预约演示
更新于：2026-05-02
Trientine tetrahydrochloride
四盐酸曲恩汀
更新于：2026-05-02
概要
基本信息
原研机构 |
非在研机构- |
最高研发阶段批准上市 |
首次获批日期 欧盟 (2017-09-05), |
最高研发阶段(中国)批准上市 |
特殊审评优先审评 (中国)、孤儿药 (澳大利亚) |
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结构/序列
分子式C6H22Cl4N4 |
InChIKeyOKHMDSCYUWAQPT-UHFFFAOYSA-N |
CAS号4961-40-4 |
关联
7
项与 四盐酸曲恩汀 相关的临床试验NCT07465718
Trientine Tetrahydrochloride Administered Once a Day for the First Line Treatment of Wilson's Disease Patients.
NCT07010575
Decentralized Study to Assess Patient Treatment Preference Comparing Their Current Standard-of-care Wilson's Disease (WD) Treatment to a New Trientine (TETA) 4HCl Formulation.
CTR20244688
盐酸曲恩汀片在健康受试者中空腹生物等效性试验
100 项与 四盐酸曲恩汀 相关的临床结果
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100 项与 四盐酸曲恩汀 相关的转化医学
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100 项与 四盐酸曲恩汀 相关的专利（医药）
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270
项与 四盐酸曲恩汀 相关的文献（医药）2026-04-01ADVANCES IN THERAPY
Patient Burden in the Treatment of Wilson Disease in the United States: An Analysis of Real-World Health Insurance Claims Data from the Komodo database
Article
作者: Strader, Carey ; Schultze, Michael ; Teynor, Megan ; Weiss, Karl Heinz ; Akumo, Divine ; Hedera, Peter ; Costantino, Halley
INTRODUCTION:
Wilson disease (WD) is a rare inherited disorder that causes copper accumulation and can be fatal if untreated. This study used real-world data from the US Komodo Health claims database to describe healthcare resource utilization (HCRU) and evaluate direct economic costs among patients with WD.
METHODS:
This retrospective observational study identified patients with WD using ICD-9/10 codes (excluding Menkes disease) between 2016 and 2019, with data spanning 2012-2020. Sociodemographic characteristics, HCRU, and costs were analyzed using SPSS v23, SAS v9.4, and R v3.6.0. The study was approved by Pearl Pathways IRB (#20-KANT-224).
RESULTS:
A total of 2115 patients with prevalent WD, including 360 ever-treated (with reimbursable WD prescriptions), were identified. During the 2-year follow-up, about 25% were hospitalized, with a mean stay of 9 days, and most visited the ER three times annually. Hepatic patients with WD were more likely to undergo liver biopsy or transplant but had fewer home health visits and less use of assistive mobility devices. Annual liver transplant costs averaged $9094.72 ± 8110.23 per prevalent WD patient and $10,147.98 ± 7030.83 per ever-treated patient. Mean annual costs per prevalent versus ever-treated patients with WD were inpatient ($716.52 ± 2675.06 vs. $252.75 ± 333.39), pharmacy ($270.35 ± 1348.79 vs. $1284.51 ± 2994.85), and outpatient ($73.93 ± 156.89 vs. $60.82 ± 62.17), respectively. Pharmacy costs for adherent patients averaged $157,505.28 for any medication, $252,617.11 for D-penicillamine, $189,328.52 for trientine, and $1574.91 for zinc. Among ever-treated patients with WD, respective costs were lower at $85,117.60, $123,190.73, $100,017.20, and $820.35.
CONCLUSION:
Estimated annual HCRU and treatment costs for patients with WD were lower than previously reported. These findings provide updated real-world insights into the economic burden of WD and highlight the cost implications of medication adherence in managing this rare disorder in the USA.
2026-01-01EUROPEAN JOURNAL OF CLINICAL PHARMACOLOGY
Further elucidation on the effect of food on the pharmacokinetics of trientine
Article
作者: Weiss, Karl Heinz ; Fu, ChauHwei ; Aliane, Verena ; Kruse, Carlot ; Scherpbier, Jan-Jaap ; van der Horst, Eric ; Abd-Elaziz, Khalid ; Dogterom, Peter ; Gerrits, Mireille
2025-12-01Current Neurology and Neuroscience Reports
Pitfalls in the Diagnosis of Wilson Disease
Review
作者: Chakravarty, Ambar ; Roy, Debasish ; Mukherjee, Angshuman
PURPOSE OF REVIEW:
Wilson disease (WD), an uncommon autosomal recessive (AR) hereditary disorder characterized by abnormal accumulation of copper primarily in the liver and secondarily in other organs like the brain, is caused by a deficiency in the ATP7B transporter gene. The key to successful therapy is early diagnosis.
RECENT FINDINGS:
Mutant genes need to be inherited from both parents for phenotypic expression. The ATP7B gene located on chromosome 13q14.3 comprises 20 introns and 21 exons, encodes a protein of 165 amino acids, and this helps in incorporation of copper into ceruloplasmin, the copper binding protein. So far, more than 800 mutations have been reported, of which 380 have confirmed involvement in the pathogenesis of WD. The most common mutations are H1069Q and R778L in European and Asian populations respectively. Approximately 90%-98% of WD subjects are heterozygous, showing different mutations in each of the alleles encoding the ATP7B. Conversely, the phenotype and the penetrance of WD can be extremely variable. Even patients carrying two disease-causing mutations do not necessarily have a demonstrable alteration of copper metabolism. Considering the possibility of late-onset disease, asymptomatic cases, and phenotypic variability, it is crucial to evaluate previous and future generations of the index case. WD ranges from being asymptomatic in some patients to result in acute liver failure and/or a variety of neuropsychiatric disorders among others. Although WD may be present at any age, is more common between the ages of 5 and 35 years. However, it should be investigated in patients with liver failure of unknown cause and those with liver disease associated with neuropsychiatric symptomatology. Diagnosis requires a combination of clinical signs and symptoms, as well as relevant diagnostic tests such as measurement of serum ceruloplasmin, urinary excretion of copper, liver biopsy or genetic testing. Treatment is lifelong and includes chelating agents (penicillamine and trientine) and inhibitors of copper absorption (zinc salts). Liver transplant is an option for patients with end-stage liver disease. The key to successful therapy is early diagnosis.
100 项与 四盐酸曲恩汀 相关的药物交易
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外链
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研发状态
10 条最早获批的记录, 后查看更多信息
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| 适应症 | 国家/地区 | 公司 | 日期 |
|---|---|---|---|
| 肝豆状核变性 | 欧盟 | 2017-09-05 | |
| 肝豆状核变性 | 冰岛 | 2017-09-05 | |
| 肝豆状核变性 | 列支敦士登 | 2017-09-05 | |
| 肝豆状核变性 | 挪威 | 2017-09-05 |
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临床结果
临床结果
适应症
分期
评价
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临床1期 | 26 | (Once Daily Dose Formulation (Treatment A)) | 憲醖艱淵鹹顧獵衊鏇鏇(壓壓選襯齋顧遞鬱範築) = 鹹襯鏇簾醖蓋窪膚淵鏇 鏇鏇觸鑰積鹽網遞膚夢 (遞餘廠艱製夢糧鬱壓範, 選繭窪選鑰構選齋繭築 ~ 蓋鹽膚餘艱製艱遞膚構) 更多 | - | 2025-08-15 | ||
(Cuprior® Comparator (Treatment B)) | 憲醖艱淵鹹顧獵衊鏇鏇(壓壓選襯齋顧遞鬱範築) = 蓋範醖築糧齋鑰衊襯鏇 鏇鏇觸鑰積鹽網遞膚夢 (遞餘廠艱製夢糧鬱壓範, 選簾鹹齋餘醖廠鹹壓製 ~ 製鏇簾積鹹糧鏇鑰憲鬱) 更多 | ||||||
临床3期 | 维持 24-h urinary copper excretion | alanine aminotransferase | 76 | 積網醖願憲醖襯廠衊選(糧醖簾築遞糧網夢積襯) = 夢夢衊願顧鑰醖齋鹽憲 選網願構願憲憲醖網餘 (願範築鏇鹹願築襯鹹製 ) | 积极 | 2022-06-24 | ||
積網醖願憲醖襯廠衊選(糧醖簾築遞糧網夢積襯) = 築構鑰醖壓繭範獵構糧 選網願構願憲憲醖網餘 (願範築鏇鹹願築襯鹹製 ) | |||||||
临床3期 | 53 | 壓築膚衊窪構繭製簾醖(鹹壓獵選觸鑰壓淵鏇遞) = 艱遞遞簾觸鹹簾遞顧觸 遞遞鑰觸構窪膚窪餘積 (窪範夢淵顧繭膚製壓餘 ) | 积极 | 2021-06-23 | |||
TETA4 | 壓築膚衊窪構繭製簾醖(鹹壓獵選觸鑰壓淵鏇遞) = 衊淵淵積願糧構簾範繭 遞遞鑰觸構窪膚窪餘積 (窪範夢淵顧繭膚製壓餘 ) |
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