Efficacy and safety of i.v. sodium benzoate in urea cycle disorders: a multicentre retrospective study

Husson, Marie-Caroline; Schiff, Manuel; Fouilhoux, Alain; Cano, Aline; Dobbelaere, Dries; Brassier, Anaïs; Mention, Karine; Arnoux, Jean‐Baptiste; Feillet, François; Chabrol, B.; Guffon, Nathalie; Elie, Caroline; Lonlay, Pascale de

doi:10.1186/s13023-016-0513-0

Cited by 35 publications

(27 citation statements)

References 24 publications

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“…For individuals with NAGS deficiency, partially responsive CPS1 deficiency and carbonic anhydrase VA deficiency administration of carglumic acid has been proven beneficial, as it mimics the function of N-acetylglutamate, the physiological activator of CPS1, stimulating the formation of carbamoylphosphate [ 2 , 12 ]. During HE, treatment is intensified stepwise by implementation of intravenous nitrogen scavenging therapy and extracorporeal detoxification using hemodialysis [ 2 , 8 , 13 ]. Liver transplantation is a curative therapy for UCDs.…”

Section: Introductionmentioning

confidence: 99%

Pharmacologic rescue of hyperammonemia-induced toxicity in zebrafish by inhibition of ornithine aminotransferase

et al. 2018

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Hyperammonemia is the common biochemical hallmark of urea cycle disorders, activating neurotoxic pathways. If untreated, affected individuals have a high risk of irreversible brain damage and mortality. Here we show that acute hyperammonemia strongly enhances transamination-dependent formation of osmolytic glutamine and excitatory glutamate, thereby inducing neurotoxicity and death in ammoniotelic zebrafish larvae via synergistically acting overactivation of NMDA receptors and bioenergetic impairment induced by depletion of 2-oxoglutarate. Intriguingly, specific and irreversible inhibition of ornithine aminotransferase (OAT) by 5-fluoromethylornithine rescues zebrafish from lethal concentrations of ammonium acetate and corrects hyperammonemia-induced biochemical alterations. Thus, OAT inhibition is a promising and effective therapeutic approach for preventing neurotoxicity and mortality in acute hyperammonemia.

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Section: Introductionmentioning

confidence: 99%

Pharmacologic rescue of hyperammonemia-induced toxicity in zebrafish by inhibition of ornithine aminotransferase

et al. 2018

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“…NaB (sodium benzoate), the metabolite of cinnamon and sodium salt of benzoic acid, is a commonly used food and beverage preservative, classified as a GRAS (Generally Recognized As Safe) compound by the FDA (Food and Drug Administration) [ 1 ]. NaB is also used as a treatment for disorders of the urea cycle and hyperammonemia because of its ability to bind and remove nitrogenous residues and decrease excess ammonia levels [ 2 ]. Since it is one of the most widely used preservatives worldwide, the biological effects of NaB have been studied in different in vitro and in vivo models.…”

Section: Introductionmentioning

confidence: 99%

Food Additive Sodium Benzoate (NaB) Activates NFκB and Induces Apoptosis in HCT116 Cells

Yılmaz

Karabay

2018

Molecules

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NaB, the metabolite of cinnamon and sodium salt of benzoic acid is a commonly used food and beverage preservative. Various studies have investigated NaB for its effects on different cellular models. However, the effects of NaB on cancer cell viability signaling is substantially unknown. In this study, the effects of NaB on viability parameters and NFκB, one of the most important regulators in apoptosis, were examined in HCT116 colon cancer cells. Cell culture, light microscopy, spectrophotometry, flow cytometry, and western blot were used as methods to determine cell viability, caspase-3 activity, NFκB, Bcl-xl, Bim, and PARP proteins, respectively. NaB (6.25 mM–50 mM) treatment inhibited cell viability by inducing apoptosis, which was evident with increased Annexin V-PE staining and caspase-3 activity. NFκB activation accompanied the induction of apoptosis in NaB treated cells. Inhibition of NFκB with BAY 11-7082 did not show a pronounced effect on cell viability but induced a more apoptotic profile, which was confirmed by increased PARP fragmentation and caspase-3 activity. This effect was mostly evident at 50 mM concentration of NaB. Bcl-xl levels were not affected by NaB or BAY 11-7082/NaB treatment; whereas, total Bim increased with NaB treatment. Inhibition of NFκB activity further increased Bim levels. Overall, these results suggest that NaB induces apoptosis and activates NFκB in HCT116 colon cancer cells. Activation of NFκB emerges as target in an attempt to protect cells against apoptosis.

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“…Most of these delays were due to the time needed to secure a central IV line for administration of SBSP ( n = 8, 57%). SBSP is hyperosmolar and requires a central line for safe administration, particularly in infants and neonates …”

Section: Discussionmentioning

confidence: 99%

Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit

Brossier

Goyer

Ziani

et al. 2019

J of Inher Metab Disea

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The purpose of the study was to evaluate the influence of establishing a protocol for the use of combined sodium benzoate and sodium phenylacetate (SBSP) (Ammonul ® ) to treat acute hyperammonemia. This was a retrospective, single-center study in a 24-bed medical and surgical pediatric intensive care unit (PICU) in a tertiary care teaching maternal-child hospital in Canada. Inclusion criteria were age < 18 years, PICU admission between 1 January 2000 and 30 June 2016, and SBSP treatment. An SBSP delivery protocol was implemented in our hospital on 30 August 2008 in order to improve management of acute hyperammonemia. Patients were assigned to one of the two groups, without or with protocol, depending on date of admission. SBSP was ordered 34 times during the study period, and 23 orders were considered for analysis (14 with and 9 without protocol). Patient characteristics were similar between groups. The median time from diagnosis to prescription was significantly shorter in the protocol group [40 min (21-82) vs 100 min (70-150), p = 0.03)] but the median time from diagnosis to administration of the treatment was equivalent [144 min (90-220) vs 195 (143-274), (p = 0.2)]. Other clinical outcomes did not differ. This study is the first to compare two SBSP delivery strategies in the treatment of acute hyperammonemia in this PICU setting. Implementation of a delivery protocol shortened the time from diagnosis of hyperammonemia to prescription of SBSP and helped us identify other parameters that can be improved to optimize treatment delivery. K E Y W O R D SPediatrics, Critical care, Hyperammonemia, Sodium phenylacetate, Sodium benzoate.

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Efficacy and safety of i.v. sodium benzoate in urea cycle disorders: a multicentre retrospective study

Cited by 35 publications

References 24 publications

Pharmacologic rescue of hyperammonemia-induced toxicity in zebrafish by inhibition of ornithine aminotransferase

Pharmacologic rescue of hyperammonemia-induced toxicity in zebrafish by inhibition of ornithine aminotransferase

Food Additive Sodium Benzoate (NaB) Activates NFκB and Induces Apoptosis in HCT116 Cells

Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit

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