glycerol phenylbutyrate

01/01/2019 - "Long-term safety and efficacy of glycerol phenylbutyrate for the management of urea cycle disorder patients."
11/01/2017 - "Safety and efficacy of glycerol phenylbutyrate for management of urea cycle disorders in patients aged 2months to 2years."
01/01/2021 - "Glycerol phenylbutyrate efficacy and safety from an open label study in pediatric patients under 2 months of age with urea cycle disorders."
09/01/2015 - "Self-reported treatment-associated symptoms among patients with urea cycle disorders participating in glycerol phenylbutyrate clinical trials."
11/01/2023 - "Transition to glycerol phenylbutyrate for the management of urea cycle disorders: clinical experiences."

01/01/2017 - "Switch from Sodium Phenylbutyrate to Glycerol Phenylbutyrate Improved Metabolic Stability in an Adolescent with Ornithine Transcarbamylase Deficiency."

03/01/2014 - "Randomized, double-blind, controlled study of glycerol phenylbutyrate in hepatic encephalopathy."
06/01/2021 - "Glycerol phenylbutyrate was the only agent with a beneficial effect on both hyperammonaemia and hepatic encephalopathy. "
08/01/2022 - "This case illustrates the successful use of glycerol phenylbutyrate in a noncirrhotic patient with Portosystemic shunt and minimal hepatic encephalopathy refractory to conventional management strategies."
12/01/2021 - "Sodium phenylacetate or glycerol phenylbutyrate is approved for urea cycle disorders, but limited clinical data are available for hepatic encephalopathy. "
07/01/2013 - "Glycerol Phenylbutyrate in Patients With Cirrhosis and Episodic Hepatic Encephalopathy: A Pilot Study of Safety and Effect on Venous Ammonia Concentration."

01/01/2016 - "In analysis of data from a phase 2 study of the effects of glycerol phenylbutyrate in patients with cirrhosis, we found that fasting levels of NH3 in blood can identify patients at risk for HE-related morbidity. "
01/01/2016 - "We performed a retrospective analysis of data from a randomized, double-blind study of 178 patients with cirrhosis given glycerol phenylbutyrate (an NH3-lowering agent) or placebo for 16 weeks. "
06/01/2010 - "Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis."
07/01/2013 - "Glycerol Phenylbutyrate in Patients With Cirrhosis and Episodic Hepatic Encephalopathy: A Pilot Study of Safety and Effect on Venous Ammonia Concentration."
01/01/2023 - "We aimed to quantify whole-body ammonia metabolism in healthy persons and patients with cirrhosis and to validate our method by examining the effects of glycerol phenylbutyrate and lactulose + rifaximin treatment. "

11/01/2019 - "In the 7 years that have elapsed since the first guideline was published, abundant novel information has accumulated, experience on newborn screening for some UCDs has widened, a novel hyperammonemia-causing genetic disorder has been reported, glycerol phenylbutyrate has been introduced as a treatment, and novel promising therapeutic avenues (including gene therapy) have been opened. "
06/01/2013 - "Glycerol phenylbutyrate is under development for treatment of urea cycle disorders (UCDs), rare inherited metabolic disorders manifested by hyperammonemia and neurological impairment. "
08/01/2022 - "Secondary Ammonia Scavenge With Glycerol Phenylbutyrate Improves Hyperammonemia Following Portosystemic Shunting."
09/01/2015 - "A severely chronically protein and calorie restricted young woman with argininosuccinate lyase deficiency developed transient refeeding syndrome (RFS) and hyperammonemia after modest diet liberalization following initiation of glycerol phenylbutyrate (GPB). "
07/01/2013 - "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 hyperammonemia. "