Zobrazeno 1 - 10
of 232
pro vyhledávání: '"James E, Heubi"'
Autor:
Jing Zhao, Kenneth D. R. Setchell, Ying Gong, Yinghua Sun, Ping Zhang, James E. Heubi, Lingjuan Fang, Yi Lu, Xinbao Xie, Jingyu Gong, Jian-She Wang
Publikováno v:
Orphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-15 (2021)
Abstract Background Biallelic variants in HSD3B7 cause 3β-hydroxy-Δ5-C27-steroid oxidoreductase (HSD3B7) deficiency, a life-threatening but treatable liver disease. The goal of this study was to obtain detailed information on the correlation betwee
Externí odkaz:
https://doaj.org/article/5f7256cd653545d5b45ec79e65978242
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:
Jason T. Blackard, Jacqueline M. Knapke, Patrick H. Ryan, Stephanie Schuckman, Jennifer Veevers, William D. Hardie, James E. Heubi
Publikováno v:
Journal of Clinical and Translational Science, Vol 6 (2022)
Abstract Introduction: Research is an important aspect of many medical students’ training. However, many medical students are not required to complete a scholarly project, and formal research training is often fragmented across the medical school
Externí odkaz:
https://doaj.org/article/95324d86673540139a2d1b6868cbe35c
Autor:
James E. Heubi, Warren P. Bishop
Publikováno v:
Case Reports in Gastroenterology, Vol 12, Iss 3, Pp 661-670 (2018)
Zellweger spectrum disorders (ZSDs) are a subgroup of peroxisomal biogenesis disorders with a generalized defect in peroxisome function. Liver disease in ZSDs has been associated with the lack of peroxisomal β-oxidation of C27-bile acid intermediate
Externí odkaz:
https://doaj.org/article/c13b0a77434d49bebbd2d6d80b0b8d38
Autor:
Heidi A. Hanson, William W. Hay, Jonathan N. Tobin, Shari L. Barkin, Mark Atkins, Margaret R. Karagas, Ann M. Dozier, Cynthia Wetmore, Michael W. Konstan, James E. Heubi
Publikováno v:
Journal of Clinical and Translational Science, Vol 2, Pp 156-162 (2018)
IntroductionEarly life exposures affect health and disease across the life course and potentially across multiple generations. The Clinical and Translational Research Institutes (CTSIs) offer an opportunity to utilize and link existing databases to c
Externí odkaz:
https://doaj.org/article/3194f0ee9f5340a584415fa292cb9b7f
Publikováno v:
Case Reports in Gastroenterology, Vol 12, Iss 2, Pp 360-372 (2018)
Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal
Externí odkaz:
https://doaj.org/article/a2927b923ef44448885a1d530884080f
Autor:
Philip J. Rosenthal, Kathleen M. Loomes, Robert H. Squires, Saul J. Karpen, Simon Horslen, John C. Magee, Emily M. Fredericks, Wen Ye, Vicky L. Ng, Ronald J. Sokol, Binita M. Kamath, Kasper S. Wang, Estella M. Alonso, Jean P. Molleston, James E. Heubi, Daniel H. Leung, Kieran Hawthorne, Lisa G. Sorensen
Publikováno v:
Journal of Pediatric Gastroenterology and Nutrition
Objective: To evaluate neurodevelopmental status among children with inherited cholestatic liver diseases with native liver and variables predictive of impairment. Methods: Participants with Alagille syndrome (ALGS), progressive familial intrahepatic
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5047fef9e423ff8915578f6a4b13dfc2
https://doi.org/10.1097/mpg.0000000000003337
https://doi.org/10.1097/mpg.0000000000003337
Autor:
Kenneth D. R., Setchell *, Nadine M., Brown, Suzanne, Summer, Eileen C., King, James E., Heubi, Sidney, Cole, Trish, Guy, Bevan, Hokin
Publikováno v:
In The Journal of Nutrition December 2013 143(12):1950-1958
Autor:
Ilia Ichetovkin, Philip J. Rosenthal, John J. Alexander, Richard J. Thompson, James E. Heubi, Saul J. Karpen, Shelley Dunn, Ronald J. Sokol, Binita M. Kamath
Publikováno v:
Journal of Pediatric Gastroenterology & Nutrition. 72:654-660
OBJECTIVES Cholestasis is caused by a wide variety of etiologies, often genetic in origin. Broad overlap in clinical presentations, particularly in newborns, renders prioritizing diagnostic investigations challenging. In this setting, a timely, compr
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::61fc123bf3a3390313cf011b446ebe7c
https://doi.org/10.1097/mpg.0000000000003094
https://doi.org/10.1097/mpg.0000000000003094
Autor:
Binita M. Kamath, Estella M. Alonso, James E. Heubi, Saul J. Karpen, Shikha S. Sundaram, Benjamin L. Shneider, Ronald J. Sokol
Publikováno v:
Clinics in liver disease. 26(3)
Malnutrition in children with chronic cholestasis is a prevalent issue and a major risk factor for adverse outcomes. Fat soluble vitamin (FSV) deficiency is an integral feature of cholestatic disease in children, often occurring within the first mont
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3b56b93fb30abd69d9648edc39581584
https://pubmed.ncbi.nlm.nih.gov/35868689
https://pubmed.ncbi.nlm.nih.gov/35868689