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(glycerol phenylbutyrate)
43 results
  • Pharmacotherapies that specifically target ammonia for the prevention and treatment of hepatic encephalopathy in adults with cirrhosis. [Review]
    Cochrane Database Syst Rev 2019; 6:CD012334Zacharias HD, Zacharias AP, … Morgan MY
  • CONCLUSIONS: There is insufficient evidence to determine the effects of these pharmacotherapies on the prevention and treatment of hepatic encephalopathy in adults with cirrhosis. They have the potential to reduce blood ammonia concentrations when compared to placebo, but their overall effects on clinical outcomes of interest and the potential harms associated with their use remain uncertain. Further evidence is needed to evaluate the potential beneficial and harmful effects of these pharmacotherapies in this clinical setting.
  • Impact of Diagnosis and Therapy on Cognitive Function in Urea Cycle Disorders. [Journal Article]
    Ann Neurol 2019; 86(1):116-128Posset R, Gropman AL, … Urea Cycle Disorders Consortium and the European Registry and Network for Intoxication Type Metabolic Diseases Consortia Study Group
  • CONCLUSIONS: Cognitive function is related to interventional and non-interventional variables. Early detection by newborn screening and early liver transplantation appear to offer greater cognitive protection, but none of the currently used nitrogen scavengers was superior with regard to long-term neurocognitive outcome. Further confirmation could determine these variables as important clinical indicators of neuroprotection for individuals with UCDs. ANN NEUROL 2019.
  • Suggested guidelines for the diagnosis and management of urea cycle disorders: First revision. [Review]
    J Inherit Metab Dis 2019; 42(6):1192-1230Häberle J, Burlina A, … Dionisi-Vici C
  • In 2012, we published guidelines summarizing and evaluating late 2011 evidence for diagnosis and therapy of urea cycle disorders (UCDs). With 1:35 000 estimated incidence, UCDs cause hyperammonemia of neonatal (~50%) or late onset that can lead to intellectual disability or death, even while effective therapies do exist. In the 7 years that have elapsed since the first guideline was published, ab…
  • Glycerol Phenylbutyrate (Ravicti) [BOOK]
    Canadian Agency for Drugs and Technologies in Health: Ottawa (ON)BOOK
  • Urea cycle disorders (UCDs) result from genetic mutations that cause defects in any of the five enzymes of the urea cycle in the liver: carbamoyl phosphate synthetase 1 (CPS1), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase, and arginase; in the co-factor producer N-acetyl glutamate synthetase; or in the ornithine transporter and citrin. The estimate…
  • GeneReviews®: Citrullinemia Type I [BOOK]
    University of Washington, Seattle: Seattle (WA)Adam MP, Ardinger HH, … Amemiya AQuinonez SC, Thoene JGBOOK
  • Citrullinemia type I (CTLN1) presents as a clinical spectrum that includes an acute neonatal form (the "classic" form), a milder late-onset form (the "non-classic" form), a form without symptoms or hyperammonemia, and a form in which women have onset of severe symptoms during pregnancy or post partum. Distinction between the clinical forms is based on clinical findings and is not clear-cut. Infan…
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