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Results: 1 to 20 of 101

1.

Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate.

Diaz GA, Krivitzky LS, Mokhtarani M, Rhead W, Bartley J, Feigenbaum A, Longo N, Berquist W, Berry SA, Gallagher R, Lichter-Konecki U, Bartholomew D, Harding CO, Cederbaum S, McCandless SE, Smith W, Vockley G, Bart SA, Korson MS, Kronn D, Zori R, Merritt JL 2nd, C S Nagamani S, Mauney J, Lemons C, Dickinson K, Moors TL, Coakley DF, Scharschmidt BF, Lee B.

Hepatology. 2013 Jun;57(6):2171-9. doi: 10.1002/hep.26058. Epub 2013 Jan 3.

PMID:
22961727
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

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

Lichter-Konecki U, Diaz GA, Merritt JL 2nd, Feigenbaum A, Jomphe C, Marier JF, Beliveau M, Mauney J, Dickinson K, Martinez A, Mokhtarani M, Scharschmidt B, Rhead W.

Mol Genet Metab. 2011 Aug;103(4):323-9. doi: 10.1016/j.ymgme.2011.04.013. Epub 2011 May 5.

PMID:
21612962
[PubMed - indexed for MEDLINE]
3.

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

Lee B, Rhead W, Diaz GA, Scharschmidt BF, Mian A, Shchelochkov O, Marier JF, Beliveau M, Mauney J, Dickinson K, Martinez A, Gargosky S, Mokhtarani M, Berry SA.

Mol Genet Metab. 2010 Jul;100(3):221-8. doi: 10.1016/j.ymgme.2010.03.014. Epub 2010 Mar 23.

PMID:
20382058
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

Ammonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate.

Smith W, Diaz GA, Lichter-Konecki U, Berry SA, Harding CO, McCandless SE, LeMons C, Mauney J, Dickinson K, Coakley DF, Moors T, Mokhtarani M, Scharschmidt BF, Lee B.

J Pediatr. 2013 Jun;162(6):1228-34, 1234.e1. doi: 10.1016/j.jpeds.2012.11.084. Epub 2013 Jan 13.

PMID:
23324524
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

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

Mokhtarani M, Diaz GA, Rhead W, Lichter-Konecki U, Bartley J, Feigenbaum A, Longo N, Berquist W, Berry SA, Gallagher R, Bartholomew D, Harding CO, Korson MS, McCandless SE, Smith W, Vockley J, Bart S, Kronn D, Zori R, Cederbaum S, Dorrani N, Merritt JL 2nd, Sreenath-Nagamani S, Summar M, Lemons C, Dickinson K, Coakley DF, Moors TL, Lee B, Scharschmidt BF.

Mol Genet Metab. 2012 Nov;107(3):308-14. doi: 10.1016/j.ymgme.2012.08.006. Epub 2012 Aug 18.

PMID:
22958974
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

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

Berry SA, Lichter-Konecki U, Diaz GA, McCandless SE, Rhead W, Smith W, Lemons C, Nagamani SC, Coakley DF, Mokhtarani M, Scharschmidt BF, Lee B.

Mol Genet Metab. 2014 May;112(1):17-24. doi: 10.1016/j.ymgme.2014.02.007. Epub 2014 Feb 21.

PMID:
24630270
[PubMed - in process]
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.

Mokhtarani M, Diaz GA, Rhead W, Berry SA, Lichter-Konecki U, Feigenbaum A, Schulze A, Longo N, Bartley J, Berquist W, Gallagher R, Smith W, McCandless SE, Harding C, Rockey DC, Vierling JM, Mantry P, Ghabril M, Brown RS Jr, Dickinson K, Moors T, Norris C, Coakley D, Milikien DA, Nagamani SC, Lemons C, Lee B, Scharschmidt BF.

Mol Genet Metab. 2013 Dec;110(4):446-53. doi: 10.1016/j.ymgme.2013.09.017. Epub 2013 Oct 8.

PMID:
24144944
[PubMed - indexed for MEDLINE]
8.

Population pharmacokinetic modeling and dosing simulations of nitrogen-scavenging compounds: disposition of glycerol phenylbutyrate and sodium phenylbutyrate in adult and pediatric patients with urea cycle disorders.

Monteleone JP, Mokhtarani M, Diaz GA, Rhead W, Lichter-Konecki U, Berry SA, Lemons C, Dickinson K, Coakley D, Lee B, Scharschmidt BF.

J Clin Pharmacol. 2013 Jul;53(7):699-710. doi: 10.1002/jcph.92. Epub 2013 Jun 15.

PMID:
23775211
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

Randomized, double-blind, controlled study of glycerol phenylbutyrate in hepatic encephalopathy.

Rockey DC, Vierling JM, Mantry P, Ghabril M, Brown RS Jr, Alexeeva O, Zupanets IA, Grinevich V, Baranovsky A, Dudar L, Fadieienko G, Kharchenko N, Klaryts'ka I, Morozov V, Grewal P, McCashland T, Reddy KG, Reddy KR, Syplyviy V, Bass NM, Dickinson K, Norris C, Coakley D, Mokhtarani M, Scharschmidt BF; HALT-HE Study Group.

Hepatology. 2014 Mar;59(3):1073-83. doi: 10.1002/hep.26611.

PMID:
23847109
[PubMed - indexed for MEDLINE]
10.

Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis.

McGuire BM, Zupanets IA, Lowe ME, Xiao X, Syplyviy VA, Monteleone J, Gargosky S, Dickinson K, Martinez A, Mokhtarani M, Scharschmidt BF.

Hepatology. 2010 Jun;51(6):2077-85. doi: 10.1002/hep.23589.

PMID:
20512995
[PubMed - indexed for MEDLINE]
Free PMC Article
11.

Rare disease clinical research network's urea cycle consortium delivers a successful clinical trial to improve alternate pathway therapy.

Mistry PK.

Hepatology. 2013 Jun;57(6):2100-2. doi: 10.1002/hep.26106. Epub 2013 Jan 7. No abstract available.

PMID:
23080135
[PubMed - indexed for MEDLINE]
12.

Assessment of plasma ammonia and glutamine concentrations in urea cycle disorders.

Serrano M, Ormazábal A, Vilaseca MA, Lambruschini N, Garcia-Romero R, Meavilla S, Perez-Dueñas B, Pineda M, Garcia-Cazorla A, Campistol J, Artuch R.

Clin Biochem. 2011 Jun;44(8-9):742-4. doi: 10.1016/j.clinbiochem.2011.03.136. Epub 2011 Apr 8.

PMID:
21497589
[PubMed - indexed for MEDLINE]
13.

Sodium phenylbutyrate decreases plasma branched-chain amino acids in patients with urea cycle disorders.

Burrage LC, Jain M, Gandolfo L, Lee BH; Members of the Urea Cycle Disorders Consortium, Nagamani SC.

Mol Genet Metab. 2014 Jul 3. pii: S1096-7192(14)00204-2. doi: 10.1016/j.ymgme.2014.06.005. [Epub ahead of print]

PMID:
25042691
[PubMed - as supplied by publisher]
14.

Long-term outcome and intervention of urea cycle disorders in Japan.

Kido J, Nakamura K, Mitsubuchi H, Ohura T, Takayanagi M, Matsuo M, Yoshino M, Shigematsu Y, Yorifuji T, Kasahara M, Horikawa R, Endo F.

J Inherit Metab Dis. 2012 Sep;35(5):777-85. doi: 10.1007/s10545-011-9427-0. Epub 2011 Dec 14.

PMID:
22167275
[PubMed - indexed for MEDLINE]
15.

Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients.

Burlina AB, Ogier H, Korall H, Trefz FK.

Mol Genet Metab. 2001 Apr;72(4):351-5.

PMID:
11286510
[PubMed - indexed for MEDLINE]
16.

Simultaneous LC-MS/MS determination of phenylbutyrate, phenylacetate benzoate and their corresponding metabolites phenylacetylglutamine and hippurate in blood and urine.

Laryea MD, Herebian D, Meissner T, Mayatepek E.

J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S321-8. doi: 10.1007/s10545-010-9172-9. Epub 2010 Aug 7.

PMID:
20694517
[PubMed - indexed for MEDLINE]
17.

Phenylbutyrate improves nitrogen disposal via an alternative pathway without eliciting an increase in protein breakdown and catabolism in control and ornithine transcarbamylase-deficient patients.

Marini JC, Lanpher BC, Scaglia F, O'Brien WE, Sun Q, Garlick PJ, Jahoor F, Lee B.

Am J Clin Nutr. 2011 Jun;93(6):1248-54. doi: 10.3945/ajcn.110.009043. Epub 2011 Apr 13.

PMID:
21490144
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Urea cycle disorder drug approved.

Guha M.

Nat Biotechnol. 2013 Apr;31(4):274. doi: 10.1038/nbt0413-274. No abstract available.

PMID:
23563410
[PubMed - indexed for MEDLINE]
19.

Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients.

Scaglia F, Carter S, O'Brien WE, Lee B.

Mol Genet Metab. 2004 Apr;81 Suppl 1:S79-85.

PMID:
15050979
[PubMed - indexed for MEDLINE]
20.

Severe hyperammonaemia in adults not explained by liver disease.

Walker V.

Ann Clin Biochem. 2012 May;49(Pt 3):214-28. doi: 10.1258/acb.2011.011206. Epub 2012 Feb 20. Review.

PMID:
22349554
[PubMed - indexed for MEDLINE]

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