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Elsevier电子期刊(11)

在“Elsevier电子期刊”中，命中：11条，耗时：0.0429786 秒

在所有数据库中总计命中：14条

1.Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate

作者：Debra Hook^a ; George A. Diaz^b ; Brendan Lee^c ; James Bartley^a ; Nicola Longo^d ; William Berquist^e ; Cynthia Le Mons^f ; Ingrid Rudolph-Angelich^g ; Marty Porter^g ; Bruce F. Scharschmidt^g ; Masoud Mokhtarani^g ; ^{masoud.mokhtarani@horizonpharma.com" class="auth_mail" title="E-mail the corresponding author}

关键词：UCD ; urea cycle disorder ; GPB ; glycerol phenylbutyrate ; NaPBA ; sodium phenylbutyrate ; NHANESs ; National Health and Nutrition Examination Surveys ; RDA ; Recommended Daily Allowances ; FAO ; Food and Agriculture Organization ; WHO ; World Health Organization ; OTC ; ornithine transcarbamylase

刊名：Molecular Genetics and Metabolism Reports

出版年：2016

2.Self-reported treatment-associated symptoms among patients with urea cycle disorders participating in glycerol phenylbutyrate clinical trials

作者：Sandesh C.S. Nagamani^a ; ^{Nagamani@bcm.edu" class="auth_mail" title="E-mail the corresponding author} ; George A. Diaz^b ; William Rhead^c ; Susan A. Berry^d ; Cynthia Le Mons^e ; Uta Lichter-Konecki^f ; James Bartley^g ; Annette Feigenbaum^h ; Andreas Schulzeⁱ ; Nicola Longo^j ; William Berquist^k ; Renata Gallagher^l ; Dennis Bartholomew^m ; Cary O. Hardingⁿ ; Mark S. Korson^o ; Shawn E. McCandless^p ; Wendy Smith^q ; Jerry Vockley^r ; David Kronn^s ; Robert Zori^t ; Stephen Cederbaum^u ; J. Lawrence Merritt II^v ; Derek Wong^u ; Dion F. Coakley^w ; Bruce F. Scharschmidt^w ; Klara Dickinson^x ; Miguel Marinoⁿ ; Brendan H. Lee^a ; Masoud Mokhtarani^w

关键词：GPB ; glycerol phenylbutyrate ; NaPBA ; sodium phenylbutyrate ; HRQoL ; health-related quality of life ; UCD ; urea cycle disorder

刊名：Molecular Genetics and Metabolism

出版年：2015

3.Glutamine and hyperammonemic crises in patients with urea cycle disorders

作者：B. Lee^a ; ^{blee@bcm.edu" class="auth_mail" title="E-mail the corresponding author} ; G.A. Diaz^b ; W. Rhead^c ; U. Lichter-Konecki^d ; A. Feigenbaum^e ; S.A. Berry^f ; C. Le Mons^g ; J. Bartley^h ; N. Longoⁱ ; S.C. Nagamani^a ; W. Berquist^j ; R.C. Gallagher^k ; C.O. Harding^l ; S.E. McCandless^m ; W. Smithⁿ ; A. Schulze^o ; M. Marino^l ; R. Rowell^p ; D.F. Coakley^q ; M. Mokhtarani^q ; B.F. Scharschmidt^q

关键词：Glycerol phenylbutyrate ; RAVICTI ; Ammonia ; Metabolic disorders

刊名：Molecular Genetics and Metabolism

出版年：2016

4.Glycerol phenylbutyrate treatment in children with urea cycle disorders: Pooled analysis of short and long-term ammonia control and outcomes

作者：Susan A. Berry^a ; ^{berry002@umn.edu" class="auth_mail} ; Uta Lichter-Konecki^b ; George A. Diaz^c ; Shawn E. McCandless^d ; William Rhead^e ; Wendy Smith^f ; Cynthia LeMons^g ; Sandesh C.S. Nagamani^h ; Dion F. Coakleyⁱ ; Masoud Mokhtaraniⁱ ; Bruce F. Scharschmidtⁱ ; Brendan Lee^h

关键词：ASL ; argininosuccinate lyase ; ASS ; argininosuccinate synthase ; AUC0&ndash ; 24 ; 24 ; hour area under the curve ; GPB ; glycerol phenylbutyrate (generic name for glyceryl tri (4-phenylbutyrate) ; also referred to as HPN-100 or RAVICTI®) ; NaPBA ; sodium phenylbutyrate ; OTC ; ornithine transcarbamylase ; PAA ; phenylacetic acid ; PAGN ; phenylacetylglutamine ; PBA ; phenylbutyric acid ; PK ; pharmacokinetic ; UCD ; urea cycle disorder

刊名：Molecular Genetics and Metabolism

出版年：May 2014

5.Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate

作者：Wendy Smith ; MD¹ ; ^{SMITHW@mmc.org} ; George A. Diaz ; MD ; PhD² ; Uta Lichter-Konecki ; MD ; PhD³ ; Susan A. Berry ; MD⁴ ; Cary O. Harding ; MD⁵ ; Shawn E. McCandless ; MD⁶ ; Cindy LeMons⁷ ; Joe Mauney ; MS⁸ ; Klara Dickinson ; BS⁹ ; Dion F. Coakley ; PharmD⁹ ; Tristen Moors ; MS⁹ ; Masoud Mokhtarani ; MD⁹ ; Bruce F. Scharschmidt ; MD⁹ ; Brendan Lee ; MD ; PhD¹⁰ ; ¹¹

关键词：ASA ; Argininosuccinic acid ; ASL ; Argininosuccinate lyase ; ASS ; Argininosuccinate synthetase ; AUC0-24 ; 24-hour area under the curve ; DSMB ; Data and safety monitoring board ; G tube ; Gastrostomy tube ; GPB ; Glycerol phenylbutyrate ; NaPBA ; Sodium phenylbutyrat

刊名：The Journal of Pediatrics

出版年：2013

6.Ammonia control in children with urea cycle disorders (UCDs); Phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate

作者：Uta Lichter-Konecki^a ; ^{ulichter@cnmc.org"" rel=""nofollow} ; G.A. Diaz^b ; J.L. Merritt II^c ; A. Feigenbaum^d ; C. Jomphe^e ; J.F. Marier^e ; M. Beliveau^e ; J. Mauney^f ; K. Dickinson^g ; A. Martinez^g ; M. Mokhtarani^g ; B. Scharschmidt^g ; W. Rhead^h

关键词：Urea cycle disorders ; Ammonia scavengers ; Hyperammonemia ; Phenylacetate ; Sodium phenylbutyrate ; Glycerol phenylbutyrate

刊名：Molecular Genetics and Metabolism

出版年：2011

7.Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio

作者：M. Mokhtarani ; G.A. Diaz ; W. Rhead ; S.A. Berry ; U. Lichter-Konecki ; A. Feigenbaum ; A. Schulze ; N. Longo ; J. Bartley ; W. Berquist ; R. Gallagher ; W. Smith ; S.E. McC ; less ; C. Harding ; D.C. Rockey ; J.M. Vierling ; P. Mantry ; M. Ghabril ; R.S. Brown Jr. ; K. Dickinson ; et al.

关键词：GEE ; generalized estimating equations ; GPB ; glycerol phenylbutyrate (generic name for glyceryl tri (4-phenylbutyrate) ; also referred to as HPN-100 or RAVICTI®) ; HE ; hepatic encephalopathy ; NaPBA ; sodium phenylbutyrate (BUPHENYL®) ; PAA ; phenylacetic acid ; PAA ; PAGN ratio ; ratio of the concentrations in 渭g/mL of PAA to PAGN in plasma ; PAGN ; phenylacetylglutamine ; PBA ; phenylbutyric acid ; SE ; safety extension ; SO ; switchover ; UCD ; urea cycle disorder

刊名：Molecular Genetics and Metabolism

出版年：December, 2013

8.Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders

作者：M. Mokhtarani^a ; ^{Masoud.mokhtarani@hyperiontx.com} ; G.A. Diaz^b ; W. Rhead^c ; U. Lichter-Konecki^d ; J. Bartley^e ; A. Feigenbaum^f ; N. Longo^g ; W. Berquist^h ; S.A. Berryⁱ ; R. Gallagher^j ; D. Bartholomew^k ; C.O. Harding^l ; M.S. Korson^m ; S.E. McCandlessⁿ ; W. Smith^o ; J. Vockley^p ; S. Bart^q ; D. Kronn^r ; R. Zori^s ; S. Cederbaum^t ; N. Dorrani^t ; J.L. Merritt II^u ; Sandesh Sreenath-Nagamani^v ; M. Summar^d ; C. LeMons^w ; K. Dickinson^a ; D.F. Coakley^a ; T.L. Moors^a ; B. Lee^v ; B.F. Scharschmidt^a

关键词：ASL ; argininosuccinate lyase deficiency ; ASS ; argininosuccinate synthetase deficiency ; AUC0&ndash ; 24 ; 24 ; hour area under the curve ; CV % ; coefficient of variation ; DSMB ; Data Safety and Monitoring Board ; GPB ; glycerol phenylbutyrate (generic name for

刊名：Molecular Genetics and Metabolism

出版年：2012

9.Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: Safety, pharmacokinetics and ammonia control

作者：Brendan Lee ; William Rhead ; George A. Diaz ; Bruce F. Scharschmidt ; Asad Mian ; Oleg Shchelochkov ; J.F. Marier ; Martin Beliveau ; Joseph Mauney ; Klara Dickinson ; Antonia Martinez ; Sharron Gargosky ; Masou

关键词：Ammonia ; Clinical trial ; Phenylacetylglutamine ; Phenylbutyrate ; Urea cycle disorders

刊名：Molecular Genetics and Metabolism

出版年：2010

10.Synthesis of triglycerides of phenylbutyric acids by lipase-catalyzed glycerolysis in a solvent-free system

作者：Chun-Sheng Chang ; Pei-Lin Wu

关键词：Glycerolysis ; Lipase ; Phenylbutyrate ; Solvent-free ; Triphenylbutyrin

刊名：Journal of Molecular Catalysis B: Enzymatic

出版年：2009

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