trientine has been researched along with Hepatolenticular Degeneration in 166 studies
Trientine: An ethylenediamine derivative used as stabilizer for EPOXY RESINS, as ampholyte for ISOELECTRIC FOCUSING and as chelating agent for copper in HEPATOLENTICULAR DEGENERATION.
TETA : An azamacrocyle in which four nitrogen atoms at positions 1, 4, 8 and 11 of a fouteen-membered ring are each substituted with a carboxymethyl group.
2,2,2-tetramine : A polyazaalkane that is decane in which the carbon atoms at positions 1, 4, 7 and 10 are replaced by nitrogens.
Hepatolenticular Degeneration: A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Drug induced colitis is a very rare side effect of trientine." | 8.91 | Trientine induced colitis during therapy for Wilson disease: a case report and review of the literature. ( Boga, S; Jain, D; Schilsky, ML, 2015) |
| "To report a case of early-decompensated liver cirrhosis secondary to discontinuation of penicillamine therapy in a patient with Wilson's disease." | 7.74 | Discontinuation of penicillamine in the absence of alternative orphan drugs (trientine-zinc): a case of decompensated liver cirrhosis in Wilson's disease. ( Awaisu, A; Aziz, NA; Ghazali, R; Hassan, Y; Ping, CC, 2007) |
| "We describe a 14-year-old boy with Wilson disease (WD) who first developed pseudo-pseudoxanthoma elasticum (PPXE) after 4." | 5.56 | Pediatric Pseudo-pseudoxanthoma Elasticum Resulting From D-Penicillamine Treatment for Wilson Disease. ( Corey, K; Hawryluk, EB; Schmidt, B; Sheu Song, J; Yu, Z, 2020) |
| "We report a pediatric patient with Wilson's disease who developed nephrotic syndrome 2 wk after beginning D-penicillamine." | 5.30 | Early onset of nephrotic syndrome after treatment with D-penicillamine in a patient with Wilson's disease. ( Alexander, S; Furuta, GT; Herrin, J; Jonas, MM; Siafakas, CG, 1998) |
| "Seven patients with Wilson's disease, treated with trientine, have been followed during 11 pregnancies." | 5.27 | The management of pregnancy in Wilson's disease treated with trientine. ( Walshe, JM, 1986) |
| "Drug induced colitis is a very rare side effect of trientine." | 4.91 | Trientine induced colitis during therapy for Wilson disease: a case report and review of the literature. ( Boga, S; Jain, D; Schilsky, ML, 2015) |
| " He was treated with trientine for more than 10 years and suffered from anemia and liver dysfunction." | 3.77 | Excess copper chelating therapy for Wilson disease induces anemia and liver dysfunction. ( Abe, S; Harada, M; Harada, R; Hiura, M; Honma, Y; Matsuhashi, T; Miyagawa, K; Shibata, M; Tabaru, A, 2011) |
| "To report a case of early-decompensated liver cirrhosis secondary to discontinuation of penicillamine therapy in a patient with Wilson's disease." | 3.74 | Discontinuation of penicillamine in the absence of alternative orphan drugs (trientine-zinc): a case of decompensated liver cirrhosis in Wilson's disease. ( Awaisu, A; Aziz, NA; Ghazali, R; Hassan, Y; Ping, CC, 2007) |
| " Through the formation of copper and protein metal complexes D-penicillamine impoverishes copper deposits causing the reduction or disappearance of hepatic and neurological symptoms; a small percentage of patients treated develops a nephrotic syndrome requiring the compulsory suspension of the drug." | 3.69 | [Wilson's disease: physiopathology, therapeutic approach and case report]. ( Bisbocci, D; Gallo, V; Riva, P; Sidoli, L, 1994) |
| " The drug was applied to 3 patients, 18 to 25 years of age, with Wilson-Konovalov disease (hepatolenticular degeneration) who in the course of chronic D-penicillamine treatment developed drug intolerance with signs of nephrotoxicity (in one patient) and myelotoxicity with leucopenia and thrombocytopenia (in two patients)." | 3.67 | [New possibilities in the treatment of Wilson-Konovalov disease (hepatolenticular degeneration)]. ( Kolarski, V, 1987) |
| "Wilson disease is an inherited disorder of copper transport." | 3.11 | Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial. ( Ala, A; Cassiman, D; Couchonnal-Bedoya, E; Cury, RG; Czlonkowska, A; D'Hollander, K; D'Inca, R; Denk, G; Dubois, N; Gondim, FAA; Kamlin, COF; Moore, J; Ott, P; Poujois, A; Schilsky, ML; Twardowschy, C; Weiss, KH; Zuin, M, 2022) |
| "Wilson disease is multisystemic and the hepatic manifestations are seen more frequently in childhood, whereas neurologic manifestations are more common in adults; presentation may range from subtle changes to end-stage liver disease with or without encephalopathy as well as neuropsychiatric manifestations." | 2.82 | Wilson Disease in Children. ( Kerkar, N; Rana, A, 2022) |
| "Tetrathiomolybdate is a better choice than trientine for preserving neurologic function in patients who present with neurologic disease." | 2.72 | 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. ( Askari, F; Brewer, GJ; Carlson, M; Dick, RB; Fink, JK; Hedera, P; Kluin, KJ; Lorincz, MT; Moretti, P; Schilsky, M; Sitterly, J; Tankanow, R, 2006) |
| "Wilson disease is an autosomal recessive disorder based on inborn error of copper metabolism." | 2.61 | [Diagnosis and treatment of Wilson disease in Japan]. ( Shimizu, N, 2019) |
| "Wilson's disease is a rare cause of secondary Immunoglobulin A nephropathy." | 2.61 | Immunoglobulin A nephropathy secondary to Wilson's disease: a case report and literature review. ( Gocho, Y; Maeda, T; Ogawa, Y; Shimamura, Y; Takizawa, H; Tsuji, K, 2019) |
| "Consideration of a diagnosis of Wilson disease is still the critical factor in testing for and establishing disease diagnosis." | 2.55 | Wilson Disease: Diagnosis, Treatment, and Follow-up. ( Schilsky, ML, 2017) |
| "Hereditary hemochromatosis and Wilson disease are autosomal recessive storage disorders of iron and copper overload, respectively." | 2.50 | Metal storage disorders: Wilson disease and hemochromatosis. ( Kanwar, P; Kowdley, KV, 2014) |
| " Standardized dosage strategies that address changes in copper pools might improve adherence and reduce side effects." | 2.50 | Clinical considerations for an effective medical therapy in Wilson's disease. ( Stremmel, W; Weiss, KH, 2014) |
| "Wilson's disease is a rare autosomal recessive disease characterised by the deposition of copper in the brain, liver; cornea, and other organs." | 2.49 | The treatment of Wilson's disease, a rare genetic disorder of copper metabolism. ( Purchase, R, 2013) |
| "As Wilson's disease is both preventable and treatable, the diagnosis must not be missed." | 2.48 | Recognition and treatment of neurologic Wilson's disease. ( Lorincz, MT, 2012) |
| " TETA is poorly absorbed with a bioavailability of 8 to 30%." | 2.46 | Triethylenetetramine pharmacology and its clinical applications. ( Lu, J, 2010) |
| "The discovery of the Wilson's disease gene has opened up a new molecular diagnostic approach, and could form the basis of future gene therapy." | 2.44 | Wilson's disease. ( Ala, A; Ashkan, K; Dooley, JS; Schilsky, ML; Walker, AP, 2007) |
| "Wilson disease is an inherited, autosomal recessive, copper accumulation and toxicity disorder that affects about 30 individuals per million." | 2.44 | Wilson disease--a practical approach to diagnosis, treatment and follow-up. ( Medici, V; Rossaro, L; Sturniolo, GC, 2007) |
| "Some of the implications are that Alzheimer's disease and other diseases of neurodegeneration and fibrotic, inflammatory, and autoimmune diseases may be treatable by lowering the availability of free copper." | 2.44 | The risks of free copper in the body and the development of useful anticopper drugs. ( Brewer, GJ, 2008) |
| "Wilson's disease is a hereditary, autosomal-recessive disease affecting copper excretion." | 2.43 | A case study: identifying a new case of Wilson's disease. ( Noble, JA, 2005) |
| "Wilson's disease is a rare autosomal recessive disease of copper accumulation and copper toxicity, due to mutations in the ATP7B gene, which leads to a failure of copper excretion in the bile." | 2.43 | Neurologically presenting Wilson's disease: epidemiology, pathophysiology and treatment. ( Brewer, GJ, 2005) |
| "Wilson disease is a rare disorder of copper metabolism that results in accumulation of copper in the liver and subsequently in other organs, mainly the central nervous system and the kidneys." | 2.42 | Wilson disease. ( El-Youssef, M, 2003) |
| "Wilson's disease is a relatively rare inherited disorder of copper accumulation and toxicity, caused by a defect in an enzyme that is part of the pathway of biliary excretion of excess copper." | 2.41 | Recognition, diagnosis, and management of Wilson's disease. ( Brewer, GJ, 2000) |
| "Wilson disease is a copper storage disease with autosomal-recessive trait that is predominantly a disorder of the adolescent and young adult." | 2.40 | [Therapy of Wilson disease]. ( Smolarek, C; Stremmel, W, 1999) |
| "Wilson's disease is an inherited disorder of copper accumulation." | 2.39 | Practical recommendations and new therapies for Wilson's disease. ( Brewer, GJ, 1995) |
| "The discovery that the gene for Wilson disease encodes a copper-transporting ATPase has greatly improved our understanding of the pathophysiology of this disorder and of copper metabolism in humans." | 2.39 | Wilson disease: genetic basis of copper toxicity and natural history. ( Schilsky, ML, 1996) |
| "Beside, once the diagnosis is made, Wilson's disease can be effectively treated." | 2.38 | [Wilson's disease]. ( Cramarossa, L; D'Angelo, D; D'Ascanio, I; Ferri, GB; Piane, E, 1992) |
| "Wilson's disease is a disorder of hepatobiliary copper excretion manifested predominantly by hepatic and neurologic copper toxicosis and inherited in an autosomal recessive pattern." | 2.38 | Wilson's disease: current status. ( Friedman, LS; Martin, P; Muñoz, SJ; Yarze, JC, 1992) |
| " Safety of TETA 4HCl treatment was based on reported adverse events (AEs)." | 1.91 | Experience on switching trientine formulations in Wilson disease: Efficacy and safety after initiation of TETA 4HCl as substitute for TETA 2HCl. ( Bourhis, H; Denk, G; Merle, U; Mohr, I; Morgil, M; Morvan, E; Obadia, MA; Poujois, A; Weiss, KH; Woimant, F, 2023) |
| "In patients with Wilson disease, defined as an excess accumulation of copper which can damage the liver, brain and other vital organs, neurological worsening can occur despite chelation therapy." | 1.91 | Neurological worsening in Wilson disease - clinical classification and outcome. ( Ala, A; Eker, E; Merle, U; Mohr, I; Pfeiffenberger, J; Poujois, A; Weiss, KH, 2023) |
| "Medical records of 77 Wilson disease patients were reviewed to collect data on hepatic and neurologic symptoms, copper (Cu) homeostasis and adverse events." | 1.72 | Multicentre, retrospective study to assess long-term outcomes of chelator based treatment with trientine in Wilson disease patients withdrawn from therapy with d -penicillamine. ( de Koning, CE; Dhawan, A; Ferenci, P; Kruse, C; Manolaki, N; van Scheppingen, D; Weiss, KH; Wijnberg, L; Zuin, M, 2022) |
| "BACKGROUND AND OBJECTIVE: There are limited data on the adverse events of D-penicillamine in Wilson's disease (WD) that can result in dose modification or treatment discontinuation." | 1.72 | Adverse Events with D-penicillamine Therapy in Hepatic Wilson's Disease: A Single-Center Retrospective Audit. ( Darak, H; Giri, S; Gopan, A; Irtaza, M; Kale, A; Kumar, S; Patra, BR; Rao, PK; Shukla, A, 2022) |
| "Investigations about weight-based dosage showed no significant difference of any laboratory parameter between the 2 cohorts." | 1.62 | Optimized Trientine-dihydrochloride Therapy in Pediatric Patients With Wilson Disease: Is Weight-based Dosing Justified? ( Ferenci, P; Fichtner, A; Hoffmann, GF; Mayr, T; Mehrabi, A; Mohr, I; Pfeiffenberger, J; Teufel-Schäfer, U; Weiler, M; Weiss, KH, 2021) |
| "We describe a 14-year-old boy with Wilson disease (WD) who first developed pseudo-pseudoxanthoma elasticum (PPXE) after 4." | 1.56 | Pediatric Pseudo-pseudoxanthoma Elasticum Resulting From D-Penicillamine Treatment for Wilson Disease. ( Corey, K; Hawryluk, EB; Schmidt, B; Sheu Song, J; Yu, Z, 2020) |
| "Subjects of Wilson disease were identified as those with International Classification of Diseases, Ninth Revision (ICD-9) code 275." | 1.48 | Modality of treatment and potential outcome of Wilson disease in Taiwan: A population-based longitudinal study. ( Chang, MH; Chen, HL; Hsu, HY; Ni, YH; Tai, CS; Wu, JF, 2018) |
| "Wilson disease is an autosomal recessive genetic disorder caused by loss-of-function mutations in the P-type copper ATPase, ATP7B, which leads to toxic accumulation of copper mainly in the liver and brain." | 1.48 | Copper(I)-binding properties of de-coppering drugs for the treatment of Wilson disease. α-Lipoic acid as a potential anti-copper agent. ( Bragina, O; Järving, I; Kabin, E; Palumaa, P; Plitz, T; Smirnova, J; Tõugu, V, 2018) |
| "We retrospectively analyzed 235 Wilson disease patients." | 1.43 | Concomitant immune-related events in Wilson disease: implications for monitoring chelator therapy. ( Ferenci, P; Gohdes, A; Gotthardt, DN; Pfeiffenberger, J; Schäfer, M; Seessle, J; Stremmel, W; Weiss, KH, 2016) |
| "Wilson's disease is a genetic disorder caused by a malfunction of ATPase 7B that leads to high accumulation of copper in the organism and consequent toxic effects." | 1.40 | Chelating polymeric beads as potential therapeutics for Wilson's disease. ( Hrubý, M; Kučka, J; Mattová, J; Nový, Z; Petřík, M; Poučková, P; Skodová, M; Stěpánek, P; Urbánek, P; Vetrík, M, 2014) |
| "Chelating agents are effective therapies for most patients with Wilson disease; D-penicillamine and trientine produce comparable outcomes, although D-penicillamine had a higher rate of adverse events." | 1.39 | Efficacy and safety of oral chelators in treatment of patients with Wilson disease. ( Ferenci, P; Ferenci-Foerster, D; Gotthardt, DN; Hefter, H; Houwen, RH; Maieron, A; Merle, U; Reuner, U; Schäfer, M; Schmidt, HH; Stauber, R; Stremmel, W; Teufel, U; Thurik, F; Trocello, JM; Weiss, KH; Wiegand, F; Zoller, H, 2013) |
| "Finally, she was diagnosed with Wilson disease." | 1.39 | An unusual cause of headache and hypertension. ( Hsia, SH; Hsiao, HJ; Lin, JJ; Lin, JL; Wu, CT, 2013) |
| "Wilson disease is an autosomal recessive disease that produces a copper accumulation in many organs, initially in the liver, progressing to liver cirrhosis, and in the brain, with different neurologic symptoms." | 1.38 | [Diagnosis and care of Wilson disease with neurological revelation]. ( Bost, M; Broussolle, E; Brunet, AS; Des Portes, V; Lachaux, A; Lion-François, L; Wagner, S, 2012) |
| "The case notes of 320 patients with Wilson disease, seen between 1960 and 1987, have been reviewed." | 1.38 | Serum 'free' copper in Wilson disease. ( Walshe, JM, 2012) |
| "Wilson's disease is an autosomal-recessive disorder caused by mutation in the ATP7B gene, with resultant impairment of biliary excretion of copper." | 1.35 | [Wilson's disease in paediatric age: diagnosis and treatment. Recent advances]. ( Palumbo, E, 2008) |
| "Wilson's disease is a rare inborn disease related to copper storage, leading to liver cirrhosis and neuropsychological deterioration." | 1.34 | Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study. ( Ferenci, P; Merle, U; Schaefer, M; Stremmel, W, 2007) |
| "Based on these findings, acute Wilson's disease was suspected." | 1.33 | [Acute liver failure and hemolysis in a 16-year-old woman. First manifestation of Wilson's disease]. ( Christl, SU; Fischbach, W; Flieger, D; Keller, R; Stremmel, W, 2005) |
| "The progression of Wilson disease (WD), a disorder of copper metabolism, can be arrested by chelation therapy." | 1.32 | Clinical correlation of brain MRI and MRS abnormalities in patients with Wilson disease. ( Davie, CA; Lees, AJ; MacManus, D; Miller, DH; Miszkiel, KA; Page, RA; Schapira, AH; Walshe, JM, 2004) |
| "Wilson's disease or hepatolenticular degeneration is an autosomal recessive disorder." | 1.32 | [Pathogenesis and treatment of Wilson's disease]. ( Csák, T; Folhoffer, A; Horváth, A; Nagy, J; Vincze, Z; Zelkó, R, 2003) |
| "In patients with Wilson's disease and neurological manifestations, treatment with D-penicillamine can cause worsening of neurological symptoms, usually in the first few weeks of treatment." | 1.32 | [Wilson's disease with severe neurological manifestations: response to trientine plus zinc therapy]. ( Amorós, I; Aragó, M; García, M; Merino, C; Primo, J; Serra, B, 2004) |
| "We describe a 19-year-old woman with haemolytic anaemia and thrombocytopenia as the initial manifestation of Wilson disease (WD)." | 1.31 | Haemolytic onset of Wilson disease in a patient with homozygous truncation of ATP7B at Arg1319. ( Baccalà, R; Belin, D; Di Raimondo, F; Garozzo, R; Horisberger, JD; Invernizzi, R; Prella, M; Schapira, M, 2001) |
| "A diagnosis of the neurological form of Wilson disease was confirmed by copper deposits in the liver obtained by a blind biopsy, and the patient was diagnosed as compound heterozygous for ATP7B mutations." | 1.31 | Ultrastructural identification of iron and copper accumulation in the liver of a male patient with Wilson disease. ( Hayashi, H; Shiono, Y; Wakusawa, S; Yano, M, 2001) |
| "We report a pediatric patient with Wilson's disease who developed nephrotic syndrome 2 wk after beginning D-penicillamine." | 1.30 | Early onset of nephrotic syndrome after treatment with D-penicillamine in a patient with Wilson's disease. ( Alexander, S; Furuta, GT; Herrin, J; Jonas, MM; Siafakas, CG, 1998) |
| "Wilson's disease is a rare inherited metabolic disorder usually characterized by liver and/or neurological degeneration." | 1.30 | [Wilson's disease]. ( Bascone, F; Bonfissuto, G; Carroccio, A; Costanza, G; Magliarisi, C; Montalto, G; Soresi, M, 1997) |
| "Now, the mass-screening for Wilson's disease is in progress." | 1.29 | [Wilson's disease--evolutive panorama of diagnosis and treatment in the last forty years]. ( Arima, M, 1995) |
| "In addition, Wilson's disease patients quite often take vitamin C in high doses in conjunction with Zn therapy, and there are indications of possible interactions among vitamin C, Zn and copper (Cu)." | 1.29 | Treatment of Wilson's disease with zinc: XI. Interaction with other anticopper agents. ( Brewer, GJ; Dick, RD; Johnson, V; Wang, Y; Yuzbasiyan-Gurkan, V, 1993) |
| "Patients with Wilson's disease contemplating pregnancy should have their hepatic function and copper status assessed." | 1.29 | Wilson's disease in pregnancy. ( Goulding, P; Hawthorne, B; Maresh, M; Nunns, D, 1995) |
| "Twenty of 320 patients with Wilson's disease initially presented with chemical and laboratory features of chronic active hepatitis, confirmed histologically in 17." | 1.28 | Prognosis of Wilsonian chronic active hepatitis. ( Scheinberg, IH; Schilsky, ML; Sternlieb, I, 1991) |
| " The bioavailability of TE was below 10% and the plasma levels of TE in non-fasted rats were significantly lower than that observed in fasted rats." | 1.28 | [Intestinal absorption and urinary excretion of triethylenetetramine for Wilson's disease in rat]. ( Iseki, K; Kobayashi, M; Miyazaki, K; Saitoh, H; Sugawara, M, 1990) |
| "Trientine proved to be an effective alternative copper chelating agent in the treatment of Wilson's disease in patients with penicillamine-induced neutropenia, thrombocytopenia, SLE, and nephrosis." | 1.28 | Treatment of Wilson's disease with triethylene tetramine hydrochloride (Trientine). ( Dubois, RS; Hambidge, KM; Rodgerson, DO, 1990) |
| "Wilson's disease is an autosomal recessive disorder characterized by an accumulation of a toxic amount of copper in the body." | 1.28 | Wilson's disease treatment by triethylene tetramine dihydrochloride (trientine, 2HCl): long-term observations. ( Hashimoto, T; Morita, J; Motohiro, T; Okano, Y; Watari, H; Yamashita, F; Yoshida, I; Yoshino, M, 1992) |
| "Seven patients with Wilson's disease, treated with trientine, have been followed during 11 pregnancies." | 1.27 | The management of pregnancy in Wilson's disease treated with trientine. ( Walshe, JM, 1986) |
| "Twenty patients with Wilson's disease in whom severe penicillamine intolerance developed have been managed with the orally active chelating agent trientine dihydrochloride (trien)." | 1.26 | Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride. ( Walshe, JM, 1982) |
| "Wilson's disease is an autosomal recessive disorder characterized by progressive cirrhosis or neurological signs." | 1.26 | Treatment of Wilson's disease with triethylene tetramine dihydrochloride. A case report. ( Berg, M; Haslam, RH; Sass-Kortsak, A; Stout, W, 1980) |
| "Sixty patients with Wilson's disease have been studied by means of computerized cranial tomography (CT)." | 1.26 | Wilson's disease. An analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents. ( Walshe, JM; Williams, FJ, 1981) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 29 (17.47) | 18.7374 |
| 1990's | 34 (20.48) | 18.2507 |
| 2000's | 43 (25.90) | 29.6817 |
| 2010's | 45 (27.11) | 24.3611 |
| 2020's | 15 (9.04) | 2.80 |
| Authors | Studies |
|---|---|
| Jacquelet, E | 1 |
| Poujois, A | 4 |
| Pheulpin, MC | 1 |
| Demain, A | 1 |
| Tinant, N | 1 |
| Gastellier, N | 1 |
| Woimant, F | 2 |
| Kumar, S | 1 |
| Patra, BR | 1 |
| Irtaza, M | 1 |
| Rao, PK | 1 |
| Giri, S | 1 |
| Darak, H | 1 |
| Gopan, A | 1 |
| Kale, A | 1 |
| Shukla, A | 1 |
| Weiss, KH | 13 |
| Kruse, C | 1 |
| Manolaki, N | 1 |
| Zuin, M | 2 |
| Ferenci, P | 6 |
| van Scheppingen, D | 1 |
| Wijnberg, L | 1 |
| de Koning, CE | 1 |
| Dhawan, A | 1 |
| Kerkar, N | 1 |
| Rana, A | 1 |
| Schilsky, ML | 10 |
| Czlonkowska, A | 2 |
| Cassiman, D | 1 |
| Twardowschy, C | 1 |
| Gondim, FAA | 1 |
| Denk, G | 2 |
| Cury, RG | 1 |
| Ott, P | 1 |
| Moore, J | 1 |
| Ala, A | 4 |
| D'Inca, R | 1 |
| Couchonnal-Bedoya, E | 1 |
| D'Hollander, K | 1 |
| Dubois, N | 1 |
| Kamlin, COF | 1 |
| Mohr, I | 4 |
| Bourhis, H | 1 |
| Obadia, MA | 1 |
| Morgil, M | 1 |
| Morvan, E | 1 |
| Merle, U | 5 |
| Pfeiffenberger, J | 6 |
| Eker, E | 1 |
| Antos, A | 1 |
| Członkowska, A | 2 |
| Smolinski, L | 1 |
| Bembenek, J | 1 |
| Przybyłkowski, A | 1 |
| Skowrońska, M | 1 |
| Kurkowska-Jastrzębska, I | 1 |
| Litwin, T | 2 |
| Shimizu, N | 2 |
| Tomiyasu, K | 1 |
| Oshima, T | 1 |
| Yoshii, M | 1 |
| Suzuki, H | 1 |
| Inamasu, J | 1 |
| Izumi, M | 1 |
| Yuan, XZ | 1 |
| Yang, RM | 2 |
| Wang, XP | 2 |
| Pietrocola, F | 1 |
| Castoldi, F | 1 |
| Zischka, H | 1 |
| Kroemer, G | 1 |
| Yu, Z | 1 |
| Sheu Song, J | 1 |
| Schmidt, B | 1 |
| Corey, K | 1 |
| Hawryluk, EB | 1 |
| Mayr, T | 1 |
| Weiler, M | 2 |
| Fichtner, A | 1 |
| Mehrabi, A | 2 |
| Hoffmann, GF | 1 |
| Teufel-Schäfer, U | 1 |
| Janka, SS | 1 |
| Bätzing, J | 1 |
| Laux, G | 1 |
| Holstiege, J | 1 |
| Wahler, S | 1 |
| Mieth, M | 1 |
| Tai, CS | 1 |
| Wu, JF | 1 |
| Chen, HL | 1 |
| Hsu, HY | 1 |
| Chang, MH | 1 |
| Ni, YH | 1 |
| Avan, A | 1 |
| de Bie, RMA | 1 |
| Hoogenraad, TU | 1 |
| Beinhardt, S | 1 |
| Gotthardt, DN | 3 |
| Haag, N | 1 |
| Freissmuth, C | 1 |
| Reuner, U | 3 |
| Gauss, A | 2 |
| Stremmel, W | 8 |
| Smirnova, J | 1 |
| Kabin, E | 1 |
| Järving, I | 1 |
| Bragina, O | 1 |
| Tõugu, V | 1 |
| Plitz, T | 1 |
| Palumaa, P | 1 |
| Shimamura, Y | 1 |
| Maeda, T | 1 |
| Gocho, Y | 1 |
| Ogawa, Y | 1 |
| Tsuji, K | 1 |
| Takizawa, H | 1 |
| Lohse, CM | 1 |
| Gotthardt, D | 2 |
| Rupp, C | 1 |
| Teufel, U | 2 |
| Harada, M | 2 |
| Honma, Y | 2 |
| Yoshizumi, T | 1 |
| Kumamoto, K | 1 |
| Oe, S | 1 |
| Harada, N | 1 |
| Tanimoto, A | 1 |
| Yabuki, K | 1 |
| Karasuyama, T | 1 |
| Yoneda, A | 1 |
| Shibata, M | 2 |
| Appenzeller-Herzog, C | 1 |
| Mathes, T | 1 |
| Heeres, MLS | 1 |
| Houwen, RHJ | 1 |
| Ewald, H | 1 |
| Sharawat, IK | 1 |
| Kurup, A | 1 |
| Sondhi, V | 1 |
| Saini, L | 1 |
| Thurik, F | 1 |
| Schäfer, M | 2 |
| Wiegand, F | 1 |
| Ferenci-Foerster, D | 1 |
| Maieron, A | 1 |
| Stauber, R | 1 |
| Zoller, H | 1 |
| Schmidt, HH | 2 |
| Hefter, H | 2 |
| Trocello, JM | 1 |
| Houwen, RH | 1 |
| Lorincz, MT | 4 |
| Teodoro, T | 1 |
| Neutel, D | 1 |
| Lobo, P | 1 |
| Geraldo, AF | 1 |
| Conceição, I | 1 |
| Rosa, MM | 1 |
| Albuquerque, L | 1 |
| Ferreira, JJ | 1 |
| Purchase, R | 1 |
| Mareček, Z | 1 |
| Brůha, R | 1 |
| Kanwar, P | 1 |
| Kowdley, KV | 1 |
| Chandok, N | 1 |
| Roberts, EA | 2 |
| Mattová, J | 1 |
| Poučková, P | 1 |
| Kučka, J | 1 |
| Skodová, M | 1 |
| Vetrík, M | 1 |
| Stěpánek, P | 1 |
| Urbánek, P | 1 |
| Petřík, M | 1 |
| Nový, Z | 1 |
| Hrubý, M | 1 |
| Hunt, DP | 1 |
| Sahani, DV | 1 |
| Corey, KE | 1 |
| Masia, R | 1 |
| Aliu, E | 1 |
| Herder, M | 1 |
| Hollis, A | 1 |
| Kim, Y | 1 |
| Koide, R | 1 |
| Kawata, A | 1 |
| Chung, EJ | 1 |
| Kim, EG | 1 |
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| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Multicentre, Retrospective and Prospective Study to Assess Long-Term Outcomes of Chelator-Based Treatment With Trientine in Wilson Disease Patients Withdrawn From Therapy With d-Penicillamine[NCT02426905] | Phase 4 | 90 participants (Anticipated) | Interventional | 2016-01-31 | Active, not recruiting | ||
| CHELATE STUDY: Trientine Tetrahydrochloride (TETA 4HCL) for the Treatment of Wilson's Disease[NCT03539952] | Phase 3 | 77 participants (Actual) | Interventional | 2018-09-03 | Completed | ||
| Single Daily Dosage of Trientine for Maintenance Treatment for Wilson Disease[NCT01472874] | 8 participants (Actual) | Interventional | 2010-01-31 | Completed | |||
| [NCT00004339] | Phase 3 | 90 participants | Interventional | 1994-01-31 | Completed | ||
| [NCT00004338] | Phase 4 | 300 participants | Interventional | 1993-10-31 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
24-hour urinary copper excretion (μg/ 24 hr) from urine collected by the patient over a 24-hour period. (NCT03539952)
Timeframe: Week 36
| Intervention | μg/24 hours (Mean) |
|---|---|
| TETA 4HCL | 274.5 |
| Penicillamine | 510.8 |
"The clinician will rate the change in the patient's Wilson's disease relative to the prior study clinic visit using a 7-point scale to a specific statement: 'Please rate the change in the overall severity of the patients Wilson's disease compared to the previous study clinic visit.~Available options were (1) very much improved; (2) much improved; (3) minimally improved; (4) no change; (5) minimally worse; (6), much worse; or (7) very much worse." (NCT03539952)
Timeframe: Week 36
| Intervention | score on a scale (Mean) |
|---|---|
| Penicillamine | 4.1 |
| TETA 4HCL | 3.9 |
The primary outcome of efficacy was serum NCC by speciation assay (μg/L), with comparative analysis of mean difference between the two groups 24 weeks after randomization. The non-inferiority margin was set at -50 μg/L. (NCT03539952)
Timeframe: Week 36
| Intervention | µg/L (Mean) |
|---|---|
| Penicillamine Arm | 46.5 |
| TETA 4HCL Arm | 58.7 |
(NCT01472874)
Timeframe: Months 1,2,3,6,9,12 (mean)
| Intervention | g/dL (Mean) |
|---|---|
| Once a Day Trientine | 0.52 |
(NCT01472874)
Timeframe: Pre Treatment (mean)
| Intervention | g/dL (Mean) |
|---|---|
| Once a Day Trientine | 0.54 |
Alanine transaminase (NCT01472874)
Timeframe: Months 1,2,3,6,9,12 (mean)
| Intervention | U/L (Mean) |
|---|---|
| Once a Day Trientine | 50.89 |
Alanine transaminase (NCT01472874)
Timeframe: Pre Treatment (mean)
| Intervention | U/L (Mean) |
|---|---|
| Once a Day Trientine | 41.38 |
(NCT01472874)
Timeframe: Months 1,2,3,6,9,12 (mean)
| Intervention | mcg/24h (Mean) |
|---|---|
| Once a Day Trientine | 0.52 |
(NCT01472874)
Timeframe: Pre Treatment (mean)
| Intervention | mcg/24h (Mean) |
|---|---|
| Once a Day Trientine | 0.54 |
(NCT01472874)
Timeframe: Months 1,2,3,6,9,12 (mean)
| Intervention | mcg/24hr (Mean) |
|---|---|
| Once a Day Trientine | 313.4 |
(NCT01472874)
Timeframe: Pre Treatment (mean)
| Intervention | mcg/24hr (Mean) |
|---|---|
| Once a Day Trientine | 287.9 |
The International Normalized Ratio (INR) is a standard way to describe the time it takes for blood to clot; an INR range of 0.8 to 1.2 is considered normal for a healthy person who is not using oral anticoagulant therapy (NCT01472874)
Timeframe: Months 1,2,3,6,9,12 (mean)
| Intervention | international normalized ratio (Mean) |
|---|---|
| Once a Day Trientine | 1.05 |
The International Normalized Ratio (INR) is a standard way to describe the time it takes for blood to clot; an INR range of 0.8 to 1.2 is considered normal for a healthy person who is not using oral anticoagulant therapy (NCT01472874)
Timeframe: Pre Treatment (mean)
| Intervention | international normalized ratio (Mean) |
|---|---|
| Once a Day Trientine | 0.99 |
(NCT01472874)
Timeframe: Months 1,2,3,6,9,12 (mean)
| Intervention | mcg/24hr (Mean) |
|---|---|
| Once a Day Trientine | 2214 |
(NCT01472874)
Timeframe: Pre Treatment (mean)
| Intervention | mcg/24hr (Mean) |
|---|---|
| Once a Day Trientine | 1959 |
41 reviews available for trientine and Hepatolenticular Degeneration
| Article | Year |
|---|---|
Wilson Disease in Children.
Topics: Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Penicillamine; Trientine | 2022 |
Early neurological deterioration in Wilson's disease: a systematic literature review and meta-analysis.
Topics: Copper; Hepatolenticular Degeneration; Humans; Nervous System Diseases; Penicillamine; Trientine | 2023 |
[Diagnosis and treatment of Wilson disease in Japan].
Topics: Adolescent; Adult; Biomarkers; Ceruloplasmin; Chelating Agents; Child; Child, Preschool; Copper; Cop | 2019 |
Wilson disease - currently used anticopper therapy.
Topics: Chelating Agents; Copper; Enzyme Inhibitors; Hepatolenticular Degeneration; Humans; Molybdenum; Peni | 2017 |
Wilson Disease: Diagnosis, Treatment, and Follow-up.
Topics: Aftercare; Biopsy; Ceruloplasmin; Chelating Agents; Copper; Copper-Transporting ATPases; Disease Man | 2017 |
Immunoglobulin A nephropathy secondary to Wilson's disease: a case report and literature review.
Topics: Chelating Agents; Drug Therapy, Combination; Glomerulonephritis, IGA; Hepatolenticular Degeneration; | 2019 |
Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta-analysis of controlled studies.
Topics: Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Liver; Molybdenum; Penicillamine; R | 2019 |
Recognition and treatment of neurologic Wilson's disease.
Topics: Ataxia; Dysarthria; Hepatolenticular Degeneration; Humans; Liver; Male; Mutation; Treatment Outcome; | 2012 |
The treatment of Wilson's disease, a rare genetic disorder of copper metabolism.
Topics: Brain; Chelating Agents; Copper; Cornea; Dimercaprol; Drug Discovery; Hepatolenticular Degeneration; | 2013 |
[Wilsons disease].
Topics: Adenosine Triphosphatases; Adult; Cation Transport Proteins; Chelating Agents; Copper; Copper-Transp | 2013 |
Metal storage disorders: Wilson disease and hemochromatosis.
Topics: Biopsy; Chelating Agents; Disease Progression; Genetic Predisposition to Disease; Genetic Testing; H | 2014 |
Clinical considerations for an effective medical therapy in Wilson's disease.
Topics: Ceruloplasmin; Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Molybdenum; Penicill | 2014 |
Fair pricing of "old" orphan drugs: considerations for Canada's orphan drug policy.
Topics: Canada; Chelating Agents; Costs and Cost Analysis; Drug Costs; Health Policy; Hepatolenticular Degen | 2015 |
Trientine induced colitis during therapy for Wilson disease: a case report and review of the literature.
Topics: Adult; Chelating Agents; Colitis; Female; Hepatolenticular Degeneration; Humans; Trientine; Withhold | 2015 |
Current Drug Managements of Wilson's Disease: From West to East.
Topics: Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Medicine, Chinese Traditional; Moly | 2016 |
The risks of free copper in the body and the development of useful anticopper drugs.
Topics: Alzheimer Disease; Angiogenesis Inhibitors; Autoimmunity; Copper; Fibrosis; Hepatolenticular Degener | 2008 |
Triethylenetetramine pharmacology and its clinical applications.
Topics: Animals; Chelating Agents; Hepatolenticular Degeneration; Humans; Trientine | 2010 |
Wilson's disease.
Topics: Adolescent; Biopsy, Needle; Blood Chemical Analysis; Child; Child, Preschool; Combined Modality Ther | 2002 |
[Wilson's disease: physiopathological, clinical and therapeutic considerations].
Topics: Adenosine Triphosphatases; Adolescent; Adult; Basal Ganglia Diseases; Brain; Cation Transport Protei | 2003 |
Antiangiogenic therapy through copper chelation.
Topics: Angiogenesis Inhibitors; Animals; Chelating Agents; Chelation Therapy; Copper; Corneal Neovasculariz | 2003 |
Wilson disease.
Topics: Adenosine Triphosphatases; Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Liver Tr | 2003 |
[Wilson disease].
Topics: Adenosine Triphosphatases; Age of Onset; Biomarkers; Cation Transport Proteins; Ceruloplasmin; Chela | 2004 |
[Wilson disease in 2003].
Topics: Adenosine Triphosphatases; Cation Transport Proteins; Chelating Agents; Copper; Copper-Transporting | 2003 |
Neurologically presenting Wilson's disease: epidemiology, pathophysiology and treatment.
Topics: Behavior; Chelating Agents; Enzyme Inhibitors; Hepatolenticular Degeneration; Humans; Molybdenum; Ne | 2005 |
A case study: identifying a new case of Wilson's disease.
Topics: Abdominal Pain; Adult; Chelating Agents; Diagnosis, Differential; Drug Monitoring; Drug Therapy, Com | 2005 |
[Wilson disease: an update].
Topics: Adenosine Triphosphatases; Cation Transport Proteins; Copper; Copper-Transporting ATPases; Diagnosis | 2006 |
Wilson's disease.
Topics: Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Liver Transplantation; Penicillamin | 2007 |
Wilson disease--a practical approach to diagnosis, treatment and follow-up.
Topics: Ceruloplasmin; Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Liver Transplantatio | 2007 |
[Current problems in Wilson's disease].
Topics: Basal Ganglia; Ceruloplasmin; Copper; Cornea; Hepatolenticular Degeneration; Humans; Kidney; Liver; | 1984 |
[Wilson's disease (hepatolenticular degeneration)].
Topics: Adolescent; Adult; Anemia, Hemolytic; Central Nervous System Diseases; Child; Combined Modality Ther | 1984 |
Practical recommendations and new therapies for Wilson's disease.
Topics: Chelating Agents; Copper; Female; Hepatolenticular Degeneration; Humans; Liver; Metallothionein; Mol | 1995 |
Wilson disease: genetic basis of copper toxicity and natural history.
Topics: Adenosine Triphosphatases; Carrier Proteins; Cation Transport Proteins; Chelating Agents; Chelation | 1996 |
[Therapy of Wilson disease].
Topics: Adolescent; Adult; Ceruloplasmin; Chelating Agents; Child; Copper; Female; Hepatolenticular Degenera | 1999 |
Recognition, diagnosis, and management of Wilson's disease.
Topics: Adolescent; Adult; Chelating Agents; Child; Copper; Hepatolenticular Degeneration; Humans; Penicilla | 2000 |
Wilson's disease and pregnancy.
Topics: Chelating Agents; Female; Hepatolenticular Degeneration; Humans; Penicillamine; Pregnancy; Pregnancy | 2000 |
Treatment of Wilson's disease: what are the relative roles of penicillamine, trientine, and zinc supplementation?
Topics: Adenosine Triphosphatases; Chelating Agents; Drug Therapy, Combination; Female; Hepatolenticular Deg | 2001 |
Diagnosis and treatment of Wilson's disease.
Topics: Chelating Agents; Copper; Dimercaprol; Enzyme Inhibitors; Female; Hepatolenticular Degeneration; Hum | 2002 |
[Wilson's disease].
Topics: Acetates; Acetic Acid; Adult; Diagnosis, Differential; Diet; Female; Hepatolenticular Degeneration; | 1992 |
Wilson's disease: current status.
Topics: Administration, Oral; Ceruloplasmin; Copper; Copper Radioisotopes; Drug Monitoring; Genetic Testing; | 1992 |
Wilson's disease: an update, with emphasis on new approaches to treatment.
Topics: Copper; Female; Hepatolenticular Degeneration; Humans; Molybdenum; Penicillamine; Pregnancy; Trienti | 1989 |
Treatment of Wilson's disease.
Topics: Copper; Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Penicillamine; Pregnancy; T | 1987 |
3 trials available for trientine and Hepatolenticular Degeneration
122 other studies available for trientine and Hepatolenticular Degeneration
| Article | Year |
|---|---|
Adherence to treatment, a challenge even in treatable metabolic rare diseases: A cross sectional study of Wilson's disease.
Topics: Adolescent; Adult; Anxiety; Chelating Agents; Child; Copper; Cross-Sectional Studies; Depression; Fe | 2021 |
Adverse Events with D-penicillamine Therapy in Hepatic Wilson's Disease: A Single-Center Retrospective Audit.
Topics: Chelating Agents; Hepatolenticular Degeneration; Humans; Penicillamine; Retrospective Studies; Trien | 2022 |
Multicentre, retrospective study to assess long-term outcomes of chelator based treatment with trientine in Wilson disease patients withdrawn from therapy with d -penicillamine.
Topics: Chelating Agents; Hepatolenticular Degeneration; Humans; Penicillamine; Retrospective Studies; Trien | 2022 |
Experience on switching trientine formulations in Wilson disease: Efficacy and safety after initiation of TETA 4HCl as substitute for TETA 2HCl.
Topics: Adult; Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Retrospective Studies; Trans | 2023 |
Triethylenetetramine (TETA).
Topics: Animals; Copper; Hepatolenticular Degeneration; Humans; Mice; No-Observed-Adverse-Effect Level; Trie | 2023 |
Neurological worsening in Wilson disease - clinical classification and outcome.
Topics: Copper; Female; Hepatolenticular Degeneration; Humans; Penicillamine; Trientine; Zinc | 2023 |
[Wilson's disease presenting as Asperger syndrome].
Topics: Administration, Oral; Adult; Asperger Syndrome; Brain; Diagnosis, Differential; Hepatolenticular Deg | 2019 |
Management Perspective of Wilson's Disease: Early Diagnosis and Individualized Therapy.
Topics: Copper; Early Diagnosis; Hepatolenticular Degeneration; Humans; Penicillamine; Trientine | 2021 |
Extending the mode of action of triethylenetetramine (trientine): Autophagy besides copper chelation.
Topics: Autophagy; Chelating Agents; Copper; Hepatocytes; Hepatolenticular Degeneration; Humans; Trientine | 2020 |
Pediatric Pseudo-pseudoxanthoma Elasticum Resulting From D-Penicillamine Treatment for Wilson Disease.
Topics: Adolescent; Adult; Hepatolenticular Degeneration; Humans; Male; Penicillamine; Pseudoxanthoma Elasti | 2020 |
Optimized Trientine-dihydrochloride Therapy in Pediatric Patients With Wilson Disease: Is Weight-based Dosing Justified?
Topics: Adolescent; Chelating Agents; Child; Copper; Hepatolenticular Degeneration; Humans; Retrospective St | 2021 |
[Retrospective cross-sectional study based on nationwide data of drug prescriptions and contractional data of outpatient offices regarding Morbus Wilson's disease].
Topics: Cross-Sectional Studies; Drug Prescriptions; Germany; Hepatolenticular Degeneration; Humans; Penicil | 2020 |
Modality of treatment and potential outcome of Wilson disease in Taiwan: A population-based longitudinal study.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Hepatolenticular Degeneration; Humans; Liver Tra | 2018 |
Wilson's Disease Should Be Treated with Zinc rather than Trientine or Penicillamine.
Topics: Hepatolenticular Degeneration; Humans; Penicillamine; Trientine; Zinc | 2017 |
Pregnancy in Wilson's disease: Management and outcome.
Topics: Adolescent; Adult; Female; Hepatolenticular Degeneration; Humans; Liver; Liver Function Tests; Pregn | 2018 |
Copper(I)-binding properties of de-coppering drugs for the treatment of Wilson disease. α-Lipoic acid as a potential anti-copper agent.
Topics: Apoptosis; Carrier Proteins; Cell Line; Cell Proliferation; Chelating Agents; Copper; Copper Transpo | 2018 |
Long-term evaluation of urinary copper excretion and non-caeruloplasmin associated copper in Wilson disease patients under medical treatment.
Topics: Adolescent; Adult; Ceruloplasmin; Chelating Agents; Child; Child, Preschool; Copper; Female; Germany | 2019 |
Idiopathic copper toxicosis: is abnormal copper metabolism a primary cause of this disease?
Topics: Carcinoma, Hepatocellular; Ceruloplasmin; Chelating Agents; Chemical and Drug Induced Liver Injury; | 2020 |
Boy with Dysarthria and Frequent Falls: A Treatable Disorder.
Topics: Accidental Falls; Brain; Chelating Agents; Child; Dysarthria; Hepatolenticular Degeneration; Humans; | 2019 |
Efficacy and safety of oral chelators in treatment of patients with Wilson disease.
Topics: Adolescent; Adult; Austria; Chelating Agents; Child; Child, Preschool; Cohort Studies; Drug-Related | 2013 |
Recovery after copper-deficiency myeloneuropathy in Wilson's disease.
Topics: Adult; Copper; Deficiency Diseases; Hepatolenticular Degeneration; Humans; Male; Nervous System Dise | 2013 |
The trientine crisis in Canada: a call to advocacy.
Topics: Canada; Chelating Agents; Consumer Advocacy; Hepatolenticular Degeneration; Humans; Prescription Fee | 2014 |
Chelating polymeric beads as potential therapeutics for Wilson's disease.
Topics: Administration, Oral; Animals; Brain; Chelating Agents; Copper; Female; Gastrointestinal Tract; Hepa | 2014 |
Case records of the Massachusetts General Hospital. Case 30-2014. A 29-year-old man with diarrhea, nausea, and weight loss.
Topics: Adult; Biopsy; Chelating Agents; Chronic Disease; Copper; Diagnosis, Differential; Diarrhea; Hepatit | 2014 |
[Magnetic Resonance Imaging Improvement in a Patient with Wilson's Disease Following Treatment with Trientine Hydrochloride and Zinc Acetate].
Topics: Adult; Drug Combinations; Hepatolenticular Degeneration; Humans; Magnetic Resonance Imaging; Male; T | 2015 |
Wilson's disease with cognitive impairment and without extrapyramidal signs: improvement of neuropsychological performance and reduction of MRI abnormalities with trientine treatment.
Topics: Brain; Chelating Agents; Cognition Disorders; Female; Hepatolenticular Degeneration; Humans; Magneti | 2016 |
Concomitant immune-related events in Wilson disease: implications for monitoring chelator therapy.
Topics: Adolescent; Adult; Antibodies, Antinuclear; Autoimmune Diseases; Chelating Agents; Child; Cross-Sect | 2016 |
Coagulation Parameters in Wilson Disease.
Topics: Adult; Biomarkers; Blood Coagulation; Blood Coagulation Factors; Chelating Agents; Cross-Sectional S | 2015 |
Zinc Therapy for Wilson Disease in Children in French Pediatric Centers.
Topics: Abdominal Pain; Adolescent; Chelating Agents; Child; Child, Preschool; Copper; Female; France; Healt | 2015 |
The spectrum of psychiatric symptoms in Wilson's disease: treatment and prognostic considerations.
Topics: Adult; Antidepressive Agents; Antipsychotic Agents; Capgras Syndrome; Chelating Agents; Depressive D | 2015 |
Pregnancy outcome after chelation therapy in Wilson disease. Evaluation of the German Embryotox Database.
Topics: Adult; Chelating Agents; Chelation Therapy; Copper; Databases, Factual; Female; Germany; Hepatolenti | 2016 |
INTERACTIVE MEDICAL CASE. Tracing the Cause of Abdominal Pain.
Topics: Abdominal Pain; Biopsy; Ceruloplasmin; Chelating Agents; Copper; Fanconi Syndrome; Female; Gallbladd | 2016 |
[Wilson's disease in paediatric age: diagnosis and treatment. Recent advances].
Topics: Adenosine Triphosphatases; Astringents; Cation Transport Proteins; Ceruloplasmin; Chelating Agents; | 2008 |
Education and imaging. Hepatobiliary and pancreatic: hypersensitivity pneumonitis induced by penicillamine.
Topics: Alveolitis, Extrinsic Allergic; Chelating Agents; Female; Hepatolenticular Degeneration; Humans; Liv | 2009 |
Treatment of Wilson's disease with tetrathiomolybdate: V. Control of free copper by tetrathiomolybdate and a comparison with trientine.
Topics: Chelating Agents; Clinical Trials as Topic; Copper; Double-Blind Method; Drug Administration Schedul | 2009 |
Oral complications associated with D-penicillamine treatment for Wilson disease: a clinicopathologic report.
Topics: Adult; Candidiasis, Oral; Cheilitis; Chelating Agents; Elastic Tissue; Female; Follow-Up Studies; Gi | 2010 |
Teaching NeuroImages: MRI reversal in Wilson disease with trientine treatment.
Topics: Adolescent; Brain; Hepatolenticular Degeneration; Humans; Magnetic Resonance Imaging; Male; Treatmen | 2010 |
Prognostic significance of neurologic examination findings in Wilson disease.
Topics: Adult; Chelating Agents; Dystonia; Female; Hepatolenticular Degeneration; Humans; Male; Molybdenum; | 2011 |
Excess copper chelating therapy for Wilson disease induces anemia and liver dysfunction.
Topics: Adult; Anemia; Ceruloplasmin; Chelating Agents; Chelation Therapy; Copper; Hemochromatosis; Hepatole | 2011 |
Serum 'free' copper in Wilson disease.
Topics: Adolescent; Adult; Ceruloplasmin; Chelating Agents; Child; Child, Preschool; Copper; Hepatolenticula | 2012 |
[Diagnosis and care of Wilson disease with neurological revelation].
Topics: Adolescent; Brain; Chelating Agents; Female; Hepatolenticular Degeneration; Humans; Liver Transplant | 2012 |
A copper for your thoughts.
Topics: Adult; Chelating Agents; Copper; Female; Hepatolenticular Degeneration; Humans; Insurance, Life; Pen | 2012 |
An unusual cause of headache and hypertension.
Topics: Adolescent; Chelating Agents; Diagnosis, Differential; Female; Headache; Hepatolenticular Degenerati | 2013 |
Histologic evolution and long-term outcome of Wilson's disease: results of a single-center experience.
Topics: Adolescent; Adult; Biopsy; Chelating Agents; Chi-Square Distribution; Child; Child, Preschool; Disea | 2013 |
Trientine-induced neurological deterioration in a patient with Wilson's disease.
Topics: Brain; Chelating Agents; Copper; Dystonia; Gait Disorders, Neurologic; Hepatolenticular Degeneration | 2013 |
[Electrophysiological impairment profile of patients with Wilson's disease].
Topics: Autonomic Nervous System Diseases; Basal Ganglia Diseases; Cerebral Cortex; Chelating Agents; Diagno | 2003 |
Treatment of Wilson's disease with zinc. XVIII. Initial treatment of the hepatic decompensation presentation with trientine and zinc.
Topics: Adult; Drug Therapy, Combination; Female; Hepatolenticular Degeneration; Humans; Liver Failure; Male | 2003 |
[Wilson's disease with severe neurological manifestations: response to trientine plus zinc therapy].
Topics: Adolescent; Chelating Agents; Hepatolenticular Degeneration; Humans; Male; Nervous System Diseases; | 2004 |
[Pathogenesis and treatment of Wilson's disease].
Topics: Chelating Agents; Copper; Hepatolenticular Degeneration; Humans; Intestinal Absorption; Penicillamin | 2003 |
Clinical correlation of brain MRI and MRS abnormalities in patients with Wilson disease.
Topics: Adult; Aged; Aspartic Acid; Atrophy; Brain; Brain Chemistry; Chelation Therapy; Copper; Dipeptides; | 2004 |
Improvement of cardiovascular autonomic dysfunction following anti-copper therapy in Wilson's disease.
Topics: Cardiovascular System; Chelating Agents; Copper; Female; Hepatolenticular Degeneration; Humans; Magn | 2005 |
Wilson's disease with depression and parkinsonism.
Topics: Adult; Antiparkinson Agents; Brain; Bromocriptine; Chelating Agents; Depressive Disorder; Female; He | 2005 |
[Acute liver failure and hemolysis in a 16-year-old woman. First manifestation of Wilson's disease].
Topics: Acute Disease; Adolescent; Anemia, Hemolytic; Biopsy, Needle; Chelating Agents; Copper; Female; Foll | 2005 |
Two male patients with Wilson's disease treated using trientine and iron reduction therapy.
Topics: Adult; Chelating Agents; Copper; Ferritins; Hemoglobins; Hepatolenticular Degeneration; Humans; Male | 2005 |
Combination treatment with penicillamine and trientine in a patient with Wilson's disease.
Topics: Adult; Chelating Agents; Drug Therapy, Combination; Hepatolenticular Degeneration; Humans; Male; Pen | 2005 |
Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study.
Topics: Adenosine Triphosphatases; Adolescent; Adult; Cation Transport Proteins; Ceruloplasmin; Chelating Ag | 2007 |
[Wilson's disease: forms of presentation in childhood].
Topics: Chelating Agents; Child; Early Diagnosis; Hepatolenticular Degeneration; Humans; Penicillamine; Prog | 2006 |
The bane of a silent illness: when Wilson's disease takes its course.
Topics: Adult; Chelating Agents; Cognitive Behavioral Therapy; Female; Hepatolenticular Degeneration; Humans | 2006 |
Discontinuation of penicillamine in the absence of alternative orphan drugs (trientine-zinc): a case of decompensated liver cirrhosis in Wilson's disease.
Topics: Adult; Chelating Agents; Fatal Outcome; Female; Hepatolenticular Degeneration; Humans; Liver Cirrhos | 2007 |
Wilson's disease.
Topics: Chelating Agents; Drug Administration Schedule; Drug Interactions; Drug Therapy, Combination; Hepato | 2007 |
Wilson disease in children: serum aminotransferases and urinary copper on triethylene tetramine dihydrochloride (trientine) treatment.
Topics: Algorithms; Chelating Agents; Child; Copper; Hepatolenticular Degeneration; Humans; Patient Complian | 2007 |
Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German patients with treated Wilson's disease.
Topics: Adult; Age of Onset; Antirheumatic Agents; Chelating Agents; Drug Therapy, Combination; Female; Germ | 2008 |
Cold comfort pharm.
Topics: Adult; Chelating Agents; Cold Temperature; Copper; Deglutition Disorders; Drug Storage; Dystonia; Fe | 2008 |
Once daily trientine for maintenance therapy of Wilson disease.
Topics: Adult; Aged; Drug Administration Schedule; Female; Hepatolenticular Degeneration; Humans; Male; Trie | 2008 |
Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride.
Topics: Adolescent; Adult; Brain; Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Male; Pen | 1982 |
Wilson's disease, an end to the search for new therapy?
Topics: Animals; Ethylenediamines; Hepatolenticular Degeneration; Humans; Penicillamine; Risk; Trientine | 1982 |
Wilson's disease: a 1984 perspective.
Topics: Adolescent; Adult; Bone and Bones; Ceruloplasmin; Child; Child, Preschool; Copper; Diagnosis, Differ | 1984 |
Treatment of Wilson's disease.
Topics: Copper; Diagnostic Errors; Hepatolenticular Degeneration; Humans; Penicillamine; Trientine; Zinc | 1983 |
Wilson's disease in one identical twin and treatment by triethylene tetramine 2HCl in another case.
Topics: Adolescent; Adult; Diseases in Twins; Ethylenediamines; Female; Hepatolenticular Degeneration; Human | 1983 |
The effect of chelation therapy on the amino aciduria and peptiduria of Wilson's disease.
Topics: Adolescent; Adult; Amino Acids; Chelating Agents; Child; Ethylenediamines; Female; Follow-Up Studies | 1983 |
Wilson's disease. An analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents.
Topics: Adolescent; Adult; Atrophy; Brain; Cerebral Ventricles; Child; Dilatation, Pathologic; Ethylenediami | 1981 |
Treatment of Wilson's disease with triethylene tetramine dihydrochloride. A case report.
Topics: Adolescent; Copper; Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Penicillamine; | 1980 |
Wilson's disease in pregnancy.
Topics: Adolescent; Copper; Female; Hepatolenticular Degeneration; Humans; Liver; Male; Penicillamine; Pregn | 1995 |
Long-term treatment of Wilson's disease with triethylene tetramine dihydrochloride (trientine).
Topics: Adolescent; Adult; Biopsy; Chelating Agents; Child; Colon; Drug Administration Schedule; Duodenum; F | 1995 |
[Wilson's disease--evolutive panorama of diagnosis and treatment in the last forty years].
Topics: Adult; Hepatolenticular Degeneration; History, 20th Century; Humans; Penicillamine; Trientine | 1995 |
The mechanisms of penicillamine, trientine, and zinc in the treatment of Wilson's disease.
Topics: Hepatolenticular Degeneration; Humans; Penicillamine; Trientine; Zinc | 1995 |
[Wilson's disease: physiopathology, therapeutic approach and case report].
Topics: Adult; Copper; Hepatolenticular Degeneration; Humans; Liver; Male; Nephrotic Syndrome; Penicillamine | 1994 |
Wilson's disease treated with trientine during pregnancy.
Topics: Adult; Ceruloplasmin; Copper; Female; Fetal Blood; Hepatolenticular Degeneration; Humans; Infant, Ne | 1995 |
Fate of orally administered triethylenetetramine dihydrochloride: a therapeutic drug for Wilson's disease.
Topics: Adult; Chromatography, High Pressure Liquid; Copper; Hepatolenticular Degeneration; Humans; Hydrolys | 1993 |
Neurological Wilson's disease.
Topics: Child; Hepatolenticular Degeneration; Humans; Penicillamine; Trientine | 1993 |
Chelation treatment of neurological Wilson's disease.
Topics: Adolescent; Adult; Cause of Death; Chelation Therapy; Copper; Drug Administration Schedule; Female; | 1993 |
[A study of trientine therapy in Wilson's disease with neurological symptoms].
Topics: Adolescent; Adult; Dysarthria; Dystonia; Female; Hepatolenticular Degeneration; Humans; Male; Penici | 1993 |
Acquired sideroblastic anaemia during treatment of Wilson's disease with triethylene tetramine dihydrochloride.
Topics: Adolescent; Anemia, Sideroblastic; Female; Hepatolenticular Degeneration; Humans; Trientine | 1993 |
Treatment of Wilson's disease with zinc: XI. Interaction with other anticopper agents.
Topics: Ascorbic Acid; Copper; Copper Radioisotopes; Drug Interactions; Drug Therapy, Combination; Hepatolen | 1993 |
[Disposition behavior and absorption mechanism of trientine, an orphan drug for Wilson's disease].
Topics: Animals; Biogenic Polyamines; Chelating Agents; Child; Female; Hepatolenticular Degeneration; Humans | 1996 |
Acquired sideroblastic anaemia induced by a copper-chelating agent.
Topics: Adolescent; Anemia, Sideroblastic; Chelating Agents; Copper; Female; Hepatolenticular Degeneration; | 1996 |
[Wilson's disease].
Topics: Adolescent; Adult; Chelating Agents; Diagnosis, Differential; Hepatolenticular Degeneration; Humans; | 1997 |
Effect of treatment of Wilson's disease on natural history of haemochromatosis.
Topics: Adult; Chelating Agents; Hemochromatosis; Hepatolenticular Degeneration; Homozygote; Humans; Liver; | 1998 |
Early onset of nephrotic syndrome after treatment with D-penicillamine in a patient with Wilson's disease.
Topics: Chelating Agents; Child; Hepatolenticular Degeneration; Humans; Male; Nephrotic Syndrome; Penicillam | 1998 |
Haemolytic onset of Wilson disease in a patient with homozygous truncation of ATP7B at Arg1319.
Topics: Adenosine Triphosphatases; Adult; Anemia, Hemolytic; Carrier Proteins; Cation Transport Proteins; Ch | 2001 |
Ultrastructural identification of iron and copper accumulation in the liver of a male patient with Wilson disease.
Topics: Adult; Biopsy; Ceruloplasmin; Chelating Agents; Copper; Electron Probe Microanalysis; Ferritins; Hep | 2001 |
Personality traits in treated Wilson's disease determined by means of the Karolinska Scales of Personality (KSP).
Topics: Adult; Aggression; Arousal; Female; Follow-Up Studies; Hepatolenticular Degeneration; Hostility; Hum | 2001 |
Dense Kayser-Fleischer ring in asymptomatic Wilson's disease (hepatolenticular degeneration).
Topics: Chelating Agents; Child; Copper; Corneal Diseases; Hepatolenticular Degeneration; Humans; Liver; Mal | 2002 |
Preparation of and clinical experiences with trien for the treatment of Wilson's disease in absolute intolerance of D-penicillamine.
Topics: Adult; Drug Tolerance; Female; Hepatolenticular Degeneration; Humans; Penicillamine; Trientine | 1977 |
The management of Wilson's disease with trienthylene tetramine 2HC1 (Trien 2HC1).
Topics: Drug Hypersensitivity; Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Male; Penici | 1979 |
[Treatment of Wilson's disease using triethylenetetramine dihydrochloride (TETA)].
Topics: Adult; Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Male; Methods; Trientine | 1978 |
The effect of certain chelating compounds on the urinary excretion of copper by the rat: observations on their clinical significance.
Topics: Animals; Chelating Agents; Copper; Drug Evaluation, Preclinical; Ethylenediamines; Hepatolenticular | 1977 |
Wilson's disease treatment by triethylene tetramine dihydrochloride (trientine, 2HCl): long-term observations.
Topics: Adolescent; Adult; Alanine Transaminase; Aspartate Aminotransferases; Chelating Agents; Copper; Fema | 1992 |
Kayser-Fleischer rings in a patient with basal cell carcinoma: fortuitous diagnosis of presymptomatic Wilson's disease.
Topics: Adult; Alkaline Phosphatase; Aspartate Aminotransferases; Blood Proteins; Carcinoma, Basal Cell; Cer | 1992 |
Treatment of Wilson's disease: penicillamine or triene?
Topics: Copper; Hepatolenticular Degeneration; Humans; Magnetic Resonance Imaging; Penicillamine; Trientine; | 1992 |
Comparison of disposition behavior and de-coppering effect of triethylenetetramine in animal model for Wilson's disease (Long-Evans Cinnamon rat) with normal Wistar rat.
Topics: Animals; Copper; Disease Models, Animal; Hepatolenticular Degeneration; In Vitro Techniques; Liver; | 1992 |
Mode of action of triethylenetetramine dihydrochloride on copper metabolism in Wilson's disease.
Topics: Adult; Copper; Drug Administration Schedule; Drug Therapy, Combination; Female; Hepatolenticular Deg | 1991 |
Triethylene-tetramine (trien) therapy for Wilson's disease.
Topics: Adolescent; Adult; Chelating Agents; Copper; Female; Hepatolenticular Degeneration; Humans; Male; Pe | 1991 |
Excellent prognosis in Wilsonian chronic active hepatitis: new data or an article of faith?
Topics: Hepatitis, Chronic; Hepatolenticular Degeneration; Humans; Liver Cirrhosis; Penicillamine; Prognosis | 1991 |
Prognosis of Wilsonian chronic active hepatitis.
Topics: Adolescent; Adult; Alanine Transaminase; Aspartate Aminotransferases; Bilirubin; Child; Copper; Fema | 1991 |
A comparison of the effects of penicillamine, trientine, and trithiomolybdate on [35S]-labeled metallothionein in vitro; implications for Wilson's disease therapy.
Topics: Animals; Copper; Cytosol; Hepatolenticular Degeneration; Liver; Male; Metallothionein; Molybdenum; P | 1991 |
[Intestinal absorption and urinary excretion of triethylenetetramine for Wilson's disease in rat].
Topics: Administration, Oral; Animals; Biological Availability; Eating; Hepatolenticular Degeneration; Intes | 1990 |
Involvement of corticospinal tract in Wilson's disease. A study of three cases with transcranial stimulation.
Topics: Adolescent; Adult; Electric Stimulation; Female; Hepatolenticular Degeneration; Humans; Male; Muscle | 1990 |
Treatment of Wilson's disease with triethylene tetramine hydrochloride (Trientine).
Topics: Administration, Oral; Adolescent; Adult; Drug Evaluation; Ethylenediamines; Female; Hepatolenticular | 1990 |
Wilson's disease presenting with features of hepatic dysfunction: a clinical analysis of eighty-seven patients.
Topics: Adolescent; Child; Copper; Female; Hepatolenticular Degeneration; Humans; Liver Function Tests; Male | 1989 |
[A case of Wilson's disease recovering from severe brain damage--special reference to trientine and D-penicillamine therapy].
Topics: Adolescent; Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Penicillamine; Trientin | 1989 |
Diagnosis and treatment of presymptomatic Wilson's disease.
Topics: Adolescent; Adult; Chelating Agents; Child; Copper; Female; Hepatolenticular Degeneration; Humans; L | 1988 |
[Control of the therapeutic prevention of copper uptake in the liver in Wilson's disease following oral administration of 64Cu].
Topics: Copper Radioisotopes; Ethylenediamines; Hepatolenticular Degeneration; Humans; Liver; Penicillamine; | 1988 |
Drug therapy in Wilson's disease.
Topics: Ethylenediamines; Hepatolenticular Degeneration; Humans; Trientine | 1988 |
[New possibilities in the treatment of Wilson-Konovalov disease (hepatolenticular degeneration)].
Topics: Adolescent; Adult; Drug Evaluation; Drug Tolerance; Female; Hepatolenticular Degeneration; Humans; P | 1987 |
Neurological abnormalities in Wilson's disease are reversible.
Topics: Adolescent; Child; Child Behavior Disorders; Ethylenediamines; Female; Hepatolenticular Degeneration | 1987 |
The use of trientine in preventing the effects of interrupting penicillamine therapy in Wilson's disease.
Topics: Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Male; Middle Aged; Patient Complian | 1987 |
[Trientin in Wilson's disease. Therapeutic possibilities in patients who cannot tolerate penicillamine].
Topics: Adult; Drug Hypersensitivity; Ethylenediamines; Hepatolenticular Degeneration; Humans; Male; Penicil | 1987 |
The management of pregnancy in Wilson's disease treated with trientine.
Topics: Adult; Ceruloplasmin; Copper; Ethylenediamines; Female; Hepatolenticular Degeneration; Humans; Pregn | 1986 |
Orphan drugs.
Topics: Caprylates; Carnitine; Chelating Agents; Cholelithiasis; Drug Industry; Glycerides; Hepatolenticular | 1986 |
[Triethylene tetramine in Wilson's disease].
Topics: Adult; Ethylenediamines; Hepatolenticular Degeneration; Humans; Male; Penicillamine; Trientine | 1986 |
Trientine for Wilson's disease.
Topics: Ethylenediamines; Hepatolenticular Degeneration; Humans; Penicillamine; Trientine | 1986 |