Zobrazeno 1 - 10
of 30
pro vyhledávání: '"Dion F. Coakley"'
Autor:
Bruce F. Scharschmidt, Jasmohan S. Bajaj, Masoud Mokhtarani, R. T. Frederick, K. Coyne, Marzena Jurek, M. Santoro, Dion F. Coakley, Nathan M. Bass, M. K. Margolis, Marwan Ghabril
Publikováno v:
Metabolic Brain Disease. 31:1081-1093
Clinical management and clinical trials of patients with overt hepatic encephalopathy (OHE) are compromised by lack of standardized and reproducible tools for its clinical diagnosis or for caregiver (CG) identification of OHE manifestations which mer
Autor:
Jitendra Ganju, Marzena Jurek, Bruce F. Scharschmidt, Masoud Mokhtarani, John M. Vierling, Richard Rowell, Dion F. Coakley
Publikováno v:
Pharmaceutical Medicine. 30:95-101
Current approaches to detection of drug-induced liver injury (DILI) are generally based on changes from normal in biochemical liver tests. However, limited information is available regarding the proportion of patients with normal versus abnormal bioc
Autor:
Dion F. Coakley, Bruce F. Scharschmidt, Sandesh C.S. Nagamani, Nicola Longo, Miguel Marino, A. Feigenbaum, Susan A. Berry, James Bartley, Masoud Mokhtarani, William E. Berquist, Andreas Schulze, Shawn E. McCandless, C. Le Mons, Wendy E. Smith, B. Lee, George A. Diaz, William J. Rhead, Renata C. Gallagher, Uta Lichter-Konecki, Richard Rowell, Cary O. Harding
Publikováno v:
Molecular Genetics and Metabolism. 117(1):27-32
Blood ammonia and glutamine levels are used as biomarkers of control in patients with urea cycle disorders (UCDs). This study was undertaken to evaluate glutamine variability and utility as a predictor of hyperammonemic crises (HACs) in UCD patients.
Autor:
George A. Diaz, Dion F. Coakley, William J. Rhead, Shawn E. McCandless, Bruce F. Scharschmidt, Wendy E. Smith, Cynthia LeMons, Sandesh C.S. Nagamani, Uta Lichter-Konecki, Masoud Mokhtarani, Susan A. Berry, Brendan Lee
Publikováno v:
Molecular Genetics and Metabolism. 112:17-24
Objective To evaluate glycerol phenylbutyrate (GPB) in the treatment of pediatric patients with urea cycle disorders (UCDs). Study design UCD patients (n=26) ages 2months through 17years were treated with GPB and sodium phenylbutyrate (NaPBA) in two
Autor:
Don C. Rockey, K. Rajender Reddy, Stephen D. Zucker, Neeral L. Shah, David C. Wolf, Robert S. Rahimi, A. Mendoza, Iryna Klaryts'ka, Dion F. Coakley, Timothy M. McCashland, Larysa Dudar, Charles D. Howell, Galyna Fadieienko, K. Gautham Reddy, O. Khrustalev, Andrey Baranovsky, Vladimir Grinevich, Olga Alexeeva, Marwan Ghabril, Robert O'Shea, Parvez S. Mantry, K. Zhidkov, Priya Grewal, Benedict Maliakkal, T. Zvyagintseva, Masoud Mokhtarani, Christopher B. O'Brien, Bruce F. Scharschmidt, V. Radchenko, B. Berk, A. Frolov, Vasyl Syplyviy, Samuel H. Sigal, Kimberly A Brown, G. Storozhakov, Robert S. Brown, Igor A. Zupanets, Hillel Tobias, Nathan M. Bass, John M. Vierling, Kirti Shetty, Michael R. Lucey, Klara Dickinson, Michael D. Voigt, Nataliya Kharchenko, Vyacheslav Morozov, Steven A. Weinman, Catherine Norris, M. Porayko, Luis A. Balart, Aijaz Ahmed
Publikováno v:
Hepatology (Baltimore, Md.)
Glycerol phenylbutyrate (GPB) lowers ammonia by providing an alternate pathway to urea for waste nitrogen excretion in the form of phenylacetyl glutamine, which is excreted in urine. This randomized, double-blind, placebo-controlled phase II trial en
Autor:
Parvez S. Mantry, Bruce F. Scharschmidt, Masoud Mokhtarani, William E. Berquist, George A. Diaz, Don C. Rockey, Nicola Longo, Marwan Ghabril, John M. Vierling, T. Moors, Douglas A. Milikien, Dion F. Coakley, Cary O. Harding, Sandesh C.S. Nagamani, Uta Lichter-Konecki, Renata C. Gallagher, Brendan Lee, Robert S. Brown, Wilbur L. Smith, Annette Feigenbaum, Cynthia LeMons, Andreas Schulze, J. Bartley, Susan A. Berry, William J. Rhead, Shawn E. McCandless, Klara Dickinson, Catherine Norris
Publikováno v:
Molecular Genetics and Metabolism. 110:446-453
Background Phenylacetic acid (PAA) is the active moiety in sodium phenylbutyrate (NaPBA) and glycerol phenylbutyrate (GPB, HPN-100). Both are approved for treatment of urea cycle disorders (UCDs) — rare genetic disorders characterized by hyperammon
Autor:
B. Lee, Dion F. Coakley, William J. Rhead, George A. Diaz, Klara Dickinson, Uta Lichter-Konecki, Jon P. R. Monteleone, Masoud Mokhtarani, Bruce F. Scharschmidt, Cynthia LeMons, Susan A. Berry
Publikováno v:
The Journal of Clinical Pharmacology. 53:699-710
Sodium phenylbutyrate and glycerol phenylbutyrate mediate waste nitrogen excretion in the form of urinary phenylacetylglutamine (PAGN) in patients with urea cycle disorders (UCDs); rare genetic disorders characterized by impaired urea synthesis and h
Autor:
Marwan Ghabril, Jasmohan S. Bajaj, Benedict Maliakkal, Dion F. Coakley, M. Santoro, John M. Vierling, Marzena Jurek, V.R. Rustgi, Richard Rowell, Bruce F. Scharschmidt, Don C. Rockey, Masoud Mokhtarani, A. M. Di Bisceglie, Charles S. Landis, A. Enriquez
Publikováno v:
Digestive diseases and sciences. 61(6)
Overt hepatic encephalopathy (OHE) is a frequent complication of decompensated cirrhosis. A multicenter prospective observational study was performed to assess the most commonly recorded presenting manifestations of OHE and its associated health-care
Autor:
Brendan Lee, Wendy E. Smith, Susan A. Berry, William E. Berquist, Bruce F. Scharschmidt, Renata C. Gallagher, Uta Lichter-Konecki, Klara Dickinson, Cary O. Harding, Annette Feigenbaum, Stephen D. Cederbaum, Miguel Marino, Andreas Schulze, Derek Wong, Dion F. Coakley, Shawn E. McCandless, George A. Diaz, Masoud Mokhtarani, William J. Rhead, David Kronn, J. Lawrence Merritt, James Bartley, Robert Zori, Sandesh C.S. Nagamani, Mark S. Korson, Dennis Bartholomew, Nicola Longo, Cynthia Le Mons, Jerry Vockley
Publikováno v:
Molecular genetics and metabolism, vol 116, iss 1-2
BackgroundHealth care outcomes have been increasingly assessed through health-related quality of life (HRQoL) measures. While the introduction of nitrogen-scavenging medications has improved survival in patients with urea cycle disorders (UCDs), they
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::54a61bc9b90d2f2874a02a9e892181eb
https://escholarship.org/uc/item/3kk4n3d6
https://escholarship.org/uc/item/3kk4n3d6
Autor:
Masoud Mokhtarani, Wendy E. Smith, B. Lee, Bruce F. Scharschmidt, Shawn E. McCandless, Uta Lichter-Konecki, George A. Diaz, J. Bartley, Cary O. Harding, Dion F. Coakley, C. Le Mons, Susan A. Berry
Publikováno v:
Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports, Vol 5, Iss C, Pp 12-14 (2015)
Molecular Genetics and Metabolism Reports, Vol 5, Iss C, Pp 12-14 (2015)
Urinary phenylacetylglutamine (U-PAGN) concentrations in spot urine samples were analyzed as a dosing biomarker during glycerol phenylbutyrate (GPB) dosing in 68 healthy adults and 66 adult and pediatric patients with urea cycle disorders who partici