cysteamine has been researched along with Cystinosis in 274 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 56 (20.44) | 18.7374 |
| 1990's | 39 (14.23) | 18.2507 |
| 2000's | 47 (17.15) | 29.6817 |
| 2010's | 85 (31.02) | 24.3611 |
| 2020's | 47 (17.15) | 2.80 |
| Authors | Studies |
|---|---|
| Cairns, D; Kay, G; Knott, RM; McCaughan, B | 1 |
| Cairns, D; Di Salvo, A; Kay, G; Knott, RM; Omran, Z | 1 |
| Cairns, D; Hector, EE; Kay, G; Knott, RM; Omran, Z | 1 |
| Benylles, A; Cairns, D; Kay, G; Knott, RM; Moloney, KA; Omran, Z | 1 |
| Albersen, M; Besouw, M; Camps, C; Cyr, D; D'Hauwers, K; de Wever, L; Goffredo, BM; Goossens, E; Hamer, R; Janssen, MCH; Kadam, P; Levtchenko, E; Monnens, L; Reda, A; Rega, LR; Spiessens, C; Taranta, A; van den Heuvel, L; Veys, K; Wetzels, A | 1 |
| Grebe, J; Grüneberg, M; Harms, E; Klank, S; Marquardt, T; Ottolenghi, C; Reunert, J; van Stein, C | 1 |
| Hohenfellner, K; Keidel, L; Luft, N; Priglinger, C; Priglinger, S; Schworm, B | 1 |
| Acquaviva, C; Alioli, C; Bacchetta, J; Bertholet-Thomas, A; Boyer, O; Gaillard, S; Hogan, J; Lemoine, S; Machuca-Gayet, I; Peyruchaud, O; Quinaux, T; Servais, A; Vasseur, S; Vrillon, I | 1 |
| Abouelwoun, II; Atia, FM; Elkhateeb, N; Elmonem, MA; Helmy, R; Selim, R; Soliman, NA | 1 |
| Harms, E; Herzig, N; Hohenfellner, K; Holla, H; Knerr, C; Köppl, C; Landfarth, T; Müller-Felber, W; Steidle, G; Vill, K | 1 |
| Besouw, M; Reda, A; Veys, K | 1 |
| Acham-Roschitz, B; Arbeiter, K; Billing, H; Büscher, A; Haffner, D; Hagenberg, M; Hohenfellner, K; Jagodzinski, C; Kanzelmeyer, N; Kemper, MJ; Kluck, R; Müller, S; Oh, J; Pavičić, L; Thumfart, J; Tönshoff, B; Weber, LT; Zivicnjak, M | 1 |
| Giancarlo, I; Gianni, P; Luca, B; Maria, SC; Matteo, F; Parrilla, R; Sergio, P; Valente, P | 1 |
| Ames, EG; Thoene, JG | 1 |
| Boddaert, N; Boisgontier, J; Hummel, A; Saitovitch, A; Servais, A | 1 |
| Ariceta, G; Elenberg, E; Hohenfellner, K; Nesterova, G; Soliman, NA; Topaloglu, R | 1 |
| Csorba, A; Kelen, K; Maka, E; Nagy, ZZ; Reusz, G; Szabó, A | 1 |
| Canalini, F; Cassiman, D; Emma, F; Goffredo, BM; Levtchenko, E; Montini, G; Pennesi, M; Peruzzi, L; Rossi, S; Verrina, E | 1 |
| Cairns, D; Hector, E; Michael Wall, G | 1 |
| Bayram-Suverza, M; Vázquez-Lara, Y; Virgen-Batista, MI | 1 |
| Chaudhry, AB; DiLeo, MV; Gertsman, I; Jimenez, J; Nischal, KK; Resnick, JL | 1 |
| Acham-Roschitz, B; Arbeiter, K; Bald, M; Benz, M; Billing, H; Boulesteix, AL; Buescher, A; Gahl, WA; Galiano, M; Haffner, D; Hohenfellner, K; Hoppe, B; John-Kroegel, U; Kettwig, M; Klaus, G; Marx-Berger, D; Moeller, K; Moser, K; Mueller, D; Nießl, C; Oh, J; Palm, K; Patzer, L; Pohl, M; Schlingmann, P; Seitz, B; Tönshoff, B; Treikauskas, U; von Vigier, RO; Wühl, E; Wygoda, S; Zirngibl, M | 1 |
| Gahl, WA; Haffner, D; Hohenfellner, K; Nießl, C; Oh, J; Okorn, C; Palm, K; Schlingmann, KP; Wygoda, S | 1 |
| Beck, BB; Bieniaś, B; Grenda, R; Kieć-Wilk, B; Kowalczyk, M; Marquardt, T; Nosek, H; Rubik, J; Sikora, P; Surowiec, P; Szymczak, M; Zaniew, M | 1 |
| Ariceta, G; Awan, A; Besouw, M; Bockenhauer, D; Haffner, D; Harms, E; Hohenfellner, K; Janssen, MCH; Kanzelmeyer, N; Levtchenko, E; Niaudet, P; Novo, R; Pape, L; Servais, A; Sikora, P; Topaloglu, R; van den Heuvel, B; Veys, K; Wühl, E; Zadora, W | 1 |
| Battafarano, G; Bellomo, F; Cairoli, S; Camassei, FD; D'Oria, V; De Leo, E; Del Fattore, A; Emma, F; Goffredo, BM; Pezzullo, M; Polishchuk, E; Polishchuk, R; Raso, R; Rega, LR; Taranta, A | 1 |
| Addobbati, R; Decorti, G; Del Savio, R; Franzin, M; Rossetto, S; Ruoso, R; Stocco, G | 1 |
| Bowman, MA; Casanova, MI; Chokshi, TJ; Cosert, K; Daley, NL; Gragg, MM; Kang, J; Leonard, BC; Mccorkell, ME; Minella, AL; Murphy, CJ; Raghunathan, VK; Thomasy, SM | 1 |
| Al-Sabban, E; Alsaad, KO; Berlingerio, SP; Bondue, T; Kouraich, A; Levtchenko, E; Marie, S; van den Heuvel, L; Veys, K | 1 |
| Haffner, D; Hohenfellner, K; Zerell, K | 1 |
| Aase, SA; Bjerre, A; Brackman, D; Forsberg, B; Gudmundsdottir, H; Radtke, M; Siva, C; Woldseth, B | 1 |
| Langman, CB | 1 |
| Bachhawat, AK; Deshpande, AA; Kanakaraj, A; Ravichandran, R; Vashist, N | 1 |
| Azimpour, K; Carlot, S; Game, D; Lashilola, S; McCarthy, M; van der Voort, J; Xu, W | 1 |
| Anglicheau, D; Antignac, C; Boddaert, N; Boisgontier, J; Funalot, B; Hummel, A; Legendre, C; Lemaitre, H; Pontoizeau, C; Saitovitch, A; Sberro-Soussan, R; Scemla, A; Servais, A; Snanoudj, R | 1 |
| Blackburn, J; Ramakrishnan, R | 1 |
| Filatova, A; Levtchenko, E; Papizh, S; Prikhodina, L; Serzhanova, V; Skoblov, M; Tabakov, V; van den Heuvel, L | 1 |
| Kasimer, RN; Langman, CB | 1 |
| D'Souza, RF; Davidson, AJ; Harrison, PT; Hollywood, JA; Holm, TM; Przepiorski, A; Sreebhavan, S; Wolvetang, EJ | 1 |
| Dixon, AM; Straatmann, C; Styres, C | 1 |
| Acquaviva-Bourdain, C; Bertholet-Thomas, A; Cochat, P; Cornu, C; Deschênes, G; Gaillard, S; Kassai, B; Mercier, C; Morin, D; Nony, P; Roche, L; Subtil, F; Vianey-Saban, C | 1 |
| Cornelissen, EAM; Elmonem, MA; Hohenfellner, K; Janssen, MC; Levtchenko, E; Prencipe, G; van den Heuvel, LP; Van Dyck, M; Veys, KRP | 1 |
| Eldin, KW; Elenberg, E; O'Mahony, CA | 1 |
| Topaloglu, R | 1 |
| Baudouin, C; Giordano, V; Labbé, A; Liang, H; Plisson, C | 1 |
| Akhan, O; Çiftci, T; Demir, H; Demir, N; Gülhan, B; Gültekingil, A; Ozaltin, F; Temucin, ÇM; Topaloglu, R; Yuce, A | 1 |
| Avti, P; Bhattacharyya, A; Bhattacharyya, J; Das, D; Kaur, H; Kaur, S; Kumar, S; Medhi, B; Prajapat, M; Prakash, A; Ram, J; Sarma, P; Shekhar, N; Singh, R | 1 |
| Acquaviva-Bourdain, C; Bacchetta, J; Bertholet-Thomas, A; Bodénan, E; Cochat, P; Deschênes, G; Gaillard, S; Kassai, B; Laudy, V; Lemoine, S; Mercier, C; Morin, D; Nony, P; Ranchin, B; Roche, L; Rouges, C; Subtil, F; Vianey-Saban, C; Zarrabian, S | 1 |
| Fedorchak, MV; Jimenez, J; Nischal, KK; Resnick, JL; Washington, MA | 1 |
| Berends, CL; Bergmann, KR; Büller, H; Burggraaf, J; de Leede, LGJ; de Loos, F; de Visser, SJ; de Vries, WS; Kamerling, IMC; Klarenbeek, NB; Moerland, M; Pagan, L; van der Wel, V; van Esdonk, MJ; Waals, H | 1 |
| Altelaar, M; Ammerlaan, CM; Berkers, CR; Berlingerio, SP; Clevers, H; Essa, K; Jamalpoor, A; Janssen, MJ; Klumperman, J; Levtchenko, E; Lilien, MR; Masereeuw, R; Pou Casellas, C; Rega, LR; Rookmaaker, MB; van der Welle, RE; van Gelder, CA; Verhaar, MC; Veys, KR; Voskuil, K; Yousef Yengej, FA; Zaal, EA | 1 |
| Antignac, C; Ariceta, G; Bertholet-Thomas, A; Bettini, C; Bockenhauer, D; Collin, S; Cornelissen, E; Deschênes, G; Devuyst, O; Emma, F; Greco, M; Haffner, D; Hoff, WV; Hohenfellner, K; Hulton, S; Janssen, M; Levtchenko, E; Niaudet, P; Novo, R; Oh, J; Ozaltin, F; Pape, L; Ravà, L; Servais, A; Topaloglu, R; Veys, K | 1 |
| Trauner, D | 1 |
| Bishop, R | 1 |
| Thoene, JG | 3 |
| Baudouin, C; Labbé, A; Le Mouhaër, J; Liang, H; Plisson, C | 1 |
| Blanco-Mendez, J; Díaz-Tomé, V; Fernández-Ferreiro, A; García-Mazás, C; Gil-Martínez, M; González-Barcia, M; Herranz, M; Lamas, MJ; Luaces-Rodríguez, A; Otero-Espinar, FJ; Rodríguez-Ares, MT; Silva-Rodríguez, J | 1 |
| Ben-Omran, T; Blom, HJ; Dewik, AN; Gallego-Villar, L; Häberle, J; Hannibal, L; Kruger, WD; Nasrallah, GK; Thöny, B | 1 |
| Hemels, M; Karabis, A; Medic, G; van der Weijden, M | 1 |
| Higashi, S; Ito, S; Matsunoshita, N; Nozu, K; Otani, M; Tokuhiro, E | 1 |
| Blanco-Méndez, J; Díaz-Tomé, V; Fernández-Ferreiro, A; Gil-Martínez, M; González-Barcia, M; Lamas, MJ; Luaces-Rodríguez, A; Otero-Espinar, FJ; Rodríguez Ares, MT; Touriño Peralba, R | 1 |
| Bhattacharya, A; Chauhan, A; Dixon, P; Fentzke, RC; Hazra, S; Konar, A | 1 |
| Ariceta, G; Giordano, V; Santos, F | 1 |
| Armas, D; Brannagan, M; Checani, GC; Confer, NF; Holt, RJ; Obaidi, M; Xie, Y | 1 |
| Bacchetta, J; Bertholet-Thomas, A; Carlier, MC; Claramunt-Taberner, D; Cochat, P; Cohen-Solal, M; Deschênes, G; Gaillard, S; Pelletier, S; Sornay-Rendu, E; Szulc, P | 1 |
| Brand, E; Canaan-Kuehl, S; Kaufeld, J; Kurschat, C; Oh, J; Pape, L; Weber, LT | 1 |
| Grandy, J; Krall, P; Nualart, D | 1 |
| Cadieux, B; Hauser, TA; Isbell, SL; Johnson, M; Pavloff, N; Williams, C | 1 |
| Gong, CX; Li, XQ; Peng, XX | 1 |
| Aktas, Z; Hasanreisoglu, M; Ozdemir, HB; Özmen, MC | 1 |
| Chauhan, A; Dixon, P | 1 |
| Janssen, MCH; Knuijt, S; Levtchenko, EN; van Rijssel, AE; Veys, K | 1 |
| Durlik, M; Gałązka, Z; Gozdowska, J; Grenda, R; Kuczborska, K; Lewandowska, D; Nazarewski, S | 1 |
| Ribeiro, AC; Ribeiro, L; Saraiva, E; Sequeira, J; Sousa-Neves, F; Varandas, R | 1 |
| Ariceta, G; Awan, A; Bacchetta, J; Bechtold, S; Bergmann, C; Cassidy, N; Deschenes, G; Elenberg, E; Gahl, WA; Greil, O; Haffner, D; Harms, E; Herzig, N; Hohenfellner, K; Hoppe, B; Koeppl, C; Levtchenko, E; Lewis, MA; Nesterova, G; Rauch, F; Santos, F; Schlingmann, KP; Servais, A; Soliman, NA; Steidle, G; Sweeney, C; Topaloglu, R; Treikauskas, U; Tsygin, A; V Vigier, R; Veys, K; Zustin, J | 1 |
| Cazals, X; Cottier, JP; Domengie, F; Favelle, O; Lauvin, MA; Nivet, H | 1 |
| Besouw, MT; Cornelissen, EA; De Paepe, A; Desmet, K; Emma, F; Greco, M; Janssen, MC; Levtchenko, EN; Lilien, MR; Malfait, F; Nobili, F; Schneider, J; Skovby, F; Symoens, S; van den Heuvel, LP | 1 |
| Morrow, T | 1 |
| Aparecido Volpini, R; Cunha Sanches, TR; de Bragança, AC; Lopes Neri, LA; Macaferri da Fonseca, FA; Moreira Filho, CA; Pache de Faria Guimaraes, L; Seguro, AC; Shimizu, MH; Sumita, NM; Vaisbich, MH | 1 |
| Baudouin, C; Charbit, M; Deschênes, G; Guest, G; Labbé, A; Loirat, C; Niaudet, P | 1 |
| Bacchetta, J; Bertholet-Thomas, A; Cochat, P; Tasic, V | 1 |
| Bagger, MJ; Cochat, P; Cornelissen, EA; Deschenes, G; Elenberg, E; Gaillard, S; Greenbaum, LA; Grimm, P; Hanna, C; Langman, CB; Morin, D; Niaudet, P; Rioux, P; Sarwal, M | 1 |
| Alexander, SI; Clayton, PA; McTaggart, SJ; Spicer, RA; Zhang, GY | 1 |
| De Leo, MG; De Matteis, MA; Ivanova, E; Levtchenko, E | 1 |
| Ariceta, G; Bedoya, R; Bravo, J; Bueno, A; Camacho, JA; Cantarell, C; Crespo, J; Daza, A; García-Nieto, V; Gil Calvo, M; Jiménez Del Cerro, LA; Justa, ML; Lara, E; Llamas, F; Muñoz, MA; Oppenheimer, F; Romero, R; Sanahuja, MJ; Santos, F; Urisarri, A; Valenciano, B; Vara, J | 1 |
| Bernardini, I; Gahl, WA; Nesterova, G; Williams, C | 1 |
| Besouw, MT; Casteels, I; Dyck, MV; Levtchenko, EN; Pinxten, AM; Veys, KR | 1 |
| Beinart, N; Graham, CD; Hackett, RA; Ostermann, M; Weinman, J | 1 |
| Bertholet-Thomas, A; Choukroun, G; Decramer, S; Goizet, C; Llanas, B; Novo, R; Servais, A | 1 |
| Bellomo, F; Bernardo, ME; Biagini, S; Cirillo, V; Conforti, A; Emma, F; Locatelli, F; Pitisci, A; Starc, N; Taranta, A | 1 |
| Besouw, MT; Cassiman, D; Claes, KJ; Levtchenko, EN; Van Dyck, M | 1 |
| Sankawa, J | 1 |
| Lyou, Y; Nangia, CS; Zhao, X | 1 |
| Berryhill, A; Bhamre, S; Chaudhuri, A; Concepcion, W; Grimm, PC | 1 |
| Ariceta, G; Camacho, JA; Fernández-Obispo, M; Fernández-Polo, A; Gamez, J; García-Villoria, J; Güell, A; Lara Monteczuma, E; Leyes, P; Martín-Begué, N; Morell, GP; Oppenheimer, F; Perelló, M; Santandreu, AV; Torra, R | 1 |
| Anderson, RJ; Cannell, SJ; Frost, L; Groundwater, PW; Hambleton, PA; Suryadevara, P | 1 |
| Bultynck, G; De Smedt, H; Elmonem, MA; Ivanova, EA; Levtchenko, EN; Mekahli, D; Missiaen, L; Pastore, A; van den Heuvel, LP | 1 |
| Abidi, K; Ben Turkia, H; Gargah, T; Hammi, Y; Jellouli, M | 1 |
| Chrystyn, H; Cotton, S; Devereux, G; Devlin, E; Fraser-Pitt, D; Griffiths, K; Norrie, J; O'Neil, D; Steele, S | 1 |
| Barshop, BA; Deschênes, G; Emma, F; Goodyer, P; Langman, CB; Levtchenko, EN; Lipkin, G; Midgley, JP; Ottolenghi, C; Servais, A; Soliman, NA; Thoene, JG | 1 |
| Ahlenstiel-Grunow, T; Drube, J; Froede, K; Kanzelmeyer, NK; Kreuzer, M; Lerch, C; Pape, L | 1 |
| Acharya, G; Isenhart, LC; Jester, JV; Lee, B; Li, F; Liu, X; Marcano, DC; Pflugfelder, SC; Shin, CS; Simpson, J | 1 |
| Cairns, D; Kay, G; Knott, R; Matthews, KH; McKenzie, B | 1 |
| Hartman, TM; Ivanova, EA; Levtchenko, EN; Pastore, A; Paul, P; Price, NPJ; Ramazani, Y; Van Den Heuvel, L; Van Schepdael, A | 1 |
| Arcolino, FO; Elmonem, MA; Levtchenko, E; van den Heuvel, L; Veys, KR | 1 |
| Bäumner, S; Weber, LT | 1 |
| Bozdağ, S; Gümüş, K; Gümüş, O; Unlü, N | 1 |
| Besouw, MT; Janssen, MC; Kremer, JA; Levtchenko, EN | 1 |
| D'Cruz, D; Horsfield, C; Krischock, L; Rigden, SP | 1 |
| Berger, JR; Dillon, DA; Goldstein, SJ; Nelson, P; Young, BA | 1 |
| Bazaraa, H; El-Baroudy, R; Rizk, A; Soliman, NA; Younan, A | 1 |
| Feksa, LR; Figueiredo, VC; Wannmacher, CM | 1 |
| Dimitriou, E; Emma, F; Karavitakis, EM; Kosma, K; Michelakakis, H; Xaidara, A | 1 |
| Coelho, D; Macário, MC; Murta, J; Quadrado, MJ; Tavares, R; Torres, A | 1 |
| Barshop, BA; Dohil, R; Fidler, M; Gangoiti, JA; Kaskel, F; Schneider, JA | 1 |
| Koch, VH; Vaisbich, MH | 1 |
| Dakshinamurty, KV; Prasad, N; Ram, R; Reddy, PN; Swarnalatha, G | 1 |
| Barshop, BA; Cabrera, BL; Dohil, R; Fidler, M; Gangoiti, JA; Schneider, JA | 2 |
| Ahmad, ZP; Johnstone, LM; Walker, AM | 1 |
| Buchan, B; Cairns, D; Heneghan, A; Kay, G; Matthews, KH | 1 |
| Fortin, A; Gros, P; Min-Oo, G; Poulin, JF | 1 |
| Levtchenko, EN; Schoeber, JP; van den Heuvel, LP; Wilmer, MJ | 1 |
| Brugnara, M; Emma, F; Greco, M; Pastore, A; Taranta, A; Zaffanello, M | 1 |
| Lim, J; Pellois, JP; Simanek, EE | 1 |
| Besouw, M; Levtchenko, E | 1 |
| Kluijtmans, LA; Levtchenko, EN; Masereeuw, R; Monnens, LA; Scheffer, PG; van den Heuvel, LP; van der Velden, TJ; Willems, PH; Wilmer, MJ | 1 |
| El-Kares, R; Goodyer, P; Mathieu, F; Midgley, JP | 1 |
| Besouw, MT; Bowker, R; Dutertre, JP; Emma, F; Gahl, WA; Greco, M; Levtchenko, EN; Lilien, MR; McKiernan, J; Nobili, F; Schneider, JA; Skovby, F; van den Heuvel, LP; Van't Hoff, WG | 1 |
| Lund, AM; Oczachowska-Kulik, AE; Pedersen, EB; Skovby, F | 1 |
| Antignac, C; Brodin-Sartorius, A; Charbit, M; Cochat, P; Guest, G; Legendre, C; Lesavre, P; Moyse, D; Niaudet, P; Ottolenghi, C; Servais, A; Tête, MJ | 1 |
| Flynn, KJ; Jester, JV; Nien, CJ; Simpson, JL | 1 |
| Bouazza, N; Chadefaux-Vekemans, B; Deschenes, G; Niaudet, P; Ottolenghi, C; Ricquier, D; Tréluyer, JM; Urien, S | 1 |
| Bagger, MJ; Cochat, P; Cornelissen, E; Deschênes, G; Gaillard, S; Greenbaum, LA; Grimm, P; Langman, CB; Matossian, D; Morin, D; Niaudet, P; Rioux, P; Sarwal, M | 1 |
| Besouw, MTP; Francois, I; Levtchenko, EN; Van Dyck, M; Van Hoyweghen, E | 1 |
| Buchan, B; Cairns, D; Kay, G; Matthews, KH | 1 |
| Engelhorn, T; Huttner, HB; Kuramatsu, JB; Marquardt, L; Roesch, J | 1 |
| Besouw, M; Cornelissen, E; Levtchenko, E; Rioux, P; Tangerman, A | 1 |
| Cabrera, BL; Dohil, R | 1 |
| Arias, A; García-Villoria, J; Hernández-Pérez, JM; Ribes, A | 1 |
| André, B; Anne, C; Aupetit, J; Chabli, A; Chadefaux-Vekemans, B; Debacker, C; Gasnier, B; Jézégou, A; Journet, A; Kieffer-Jaquinod, S; Llinares, E; O'Regan, S; Sagné, C | 1 |
| Gahl, WA; Schneider, JA; Thoene, JG | 1 |
| Geelen, JM; Levtchenko, EN; Monnens, LA | 1 |
| Grupcheva, CN; McGhee, C; Ormonde, SE | 1 |
| Del Monte, M; Gahl, W; Granet, DB; Kaiser-Kupfer, MI; Lindblad, AS; Reed, GF; Rubin, B; Schneider, JA; Thoene, J; Thompson, D; Tsilou, ET | 1 |
| Deutsch, R; Dohil, R; Newbury, RO; Schneider, JA; Sellers, ZM | 1 |
| Gahl, WA | 1 |
| Belldina, EB; Brundage, RC; Huang, MY; Schneider, JA; Tracy, TS | 1 |
| Blom, H; de Graaf-Hess, AC; Levtchenko, EN; Monnens, LA; van den Heuvel, LP; Wilmer, M | 1 |
| de Souza Wyse, AT; Rosa, TG; Wajner, M; Wannmacher, CM | 1 |
| Blom, H; de Graaf-Hess, A; Levtchenko, E; Monnens, L; van den Heuvel, L; Wilmer, M | 1 |
| Bendavid, C; Gahl, WA; Haddad, BR; Kleta, R; Long, R; Muenke, M; Ouspenskaia, M | 1 |
| Schneider, JA | 4 |
| Khan, AO; Latimer, B | 1 |
| Gahl, WA; Kleta, R | 1 |
| Bass, D; Berry, G; DiDomenico, P; Fridge, J; Sarwal, M | 1 |
| de Souza Wyse, AT; Dutra-Filho, CS; Fleck, RM; Giacomazzi, J; Parissoto, D; Rodrigues Junior, V; Wajner, M; Wannmacher, CM | 1 |
| Almajid, P; Bernardini, I; Gahl, WA; Kleta, R; Sonies, BC | 1 |
| Herrine, SK; Navarro, VJ; Rossi, S | 1 |
| Barshop, B; Deutsch, R; Dohil, R; Fidler, M; Newbury, R; Schneider, J; Sellers, Z | 1 |
| Blom, HJ; de Graaf-Hess, AC; Levtchenko, EN; Monnens, LA; van Dael, CM; van den Heuvel, LP; Wilmer, MJ | 1 |
| Levtchenko, E; Monnens, L | 1 |
| Balog, J; Caruso, RC; Gahl, WA; Iwata, F; Kaiser-Kupfer, MI; Reed, G; Rubin, BI; Tsilou, ET | 1 |
| Kleta, R | 1 |
| Barshop, BA; Deutsch, R; Dohil, R; Fidler, M; Gangoiti, J; Martin, M; Schneider, JA | 1 |
| Zachwieja, J | 1 |
| Barshop, BA; Deutsch, R; Dohil, R; Fidler, MC; Gangoiti, JA; Martin, M; Schneider, JA | 1 |
| Besouw, M; Blom, H; de Graaf-Hess, A; Levtchenko, E; Tangerman, A | 1 |
| Arevalo do Amaral, MF; Dutra-Filho, CS; Duval Wannmacher, CM; Feksa, LR; Koch, GW; Rech, VC; Terezinha de Souza Wyse, A; Wajner, M | 1 |
| Besouw, MT; Levtchenko, EN; Noordam, K; Willemsen, MA | 1 |
| Bendel-Stenzel, MR; Dohil, R; Kim, Y; Steinke, J | 1 |
| Balog, JZ; Gahl, WA; Kleta, R | 1 |
| Gahl, W; Nesterova, G | 1 |
| Athaydes, GA; Dornelles, PK; Feksa, LR; Fleck, RM; Rech, VC; Rodrigues-Junior, V; Wannmacher, CM | 1 |
| Levtchenko, EN; van den Heuvel, LP; Wilmer, MJ | 1 |
| Borowitz, MJ; Burns, KH; Confer, B; Emadi, A; Streiff, MB | 1 |
| Segal, S; States, B | 2 |
| Dumoulin, R; Frederich, A; Guibaud, P; Louis, JJ; Zabot, MT | 1 |
| Baltassat, P; Bureau, J; Dumoulin, R; Frederich, A; Guibaud, P; Larbre, F; Louis, JJ; Parchoux, B; Zabot, MT | 1 |
| Schulman, JD; Steinherz, R; Tietze, F | 1 |
| Harms, E; Oude Elferink, RP; Strijland, A; Tager, JM | 1 |
| Gahl, WA; Schneider, J; Schulman, JD; Thoene, JG | 1 |
| Jonas, AJ; Pellett, OL; Schneider, JA; Smith, ML; Thoene, JG | 1 |
| Gahl, WA; Hubbard, VS; Orloff, S | 1 |
| Chiummariello, R; D'Eufemia, P; Martino, F; Ruberto, U | 1 |
| Lee, J; Segal, S; States, B | 2 |
| Avner, ED; Ellis, D; Jaffe, R | 1 |
| Cantani, A; Ciarnella Cantani, A; Giardini, O | 1 |
| Bergonzi, E; Bühlmann, C; Herren, A; Lavanchy, P; Lüthy, C; Oetliker, O; Wyss, SR | 1 |
| Jonas, AJ; Schneider, JA | 1 |
| Butler, JD; Mukherjee, AB; Orloff, S; Schulman, JD; Towne, D | 1 |
| Nissim, I; Schneider, A; Segal, S; Yudkoff, M | 1 |
| Corden, BJ; Schneider, JA; Schulman, JD; Thoene, JG | 1 |
| Lemons, R; Thoene, JG | 1 |
| Foreman, JW; Segal, S; Yudkoff, M | 1 |
| Berry, KA; Clark, KF; Gahl, WA; Greene, AA; Markello, TC; Noonan, PK; Reisch, JS; Schneider, JA; Thoene, JG | 1 |
| Briones, P; Camacho, JA; Farré, C; Mas, A; Vilaseca, MA | 1 |
| Gahl, WA; Kimonis, VE; Markello, TC; Rose, SR; Troendle, J; Yang, ML | 1 |
| Gräf, M; Kalinowski, HO | 1 |
| Gretz, N; Manz, F | 1 |
| Gahl, WA; Kaiser-Kupfer, MI; Krasnewich, D; Theodoropoulos, DS | 1 |
| Bernardini, IM; Gahl, WA; Markello, TC | 1 |
| Bernardini, I; Gahl, WA; Hyman, PE; Ingelfinger, J; Mohan, P; Tangerman, A | 1 |
| Ohno, K; Yano, T | 1 |
| Gretz, N; van't Hoff, WG | 1 |
| Berthélémé, JP; Broyer, M; Guest, G; Labrousse, F; Poisson, M; Tête, MJ | 1 |
| Bianchetti, MG; Kernland, K; Lüthy, CM; Wermuth, B | 1 |
| Murphy, JL; Wenner, WJ | 1 |
| Broyer, M; Gretz, N; Haffner, D; Mehls, O; Offner, G; van't Hoff, WG; Wühl, E | 1 |
| Dionisi-Vici, C; Federici, G; Greco, M; Pastore, A; Ribes, A; Rizzo, C; Rizzoni, G | 1 |
| Blom, H; de Graaf-Hess, A; Trijbels, F | 1 |
| Brodehl, J; Offner, G; Schubert, M; Vester, U | 1 |
| Ohno, K | 1 |
| Blom, HJ; de Graaf-Hess, A; Levtchenko, EN; Monnens, LA | 1 |
| Butler, JD; Pellefigue, F; Schulman, JD; Spielberg, SP; Tietze, F | 1 |
| Pollard, AC; Roy, LP | 1 |
| Humphrey, C | 1 |
| Crawhall, JC; Oshima, RG; Schneider, JA; Thoene, JG | 1 |
| Crocker, JF; DeWolfe, MS; Girardin, EP | 1 |
| Schulman, JD | 1 |
| Crawhall, JC; Hsiung, M; Itiaba, K; Yeo, YY | 1 |
| Crawhall, JC; Olson, DL; Oshima, RG; Schneider, JA; Thoene, JG | 1 |
| Kamoshita, S; Watanabe, H | 1 |
| Bernardini, I; Charnas, L; Dalakas, MC; Gahl, WA; Ishak, KG; Markello, TC | 1 |
| Ayadi, A; Besbes, A; Hammami, M; Pousse, H; Sfar, MT; Slimane, MN | 1 |
| Gräf, M; Grote, A; Wagner, F | 1 |
| Baker, T; Chantler, C; Dalton, RN; Duke, LC; Haycock, GB; Smith, SP; van't Hoff, WG | 1 |
| Broyer, M; Ged, C; Jean, G; Kamoun, P; Tete, MJ | 1 |
| Bradbury, JA; Brocklebank, T; Danjoux, JP; Spencer, M; Voller, J | 1 |
| Adamson, M; Adelman, RD; Clark, KF; Gahl, WA; Murphy, JL; Reznik, VM; Schneider, JA | 1 |
| Cantani, A; D'Eufemia, P; Giardini, O | 1 |
| Strife, CF; Strife, JL; Wacksman, J | 1 |
| Milot, J; O'Regan, S; Quigley, M; Richler, M | 1 |
| Jones, NP; Noble, JL; Postlethwaite, RJ | 1 |
| Berger, R; Bijsterveld, K; Chapman, TE; Reijngoud, DJ; Schierbeek, H; van Luijk, WH | 1 |
| Caruso, RC; Datiles, MB; Gahl, WA; Gazzo, MA; Kaiser-Kupfer, MI; Kuehl, EM | 1 |
| Broyer, M; Tete, MJ | 1 |
| Forster, S; Lloyd, JB; Scarlett, L | 1 |
| Adamson, MD; Andersson, HC; Ekman, EF; Gahl, WA; Kaler, SG; Markello, TC; Sonies, BC | 1 |
| Butler, JD | 1 |
| Gahl, WA; Reed, GF; Schneider, JA; Schulman, JD; Thoene, JG | 1 |
| Raub, W | 1 |
| Foreman, JW | 1 |
| Butler, JD; Gahl, WA; Tietze, F | 1 |
| Gahl, WA; Kaiser-Kupfer, MI; Kuwabara, T; O'Regan, S; Schneider, JA; Thoene, JG | 1 |
| Bernar, J; Bernardini, I; Dalakas, M; Gahl, WA; Harper, GS; Hoeg, JM; Hurko, O; Rizzo, WB | 1 |
| Clark, KF; Gahl, WA; Schneider, JA; Smolin, LA; Thoene, JG | 1 |
| Caruso, RC; Gahl, WA; Kaiser-Kupfer, MI; Minkler, DS | 1 |
| Corden, BJ; Denman, DW; Gahl, WA; Jonas, AJ; Reed, GF; Rizzo, WB; Schlesselman, JJ; Schneider, JA; Schulman, JD; Thoene, JG | 1 |
| Baten, E; Bruyneel, P; Hoogmartens, J; Proesmans, W | 1 |
| Broyer, M; Dhermy, P; Dufier, JL; Gagnadoux, MF; Gubler, MC | 1 |
| Bernardini, I; Gahl, WA; Harper, GS; Hurko, O; Zuurveld, J | 1 |
| Fujikawa, L; Gahl, WA; Jain, S; Kaiser-Kupfer, MI; Kuwabara, T | 1 |
| Bercu, BB; Gahl, WA | 1 |
| Kooistra, T; Lloyd, JB | 1 |
| Christensen, HN; Pisoni, RL; Thoene, JG | 1 |
| Danpure, CJ; Fyfe, DA; Jennings, PR | 1 |
| Fisher, E; Gahl, WA; Gregg, RE; Hoeg, JM | 1 |
| Butler, JD; Gahl, WA; Schulman, JD; Tietze, F | 1 |
| Butler, JD; Gahl, WA; Thoene, JG; Wittwer, CT; Zatz, M | 1 |
| Blumberg, A; da Silva, VA; Lavanchy, P; Lüthy, CM; Oetliker, OH; Suter, H; Wyss, SR; Zurbrügg, RP | 1 |
| Bump, EA; Greene, AA; Harms, E; Jonas, AJ; Miller, AL; Pellett, OL; Schneider, JA; Smith, ML | 1 |
33 review(s) available for cysteamine and Cystinosis
| Article | Year |
|---|---|
Fertility in Cystinosis.
Topics: Animals; Biomarkers; Cysteamine; Cystinosis; Disease Models, Animal; Fertility; Humans; Models, Biological | 2021 |
Programmed Cell Death in Cystinosis.
Topics: Apoptosis; Autophagy; Cysteamine; Cystinosis; Humans; Kidney Transplantation | 2022 |
Central Nervous System Complications in Cystinosis: The Role of Neuroimaging.
Topics: Adult; Central Nervous System Diseases; Child; Cysteamine; Cystine; Cystinosis; Diffusion Tensor Imaging; Gray Matter; Humans | 2022 |
Newborn Screening: Review of its Impact for Cystinosis.
Topics: Adult; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant, Newborn; Neonatal Screening; Pilot Projects | 2022 |
Biomarkers in Nephropathic Cystinosis: Current and Future Perspectives.
Topics: Biomarkers; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Quality of Life | 2022 |
Adult complications of nephropathic cystinosis: a systematic review.
Topics: Adult; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Kidney Failure, Chronic; Kidney Transplantation | 2021 |
Topics: Child; Child, Preschool; Consumer Organizations; Cysteamine; Cystinosis; Drug Industry; France; Humans; Rare Diseases; Universities | 2020 |
Nephropathic cystinosis: an update on genetic conditioning.
Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Mutation | 2021 |
Efficacy and Safety of Topical Cysteamine in Corneal Cystinosis: A Systematic Review and Meta-Analysis.
Topics: Corneal Diseases; Cysteamine; Cystine Depleting Agents; Cystinosis; Humans; Ophthalmic Solutions; Visual Acuity | 2021 |
Cysteamine revisited: repair of arginine to cysteine mutations.
Topics: Animals; Apolipoprotein E3; Arginine; Argininosuccinate Lyase; Cystathionine beta-Synthase; Cysteamine; Cysteine; Cystinosis; Homeostasis; Humans; Hydrogen-Ion Concentration; Molecular Conformation; Mutation; Mutation, Missense; Oxidation-Reduction; Sulfhydryl Compounds; Thromboplastin | 2017 |
A systematic literature review of cysteamine bitartrate in the treatment of nephropathic cystinosis.
Topics: Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Randomized Controlled Trials as Topic | 2017 |
Effects of long-term cysteamine treatment in patients with cystinosis.
Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystine Depleting Agents; Cystinosis; Disease Progression; Drug Administration Schedule; Genetic Predisposition to Disease; Humans; Mutation; Time Factors; Treatment Outcome | 2019 |
[Cystinosis : Diagnosis, cystine-depleting therapy, and transition].
Topics: Adult; Child; Child, Preschool; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Humans; Kidney; Lysosomes; Male; Quality of Life | 2018 |
Cystinosis: clinical presentation, pathogenesis and treatment.
Topics: Cysteamine; Cystinosis; Humans; Treatment Outcome | 2014 |
Cystinosis: a new perspective.
Topics: Child; Child, Preschool; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant; Kidney Transplantation | 2016 |
[Cystinosis in adults: A systemic disease].
Topics: Adolescent; Adult; Cysteamine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Humans; Kidney Failure, Chronic; Kidney Transplantation; Renal Insufficiency | 2015 |
Nephropathic cystinosis: an update.
Topics: Cysteamine; Cystinosis; Female; Forecasting; Genetic Therapy; Humans; Infant; Infant, Newborn; Male; Nephrotic Syndrome; Prognosis; Risk Assessment; Severity of Illness Index; Stem Cell Transplantation; Treatment Outcome | 2017 |
Cystinosis and lupus erythematosus: coincidence or causation.
Topics: Child; Cysteamine; Cystinosis; Follow-Up Studies; Humans; Lupus Erythematosus, Systemic; Lupus Nephritis; Male | 2010 |
Cystinosis: practical tools for diagnosis and treatment.
Topics: Cysteamine; Cystinosis; Diagnosis, Differential; Humans; Kidney | 2011 |
[Improved prognosis of cystinosis achieved by treatment with cysteamine and by kidney transplantation].
Topics: Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Kidney Failure, Chronic; Kidney Transplantation; Prognosis; Treatment Outcome | 2011 |
Cystinosis.
Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Glycoproteins; Humans; Membrane Proteins; Membrane Transport Proteins; Mutation | 2002 |
Early oral cysteamine therapy for nephropathic cystinosis.
Topics: Administration, Oral; Cysteamine; Cystinosis; Humans; Infant; Infant, Newborn; Neonatal Screening; Radiation-Protective Agents | 2003 |
[From gene to disease: cystinosis].
Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Gene Deletion; Glycoproteins; Humans; Membrane Proteins; Membrane Transport Proteins; Renal Insufficiency | 2004 |
Pharmacological treatment of nephropathic cystinosis with cysteamine.
Topics: Administration, Oral; Adolescent; Adult; Child; Child, Preschool; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant | 2004 |
[Cystinosis--an orphan disease. Pathogenesis and current treatment].
Topics: Adolescent; Adult; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Humans; Infant; Infant, Newborn; Kidney Diseases; Rare Diseases; Renal Insufficiency | 2006 |
Nephropathic cystinosis: late complications of a multisystemic disease.
Topics: Administration, Oral; Adolescent; Adult; Child; Cysteamine; Cystinosis; Disease Progression; Fanconi Syndrome; Genetic Predisposition to Disease; Humans; Kidney Failure, Chronic; Kidney Transplantation; Life Expectancy; Male; Middle Aged; Severity of Illness Index; Treatment Outcome | 2008 |
Genetic disorders of glutathione and sulfur amino-acid metabolism. New biochemical insights and therapeutic approaches.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Sulfur; Ascorbic Acid; Cysteamine; Cystinosis; Erythrocytes; Glucosephosphate Dehydrogenase Deficiency; Glutathione; Glutathione Synthase; Homocystinuria; Humans; Leukocytes; Vitamin E | 1980 |
Recent advances in the treatment of cystinosis.
Topics: Adolescent; Child; Child, Preschool; Cysteamine; Cystinosis; Humans | 1995 |
[Lysosomal membrane transport disorders--cystinosis and sialic acid storage disorders (Salla disease, ISSD)].
Topics: Biological Transport; Cysteamine; Cystine; Cystinosis; Humans; Lysosomal Storage Diseases; N-Acetylneuraminic Acid; Sialic Acids | 1995 |
[Cystinosis].
Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystinosis; Diagnosis, Differential; Gene Deletion; Genes, Recessive; Glycoproteins; Humans; Membrane Proteins; Membrane Transport Proteins; Nervous System Diseases | 2000 |
Cystine depletion of cystinotic cells by aminothiols.
Topics: Cell-Free System; Cystamine; Cysteamine; Cysteine; Cystinosis; Fibroblasts; Humans; Penicillamine; Sulfhydryl Compounds | 1977 |
Cystinosis.
Topics: Cysteamine; Cystine; Cystinosis; Humans; Kidney | 1989 |
NIH conference. Cystinosis: progress in a prototypic disease.
Topics: Adolescent; Adult; Biological Transport; Child; Child, Preschool; Crystallization; Cystaphos; Cysteamine; Cystine; Cystinosis; Eye Diseases; Female; Growth; Humans; Kidney Transplantation; Male; Ophthalmic Solutions; Prodrugs; Quebec | 1988 |
21 trial(s) available for cysteamine and Cystinosis
| Article | Year |
|---|---|
A novel sustained-release cysteamine bitartrate formulation for the treatment of cystinosis: Pharmacokinetics and safety in healthy male volunteers.
Topics: Adult; Area Under Curve; Cross-Over Studies; Cysteamine; Cystine Depleting Agents; Cystinosis; Delayed-Action Preparations; Healthy Volunteers; Humans; Male; Netherlands; Young Adult | 2021 |
A New Viscous Cysteamine Eye Drops Treatment for Ophthalmic Cystinosis: An Open-Label Randomized Comparative Phase III Pivotal Study.
Topics: Adolescent; Cornea; Corneal Diseases; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Male; Microscopy, Confocal; Ophthalmic Solutions; Retrospective Studies; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity; Young Adult | 2017 |
A Phase 1 Pharmacokinetic Study of Cysteamine Bitartrate Delayed-Release Capsules Following Oral Administration with Orange Juice, Water, or Omeprazole in Cystinosis.
Topics: Administration, Oral; Adolescent; Adult; Citrus sinensis; Cysteamine; Cystine Depleting Agents; Cystinosis; Delayed-Action Preparations; Drug Interactions; Europe; Female; Fruit and Vegetable Juices; Humans; Male; Middle Aged; Omeprazole; Proton Pump Inhibitors; Water; Young Adult | 2018 |
Teenagers and young adults with nephropathic cystinosis display significant bone disease and cortical impairment.
Topics: Absorptiometry, Photon; Adolescent; Adult; Age Factors; Biomarkers; Bone Density; Bone Diseases; Child; Cortical Bone; Cysteamine; Cystinosis; Female; Fibroblast Growth Factor-23; Humans; Male; Pilot Projects; Prevalence; Prospective Studies; Quality of Life; Radius; Severity of Illness Index; Tibia; Tomography, X-Ray Computed; Young Adult | 2018 |
N-acetyl-cysteine is associated to renal function improvement in patients with nephropathic cystinosis.
Topics: Acetylcysteine; Antioxidants; Child; Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Humans; Kidney Function Tests; Male; Oxidative Stress | 2014 |
Quality of life is improved and kidney function preserved in patients with nephropathic cystinosis treated for 2 years with delayed-release cysteamine bitartrate.
Topics: Child; Cysteamine; Cystine Depleting Agents; Cystinosis; Delayed-Action Preparations; Female; Glomerular Filtration Rate; Humans; Male; Prospective Studies; Quality of Life; Time Factors | 2014 |
Population pharmacokinetics and pharmacodynamics of cysteamine in nephropathic cystinosis patients.
Topics: Adolescent; Adult; Body Weight; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Fanconi Syndrome; Female; Humans; Infant; Male; Nephrotic Syndrome; Radiation-Protective Agents; Treatment Outcome; Young Adult | 2011 |
A randomized controlled crossover trial with delayed-release cysteamine bitartrate in nephropathic cystinosis: effectiveness on white blood cell cystine levels and comparison of safety.
Topics: Adolescent; Adult; Child; Cross-Over Studies; Cysteamine; Cystine; Cystinosis; Delayed-Action Preparations; Fanconi Syndrome; Female; Humans; Leukocytes; Male; Nephrotic Syndrome; Young Adult | 2012 |
A multicentre randomised double masked clinical trial of a new formulation of topical cysteamine for the treatment of corneal cystine crystals in cystinosis.
Topics: Administration, Topical; Adolescent; Adult; Child; Child, Preschool; Corneal Diseases; Cysteamine; Cystinosis; Double-Blind Method; Female; Humans; Male; Prospective Studies; Radiation-Protective Agents; Treatment Outcome | 2003 |
Steady-state pharmacokinetics and pharmacodynamics of cysteamine bitartrate in paediatric nephropathic cystinosis patients.
Topics: Adolescent; Child; Child, Preschool; Cysteamine; Cystinosis; Female; Humans; Male; Radiation-Protective Agents | 2003 |
Evaluation of specific therapies for cystinosis.
Topics: Clinical Trials as Topic; Creatinine; Cysteamine; Cystinosis; Humans | 1983 |
Cysteamine treatment of nephropathic cystinosis.
Topics: Clinical Trials as Topic; Cysteamine; Cystinosis; Humans | 1983 |
Effects of early cysteamine therapy on thyroid function and growth in nephropathic cystinosis.
Topics: Adolescent; Adult; Age Determination by Skeleton; Child; Child, Preschool; Cysteamine; Cystinosis; Humans; Infant; Retrospective Studies; Thyroid Gland; Thyroxine; Time Factors | 1995 |
Clinical polymorphism of cystinosis encephalopathy. Results of treatment with cysteamine.
Topics: Adolescent; Adult; Atrophy; Brain; Brain Diseases; Child; Cysteamine; Cystinosis; Female; Humans; Kidney Transplantation; Magnetic Resonance Imaging; Male; Polymorphism, Genetic | 1996 |
The effects of cysteamine on the upper gastrointestinal tract of children with cystinosis.
Topics: Adolescent; Child; Child, Preschool; Cysteamine; Cystinosis; Duodenum; Endoscopy, Gastrointestinal; Female; Gastric Acid; Gastric Juice; Gastric Mucosa; Gastrins; Humans; Infant; Intestinal Mucosa; Male | 1997 |
Treatment with recombinant human growth hormone in short children with nephropathic cystinosis: no evidence for increased deterioration rate of renal function. The European Study Group on Growth Hormone Treatment in Short Children with Nephropathic Cystin
Topics: Child; Creatinine; Cysteamine; Cystinosis; Female; Glomerular Filtration Rate; Growth Disorders; Human Growth Hormone; Humans; Kidney; Kidney Function Tests; Male; Recombinant Proteins | 1998 |
Distal myopathy in nephropathic cystinosis.
Topics: Adolescent; Adult; Child; Cysteamine; Cystinosis; Electromyography; Female; Hand; Humans; Kidney Failure, Chronic; Male; Muscle Weakness; Muscular Diseases; Neural Conduction | 2000 |
A randomised placebo-controlled trial of topical cysteamine therapy in patients with nephropathic cystinosis.
Topics: Adolescent; Child; Contrast Sensitivity; Cornea; Cysteamine; Cystine; Cystinosis; Double-Blind Method; Female; Humans; Kidney Failure, Chronic; Male; Ophthalmic Solutions; Visual Acuity | 1991 |
A randomized placebo-controlled trial of cysteamine eye drops in nephropathic cystinosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Corneal Diseases; Crystallization; Cysteamine; Cystinosis; Double-Blind Method; Female; Humans; Infant; Male; Ophthalmic Solutions; Randomized Controlled Trials as Topic | 1990 |
Cysteamine therapy for children with nephropathic cystinosis.
Topics: Administration, Oral; Child; Child, Preschool; Clinical Trials as Topic; Cysteamine; Cystine; Cystinosis; Growth; Humans; Kidney Diseases; Kidney Glomerulus; Kidney Tubules | 1987 |
Removal of corneal crystals by topical cysteamine in nephropathic cystinosis.
Topics: Administration, Topical; Adolescent; Age Factors; Animals; Corneal Diseases; Crystallization; Cysteamine; Cystinosis; Female; Humans; In Vitro Techniques; Infant; Kidney Diseases; Male; Ophthalmic Solutions; Rabbits | 1987 |
220 other study(ies) available for cysteamine and Cystinosis
| Article | Year |
|---|---|
A potential new prodrug for the treatment of cystinosis: design, synthesis and in-vitro evaluation.
Topics: Cells, Cultured; Cysteamine; Cystinosis; Epithelial Cells; Fibroblasts; Humans; Molecular Structure; Prodrugs | 2008 |
PEGylated derivatives of cystamine as enhanced treatments for nephropathic cystinosis.
Topics: Cells, Cultured; Cystamine; Cystinosis; Fanconi Syndrome; Fibroblasts; Humans; Nephrotic Syndrome; Polyethylene Glycols; Prodrugs | 2011 |
Folate pro-drug of cystamine as an enhanced treatment for nephropathic cystinosis.
Topics: Administration, Oral; Cell Line; Cystamine; Cystinosis; Fanconi Syndrome; Humans; Nephrotic Syndrome; Prodrugs | 2011 |
Synthesis and in vitro evaluation of novel pro-drugs for the treatment of nephropathic cystinosis.
Topics: Cystamine; Cystinosis; Fanconi Syndrome; Fibroblasts; Glutarates; Humans; Nephrotic Syndrome; Prodrugs; Succinic Acid | 2011 |
Human and animal fertility studies in cystinosis reveal signs of obstructive azoospermia, an altered blood-testis barrier and a subtherapeutic effect of cysteamine in testis.
Topics: Adult; Animals; Azoospermia; Blood-Testis Barrier; Cysteamine; Cystine Depleting Agents; Cystinosis; Disease Models, Animal; Extracellular Matrix Proteins; Humans; Infertility, Male; Male; Mice; Mice, Inbred C57BL; Middle Aged; Neoplasm Proteins; Retrospective Studies; Testis; Young Adult; Zonula Occludens-1 Protein | 2021 |
A comparison of immediate release and delayed release cysteamine in 17 patients with nephropathic cystinosis.
Topics: Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Quality of Life; Retrospective Studies | 2021 |
Spectral domain optical coherence tomography-based retinochoroidal cystine crystal score: a window into infantile nephropathic cystinosis.
Topics: Adolescent; Adult; Child; Cornea; Cross-Sectional Studies; Cysteamine; Cystine; Cystinosis; Female; Humans; Male; Tomography, Optical Coherence; Young Adult | 2023 |
Response to Cysteamine in Osteoclasts Obtained from Patients with Nephropathic Cystinosis: A Genotype/Phenotype Correlation.
Topics: Adolescent; Adult; Amino Acid Transport Systems, Neutral; Cell Differentiation; Child; Child, Preschool; Cysteamine; Cystinosis; Female; Genetic Association Studies; Genotype; Humans; Leukocytes, Mononuclear; Male; Middle Aged; Mutation; Osteoclasts; Phenotype | 2021 |
Clinical and neurophysiological characterization of early neuromuscular involvement in children and adolescents with nephropathic cystinosis.
Topics: Adolescent; Child; Cohort Studies; Cysteamine; Cystinosis; Fanconi Syndrome; Female; Humans; Male; Muscular Diseases | 2022 |
Neuromuscular conditions and the impact of cystine-depleting therapy in infantile nephropathic cystinosis: A cross-sectional analysis of 55 patients.
Topics: Adult; Cross-Sectional Studies; Cysteamine; Cystine; Cystinosis; Humans; Neuromuscular Diseases | 2022 |
Body growth, upper arm fat area, and clinical parameters in children with nephropathic cystinosis compared with other pediatric chronic kidney disease entities.
Topics: Adipose Tissue; Adolescent; Arm; Child; Child, Preschool; Cysteamine; Cystinosis; Fanconi Syndrome; Female; Growth Hormone; Humans; Male; Prospective Studies; Renal Insufficiency, Chronic | 2022 |
Analysis of tear film in cystinosis patients treated with topical viscous cysteamine hydrochloride (Cystadrops
Topics: Cornea; Cysteamine; Cystinosis; Dry Eye Syndromes; Humans; Osmolar Concentration; Reproducibility of Results; Tears | 2022 |
Ocular treatment of cystinosis with eye drop containing cysteamine
Topics: Cornea; Corneal Diseases; Cysteamine; Cystine; Cystinosis; Humans; Photophobia; Quality of Life; Visual Acuity | 2022 |
Evaluation of NACA and diNACA in human cystinosis fibroblast cell cultures as potential treatments for cystinosis.
Topics: Cell Culture Techniques; Cysteamine; Cysteine; Cystine; Cystinosis; Fibroblasts; Humans | 2022 |
Importance of adherence to topical cysteamine in infantile ocular cystinosis: An illustrative case.
Topics: Cysteamine; Cystinosis; Eye; Face; Humans | 2022 |
Ocular biodistribution of cysteamine delivered by a sustained release microsphere/thermoresponsive gel eyedrop.
Topics: Animals; Chromatography, Liquid; Cornea; Cysteamine; Cystinosis; Delayed-Action Preparations; Microspheres; Ophthalmic Solutions; Rabbits; Tandem Mass Spectrometry; Tissue Distribution | 2022 |
Relationship between age at initiation of cysteamine treatment, adherence with therapy, and glomerular kidney function in infantile nephropathic cystinosis.
Topics: Child; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant; Infant, Newborn; Kidney | 2022 |
Beneficial effects of starting oral cysteamine treatment in the first 2 months of life on glomerular and tubular kidney function in infantile nephropathic cystinosis.
Topics: Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Infant; Infant, Newborn; Kidney | 2022 |
Nephropathic cystinosis in Poland: a 40-year retrospective study.
Topics: Adult; Child; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Kidney Failure, Chronic; Poland; Retrospective Studies; Young Adult | 2022 |
Outcome of infantile nephropathic cystinosis depends on early intervention, not genotype: A multicenter sibling cohort study.
Topics: Adolescent; Adult; Child; Child, Preschool; Cohort Studies; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant, Newborn; Kidney Failure, Chronic; Retrospective Studies; Siblings; Young Adult | 2023 |
Genistein improves renal disease in a mouse model of nephropathic cystinosis: a comparison study with cysteamine.
Topics: Animals; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Female; Genistein; Kidney; Kidney Diseases; Mice | 2023 |
A new proof of evidence of cysteamine quantification for therapeutic drug monitoring in patients with cystinosis.
Topics: Cysteamine; Cystine; Cystinosis; Drug Monitoring; Humans | 2022 |
The TGM2 inhibitor cysteamine hydrochloride does not impact corneal epithelial and stromal wound healing in vitro and in vivo.
Topics: Animals; Cicatrix; Cornea; Corneal Diseases; Corneal Injuries; Cysteamine; Cystinosis; Epithelium, Corneal; Protein Glutamine gamma Glutamyltransferase 2; Rabbits; Wound Healing | 2023 |
The Pitfall of White Blood Cell Cystine Measurement to Diagnose Juvenile Cystinosis.
Topics: Adolescent; Adult; Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Humans; Leukocytes | 2023 |
Cystinosis.
Topics: Cysteamine; Cystine; Cystinosis; Humans; Mutation | 2023 |
The effects of transitioning from immediate release to extended release cysteamine therapy in Norwegian patients with nephropathic cystinosis: a retrospective study.
Topics: Adult; Child; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Retrospective Studies | 2023 |
Long-term clinical benefits of delayed-release cysteamine bitartrate capsules in patients with nephropathic cystinosis (response to "A comparison of immediate release and delayed release cysteamine in 17 patients with nephropathic cystinosis").
Topics: Capsules; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Retrospective Studies | 2023 |
Cystinosis: Status of research and treatment in India and the world.
Topics: Cysteamine; Cystine; Cystinosis; Humans; India; Mutation | 2023 |
Impact of compliance to oral cysteamine treatment on the costs of Kidney failure in patients with nephropathic cystinosis in the United Kingdom.
Topics: Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Patient Compliance; Renal Dialysis; Renal Insufficiency; United Kingdom | 2023 |
Central nervous system complications in adult cystinosis patients.
Topics: Adolescent; Adult; Aged; Case-Control Studies; Central Nervous System Diseases; Cerebrovascular Circulation; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Female; Gray Matter; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Young Adult | 2020 |
Short stature: making a crystal clear diagnosis.
Topics: Child, Preschool; Cysteamine; Cystine Depleting Agents; Cystinosis; Diagnosis, Differential; Dwarfism; Female; Humans; Prognosis; Rickets | 2019 |
CTNS mRNA molecular analysis revealed a novel mutation in a child with infantile nephropathic cystinosis: a case report.
Topics: Amino Acid Transport Systems, Neutral; Child, Preschool; Consanguinity; Cysteamine; Cystine Depleting Agents; Cystinosis; DNA Mutational Analysis; Exons; Fibroblasts; Humans; Infant; Introns; Male; Mutation; Real-Time Polymerase Chain Reaction; RNA, Messenger | 2019 |
Use of Human Induced Pluripotent Stem Cells and Kidney Organoids To Develop a Cysteamine/mTOR Inhibition Combination Therapy for Cystinosis.
Topics: Amino Acid Transport Systems, Neutral; Animals; Autophagy; Cell Line; CRISPR-Cas Systems; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Drug Evaluation, Preclinical; Drug Therapy, Combination; Everolimus; Gene Editing; Heterografts; Humans; Induced Pluripotent Stem Cells; Lysosomes; Mice; Mice, SCID; Organoids; Phenotype; TOR Serine-Threonine Kinases | 2020 |
An Infant With Vomiting, Constipation, and Weight Loss.
Topics: Calcitriol; Citric Acid; Constipation; Cysteamine; Cystinosis; Diagnosis, Differential; Fanconi Syndrome; Humans; Infant; Male; Phosphorus; Potassium; Sodium; Sodium Citrate; Vomiting; Weight Loss | 2020 |
Chitotriosidase as a Novel Biomarker for Therapeutic Monitoring of Nephropathic Cystinosis.
Topics: Adolescent; Adult; Biomarkers; Child; Cysteamine; Cystine; Cystinosis; Drug Monitoring; Female; Hexosaminidases; Humans; Inflammation; Interleukin-18; Interleukin-1beta; Interleukin-6; Leukocytes; Macrophage Activation; Male; Medication Adherence; Peptide Fragments; Prospective Studies; Young Adult | 2020 |
Ruptured gallbladder in cystinosis renal transplant recipient.
Topics: Cysteamine; Cystinosis; Gallbladder; Humans; Kidney; Kidney Transplantation | 2020 |
Long-term follow-up of cystinosis patients treated with 0.55% cysteamine hydrochloride.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Cornea; Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Follow-Up Studies; Humans; Male; Middle Aged; Ophthalmic Solutions; Retrospective Studies; Time Factors; Visual Acuity; Young Adult | 2021 |
Cystinosis beyond kidneys: gastrointestinal system and muscle involvement.
Topics: Child; Cysteamine; Cystine; Cystinosis; Humans; Infant; Kidney; Muscles | 2020 |
Adherence to cysteamine in nephropathic cystinosis: A unique electronic monitoring experience for a better understanding. A prospective cohort study: CrYSTobs.
Topics: Adolescent; Adult; Child; Child, Preschool; Cysteamine; Cystinosis; Electronics; Fanconi Syndrome; Female; Humans; Male; Prospective Studies; Young Adult | 2021 |
A sustained release cysteamine microsphere/thermoresponsive gel eyedrop for corneal cystinosis improves drug stability.
Topics: Cornea; Cysteamine; Cystinosis; Delayed-Action Preparations; Drug Stability; Humans; Microspheres; Ophthalmic Solutions | 2021 |
Cysteamine-bicalutamide combination therapy corrects proximal tubule phenotype in cystinosis.
Topics: Amino Acid Transport Systems, Neutral; Anilides; Animals; Cysteamine; Cystinosis; Humans; Nitriles; Phenotype; Tosyl Compounds; Zebrafish | 2021 |
An international cohort study spanning five decades assessed outcomes of nephropathic cystinosis.
Topics: Adult; Child, Preschool; Cohort Studies; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Humans | 2021 |
Neurocognitive Complications of Cystinosis.
Topics: Adult; Brain; Child; Child, Preschool; Cysteamine; Cystinosis; Humans; Neurocognitive Disorders | 2017 |
Ocular Complications of Infantile Nephropathic Cystinosis.
Topics: Cysteamine; Cystinosis; Eye; Eye Diseases; Humans; Infant | 2017 |
Myopathy and Less Frequent Complications of Cystinosis.
Topics: Cardiovascular Diseases; Cysteamine; Cystinosis; Gastrointestinal Diseases; Humans; Muscular Diseases | 2017 |
Cysteamine polysaccharide hydrogels: Study of extended ocular delivery and biopermanence time by PET imaging.
Topics: Administration, Ophthalmic; Animals; Carrageenan; Cattle; Cells, Cultured; Corneal Keratocytes; Cysteamine; Cystinosis; Delayed-Action Preparations; Drug Carriers; Humans; Hydrogels; Male; Polysaccharides, Bacterial; Positron Emission Tomography Computed Tomography; Rats; Rats, Sprague-Dawley | 2017 |
Diagnostic challenge in a patient with nephropathic juvenile cystinosis: a case report.
Topics: Child; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Male; Nephrotic Syndrome; Slit Lamp Microscopy | 2017 |
[Cysteamine ophthalmic hydrogel for the treatment of ocular cystinosis].
Topics: Administration, Topical; Chemistry, Pharmaceutical; Cysteamine; Cystinosis; Eye Diseases; Humans; Hydrogel, Polyethylene Glycol Dimethacrylate; Ophthalmic Solutions | 2017 |
In vitro drug release and in vivo safety of vitamin E and cysteamine loaded contact lenses.
Topics: Animals; Contact Lenses, Hydrophilic; Cornea; Cysteamine; Cystine Depleting Agents; Cystinosis; Diffusion; Drug Delivery Systems; Drug Liberation; Drug Stability; Female; Male; Models, Animal; Models, Biological; Oxidation-Reduction; Rabbits; Time Factors; Vitamin E | 2018 |
[Nephropatic cystinosis: report of one case].
Topics: Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Humans; Infant; Polymerase Chain Reaction; Prenatal Diagnosis | 2018 |
The effect of food and liquid pH on the integrity of enteric-coated beads from cysteamine bitartrate delayed-release capsules.
Topics: Capsules; Cysteamine; Cystinosis; Food; Fruit and Vegetable Juices; Humans; Hydrogen-Ion Concentration; Temperature | 2018 |
Access to Orphan Drugs is a Challenge for Sustainable Management of Cystinosis in China.
Topics: China; Cysteamine; Cystinosis; Humans; Male; Orphan Drug Production | 2018 |
Topics: Adult; Anterior Eye Segment; Cornea; Corneal Diseases; Crystallization; Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Follow-Up Studies; Humans; Microscopy, Confocal; Ophthalmic Solutions; Tomography, Optical Coherence; Visual Acuity | 2019 |
Carbon Black Tinted Contact Lenses for Reduction of Photophobia in Cystinosis Patients.
Topics: Animals; Antihypertensive Agents; Contact Lenses, Hydrophilic; Corneal Diseases; Cysteamine; Cystine Depleting Agents; Cystinosis; Drug Delivery Systems; Elastic Modulus; Microscopy, Electron, Scanning; Photophobia; Rabbits; Refractometry; Soot; Spectrophotometry, Ultraviolet; Timolol; Vitamin E; Vitamins | 2019 |
Swallowing dysfunction in patients with nephropathic cystinosis.
Topics: Adult; Cysteamine; Cystinosis; Deglutition; Deglutition Disorders; Female; Humans; Kidney Diseases; Male; Mastication; Middle Aged; Myotonic Dystrophy; Young Adult | 2019 |
Therapeutic Problems and Pregnancy in a Patient With Infantile Nephropathic Cystinosis: A Case Report.
Topics: Adult; Cesarean Section; Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Humans; Kidney Transplantation; Pregnancy; Pregnancy Complications; Pregnancy Outcome | 2019 |
Ocular manifestations of intermediate cystinosis: To treat or not to treat?
Topics: Adult; Cornea; Corneal Diseases; Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Follow-Up Studies; Humans; Microscopy, Confocal; Slit Lamp Microscopy; Time Factors; Tomography, Optical Coherence; Visual Acuity | 2020 |
Management of bone disease in cystinosis: Statement from an international conference.
Topics: Administration, Oral; Bone Diseases; Cysteamine; Cystinosis; Disease Management; Fanconi Syndrome; Female; Humans; Male | 2019 |
Cystinosis encephalopathy: MRI perivascular enhancement with micronodular T2* hypointensity.
Topics: Brain; Brain Diseases, Metabolic, Inborn; Cerebral Arteries; Child; Cysteamine; Cystinosis; Follow-Up Studies; Humans; Image Enhancement; Image Interpretation, Computer-Assisted; Immunosuppressive Agents; Kidney Transplantation; Magnetic Resonance Imaging; Male; Medication Adherence; Neurologic Examination | 2013 |
Copper deficiency in patients with cystinosis with cysteamine toxicity.
Topics: Adenosine Triphosphatases; Adolescent; Adult; Biomarkers; Cation Transport Proteins; Ceruloplasmin; Child; Child, Preschool; Collagen; Collagen Type I; Collagen Type I, alpha 1 Chain; Copper; Copper Transporter 1; Copper-Transporting ATPases; Cysteamine; Cystinosis; Fanconi Syndrome; Female; Genetic Markers; Humans; Male; Polymorphism, Genetic; Protective Agents; Protein-Lysine 6-Oxidase; Renal Agents; Sequence Analysis, DNA; Young Adult | 2013 |
Do comparable efficacy & convenient dosing justify Procysbi's extremely high price?
Topics: Cysteamine; Cystinosis; Delayed-Action Preparations; Humans; Lysosomal Storage Diseases | 2013 |
A new gel formulation of topical cysteamine for the treatment of corneal cystine crystals in cystinosis: the Cystadrops OCT-1 study.
Topics: Administration, Topical; Adolescent; Adult; Child; Cornea; Crystallization; Cysteamine; Cystine; Cystinosis; Female; Gels; Humans; Male; Ophthalmic Solutions; Young Adult | 2014 |
Nephropathic cystinosis--a gap between developing and developed nations.
Topics: Cysteamine; Cystinosis; Developed Countries; Developing Countries; Humans; Surveys and Questionnaires; Treatment Outcome | 2014 |
Patient and graft survival following kidney transplantation in recipients with cystinosis: a cohort study.
Topics: Adult; Australia; Cause of Death; Cohort Studies; Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Graft Survival; Humans; Kidney Failure, Chronic; Kidney Transplantation; Male; Nervous System Diseases; Patient Outcome Assessment; Survival Analysis; Vascular Diseases | 2015 |
Cysteamine (Cystagon®) adherence in patients with cystinosis in Spain: successful in children and a challenge in adolescents and adults.
Topics: Adolescent; Adult; Child; Child, Preschool; Cohort Studies; Cysteamine; Cystine Depleting Agents; Cystinosis; Female; Health Knowledge, Attitudes, Practice; Humans; Infant; Infant, Newborn; Male; Medication Adherence; Prognosis; Self Care; Spain; Young Adult | 2015 |
Cystinosis: renal glomerular and renal tubular function in relation to compliance with cystine-depleting therapy.
Topics: Administration, Oral; Adolescent; Adult; Age Factors; Biomarkers; Child; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Female; Humans; Kidney; Kidney Failure, Chronic; Kidney Function Tests; Leukocytes; Linear Models; Male; Maximum Tolerated Dose; Medication Adherence; Middle Aged; Retrospective Studies; Risk Factors; Time Factors; Treatment Outcome; Young Adult | 2015 |
Mood and illness experiences of adults with cystinosis.
Topics: Adolescent; Adult; Affect; Anxiety; Cysteamine; Cystine Depleting Agents; Cystinosis; Depression; Female; Humans; Male; Middle Aged; Psychiatric Status Rating Scales; Surveys and Questionnaires; Young Adult | 2015 |
Cysteamine treatment restores the in vitro ability to differentiate along the osteoblastic lineage of mesenchymal stromal cells isolated from bone marrow of a cystinotic patient.
Topics: Adolescent; Bone Marrow; Cell Culture Techniques; Cell Differentiation; Cell Lineage; Cell Proliferation; Child; Cysteamine; Cystinosis; Humans; Immunophenotyping; Leukocytes, Mononuclear; Mesenchymal Stem Cells; Osteoblasts; Young Adult | 2015 |
Management dilemmas in pediatric nephrology: Cystinosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cysteamine; Cystinosis; Disease Management; Fatal Outcome; Humans; Infant; Infant, Newborn; Kidney Transplantation; Male; Nephrology; Pediatrics; Pedigree; Renal Insufficiency; Young Adult | 2015 |
[Quality of life improves].
Topics: Administration, Oral; Child; Child, Preschool; Cysteamine; Cystinosis; Delayed-Action Preparations; Fanconi Syndrome; Humans; Microspheres; Quality of Life | 2015 |
Pancytopenia in a patient with cystinosis secondary to myelosuppression from cystine crystal deposition: a case report.
Topics: Adult; Bone Marrow; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Female; Flow Cytometry; Humans; Pancytopenia | 2015 |
Cysteamine in renal transplantation: A report of two patients with nephropathic cystinosis and the successful re-initiation of cysteamine therapy during the immediate post-transplant period.
Topics: Adolescent; Child; Corneal Diseases; Cysteamine; Cystinosis; Drug Administration Schedule; Fanconi Syndrome; Female; Humans; Immunosuppression Therapy; Immunosuppressive Agents; Inpatients; Kidney Transplantation; Male; Postoperative Period; Renal Insufficiency; Tacrolimus; Treatment Outcome | 2016 |
Cystinosis in adult and adolescent patients: Recommendations for the comprehensive care of cystinosis.
Topics: Adolescent; Adult; Amino Acid Transport Systems, Neutral; Comprehensive Health Care; Corneal Diseases; Cysteamine; Cystinosis; Disease Management; Early Diagnosis; Endocrine System Diseases; Genetic Counseling; Humans; Interdisciplinary Communication; Kidney Failure, Chronic; Kidney Transplantation; Nervous System Diseases; Patient Education as Topic; Quality of Life; Self Care; Transition to Adult Care | 2015 |
Synthesis of diacylated γ-glutamyl-cysteamine prodrugs, and in vitro evaluation of their cytotoxicity and intracellular delivery of cysteamine.
Topics: Cell Line; Cell Survival; Cysteamine; Cystinosis; Humans; Prodrugs | 2016 |
Altered mTOR signalling in nephropathic cystinosis.
Topics: Amino Acid Transport Systems, Neutral; Autophagy; Cells, Cultured; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Kidney Failure, Chronic; Kidney Tubules, Proximal; Lysosomes; Mechanistic Target of Rapamycin Complex 1; Signal Transduction | 2016 |
[Peculiarity of infantile cystinosis in Tunisian children].
Topics: Adolescent; Child; Child, Preschool; Cysteamine; Cystine Depleting Agents; Cystinosis; Disease Progression; Female; Humans; Infant; Kidney Failure, Chronic; Kidney Function Tests; Male; Retrospective Studies; Tunisia | 2015 |
An Open-Label Investigation of the Pharmacokinetics and Tolerability of Oral Cysteamine in Adults with Cystic Fibrosis.
Topics: Adult; Area Under Curve; Cysteamine; Cystic Fibrosis; Cystinosis; Female; Half-Life; Humans; Intestinal Absorption; Male; Sputum; Treatment Outcome; Young Adult | 2016 |
Controversies and research agenda in nephropathic cystinosis: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.
Topics: Adolescent; Adult; Age Factors; Amino Acid Transport Systems, Neutral; Child; Congresses as Topic; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Genetic Testing; Genetic Therapy; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppression Therapy; Infant; Kidney Failure, Chronic; Kidney Transplantation; Lysosomes; Mutation; Rare Diseases; Renal Dialysis | 2016 |
Switching from immediate- to extended-release cysteamine in nephropathic cystinosis patients: a retrospective real-life single-center study.
Topics: Adolescent; Child; Child, Preschool; Cohort Studies; Cysteamine; Cystine; Cystinosis; Delayed-Action Preparations; Drug Compounding; Female; Glomerular Filtration Rate; Humans; Infant; Leukocytes; Male; Renal Agents; Retrospective Studies; Treatment Outcome | 2017 |
Synergistic Cysteamine Delivery Nanowafer as an Efficacious Treatment Modality for Corneal Cystinosis.
Topics: Animals; Cornea; Cysteamine; Cystine; Cystinosis; Drug Delivery Systems; Female; Mass Spectrometry; Mice; Mice, Inbred C57BL; Ophthalmic Solutions; Treatment Outcome | 2016 |
Preformulation of cysteamine gels for treatment of the ophthalmic complications in cystinosis.
Topics: Acrylic Resins; Animals; Cattle; Cellulose; Chemistry, Pharmaceutical; Cornea; Cysteamine; Cystinosis; Drug Delivery Systems; Eye Diseases; Gels; Humans; Hyaluronic Acid; Ophthalmic Solutions; Polymers; Rheology; Solubility | 2016 |
Evaluation of carbohydrate-cysteamine thiazolidines as pro-drugs for the treatment of cystinosis.
Topics: Arabinose; Biotransformation; Cysteamine; Cystine Depleting Agents; Cystinosis; Drug Design; Extracellular Space; Fibroblasts; Glucose; Humans; Hydrolysis; Lactose; Molecular Structure; Primary Cell Culture; Prodrugs; Ribose; Sulfides; Thiazolidines | 2017 |
Conversion from immediate- to extended-release cysteamine may decrease disease control and increase additional side effects.
Topics: Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Retrospective Studies | 2017 |
Formulation and in vitro evaluation of cysteamine hydrochloride viscous solutions for the treatment of corneal cystinosis.
Topics: Administration, Topical; Adult; Animals; Benzalkonium Compounds; Chemistry, Pharmaceutical; Corneal Diseases; Cysteamine; Cystinosis; Drug Administration Schedule; Drug Stability; Excipients; Eye; Female; Hot Temperature; Humans; Hydrogen-Ion Concentration; Hypromellose Derivatives; Kinetics; Male; Methylcellulose; Ophthalmic Solutions; Orphan Drug Production; Osmolar Concentration; Preservatives, Pharmaceutical; Rabbits; Solubility; Sterilization; Toxicity Tests, Acute; Viscosity | 2008 |
Fertility status in male cystinosis patients treated with cysteamine.
Topics: Adult; Azoospermia; Cysteamine; Cystine; Cystinosis; Female; Fertility; Follicle Stimulating Hormone; Glomerular Filtration Rate; Humans; Luteinizing Hormone; Male; Organ Size; Pregnancy; Semen Analysis; Testis; Testosterone; Young Adult | 2010 |
Drug-induced lupus and antiphospholipid syndrome associated with cysteamine therapy.
Topics: Adolescent; Antibodies, Antinuclear; Antiphospholipid Syndrome; Blood Sedimentation; Cysteamine; Cystinosis; Female; Humans; Kidney Transplantation; Lupus Nephritis | 2009 |
Cystinosis of the brain and spinal cord with associated vasculopathy.
Topics: Adult; Anti-Inflammatory Agents; Biopsy, Needle; Central Nervous System; Crystallization; Cysteamine; Cystine; Cystinosis; Gait Disorders, Neurologic; Humans; Immunosuppressive Agents; Kidney Transplantation; Magnetic Resonance Imaging; Male; Myelitis; Postoperative Complications; Reoperation; Sensation Disorders; Spinal Cord Compression; Vasculitis, Central Nervous System | 2009 |
Nephropathic cystinosis in children: An overlooked disease.
Topics: Administration, Oral; Administration, Topical; Child; Child, Preschool; Cornea; Corneal Diseases; Cysteamine; Cystinosis; Diagnostic Techniques, Ophthalmological; Disease Progression; Fanconi Syndrome; Female; Humans; Infant; Kidney Failure, Chronic; Male; Medication Adherence; Ophthalmic Solutions; Predictive Value of Tests; Severity of Illness Index; Treatment Outcome | 2009 |
Cysteamine prevents inhibition of adenylate kinase caused by cystine in rat brain cortex.
Topics: Adenylyl Cyclases; Animals; Brain Chemistry; Brain Diseases, Metabolic; Cerebral Cortex; Creatine Kinase; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Drug Interactions; Energy Metabolism; Homeostasis; Lysosomes; Rats; Rats, Wistar | 2009 |
Chitotriosidase plasma activity in nephropathic cystinosis.
Topics: Child, Preschool; Cysteamine; Cystinosis; Fanconi Syndrome; Hexosaminidases; Humans; Nephrotic Syndrome | 2009 |
Evaluation of treatment with cysteamine eyedrops for cystinosis with confocal microscopy.
Topics: Corneal Diseases; Crystallization; Crystallography; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Kidney Diseases; Kidney Transplantation; Medical Records; Microscopy, Confocal; Ophthalmic Solutions; Photophobia; Treatment Outcome; Visual Acuity; Young Adult | 2009 |
Twice-daily cysteamine bitartrate therapy for children with cystinosis.
Topics: Adolescent; Child; Cysteamine; Cystinosis; Delayed-Action Preparations; Female; Gastrins; Humans; Leukocytes; Radiation-Protective Agents | 2010 |
Report of a Brazilian multicenter study on nephropathic cystinosis.
Topics: Adolescent; Adult; Brazil; Child; Child, Preschool; Comorbidity; Cysteamine; Cystinosis; Female; Glomerular Filtration Rate; Humans; Hypothyroidism; Infant; Kidney Diseases; Leukocytes; Male; Young Adult | 2010 |
Quiz page. Fanconi syndrome caused by cystinosis, intermediate type.
Topics: Adult; Cysteamine; Cystine; Cystinosis; Diagnosis, Differential; Fanconi Syndrome; Humans; Male; Nephritis, Interstitial; Radiation-Protective Agents; Sjogren's Syndrome | 2009 |
Long-term treatment of cystinosis in children with twice-daily cysteamine.
Topics: Adolescent; Child; Creatinine; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Leukocytes; Male; Tablets, Enteric-Coated | 2010 |
Gel formulations for treatment of the ophthalmic complications in cystinosis.
Topics: Acrylic Resins; Adhesiveness; Animals; Cattle; Cornea; Corneal Diseases; Cysteamine; Cystinosis; Drug Carriers; Drug Compounding; Gels; Hydrogen-Ion Concentration; In Vitro Techniques; Molecular Structure; Phenylalanine; Rheology; Solubility | 2010 |
Cysteamine, the molecule used to treat cystinosis, potentiates the antimalarial efficacy of artemisinin.
Topics: Animals; Antimalarials; Artemisinins; Cysteamine; Cystinosis; Dose-Response Relationship, Drug; Drug Resistance; Drug Synergism; Drug Therapy, Combination; Humans; Malaria; Mice; Mice, Inbred C57BL; Plasmodium chabaudi | 2010 |
Pharmacokinetics of enteric-coated cysteamine bitartrate in healthy adults: a pilot study.
Topics: Adolescent; Area Under Curve; Cysteamine; Cystinosis; Female; Gastrins; Humans; Intestinal Absorption; Male; Radiation-Protective Agents; Tablets, Enteric-Coated; Young Adult | 2010 |
Long-term outcome of nephropathic cystinosis: a 20-year single-center experience.
Topics: Adolescent; Angiotensin-Converting Enzyme Inhibitors; Child; Child, Preschool; Cysteamine; Cystinosis; Disease Progression; Drug Therapy, Combination; Early Diagnosis; Fanconi Syndrome; Female; Humans; Infant; Italy; Kaplan-Meier Estimate; Kidney; Kidney Failure, Chronic; Logistic Models; Male; Nephrotic Syndrome; Retrospective Studies; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome | 2010 |
A retro-inverso TAT-like peptide designed to deliver cysteamine to cells.
Topics: Chromatography, High Pressure Liquid; Cysteamine; Cystinosis; Disulfides; Drug Delivery Systems; Drug Design; Gene Products, tat; Peptides; Retroviridae; Spectrometry, Fluorescence; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Sulfhydryl Compounds | 2010 |
Pharmacokinetics of cysteamine in a cystinosis patient treated with hemodialysis.
Topics: Area Under Curve; Cysteamine; Cystinosis; Humans; Kidney Failure, Chronic; Male; Renal Dialysis; Young Adult | 2011 |
Cysteamine restores glutathione redox status in cultured cystinotic proximal tubular epithelial cells.
Topics: Adenosine Triphosphate; Adolescent; Cell Proliferation; Cells, Cultured; Child; Child, Preschool; Cysteamine; Cystinosis; Epithelial Cells; Female; Glutathione; Humans; Infant; Kidney Tubules, Proximal; Male; Oxidation-Reduction; Reactive Oxygen Species | 2011 |
Natural history of adolescent-onset cystinosis.
Topics: Adolescent; Age of Onset; Amino Acid Transport Systems, Neutral; Base Sequence; Child; Cysteamine; Cystinosis; Exons; Gene Deletion; Humans; Kidney Transplantation; Male; Middle Aged; Pedigree; Radiation-Protective Agents | 2011 |
Cysteamine toxicity in patients with cystinosis.
Topics: Child, Preschool; Cysteamine; Cystinosis; Drug Eruptions; Humans; Infant; Male | 2011 |
Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults.
Topics: Adolescent; Adult; Age Factors; Amino Acid Transport Systems, Neutral; Child; Child, Preschool; Cysteamine; Cystinosis; Diabetes Complications; Disease Progression; Educational Status; Employment; Fanconi Syndrome; Female; Follow-Up Studies; Humans; Hypothyroidism; Infant; Kaplan-Meier Estimate; Kidney Failure, Chronic; Male; Middle Aged; Nephrotic Syndrome; Nervous System Diseases; Neuromuscular Diseases; Radiation-Protective Agents; Young Adult | 2012 |
Evaluation of topical cysteamine therapy in the CTNS(-/-) knockout mouse using in vivo confocal microscopy.
Topics: Administration, Ophthalmic; Amino Acid Transport Systems, Neutral; Animals; Cornea; Crystallization; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Humans; Mice; Mice, Knockout; Microscopy, Confocal; Ophthalmic Solutions; Treatment Outcome; Visual Acuity | 2011 |
Detailed studies of growth hormone secretion in cystinosis patients.
Topics: Biomarkers; Body Height; Case-Control Studies; Child; Child, Preschool; Circadian Rhythm; Cysteamine; Cystinosis; Female; Glucagon; Growth Disorders; Hormone Replacement Therapy; Human Growth Hormone; Humans; Infant; Male; Time Factors; Treatment Outcome | 2012 |
Suppository formulations as a potential treatment for nephropathic cystinosis.
Topics: Chemistry, Pharmaceutical; Cysteamine; Cystine; Cystinosis; Drug Stability; Excipients; Fanconi Syndrome; Nephrotic Syndrome; Polyethylene Glycols; Solubility; Suppositories | 2012 |
Posterior reversible encephalopathy syndrome in cystinosis.
Topics: Anticonvulsants; Cysteamine; Cystinosis; Diffusion Magnetic Resonance Imaging; Electroencephalography; Female; Humans; Kidney Diseases; Magnetic Resonance Imaging; Peritoneal Dialysis; Posterior Leukoencephalopathy Syndrome; Steroids; Young Adult | 2013 |
Halitosis in cystinosis patients after administration of immediate-release cysteamine bitartrate compared to delayed-release cysteamine bitartrate.
Topics: Adolescent; Child; Cysteamine; Cystinosis; Exhalation; Halitosis; Humans; Male; Sulfides; Young Adult | 2012 |
Treatment of cystinosis with delayed-release cysteamine: 6-year follow-up.
Topics: Adolescent; Biomarkers; Chemistry, Pharmaceutical; Creatinine; Cysteamine; Cystinosis; Delayed-Action Preparations; Drug Administration Schedule; Fanconi Syndrome; Female; Glomerular Filtration Rate; Humans; Kidney; Leukocyte Count; Male; Nephrotic Syndrome; Time Factors; Treatment Outcome; Young Adult | 2013 |
Improvement of the cystine measurement in granulocytes by liquid chromatograhy-tandem mass spectrometry.
Topics: Case-Control Studies; Child, Preschool; Chromatography, High Pressure Liquid; Cysteamine; Cystine; Cystinosis; Granulocytes; Humans; Infant; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Time Factors | 2013 |
Heptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapy.
Topics: Amino Acid Motifs; Amino Acid Sequence; Amino Acid Transport Systems, Basic; Animals; Base Sequence; Caenorhabditis elegans Proteins; Canavanine; Cysteamine; Cystinosis; DEAD-box RNA Helicases; DNA, Complementary; Drosophila Proteins; Electrophysiological Phenomena; Female; Genes, Fungal; Humans; Membrane Proteins; Molecular Sequence Data; Oocytes; Protein Structure, Secondary; Rats; Recombinant Proteins; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Species Specificity; Vacuoles; Xenopus laevis | 2012 |
Follow-up and treatment of adults with cystinosis in the Netherlands.
Topics: Adult; Central Nervous System Diseases; Cysteamine; Cystinosis; Endocrine System Diseases; Fanconi Syndrome; Female; Follow-Up Studies; Humans; Kidney Transplantation; Male; Muscular Diseases; Netherlands; Postoperative Complications; Retrospective Studies; Socioeconomic Factors; Vision Disorders; Visual Acuity | 2002 |
In vivo confocal microscopy of the cornea in nephropathic cystinosis.
Topics: Adult; Cornea; Corneal Diseases; Crystallization; Cysteamine; Cystinosis; Humans; Kidney Diseases; Male; Microscopy, Confocal | 2002 |
The evaluation and treatment of gastrointestinal disease in children with cystinosis receiving cysteamine.
Topics: Anti-Ulcer Agents; Child; Child, Preschool; Cysteamine; Cystinosis; Female; Gastric Acid; Gastrins; Gastrointestinal Diseases; Gastroscopy; Humans; Male; Omeprazole; Stomach | 2003 |
Cysteamine prevents and reverses the inhibition of pyruvate kinase activity caused by cystine in rat heart.
Topics: Animals; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Enzyme Activation; Heart; Myocardium; Organ Culture Techniques; Pyruvate Kinase; Rats; Rats, Wistar | 2004 |
Comparison of cystine determination in mixed leukocytes vs polymorphonuclear leukocytes for diagnosis of cystinosis and monitoring of cysteamine therapy.
Topics: Cysteamine; Cystine; Cystinosis; Humans; Neutrophils | 2004 |
FISH diagnosis of the common 57-kb deletion in CTNS causing cystinosis.
Topics: Alleles; Amino Acid Transport Systems, Neutral; Cysteamine; Cystinosis; Databases as Topic; Exons; Gene Deletion; Glycoproteins; Humans; In Situ Hybridization, Fluorescence; Membrane Proteins; Membrane Transport Proteins; Models, Genetic; Mutation; Nucleic Acid Hybridization; Polymerase Chain Reaction | 2004 |
Treatment of cystinosis: simple in principle, difficult in practice.
Topics: Adolescent; Child; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Humans; Patient Compliance | 2004 |
Successful use of topical cysteamine formulated from the oral preparation in a child with keratopathy secondary to cystinosis.
Topics: Administration, Topical; Capsules; Chemistry, Pharmaceutical; Child; Corneal Diseases; Cysteamine; Cystinosis; Humans; Male; Ophthalmic Solutions; Pharmaceutical Preparations; Photophobia; Retrospective Studies | 2004 |
Noncirrhotic portal hypertension in association with juvenile nephropathic cystinosis: case presentation and review of the literature.
Topics: Adult; Biopsy; Creatinine; Cysteamine; Cysteine; Cystinosis; Female; Humans; Hypertension, Portal; Kidney Transplantation; Kupffer Cells; Liver; Liver Diseases; Tomography, X-Ray Computed | 2004 |
Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex.
Topics: Adenosine Diphosphate; Animals; Binding, Competitive; Cerebral Cortex; Creatine Kinase; Cysteamine; Cystine; Cystinosis; Cytosol; Dose-Response Relationship, Drug; Enzyme Activation; Enzyme Inhibitors; Glutathione; Mitochondria; Nerve Degeneration; Phosphocreatine; Rats; Rats, Wistar; Subcellular Fractions | 2005 |
Swallowing dysfunction in 101 patients with nephropathic cystinosis: benefit of long-term cysteamine therapy.
Topics: Adolescent; Adult; Age Factors; Amino Acid Transport Systems, Neutral; Child; Cysteamine; Cystinosis; Deglutition Disorders; Female; Glycoproteins; Humans; Male; Membrane Proteins; Membrane Transport Proteins; Middle Aged; Mutation; Severity of Illness Index | 2005 |
Cystinosis as a cause of noncirrhotic portal hypertension.
Topics: Adult; Cysteamine; Cystinosis; Fatal Outcome; Humans; Hypertension, Portal; Male; Portasystemic Shunt, Transjugular Intrahepatic | 2005 |
Esomeprazole therapy for gastric acid hypersecretion in children with cystinosis.
Topics: Child; Child, Preschool; Cysteamine; Cystinosis; Enzyme Inhibitors; Esomeprazole; Female; Gastric Acid; Gastrins; Gastrointestinal Diseases; Gastroscopy; Humans; Intestinal Mucosa; Male; Retrospective Studies; Severity of Illness Index | 2005 |
Strict cysteamine dose regimen is required to prevent nocturnal cystine accumulation in cystinosis.
Topics: Adolescent; Child; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Male; Neutrophils; Time Factors | 2006 |
Development of Fanconi syndrome during infancy in a patient with cystinosis.
Topics: Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant; Male; Renal Agents | 2006 |
Nephropathic cystinosis: posterior segment manifestations and effects of cysteamine therapy.
Topics: Administration, Oral; Adolescent; Adult; Child; Child, Preschool; Cross-Sectional Studies; Cysteamine; Cystinosis; Electroretinography; Female; Humans; Infant; Male; Retina; Retinal Diseases; Visual Acuity; Visual Fields | 2006 |
A deeper look into cysteamine absorption for the treatment of cystinosis.
Topics: Cysteamine; Cystinosis; Fanconi Syndrome; Gastrins; Humans; Intestine, Small | 2006 |
Understanding intestinal cysteamine bitartrate absorption.
Topics: Adolescent; Adult; Area Under Curve; Cysteamine; Cystine; Cystinosis; Delayed-Action Preparations; Female; Gastrins; Humans; Intestinal Absorption; Leukocytes; Male; Quality of Life | 2006 |
Pharmacokinetics of cysteamine bitartrate following gastrointestinal infusion.
Topics: Adult; Area Under Curve; Cysteamine; Cystinosis; Female; Humans; Intestinal Absorption; Male; Nootropic Agents; Prospective Studies | 2007 |
The origin of halitosis in cystinotic patients due to cysteamine treatment.
Topics: Adolescent; Adult; Breath Tests; Child; Cysteamine; Cystinosis; Female; Halitosis; Humans; Male; Sulfhydryl Compounds; Sulfides | 2007 |
Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester.
Topics: Animals; Catalase; Cysteamine; Cystine; Cystinosis; Drug Interactions; Fluoresceins; Glutathione Peroxidase; Hydrogen Peroxide; Kidney; Lipid Peroxidation; Oxidation-Reduction; Oxidative Stress; Proteins; Random Allocation; Rats; Rats, Wistar; Superoxide Dismutase; Thiobarbituric Acid Reactive Substances | 2007 |
Growth hormone producing prolactinoma in juvenile cystinosis: a simple coincidence?
Topics: Administration, Oral; Administration, Topical; Body Height; Cabergoline; Child; Cysteamine; Cystinosis; Dopamine Agonists; Ergolines; Headache; Human Growth Hormone; Humans; Male; Ophthalmic Solutions; Pituitary Neoplasms; Prolactinoma; Radiation-Protective Agents; Treatment Outcome | 2008 |
Intravenous delivery of cysteamine for the treatment of cystinosis: association with hepatotoxicity.
Topics: Chemical and Drug Induced Liver Injury; Combined Modality Therapy; Cysteamine; Cystinosis; Drug Therapy, Combination; Female; Gastrointestinal Diseases; Gastrointestinal Motility; Humans; Hypothyroidism; Infant; Injections, Intravenous; Kidney Transplantation; Radiation-Protective Agents; Renal Insufficiency | 2008 |
Nephropathic cystinosis in adults: natural history and effects of oral cysteamine therapy.
Topics: Administration, Oral; Adolescent; Adult; Amino Acid Transport Systems, Neutral; Cysteamine; Cystinosis; Fanconi Syndrome; Female; Gene Deletion; Humans; Kidney Transplantation; Kidney Tubules; Male; Middle Aged; Retrospective Studies | 2007 |
Cysteamine prevents inhibition of thiol-containing enzymes caused by cystine or cystine dimethylester loading in rat brain cortex.
Topics: Animals; Antioxidants; Cerebral Cortex; Creatine Kinase; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Enzyme Inhibitors; Lysosomes; Oxidation-Reduction; Oxidative Stress; Pyruvate Kinase; Rats; Rats, Wistar; Sulfhydryl Compounds | 2008 |
The use of CDME in cystinosis research.
Topics: Animals; Antioxidants; Biomedical Research; Cysteamine; Cystinosis; Rats; Rats, Wistar | 2008 |
Hematological manifestations of nephropathic cystinosis.
Topics: Adult; Antilymphocyte Serum; Bone Marrow; Combined Modality Therapy; Crystallization; Cysteamine; Cystine; Cystinosis; Humans; Immunosuppressive Agents; Kidney Failure, Chronic; Kidney Transplantation; Macrophages; Male; Pancytopenia; Postoperative Complications; T-Lymphocytes | 2008 |
Levels of gamma-glutamyltranspeptidase in cultured skin fibroblasts from cystinotics and normals.
Topics: Adult; Cell Line; Child; Cysteamine; Cystinosis; Fibroblasts; gamma-Glutamyltransferase; Humans; Skin | 1980 |
[Treatment of cystinosis with cysteamine].
Topics: Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; In Vitro Techniques; Infant; Male | 1983 |
[Cysteamine in the treatment of cystinosis in children. In vitro and in vivo studies].
Topics: Cells, Cultured; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Eye Diseases; Female; Fibroblasts; Growth; Humans; Infant; Kidney; Leukocytes; Male | 1984 |
Effects of cysteamine and other thiols on cystine egress from isolated lysosome-rich fractions of cystinotic and normal leucocytes.
Topics: Amino Acids; Ascorbic Acid; Cysteamine; Cystine; Cystinosis; Glutathione; Half-Life; Humans; In Vitro Techniques; Leukocytes; Lysosomes; Sulfhydryl Compounds | 1983 |
The intralysosomal pH in cultured human skin fibroblasts in relation to cystine accumulation in patients with cystinosis.
Topics: Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Hydrogen-Ion Concentration; Lysosomes | 1983 |
Hepatotoxicity of cysteamine?
Topics: Chemical and Drug Induced Liver Injury; Cysteamine; Cystinosis; Humans | 1983 |
Renal cell culture using autopsy material from children with cystinosis.
Topics: Autopsy; Cells, Cultured; Child, Preschool; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Kidney; Male; Pantetheine | 1984 |
Decreased sweat production in cystinosis.
Topics: Adolescent; Child; Child, Preschool; Cysteamine; Cystinosis; Humans; Infant; Sweat | 1984 |
[A case of nephropathic cystinosis associated with hyperlipidemia].
Topics: Child; Cysteamine; Cystinosis; Female; Humans; Hyperlipidemias; Light; Vision Disorders | 1984 |
Handling of L-[35S]cystine by cysteamine-pretreated cystinotic and normal fibroblasts.
Topics: Cell Line; Cycloheximide; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Isotope Labeling; Sulfur Radioisotopes | 1983 |
Veno-occlusive disease of the liver associated with cysteamine treatment of nephropathic cystinosis.
Topics: Child; Cysteamine; Cystinosis; Hepatic Veins; Humans; Hypertension, Portal; Male; Stomach; Varicose Veins; Vascular Diseases | 1983 |
Nephropathic cystinosis: ineffectiveness of cysteamine therapy for ocular changes.
Topics: Child; Child, Preschool; Cysteamine; Cystinosis; Eye Diseases; Humans; Infant; Infant, Newborn; Kidney Diseases | 1983 |
Treatment of cystinosis with cysteamine. A pilot study determining dose and form of application.
Topics: Child, Preschool; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Humans; Infant; Kidney Transplantation; Pilot Projects; Postoperative Complications; Renal Dialysis | 1981 |
Plasma cysteamine concentrations in children treated for cystinosis.
Topics: Administration, Oral; Child; Child, Preschool; Cysteamine; Cystinosis; Humans; Infant; Leukocytes | 1982 |
Effect of cysteamine on amino acid pools of cultured fibroblasts and the uptake of L-proline.
Topics: Amino Acids; Biological Transport; Cell Line; Cysteamine; Cystinosis; Fibroblasts; Humans; Proline | 1982 |
Uptake of cystine by cystine-depleted fibroblasts from patients with cystinosis.
Topics: Biological Transport; Cell Line; Child; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Kinetics; Skin | 1981 |
Cysteamine therapy in nephropathic cystinosis.
Topics: Adult; Child; Child, Preschool; Creatinine; Cysteamine; Cystinosis; Humans; Infant; Kidney; Male | 1981 |
Pantetheinase activity and cysteamine content in cystinotic and normal fibroblasts and leukocytes.
Topics: Amidohydrolases; Cysteamine; Cystinosis; Fibroblasts; GPI-Linked Proteins; Humans; Leukocytes; Mercaptoethylamines; Pantetheine | 1981 |
Cysteamine inhibition of [15N]-glycine turnover in cystinosis and of glycine cleavage system in vitro.
Topics: Coenzyme A; Cysteamine; Cystinosis; Decarboxylation; Glycine; Humans; In Vitro Techniques; Kinetics; Mitochondria, Liver; Nitrogen Isotopes | 1981 |
Adverse reactions to oral cysteamine use in nephropathic cystinosis.
Topics: Child; Child, Preschool; Cysteamine; Cystinosis; Female; Humans; Kidney Diseases; Male | 1981 |
Cystine depletion of cystinotic tissues by phosphocysteamine (WR638).
Topics: Child; Cystaphos; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Fibroblasts; Humans; Male; Organothiophosphorus Compounds | 1980 |
Effects of cysteamine therapy in nephropathic cystinosis.
Topics: Amino Acids; Blood Glucose; Child; Child, Preschool; Creatinine; Cysteamine; Cystinosis; Growth; Humans; Lactates; Leukocytes; Male; Phosphates; Pyruvates | 1981 |
Biochemical follow-up in late-treated nephropathic cystinosis.
Topics: Carnitine; Creatinine; Cysteamine; Cysteine; Cystinosis; Disease Progression; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Kidney Function Tests; Male; Oxidants | 1995 |
[1H-NMR spectroscopic study of cysteamine eyedrops].
Topics: Cornea; Corneal Diseases; Cystamine; Cysteamine; Cystinosis; Dose-Response Relationship, Drug; Drug Stability; Humans; Magnetic Resonance Spectroscopy; Ophthalmic Solutions; Oxidation-Reduction | 1995 |
Approval of cysteamine for patients with cystinosis.
Topics: Cysteamine; Cystinosis; Drug Approval; Humans | 1995 |
Progression of chronic renal failure in a historical group of patients with nephropathic cystinosis. European Collaborative Study on Cystinosis.
Topics: Child, Preschool; Creatinine; Cysteamine; Cystinosis; Disease Progression; Humans; Kidney Failure, Chronic; Kidney Function Tests; Retrospective Studies; Survival Rate | 1994 |
Cysteamine for cystinosis.
Topics: Cysteamine; Cystinosis; Humans; Orphan Drug Production | 1994 |
Classic nephropathic cystinosis as an adult disease.
Topics: Adult; Central Nervous System Diseases; Cohort Studies; Cost of Illness; Cysteamine; Cystinosis; Endocrine System Diseases; Female; Graft Survival; Growth; Humans; Kidney Transplantation; Male; Muscular Diseases; Survival Analysis; Visual Acuity | 1993 |
Improved renal function in children with cystinosis treated with cysteamine.
Topics: Child; Child, Preschool; Creatinine; Cysteamine; Cystinosis; Humans; Kidney; Kidney Transplantation; Renal Insufficiency | 1993 |
Intravenous cysteamine therapy for nephropathic cystinosis.
Topics: Child, Preschool; Cysteamine; Cystine; Cystinosis; Gastrointestinal Diseases; Gastrointestinal Motility; Humans; Injections, Intravenous; Leukocytes; Male; Microscopy, Electron; Sulfides | 1995 |
The treatment of cystinosis with cysteamine and phosphocysteamine in the United Kingdom and Eire.
Topics: Adolescent; Child; Child, Preschool; Cystaphos; Cysteamine; Cystine; Cystinosis; Female; Growth; Humans; Infant; Ireland; Kidney Function Tests; Leukocytes; Male; Retrospective Studies; United Kingdom | 1995 |
Effectiveness of cysteamine and mesna in decreasing intracellular cystine content in cystinosis.
Topics: Cells, Cultured; Cysteamine; Cystine; Cystinosis; Expectorants; Humans; Mesna; Radiation-Protective Agents | 1996 |
Pyroglutamic aciduria and nephropathic cystinosis.
Topics: 3-Hydroxybutyric Acid; Case-Control Studies; Child, Preschool; Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant; Ketoglutaric Acids; Lactates; Malates; Pyrrolidonecarboxylic Acid; Succinates | 1999 |
Cysteamine: new preparation. Chronic treatment of cystinosis.
Topics: Adolescent; Child; Child, Preschool; Clinical Trials as Topic; Cysteamine; Cystinosis; Europe; Humans; Infant; Radiation-Protective Agents; Treatment Outcome | 1999 |
New method for determining cystine in leukocytes and fibroblasts.
Topics: Adult; Borohydrides; Bridged Bicyclo Compounds; Cell Extracts; Chromatography, High Pressure Liquid; Cysteamine; Cystine; Cystinosis; Ethylmaleimide; Fibroblasts; Fluorometry; Humans; Leukocytes; Middle Aged; Reproducibility of Results | 1999 |
Negligible urinary cysteamine loss in cystinosis patients with Fanconi syndrome.
Topics: Adolescent; Child; Cysteamine; Cystinosis; Fanconi Syndrome; Humans | 2002 |
Depletion of cystine in cystinotic fibroblasts by drugs enclosed in liposomes.
Topics: Animals; Culture Techniques; Cysteamine; Cystine; Cystinosis; Fibroblasts; Hydrogen-Ion Concentration; Liposomes; Mice; Phosphatidylcholines | 1978 |
Cysteamine therapy for cystinosis.
Topics: Administration, Oral; Child, Preschool; Cysteamine; Cystine; Cystinosis; Female; Follow-Up Studies; Humans; Infant; Leukocytes; Male | 1978 |
Cysteamine for cystinosis.
Topics: Animals; Cysteamine; Cystinosis; Duodenal Ulcer; Humans; Rats | 1978 |
Treatment of cystinosis with cysteamine.
Topics: Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant; Male | 1979 |
Approaches to the treatment of inborn errors of sulphur amino acid and peptide metabolism.
Topics: Amino Acids, Sulfur; Ascorbic Acid; Aspirin; Cysteamine; Cystinosis; Dipyridamole; Glutathione; Glutathione Synthase; Homocystinuria; Humans; Metabolism, Inborn Errors; Pyridoxine; Vitamin E | 1979 |
Cysteamine, penicillamine, glutathione, and their derivatives analyzed by automated ion exchange column chromatography.
Topics: Animals; Autoanalysis; Chromatography, Ion Exchange; Cysteamine; Cystinosis; Ethylmaleimide; Fibroblasts; Glutathione; Humans; Iodoacetamide; Iodoacetates; Male; Penicillamine; Rats | 1978 |
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
Topics: Child; Cystamine; Cysteamine; Cystine; Cystinosis; Female; Fibroblasts; Glutathione; Humans; Leukocytes; Models, Biological; Oxidation-Reduction | 1976 |
[Cystinosis].
Topics: Administration, Oral; Cysteamine; Cystine; Cystinosis; Eye; Genes, Recessive; Humans; Kidney; Kidney Transplantation; Lysosomes | 1992 |
Parenchymal organ cystine depletion with long-term cysteamine therapy.
Topics: Adolescent; Child; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Infant; Kidney; Kidney Diseases; Liver; Lung; Male; Microscopy, Electron, Scanning; Muscles; Pancreas; Spleen | 1992 |
[Infantile cystinosis. A new Tunisian case].
Topics: Child, Preschool; Cysteamine; Cystine; Cystinosis; Humans; Leukocytes; Male; Ophthalmoscopy; Radiography | 1992 |
[Cysteamine eyedrops for treatment of corneal cysteine deposits in infantile cystinosis].
Topics: Child, Preschool; Cornea; Corneal Opacity; Crystallization; Cysteamine; Cystine; Cystinosis; Humans; Male; Ophthalmic Solutions | 1992 |
Effects of oral phosphocysteamine and rectal cysteamine in cystinosis.
Topics: Administration, Oral; Administration, Rectal; Adolescent; Child; Child, Preschool; Circadian Rhythm; Cystaphos; Cysteamine; Cystine; Cystinosis; Female; Humans; Infant; Leukocytes; Male; Time Factors | 1991 |
[Intra-leukocyte cystine in cystinosis treated with cysteamine].
Topics: Adolescent; Adult; Binding, Competitive; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Infant; Leukocytes; Male; Patient Compliance; Radioimmunoassay | 1991 |
Treatment of cystinosis with cysteamine from early infancy.
Topics: Child, Preschool; Cysteamine; Cystinosis; Drug Administration Schedule; Family; Female; Humans; Infant, Newborn; Male; Pregnancy; Prenatal Diagnosis | 1991 |
[The treatment of cystinosis with cysteamine].
Topics: Cysteamine; Cystinosis; Humans; Male | 1991 |
Acquired structural genitourinary abnormalities contributing to deterioration of renal function in older patients with nephropathic cystinosis.
Topics: Creatinine; Cysteamine; Cystinosis; Female; Glomerular Filtration Rate; Humans; Infant; Kidney Failure, Chronic; Male; Ultrasonography | 1991 |
Ocular manifestations of nephropathic cystinosis. The French-Canadian experience in a genetically homogeneous population.
Topics: Adolescent; Adult; Canada; Child; Child, Preschool; Cysteamine; Cystinosis; Eye Diseases; Humans; Infant; Kidney Diseases; Kidney Transplantation; Light | 1991 |
Clearance of corneal crystals in nephropathic cystinosis by topical cysteamine 0.5%.
Topics: Administration, Topical; Child, Preschool; Cornea; Corneal Diseases; Crystallization; Cysteamine; Cystinosis; Female; Humans | 1991 |
Stable isotope dilution analysis of cystine in granulocyte suspensions as cysteine: a powerful method for the diagnosis, the follow-up, and treatment of patients with cystinosis.
Topics: Adolescent; Adult; Cell Separation; Child; Cysteamine; Cysteine; Cystine; Cystinosis; Gas Chromatography-Mass Spectrometry; Granulocytes; Humans; Indicator Dilution Techniques; Reference Values | 1990 |
[Treatment of cystinosis using cysteamine].
Topics: Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Female; Growth; Humans; Infant; Kidney; Leukocytes; Male; Time Factors | 1990 |
Mechanism of cystine reaccumulation by cystinotic fibroblasts in vitro.
Topics: Biological Transport; Cell Membrane; Cells, Cultured; Colchicine; Cysteamine; Cystine; Cystinosis; Fibroblasts; Glutamates; Humans; Kinetics; Lysosomes | 1990 |
Swallowing dysfunction in nephropathic cystinosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cysteamine; Cystinosis; Deglutition; Deglutition Disorders; Female; Humans; Kidney Diseases; Kidney Transplantation; Male; Mouth | 1990 |
Depletion of cystine in cystinotic fibroblasts by homocysteine. Synergism of cysteamine with various reducing agents in depletion of cystine from cystinotic fibroblasts.
Topics: Ascorbic Acid; Cells, Cultured; Cysteamine; Cystine; Cystinosis; Fibroblasts; Glutathione; Homocysteine; Humans | 1990 |
Predicted reciprocal serum creatinine at age 10 years as a measure of renal function in children with nephropathic cystinosis treated with oral cysteamine.
Topics: Administration, Oral; Age Factors; Child; Child, Preschool; Creatinine; Cysteamine; Cystinosis; Humans; Kidney Function Tests | 1990 |
From the National Institutes of Health.
Topics: Corneal Diseases; Cysteamine; Cystinosis; Growth Hormone; Humans; Lymphocyte Activation; National Institutes of Health (U.S.); Paraparesis, Tropical Spastic; Prednisone; Protein Deficiency; United States | 1990 |
Cystine depletion by WR-1065 in cystinotic cells. Mechanism of action.
Topics: Amifostine; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Humans; In Vitro Techniques; Mercaptoethylamines; Radiation-Protective Agents; Sulfur Radioisotopes | 1985 |
Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.
Topics: Administration, Oral; Biological Transport; Carnitine; Cells, Cultured; Child; Child, Preschool; Cholesterol; Cysteamine; Cystinosis; Fanconi Syndrome; Fatty Acids, Nonesterified; Humans; Infant; Kidney Transplantation; Lipid Metabolism; Metabolic Clearance Rate; Muscles; Oculocerebrorenal Syndrome; Renal Tubular Transport, Inborn Errors; Triglycerides | 1988 |
A comparison of the effectiveness of cysteamine and phosphocysteamine in elevating plasma cysteamine concentration and decreasing leukocyte free cystine in nephropathic cystinosis.
Topics: Administration, Oral; Child; Child, Preschool; Cystaphos; Cysteamine; Cystine; Cystinosis; Gastrointestinal Contents; Humans; Infant; Leukocytes; Organothiophosphorus Compounds; Patient Acceptance of Health Care; Vomiting | 1988 |
Long-term ocular manifestations in nephropathic cystinosis.
Topics: Adolescent; Adult; Child; Cornea; Cysteamine; Cystinosis; Electroretinography; Eye Diseases; Female; Fluorescein Angiography; Humans; Iris; Kidney Diseases; Kidney Transplantation; Lens Capsule, Crystalline; Male; Retina; Visual Acuity | 1986 |
Nephropathic cystinosis: effect of long-term cysteamine therapy.
Topics: Body Height; Child, Preschool; Creatinine; Cysteamine; Cystinosis; Humans; Male | 1987 |
Ocular changes in long-term evolution of infantile cystinosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cysteamine; Cystinosis; Eye Diseases; Female; Follow-Up Studies; Humans; Infant; Kidney Diseases; Male; Ophthalmic Solutions | 1987 |
Cystine storage in cultured myotubes from patients with nephropathic cystinosis.
Topics: Cells, Cultured; Centrifugation, Density Gradient; Cysteamine; Cystine; Cystinosis; Humans; Muscles | 1987 |
Treatment of infantile nephropathic cystinosis with cysteamine.
Topics: Child; Child, Preschool; Cysteamine; Cystinosis; Female; Humans; Infant; Kidney Diseases; Male | 1986 |
Blunted prolactin response to thyrotropin-releasing hormone stimulation in cystinotic children receiving cysteamine.
Topics: Administration, Oral; Child; Child, Preschool; Cysteamine; Cystinosis; Female; Humans; Infant; Male; Prolactin; Thyrotropin; Thyrotropin-Releasing Hormone; Time Factors | 1985 |
A study of the low beta-galactosidase activity in cystinotic fibroblasts: effects of cysteamine.
Topics: beta-Galactosidase; Cells, Cultured; Cysteamine; Cystinosis; Fibroblasts; Galactosidases; Humans; Hydrolysis; Leupeptins | 1985 |
Detection and characterization of carrier-mediated cationic amino acid transport in lysosomes of normal and cystinotic human fibroblasts. Role in therapeutic cystine removal?
Topics: Amino Acids; Biological Transport, Active; Cell Line; Chloroquine; Cysteamine; Cystinosis; Electrophoresis; Fibroblasts; Humans; Hydrogen-Ion Concentration; Lysine; Lysosomes | 1985 |
Further studies on the effect of chloroquine on the uptake, metabolism and intracellular translocation of [35S]cystine in cystinotic fibroblasts.
Topics: Biological Transport; Cells, Cultured; Chloroquine; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Hydrogen-Ion Concentration; Lysosomes | 1986 |
In vivo alteration of a mutant human protein using the free thiol cysteamine.
Topics: Apolipoprotein E2; Apolipoprotein E3; Apolipoproteins E; Child, Preschool; Cysteamine; Cystinosis; Humans; Hyperlipoproteinemia Type III; Isoelectric Focusing; Male; Middle Aged; Mutation | 1985 |
Cysteamine depletes cystinotic leucocyte granular fractions of cystine by the mechanism of disulphide interchange.
Topics: Cystamine; Cysteamine; Cystine; Cystinosis; Cytoplasmic Granules; Disulfides; Ethylmaleimide; Half-Life; Humans; Leukocytes | 1985 |
Metabolism of pantethine in cystinosis.
Topics: Adolescent; Amidohydrolases; Animals; Child; Cysteamine; Cystine; Cystinosis; Diarrhea; Female; GPI-Linked Proteins; Humans; Intestinal Absorption; Intestinal Mucosa; Kinetics; Leukocytes; Male; Pantetheine; Rats; Rats, Inbred Strains; Sulfhydryl Compounds | 1985 |
Long-term treatment of infantile nephropathic cystinosis with cysteamine.
Topics: Bone Marrow; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Growth; Humans; Infant; Infant, Newborn; Kidney Failure, Chronic; Leukocytes; Male | 1985 |
Therapy of cystinosis.
Topics: Cysteamine; Cystinosis; Fanconi Syndrome; Humans; Infant | 1985 |
Lysosomal cystine storage in cystinosis and mucolipidosis type II.
Topics: Cells, Cultured; Cysteamine; Cysteine; Cystine; Cystinosis; Fibroblasts; Humans; Leukocytes; Liver; Lysosomes; Mucolipidoses | 1985 |
Therapy of cystinosis.
Topics: Cysteamine; Cystinosis; Humans; Orphan Drug Production; Pantetheine | 1985 |