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Unbound Medicine.
(sodium phenylacetate sodium benzoate)
69 results
  • GeneReviews®: Hyperornithinemia-Hyperammonemia-Homocitrullinuria Syndrome [BOOK]
    . University of Washington, Seattle: Seattle (WA)Adam MP, Ardinger HH, … Amemiya ACamacho J, Rioseco-Camacho NBOOK
  • Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a disorder of the urea cycle and ornithine degradation pathway. Clinical manifestations and age of onset vary among individuals even in the same family. Neonatal onset (~8% of affected individuals). Manifestations of hyperammonemia usually begin 24-48 hours after feeding begins and can include lethargy, somnolence, refusal to fe…
  • Acute pediatric hyperammonemia: current diagnosis and management strategies. [Review]
    Hepat Med. 2018; 10:105-115.Savy N, Brossier D, … Jouvet P
  • Acute hyperammonemia may induce a neurologic impairment leading to an acute life-threatening condition. Coma duration, ammonia peak level, and hyperammonemia duration are the main risk factors of hyperammonemia-related neurologic deficits and death. In children, hyperammonemia is mainly caused by severe liver failure and inborn errors of metabolism. In an acute setting, obtaining reliable plasma …
  • GeneReviews®: Lysinuric Protein Intolerance [BOOK]
    . University of Washington, Seattle: Seattle (WA)Adam MP, Ardinger HH, … Amemiya ANunes V, Niinikoski HBOOK
  • Lysinuric protein intolerance (LPI) typically presents after an infant is weaned from breast milk or formula; variable findings include recurrent vomiting and episodes of diarrhea, episodes of stupor and coma after a protein-rich meal, poor feeding, aversion to protein-rich food, failure to thrive, hepatosplenomegaly, and muscular hypotonia. Over time, findings include: poor growth, osteoporosis,…
  • Prenatal treatment of ornithine transcarbamylase deficiency. [Case Reports]
    Mol Genet Metab. 2018 03; 123(3):297-300.Wilnai Y, Blumenfeld YJ, … Enns GM
  • CONCLUSIONS: Prenatal treatment of mothers who harbor severe OTCD mutations and carry affected male fetuses with intravenous Ammonul and arginine, followed by immediate institution of maintenance infusions after delivery, results in therapeutic levels of benzoate and phenylacetate in the newborn at delivery and, in conjunction with high-caloric enteral nutrition, prevents acute hyperammonemia and neurological decompensation. Following initial medical management, early liver transplantation may improve developmental outcome.
  • Saline is as effective as nitrogen scavengers for treatment of hyperammonemia. [Journal Article]
    Sci Rep. 2017 10 13; 7(1):13112.van Straten G, de Sain-van der Velden MGM, … Verhoeven-Duif NM
  • Urea cycle enzyme deficiency (UCED) patients with hyperammonemia are treated with sodium benzoate (SB) and sodium phenylacetate (SPA) to induce alternative pathways of nitrogen excretion. The suggested guidelines supporting their use in the management of hyperammonemia are primarily based on non-analytic studies such as case reports and case series. Canine congenital portosystemic shunting (CPSS)…
  • A Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrate. [Case Reports]
    Case Rep Pediatr. 2017; 2017:1045031.Köse M, Canda E, … Çoker M
  • In treatment of metabolic imbalances caused by maple syrup urine disease (MSUD), peritoneal dialysis, and hemofiltration, pharmacological treatments for elimination of toxic metabolites can be used in addition to basic dietary modifications. Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate (NaPB) in urea-cycle disorder cases has been associated with a reduction in branched-chai…
  • Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma. [Case Reports]
    Mol Genet Metab. 2017 05; 121(1):9-15.O'Donnell-Luria AH, Lin AP, … Frank NY
  • Acute idiopathic hyperammonemia in an adult patient is a life-threatening condition often resulting in a rapid progression to irreversible cerebral edema and death. While ammonia-scavenging therapies lower blood ammonia levels, in comparison, clearance of waste nitrogen from the brain may be delayed. Therefore, we used magnetic resonance spectroscopy (MRS) to monitor cerebral glutamine levels, th…
  • Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. [Case Reports]
    Ital J Pediatr. 2017 Jan 19; 43(1):12.Muñoz-Bonet JI, Ortega-Sánchez MD, León Guijarro JL
  • CONCLUSIONS: The alcohol consumption must be avoided in patients with HMG-CoA lyase deficiency. In our patient hyperamoniemia was effectively treated with sodium phenylacetate and sodium benzoate. Magnetic resonance imaging showed and quantified the cytotoxic brain edema. Belatedly, a cerebral vasospasm was an additional mechanism of cerebral injury. None of these observations has been previously reported.
  • 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…
  • Hepatic Encephalopathy: Pharmacological Therapies Targeting Ammonia. [Review]
    Semin Liver Dis. 2016 Feb; 36(1):48-55.Rahimi RS, Rockey DC
  • Hepatic encephalopathy (HE) is a major complication in patients with decompensated cirrhosis, leading to higher readmission rates causing a profound burden of disease and considerable health care costs. Because ammonia is thought to play a crucial role in the pathogenesis of HE, therapies directed at reducing ammonia levels are now being aggressively developed. Ammonia scavengers such as AST-120 …
  • Recent advances in the treatment of hyperammonemia. [Review]
    Adv Drug Deliv Rev. 2015 Aug 01; 90:55-68.Matoori S, Leroux JC
  • Ammonia is a neurotoxic agent that is primarily generated in the intestine and detoxified in the liver. Toxic increases in systemic ammonia levels predominantly result from an inherited or acquired impairment in hepatic detoxification and lead to potentially life-threatening neuropsychiatric symptoms. Inborn deficiencies in ammonia detoxification mainly affect the urea cycle, an endogenous metabo…
  • Late-onset ornithine transcarbamylase deficiency: treatment and outcome of hyperammonemic crisis. [Case Reports]
    Pediatrics. 2014 Apr; 133(4):e1072-6.Bergmann KR, McCabe J, … Gupta S
  • Hyperammonemic crises in ornithine transcarbamylase deficiency (OTC) can be associated with devastating cerebral edema resulting in severe long-term neurologic impairment and death. We present an 8-year-old boy who had late-onset OTC deficiency in which early and aggressive management of hyperammonemia and associated cerebral edema, including therapeutic hypothermia and barbiturate-induced coma, …
  • Fatal hyperammonemic brain injury from valproic Acid exposure. [Case Reports]
    Case Rep Neurol. 2012 Sep; 4(3):224-30.Bega D, Vaitkevicius H, … Chou SH
  • CONCLUSIONS: Cerebral edema secondary to hyperammonemia is potentially reversible if recognized early. Ammonia excretion can be facilitated by initiation of hemodialysis and administration of scavenging agents (sodium phenylacetate and sodium benzoate). Severe hyperammonemia can result from valproic acid exposure even in the absence of hepatotoxicity or inborn errors of metabolism. It is important to check serum ammonia in any patient with encephalopathy who has had recent valproic acid exposure.
  • Acute liver failure following cleft palate repair: a case of therapeutic acetaminophen toxicity. [Case Reports]
    Cleft Palate Craniofac J. 2013 Nov; 50(6):747-50.Iorio ML, Cheerharan M, … Boyajian M
  • Background : Acetaminophen is a widely used analgesic and antipyretic agent in the pediatric population. While the hepatotoxic effects of the drug have been well recognized in cases of acute overdose and chronic supratherapeutic doses, the toxic effects of acetaminophen are rarely documented in cases where therapeutic guidelines are followed. Case : An 8-month-old boy underwent cleft palate repai…
  • Phenylbutyrate therapy for maple syrup urine disease. [Controlled Clinical Trial]
    Hum Mol Genet. 2011 Feb 15; 20(4):631-40.Brunetti-Pierri N, Lanpher B, … Lee B
  • Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate in urea cycle disorder patients has been associated with a selective reduction in branched-chain amino acids (BCAA) in spite of adequate dietary protein intake. Based on this clinical observation, we investigated the potential of phenylbutyrate treatment to lower BCAA and their corresponding α-keto acids (BCKA) in patients with c…
  • Current trends in the treatment of hepatic encephalopathy. [Journal Article]
    Ther Clin Risk Manag. 2009 Jun; 5(3):617-26.Al Sibae MR, McGuire BM
  • Hepatic encephalopathy (HE) is a common reversible neuropsychiatric syndrome associated with chronic and acute liver dysfunction and significant morbidity and mortality. Although a clear pathogenesis is yet to be determined, elevated ammonia in the serum and central nervous system are the mainstay for pathogenesis and treatment. Management includes early diagnosis and prompt treatment of precipit…
  • Ammonia toxicity and its prevention in inherited defects of the urea cycle. [Review]
    Diabetes Obes Metab. 2009 Sep; 11(9):823-35.Walker V
  • The urea cycle is the final pathway for removal of surplus nitrogen from the body, and the major route in humans for detoxification of ammonia. The full complement of enzymes is expressed only in liver. Inherited deficiencies of urea cycle enzymes lead to hyperammonaemia, which causes brain damage. Severe defects present with hyperammonaemic crises in neonates. Equally devastating episodes may oc…
  • The role of molecular testing and enzyme analysis in the management of hypomorphic citrullinemia. [Case Reports]
    Am J Med Genet A. 2008 Nov 15; 146A(22):2885-90.Dimmock DP, Trapane P, … Fang P
  • Expanded newborn screening detects patients with modest elevations in citrulline; however it is currently unclear how to treat these patients and how to counsel their parents. In order to begin to address these issues, we compared the clinical, biochemical, and molecular features of 10 patients with mildly elevated citrulline levels. Three patients presented with clinical illness whereas seven ca…
  • Gateways to clinical trials. [Bibliography]
    Methods Find Exp Clin Pharmacol. 2007 Nov; 29(9):625-55.Bayés M, Rabasseda X, Prous JR
  • 1-Octanol, 9vPnC-MnCc; Abiraterone acetate, Adalimumab, Adefovir dipivoxil, Alemtuzumab, Aliskiren fumarate, Aminolevulinic acid hexyl ester, Amlodipine besylate/atorvastatin calcium, Amrubicin hydrochloride, Anakinra, Aripiprazole, ARRY-520, AS-1404, Asimadoline, Atazanavir sulfate, AVE-0277, Azelnidipine; Bevacizumab, Bimatoprost, Boceprevir, Bortezomib, Bosentan, Botulinum toxin type B; Certol…
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