Page last updated: 2024-08-22

tiletamine hydrochloride and Tyrosinemias

tiletamine hydrochloride has been researched along with Tyrosinemias in 133 studies

Research

Studies (133)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's41 (30.83)29.6817
2010's77 (57.89)24.3611
2020's15 (11.28)2.80

Authors

AuthorsStudies
Höller, A; Janzen, N; Jörg-Streller, M; Karall, D; Ramoni, A; Ramoser, G; Scholl-Bürgi, S; Zöggeler, T1
Aktuglu Zeybek, AC; Cansever, MS; Erel, O; Iscan, HZ; Kiykim, E; Neselioglu, S; Zubarioglu, T1
Balouch, F; Bhushan, S; Ee, L; Hodgkinson, P; Lampe, G; Lewindon, P; McGill, J; Noble, C1
Arnoux, JB; Bou-Gharios, G; Davison, AS; Gallagher, JA; Hughes, AT; Imrich, R; M, K; Milan, AM; Norman, BP; Olsson, B; Ranganath, LR; Rudebeck, M1
Heiner-Fokkema, MR; Lubout, CMA; Rodenburg, IL; van der Klauw, MM; van Ginkel, WG; van Spronsen, FJ; van Vliet, K; Wolffenbuttel, BHR1
Bou-Gharios, G; Gallagher, JA; Hughes, AT; Hughes, JH; Jarvis, JC; Judd, S; Milan, AM; Ranganath, LR; Sutherland, H; Wilson, PJM1
Aldámiz-Echevarría, L; Bueno, M; Couce, ML; Díaz-Fernández, C; García-Volpe, C; Gil, D; Hernández, T; López-Ruzafa, E; Martín-Hernández, E; Navas, V; Peña-Quintana, L; Pintos, G; Roca, I; Sánchez-Pintos, P; Sánchez-Valverde, F; Vitoria, I1
Chelliah, Y; Chen, J; Cox, KH; de Groot, M; Kumar, V; Siepka, SM; Takahashi, JS; Tu, B; Yang, S1
Harding, CO; Heiner-Fokkema, MR; Hollak, CEM; Rodenburg, IL; van Ginkel, WG; van Spronsen, FJ1
Burgerhof, JGM; Daly, A; de Blaauw, P; Heiner-Fokkema, MR; Kienstra, NS; MacDonald, A; Rodenburg, IL; Santra, S; van de Krogt, J; van Ginkel, WG; van Reemst, HE; van Spronsen, FJ1
Bierau, J; Cassiman, D; Daly, A; De Laet, C; Eyskens, F; Gissen, P; Goyens, PJ; Huijbregts, SCJ; Jahja, R; Jameson, EA; MacDonald, A; McKiernan, PJ; Morris, AAM; Mumford, N; Rahman, Y; Timmer, C; van Ginkel, WG; van Hasselt, PM; van Spronsen, FJ; van Vliet, K; Vara, R; Wilcox, G1
Hong, F; Huang, X; Liu, C; Qian, G; Shu, Q; Tong, F; Wu, D; Yang, R; Zhang, T; Zhao, Z; Zhou, X1
Almuqbil, M; Chinsky, JM; Knoll, J1
Äärelä, L; Hiltunen, P; Kurppa, K; Nevalainen, PI1
Gavrilov, DK; Matern, D; Netzel, BC; Oglesbee, D; Pino, GB; Raymond, KM; Rinaldo, P; Schultz, MJ; Singh, RH; Smith, WE; Tortorelli, S1
Äärelä, L; Heikinheimo, M; Hiltunen, P; Huhtala, H; Kivelä, L; Kurppa, K; Nevalainen, PI; Soini, T; Vuorela, N1
Branson, S; Casimir, G; Claes, P; Colemonts-Vroninks, H; De Kock, J; Goyens, P; Marcelis, L; Martens, GA; Neuckermans, J; Vanhaecke, T1
Bärhold, F; Beblo, S; Bogovic, N; Dahl, SV; Das, AM; Dokoupil, K; Fekete, A; Fischer, A; Freisinger, P; Heddrich-Ellerbrok, M; Hochuli, M; Jörg-Streller, M; Korpel, V; Lahl, J; Lier, D; Mayorandan, S; Meyer, U; Möslinger, D; Neugebauer, AK; Och, U; Rohde, C; Rosenbaum-Fabian, S; Thimm, EM; van Teeffelen-Heithoff, A1
Couce, ML; Das, AM; de Laet, C; Dionisi-Vici, C; Lund, AM; Rudebeck, M; Schiff, M; Spada, M; Sparve, E; Spiekerkoetter, U; Szamosi, J; Vara, R1
Introne, WJ1
Aldámiz-Echevarría, L; Andrade, F; Couce, ML; González-Lamuño, D; Sánchez-Pintos, P1
Hakeem, A; Jalan, A; Menon, J; Reddy, MS; Rela, M; Shanmugam, N; Valamparampil, JJ; Vij, M1
Morrow, G; Tanguay, RM1
Angileri, F; Tanguay, RM; Vogel, A1
Dubois, J; Halac, U; Mitchell, GA1
Dionisi-Vici, C; Maiorana, A1
Pennings, JP; van Ginkel, WG; van Spronsen, FJ1
Huijbregts, SCJ; Jahja, R; van Ginkel, WG; van Spronsen, FJ1
Endo, F; Ito, M; Nakamura, K; Shigematsu, Y1
Aktuglu-Zeybek, AC; Cansever, MS; Kiykim, E1
Lock, EA1
Alvarez, F; Atkinson, S; Bouchard, M; Brunel-Guitton, C; Buhas, D; Bussières, JF; Dubois, J; Fenyves, D; Goodyer, P; Gosselin, M; Halac, U; Labbé, P; Laframboise, R; Maranda, B; Melançon, S; Merouani, A; Mitchell, GA; Mitchell, J; Parizeault, G; Pelletier, L; Phan, V; Turcotte, JF1
Das, AM; Meyer, U; van Rijn, M; van Spronsen, FJ1
Mitchell, GA; Yang, H1
Chinsky, JM; Coakley, K; Ficicioglu, C; Grompe, M; Gucsavas-Calikoglu, M; Mitchell, G; Scott, CR; Singh, R; van Karnebeek, CDM; Waisbren, SE; Wasserstein, MP1
Clarke, A; Fraser, H; Freeman, K; Geppert, J; Johnson, S; Stinton, C; Sutcliffe, P; Taylor-Phillips, S1
Burgerhof, JGM; Daly, A; de Blaauw, P; Heiner-Fokkema, MR; Kienstra, NS; MacDonald, A; McKiernan, PJ; van Dam, E; van Ginkel, WG; van Reemst, HE; van Spronsen, FJ1
Christensen, M; Lund, AM; Sundberg, J; Wibrand, F1
Amoano, K; Hayashi, A; Hirayama, M; Inoue, M; Kurihara, K; Sakurai, H; Toyoda, H; Uchida, K1
Adam, D1
Barchanska, H; Mrachacz, M; Rola, R; Szczepankiewicz, W1
Aydogdu, S; Farajov, R; Iakobadze, Z; Karaca, CA; Kilic, M; Yilmaz, C1
Boels, D; Bretaudeau, M; Harry, P; Monteil-Ganière, C; Turcant, A1
Kassel, R; Rudnick, DA; Sprietsma, L1
Al Jurayyan, NA; Al-Jarallah, AS; Al-Nemri, A; Babiker, A; Hasanato, R; Kambal, MA; Mohamed, S1
Bendadi, F; de Koning, TJ; de Sain, MG; Prinsen, HC; Sinnema, G; van Hasselt, PM; van Spronsen, FJ; Verhoeven-Duif, N; Visser, G1
Bartlett, DC; Lloyd, C; McKiernan, PJ; Newsome, PN1
Janovszky, A; Karg, E; László, A; Rózsa, M; Sallay, E; Tiszlavicz, L; Túri, S; Ugarte, M; Várkonyi, A; Wittmann, G1
Chapchap, P; Feier, FH; Fonseca, EA; Leite, KM; Miura, IK; Porta, A; Porta, G; Pugliese, R; Seda Neto, J1
Aldamiz-Echevarria, LJ; Bliksrud, YT; Brunner-Krainz, M; Cocho de Juan, JA; Couce Pico, ML; Das, AM; de Baulny, HO; de Laet, C; Dionisi-Vici, C; Endig, J; Ernst, S; Freisinger, P; Gautschi, M; Gokcay, G; Hochuli, M; Jordan, J; Lotz-Havla, AS; Maiorana, A; Mandel, H; Mayorandan, S; McKiernan, P; Meyer, U; Moeslinger, D; Morlot, S; Sander, J; Santer, R; Scholl-Bürgi, S; Segarra, NG; Spiekerkoetter, U; Thimm, E; van Spronsen, F; Vogel, A; Zeman, J1
Boenzi, S; Di Ciommo, VM; Dionisi-Vici, C; Emma, F; Maiorana, A; Malamisura, M1
Altay, S; Aydin, A; Cansever, S; Erkan, T; Kiykim, E; Soyucen, E; Zeybek, AC; Zubarioglu, T1
Lock, E; Ranganath, LR; Timmis, O1
Chakrapani, A; McKiernan, PJ; Preece, MA1
Barshop, BA; Gangoiti, JA; Gertsman, I; Nyhan, WL1
Hadžić, N; Vara, R1
Iwai, M; Katashima, M; Minoura, H; Takahashi, H; Taniuchi, Y1
Shah, F; Shah, I1
Horoz, ÖÖ; İncecik, F; Kör, D; Öktem, M; Önenli Mungan, N; Sander, J; Yıldızdaş, D1
Barnby, E; Coker, SB; Hillgartner, MA; Koenig, AE; MacGregor, GG; Moore, ME1
Derks, G; Dunster, JL; Mistry, P; Salazar, JD; Ward, JP1
Amiot, B; Chen, H; DeGrado, TR; Elgilani, F; Freeman, BL; Glorioso, J; Grompe, M; Harding, CO; Hickey, RD; Ho, CS; Ibrahim, SH; Ikeda, Y; Jiang, H; Lillegard, JB; Mao, SA; Marler, R; Nyberg, SL; O'Connor, MK; Peng, KW; Rinaldo, P; Russell, SJ; Suksanpaisan, L1
Barzi, M; Bissig, KD; Bissig-Choisat, B; Borowiak, M; Elsea, SH; Hubert, L; Legras, X; Mi, T; Pankowicz, FP; Ravishankar, M; Sumazin, P; Sun, Q; Tomolonis, JA; Yang, D1
Gokay, S; Kardas, F; Kendirci, M; Ustkoyuncu, PS1
Peake, RW1
Mannion, MA; Mayne, P; Monavari, AA; Smith, A1
Hulton, SA; McKiernan, PJ; Preece, MA; Santra, S1
Finegold, M; Grompe, M; Lee, AY; Rothfuss, A; Sharma, AD; Vogel, A; Wang, Z; Willenbring, H1
Buitrago-Molina, LE; Cañón, JM; Finegold, M; Geffers, R; Grompe, M; Lamlé, J; Manns, MP; Marhenke, S; Sporn, M; Vogel, A; Yamamoto, M1
Gradowska, W; Gryff-Keller, A; Lamparska, D; Szczeciński, P1
Herebian, D; Lamshöft, M; Laryea, M; Mayatepek, E; Spiekerkötter, U; Thimm, E1
Gryff-Keller, A; Molchanov, S1
Al-Dirbashi, O; El-Hawary, M; El-Karaksy, H; El-Koofy, N; El-Raziky, M; El-Sayed, R; Rashed, M1
Aktuğlu-Zeybek, AC; Cansever, MS; Erim, FB1
Herebian, D; Lamshöft, M; Mayatepek, E; Spiekerkoetter, U1
Dahlke, MH; Doenecke, A; Eggenhofer, E; Geissler, EK; Piso, P; Popp, FC; Renner, P; Schlitt, HJ; Slowik, P1
Callea, F; Castellano, A; Dionisi-Vici, C; Francalanci, P; Holme, E; Jenkner, A; Nobili, V1
Das, AM; Demirkol, M; Gökcay, G; Janzen, N; Ozer, I; Peter, M; Sander, J; Sander, S; Terhardt, M1
Assmann, B; Herebian, D; Klee, D; Mayatepek, E; Spiekerkoetter, U; Thimm, E1
Celli, M; D'Eufemia, P; Finocchiaro, R; Tetti, M1
Aldámiz-Echevarría, L; Andrade, F; Lage, S; Prieto, JA1
El-Hennawy, A; El-Karaksy, H; El-Kiki, H; El-Koofy, N; El-Raziky, M; El-Sayed, R; Fahmy, M; Mohsen, N; Rashed, MS1
Carton, D; Dan, B; De Laet, C; François, B; Goyens, PJ; Jaeken, J; Munoz, VT; Sokal, EM1
Aldámiz-Echevarría, L; Couce, ML; Dalmau, J; del Toro, M; Pintos-Morell, G1
Bleidissel, M; Cantz, T; Gentile, L; Greber, B; Han, DW; Liu, N; Ott, M; Rittelmeyer, I; Rudolph, C; Schambach, A; Schöler, HR; Sgodda, M; Sharma, AD; Steinemann, D; Wu, G; Zaehres, H1
Herebian, D; Kamp, G; Klee, D; Mayatepek, E; Molke, B; Richter-Werkle, R; Spiekerkoetter, U; Thimm, E1
Laidman, J1
Boenzi, S; Della Bona, ML; Dionisi-Vici, C; la Marca, G; Malvagia, S; Martinelli, D; Materazzi, S1
Herebian, D; Schlune, A; Spiekerkoetter, U; Thimm, E1
Bartlett, DC; Holme, E; Lloyd, C; McKiernan, PJ; Newsome, PN; Preece, MA1
Claerhout, I; Jaeken, J; Kestelyn, P; Schauwvlieghe, PP1
Davit-Spraul, A; Poggi-Bach, J; Romdhane, H1
Arias, C; Cabello, JF; Castro, G; Cornejo, V; de la Parra, A; Fernández, E; Raimann, E1
Alvarez, F; Bussières, JF; Chevalier, I; Dallaire, L; Dubois, J; Faucher, F; Fenyves, D; Goodyer, P; Grenier, A; Holme, E; Laframboise, R; Lambert, M; Larochelle, J; Lindstedt, S; Maranda, B; Melançon, S; Merouani, A; Mitchell, GA; Mitchell, J; Parizeault, G; Pelletier, L; Phan, V; Rinaldo, P; Scott, CR; Scriver, C1
van der Lelij, A; Visser, G; Wisse, RP; Wittebol-Post, D1
Benoist, JF; Davit-Spraul, A; Garcia Segarra, N; Grenèche, MO; Imbard, A; Ogier de Baulny, H; Roche, S1
de Baulny, HO; de Laet, C; Dionisi-Vici, C; Leonard, JV; McKiernan, P; Mitchell, G; Monti, L; Pintos-Morell, G; Spiekerkötter, U1
Ahmad, S; Lueder, GT; Teckman, JH1
Hanauske-Abel, HM; Levy, J; Newfield, RS; Popowicz, A; Remotti, H1
Bodamer, OA; Crone, J; Holme, E; Huber, WD; Möslinger, D; Schima, W; Stöckler Ipsiroglu, S1
Gissen, P; McKiernan, PJ; Preece, MA; Willshaw, HA1
Dalton, TP; Dieter, MZ; Freshwater, SL; Miller, ML; Nebert, DW; Shertzer, HG1
Berger, R; Jacobs, SM; Klomp, LW; Koornneef, LP; Luijerink, MC; van Beurden, EA; van den Berg, IE1
Yang, DY1
Al-Dhalimy, M; Finegold, M; Grompe, M; Groopman, J; Iordanov, MS; Ou, CN; Ryabinina, O; van Den Berg, IE; Vogel, A1
Berger, R; Grompe, M; Jacobs, SM; Klomp, LW; Luijerink, MC; Malingré, HE; van Beurden, EA; van den Berg, IE1
Joshi, SN; Venugopalan, P1
Bijleveld, CM; van Maldegem, BT; van Spronsen, FJ; Wijburg, FA1
André, N; Jubin, V; Ovaert, C; Roquelaure, B1
Al Amoudi, M; Al-Ahaidib, LY; Al-Dbaas, A; Al-Dirbashi, OY; Al-Hassnan, Z; Al-Owain, M; Al-Sayed, MM; Ni Luanaigh, M; Rahbeeni, Z; Rashed, MS1
Bergeron, A; Jorquera, R; Orejuela, D; Tanguay, RM1
Berger, R; Jacobs, SM; Klomp, LW; van Beurden, DH; van den Berg, IE1
Ellis, M; Gaskin, P; Lock, EA; Provan, WM; Smith, LL1
Finegold, M; Jorquera, R; Langlois, C; Shroads, AL; Stacpoole, PW; Tanguay, RM1
Arora, N; Kelly, DA; McKiernan, PJ; Stumper, O; Wright, J1
McKiernan, PJ1
Bijleveld, CM; Koelink, CJ; van den Heuvel-Eibrink, MM; van der Ploeg, A; van Hasselt, P; van Spronsen, FJ; Wijburg, FA1
Cohen, AF; van Bronswijk, H1
Bergeron, A; Finegold, MJ; Jorquera, R; Langlois, C; Orejuela, D; Rhead, WJ; Tanguay, RM1
Bergeron, A; Finegold, MJ; Jorquera, R; Orejuela, D; Tanguay, RM1
Bernard, O; de Baulny, HO; Dobbelaere, D; Guffon, N; Masurel-Paulet, A; Poggi-Bach, J; Rolland, MO; Sarles, J; Touati, G1
Baumann, U; Santra, S1
Ketteler, K; Mayatepek, E; Perot, C; Schiff, M; Schlump, JU; Spiekerkoetter, U; Wendel, U1
Sass, JO; Skladal, D1
Blum, D; Clercx, A; De Laet, C; Goyens, P; Vamos, E; Vanden Eijnden, S1
Heikinheimo, M; Pitkänen, ST; Salo, MK1
Holme, E; Lindstedt, S1
González Pascual, E; Holme, E; Lambruschini Ferri, N; Mas Comas, A; Ros Viladoms, J; Vilaseca Buscà, MA1
Eksborg, S; Hall, MG; Lumholtz, B; Provan, WM; Wilks, MF1
Al-Dhalimy, M; Finegold, M; Grompe, M; Overturf, K1
Schwetz, BA1

Reviews

20 review(s) available for tiletamine hydrochloride and Tyrosinemias

ArticleYear
Long-Term Outcomes and Practical Considerations in the Pharmacological Management of Tyrosinemia Type 1.
    Paediatric drugs, 2019, Volume: 21, Issue:6

    Topics: Cyclohexanones; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias

2019
Biochemical and Clinical Aspects of Hereditary Tyrosinemia Type 1.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Humans; Kidney; Liver; Liver Failure; Nitrobenzoates; Tyrosine; Tyrosinemias

2017
Molecular Pathogenesis of Liver Injury in Hereditary Tyrosinemia 1.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Animals; Cyclohexanones; Humans; Hydrolases; Liver; Liver Diseases; Nitrobenzoates; Signal Transduction; Tyrosinemias

2017
The Liver in Tyrosinemia Type I: Clinical Management and Course in Quebec.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Humans; Hydrolases; Liver; Liver Diseases; Nitrobenzoates; Quebec; Tyrosinemias

2017
NTBC and Correction of Renal Dysfunction.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Animals; Cyclohexanones; Humans; Kidney; Kidney Diseases; Liver; Liver Diseases; Nitrobenzoates; Tyrosinemias

2017
Liver Cancer in Tyrosinemia Type 1.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Biomarkers, Tumor; Carcinoma, Hepatocellular; Cyclohexanones; Early Detection of Cancer; Humans; Liver Neoplasms; Nitrobenzoates; Tyrosinemias

2017
Neurological and Neuropsychological Problems in Tyrosinemia Type I Patients.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Humans; Nervous System Diseases; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Hereditary Tyrosinemia Type 1 in Turkey.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Humans; Hydrolases; Liver; Liver Diseases; Nitrobenzoates; Turkey; Tyrosine; Tyrosinemias

2017
From Weed Killer to Wonder Drug.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Animals; Biological Products; Cyclohexanones; Enzyme Inhibitors; Humans; Liver; Nitrobenzoates; Tyrosine; Tyrosinemias

2017
The Québec NTBC Study.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Enzyme Inhibitors; Heptanoates; Humans; Infant, Newborn; Liver Diseases; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Quebec; Tyrosinemias

2017
Dietary Considerations in Tyrosinemia Type I.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Diet; Humans; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Remaining Challenges in the Treatment of Tyrosinemia from the Clinician's Viewpoint.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Animals; Cyclohexanones; Diet; Diet Therapy; Humans; Infant, Newborn; Kidney Diseases; Liver Failure; Liver Neoplasms; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Tyrosinemias

2017
Diagnosis and treatment of tyrosinemia type I: a US and Canadian consensus group review and recommendations.
    Genetics in medicine : official journal of the American College of Medical Genetics, 2017, Volume: 19, Issue:12

    Topics: Canada; Cyclohexanones; Diet Therapy; Disease Management; Female; Genetic Counseling; Genotype; Humans; Infant, Newborn; Liver Transplantation; Medication Adherence; Neonatal Screening; Nitrobenzoates; Phenotype; Pregnancy; Pregnancy Complications; Tyrosinemias; United States

2017
Evaluation of pre-symptomatic nitisinone treatment on long-term outcomes in Tyrosinemia type 1 patients: a systematic review.
    Orphanet journal of rare diseases, 2017, 09-11, Volume: 12, Issue:1

    Topics: Cyclohexanones; Humans; Infant, Newborn; Neonatal Screening; Nitrobenzoates; Tyrosinemias

2017
The role of nitisinone in tyrosine pathway disorders.
    Current rheumatology reports, 2014, Volume: 16, Issue:11

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Biomedical Research; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Off-Label Use; Tyrosinemias

2014
Recommendations for the management of tyrosinaemia type 1.
    Orphanet journal of rare diseases, 2013, Jan-11, Volume: 8

    Topics: Bone Density; Cyclohexanones; Female; Humans; Infant, Newborn; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Pregnancy; Psychometrics; Tyrosinemias

2013
4-Hydroxyphenylpyruvate dioxygenase as a drug discovery target.
    Drug news & perspectives, 2003, Volume: 16, Issue:8

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Cyclohexanones; Drug Design; Enzyme Inhibitors; Forecasting; Isoxazoles; Molecular Structure; Nitriles; Nitrobenzoates; Oxygenases; Sulfones; Tyrosinemias

2003
Experience of nitisinone for the pharmacological treatment of hereditary tyrosinaemia type 1.
    Expert opinion on pharmacotherapy, 2008, Volume: 9, Issue:7

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Carcinoma, Hepatocellular; Child; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Diseases; Liver Neoplasms; Monitoring, Physiologic; Nitrobenzoates; Tyrosinemias

2008
Hereditary tyrosinaemia type I: from basics to progress in treatment.
    Annals of medicine, 2000, Volume: 32, Issue:8

    Topics: Animals; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Transplantation; Mice; Mutation; Nitrobenzoates; Pedigree; Tyrosine Transaminase; Tyrosinemias

2000
Nontransplant treatment of tyrosinemia.
    Clinics in liver disease, 2000, Volume: 4, Issue:4

    Topics: Age Factors; Animals; Carcinoma, Hepatocellular; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Liver Neoplasms; Nitrobenzoates; Risk Factors; Tyrosinemias

2000

Trials

4 trial(s) available for tiletamine hydrochloride and Tyrosinemias

ArticleYear
Long-term safety and outcomes in hereditary tyrosinaemia type 1 with nitisinone treatment: a 15-year non-interventional, multicentre study.
    The lancet. Diabetes & endocrinology, 2021, Volume: 9, Issue:7

    Topics: Adolescent; Chemical and Drug Induced Liver Injury; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Kidney Diseases; Longitudinal Studies; Male; Neonatal Screening; Nitrobenzoates; Prospective Studies; Time Factors; Treatment Outcome; Tyrosinemias

2021
Neuropsychological outcome of NTBC-treated patients with tyrosinaemia type 1.
    Developmental medicine and child neurology, 2011, Volume: 53, Issue:10

    Topics: Adolescent; Belgium; Child; Child, Preschool; Cognition Disorders; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Intelligence; Intelligence Tests; Male; Neuropsychological Tests; Nitrobenzoates; Phenylalanine; Treatment Outcome; Tyrosine; Tyrosinemias

2011
Ophthalmic follow-up of patients with tyrosinaemia type I on NTBC.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:1

    Topics: Child; Child, Preschool; Corneal Opacity; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Nitrobenzoates; Tyrosine; Tyrosinemias

2003
Pharmacokinetics and pharmacodynamics of NTBC (2-(2-nitro-4-fluoromethylbenzoyl)-1,3-cyclohexanedione) and mesotrione, inhibitors of 4-hydroxyphenyl pyruvate dioxygenase (HPPD) following a single dose to healthy male volunteers.
    British journal of clinical pharmacology, 2001, Volume: 52, Issue:2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Administration, Oral; Area Under Curve; Chemistry, Pharmaceutical; Cyclohexanones; Enzyme Inhibitors; Half-Life; Humans; Male; Nitrobenzoates; Therapeutic Equivalency; Tyrosine; Tyrosinemias

2001

Other Studies

109 other study(ies) available for tiletamine hydrochloride and Tyrosinemias

ArticleYear
Nitisinone treatment during two pregnancies and breastfeeding in a woman with tyrosinemia type 1 - a case report.
    Journal of pediatric endocrinology & metabolism : JPEM, 2022, Feb-23, Volume: 35, Issue:2

    Topics: Breast Feeding; Cyclohexanones; Female; Humans; Infant, Newborn; Nitrobenzoates; Pregnancy; Pregnancy Complications; Tyrosinemias; Young Adult

2022
Evaluation of dynamic thiol/disulfide homeostasis in hereditary tyrosinemia type 1 patients.
    Pediatric research, 2022, Volume: 92, Issue:2

    Topics: Carcinoma, Hepatocellular; Cyclohexanones; Disulfides; Homeostasis; Humans; Liver Neoplasms; Nitrobenzoates; Oxidative Stress; Sulfhydryl Compounds; Tyrosine; Tyrosinemias

2022
Hepatocellular carcinoma requiring liver transplantation in hereditary tyrosinemia type 1 despite nitisinone therapy and α1-fetoprotein normalization.
    Pediatric transplantation, 2022, Volume: 26, Issue:7

    Topics: alpha-Fetoproteins; Carcinoma, Hepatocellular; Child; Cyclohexanones; Female; Humans; Liver Cirrhosis; Liver Neoplasms; Liver Transplantation; Nitrobenzoates; Tyrosinemias

2022
Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria.
    Scientific reports, 2022, 09-27, Volume: 12, Issue:1

    Topics: Alkaptonuria; Brain Diseases, Metabolic, Inborn; Cyclohexanones; Homogentisic Acid; Humans; Nitrobenzoates; Phenylalanine; Phenylpropionates; Tyrosine; Tyrosinemias

2022
Biomarkers of Micronutrients in Regular Follow-Up for Tyrosinemia Type 1 and Phenylketonuria Patients.
    Nutrients, 2019, Aug-27, Volume: 11, Issue:9

    Topics: Adolescent; Adult; Aged; Amino Acids; Biomarkers; Biopterins; Child; Child, Preschool; Cyclohexanones; Diet, Protein-Restricted; Dietary Supplements; Enzyme Inhibitors; Female; Humans; Infant; Male; Micronutrients; Middle Aged; Nitrobenzoates; Nutritional Status; Phenylketonurias; Retrospective Studies; Risk Assessment; Risk Factors; Tyrosinemias; Vitamin B 12 Deficiency; Vitamin D Deficiency; Young Adult

2019
Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:2

    Topics: Alkaptonuria; Animals; Cyclohexanones; Diet, Protein-Restricted; Female; Humans; Male; Mice; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2020
Evolution of tyrosinemia type 1 disease in patients treated with nitisinone in Spain.
    Medicine, 2019, Volume: 98, Issue:39

    Topics: Adult; Child; Cognitive Dysfunction; Cyclohexanones; Delayed Diagnosis; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant, Newborn; Kidney Diseases; Male; Needs Assessment; Neonatal Screening; Nitrobenzoates; Obesity; Prognosis; Quality of Life; Retrospective Studies; Spain; Time-to-Treatment; Tyrosinemias

2019
Tissue-specific FAH deficiency alters sleep-wake patterns and results in chronic tyrosinemia in mice.
    Proceedings of the National Academy of Sciences of the United States of America, 2019, 10-29, Volume: 116, Issue:44

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Cells, Cultured; Circadian Rhythm; Cyclohexanones; Enzyme Inhibitors; Enzyme Stability; HEK293 Cells; Homozygote; Humans; Hydrolases; Kidney; Liver; Male; Mice; Mice, Inbred C57BL; Mutation; Nitrobenzoates; Organ Specificity; Sleep; Suprachiasmatic Nucleus; Tyrosinemias

2019
The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients.
    Nutrients, 2019, Nov-18, Volume: 11, Issue:11

    Topics: Adolescent; Adult; Child; Cyclohexanones; Dietary Supplements; Enzyme Inhibitors; Female; Heptanoates; Humans; Linear Models; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias; Young Adult

2019
Emotional and behavioral problems, quality of life and metabolic control in NTBC-treated Tyrosinemia type 1 patients.
    Orphanet journal of rare diseases, 2019, 12-04, Volume: 14, Issue:1

    Topics: Adolescent; Adult; Child; Cyclohexanones; Humans; Male; Nitrobenzoates; Phenylalanine; Quality of Life; Tyrosine; Tyrosinemias; Young Adult

2019
[Screening for hereditary tyrosinemia and genotype analysis in newborns].
    Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences, 2019, 06-25, Volume: 48, Issue:4

    Topics: Child; Cyclohexanones; Genotype; Humans; Infant; Infant, Newborn; Neonatal Screening; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosinemias

2019
Late Development of Hepatocellular Carcinoma in Tyrosinemia Type 1 Despite Nitisinone (NTBC) Treatment.
    Journal of pediatric gastroenterology and nutrition, 2020, Volume: 71, Issue:2

    Topics: Carcinoma, Hepatocellular; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Neoplasms; Nitrobenzoates; Tyrosinemias

2020
First Scandinavian case of successful pregnancy during nitisinone treatment for type 1 tyrosinemia.
    Journal of pediatric endocrinology & metabolism : JPEM, 2020, May-26, Volume: 33, Issue:5

    Topics: Adolescent; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Tyrosine; Tyrosinemias

2020
Laboratory monitoring of patients with hereditary tyrosinemia type I.
    Molecular genetics and metabolism, 2020, Volume: 130, Issue:4

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Amino Acids; Biomarkers; Case-Control Studies; Child; Child, Preschool; Cyclohexanones; Female; Follow-Up Studies; Heptanoates; Humans; Infant; Infant, Newborn; Laboratories; Male; Middle Aged; Nitrobenzoates; Prognosis; Reference Standards; Specimen Handling; Tyrosinemias; Young Adult

2020
Type 1 tyrosinemia in Finland: a nationwide study.
    Orphanet journal of rare diseases, 2020, 10-12, Volume: 15, Issue:1

    Topics: Child; Child, Preschool; Cyclohexanones; Female; Finland; Humans; Infant; Infant, Newborn; Liver; Male; Neonatal Screening; Nitrobenzoates; Prognosis; Tyrosinemias

2020
Oxidative Stress, Glutathione Metabolism, and Liver Regeneration Pathways Are Activated in Hereditary Tyrosinemia Type 1 Mice upon Short-Term Nitisinone Discontinuation.
    Genes, 2020, 12-22, Volume: 12, Issue:1

    Topics: Animals; Cyclohexanones; Disease Models, Animal; Glutathione; Humans; Hydrolases; Liver; Liver Regeneration; Medication Adherence; Mice; Mice, Knockout; NF-E2-Related Factor 2; Nitrobenzoates; Oxidative Stress; Oxidoreductases; Signal Transduction; Tyrosine; Tyrosinemias; Withholding Treatment

2020
Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome.
    Nutrients, 2020, Dec-31, Volume: 13, Issue:1

    Topics: Adolescent; Austria; Child; Child, Preschool; Combined Modality Therapy; Cyclohexanones; Diet, Protein-Restricted; Dietary Proteins; Enzyme Inhibitors; Female; Germany; Humans; Male; Nitrobenzoates; Patient Compliance; Phenylalanine; Practice Guidelines as Topic; Prospective Studies; Retrospective Studies; Surveys and Questionnaires; Switzerland; Treatment Outcome; Tyrosine; Tyrosinemias; Young Adult

2020
Nitisinone: two decades treating hereditary tyrosinaemia type 1.
    The lancet. Diabetes & endocrinology, 2021, Volume: 9, Issue:7

    Topics: Cyclohexanones; Humans; Nitrobenzoates; Tyrosinemias

2021
Treatment adherence in tyrosinemia type 1 patients.
    Orphanet journal of rare diseases, 2021, 06-03, Volume: 16, Issue:1

    Topics: Adolescent; Adult; Child; Child, Preschool; Cyclohexanones; Humans; Infant; Nitrobenzoates; Phenylalanine; Prognosis; Treatment Adherence and Compliance; Tyrosine; Tyrosinemias; Young Adult

2021
Liver Transplantation: A Safe and Definitive Alternative to Lifelong Nitisinone for Tyrosinemia Type 1.
    Indian journal of pediatrics, 2022, Volume: 89, Issue:5

    Topics: Carcinoma, Hepatocellular; Child; Cyclohexanones; Humans; Liver Neoplasms; Liver Transplantation; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias

2022
Tyrosinemia Type I in Japan: A Report of Five Cases.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Child, Preschool; Cyclohexanones; Female; Humans; Infant; Japan; Liver; Liver Transplantation; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Daily variation of NTBC and its relation to succinylacetone in tyrosinemia type 1 patients comparing a single dose to two doses a day.
    Journal of inherited metabolic disease, 2018, Volume: 41, Issue:2

    Topics: Adolescent; Child; Child, Preschool; Chromatography, High Pressure Liquid; Cyclohexanones; Diet, Protein-Restricted; Dried Blood Spot Testing; Drug Administration Schedule; Drug Monitoring; Female; Humans; Infant; Male; Nitrobenzoates; Prospective Studies; Tandem Mass Spectrometry; Time Factors; Treatment Outcome; Tyrosinemias; Young Adult

2018
Simultaneous quantification of succinylacetone and nitisinone for therapeutic drug monitoring in the treatment of Tyrosinemia type 1.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2018, Jan-01, Volume: 1072

    Topics: Chromatography, Liquid; Cyclohexanones; Drug Monitoring; Heptanoates; Humans; Linear Models; Nitrobenzoates; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Tyrosinemias

2018
Discontinuation of NTBC after liver transplantation in tyrosinemia type 1.
    Pediatrics international : official journal of the Japan Pediatric Society, 2018, Volume: 60, Issue:11

    Topics: Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Liver Transplantation; Magnetic Resonance Imaging; Male; Nitrobenzoates; Tomography, X-Ray Computed; Tyrosinemias; Withholding Treatment

2018
A father's fight to help his sons - and fix clinical trials.
    Nature, 2019, Volume: 565, Issue:7738

    Topics: Adolescent; Alkaptonuria; Animals; Autopsy; Child; Clinical Trials as Topic; Compassionate Use Trials; Cyclohexanones; Disease Models, Animal; Disease Progression; Drug Approval; Endpoint Determination; Female; Homogentisic Acid; Humans; Infant; Male; Mice; National Health Programs; National Institutes of Health (U.S.); Nitrobenzoates; Observational Studies as Topic; Off-Label Use; Orphan Drug Production; Rare Diseases; Rats; Sample Size; Tyrosine; Tyrosinemias; United States; United States Food and Drug Administration

2019
LC-MS/MS study of the degradation processes of nitisinone and its by-products.
    Journal of pharmaceutical and biomedical analysis, 2019, Jul-15, Volume: 171

    Topics: Chromatography, High Pressure Liquid; Cyclohexanones; Drug Stability; Gastric Juice; Humans; Hydrogen-Ion Concentration; Limit of Detection; Models, Biological; Molecular Structure; Nitrobenzoates; Saliva; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Tyrosinemias

2019
Live donor liver transplantation for type 1 tyrosinemia: An analysis of 15 patients.
    Pediatric transplantation, 2019, Volume: 23, Issue:6

    Topics: Adolescent; Adult; Carcinoma, Hepatocellular; Child; Child, Preschool; Cyclohexanones; Databases, Factual; Female; Humans; Infant; Infant, Newborn; Liver Neoplasms; Liver Transplantation; Living Donors; Male; Neonatal Screening; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias

2019
Triketone toxicity: a report on two cases of sulcotrione poisoning.
    Human & experimental toxicology, 2013, Volume: 32, Issue:7

    Topics: 2-Methyl-4-chlorophenoxyacetic Acid; 2,4-Dichlorophenoxyacetic Acid; Adult; Cyclohexanones; Herbicides; Humans; Male; Mesylates; Tyrosinemias; Vomiting

2013
Pregnancy in an NTBC-treated patient with hereditary tyrosinemia type I.
    Journal of pediatric gastroenterology and nutrition, 2015, Volume: 60, Issue:1

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Combined Modality Therapy; Contraindications; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Phenylalanine; Pregnancy; Pregnancy Complications; Term Birth; Tyrosine; Tyrosinemias

2015
Tyrosinemia type 1: a rare and forgotten cause of reversible hypertrophic cardiomyopathy in infancy.
    BMC research notes, 2013, Sep-09, Volume: 6

    Topics: Cardiomyopathy, Hypertrophic; Cyclohexanones; Food, Formulated; Heptanoates; Homozygote; Humans; Hydrolases; Infant; Male; Mutation; Nitrobenzoates; Siblings; Treatment Outcome; Tyrosinemias

2013
Impaired cognitive functioning in patients with tyrosinemia type I receiving nitisinone.
    The Journal of pediatrics, 2014, Volume: 164, Issue:2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Child; Child Development; Child, Preschool; Cognition; Cognitive Dysfunction; Cross-Sectional Studies; Cyclohexanones; Disease Progression; Enzyme Inhibitors; Female; Humans; Incidence; Male; Netherlands; Nitrobenzoates; Prognosis; Risk Factors; Tyrosinemias; Young Adult

2014
Early nitisinone treatment reduces the need for liver transplantation in children with tyrosinaemia type 1 and improves post-transplant renal function.
    Journal of inherited metabolic disease, 2014, Volume: 37, Issue:5

    Topics: Adolescent; Adult; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Female; Glomerular Filtration Rate; Humans; Hypertension; Kidney; Liver Transplantation; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias; Young Adult

2014
The fate of tyrosinaemic Hungarian patients before the NTBC aera.
    Ideggyogyaszati szemle, 2013, Nov-30, Volume: 66, Issue:11-12

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Carcinoma, Hepatocellular; Carcinoma, Renal Cell; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Fatal Outcome; Homozygote; Humans; Hungary; Hydrolases; Kidney Neoplasms; Liver Neoplasms; Male; Nitrobenzoates; Treatment Outcome; Tyrosine; Tyrosinemias

2013
HCC prevalence and histopathological findings in liver explants of patients with hereditary tyrosinemia type 1.
    Pediatric blood & cancer, 2014, Volume: 61, Issue:9

    Topics: Carcinoma, Hepatocellular; Child; Child, Preschool; Cyclohexanones; Drug Resistance; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Liver; Liver Neoplasms; Liver Transplantation; Male; Nitrobenzoates; Prognosis; Retrospective Studies; Tomography, X-Ray Computed; Tyrosinemias

2014
Cross-sectional study of 168 patients with hepatorenal tyrosinaemia and implications for clinical practice.
    Orphanet journal of rare diseases, 2014, Aug-01, Volume: 9

    Topics: Adolescent; Child; Child, Preschool; Cross-Sectional Studies; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Liver Failure; Liver Transplantation; Male; Neonatal Screening; Nitrobenzoates; Rare Diseases; Renal Insufficiency; Retrospective Studies; Surveys and Questionnaires; Treatment Outcome; Tyrosinemias; Young Adult

2014
Early effect of NTBC on renal tubular dysfunction in hereditary tyrosinemia type 1.
    Molecular genetics and metabolism, 2014, Volume: 113, Issue:3

    Topics: Biomarkers; Child, Preschool; Cyclohexanones; Female; Humans; Infant; Kidney Tubules; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias

2014
Hereditary tyrosinemia type 1 in Turkey: twenty year single-center experience.
    Pediatrics international : official journal of the Japan Pediatric Society, 2015, Volume: 57, Issue:2

    Topics: Acute Disease; Child; Child, Preschool; Chronic Disease; Cyclohexanones; Diet Therapy; Early Diagnosis; Enzyme Inhibitors; Female; Hepatomegaly; Humans; Infant; Infant, Newborn; Kidney Diseases; Liver Transplantation; Living Donors; Male; Nitrobenzoates; Prognosis; Retrospective Studies; Splenomegaly; Turkey; Tyrosinemias

2015
Outcome of children with hereditary tyrosinaemia following newborn screening.
    Archives of disease in childhood, 2015, Volume: 100, Issue:8

    Topics: alpha-Fetoproteins; Cyclohexanones; Enzyme Inhibitors; Female; Genotype; Humans; Hydrolases; Infant, Newborn; Male; Mutation; Neonatal Screening; Nitrobenzoates; Prothrombin Time; Treatment Outcome; Tyrosinemias

2015
Perturbations of tyrosine metabolism promote the indolepyruvate pathway via tryptophan in host and microbiome.
    Molecular genetics and metabolism, 2015, Volume: 114, Issue:3

    Topics: Aldehydes; Alkaptonuria; Cell Line, Tumor; Cyclohexanones; Gastrointestinal Microbiome; Humans; Indoles; Mass Spectrometry; Metabolomics; Nitrobenzoates; Phenylpyruvic Acids; Symbiosis; Tryptophan; Tyrosine; Tyrosinemias

2015
Neonatal screening for hereditary tyrosinaemia: are we there yet?
    Archives of disease in childhood, 2015, Volume: 100, Issue:8

    Topics: Cyclohexanones; Enzyme Inhibitors; Female; Humans; Male; Neonatal Screening; Nitrobenzoates; Tyrosinemias

2015
[Pharmacological and clinical profile of nitisinone (Orfadin(®) Capsules): a therapeutic agent for hereditary tyrosinemia type 1].
    Nihon yakurigaku zasshi. Folia pharmacologica Japonica, 2015, Volume: 146, Issue:6

    Topics: Animals; Capsules; Clinical Trials as Topic; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Signal Transduction; Tyrosinemias

2015
Tyrosinemia type I: Case series with response to treatment to NTBC.
    Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology, 2016, Volume: 35, Issue:3

    Topics: Child, Preschool; Cyclohexanones; Female; Humans; Infant; Male; Nitrobenzoates; Treatment Outcome; Tyrosinemias

2016
Tyrosinemia type 1 and irreversible neurologic crisis after one month discontinuation of nitisone.
    Metabolic brain disease, 2016, Volume: 31, Issue:5

    Topics: Cyclohexanones; Fatal Outcome; Humans; Infant; Male; Nervous System Diseases; Nitrobenzoates; Time Factors; Tyrosine; Tyrosinemias

2016
Tyrosinemia type I and not treatment with NTBC causes slower learning and altered behavior in mice.
    Journal of inherited metabolic disease, 2016, Volume: 39, Issue:5

    Topics: Animals; Behavior, Animal; Cyclohexanones; Disease Models, Animal; Hydrolases; Learning; Memory, Long-Term; Mice; Nitrobenzoates; Tyrosine; Tyrosinemias

2016
Predicting tyrosinaemia: a mathematical model of 4-hydroxyphenylpyruvate dioxygenase inhibition by nitisinone in rats.
    Mathematical medicine and biology : a journal of the IMA, 2017, 09-01, Volume: 34, Issue:3

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Computer Simulation; Cyclohexanones; Enzyme Inhibitors; Kinetics; Liver; Mathematical Concepts; Models, Animal; Models, Biological; Nitrobenzoates; Rats; Tyrosine; Tyrosinemias

2017
Curative ex vivo liver-directed gene therapy in a pig model of hereditary tyrosinemia type 1.
    Science translational medicine, 2016, 07-27, Volume: 8, Issue:349

    Topics: Animals; Cyclohexanones; Disease Models, Animal; Genetic Therapy; Hepatocytes; Hydrolases; Immunohistochemistry; Liver; Nitrobenzoates; Swine; Transplantation, Homologous; Tyrosinemias

2016
Reprogramming metabolic pathways in vivo with CRISPR/Cas9 genome editing to treat hereditary tyrosinaemia.
    Nature communications, 2016, 08-30, Volume: 7

    Topics: Animals; Cell Line; Cell Proliferation; CRISPR-Cas Systems; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Exons; Gene Editing; Genetic Therapy; Hepatocytes; Humans; Hydrolases; Liver; Metabolic Networks and Pathways; Mice; Mice, Inbred Strains; Mice, Knockout; Nitrobenzoates; Oxidoreductases; Phenotype; Tyrosinemias

2016
The outcome of seven patients with hereditary tyrosinemia type 1.
    Journal of pediatric endocrinology & metabolism : JPEM, 2016, Oct-01, Volume: 29, Issue:10

    Topics: Biomarkers; Child; Child, Preschool; Cyclohexanones; Echocardiography; Enzyme Inhibitors; Female; Gas Chromatography-Mass Spectrometry; Humans; Infant; Magnetic Resonance Imaging; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias; Ultrasonography

2016
In brief: Nitisinone (Orfadin) for hereditary tyrosinemia.
    The Medical letter on drugs and therapeutics, 2016, Oct-10, Volume: 58, Issue:1505

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Administration, Oral; Capsules; Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Humans; Nitrobenzoates; Pharmaceutical Solutions; Treatment Outcome; Tyrosinemias

2016
Deranged Liver Function in a Neonate.
    Clinical chemistry, 2016, Volume: 62, Issue:11

    Topics: Biomarkers; Cyclohexanones; Enzyme Inhibitors; Female; Gas Chromatography-Mass Spectrometry; Heptanoates; Humans; Infant, Newborn; Liver Diseases; Neonatal Screening; Nitrobenzoates; Tyrosinemias; Urinalysis

2016
Type 1 Tyrosinaemia.
    Irish medical journal, 2016, Jun-10, Volume: 109, Issue:6

    Topics: Biomarkers; Cyclohexanones; Enzyme Inhibitors; Heptanoates; Humans; Hydrolases; Hypoglycemia; Ireland; Liver Transplantation; Mutation; Nitrobenzoates; Tyrosinemias

2016
Renal tubular function in children with tyrosinaemia type I treated with nitisinone.
    Journal of inherited metabolic disease, 2008, Volume: 31, Issue:3

    Topics: Child; Child, Preschool; Cyclohexanones; Female; Humans; Infant; Infant, Newborn; Kidney; Kidney Tubules; Male; Nitrobenzoates; Proteinuria; Retrospective Studies; Tyrosinemias; Ultrasonography

2008
Loss of p21 permits carcinogenesis from chronically damaged liver and kidney epithelial cells despite unchecked apoptosis.
    Cancer cell, 2008, Jul-08, Volume: 14, Issue:1

    Topics: Animals; Apoptosis; Carcinoma, Hepatocellular; Cell Cycle; Cell Proliferation; Cyclin-Dependent Kinase Inhibitor p21; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Hepatectomy; Hepatocytes; Hydrolases; Kidney Diseases, Cystic; Kidney Neoplasms; Kidney Tubules, Proximal; Liver Neoplasms; Liver Regeneration; Mice; Mice, Knockout; Neoplasms; Nitrobenzoates; Tyrosinemias

2008
Activation of nuclear factor E2-related factor 2 in hereditary tyrosinemia type 1 and its role in survival and tumor development.
    Hepatology (Baltimore, Md.), 2008, Volume: 48, Issue:2

    Topics: Animals; Cyclohexanones; DNA Damage; Dose-Response Relationship, Drug; Glutathione; Hepatitis; Hydrolases; Imidazoles; Inactivation, Metabolic; Liver; Liver Cirrhosis; Liver Failure; Liver Neoplasms; Mice; Mice, Knockout; NF-E2-Related Factor 2; Nitrobenzoates; Oleanolic Acid; Oxidative Stress; Tyrosinemias

2008
Identification of 2-[2-nitro-4-(trifluoromethyl)benzoyl]- cyclohexane-1,3-dione metabolites in urine of patients suffering from tyrosinemia type I with the use of 1H and 19F NMR spectroscopy.
    Acta biochimica Polonica, 2008, Volume: 55, Issue:4

    Topics: Child; Child, Preschool; Cyclohexanones; Female; Fluorine; Humans; Magnetic Resonance Spectroscopy; Male; Nitrobenzoates; Protons; Tyrosinemias

2008
Liquid chromatography tandem mass spectrometry method for the quantitation of NTBC (2-(nitro-4-trifluoromethylbenzoyl)1,3-cyclohexanedione) in plasma of tyrosinemia type 1 patients.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2009, May-15, Volume: 877, Issue:14-15

    Topics: Chromatography, Liquid; Cyclohexanones; Humans; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosinemias

2009
Inhibition of 4-hydroxyphenylpyruvate dioxygenase by 2-[2-nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione.
    Acta biochimica Polonica, 2009, Volume: 56, Issue:3

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Binding Sites; Cyclohexanones; Herbicides; Humans; Models, Biological; Molecular Structure; Tyrosinemias

2009
Clinical practice. NTBC therapy for tyrosinemia type 1: how much is enough?
    European journal of pediatrics, 2010, Volume: 169, Issue:6

    Topics: Child, Preschool; Cost-Benefit Analysis; Cyclohexanones; Dose-Response Relationship, Drug; Drug Costs; Egypt; Enzyme Inhibitors; Female; Heptanoates; Humans; Infant; Nitrobenzoates; Tyrosinemias

2010
Determination of NTBC in serum samples from patients with hereditary tyrosinemia type I by capillary electrophoresis.
    Talanta, 2010, Mar-15, Volume: 80, Issue:5

    Topics: Cyclohexanones; Electrophoresis, Capillary; Enzyme Inhibitors; Humans; Limit of Detection; Nitrobenzoates; Time Factors; Tyrosinemias

2010
Identification of NTBC metabolites in urine from patients with hereditary tyrosinemia type 1 using two different mass spectrometric platforms: triple stage quadrupole and LTQ-Orbitrap.
    Rapid communications in mass spectrometry : RCM, 2010, Volume: 24, Issue:6

    Topics: Cyclohexanones; Humans; Mass Spectrometry; Models, Molecular; Molecular Weight; Nitrobenzoates; Toluene; Tyrosinemias

2010
High volume naked DNA tail-vein injection restores liver function in Fah-knock out mice.
    Journal of gastroenterology and hepatology, 2010, Volume: 25, Issue:5

    Topics: Animals; Bilirubin; Biomarkers; Cell Proliferation; Cyclohexanones; Dependovirus; Disease Models, Animal; Genetic Therapy; Genetic Vectors; Hepatocytes; Hydrolases; Injections, Intravenous; L-Lactate Dehydrogenase; Liver; Liver Function Tests; Mice; Mice, Inbred BALB C; Mice, Knockout; Nitrobenzoates; Plasmids; Serum Albumin; Tail; Time Factors; Transfection; Tyrosinemias; Urea

2010
Tyrosinemia type 1: metastatic hepatoblastoma with a favorable outcome.
    Pediatrics, 2010, Volume: 126, Issue:1

    Topics: Adolescent; Biopsy, Needle; Carcinoma, Hepatocellular; Chemotherapy, Adjuvant; Cyclohexanones; Diagnosis, Differential; Female; Follow-Up Studies; Hepatectomy; Hepatoblastoma; Humans; Immunohistochemistry; Liver Neoplasms; Nitrobenzoates; Risk Assessment; Treatment Outcome; Tyrosinemias

2010
Monitoring tyrosinaemia type I: Blood spot test for nitisinone (NTBC).
    Clinica chimica acta; international journal of clinical chemistry, 2011, Jan-14, Volume: 412, Issue:1-2

    Topics: Blood Chemical Analysis; Cyclohexanones; Follow-Up Studies; Humans; Male; Nitrobenzoates; Specimen Handling; Treatment Outcome; Tyrosinemias

2011
Increase of CSF tyrosine and impaired serotonin turnover in tyrosinemia type I.
    Molecular genetics and metabolism, 2011, Volume: 102, Issue:2

    Topics: Brain; Child; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Male; Nitrobenzoates; Psychomotor Performance; Serotonin; Tyrosine; Tyrosinemias

2011
Tyrosinemia type I: long-term outcome in a patient treated with doses of NTBC lower than recommended.
    European journal of pediatrics, 2011, Volume: 170, Issue:6

    Topics: Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Tyrosinemias

2011
Comparison of plasma and dry blood spots as samples for the determination of nitisinone (NTBC) by high-performance liquid chromatography-tandem mass spectrometry. Study of the stability of the samples at different temperatures.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2011, Apr-01, Volume: 879, Issue:11-12

    Topics: Adolescent; Analysis of Variance; Blood Specimen Collection; Child; Child, Preschool; Chromatography, High Pressure Liquid; Cyclohexanones; Drug Stability; Female; Humans; Male; Nitrobenzoates; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Temperature; Time Factors; Tyrosinemias

2011
Hereditary tyrosinemia type 1 from a single center in Egypt: clinical study of 22 cases.
    World journal of pediatrics : WJP, 2011, Volume: 7, Issue:3

    Topics: Child, Preschool; Cyclohexanones; Egypt; Enzyme Inhibitors; Female; Humans; Infant; Liver Transplantation; Male; Nitrobenzoates; Tyrosinemias

2011
Tyrosinemia type 1 in Spain: mutational analysis, treatment and long-term outcome.
    Pediatrics international : official journal of the Japan Pediatric Society, 2011, Volume: 53, Issue:6

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Child; Child, Preschool; Cyclohexanones; DNA; DNA Mutational Analysis; Enzyme Inhibitors; Female; Follow-Up Studies; Genotype; Humans; Incidence; Male; Mutation; Nitrobenzoates; Retrospective Studies; Spain; Time Factors; Treatment Outcome; Tyrosine; Tyrosinemias

2011
Generation of healthy mice from gene-corrected disease-specific induced pluripotent stem cells.
    PLoS biology, 2011, Volume: 9, Issue:7

    Topics: Animals; Cell Survival; Cells, Cultured; Chromosomes; Cyclohexanones; Disease Models, Animal; Female; Fetus; Fibroblasts; Genetic Complementation Test; Genetic Therapy; Genetic Vectors; Humans; Hydrolases; Induced Pluripotent Stem Cells; Lentivirus; Liver; Mice; Mice, Knockout; Nitrobenzoates; Pregnancy; Promoter Regions, Genetic; Spleen Focus-Forming Viruses; Tetraploidy; Tyrosinemias

2011
Neurocognitive outcome in patients with hypertyrosinemia type I after long-term treatment with NTBC.
    Journal of inherited metabolic disease, 2012, Volume: 35, Issue:2

    Topics: Cerebrum; Child; Child, Preschool; Cognition; Cognition Disorders; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Infant; Language Development; Long-Term Care; Male; Motor Activity; Nitrobenzoates; Psychometrics; Time; Treatment Outcome; Tyrosine; Tyrosinemias

2012
Grown up mice from gene-corrected iPS cells.
    Circulation research, 2011, Nov-11, Volume: 109, Issue:11

    Topics: Animals; Cell Differentiation; Cyclohexanones; Embryo Transfer; Gene Silencing; Genetic Complementation Test; Genetic Vectors; Hydrolases; Induced Pluripotent Stem Cells; Lentivirus; Liver; Mice; Mice, Knockout; Mice, Transgenic; Nitrobenzoates; Transduction, Genetic; Transgenes; Tyrosinemias

2011
LC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I.
    Analytical chemistry, 2012, Jan-17, Volume: 84, Issue:2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Biomarkers; Child; Child, Preschool; Chromatography, Liquid; Cyclohexanones; Diet, Protein-Restricted; Drug Monitoring; Enzyme Inhibitors; Heptanoates; Humans; Infant; Infant, Newborn; Kidney; Liver; Methionine; Nitrobenzoates; Phenylalanine; Tandem Mass Spectrometry; Tyrosine; Tyrosinemias

2012
Single dose NTBC-treatment of hereditary tyrosinemia type I.
    Journal of inherited metabolic disease, 2012, Volume: 35, Issue:5

    Topics: Cyclohexanones; Drug Administration Schedule; Female; Heptanoates; Humans; Infant; Infant, Newborn; Male; Nitrobenzoates; Tyrosinemias

2012
Plasma succinylacetone is persistently raised after liver transplantation in tyrosinaemia type 1.
    Journal of inherited metabolic disease, 2013, Volume: 36, Issue:1

    Topics: Adolescent; Child; Child, Preschool; Cyclohexanones; Heptanoates; Humans; Infant; Liver Transplantation; Nitrobenzoates; Porphobilinogen Synthase; Retrospective Studies; Tyrosinemias

2013
Confocal microscopy of corneal crystals in a patient with hereditary tyrosinemia type I, treated with NTBC.
    Cornea, 2013, Volume: 32, Issue:1

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Corneal Opacity; Crystallization; Cyclohexanones; Enzyme Inhibitors; Epithelium, Corneal; Fluorescein; Humans; Male; Microscopy, Confocal; Nitrobenzoates; Staining and Labeling; Tyrosinemias

2013
Simple and fast quantification of nitisone (NTBC) using liquid chromatography-tandem mass spectrometry method in plasma of tyrosinemia type 1 patients.
    Journal of chromatographic science, 2012, Volume: 50, Issue:5

    Topics: Adolescent; Child; Child, Preschool; Chromatography, High Pressure Liquid; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Nitrobenzoates; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Tyrosinemias

2012
[Clinical follow up of Chilean patients with tyrosinemia type 1 treated with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-ciclohexanedione (NTBC)].
    Revista medica de Chile, 2012, Volume: 140, Issue:2

    Topics: Child, Preschool; Chile; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Liver Neoplasms; Liver Transplantation; Male; Nitrobenzoates; Retrospective Studies; Time Factors; Treatment Outcome; Tyrosinemias

2012
Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec.
    Molecular genetics and metabolism, 2012, Volume: 107, Issue:1-2

    Topics: Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Infant, Newborn; Kidney; Liver; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Quebec; Treatment Outcome; Tyrosinemias

2012
Corneal depositions in tyrosinaemia type I during treatment with Nitisinone.
    BMJ case reports, 2012, Nov-30, Volume: 2012

    Topics: Adolescent; Corneal Diseases; Cyclohexanones; Enzyme Inhibitors; Eye Pain; Humans; Male; Nitrobenzoates; Patient Compliance; Photophobia; Tyrosinemias

2012
Maternal and fetal tyrosinemia type I.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: Biomarkers; Child Development; Consanguinity; Cyclohexanones; Diet, Protein-Restricted; DNA Mutational Analysis; Female; Genetic Predisposition to Disease; Heptanoates; Heredity; Heterozygote; Homozygote; Humans; Hydrolases; Infant; Infant, Newborn; Live Birth; Mutation; Nitrobenzoates; Pedigree; Phenotype; Pregnancy; Tyrosine; Tyrosinemias; Young Adult

2010
Corneal opacities associated with NTBC treatment.
    American journal of ophthalmology, 2002, Volume: 134, Issue:2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Child, Preschool; Cornea; Corneal Opacity; Cyclohexanones; Diet, Protein-Restricted; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias

2002
Tyrosinemia I, a model for human diseases mediated by 2-oxoacid-utilizing dioxygenases: hepatotoxin suppression by NTBC does not normalize hepatic collagen metabolism.
    Journal of pediatric gastroenterology and nutrition, 2002, Volume: 35, Issue:1

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Collagen; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Liver; Models, Molecular; Molecular Structure; Nitrobenzoates; Procollagen-Proline Dioxygenase; Tyrosinemias

2002
Reversibility of cirrhotic regenerative liver nodules upon NTBC treatment in a child with tyrosinaemia type I.
    Acta paediatrica (Oslo, Norway : 1992), 2003, Volume: 92, Issue:5

    Topics: Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Infant, Newborn; Liver Cirrhosis; Liver Regeneration; Male; Nitrobenzoates; Remission Induction; Tyrosinemias

2003
Pharmacological rescue of the 14CoS/14CoS mouse: hepatocyte apoptosis is likely caused by endogenous oxidative stress.
    Free radical biology & medicine, 2003, Aug-15, Volume: 35, Issue:4

    Topics: Animals; Apoptosis; Caspase 3; Caspases; Cyclohexanones; Free Radicals; Glutamate-Cysteine Ligase; Glutathione; Hepatocytes; Heterozygote; Humans; Hydrolases; Kidney; Liver; Mice; Mice, Knockout; Mice, Transgenic; Microscopy, Electron; Models, Chemical; Nitrobenzoates; Oxidative Stress; Protein Structure, Tertiary; RNA; RNA, Messenger; Time Factors; Tyrosine; Tyrosinemias

2003
Extensive changes in liver gene expression induced by hereditary tyrosinemia type I are not normalized by treatment with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC).
    Journal of hepatology, 2003, Volume: 39, Issue:6

    Topics: Animals; Blotting, Northern; Cyclohexanones; DNA, Complementary; Enzyme Inhibitors; Female; Gene Expression; Gene Expression Profiling; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Nitrobenzoates; Phenotype; Reproducibility of Results; Tyrosinemias

2003
Chronic liver disease in murine hereditary tyrosinemia type 1 induces resistance to cell death.
    Hepatology (Baltimore, Md.), 2004, Volume: 39, Issue:2

    Topics: Acetaminophen; Analgesics, Non-Narcotic; Animals; Apoptosis; Cell Survival; Central Nervous System Depressants; Chronic Disease; Cyclohexanones; Enzyme Inhibitors; Ethanol; Fas Ligand Protein; fas Receptor; Glutathione; Homogentisic Acid; HSP70 Heat-Shock Proteins; Hydrolases; Liver Failure, Acute; Membrane Glycoproteins; Mice; Mice, Mutant Strains; Necrosis; NF-kappa B; Nitrobenzoates; Oxidative Stress; Proto-Oncogene Proteins c-jun; Tyrosinemias; Up-Regulation

2004
Renal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1.
    Kidney international, 2004, Volume: 66, Issue:3

    Topics: Animals; Caspase 3; Caspase 9; Caspases; Cyclohexanones; Disease Models, Animal; DNA Fragmentation; Enzyme Inhibitors; Hepatocytes; Homogentisic Acid; Hydrolases; In Situ Nick-End Labeling; Kidney Tubules, Proximal; Mice; Mice, Knockout; Nitrobenzoates; Survival Rate; Tyrosine; Tyrosinemias

2004
Experience with NTBC therapy in hereditary tyrosinaemia type I: an alternative to liver transplantation.
    Annals of tropical paediatrics, 2004, Volume: 24, Issue:3

    Topics: Child, Preschool; Combined Modality Therapy; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Liver Transplantation; Male; Nitrobenzoates; Treatment Outcome; Tyrosinemias

2004
Hepatocellular carcinoma in hereditary tyrosinemia type I despite 2-(2 nitro-4-3 trifluoro- methylbenzoyl)-1, 3-cyclohexanedione treatment.
    Journal of pediatric gastroenterology and nutrition, 2005, Volume: 40, Issue:1

    Topics: Carcinoma, Hepatocellular; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Neoplasms; Liver Transplantation; Male; Nitrobenzoates; Treatment Outcome; Tyrosinemias

2005
Successful treatment of severe cardiomyopathy with NTBC in a child with tyrosinaemia type I.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Cardiomyopathy, Hypertrophic; Cyclohexanones; Echocardiography; Enzyme Inhibitors; Humans; Hypertrophy, Left Ventricular; Infant; Male; Nitrobenzoates; Tyrosine; Tyrosinemias

2005
Tandem mass spectrometric assay of succinylacetone in urine for the diagnosis of hepatorenal tyrosinemia.
    Analytical biochemistry, 2005, Apr-15, Volume: 339, Issue:2

    Topics: Adolescent; Adult; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Heptanoates; Humans; Infant; Infant, Newborn; Mass Spectrometry; Middle Aged; Neonatal Screening; Nitrobenzoates; Reproducibility of Results; Tyrosinemias

2005
Involvement of endoplasmic reticulum stress in hereditary tyrosinemia type I.
    The Journal of biological chemistry, 2006, Mar-03, Volume: 281, Issue:9

    Topics: Acetoacetates; Animals; Cell Line; Cricetinae; Cyclohexanones; Endoplasmic Reticulum; Endoplasmic Reticulum Chaperone BiP; Enzyme Inhibitors; Eukaryotic Initiation Factor-2; Heat-Shock Proteins; Humans; Hydrolases; Liver; Lung; Mice; Mice, Knockout; Molecular Chaperones; Nitrobenzoates; Proteasome Endopeptidase Complex; Signal Transduction; Transcription, Genetic; Tyrosinemias

2006
Kidneys of mice with hereditary tyrosinemia type I are extremely sensitive to cytotoxicity.
    Pediatric research, 2006, Volume: 59, Issue:3

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Cyclohexanones; Enzyme Inhibitors; Female; Homogentisic Acid; Humans; Hydrolases; In Situ Nick-End Labeling; Kidney; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Nitrobenzoates; Tyrosinemias

2006
Tyrosinemia produced by 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione (NTBC) in experimental animals and its relationship to corneal injury.
    Toxicology and applied pharmacology, 2006, Aug-15, Volume: 215, Issue:1

    Topics: Animals; Cornea; Corneal Injuries; Cyclohexanones; Dogs; Dose-Response Relationship, Drug; Homogentisate 1,2-Dioxygenase; Macaca mulatta; Male; Nitrobenzoates; Rabbits; Tyrosine Transaminase; Tyrosinemias

2006
Evaluation of dichloroacetate treatment in a murine model of hereditary tyrosinemia type 1.
    Biochemical pharmacology, 2006, May-28, Volume: 71, Issue:11

    Topics: Animals; Cyclohexanones; Dichloroacetic Acid; Female; Glutathione; Glutathione Disulfide; Hydrolases; Kidney; Liver; Male; Mice; Nitrobenzoates; Oxidative Stress; Tyrosine; Tyrosinemias; Weight Loss

2006
Cardiomyopathy in tyrosinaemia type I is common but usually benign.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:1

    Topics: Adolescent; Cardiomyopathies; Child; Child, Preschool; Cyclohexanones; Echocardiography; Enzyme Inhibitors; Female; Humans; Infant; Infant, Newborn; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias

2006
Nitisinone in the treatment of hereditary tyrosinaemia type 1.
    Drugs, 2006, Volume: 66, Issue:6

    Topics: Carcinoma, Hepatocellular; Cyclohexanones; Enzyme Inhibitors; Humans; Kidney Diseases; Liver Diseases; Liver Failure; Liver Neoplasms; Liver Transplantation; Monitoring, Physiologic; Nitrobenzoates; Tyrosine; Tyrosinemias

2006
Tyrosinemia type I treated by NTBC: how does AFP predict liver cancer?
    Molecular genetics and metabolism, 2006, Volume: 89, Issue:4

    Topics: alpha-Fetoproteins; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Infant; Liver Neoplasms; Male; Nitrobenzoates; Prognosis; Tyrosinemias

2006
[New drugs; nitisinone].
    Nederlands tijdschrift voor geneeskunde, 2006, Nov-18, Volume: 150, Issue:46

    Topics: Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias

2006
Nitisinone: new drug. Type 1 tyrosinemia: an effective drug.
    Prescrire international, 2007, Volume: 16, Issue:88

    Topics: Child; Child, Preschool; Clinical Trials as Topic; Cost-Benefit Analysis; Cyclohexanones; Drug Approval; Enzyme Inhibitors; Europe; Humans; Infant; Infant, Newborn; Nitrobenzoates; Survival Rate; Time Factors; Tyrosinemias

2007
Rescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment.
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:3

    Topics: Animals; Animals, Newborn; Antioxidants; Ascorbic Acid; Carcinoma, Hepatocellular; Cyclohexanones; Disease Models, Animal; Drug Therapy, Combination; Female; Glutathione; Hydrolases; Liver Neoplasms; Mice; Mice, Knockout; Nitrobenzoates; Oxidative Stress; Pregnancy; Tyrosinemias

2008
Hepatic stress in hereditary tyrosinemia type 1 (HT1) activates the AKT survival pathway in the fah-/- knockout mice model.
    Journal of hepatology, 2008, Volume: 48, Issue:2

    Topics: Animals; bcl-X Protein; Carcinoma, Hepatocellular; Cyclohexanones; Disease Models, Animal; Hydrolases; Liver Neoplasms; Mice; Mice, Knockout; Nitrobenzoates; Proto-Oncogene Proteins c-akt; Proto-Oncogene Proteins c-bcl-2; Signal Transduction; Tyrosinemias

2008
NTBC treatment in tyrosinaemia type I: long-term outcome in French patients.
    Journal of inherited metabolic disease, 2008, Volume: 31, Issue:1

    Topics: Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Follow-Up Studies; France; Humans; Infant; Infant, Newborn; Liver; Liver Function Tests; Nitrobenzoates; Patient Compliance; Retrospective Studies; Time Factors; Treatment Failure; Tyrosinemias

2008
Severe neurological crisis in a patient with hereditary tyrosinaemia type I after interruption of NTBC treatment.
    Journal of inherited metabolic disease, 2008, Volume: 31 Suppl 2

    Topics: Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Humans; Hypertension; Infant; Male; Medication Adherence; Nitrobenzoates; Polyneuropathies; Respiration, Artificial; Respiratory Insufficiency; Respiratory Paralysis; Tyrosinemias

2008
Elevated plasma bile acid concentrations in two sisters with tyrosinaemia type I.
    Journal of paediatrics and child health, 2000, Volume: 36, Issue:1

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Bile Acids and Salts; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Infant; Infant, Newborn; Nitrobenzoates; Nuclear Family; Tyrosinemias

2000
Cutaneous porphyria in a neonate with tyrosinaemia type 1.
    European journal of pediatrics, 2000, Volume: 159, Issue:7

    Topics: Combined Modality Therapy; Consanguinity; Coproporphyrins; Cyclohexanones; Enzyme Inhibitors; Humans; Infant, Newborn; Liver Failure; Male; Nitrobenzoates; Photosensitivity Disorders; Phototherapy; Porphyria Cutanea Tarda; Tyrosinemias

2000
[Evolution of a case of tyrosinemia type I treated with NTBC].
    Anales espanoles de pediatria, 2001, Volume: 54, Issue:3

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Carcinoma, Hepatocellular; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Neoplasms; Male; Nitrobenzoates; Palliative Care; Tyrosinemias

2001
Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I.
    Molecular genetics and metabolism, 2002, Volume: 75, Issue:1

    Topics: Animals; Cyclohexanones; Diet; Disease Models, Animal; Enzyme Inhibitors; Hydrolases; Mice; Mice, Knockout; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2002
From the Food and Drug Administration.
    JAMA, 2002, Mar-06, Volume: 287, Issue:9

    Topics: Adult; Adverse Drug Reaction Reporting Systems; Anti-Inflammatory Agents, Non-Steroidal; Antidepressive Agents, Second-Generation; Antineoplastic Agents; Arthritis, Psoriatic; Benzamides; Chemical and Drug Induced Liver Injury; Cyclohexanones; Enzyme Inhibitors; Etanercept; Gastrointestinal Neoplasms; Humans; Imatinib Mesylate; Immunoglobulin G; Infant; Liver Failure; Nitrobenzoates; Orphan Drug Production; Piperazines; Pyrimidines; Receptors, Tumor Necrosis Factor; Recombinant Fusion Proteins; Triazoles; Tyrosinemias; United States; United States Food and Drug Administration

2002