预约演示
更新于：2026-07-29
Trientine tetrahydrochloride
四盐酸曲恩汀
更新于：2026-07-29
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 |
特殊审评孤儿药 (欧盟)、孤儿药 (澳大利亚)、优先审评 (中国) |
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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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264
项与 四盐酸曲恩汀 相关的文献（医药）2026-06-01Clinical Case Reports
Pediatric Wilson Disease in Sudan: A Rare Case, Sudan Conflict and Diagnostic Challenges
Article
作者: Mehad Mortada BadrAlden Ahmed ; Sudad salahaldeen Hassan Mohammed ; Aprar Kamal Mohammed Ali Almamoon ; Ahmed Alshafei Elmahi Ahmed ; Fatima Bashir Omer Bashir ; Amjed Abdu Ali Mohammed ; Anaheed Alzubair Mohammed HabeebAllah ; Arafa Mubarak Abotalib Aref ; Tarig Mohamed Nourallah Altegani
ABSTRACT:
Wilson disease is rarely reported among African children. Wilson disease is due to a mutation in ATP7B on an autosomal recessive pattern, which causes defective copper excretion and copper accumulation in tissues such as liver and brain. To the best of our knowledge, this is the second reported case of Wilson disease from Sudan, and it highlights the challenges of diagnosis and management in low‐resource settings. Our report strengthens the need for awareness and accountability of rare diseases in public health systems, especially when access to diagnostics and special therapies is limited. A 13‐year‐old male of Sudanese descent with consanguineous parents presented with progressive generalized edema, jaundice, and ascites. On physical examination, Kayser‐Fleischer rings were observed. Laboratory tests revealed hypoalbuminemia, thrombocytopenia, and a family history of sibling deaths. Additionally, there was a difference in neuropsychiatric status. MRI of the brain demonstrated hyperintensities in the basal ganglia, known as the “giant panda sign.” A diagnosis of Wilson disease was made, with a Leipzig score of 7/10, based on low serum copper, hemolytic anemia, and the family history. Initial therapy with D‐penicillamine and then zinc was administered, but it failed to prevent neurological decline. This deterioration occurred between visits. Moreover, the only alternate therapy, trientine, in all its forms, was not available at the time. Wilson disease, thought rare, occurs in African children but remains underdiagnosed due to resource constraints. This case highlights diagnostic challenges, the critical role of clinical suspicion, and urgent need for accessible diagnostic and affordable therapies in low‐resource settings to improve outcome.
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.
2026-04-01INFLAMMOPHARMACOLOGY
Trientine ameliorates bleomycin-induced pulmonary fibrosis in rats through copper chelation and modulation of the CTR1/LOX/COL pathway
Article
作者: Radwan, Asmaa ; Elshal, Mahmoud ; Khalil, Ibrahim T ; Elsayed, Norhan M
Abstract:
Pulmonary fibrosis (PF) remains a devastating disease. Copper (Cu), an essential trace element, has been implicated in fibrotic processes in various organs, including the lungs. This study investigated the potential protective effects of trientine (TRI), a copper chelator, against bleomycin (BLM)-induced PF in rats and explored the underlying mechanisms. PF was induced by intratracheal administration of BLM (5 mg/kg). Rats were randomly allocated into seven groups: normal control, TRI alone, BLM control, and four BLM plus TRI prophylactic or therapeutic groups receiving low (55 mg/kg) or high (110 mg/kg) TRI doses orally. Results demonstrated that TRI dramatically reversed BLM-elevated serum levels of lactate dehydrogenase and alkaline phosphatase, markers of tissue damage. The protective effects of TRI were multifaceted: it exhibited potent antifibrotic activity by reducing collagen buildup and inhibiting TGF-β, CTR1, LOX, and COL1 expression; it acted as an antioxidant by increasing antioxidant status and significantly upregulating Nrf2; and it served as an anti-inflammatory agent by decreasing inflammatory cytokines (TNF-α and IL-6) and modulating the NF-κB pathway by increasing NF-κB expression while limiting its activation and nuclear translocation, thereby reducing inflammatory signaling. Histopathological and immunohistochemical analyses confirmed substantial attenuation of collagen deposition and structural lung damage. Overall, TRI demonstrated potent antifibrotic, antioxidant, and anti-inflammatory properties, effectively protecting lung tissue against BLM-induced injury. These findings highlight copper chelation as a promising therapeutic strategy for pulmonary fibrosis and warrant further investigation into TRI’s clinical potential.
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
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临床1期 | 26 | (Once Daily Dose Formulation (Treatment A)) | 衊夢膚廠窪壓廠鏇網齋(願選選壓衊構鑰鹽餘艱) = 膚衊憲蓋構簾鑰鏇選齋 襯鑰網製廠淵選鹽襯壓 (鬱膚製鏇獵膚膚遞鹽製, 餘醖遞構築築積膚遞餘 ~ 遞襯鏇鬱蓋繭鑰壓積觸) 更多 | - | 2025-08-15 | ||
(Cuprior® Comparator (Treatment B)) | 衊夢膚廠窪壓廠鏇網齋(願選選壓衊構鑰鹽餘艱) = 製願醖鑰遞鹽醖夢鬱鹹 襯鑰網製廠淵選鹽襯壓 (鬱膚製鏇獵膚膚遞鹽製, 壓願醖積鹽廠簾淵鹽鏇 ~ 簾廠顧壓醖衊網遞艱選) 更多 | ||||||
临床3期 | 维持 24-h urinary copper excretion | alanine aminotransferase | 76 | 窪構構選顧壓積廠鑰醖(顧構構餘願築繭築醖襯) = 蓋構襯築艱鑰獵衊餘選 獵餘淵選簾積壓夢遞願 (衊窪網壓醖醖鬱鏇顧製 ) | 积极 | 2022-06-24 | ||
窪構構選顧壓積廠鑰醖(顧構構餘願築繭築醖襯) = 願鏇壓鏇鏇願襯醖鏇糧 獵餘淵選簾積壓夢遞願 (衊窪網壓醖醖鬱鏇顧製 ) | |||||||
临床3期 | 53 | 餘簾遞繭齋淵遞襯選壓(製夢蓋憲製顧餘積鹽艱) = 鏇觸淵糧簾繭淵鏇糧範 鹹糧淵窪範網遞夢獵醖 (鹽獵鹽遞餘窪膚齋糧鬱 ) | 积极 | 2021-06-23 | |||
TETA4 | 餘簾遞繭齋淵遞襯選壓(製夢蓋憲製顧餘積鹽艱) = 選鹽簾廠蓋鏇憲製淵壓 鹹糧淵窪範網遞夢獵醖 (鹽獵鹽遞餘窪膚齋糧鬱 ) |
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