- |||||||||| sodium phenylacetate/sodium benzoate / Generic mfg.
Acquired Hyperammonemia and L-carnitine Deficiency Mimicking Urea Cycle Disorder (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2529;
We present a case of nutritionally driven acquired urea cycle disorder with carnitine deficiency leading to severe hyperammonemia, encephalopathy and extensive MRI changes
- |||||||||| sodium phenylacetate/sodium benzoate / Generic mfg.
A case of Ornithine Transcarbamylase Deficiency (MTCC - Exhibit Halls DE) - Jan 10, 2024 - Abstract #ACMG2024ACMG_527;
While the variant (c.238A>G, p.K80E) exhibited a considerably milder clinical presentation in the female patient, it manifested as a markedly more severe phenotype in our male patient, with early and aggressive onset. Another crucial point to consider is that in cases where patients exhibit a substantial increase in ammonia levels, the RRT approach to be employed is CVVH as the use of Aquadex alone has proven insufficient for effectively eliminating excess ammonia.
- |||||||||| Ravicti (glycerol phenylbutyrate) / Horizon Therapeutics, Immedica
Severe Hyperammonemia and Coma in a Post-partum Patient Secondary to a Urea Cycle Disorder (Walter E. Washington Convention Center, Area K, Hall C (Lower Level)) - Mar 25, 2023 - Abstract #ATS2023ATS_8078;
RRT was later discontinued after initiating IV ammonia scavenger therapy with Ammonul (sodium phenylacetate and sodium benzoate)... Awareness of late-onset UCD in MICU patients as a cause of encephalopathy and hyperammonemia is necessary for early diagnosis and timely management to prevent life-threatening complications of hyperammonemia.
- |||||||||| Xifaxan (rifaximin) / Alfa Wassermann, Bausch Health, Norgine, ASKA Pharma
HYPERAMMONEMIA IN FIBROLAMELLAR HEPATOCARCINOMA. (Rehearsal Room (Sunset Center)) - Dec 20, 2021 - Abstract #WSMRF2022WSMRF_551;
She was experiencing persistent hyperammonemia ranging from 200?250 umol/L despite CRRT, so she was treated with intravenous arginine and Ammonul (sodium phenylacetate and sodium benzoate)...Other medication options, including lactulose, rifaximin, and other oral medications were not able to be given due to her ongoing GI bleed...ODC in turn catalyzes the decarboxylation of ornithine in polyamine synthesis, shunting ornithine away from the urea cycle and inhibiting OTC activity. In order to investigate this proposed mechanism, we intend to conduct further studies to measure polyamine synthesis, a downstream effect of the aforementioned mechanism.
- |||||||||| sodium phenylacetate/sodium benzoate / Generic mfg.
Review, Journal: Continuous Renal Replacement Therapy for Two Neonates With Hyperammonemia. (Pubmed Central) - Nov 24, 2021
Continuous renal replacement therapy was more effective than scavenger therapy alone (Ammonul™) for rapid removal of ammonia in low weight infants (as low as 2.5 kg). Continuous renal replacement therapy is technically feasible in low weight infants with severe hyperammonemia secondary to an inborn error of metabolism.
- |||||||||| Ammonul (sodium phenylacetate/sodium benzoate) / Bausch Health, divalproex sodium XR / Generic mfg., escitalopram / Generic mfg.
[VIRTUAL] A Rare Case of Late Onset Ornithine Transcarbamylase Deficiency Causing Acute Metabolic Encephalopathy (ATS 2020 Virtual) - Jul 6, 2020 - Abstract #ATSI2020ATS-I_5555;
He was started on valproic acid and escitalopram in the Emergency Department (ED), but deteriorated quickly with development of vomiting, hypotension, worsening mental status, and status epilepticus...The patient's exposure to Depakote may have precipitated hyperammonemia triggering acute decompensation of his underlying disease and eventual death...Currently, there are 3 FDA approved medications, namely Buphenyl (sodium phenylbutyrate), Ravicti (glycerol phenylgutyrate), and Ammonul (sodium phenylacetate and sodium benzoate). This case highlights a number of interesting points regarding identification and triggers of OTC deficiency.
- |||||||||| Ammonul (sodium phenylacetate/sodium benzoate) / Bausch Health, divalproex sodium XR / Generic mfg., escitalopram / Generic mfg.
A Rare Case of Late Onset Ornithine Transcarbamylase Deficiency Causing Acute Metabolic Encephalopathy (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area E) - Mar 15, 2020 - Abstract #ATS2020ATS_8148;
He was started on valproic acid and escitalopram in the Emergency Department (ED), but deteriorated quickly with development of vomiting, hypotension, worsening mental status, and status epilepticus...The patient’s exposure to Depakote may have precipitated hyperammonemia triggering acute decompensation of his underlying disease and eventual death...Treatment options for patients with OTC deficiency are relatively limited and include dietary modifications and ammonia-lowering medications. Currently, there are 3 FDA approved medications, namely Buphenyl (sodium phenylbutyrate), Ravicti (glycerol phenylgutyrate), and Ammonul (sodium phenylacetate and sodium benzoate).This case highlights a number of interesting points regarding identification and triggers of OTC deficiency.
- |||||||||| sodium phenylacetate/sodium benzoate / Generic mfg.
Trial completion: A Study to Evaluate the Pharmacokinetics (PK), Safety and Tolerability of TAK-123 After Intravenous Infusion in Japanese Healthy Adult Male Participants (clinicaltrials.gov) - Dec 23, 2019
P1, N=10, Completed,
Currently, there are 3 FDA approved medications, namely Buphenyl (sodium phenylbutyrate), Ravicti (glycerol phenylgutyrate), and Ammonul (sodium phenylacetate and sodium benzoate).This case highlights a number of interesting points regarding identification and triggers of OTC deficiency. Active, not recruiting --> Completed
- |||||||||| sodium phenylacetate/sodium benzoate / Generic mfg.
Journal: Prenatal treatment of ornithine transcarbamylase deficiency. (Pubmed Central) - Feb 8, 2019
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.
- |||||||||| sodium phenylacetate/sodium benzoate / Generic mfg.
Enrollment change, Trial termination: Efficacy and Safety Study of Ammonul (clinicaltrials.gov) - Sep 10, 2015
P2, N=1, Terminated,
Following initial medical management, early liver transplantation may improve developmental outcome. N=105 --> 1 | Withdrawn --> Terminated; Study was terminated due to lack of enrollment and business decisions.
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