Trientine hydrochloride is
N,N' -bis (2-aminoethyl)-1,2-ethanediamine dihydrochloride. It is a white to pale yellow crystalline hygroscopic powder. It is freely soluble in water, soluble in methanol, slightly soluble in ethanol, and insoluble in chloroform and ether.
SYPRINE is indicated in the treatment of patients with Wilson's disease who are intolerant of penicillamine. Clinical experience with SYPRINE is limited and alternate dosing regimens have not been well-characterized; all endpoints in determining an individual patient's dose have not been well defined. SYPRINE and penicillamine cannot be considered interchangeable. SYPRINE should be used when continued treatment with penicillamine is no longer possible because of intolerable or life endangering side effects.
Unlike penicillamine, SYPRINE is not recommended in cystinuria or rheumatoid arthritis. The absence of a sulfhydryl moiety renders it incapable of binding cystine and, therefore, it is of no use in cystinuria. In 15 patients with rheumatoid arthritis, SYPRINE was reported not to be effective in improving any clinical or biochemical parameter after 12 weeks of treatment.
SYPRINE is not indicated for treatment of biliary cirrhosis.
Published Studies Related to Syprine (Trientine)
Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double-blind study of treatment of the neurologic presentation of Wilson disease. [2006.04]
OBJECTIVE: To compare tetrathiomolybdate and trientine in treating patients with the neurologic presentation of Wilson disease for the frequency of neurologic worsening, adverse effects, and degree of neurologic recovery... CONCLUSION: Tetrathiomolybdate is a better choice than trientine for preserving neurologic function in patients who present with neurologic disease.
Metal contents of liver parenchyma after percutaneous ethanol injection or radiofrequency ablation in patients with hepatocellular carcinoma before and after trientine hydrochloride therapy. [2004.06]
We administered trientine hydrochloride, a drug used in the treatment of Wilson's disease, to patients with hepatocellular carcinoma after radical treatment with percutaneous ethanol injection or radiofrequency ablation, and examined its effect on the reduction of liver-tissue copper content... In patients with hepatocellular carcinoma, trientine hydrochloride therapy may significantly reduce copper content in liver tissue.
Evaluation of Cuprimine(R) and Syprine(R) for decorporation of radioisotopes of cesium, cobalt, iridium and strontium. [2011.08]
Cuprimine(R) and Syprine(R) are therapeutics approved by the USFDA to treat copper overload in Wilson Disease (a genetic defect in copper transport) by chelation and accelerated excretion of internally-deposited copper... Based on these encouraging findings, further studies to evaluate the dose-response profiles and timing of the chelator administration post exposure to radionuclides are warranted.
Evaluation of Cuprimine and Syprine for decorporation of (60)Co and (210)Po. [2010.03]
The acknowledged risk of deliberate release of radionuclides into local environments by terrorist activities has prompted a drive to improve novel materials and methods for removing internally deposited radionuclides. These decorporation treatments will also benefit workers in the nuclear industry, should an exposure occur.
Triethylene tetramine dihydrochloride (trientine) in children with Wilson disease: experience at King's College Hospital and review of the literature. [2009.09]
Our aim was to review our experience of trientine as chelation therapy in children with Wilson disease (WD) and compare to that reported in the literature. We made a retrospective review of the medical notes of 16 of 96 (17%) children diagnosed with WD between 1981 and 2006... Whilst the current evidence is low quality, it appears that trientine is as efficacious as penicillamine and small population studies show a lower side effect profile.
Clinical Trials Related to Syprine (Trientine)
Trientine Hydrochloride for the Prevention of Macular Edema After Cataract Surgery in Patients With Type 2 Diabetes Mellitus [Not yet recruiting]
The primary purpose of the protocol is to evaluate whether Trientine Hydrochloride, a copper
chelator which is an agent that binds with and removes copper, will be effective in
minimizing macular edema after cataract surgery in individuals with type 2 diabetes. It is
our hypothesis that there will be a reduction in copper-attributed inflammation after
surgery resulting a decrease in edema.
Trientine and Carboplatin in Advanced Malignancies [Recruiting]
The goal of this clinical research study is to find the highest tolerable dose of the
combination of trientine and carboplatin that can be given to patients with advanced cancer.
The safety of this drug combination will also be studied.
Trientine Hydrochloride for the Prevention of Macular Edema Associated With Pan-retinal Photocoagulation for Severe Non-proliferative and Proliferative Diabetic Retinopathy [Recruiting]
To evaluate the effects of Trientine Hydrochloride in prevention of post-laser (pan-retinal
photocoagulation) macular edema in the eyes for subjects with diabetic retinopathy.
Trientine hydrochloride can limit secondary inflammatory damage to retinal vessels following
the administration of pan-retinal photocoagulation therapy for severe non-proliferative
diabetic retinopathy or retinal neovascularization due to diabetic retinopathy, resulting in
less macular edema and improved visual outcomes.
Study of Tetrathiomolybdate in Patients With Wilson Disease [Completed]
Evaluate the safety and efficacy of ammonium tetrathiomolybdate alone and compared with
trientine therapy as initial treatment in patients with Wilson disease presenting
Efficacy of Invitro Expanded Bone Marrow Derived Allogeneic Mesenchymal Stem Cell Transplantation Via Portal Vein or Hepatic Artery or Peripheral Vein in Patients With Wilson Cirrhosis [Recruiting]
Wilson's disease is an autosomal recessive genetically inherited disorder of copper
metabolism, causing neurological, psychiatric and liver disease. The ATP7B gene on the 13th
chromosome is responsible for the disease. Liver has a critical role on copper metabolism.
It is the main site of copper accumulation and bile secretion is the only physiologic way of
copper elimination. Due to defective production of ceruloplasmin which carries copper, wide
amount of free copper precipitates throughout the body but particularly in the liver, eyes
and brain. Patients are bound to lifelong chelating agents such as penicillamine, trientine
and tetramine dihydrochloride. Unfortunately, these medications may cause severe
side-effects such as hypersensitivity reactions, bone marrow suppression, auto-immune
disease and sideroblastic anemia. Medical treatment of liver cirrhosis, the last stage of
the illness that leads to morbidity and mortality in the Wilson Disease, is difficult. Liver
transplantation is still the most effective treatment for the patients with liver cirrhosis
in Wilson Disease. However, serious problems are accompanied with liver transplantation.
Lack of liver donors, complications during and after the surgery, graft rejection and high
costs are the main problems.
There are cells in the human body that are capable to renew themselves and differentiate to
a diverse range of specialized cell types. These are called "stem cells". Stem cells can be
differentiated to specialized cells in appropriate medias in the laboratory. Recently, the
differentiation potential of mesenchymal stem cells into hepatocytes is proved by
demonstrating hepatocytes containing Y chromosome in the female who has had bone marrow
transplantation from male donors. In many laboratory studies, it is observed that human bone
marrow derived mesenchymal stem cells, transplanted to animals with induced liver damage,
differentiate into the albumin producing hepatocytes without fusion. By these studies, it is
understood that mesenchymal stem cells are more potent than other bone marrow elements in
context of differentiation to hepatocytes. Even though the number of studies on human for
the same purpose is few, findings are supporting those of animal experiments. Mesenchymal
stem cells are non-immunogenic. Safety and feasibility of allogeneic transplantations
between individuals without need of immunosuppressive drug regimen are proven. Proofs of
correcting metabolic defects by this way are also presented in some publications. For the
reasons mentioned above, allogeneic mesenchymal stem cell transplantation is a promising
treatment modality especially for the hereditary metabolic diseases. By this way,
non-immunogeneic mesenchymal stem cells which have healthy genetic structure, can
manufacture the required enzyme, will be repopulated in the damaged tissue and contribute to
the clinical improvement.
In this study, mesenchymal stem cells will be derived from healthy volunteer donor's bone
marrow and be expanded in-vitro, and then 1 million cells per kg will be infused to patients
with liver cirrhosis related to Wilson disease, 50 million cells via hepatic artery and the
remaining cells via peripheral vein. It is aimed to enable liver regeneration, decrease
fibrosis rate, improve patient's health conditions, increase ceruloplasmin synthesis,
ameliorate disorder of copper metabolism, decrease the need for chelating agents, increase
living standards of patients, and prolong waiting time for liver transplantation. Finally it
is aimed to establish a new and regenerative treatment protocol alternative to liver
transplantation. For observation of clinical and laboratory improvement, patients are
planned to be monitored by histopathologic examination of liver biopsies before and at 6th
month after the treatment, monthly biochemical and hematologic blood tests and periodic
radiologic examinations. This is a hopeful, avant garde and sophisticated study which may
constitute new horizons in context of cellular therapies.
Page last updated: 2011-12-09