrick McKiernan, Hasan Özen, Sanjay R Rajwal, Bertrand Roquelaure, Mohammad Shagrani, Eyal Shteyer, Nisreen Soufi, Ekkehard Sturm, Mary Elizabeth Tessier, Henkjan J Verkade, Patrick Horn
Publikováno v:
The lancet. Gastroenterology & hepatology, 7(9), 830-842. HANLEY & BELFUS-ELSEVIER INC
Thompson, R J, Arnell, H, Artan, R, Baumann, U, Calvo, P L, Czubkowski, P, Dalgic, B, D'Antiga, L, Durmaz, Ö, Fischler, B, Gonzalès, E, Grammatikopoulos, T, Gupte, G, Hardikar, W, Houwen, R H J, Kamath, B M, Karpen, S J, Kjems, L, Lacaille, F, Lachaux, A, Lainka, E, Mack, C L, Mattsson, J P, McKiernan, P, Özen, H, Rajwal, S R, Roquelaure, B, Shagrani, M, Shteyer, E, Soufi, N, Sturm, E, Tessier, M E, Verkade, H J & Horn, P 2022, ' Odevixibat treatment in progressive familial intrahepatic cholestasis : a randomised, placebo-controlled, phase 3 trial ', The Lancet Gastroenterology & Hepatology . https://doi.org/10.1016/S2468-1253(22)00093-0
Thompson, R J, Arnell, H, Artan, R, Baumann, U, Calvo, P L, Czubkowski, P, Dalgic, B, D'Antiga, L, Durmaz, Ö, Fischler, B, Gonzalès, E, Grammatikopoulos, T, Gupte, G, Hardikar, W, Houwen, R H J, Kamath, B M, Karpen, S J, Kjems, L, Lacaille, F, Lachaux, A, Lainka, E, Mack, C L, Mattsson, J P, McKiernan, P, Özen, H, Rajwal, S R, Roquelaure, B, Shagrani, M, Shteyer, E, Soufi, N, Sturm, E, Tessier, M E, Verkade, H J & Horn, P 2022, ' Odevixibat treatment in progressive familial intrahepatic cholestasis : a randomised, placebo-controlled, phase 3 trial ', The Lancet Gastroenterology & Hepatology . https://doi.org/10.1016/S2468-1253(22)00093-0
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited paediatric liver diseases resulting from mutations in genes that impact bile secretion. We aimed to evaluate the effects of odevixibat, an ileal bile acid transp