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更新于：2026-09-07
Sodium Benzoate/Sodium phenylacetate
苯甲酸钠/苯乙酸钠
更新于：2026-09-07
概要
基本信息
最高研发阶段批准上市 |
最高研发阶段(中国)- |
特殊审评孤儿药 (美国) |
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关联
2
项与 苯甲酸钠/苯乙酸钠 相关的临床试验NCT04155567
A Single Center, Open-Label, Phase 1 Study to Evaluate the Pharmacokinetics, Safety and Tolerability of TAK-123 After Intravenous Infusion in Japanese Healthy Adult Male Subjects
NCT00597909
A Phase 2, Randomized, Double-Blind, Placebo-Controlled Study of the Efficacy and Safety of Two Doses of AMMONUL® (Sodium Phenylacetate and Sodium Benzoate) Injection 10% / 10% in Subjects With Grade 3 or 4 Hepatic Encephalopathy
100 项与 苯甲酸钠/苯乙酸钠 相关的临床结果
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100 项与 苯甲酸钠/苯乙酸钠 相关的转化医学
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100 项与 苯甲酸钠/苯乙酸钠 相关的专利（医药）
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15
项与 苯甲酸钠/苯乙酸钠 相关的文献（医药）2023-02-01Drug metabolism and pharmacokinetics
Pharmacokinetics, safety, and tolerability of sodium phenylacetate and sodium benzoate in healthy Japanese volunteers: A phase I, single-center, open-label study
Article
作者: Endo, Fumio ; Dote, Nobuhito ; Koumura, Emiko ; Shimizu, Kohei ; Sano, Yuuhei ; Nakamura, Kimitoshi
TAK-123, a combination of sodium phenylacetate (NaPA) and sodium benzoate (NaBZ), is an intravenously administered drug developed for the treatment of acute hyperammonemia in infants, children, and adults with urea cycle enzyme deficiencies. The aim of the current study was to evaluate the pharmacokinetics, safety, and tolerability after intravenous infusion of TAK-123 in Japanese healthy adult volunteers. Ten volunteers received a 3.75 g/m2 loading dose of TAK-123 over a period of 1.5 h followed by a maintenance infusion of the same dose over 24 h. Phenylacetate (PA) and benzoate (BZ) and their respective metabolites, phenylacetylglutamine (PAG) and hippurate (HIP) were measured over a 24-h period using a high-performance liquid chromatography/tandem mass spectrometry method. Non-compartmental analysis was performed using WinNonlin® Professional. During the loading dose, plasma levels of both PA and BZ peaked at 1.5 h. Plasma PA levels plateaued and were maintained up to 6.5 h, whereas plasma BZ levels declined rapidly after switching to maintenance infusion. Urinary excretion ratios of PAG and HIP at 48 h after the administration were 99.3% and 104%, respectively, suggesting that almost all NaPA and NaBZ were metabolized and excreted into urine. Overall, TAK-123 was well-tolerated in healthy Japanese adults.
2022-06-01Molecular genetics and metabolism reports
Compound heterozygote variants: c.848A > G; p.Glu283Gly and c.890C > T; p.Ala297Val, of Isovaleric acid-CoA dehydrogenase (IVD) gene causing severe Isovaleric acidemia with hyperammonemia
Article
作者: Enchautegui-Colon, Yazmin ; Lin, Hsin-Ti ; DeMarzo, Danielle ; Zimmerman, Chelsea ; Chou, Maxwell ; Tsai, Anne Chun-Hui ; Bolen, Jessica
With the execution of expanded newborn screen (NBS) program nationwide, it is uncommon to see severe hyperammonemia associated with isovaleric acidemia (IVA). We present a seven-day-old boy with severe IVA complicated by hyperammonemia. This child was flagged by NBS at 4 days old, but confirmatory testing was delayed due to COVID19 pandemic and parental skepticism. His parents did not adhere to the leucine-restricted diet as recommended. On day 7, the patient presented to the ER with ammonia of 588 μg/dL. Ammonia subsequently rose to >1000 μg/dL. This child received carnitine, 1 dose of Ammonul (sodium benzoate and sodium phenylacetate), arginine, carglumic acid (Carbaglu) and CRRT. Plasma amino acid assay revealed a glutamine level of 256 μmol/L, which is below the lower limit of normal upon arrival to ER and PICU. The hyperammonemia was corrected in 15 h and with the continued use of carglumic acid for 3 days, there was no rebound of hyperammonemia. However, the patient suffered from bone marrow suppression associated with the organic acidemia and required frequent platelet transfusions, as well as G-CSF for neutropenia. The management of this patient provides supporting evidence of the many theoretic metabolic "facts" including why Ammonul is not helpful in organic acidemias.
2020-04-01European journal of cancer (Oxford, England : 1990)1区 · 医学
5-Fluorouracil-induced hyperammonaemic encephalopathy: A French national survey
1区 · 医学
Article
作者: Mathieu Jozwiak ; Antoine Hollebecque ; Agnès Lillo-Le Louët ; Michel Ducreux ; Laure Thomas ; Valérie Boige ; Dominique Hillaire-Buys ; Louise Gaboriau ; David Malka ; Alice Boilève ; Laurent Chouchana
BACKGROUND:
5-Fluorouracil (5-FU)-induced hyperammonaemic encephalopathy is a rare but serious 5-FU adverse drug reaction (ADR). Given the growing number of cancers treated with 5-FU and the paucity of data regarding this ADR, we performed a retrospective national survey to better characterise 5-FU-induced hyperammonaemic encephalopathy.
PATIENTS AND METHODS:
Since inception of the French pharmacovigilance database, we identified all patients who experienced 5-FU-induced hyperammonaemic encephalopathy. Variables regarding demographics, characteristics, management and outcome of patients were collected.
RESULTS:
From 1986 to 2018, 30 patients were included. 5-FU-induced hyperammonaemic encephalopathy started 2 [1-4] days after 5-FU infusion onset. Most common neurological disorders were consciousness impairment, seizures and confusion. hyperammonaemia tended to be higher in patients with the lowest Glasgow score and admitted in intensive care unit (ICU) compared to non-ICU patients (250 [133-522] versus 139 [68-220] μmol/L respectively, p = NS). Dihydropyrimidine dehydrogenase deficiency was found in 27% of tested patients (n = 3/11). Encephalopathy-induced mortality was 17%, 57% of patients were admitted in ICU and 70% had a complete neurological recovery within 5 [2-10] days. A 5-FU rechallenge was considered in 14 (67%) patients with neurological recovery and a relapse was observed in 57% of them. No 5-FU-induced hyperammonaemic encephalopathy relapse was observed as long as 5-FU rechallenge was performed with decreased 5-FU dosage.
CONCLUSION:
We report the largest cohort of 5-FU-induced hyperammonaemic encephalopathy cases so far. This ADR should be suspected and ammonaemia measured in all patients experiencing neurological disorders after 5-FU administration. In patients with complete neurological recovery, a 5-FU rechallenge could be cautiously considered.
100 项与 苯甲酸钠/苯乙酸钠 相关的药物交易
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外链
| KEGG | Wiki | ATC | Drug Bank |
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| D10205 | - | - | - |
研发状态
批准上市
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| 适应症 | 国家/地区 | 公司 | 日期 |
|---|---|---|---|
| 高氨血症 | 美国 | 1987-12-23 | |
| 尿素循环障碍 | 美国 | 1987-12-23 |
未上市
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| 适应症 | 最高研发状态 | 国家/地区 | 公司 | 日期 |
|---|---|---|---|---|
| 消化系统疾病 | 申请上市 | 加拿大 | 2026-03-01 |
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临床结果
临床结果
适应症
分期
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临床1期 | - | 10 | NaBZ+NaPA+TAK-123 | 艱製積窪齋鏇積顧餘窪(廠淵製範選網顧憲顧襯) = 簾窪夢膚齋遞顧餘繭鏇 積夢窪願窪鏇觸願夢齋 (遞構繭築鹹憲艱構餘齋, 5.2) 更多 | - | 2021-02-21 | |
临床2期 | 1 | 願壓艱鹹齋糧齋蓋獵憲(鹹蓋淵齋選獵艱顧齋餘) = 觸齋簾構鏇糧願簾鬱範 糧繭憲鬱製餘鹽衊艱壓 (鬱鏇構鹽遞餘艱簾憲窪, 鬱夢壓鹽簾網壓選範鹹 ~ 淵鬱窪顧網繭製願遞觸) 更多 | - | 2016-03-31 | |||
願壓艱鹹齋糧齋蓋獵憲(鹹蓋淵齋選獵艱顧齋餘) = 選選窪鹹淵夢襯襯觸觸 糧繭憲鬱製餘鹽衊艱壓 (鬱鏇構鹽遞餘艱簾憲窪, 範願齋構淵範衊糧鏇襯 ~ 鹽鹽鏇壓簾衊壓製糧製) 更多 |
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