Masoud Mokhtarani
Overview
Explore the profile of Masoud Mokhtarani including associated specialties, affiliations and a list of published articles.
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14
Citations
633
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Recent Articles
1.
Ghabril M, Zupanets I, Vierling J, Mantry P, Rockey D, Wolf D, et al.
Clin Pharmacol Drug Dev
. 2016 Apr;
2(3):278-84.
PMID: 27121790
Glycerol tri-(4-phenylbutyrate) (glycerol phenylbutyrate, GPB, HPN-100) mediates waste nitrogen excretion through conjugation with glutamine to form phenylacetylglutamine which is excreted in urine. This pilot study was performed to assess tolerability...
2.
Hook D, Diaz G, Lee B, Bartley J, Longo N, Berquist W, et al.
Mol Genet Metab Rep
. 2016 Mar;
6:34-40.
PMID: 27014577
Background: Little prospectively collected data are available comparing the dietary intake of urea cycle disorder (UCD) patients to UCD treatment guidelines or to healthy individuals. Objective: To examine the protein...
3.
Vierling J, Mokhtarani M, Brown Jr R, Mantry P, Rockey D, Ghabril M, et al.
Clin Gastroenterol Hepatol
. 2015 Dec;
14(6):903-906.e1.
PMID: 26707685
Background & Aims: There is controversy over the use of measuring blood levels of ammonia (NH3) in the management of patients with overt hepatic encephalopathy (HE). Methods: We performed a...
4.
Nagamani S, Diaz G, Rhead W, Berry S, Le Mons C, Lichter-Konecki U, et al.
Mol Genet Metab
. 2015 Aug;
116(1-2):29-34.
PMID: 26296711
Background: Health 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...
5.
Lee B, Diaz G, Rhead W, Lichter-Konecki U, Feigenbaum A, Berry S, et al.
Genet Med
. 2014 Dec;
17(7):561-8.
PMID: 25503497
Purpose: The aim of this study was to examine predictors of ammonia exposure and hyperammonemic crises in patients with urea cycle disorders. Methods: The relationships between fasting ammonia, daily ammonia...
6.
Berry S, Lichter-Konecki U, Diaz G, McCandless S, Rhead W, Smith W, et al.
Mol Genet Metab
. 2014 Mar;
112(1):17-24.
PMID: 24630270
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...
7.
Rockey D, Vierling J, Mantry P, Ghabril M, Brown Jr R, Alexeeva O, et al.
Hepatology
. 2013 Jul;
59(3):1073-83.
PMID: 23847109
Unlabelled: 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,...
8.
Smith W, Diaz G, Lichter-Konecki U, Berry S, Harding C, McCandless S, et al.
J Pediatr
. 2013 Jan;
162(6):1228-34, 1234.e1.
PMID: 23324524
Objectives: To examine ammonia levels, pharmacokinetics, and safety of glycerol phenylbutyrate (GPB; also referred to as HPN-100) and sodium phenylbutyrate (NaPBA) in young children with urea cycle disorders (UCDs). Study...
9.
Diaz G, Krivitzky L, Mokhtarani M, Rhead W, Bartley J, Feigenbaum A, et al.
Hepatology
. 2012 Sep;
57(6):2171-9.
PMID: 22961727
Unlabelled: Glycerol phenylbutyrate is under development for treatment of urea cycle disorders (UCDs), rare inherited metabolic disorders manifested by hyperammonemia and neurological impairment. We report the results of a pivotal...
10.
Lane N, Schnitzer T, Birbara C, Mokhtarani M, Shelton D, Smith M, et al.
N Engl J Med
. 2010 Oct;
363(16):1521-31.
PMID: 20942668
Background: Increased expression of nerve growth factor in injured or inflamed tissue is associated with increased pain. This proof-of-concept study was designed to investigate the safety and analgesic efficacy of...