Compounds > carbostyril
Page last updated: 2024-08-17

carbostyril and Cystic Fibrosis of Pancreas

carbostyril has been researched along with Cystic Fibrosis of Pancreas in 683 studies

Research

Studies (683)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's2 (0.29)18.2507
2000's5 (0.73)29.6817
2010's443 (64.86)24.3611
2020's233 (34.11)2.80

Authors

AuthorsStudies
Bengtson, CD; He, J; Kim, MD; Salathe, MA1
Lahiri, T; Sullivan, JS1
Davies, G; Granger, E; Keogh, RH1
Anderson, A; Lubsch, L; McCoy, L; Pettit, RS; Wright, BA1
Alyan, N; Breuer, O; Cohen-Cymberknoh, M; Kerem, E; Koretz, S; Reiter, J; Shoseyov, D; Wexler, I1
Chróinín, MN; Croinin, K; Crowley, J; Mullane, D1
Cagnina, RE; Cernadas, M; Kennedy, JC; Marchetti, P; Putman, MS; Sawicki, GS; Scully, KJ; Uluer, A1
Amato, A; Campagna, G; Carnovale, V; Ferrari, G; Majo, F; Padoan, R; Pepe, A; Quattrucci, S; Salvatore, D; Salvatore, M; Taruscio, D1
Bruegel, M; Habler, K; Kalla, AS; Nährig, S; Paal, M; Rychlik, M; Teupser, D; Vogeser, M1
Allard, PM; Commun, C; Dijoux-Franca, MG; Doléans-Jordheim, A; Gioia, B; Michalet, S; Ngoc, VTN; Nouwade, K; Wolfender, JL1
Bacon, DR; Ebert, CS; Goralski, JL; Kimple, AJ; Lee, SE; Nouraie, M; Senior, BA; Shaffer, AD; Stapleton, A; Thorp, BD; Zemke, AC1
Antell, MH; Bourque, B; Diloreto, S; Heo, S; Rotolo, SM; Safirstein, J; Young, DC1
Bilton, D; Carr, SB; Daniel, RM; Keogh, RH; Newsome, SJ1
Dittrich, AM; Hansen, G; Junge, S; Nietert, M; Pallenberg, ST; Ringshausen, FC; Sauer-Heilborn, A; Tümmler, B1
Kemper, EM; Mathôt, RAA; Mouissie, LM; Terheggen-Lagro, SWJ; Vonk, SEM1
Blaikie, L; Collins, S; Daniels, T; Edwards, V; Ketchell, I; McCulloch, A; Shaw, N; Smith, T1
Autry, E; Bomersback, T; Hewlett, J; Kormelink, L; Lubsch, L; Ragan, H; Safirstein, J; Shanley, L1
Bartels, AR; Ketchen, NK; Rasmussen, LN; Singh, SB; Wright, BA1
Begnel, L; Litvin, M; Petersen, MC; Wallendorf, M1
Bijvelds, MJC; de Jonge, HR; Janssens, HM; Meijsen, KF; Roest, HP; Roos, FJM; van der Laan, LJW; Verstegen, MMA1
Ahluwalia, N; Bruinsma, BG; Downey, DG; Duckers, J; Harris, C; Lam, AP; Lou, Y; MacGregor, G; Mall, MA; McKone, EF; Moskowitz, SM; Sutharsan, S; Tian, S; Tullis, E; Van Braeckel, E; Wainwright, CE; Waltz, D; Watson, D; Yuan, J1
Collins, B; Cotey, A; Esther, CRJ; Fortner, C; Trimble, A1
Cruz, VB; Konczal, LL; Tang, TY1
Avolio, J; Gonska, T; Grasemann, H; Jacobi, E; Ratjen, F; Shaw, M; Solomon, M1
Zhang, B; Zhang, S1
Burgel, PR; Campredon, A; Chassagnon, G; Vakalopoulou, M1
Burgel, PR; Burnet, E; Chiron, R; Da Silva, J; Danner-Boucher, I; Douvry, B; Durieu, I; Grenet, D; Hamidfar, R; Lemonnier, L; Leroy, S; Martin, C; Mely, L; Montcouquiol, S; Murris-Espin, M; Paillasseur, JL; Ramel, S; Reynaud-Gaubert, M1
Breneman, A; Gallitano, SM; Soliman, YS1
Aylward, SC; Jordan, CO; Kopp, BT; Paul, GR; Wisniewski, BL1
Abou Alaiwa, M; Cavanaugh, JE; Harris, LM; Hornick, DB; Miller, AC; Polgreen, PM; Stoltz, DA1
Ahluwalia, N; Campbell, D; Chilvers, M; Davies, JC; Harris, RS; McNamara, J; Naehrlich, L; Paz-Diaz, H; Saunders, C; Sawicki, GS; Sermet-Gaudelus, I; Shih, JL; Wainwright, CE1
Bratcher, PE; Shaughnessy, CA; Zeitlin, PL1
Borgo, C; Capurro, V; Castellani, C; Cresta, F; D'Amore, C; Pedemonte, N; Salvi, M; Sondo, E; Tomati, V1
Almond, LM; Beringer, PM; Chung, PS; Hong, E; Rao, AP1
Al-Aloul, M; FitzMaurice, TS; Iyer, K; Nazareth, D; Walshaw, M1
Branstetter, BF; Goralski, JL; Kimple, AJ; Lee, SE; Nouraie, SM; Pilewski, JM; Senior, BA; Shaffer, AD; Stapleton, AL; Zemke, AC1
Aksit, MA; Bridges, RJ; Cutting, GR; Faino, AV; Gibson, RL; Goralski, JL; Ling, H; Merlo, CA; Montemayor, K; Onchiri, FM; Osorio, DL; Patel, SU; Paul, KC; Raraigh, KS; Sciortino, S; Sharma, N; Sheppard, DN; Thakerar, A; Wang, Y; West, NE; Worthington, EN1
Baffico, AM; Bandiera, T; Bocciardi, R; Capurro, V; Castellani, C; Coviello, D; Cresta, F; Galietta, LJV; Iacomino, M; Lena, M; Pastorino, C; Pedemonte, N; Pesce, E; Sondo, E; Tomati, V; Zara, F1
Fortunak, J; Guo, J; Hill, A; Wang, J; Zhang, J1
Borowitz, D; Clancy, JP; Gabel, ME; Gelfond, D; Roach, C; Rowe, SM; Sagel, SD; Wang, H1
Amati, F; Blasi, F; Carugo, S; Contarini, M; De Petro, C; Gramegna, A; Leonardi, G; Meazza, R1
Joshi, S; Muirhead, C; Verzasconi, D1
Bartlett, LE; Diamond, JM; Faro, A; Goss, CH; Guimbellot, JS; Pilewski, JM; Ramos, KJ; Valapour, M; Wai, TH1
Cipolli, M; Costa, E; Girotti, S; Leufkens, HGM; Pauro, F1
Bignamini, E; Cimino, G; Esposito, I; Leonetti, G; Manunza, D; Pepe, A; Salvatore, D; Troiani, P; Zanda, M1
Ben-Meir, E; Grasemann, H1
Bass, JL; Walter, E1
Berdella, MN; Januska, MN; Langfelder-Schwind, E1
Bansal, A; Goss, CH; Hee Wai, T; Kapnadak, SG; Mayer-Hamblett, N; Ramos, KJ; Rodriguez, PJ; Stanojevic, S; Stephenson, AL; Sykes, J1
Jensen, JH; Leo-Hansen, C; Olesen, HV; Pedersen, CL; Philipsen, LKD; Rysgaard, UK; Sørensen, L1
Esther, CR; Jain, R; Keller, A; Magaret, A; Mayer-Hamblett, N; Putman, MS; Taylor-Cousar, JL; VanDalfsen, JM; Vu, PT; Wilson, A1
Edwards, C; Lee, T; Naisbitt, DJ; Nissenbaum, C; Ogese, MO; Pirmohamed, M; Semic-Jusufagic, A; Wilkinson, M1
Deane, J; Einarsson, GG; Elborn, JS; Eustace, JA; Fouhy, F; Hill, C; McCarthy, M; Mm, T; Murphy, DM; Plant, BJ; Rea, M; Ronan, NJ; Ross, RP; Shanahan, F; Stanton, C1
Arnaud, F; Bec, R; Bermudez, J; Coiffard, B; Di Bisceglie, M; Dufeu, N; Gaubert, JY; Habert, P; Naud, R; Reynaud-Gaubert, M1
Blanco Aparicio, M; Carrasco Hernández, L; Diab Cáceres, L; García Clemente, M; Girón Fernández, MV; Girón Moreno, RM; Gómez Bonilla, A; Gómez Crespo, B; González Torres, L; Olveira Fuster, C; Peláez, A; Prados Sanchez, C; Quintana Gallego, E; Solís García, M; Tejedor Ortiz, MT; Zamarrón de Lucas, E1
Neeland, MR; Ranganathan, S; Shanthikumar, S1
Barton, L; Mahlen, T; Roberts, D1
Acosta, EP; Anderson, JD; Guimbellot, JS; Parker, KL; Rowe, SM; Ryan, KJ; Victoria Odom, L1
Borgia, F; Costa, S; Cristadoro, S; Di Bartolomeo, L; Lentini, M; Li Pomi, F; Lombardo, M; Lucanto, MC; Vaccaro, M1
Almulhem, M; Brodlie, M; Graham, S; Haq, I; Harnett, N; Visram, S; Ward, C1
Baroni, D; Ludovico, A; Moran, O1
Caverly, LJ; Hisert, KB; Riquelme, SA1
Cohen-Cymberknoh, M; Jain, R; Shteinberg, M; Taylor-Cousar, JL1
Flight, WG; Heath, L; Koutoukidis, DA; Snowball, JE1
Ademhan Tural, D; Asfuroğlu, P; Başaran, E; Başkan, AK; Bingöl, A; Çağlar, HT; Çakır, E; Çaltepe, G; Can, D; Canıtez, Y; Cinel, G; Çobanoğlu, N; Çokuğraş, H; Çollak, A; Damadoğlu, E; Demir, E; Doğru, D; Emiralioğlu, N; Eryılmaz Polat, S; Hangül, M; Harmancı, K; Irmak, İ; Kartal Öztürk, G; Keskin, Ö; Kılıç, M; Kılınç, AA; Kiper, N; Korkmaz Ekren, P; Köse, M; Özcan, G; Özçelik, U; Özdemir, A; Özdoğan, Ş; Özkan Tabakçı, S; Özsezen, B; Pekcan, S; Sapan, N; Selimoğlu Şen, H; Şen, V; Şişmanlar Eyüboğlu, T; Tana Aslan, A; Topal, E; Ufuk Altıntaş, D; Ünal, G; Uytun, S; Uzuner, S; Yalçın, E; Yazan, H; Yılmaz, Aİ; Yüksel, H1
Downey, DG; Robinson, PD; Shteinberg, M; Taylor-Cousar, JL1
Beiersdorf, N; Groten, T; Jaudszus, A; Lorenz, M; Mainz, JG; Michl, RK; Schneider, U1
Bell, SC; Fridman, M; Kinnman, N; Macey, J; MacGregor, G; Madge, S; Mainz, JG; Narayanan, S; Suthoff, ED1
Connett, GJ1
Ahmadi, S; Bartlett, C; Bear, CE; Gonska, T; Ip, W; Jiang, J; Laselva, O; Lew, A; Moraes, TJ; Ouyang, H; Wellhauser, L; Wu, YS; Xia, S1
Alper, CJ; Bacon, R; Greenwood, BC; Jeffrey, PL; Lenz, K; Stevens, K; Tesell, MA1
Fitch, J; Hayes, D; Jaramillo, L; Kopp, BT; Mejias, A; Palacios, S; Partida-Sanchez, S; Ramilo, O; Robledo-Avila, F; Shrestha, CL; White, P; Woodley, F; Zhang, S1
Cui, G; Hong, JS; Imhoff, BR; McCarty, NA; Rab, A; Sorscher, EJ; Stauffer, BB1
Bellaire, S; Bwirire, D; Conrath, K; Corveleyn, S; Fischer, R; Gleiber, W; Hector, A; Kempa, A; Muller, K; Pano, A; Santermans, E; Schwarz, C; Sutharsan, S; Van de Steen, O; van Koningsbruggen-Rietschel, S; Van Osselaer, N1
Harris, JK; Heltshe, SL; Robertson, CE; Rowe, SM; Sagel, SD; Stevens, MJ; Wagner, BD; Zemanick, ET1
Akshintala, VS; Cebotaru, L; Cutting, GR; Dezube, R; Faghih, M; Jennings, MT; Kamal, A; Lechtzin, N; Merlo, CA; Singh, VK; West, NE; Whitcomb, DC1
Hollis, A1
Jain, M; Sala, MA2
Cuyx, S; De Boeck, K1
Chou, FY; Hamilton, CM; Lam, GY; Quon, BS; Sergeev, V; Wilcox, PG1
Barr, HL; Barrett, DA; Cámara, M; Fogarty, A; Halliday, N; Knox, A; Nash, EF; Smyth, AR; Stewart, I; Webb, K; Whitehouse, JL; Williams, P1
Gifford, AH; Heltshe, SL1
Douros, K; Loukou, I; Moustaki, M; Plyta, M1
Avramescu, RG; Bagdany, M; Bensinger, D; Da Fonte, DF; Lukacs, GL; Matouk, E; Premchandar, A; Schmidt, B; Stubba, D; Veit, G; Xu, H1
Accurso, FJ; Zemanick, ET1
Ahluwalia, N; Downey, DG; Heijerman, HGM; Mall, MA; Marigowda, G; McCoy, KS; McKee, CM; McKone, EF; Moskowitz, SM; Ramsey, BW; Rowe, SM; Simard, C; Sosnay, PR; Taylor-Cousar, JL; Tullis, E; Van Braeckel, E; Waltz, D; Welter, JJ; Xuan, F; Zhang, Y1
Dřevínek, P; Jain, R; Lands, LC; Mall, MA; Marigowda, G; McKee, CM; McKone, EF; Middleton, PG; Moskowitz, SM; Nair, N; Polineni, D; Ramsey, BW; Rowe, SM; Savage, J; Simard, C; Taylor-Cousar, JL; Tian, S; Tullis, E; Vermeulen, F; Waltz, D; Xuan, F1
Ehrhardt, A; Hong, JS; Joshi, D; Sorscher, EJ1
Daley, CP; Pallin, M1
Higgins, M; Jones, MC; Lan, L; Nick, JA; St Clair, C1
Hoy, SM1
Magaret, AS; Mayer-Hamblett, N; VanDevanter, D1
Guimbellot, JS; Heltshe, SL; Jain, M; Jovanovic, B; Rowe, SM; Sagel, SD; Secunda, KE1
Barker, D; Burr, L; Dorahy, D; France, M; Greville, H; Middleton, P; Tong, K; Visser, S; Wainwright, C; Wark, P1
Mall, MA; Mayer-Hamblett, N; Rowe, SM1
Hinzpeter, A; Sermet-Gaudelus, I; Sheppard, DN1
Mulrennan, S; Popowicz, N; Vekaria, S; White, SW1
Belarski, E; Pettit, R1
Durieu, I; Garcia, S; Nave, V; Olivereau, L; Perceval, M; Rabilloud, M; Reynaud, Q1
Aschenbrenner, DS1
Carson, S; Christianson, MS; Claudio, AT; Lechtzin, N; Montemayor, K; West, NE1
Acosta, EP; Anderson, JD; Esther, CR; Guimbellot, JS; Kersh, L; Liu, Z; Rowe, SM; Ryan, KJ1
McGarry, ME1
Cryan, SA; Cutrona, MB; De Santi, C; Fernández Fernández, E; Gaul, R; Glasgow, A; Greene, CM; Harvey, BJ; Hawkins, F; Henshall, DC; Hurley, K; Linnane, B; MacLoughlin, R; McNally, P; Mitash, N; Mu, F; Oglesby, IK; Raoof, R; Simpson, JC; Swiatecka-Urban, A; Vencken, S1
Beekman, JM; de Winter-de Groot, KM; Hagemeijer, MC; Janssens, HM; Korkmaz, C; Kruisselbrink, E; van der Ent, CK; van Haaren, P; Van Mourik, P1
Bear, CE; Deber, CM; Laselva, O; Stone, TA1
Clifton, I; Etherington, C; Holbrook, J; Jarosz-Griffiths, HH; Lara-Reyna, S; Martinon, F; McDermott, MF; Mehta, A; Peckham, D; Savic, S; Scambler, T; Spoletini, G; Whitaker, P; Wong, CH1
Daniels, MLA; Ebert, CS; Farzal, Z; Kimple, AJ; Lee, SE; Senior, BA; Thorp, BD; Zanation, AM1
Fajac, I; Girodon, E1
Abely, M; Bounyar, L; Chatron, E; Chiron, R; Cosson, L; Dominique, S; Durieu, I; Gerardin, M; Kessler, L; Kessler, R; Mankikian, J; Melly, L; Misgault, B; Porzio, M; Reynaud, Q; Ronsin-Pradel, O; Touzet, S; Troussier, F; Weiss, L1
Berkers, GG; Burghard, MM; de Winter-de Groot, KK; Ghijsen, SS; Heijerman, HH; Hollander-Kraaijeveld, FF; Hulzebos, HE; Takken, TT; van der Ent, CK1
Cavallaro, G; Di Leonardo, A; Giammona, G; Lentini, L; Porsio, B; Quaglia, F; Ungaro, F1
Edwards, LJ; Hathorne, HY; Mutyam, V; Peabody Lever, JE; Peng, N; Rowe, SM; Sharma, J1
Hjelm, M; Shaikhkhalil, AK1
Cottrill, KA; Farinha, CM; McCarty, NA1
Horgan, M; Kerr, C; Morrissy, D; Plant, BJ1
Doull, I2
Egan, ME2
Aller, SG; Bacsa, J; Breton, GW; Doiron, JE; Le, CA; Martin, KL; Turlington, M1
Carr, SB; Davies, JC; Jones, D; Nichols, AL1
Kessler, L1
Afzal, S; Çolak, Y; Nordestgaard, BG1
Françoise, A; Héry-Arnaud, G1
Barrow, KA; Debley, JS; McKone, EF; Nichols, DP; Rich, LM; Singh, P1
Aalbers, BL; Arets, HGM; Bronsveld, I; de Kiviet, AC; de Winter-de Groot, KM; Heijerman, HGM; Hofland, RW; Kruijswijk, MA; Michel, S; Schotman, S; van der Ent, CK; van Oirschot-van de Ven, MMM1
Al Shadfan, LM; Altıntaş, DU; Aslan, AT; Başaran, AE; Bingöl, A; Çakır, E; Çaltepe, G; Can, D; Çekiç, Ş; Çelebioğlu, E; Cinel, G; Çobanoğlu, N; Çokuğraş, H; Demir, E; Doğru, D; Emiralioğlu, N; Ercan, Ö; Gürsoy, TR; Hangül, M; Harmancı, K; Karagöz, D; Kartal Öztürk, G; Kılıç, M; Kılınç, AA; Kiper, N; Korkmaz Ekren, P; Köse, M; Özcan, G; Özçelik, U; Özdemir, A; Özdoğan, Ş; Pekcan, S; Sapan, N; Şen, HS; Şen, V; Şişmanlar Eyüboğlu, T; Süleyman, A; Tamay, Z; Topal, E; Tuğcu, G; Yalçın, E; Yazan, H; Yıldırım, GK; Yılmaz, Ö; Yüksel, H1
Becq, F; Cantereau, A; Froux, L; Llinares, J1
Boyle, KG; Dhillon, SS; Guenette, JA; Mitchell, RA; Quon, BS; Ramsook, AH; Wilcox, PG1
Beekman, JM; Berkers, G; de Jonge, HR; de Rijke, YB; Heijerman, HG; Janssens, HM; Kemper, EM; Koppelman, GH; Kruisselbrink, E; Majoor, CJ; Roukema, J; Suen, SW; van der Ent, CK; van der Meer, R; van Mourik, P; Vonk, AM1
Ballmann, M; Glass, A; Holl, R; Prinz, N1
Keeling, KM; Rowe, SM; Sharma, J1
Clancy, JP; Donaldson, SH; Khan, U; Ratjen, F; Rowe, SM; Sagel, SD; Shaw, M1
Bridges, RJ; Hastings, ML; Michaels, WE1
Mall, MA; Naisbitt, DJ; Ogese, MO; Roehmel, JF; Rohrbach, A1
Cholon, DM; Esther, CR; Gentzsch, M; Guhr Lee, TN; Quinney, NL1
Ahluwalia, N; Campbell, D; Ellemunter, H; Kerem, E; Munck, A; Owen, CA; Wainwright, C; Wang, LT1
Birket, SE; Chambers, A; Davis, JM; Fernandez-Petty, CM; Fields, A; Fu, L; Henderson, AG; Hong, J; Oden, AM; Rowe, SM; Sorscher, EJ; Tang, L; Tearney, GJ; Wen, H; Zhao, G1
Caputo, M; Carnovale, V; Castaldo, G; Cernera, G; Comegna, M; Corso, G; Gelzo, M; Iacotucci, P1
George, A; Goetz, DM; Sawicki, GS; Smith, B1
Brown, J; de Vries, JM; Fabbrini, AL; Green, D; Kidder, M; Kucera, JN; Wilsey, M1
Abely, M; Biouhee, T; Bui, S; Burgel, PR; Chiron, R; Corvol, H; Dehillotte, C; Durieu, I; Hubert, D; Lemonnier, L; Macey, J; Marguet, C; Martin, C; Mely, L; Munck, A; Murris-Espin, M; Paillasseur, JL; Porzio, M; Prevotat, A; Reix, P; Sermet-Gaudelus, I; Silva, JD1
Aureli, M; Bassi, R; Cabrini, G; Chiricozzi, E; Dechecchi, MC; Lippi, G; Loberto, N; Mancini, G; Mauri, L; Olioso, D; Pedemonte, N; Pesce, E; Schiumarini, D; Sonnino, S; Tamanini, A1
Finnegan, R; O'Grady, E; Ryan, S; Smyth, A; Williamson, M1
Aridgides, DS; Armstrong, DA; Ashare, A; Dessaint, JA; Hampton, TH; Hazlett, HF; Mellinger, DL; Nymon, AB1
Afonso, S; Amaral, MD; Botelho, HM; da Paula, AC; Farinha, CM; Felício, V; Lobo, MJ; Uliyakina, I1
Aissat, A; Bizard, L; Decrouy, X; Degrugillier, F; Fanen, P; Jiang, C; Prulière-Escabasse, V; Rotin, D; Simon, S; Simonneau, B1
Armirotti, A; Bandiera, T; Bertozzi, SM; Braccia, C; Giraudo, A; Liessi, N; Pedemonte, N; Pesce, E1
Finkbeiner, WE; Haggie, PM; Janahi, IA; Nielson, DW; Phuan, PW; Rivera, AA; Tan, JA; Thomas, MM; Verkman, AS1
Rayment, JH; Smith, H1
Arest, CF; De Jong, E; Falsafi, R; Garratt, LW; Hancock, REW; Hillas, J; Iosifidis, T; Kicic, A; Kicic-Starcevich, E; Lannigan, FJ; Lee, AHY; Ling, KM; Looi, K; Martinovich, KM; Montgomery, ST; Shaw, NC; Smith, ML; Stick, SM; Sutanto, EN; Vijayasekaran, S1
Beekman, JM; Graeber, SY; Hirtz, S; Kruisselbrink, E; Mall, MA; van der Ent, CK; van Mourik, P; Vonk, AM1
Berkers, G; Bittermann, J; de Winter-de Groot, K; Gostelie, R; Kipshagen, PV; Ligtenberg-van der Drift, I; Speleman, L; Stegeman, I1
Hisert, KB1
Dansky, D; DiMango, E; Francis, SF; Gudis, D; Keating, C; Overdevest, J1
Amaral, MD; Botelho, HM; Centeio, R; Clarke, LA; Doušová, T; Farinha, CM; Holubová, A; Hwang, TC; Kunzelmann, K; Railean, V; Ramalho, S; Silva, IAL; Valášková, I; Yeh, JT1
Clausen, E; Dezube, R; Grant, JJ; Hong, G; Safirstein, J; Savant, D1
Beaufils, F; Bui, S; Burgel, PR; Delhaes, L; Enaud, R; Fayon, M; Lamireau, T; Mas, E; Maumus, P; Mittaine, M; Tétard, C1
Bartlett, C; Bear, CE; Gonska, T; Gunawardena, TNA; Laselva, O; Moraes, TJ; Ouyang, H; Popa, A1
Middleton, PG; Palacios, AC; Song, Y; Thiagalingam, A1
Xia, X1
Biglia, C; Calderazzo, MA; Caloiero, M; Cresta, F; De Venuto, D; Francalanci, M; Leonetti, G; Majo, F; Messore, B; Pisi, G; Pizzamiglio, G; Salvatore, D; Taccetti, G; Terlizzi, V1
Ratjen, F; Stanojevic, S; Stephenson, AL; Sykes, J; Tullis, E; Vukovojac, K1
Carter, S; Gray, RD; Grogan, B; Hardisty, GR; Law, SM; McKone, EF; Singh, PK1
DeVito, NJ; Holman, B; Vassar, M1
McGarry, M1
Ejiofor, LCK; Jensen-Fangel, S; Mathiesen, IHM; Olesen, HV; Pedersen, CL; Philipsen, LKD; Pressler, T; Skov, M1
Akong, K; Lim, M; Munce, D1
Antos, N; Beisang, D; Billings, J; Frederick, C; Heltshe, SL; Mann, M; Moheet, A; Moran, A; Rowe, SM; Sagel, SD; VanDalfsen, JM; Zhang, L1
Edelman, A; Gauthier, S; Guerrera, IC; Hinzpeter, A; Jung, V; Martignetti, L; Nguyen-Khoa, T; Pranke, I; Semeraro, M; Sermet-Gaudelus, I; Stoven, V1
Hutchinson, I; McNally, P1
Ahluwalia, N; Campbell, D; Davies, JC; Harris, RS; Haseltine, E; Naehrlich, L; Owen, CA; Panorchan, P; Saunders, C; Sermet-Gaudelus, I; Short, C; Wainwright, CE1
Chapman, RH; Kuntz, KM; Wherry, K; Williamson, I1
Cheung, A; Haggie, PM; Kurth, MJ; Lipman, E; Phuan, PW; Son, JH; Tantillo, DJ; Tsui, KY; Verkman, AS; Zhu, JS1
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Retsch-Bogart, GZ1
Arumugam, V; Ashlock, M; Burton, B; Cao, D; Decker, C; Frizzell, RA; Grootenhuis, PD; Hadida, S; Hazlewood, A; Joubran, J; McCartney, J; Negulescu, P; Neuberger, T; Olson, ER; Singh, A; Turnbull, A; Van Goor, F; Wine, JJ; Yang, J; Young, C; Zhou, J1
Accurso, FJ; Aitken, ML; Ashlock, MA; Boyle, MP; Campbell, PW; Clancy, JP; Donaldson, SH; Dong, Q; Dunitz, JM; Durie, PR; Freedman, SD; Hornick, DB; Konstan, MW; Mayer-Hamblett, N; Moss, RB; Olson, ER; Ordoñez, CL; Pilewski, JM; Ramsey, BW; Rose, LM; Rowe, SM; Rubenstein, RC; Sagel, SD; Stone, AJ; Uluer, AZ; Zha, J1
Welsh, MJ1
Coulter, WA; Dooley, JS; Elborn, JS; Goldsmith, CE; Lowery, CJ; Maeda, Y; Mason, C; Matsuda, M; Millar, BC; Moore, JE; Murayama, M; Rendall, JC1
Kassen, R; Wong, A1
Antoniu, SA1
Borja Segade, J; Castro Jiménez, A; Feo Brito, FJ; Galindo Bonilla, PA; García Rodríguez, R; Gómez Torrijos, E; Lara de la Rosa, P1
Dolgin, E2
Bear, CE; Kim Chiaw, P; Yu, W1
Sheridan, C1
Opar, A1
Bolger, GB; Clancy, JP; Ehrhardt, A; Fan, L; Li, Y; Mitchell, LH; Naren, AP; Pyle, LC; Ren, A; Rowe, SM; Sorscher, EJ1
Bell, SC; Davies, J; Dong, Q; Dřevínek, P; Elborn, JS; Griese, M; Konstan, MW; McElvaney, NG; McKone, EF; Moss, R; Ordoñez, C; Ramsey, BW; Ratjen, F; Rodriguez, S; Rowe, SM; Sermet-Gaudelus, I; Tullis, E; Wainwright, CE; Yen, K1
Bozdoğan, B; Coban, AY; Durupınar, B; Erturan, Z; Tanrıverdi Çaycı, Y; Yıldırım, T1
Farrell, PM; Sanders, DB1
Burton, B; Cao, D; Hoffman, BJ; Huang, CJ; Johnson, JP; Joubran, J; Seepersaud, S; Sussky, K; Urrutia, A; Van Goor, F; Worley, J; Yu, H1
Kaiser, J1
Borowitz, DS; Flume, PA; Geller, DE; Li, H; Liou, TG; Ordoñez, CL; Yen, K1
Beall, R1
Ledford, H1
Bush, A; Simmonds, NJ1
Corbyn, Z1
Davis, PB; Kirkpatrick, P; Yasothan, U1
Morrow, T1
Hull, J1
Davies, JC; Pabary, R; Thursfield, R1
Alagely, A; Barrett, PM; Topol, EJ1
Arora, K; Donnellan, CA; Kappes, JC; Khan, FA; Naren, AP; Penmatsa, H; Ren, A; Srinivasan, S; Stokes, DC; Yarlagadda, S; Zhang, W1
Hoffman, LR; Ramsey, BW1
Polenakovik, HM; Sanville, B1
Anderson, L; Boucher, R; Collier, DN; Gilligan, P; Knowles, M; McKnight, SL; Noah, TL; Pesci, EC; Schwab, U1
Aebersold, R; Eng, J; Goodlett, DR; Guina, T; Miller, SI; Purvine, SO; Yi, EC1
Marchese, A; Roveta, S; Schito, AM; Schito, GC1
Kitade, M; Moritaka, T; Nakanishi, N; Ueda, N1
Hodson, ME1

Reviews

110 review(s) available for carbostyril and Cystic Fibrosis of Pancreas

ArticleYear
Ethical Dilemma: Elexacaftor-Tezacaftor-Ivacaftor or Lung Transplantation in Cystic Fibrosis and End-Stage Lung Disease?
    Chest, 2022, Volume: 161, Issue:3

    Topics: Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Lung; Lung Transplantation; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: Early report.
    European journal of radiology, 2022, Volume: 154

    Topics: Adult; Aminophenols; Benzodioxoles; Cystic Fibrosis; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Reproducibility of Results; Retrospective Studies; Tomography, X-Ray Computed

2022
The Impact of Highly Effective Modulator Therapy on Cystic Fibrosis Microbiology and Inflammation.
    Clinics in chest medicine, 2022, Volume: 43, Issue:4

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Inflammation; Mutation; Quality of Life; Quinolones

2022
CFTR modulator therapy: transforming the landscape of clinical care in cystic fibrosis.
    Lancet (London, England), 2023, 09-30, Volume: 402, Issue:10408

    Topics: Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Mutation; Quinolones

2023
Lumacaftor-ivacaftor in the treatment of cystic fibrosis: design, development and place in therapy.
    Drug design, development and therapy, 2019, Volume: 13

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Design; Drug Therapy, Combination; Humans; Quinolones

2019
Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis.
    Seminars in respiratory and critical care medicine, 2019, Volume: 40, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Indoles; Mutation; Quinolones; Randomized Controlled Trials as Topic

2019
The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis.
    Annals of the American Thoracic Society, 2020, Volume: 17, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Quinolones; Signal Transduction

2020
Cystic fibrosis precision therapeutics: Emerging considerations.
    Pediatric pulmonology, 2019, Volume: 54 Suppl 3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Precision Medicine; Quinolones

2019
Elexacaftor/Ivacaftor/Tezacaftor: First Approval.
    Drugs, 2019, Volume: 79, Issue:18

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; United States; United States Food and Drug Administration

2019
Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications.
    American journal of respiratory and critical care medicine, 2020, 05-15, Volume: 201, Issue:10

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Sodium Channel Blockers; Humans; Indoles; Molecular Targeted Therapy; Mucociliary Clearance; Mutation; Precision Medicine; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2020
Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy: A Review for the Otolaryngologist.
    American journal of rhinology & allergy, 2020, Volume: 34, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chronic Disease; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Humans; Indoles; Mutation; Nasal Mucosa; Otolaryngologists; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2020
The bidirectional relationship between CFTR and lipids.
    Communications biology, 2020, 04-20, Volume: 3, Issue:1

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Epithelial Cells; Humans; Lung; Membrane Lipids; Membrane Microdomains; Mutation; Protein Conformation; Protein Stability; Protein Transport; Quinolones; Structure-Activity Relationship

2020
Cystic fibrosis 2019: Year in review.
    Paediatric respiratory reviews, 2020, Volume: 35

    Topics: Administration, Inhalation; Aminophenols; Aminopyridines; Anti-Bacterial Agents; Azithromycin; Benzodioxoles; Carrier State; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Exercise; Health Services Accessibility; Healthcare Disparities; Humans; Indoles; Inflammation; Physical Therapy Modalities; Pseudomonas Infections; Pyrazoles; Pyridines; Quinolines; Quinolones; Saline Solution, Hypertonic

2020
Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update.
    Current opinion in pediatrics, 2020, Volume: 32, Issue:3

    Topics: Aminophenols; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quality of Life; Quinolones; Treatment Outcome

2020
Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor.
    Paediatric respiratory reviews, 2020, Volume: 35

    Topics: Adolescent; Age Factors; Aminophenols; Carrier Proteins; Chloride Channel Agonists; Cystic Fibrosis; Duration of Therapy; Enzyme Replacement Therapy; Exocrine Pancreatic Insufficiency; Feces; Female; Humans; Pancreatic Elastase; Quinolones; Recovery of Function

2020
The Microbiome in Cystic Fibrosis Pulmonary Disease.
    Genes, 2020, 05-11, Volume: 11, Issue:5

    Topics: Aminophenols; Animals; Archaea; Bacteria; Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Disease Progression; Dysbiosis; Fungi; Gastrointestinal Microbiome; Genotype; High-Throughput Nucleotide Sequencing; Humans; Lung; Mammals; Metagenomics; Microbiota; Organ Specificity; Prognosis; Quinolones; Viruses

2020
Pharmacological approaches for targeting cystic fibrosis nonsense mutations.
    European journal of medicinal chemistry, 2020, Aug-15, Volume: 200

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Codon, Nonsense; Cystic Fibrosis; Dose-Response Relationship, Drug; Humans; Indoles; Molecular Structure; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Structure-Activity Relationship

2020
Development of elexacaftor - tezacaftor - ivacaftor: Highly effective CFTR modulation for the majority of people with Cystic Fibrosis.
    Expert review of respiratory medicine, 2021, Volume: 15, Issue:6

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Entering the era of highly effective modulator therapies.
    Pediatric pulmonology, 2021, Volume: 56 Suppl 1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Modulators of CFTR. Updates on clinical development and future directions.
    European journal of medicinal chemistry, 2021, Mar-05, Volume: 213

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Development; Humans; Indoles; Quinolones

2021
Elexacaftor-tezacaftor-ivacaftor: The new paradigm to treat people with cystic fibrosis with at least one p.Phe508del mutation.
    Current opinion in pharmacology, 2021, Volume: 57

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Cystic fibrosis transmembrane conductance regulator modulators for cystic fibrosis: a new dawn?
    Archives of disease in childhood, 2021, Volume: 106, Issue:10

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genetic Variation; Humans; Indoles; Outcome Assessment, Health Care; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
A new era for people with cystic fibrosis.
    European journal of pediatrics, 2021, Volume: 180, Issue:9

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quality of Life; Quinolones

2021
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis.
    International journal of molecular sciences, 2021, Jul-16, Volume: 22, Issue:14

    Topics: Aminophenols; Aminopyridines; Anti-Inflammatory Agents; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Inflammation; Ion Transport; Lung; Macrophages; Pneumonia; Quinolones; Signal Transduction

2021
New treatments targeting the basic defects in cystic fibrosis.
    Presse medicale (Paris, France : 1983), 2017, Volume: 46, Issue:6 Pt 2

    Topics: Adolescent; Adult; Aminophenols; Child; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Exocrine Pancreatic Insufficiency; Genetic Therapy; Humans; Infant; Infant, Newborn; Interdisciplinary Communication; Intersectoral Collaboration; Life Expectancy; Phenotype; Prognosis; Quinolones; Young Adult

2017
Innovating cystic fibrosis clinical trial designs in an era of successful standard of care therapies.
    Current opinion in pulmonary medicine, 2017, Volume: 23, Issue:6

    Topics: Aminophenols; Aminopyridines; Anti-Bacterial Agents; Benzodioxoles; Chloride Channel Agonists; Clinical Trials as Topic; Comparative Effectiveness Research; Cystic Fibrosis; Disease Progression; Drug Combinations; Humans; Pseudomonas Infections; Quinolones; Standard of Care; Treatment Outcome

2017
Comparing effectiveness and outcomes in asthma and cystic fibrosis.
    Paediatric respiratory reviews, 2017, Volume: 24

    Topics: Administration, Inhalation; Adrenal Cortex Hormones; Adrenergic beta-Agonists; Aminophenols; Anti-Bacterial Agents; Asthma; Chloride Channel Agonists; Comparative Effectiveness Research; Cost-Benefit Analysis; Cystic Fibrosis; Equivalence Trials as Topic; Humans; Leukotriene Antagonists; Precision Medicine; Quality-Adjusted Life Years; Quinolones; Treatment Outcome

2017
Effects of new and emerging therapies on gastrointestinal outcomes in cystic fibrosis.
    Current opinion in pulmonary medicine, 2017, Volume: 23, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Body Mass Index; Chloride Channel Agonists; Cystic Fibrosis; Digestive System; Drug Combinations; Gastroesophageal Reflux; Humans; Hydrogen-Ion Concentration; Intestinal Diseases; Liver Diseases; Probiotics; Quinolones; Treatment Outcome; Weight Gain

2017
Diagnosis, follow-up and treatment of cystic fibrosis-related liver disease.
    Current opinion in pulmonary medicine, 2017, Volume: 23, Issue:6

    Topics: Aftercare; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cholagogues and Choleretics; Cystic Fibrosis; Drug Combinations; Elasticity Imaging Techniques; Humans; Liver Diseases; Liver Transplantation; Quinolones; Ursodeoxycholic Acid

2017
The safety of lumacaftor and ivacaftor for the treatment of cystic fibrosis.
    Expert opinion on drug safety, 2017, Volume: 16, Issue:11

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytochrome P-450 CYP3A; Drug Combinations; Drug Interactions; Humans; Mutation; Quinolones

2017
Lumacaftor/ivacaftor, a novel agent for the treatment of cystic fibrosis patients who are homozygous for the F580del CFTR mutation.
    Expert review of clinical pharmacology, 2017, Volume: 10, Issue:10

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Forced Expiratory Volume; Humans; Molecular Targeted Therapy; Mutation; Quinolones

2017
Transformative therapies for rare CFTR missense alleles.
    Current opinion in pharmacology, 2017, Volume: 34

    Topics: Alleles; Aminophenols; Animals; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Variation; Humans; Mutation, Missense; Quinolones

2017
CFTR potentiators: from bench to bedside.
    Current opinion in pharmacology, 2017, Volume: 34

    Topics: Adenosine Triphosphate; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Precision Medicine; Quinolones

2017
Corrector combination therapies for F508del-CFTR.
    Current opinion in pharmacology, 2017, Volume: 34

    Topics: Aminophenols; Binding Sites; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Mutation; Quinolones

2017
Cystic fibrosis transmembrane conductance regulator modulators: precision medicine in cystic fibrosis.
    Current opinion in pediatrics, 2018, Volume: 30, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Clinical Trials, Phase II as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Indoles; Precision Medicine; Quinolones; Respiratory System Agents

2018
Cystic fibrosis papers of the year 2017.
    Paediatric respiratory reviews, 2018, Volume: 27

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Management; Drug Combinations; Humans; Lung; Membrane Transport Modulators; Mutation; Quinolones; Respiratory Function Tests; Time

2018
The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Canada; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genetic Therapy; Humans; Incidence; Mutation, Missense; Precision Medicine; Program Development; Quinolones; RNA

2019
The expanding horizon of alkyl quinolone signalling and communication in polycellular interactomes.
    FEMS microbiology letters, 2018, 05-01, Volume: 365, Issue:9

    Topics: Animals; Cystic Fibrosis; Host-Pathogen Interactions; Humans; Microbial Interactions; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Signal Transduction

2018
Ion Channel Modulators in Cystic Fibrosis.
    Chest, 2018, Volume: 154, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genotype; Humans; Indoles; Ion Channels; Precision Medicine; Quinolones

2018
Cystic Fibrosis: Translating Molecular Mechanisms into Effective Therapies.
    Annals of the American Thoracic Society, 2018, Volume: 15, Issue:8

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Development; Humans; Indoles; Molecular Targeted Therapy; Mutation; Quinolones

2018
Recent advances in developing therapeutics for cystic fibrosis.
    Human molecular genetics, 2018, 08-01, Volume: 27, Issue:R2

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Genotype; Humans; Mutation; Phenotype; Quinolones

2018
Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
    The Cochrane database of systematic reviews, 2018, 08-02, Volume: 8

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genetic Therapy; Humans; Indoles; Mutation; Phenylbutyrates; Quinolones; Randomized Controlled Trials as Topic

2018
Tezacaftor for the treatment of cystic fibrosis.
    Expert review of respiratory medicine, 2018, Volume: 12, Issue:9

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genotype; Humans; Indoles; Mutation; Quinolones

2018
Theratyping in cystic fibrosis.
    Current opinion in pulmonary medicine, 2018, Volume: 24, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Gene Editing; Genetic Therapy; Genotype; Humans; Indoles; Mutation; Organoids; Oxadiazoles; Precision Medicine; Quinolones

2018
CFTR modulator therapy in patients with cystic fibrosis and an organ transplant.
    Paediatric respiratory reviews, 2018, Volume: 27

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Male; Molecular Targeted Therapy; Organ Transplantation; Quinolones; Young Adult

2018
Efficacy and Safety of CFTR Corrector and Potentiator Combination Therapy in Patients with Cystic Fibrosis for the F508del-CFTR Homozygous Mutation: A Systematic Review and Meta-analysis.
    Advances in therapy, 2019, Volume: 36, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Combined Modality Therapy; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Homozygote; Humans; Mutation; Quinolones

2019
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
    The Cochrane database of systematic reviews, 2019, 01-07, Volume: 1

    Topics: Adult; Age Factors; Aminophenols; Child; Chloride Channel Agonists; Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Humans; Molecular Targeted Therapy; Mucociliary Clearance; Mutation; Quality of Life; Quinolones; Randomized Controlled Trials as Topic

2019
Cystic fibrosis transmembrane conductance regulator modulators: Present and future in cystic fibrosis treatment. A review.
    Archivos argentinos de pediatria, 2019, 04-01, Volume: 117, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Precision Medicine; Quality of Life; Quinolones

2019
Prevention of cystic fibrosis: The beginning of the end?
    Science translational medicine, 2019, 03-27, Volume: 11, Issue:485

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Ferrets; Pregnancy; Quinolones

2019
Safety and efficacy of treatment with lumacaftor in combination with ivacaftor in younger patients with cystic fibrosis.
    Expert review of respiratory medicine, 2019, Volume: 13, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Child, Preschool; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Quinolones; Treatment Outcome

2019
Newborn screening for cystic fibrosis: Is there benefit for everyone?
    Paediatric respiratory reviews, 2019, Volume: 31

    Topics: Aminophenols; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; False Positive Reactions; Health Policy; Humans; Infant, Newborn; Mutation; Neonatal Screening; Penetrance; Phenotype; Quinolones; Risk Assessment; Sweat

2019
Clinical papers of the year 2018 - Cystic fibrosis.
    Paediatric respiratory reviews, 2020, Volume: 33

    Topics: Administration, Inhalation; Aminophenols; Aminopyridines; Anti-Bacterial Agents; Azithromycin; Benzodioxoles; Bronchoalveolar Lavage; Bronchoalveolar Lavage Fluid; Chloride Channel Agonists; Cough; Cross Infection; Cystic Fibrosis; Disease Progression; Drug Combinations; Drug Therapy, Combination; Humans; Indoles; Lung Transplantation; Microbiological Techniques; Mycobacterium abscessus; Mycobacterium Infections, Nontuberculous; Nebulizers and Vaporizers; Proton Pump Inhibitors; Pseudomonas aeruginosa; Pseudomonas Infections; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Randomized Controlled Trials as Topic; Saline Solution, Hypertonic; Specimen Handling; Sputum; Treatment Outcome; Tuberculosis, Pulmonary

2020
A Systematic Review of the Clinical Efficacy and Safety of CFTR Modulators in Cystic Fibrosis.
    Scientific reports, 2019, 05-10, Volume: 9, Issue:1

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Gene Deletion; Humans; Indoles; Polymorphism, Single Nucleotide; Quinolones; Treatment Outcome

2019
Cystic Fibrosis-Associated Liver Disease in Lung Transplant Recipients.
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society, 2019, Volume: 25, Issue:8

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Incidence; Liver Cirrhosis; Liver Transplantation; Lung Transplantation; Mutation; Postoperative Care; Quinolones; Survival Rate; Transplant Recipients; Treatment Outcome; Ursodeoxycholic Acid

2019
A systematic Cochrane Review of correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
    Paediatric respiratory reviews, 2019, Volume: 30

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Indoles; Quinolones; Treatment Outcome

2019
Pharmacological analysis of CFTR variants of cystic fibrosis using stem cell-derived organoids.
    Drug discovery today, 2019, Volume: 24, Issue:11

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biological Assay; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; Molecular Targeted Therapy; Mutation; Organoids; Pharmacogenomic Variants; Quinolones; Stem Cells

2019
Beyond cystic fibrosis transmembrane conductance regulator therapy: a perspective on gene therapy and small molecule treatment for cystic fibrosis.
    Gene therapy, 2019, Volume: 26, Issue:9

    Topics: Aminophenols; Animals; CRISPR-Cas Systems; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gene Transfer Techniques; Genetic Therapy; Genetic Vectors; Humans; Quinolones

2019
Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.
    European respiratory review : an official journal of the European Respiratory Society, 2013, Mar-01, Volume: 22, Issue:127

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Discovery; Genetic Predisposition to Disease; Genetic Therapy; High-Throughput Screening Assays; Humans; Molecular Targeted Therapy; Mutation; Phenotype; Precision Medicine; Prognosis; Protein Conformation; Quinolones; Respiratory System Agents; Structure-Activity Relationship

2013
Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.
    European respiratory review : an official journal of the European Respiratory Society, 2013, Mar-01, Volume: 22, Issue:127

    Topics: Adolescent; Adult; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Predisposition to Disease; Humans; Lung; Molecular Targeted Therapy; Mutation; Patient Selection; Phenotype; Precision Medicine; Quinolones; Respiratory System Agents; Sweat; Time Factors; Treatment Outcome; Young Adult

2013
Cystic fibrosis in the era of genomic medicine.
    Current opinion in pediatrics, 2013, Volume: 25, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genomics; Humans; Molecular Targeted Therapy; Mutation; Quinolones; Respiratory Tract Infections

2013
[Cystic fibrosis: new treatments targeting the CFTR protein].
    Revue des maladies respiratoires, 2013, Volume: 30, Issue:4

    Topics: Adult; Aminophenols; Animals; Combined Modality Therapy; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Molecular Targeted Therapy; Quinolones; Therapies, Investigational

2013
Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors.
    Paediatric drugs, 2013, Volume: 15, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Molecular Targeted Therapy; Mutation; Quinolones

2013
Cystic fibrosis transmembrane regulator correctors and potentiators.
    Cold Spring Harbor perspectives in medicine, 2013, Jul-01, Volume: 3, Issue:7

    Topics: Aminophenols; Aminopyridines; Bayes Theorem; Benzodioxoles; Chloride Channels; Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Discovery; Humans; Mutation; Quinolones; Randomized Controlled Trials as Topic; Technology, Pharmaceutical

2013
Advances in personalized medicine - medicinal chemistry and pharmacology of vemurafenib and ivacaftor.
    Die Pharmazie, 2013, Volume: 68, Issue:7

    Topics: Aminophenols; Animals; Chemistry, Pharmaceutical; Clinical Trials, Phase I as Topic; Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Drug Evaluation, Preclinical; Drug Resistance, Neoplasm; Humans; Indoles; Mitogen-Activated Protein Kinases; Precision Medicine; Quinolones; Sulfonamides; Vemurafenib

2013
Carrier screening for cystic fibrosis in the new era of medications that restore CFTR function.
    Lancet (London, England), 2014, Mar-08, Volume: 383, Issue:9920

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Carrier Screening; Humans; Molecular Targeted Therapy; Mutation; Quinolones

2014
Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence.
    Therapeutic advances in respiratory disease, 2013, Volume: 7, Issue:5

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Predisposition to Disease; Humans; Lung; Molecular Targeted Therapy; Mutation; Phenotype; Quinolones; Respiratory System Agents; Treatment Outcome

2013
Ivacaftor: a review of its use in patients with cystic fibrosis.
    Drugs, 2013, Volume: 73, Issue:14

    Topics: Aminophenols; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Double-Blind Method; Humans; Quinolones

2013
Development, clinical utility, and place of ivacaftor in the treatment of cystic fibrosis.
    Drug design, development and therapy, 2013, Volume: 7

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Design; Humans; Molecular Targeted Therapy; Mutation; Quality of Life; Quinolones

2013
Modifying disease in cystic fibrosis: current and future therapies on the horizon.
    Current opinion in pulmonary medicine, 2013, Volume: 19, Issue:6

    Topics: Aminophenols; Biomarkers; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genetic Markers; Genetic Therapy; Humans; Male; Mannitol; Mutation; Phenotype; Quinolones; Respiratory System Agents; Saline Solution, Hypertonic

2013
CFTR protein repair therapy in cystic fibrosis.
    Archivos de bronconeumologia, 2014, Volume: 50, Issue:4

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Molecular Targeted Therapy; Mutation; Quinolones

2014
Inhaled antibiotics to treat lung infection.
    Pharmaceutical patent analyst, 2013, Volume: 2, Issue:5

    Topics: Administration, Inhalation; Aerosols; Aminoglycosides; Animals; Anti-Bacterial Agents; Chemistry, Pharmaceutical; Cystic Fibrosis; Dry Powder Inhalers; Humans; Lung Diseases; Monobactams; Nebulizers and Vaporizers; Patents as Topic; Quinolones; Respiratory Tract Infections

2013
[Therapeutic update in cystic fibrosis].
    La Revue de medecine interne, 2014, Volume: 35, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Oxadiazoles; Quinolones

2014
A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect.
    The Lancet. Respiratory medicine, 2013, Volume: 1, Issue:2

    Topics: Aminophenols; Biological Transport; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forecasting; Humans; Mutation; Oxadiazoles; Precision Medicine; Protein Biosynthesis; Quinolones; Respiratory System Agents; Treatment Outcome

2013
Ivacaftor for the treatment of patients with cystic fibrosis and the G551D mutation: a systematic review and cost-effectiveness analysis.
    Health technology assessment (Winchester, England), 2014, Volume: 18, Issue:18

    Topics: Adolescent; Adult; Age Factors; Aminophenols; Child; Cost-Benefit Analysis; Cystic Fibrosis; England; Female; Humans; Lung Transplantation; Male; Models, Economic; Mutation; Quality-Adjusted Life Years; Quinolones; Randomized Controlled Trials as Topic; Respiratory Function Tests; Sex Factors; State Medicine

2014
Cystic fibrosis: a review of pathophysiology and current treatment recommendations.
    South Dakota medicine : the journal of the South Dakota State Medical Association, 2014, Volume: 67, Issue:4

    Topics: Aminophenols; Anti-Bacterial Agents; Anti-Inflammatory Agents; Bronchodilator Agents; Cystic Fibrosis; Enzyme Replacement Therapy; Expectorants; Humans; Quinolones; Vitamins

2014
[Two news drugs (ivacaftor & bedaquiline), one biomarker (florbetapir) and a re-positioned drug (propranolol) on the market].
    Annales pharmaceutiques francaises, 2014, Volume: 72, Issue:4

    Topics: Adrenergic beta-Antagonists; Adult; Alzheimer Disease; Aminophenols; Aniline Compounds; Antitubercular Agents; Cystic Fibrosis; Diarylquinolines; Drug Resistance, Bacterial; Ethylene Glycols; Hemangioma; Humans; Infant; Propranolol; Quinolones; Radionuclide Imaging; Radiopharmaceuticals; Tuberculosis; Tuberculosis, Multidrug-Resistant

2014
Ivacaftor for patients with cystic fibrosis.
    Expert review of respiratory medicine, 2014, Volume: 8, Issue:5

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2014
Novel personalized therapies for cystic fibrosis: treating the basic defect in all patients.
    Journal of internal medicine, 2015, Volume: 277, Issue:2

    Topics: Aminoglycosides; Aminophenols; Anti-Bacterial Agents; Anti-Inflammatory Agents; Biomarkers; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Evidence-Based Medicine; Frameshift Mutation; Genistein; Humans; Oxadiazoles; Phenotype; Precision Medicine; Purinergic Antagonists; Quinolones; Rare Diseases; Severity of Illness Index; Sweat Glands

2015
Cystic fibrosis--what are the prospects for a cure?
    European journal of internal medicine, 2014, Volume: 25, Issue:9

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Quinolones

2014
Inhaled antibiotics beyond aminoglycosides, polymyxins and aztreonam: A systematic review.
    International journal of antimicrobial agents, 2015, Volume: 45, Issue:3

    Topics: Administration, Inhalation; Anti-Bacterial Agents; Bacterial Infections; beta-Lactams; Bronchiectasis; Cystic Fibrosis; Fosfomycin; Humans; Pneumonia, Ventilator-Associated; Quinolones; Respiratory Tract Infections; Treatment Outcome

2015
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
    The Cochrane database of systematic reviews, 2015, Mar-26, Issue:3

    Topics: Adult; Age Factors; Aminophenols; Child; Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Humans; Molecular Targeted Therapy; Mucociliary Clearance; Mutation; Quality of Life; Quinolones; Randomized Controlled Trials as Topic

2015
F508del-cystic fibrosis transmembrane regulator correctors for treatment of cystic fibrosis: a patent review.
    Expert opinion on therapeutic patents, 2015, Volume: 25, Issue:9

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Design; Humans; Mutation; Patents as Topic; Quinolones

2015
Potentiators for cystic fibrosis - targeting the underlying molecular defect.
    Paediatric respiratory reviews, 2015, Volume: 16, Issue:3

    Topics: Adult; Aminophenols; Child; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones; Respiratory Function Tests

2015
New and Emerging Treatments for Cystic Fibrosis.
    Drugs, 2015, Volume: 75, Issue:11

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Design; Humans; Mutation; Quinolones

2015
Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.
    Pediatric pulmonology, 2015, Volume: 50 Suppl 40

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Design; Homozygote; Humans; Mice; Molecular Targeted Therapy; Mutation; Protein Folding; Quinolones

2015
Targeting ion channels in cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:5

    Topics: Aminophenols; Anoctamin-1; Antiporters; Chloride Channels; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Sodium Channels; Humans; Ion Transport; Mutation; Neoplasm Proteins; Quinolones; Sulfate Transporters

2015
Cystic Fibrosis: A Novel Pharmacologic Approach to Cystic Fibrosis Transmembrane Regulator Modulation Therapy.
    The Journal of the American Osteopathic Association, 2015, Volume: 115, Issue:9

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; DNA Mutational Analysis; Humans; Mutation; Oxadiazoles; Quinolones

2015
Improving complex medical care while awaiting next-generation CFTR potentiators and correctors: The current pipeline of therapeutics.
    Pediatric pulmonology, 2015, Volume: 50 Suppl 40

    Topics: Aminophenols; Anti-Bacterial Agents; Anti-Inflammatory Agents; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy; Gene Deletion; Homozygote; Humans; Quality of Life; Quinolones; Treatment Outcome

2015
Update in Cystic Fibrosis 2014.
    American journal of respiratory and critical care medicine, 2015, Sep-15, Volume: 192, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Markers; Humans; Mucociliary Clearance; Quinolones; Treatment Outcome

2015
Targeted therapies to improve CFTR function in cystic fibrosis.
    Genome medicine, 2015, Sep-24, Volume: 7

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Molecular Targeted Therapy; Quinolones

2015
Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.
    Therapeutic advances in respiratory disease, 2015, Volume: 9, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Drug Combinations; Genetic Predisposition to Disease; Humans; Lung; Mutation; Patient Selection; Phenotype; Precision Medicine; Quinolones; Respiratory System Agents; Treatment Outcome

2015
[New therapies for cystic fibrosis targeting the CFTR gene or the CFTR protein].
    Revue des maladies respiratoires, 2016, Volume: 33, Issue:8

    Topics: Age Factors; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genetic Therapy; Humans; Molecular Targeted Therapy; Quinolones

2016
[Treatment of Cystic Fibrosis with CFTR Modulators].
    Pneumologie (Stuttgart, Germany), 2016, Volume: 70, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers, Tumor; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Drug Combinations; Evidence-Based Medicine; Genetic Markers; Genetic Predisposition to Disease; Humans; Precision Medicine; Quinolones; Treatment Outcome

2016
Advancing clinical development pathways for new CFTR modulators in cystic fibrosis.
    Thorax, 2016, Volume: 71, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Clinical Trials as Topic; Critical Pathways; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genetic Therapy; Genotype; Humans; Molecular Targeted Therapy; Mutation; Quality of Life; Quinolones; Randomized Controlled Trials as Topic; Treatment Outcome

2016
A safety evaluation of ivacaftor for the treatment of cystic fibrosis.
    Expert opinion on drug safety, 2016, Volume: 15, Issue:5

    Topics: Aminophenols; Animals; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Interactions; Humans; Mutation; Quality of Life; Quinolones; Rats

2016
New and emerging targeted therapies for cystic fibrosis.
    BMJ (Clinical research ed.), 2016, Mar-30, Volume: 352

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Discovery; Drug Therapy, Combination; Forced Expiratory Volume; Forecasting; Genetic Therapy; Homozygote; Humans; Molecular Targeted Therapy; Mutation; Oxadiazoles; Phosphodiesterase 5 Inhibitors; Practice Guidelines as Topic; Precision Medicine; Quinolones

2016
Cystic fibrosis: a model system for precision medicine.
    Current opinion in pediatrics, 2016, Volume: 28, Issue:3

    Topics: Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Genotype; Humans; Mutation; Precision Medicine; Quinolones; United States

2016
Controversies with Kalydeco: Newspaper coverage in Canada and the United States of the cystic fibrosis "wonder drug".
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016, Volume: 15, Issue:5

    Topics: Aminophenols; Canada; Cross-Cultural Comparison; Cystic Fibrosis; Drug Costs; Health Services Accessibility; Humans; Newspapers as Topic; Orphan Drug Production; Quinolones; Reimbursement Mechanisms; United States

2016
Lumacaftor/ivacaftor combination for cystic fibrosis patients homozygous for Phe508del-CFTR.
    Drugs of today (Barcelona, Spain : 1998), 2016, Volume: 52, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Mutation; Precision Medicine; Quinolones

2016
The impact of 12 months treatment with ivacaftor on Scottish paediatric patients with cystic fibrosis with the G551D mutation: a review.
    Archives of disease in childhood, 2018, Volume: 103, Issue:1

    Topics: Aminophenols; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones; Scotland; Treatment Outcome

2018
Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis.
    The Lancet. Respiratory medicine, 2016, Volume: 4, Issue:8

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Therapy, Combination; Female; Forced Expiratory Volume; Homozygote; Humans; Lung; Male; Middle Aged; Quinolones; Randomized Controlled Trials as Topic; Respiratory Function Tests; Treatment Outcome; Young Adult

2016
Lumacaftor/Ivacaftor: A Review in Cystic Fibrosis.
    Drugs, 2016, Volume: 76, Issue:12

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Lung; Mutation; Quinolones

2016
New Therapeutic Approaches to Modulate and Correct Cystic Fibrosis Transmembrane Conductance Regulator.
    Pediatric clinics of North America, 2016, Volume: 63, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Molecular Targeted Therapy; Mutation; Quinolones

2016
Can Cystic Fibrosis Patients Finally Catch a Breath With Lumacaftor/Ivacaftor?
    Clinical pharmacology and therapeutics, 2017, Volume: 101, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Combinations; Humans; Quinolones; United States; United States Food and Drug Administration

2017
Adherence to therapies in cystic fibrosis: a targeted literature review.
    Expert review of respiratory medicine, 2017, Volume: 11, Issue:2

    Topics: Administration, Inhalation; Administration, Oral; Aminophenols; Anti-Bacterial Agents; Cystic Fibrosis; Deoxyribonuclease I; Humans; Medication Adherence; Quinolones; Recombinant Proteins; Saline Solution, Hypertonic

2017
Update on new pulmonary therapies.
    Current opinion in pulmonary medicine, 2009, Volume: 15, Issue:6

    Topics: Aminophenols; Aminopyridines; Anti-Infective Agents; Anti-Inflammatory Agents; Benzodioxoles; Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Markers; Genetic Therapy; Humans; Mutation; Oxadiazoles; Quinolones; Respiratory System Agents; Saline Solution, Hypertonic

2009
Parallel evolution and local differentiation in quinolone resistance in Pseudomonas aeruginosa.
    Microbiology (Reading, England), 2011, Volume: 157, Issue:Pt 4

    Topics: Anti-Bacterial Agents; Bacterial Proteins; Cystic Fibrosis; DNA Gyrase; DNA Topoisomerase IV; DNA-Binding Proteins; Drug Resistance, Bacterial; Evolution, Molecular; Humans; Mutation, Missense; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Repressor Proteins; Transcription Factors

2011
Cystic fibrosis transmembrane conductance regulator dysfunction and its treatment.
    Journal of the Royal Society of Medicine, 2012, Volume: 105 Suppl 2

    Topics: Aminophenols; Clinical Trials, Phase II as Topic; Codon, Terminator; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drugs, Investigational; Female; Forecasting; Gene Expression Regulation; Genetic Predisposition to Disease; Humans; Ion Transport; Male; Molecular Targeted Therapy; Mutation; Needs Assessment; Quinolones; Randomized Controlled Trials as Topic; Sodium Channels

2012
Cystic fibrosis in an era of genomically guided therapy.
    Human molecular genetics, 2012, Oct-15, Volume: 21, Issue:R1

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Predisposition to Disease; Humans; Molecular Targeted Therapy; Mutation; Orphan Drug Production; Quinolones

2012
Cystic fibrosis therapeutics: the road ahead.
    Chest, 2013, Volume: 143, Issue:1

    Topics: Aminophenols; Anti-Bacterial Agents; Anti-Inflammatory Agents; Cystic Fibrosis; Drug Therapy; Humans; Quinolones

2013

Trials

61 trial(s) available for carbostyril and Cystic Fibrosis of Pancreas

ArticleYear
Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial.
    The Lancet. Respiratory medicine, 2022, Volume: 10, Issue:3

    Topics: Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quality of Life; Quinolones

2022
A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:4

    Topics: Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Homozygote; Humans; Indoles; Mutation; Quinolones

2022
Prospectively evaluating maternal and fetal outcomes in the era of CFTR modulators: the MAYFLOWERS observational clinical trial study design.
    BMJ open respiratory research, 2022, Volume: 9, Issue:1

    Topics: Aminophenols; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Lactation; Multicenter Studies as Topic; Observational Studies as Topic; Pregnancy; Prospective Studies; Quinolones

2022
GLPG2737 in lumacaftor/ivacaftor-treated CF subjects homozygous for the F508del mutation: A randomized phase 2A trial (PELICAN).
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:2

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Homozygote; Humans; Male; Mutation; Quinolones; Respiratory Function Tests; Sweat; Treatment Outcome

2020
Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.
    Lancet (London, England), 2019, 11-23, Volume: 394, Issue:10212

    Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Therapy, Combination; Female; Humans; Indoles; Male; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sweat

2019
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
    The New England journal of medicine, 2019, 11-07, Volume: 381, Issue:19

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Genotype; Humans; Indoles; Male; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sweat; Young Adult

2019
Ivacaftor in cystic fibrosis with residual function: Lung function results from an N-of-1 study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:1

    Topics: Adolescent; Aminophenols; Chloride Channel Agonists; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Forced Expiratory Volume; Functional Residual Capacity; Humans; Male; Mutation; Quinolones; Respiratory Function Tests; Treatment Outcome; Young Adult

2020
Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis.
    Respiratory research, 2020, Jun-01, Volume: 21, Issue:1

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Exercise Test; Female; Forced Expiratory Volume; Humans; Male; Physical Exertion; Pulmonary Ventilation; Quinolones; Treatment Outcome; Young Adult

2020
Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:6

    Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Curcumin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genistein; Humans; Male; Organoids; Quinolones

2020
Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:6

    Topics: Adult; Alleles; Aminophenols; Benzodioxoles; Body Mass Index; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Genotype; Humans; Indoles; Male; Quinolones; Respiratory Function Tests

2020
Patients with cystic fibrosis and advanced lung disease benefit from lumacaftor/ivacaftor treatment.
    Pediatric pulmonology, 2020, Volume: 55, Issue:12

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Forced Expiratory Volume; Humans; Lung; Male; Middle Aged; Quality of Life; Quinolones; Young Adult

2020
A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous for F508del or heterozygous for the F508del-CFTR mutati
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:1

    Topics: Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Heterozygote; Homozygote; Humans; Indoles; Male; Mutation; Quinolones; Treatment Outcome

2021
Ivacaftor in People with Cystic Fibrosis and a
    Annals of the American Thoracic Society, 2021, Volume: 18, Issue:3

    Topics: Aminophenols; Bayes Theorem; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Humans; Mutation; Quinolones

2021
G970R-CFTR Mutation (c.2908G>C) Results Predominantly in a Splicing Defect.
    Clinical and translational science, 2021, Volume: 14, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Biopsy; Cell Line; Cells, Cultured; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exons; Female; Humans; Intestinal Mucosa; Male; Mutation; Organoids; Precision Medicine; Primary Cell Culture; Quinolones; Rectum; RNA Splicing; Treatment Outcome; Young Adult

2021
A phase 3, randomized, double-blind, parallel-group study to evaluate tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for F508del-CFTR and a gating mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Heterozygote; Humans; Indoles; Ion Channel Gating; Male; Mutation; Quinolones; Respiratory Function Tests

2021
VO
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:3

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Double-Blind Method; Exercise Test; Exercise Tolerance; Female; Humans; Male; Oxygen Consumption; Quinolones

2021
Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study.
    The Lancet. Respiratory medicine, 2021, Volume: 9, Issue:7

    Topics: Aminophenols; Aminopyridines; Australia; Benzodioxoles; Canada; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Europe; Female; Humans; Male; Mutation; Quinolones; Time; Treatment Outcome; United States

2021
Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with open-label
    Nutrition (Burbank, Los Angeles County, Calif.), 2021, Volume: 85

    Topics: Adult; Aminophenols; Body Composition; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2021
Ivacaftor Reduces Inflammatory Mediators in Upper Airway Lining Fluid From Cystic Fibrosis Patients With a G551D Mutation: Serial Non-Invasive Home-Based Collection of Upper Airway Lining Fluid.
    Frontiers in immunology, 2021, Volume: 12

    Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Inflammation Mediators; Longitudinal Studies; Male; Middle Aged; Mutation; Nasal Lavage; Nasal Lavage Fluid; Quinolones; Young Adult

2021
CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy.
    Pediatric pulmonology, 2017, Volume: 52, Issue:7

    Topics: Adolescent; Adult; Aminophenols; C-Reactive Protein; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Leukocytes, Mononuclear; Male; Mutation; Neutrophils; Quinolones; Respiratory Function Tests; Sweat; Young Adult

2017
The pharmacokinetic interaction between ivacaftor and ritonavir in healthy volunteers.
    British journal of clinical pharmacology, 2017, Volume: 83, Issue:10

    Topics: Adult; Aminophenols; Area Under Curve; Chloride Channel Agonists; Chromatography, Liquid; Cross-Over Studies; Cystic Fibrosis; Cytochrome P-450 CYP3A; Cytochrome P-450 CYP3A Inhibitors; Drug Administration Schedule; Drug Interactions; Female; Half-Life; Healthy Volunteers; Humans; Male; Quinolones; Ritonavir; Tandem Mass Spectrometry; Young Adult

2017
Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment.
    Clinical science (London, England : 1979), 2017, Aug-01, Volume: 131, Issue:15

    Topics: Adolescent; Adult; Aged; Aminophenols; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exercise Test; Female; Humans; Lung; Male; Middle Aged; Mutation, Missense; Oxygen; Quality of Life; Quinolones; Young Adult

2017
Antibiotic exposure and interpersonal variance mask the effect of ivacaftor on respiratory microbiota composition.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:1

    Topics: Adult; Aminophenols; Analysis of Variance; Anti-Bacterial Agents; Chloride Channel Agonists; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Forced Expiratory Volume; Humans; Male; Microbiota; Mutation; Outcome Assessment, Health Care; Quinolones; Respiratory System; Sputum

2018
Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis.
    The New England journal of medicine, 2017, 11-23, Volume: 377, Issue:21

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Heterozygote; Humans; Indoles; Male; Mutation; Quality of Life; Quinolones; Young Adult

2017
Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.
    The New England journal of medicine, 2017, 11-23, Volume: 377, Issue:21

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Body Mass Index; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Homozygote; Humans; Indoles; Male; Mutation; Quality of Life; Quinolones; Young Adult

2017
Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study.
    The Lancet. Respiratory medicine, 2018, Volume: 6, Issue:7

    Topics: Aminophenols; Australia; Canada; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Infant; Male; Mutation; Quinolones; Treatment Outcome; United Kingdom; United States

2018
Lumacaftor/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:1

    Topics: Adolescent; Adult; Aged; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Disease Progression; Double-Blind Method; Drug Combinations; Female; Follow-Up Studies; Forced Expiratory Volume; Humans; Lung; Male; Middle Aged; Quinolones; Respiratory Function Tests; Retrospective Studies; Treatment Outcome; Young Adult

2019
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
    The New England journal of medicine, 2018, 10-25, Volume: 379, Issue:17

    Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Genotype; Humans; Indoles; Male; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sweat; Young Adult

2018
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
    The New England journal of medicine, 2018, 10-25, Volume: 379, Issue:17

    Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Genotype; Humans; Indoles; Male; Mutation; Pyrazoles; Pyrrolidines; Quinolones; Sweat; Young Adult

2018
Impact of CFTR modulation with Ivacaftor on Gut Microbiota and Intestinal Inflammation.
    Scientific reports, 2018, 12-13, Volume: 8, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dysbiosis; Enterobacteriaceae; Female; Gastrointestinal Microbiome; Humans; Inflammation; Intestinal Diseases; Male; Middle Aged; Mutation; Quinolones

2018
Measuring recovery in health-related quality of life during and after pulmonary exacerbations in patients with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:5

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Disease Progression; Drug Combinations; Female; Humans; Male; Quality of Life; Quinolones; Recovery of Function; Time Factors; Treatment Outcome

2019
Safety, pharmacokinetics, and pharmacodynamics of lumacaftor and ivacaftor combination therapy in children aged 2-5 years with cystic fibrosis homozygous for F508del-CFTR: an open-label phase 3 study.
    The Lancet. Respiratory medicine, 2019, Volume: 7, Issue:4

    Topics: Age Factors; Aminophenols; Aminopyridines; Benzodioxoles; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Female; Homozygote; Humans; Male; Quinolones

2019
An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5 years (KLIMB).
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:6

    Topics: Aminophenols; Body Mass Index; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Ion Channel Gating; Liver Function Tests; Male; Pancreas; Quinolones; Sodium Chloride; Sweat; Transaminases; Treatment Outcome; Weight Gain

2019
CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:5

    Topics: Administration, Oral; Adult; Aminophenols; Benzoates; Benzopyrans; Biological Availability; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Monitoring; Drug Therapy, Combination; Female; Humans; Male; Mutation; Quinolones; Respiratory Function Tests; Sweat; Treatment Outcome

2019
GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1).
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:5

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Drug Monitoring; Drug Substitution; Female; Humans; Male; Pyrans; Pyrazoles; Quinolones; Respiratory Function Tests; Sweat; Treatment Outcome; Withholding Treatment

2019
A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11 years with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:5

    Topics: Aminophenols; Benzodioxoles; Biological Availability; Child; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Drug Monitoring; Drug Therapy, Combination; Female; Humans; Indoles; Male; Mutation; Quinolones; Respiratory Function Tests; Sweat; Treatment Outcome

2019
Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.
    American journal of respiratory and critical care medicine, 2013, Jun-01, Volume: 187, Issue:11

    Topics: Administration, Oral; Alleles; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; Dose-Response Relationship, Drug; Double-Blind Method; Forced Expiratory Volume; Humans; Lung; Mutation; Quinolones; Respiratory Function Tests; Treatment Outcome

2013
Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation.
    PloS one, 2013, Volume: 8, Issue:7

    Topics: Adult; Amino Acid Substitution; Aminophenols; Biological Transport; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Demography; Epithelial Sodium Channels; Female; Humans; Male; Membrane Potentials; Middle Aged; Mutation; Nose; Placebos; Quinolones; Sample Size; Sodium; Solutions; Young Adult

2013
Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: a small pilot study.
    Pediatric diabetes, 2013, Volume: 14, Issue:6

    Topics: Adolescent; Adult; Amino Acid Substitution; Aminophenols; Child; Cross-Sectional Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diabetes Mellitus; Female; Glucose Intolerance; Glucose Tolerance Test; Humans; Insulin; Insulin Secretion; Insulin-Secreting Cells; Male; Middle Aged; Mutation; Pilot Projects; Prediabetic State; Quinolones

2013
Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial.
    The Lancet. Respiratory medicine, 2013, Volume: 1, Issue:8

    Topics: Adolescent; Adult; Aminophenols; Child; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Forced Expiratory Volume; Humans; Male; Mucociliary Clearance; Mutation; Quinolones; Respiratory System Agents; Spirometry; Young Adult

2013
Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:2

    Topics: Adult; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Double-Blind Method; Drug Monitoring; Female; Humans; Male; Mutation; Nasal Mucosa; Quinolones; Reproducibility of Results; Respiratory System Agents; Specimen Handling; Sweat; Treatment Outcome

2014
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial.
    The Lancet. Respiratory medicine, 2014, Volume: 2, Issue:7

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Base Sequence; Benzodioxoles; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Therapy, Combination; Female; Forced Expiratory Volume; Heterozygote; Homozygote; Humans; Male; Quinolones; Sequence Deletion; Sweat; Young Adult

2014
Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:6

    Topics: Adolescent; Adult; Aminophenols; Body Mass Index; Child; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Forced Expiratory Volume; Humans; Ion Channel Gating; Male; Middle Aged; Mutation; Outcome Assessment, Health Care; Quinolones; Young Adult

2014
Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST).
    The Lancet. Respiratory medicine, 2014, Volume: 2, Issue:11

    Topics: Adolescent; Adult; Aminophenols; Child; Cough; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Female; Forced Expiratory Volume; Humans; Male; Mutation; Quinolones; Respiratory Tract Infections; Time Factors; Weight Gain; Young Adult

2014
Effect of ivacaftor in patients with advanced cystic fibrosis and a G551D-CFTR mutation: Safety and efficacy in an expanded access program in the United States.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016, Volume: 15, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Animals; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Drug Monitoring; Female; Humans; Male; Mutation; Quinolones; Respiratory Function Tests; Treatment Outcome

2016
Efficacy response in CF patients treated with ivacaftor: post-hoc analysis.
    Pediatric pulmonology, 2015, Volume: 50, Issue:5

    Topics: Adolescent; Adult; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Female; Forced Expiratory Volume; Humans; Male; Mutation; Numbers Needed To Treat; Quinolones; Respiratory Function Tests; Surveys and Questionnaires; Sweat; Weight Gain; Young Adult

2015
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
    The New England journal of medicine, 2015, 07-16, Volume: 373, Issue:3

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Therapy, Combination; Female; Forced Expiratory Volume; Homozygote; Hospitalization; Humans; Male; Middle Aged; Mutation; Quinolones; Young Adult

2015
Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial.
    The Lancet. Respiratory medicine, 2015, Volume: 3, Issue:7

    Topics: Adolescent; Adult; Aged; Aminophenols; Child; Chloride Channels; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Forced Expiratory Volume; Humans; Male; Middle Aged; Mutation; Quinolones; Respiratory System Agents; Treatment Outcome; Young Adult

2015
Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trial.
    Health and quality of life outcomes, 2015, Jul-02, Volume: 13

    Topics: Adolescent; Adult; Aged; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Humans; Male; Middle Aged; Molecular Targeted Therapy; Mutation; Patient Outcome Assessment; Quality of Life; Quinolones; Young Adult

2015
Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor.
    Digestive diseases and sciences, 2016, Volume: 61, Issue:1

    Topics: Administration, Oral; Adolescent; Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Administration Schedule; Female; Genetic Predisposition to Disease; Humans; Male; Membrane Transport Modulators; Mutation; Nutritional Status; Phenotype; Quinolones; Surveys and Questionnaires; Time Factors; Treatment Outcome; Weight Gain; Young Adult

2016
Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study.
    The Lancet. Respiratory medicine, 2016, Volume: 4, Issue:2

    Topics: Aminophenols; Child, Preschool; Chloride Channel Agonists; Chlorides; Cough; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Genotype; Humans; Male; Mutation; Quinolones; Sweat

2016
Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Biological Variation, Population; Biomarkers; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Middle Aged; Mutation; Quinolones; Retrospective Studies; Sweat

2017
Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study.
    The Lancet. Respiratory medicine, 2017, Volume: 5, Issue:2

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cough; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Double-Blind Method; Drug Administration Schedule; Drug Combinations; Female; Forced Expiratory Volume; Homozygote; Humans; Lung; Male; Mutation; Quinolones; Time; Treatment Outcome; Young Adult

2017
In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.
    Pediatric pulmonology, 2017, Volume: 52, Issue:4

    Topics: Administration, Oral; Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Middle Aged; Quinolones; Sweat; Treatment Outcome; Young Adult

2017
Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:2

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Administration Schedule; Drug Monitoring; Female; Forced Expiratory Volume; Helium; Humans; Isotopes; Magnetic Resonance Imaging; Male; Middle Aged; Mutation; Outcome Assessment, Health Care; Pilot Projects; Pulmonary Ventilation; Quinolones; Single-Blind Method

2017
Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor.
    Pediatrics, 2017, Volume: 139, Issue:2

    Topics: Aminophenols; Body Height; Body Weight; Child; Cystic Fibrosis; Double-Blind Method; Female; Follow-Up Studies; Humans; Longitudinal Studies; Male; Quinolones; Treatment Outcome

2017
Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:3

    Topics: Adult; Aldehyde Oxidoreductases; Aminophenols; Aminopyridines; Benzodioxoles; Biological Availability; Biphenyl Compounds; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytochrome P-450 CYP3A Inducers; Dose-Response Relationship, Drug; Drug Combinations; Drug Interactions; Drug Monitoring; Female; Humans; Male; Membrane Transport Modulators; Mutation; Pharmacogenetics; Quinolones; Rifampin; Treatment Outcome

2017
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
    The New England journal of medicine, 2010, Nov-18, Volume: 363, Issue:21

    Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Forced Expiratory Volume; Humans; Ion Channels; Male; Membrane Potentials; Middle Aged; Mutation; Nasal Mucosa; Quinolones; Sweat; Young Adult

2010
Cystic fibrosis transmembrane regulator potentiators as promising cystic fibrosis therapies.
    Expert opinion on investigational drugs, 2011, Volume: 20, Issue:3

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Aminophenols; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Humans; Ion Transport; Middle Aged; Mutation; Quinolones; Young Adult

2011
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.
    The New England journal of medicine, 2011, Nov-03, Volume: 365, Issue:18

    Topics: Administration, Oral; Adolescent; Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Female; Forced Expiratory Volume; Humans; Male; Middle Aged; Mutation; Quinolones; Young Adult

2011
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.
    Chest, 2012, Volume: 142, Issue:3

    Topics: Adolescent; Adult; Alleles; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Homozygote; Humans; Male; Middle Aged; Mutation; Pharmacogenetics; Quinolones; Sweat; Treatment Outcome; Young Adult

2012

Other Studies

512 other study(ies) available for carbostyril and Cystic Fibrosis of Pancreas

ArticleYear
Cystic Fibrosis-related Diabetes Is Associated with Worse Lung Function Trajectory despite Ivacaftor Use.
    American journal of respiratory and critical care medicine, 2021, 12-01, Volume: 204, Issue:11

    Topics: Adolescent; Adult; Age Factors; Aminophenols; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Diabetes Mellitus; Female; Forced Expiratory Volume; Humans; Male; Quinolones; Young Adult

2021
Recent advances in the early treatment of cystic fibrosis: Bridging the gap to highly effective modulator therapy.
    Pediatric pulmonology, 2022, Volume: 57 Suppl 1

    Topics: Adolescent; Adult; Aminophenols; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gastrointestinal Diseases; Humans; Infant; Mutation; Nutritional Status; Quinolones

2022
Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Aminophenols; Anti-Bacterial Agents; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2022
Early insurance coverage of elexacaftor/tezacaftor/ivacaftor for cystic fibrosis.
    Pediatric pulmonology, 2021, Volume: 56, Issue:12

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Insurance Coverage; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Restoration of exocrine pancreatic function in child with lumacaftor/ivacaftor therapy in cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Quinolones

2022
The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Adult; Aminophenols; Benzodioxoles; Blood Glucose; Blood Glucose Self-Monitoring; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Hypoglycemia; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Elexacaftor/tezacaftor/ivacaftor for CFTR variants giving rise to diagnostic uncertainty: Personalised medicine or over-medicalisation?
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Precision Medicine; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Uncertainty

2022
Isotope dilution LC-MS/MS quantification of the cystic fibrosis transmembrane conductance regulator (CFTR) modulators ivacaftor, lumacaftor, tezacaftor, elexacaftor, and their major metabolites in human serum.
    Clinical chemistry and laboratory medicine, 2022, 01-26, Volume: 60, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chromatography, Liquid; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Isotopes; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Tandem Mass Spectrometry

2022
Alkyl-Quinolones derivatives as potential biomarkers for Pseudomonas aeruginosa infection chronicity in Cystic Fibrosis.
    Scientific reports, 2021, 10-20, Volume: 11, Issue:1

    Topics: Biomarkers; Cystic Fibrosis; Humans; Persistent Infection; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Quorum Sensing

2021
Olfaction before and after initiation of elexacaftor-tezacaftor-ivacaftor in a cystic fibrosis cohort.
    International forum of allergy & rhinology, 2022, Volume: 12, Issue:2

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Smell

2022
Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Affect; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mental Fatigue; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Somatoform Disorders

2022
Using Negative Control Outcomes and Difference-in-Differences Analysis to Estimate Treatment Effects in an Entirely Treated Cohort: The Effect of Ivacaftor in Cystic Fibrosis.
    American journal of epidemiology, 2022, 02-19, Volume: 191, Issue:3

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2022
CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3

    Topics: Adrenergic Agents; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sweat

2022
No drug-drug interaction between tezacaftor-ivacaftor and clofazimine: A case report.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:1

    Topics: Adolescent; Aminophenols; Anti-Inflammatory Agents; Benzodioxoles; Chloride Channel Agonists; Clofazimine; Cystic Fibrosis; Drug Combinations; Drug Interactions; Female; Humans; Indoles; Quinolones

2022
Optimising the care and quality of life of people with cystic fibrosis: the influence of cystic fibrosis transmembrane conductance regulator modulators.
    British journal of hospital medicine (London, England : 2005), 2021, Nov-02, Volume: 82, Issue:11

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quality of Life; Quinolones

2021
The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis postliver transplant: A case series.
    Pediatric pulmonology, 2022, Volume: 57, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quality of Life; Quinolones; Retrospective Studies; Young Adult

2022
Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients.
    Pediatric pulmonology, 2022, Volume: 57, Issue:3

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Indoles; Male; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Vitamin D; Vitamins; Young Adult

2022
Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Adult; Aminophenols; Benzodioxoles; Body Weight; Cardiometabolic Risk Factors; Chloride Channel Agonists; Cholesterol; Cystic Fibrosis; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Retrospective Studies

2022
Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3

    Topics: Aminophenols; Benzodioxoles; Bicarbonates; Chloride Channel Agonists; Chloride-Bicarbonate Antiporters; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Epithelial Cells; Humans; Indoles; Organoids; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Drug exposure to infants born to mothers taking Elexacaftor, Tezacaftor, and Ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:4

    Topics: Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Female; Humans; Indoles; Infant; Maternal Exposure; Mothers; Placenta; Pregnancy; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Idiopathic chronic pancreatitis treated with ivacaftor in a CFTR carrier with methylmalonic acidemia.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Mutation; Pancreatitis, Chronic; Quinolones

2022
Aquagenic wrinkling of the palms in cystic fibrosis patients treated with ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones; Skin Aging; Water

2022
Radiomics-derived morphological features predict pulmonary function response during lumacaftor/ivacaftor therapy in patients with cystic fibrosis.
    The European respiratory journal, 2022, Volume: 60, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Humans; Quinolones; Tomography, X-Ray Computed; Unsupervised Machine Learning

2022
Diversity of approaches in artificial intelligence: an opportunity for discoveries in thoracic imaging.
    The European respiratory journal, 2022, Volume: 60, Issue:1

    Topics: Aminophenols; Aminopyridines; Artificial Intelligence; Benzodioxoles; Cystic Fibrosis; Humans; Quinolones; Tomography, X-Ray Computed; Unsupervised Machine Learning

2022
Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Lung Transplantation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
An acneiform eruption associated with elexacaftor/tezacaftor/ivacaftor treatment.
    Dermatology online journal, 2021, Nov-15, Volume: 27, Issue:11

    Topics: Acneiform Eruptions; Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Drug Eruptions; Humans; Indoles; Male; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Hypervitaminosis A with fulminant secondary intracranial hypertension following personalized medicine-based Elexacaftor/Tezacaftor/Ivacaftor initiation in a preadolescent with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Hypervitaminosis A; Indoles; Intracranial Hypertension; Precision Medicine; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
The Rapid Reduction of Infection-Related Visits and Antibiotic Use Among People With Cystic Fibrosis After Starting Elexacaftor-Tezacaftor-Ivacaftor.
    Clinical infectious diseases : an official publication of the Infectious Diseases Society of America, 2022, 09-30, Volume: 75, Issue:7

    Topics: Aminophenols; Anti-Bacterial Agents; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:4

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dimethyl Sulfoxide; Drug Combinations; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Targeting the E1 ubiquitin-activating enzyme (UBA1) improves elexacaftor/tezacaftor/ivacaftor efficacy towards F508del and rare misfolded CFTR mutants.
    Cellular and molecular life sciences : CMLS, 2022, Mar-16, Volume: 79, Issue:4

    Topics: Aminophenols; Benzodioxoles; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Drug Therapy, Combination; Enzyme Inhibitors; Humans; Indoles; Mutation; Protein Folding; Pyrazoles; Pyridines; Pyrimidines; Pyrrolidines; Quinolones; Sequence Deletion; Sulfides; Sulfonamides; Ubiquitin-Activating Enzymes

2022
Physiologically-Based Pharmacokinetic-Led Guidance for Patients With Cystic Fibrosis Taking Elexacaftor-Tezacaftor-Ivacaftor With Nirmatrelvir-Ritonavir for the Treatment of COVID-19.
    Clinical pharmacology and therapeutics, 2022, Volume: 111, Issue:6

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; COVID-19 Drug Treatment; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytochrome P-450 CYP3A; Drug Combinations; Drug Interactions; Humans; Indoles; Lactams; Leucine; Mutation; Nitriles; Proline; Pyrazoles; Pyridines; Pyrrolidines; Quinolines; Quinolones; Ritonavir

2022
Elexacaftor/Tezacaftor/Ivacaftor as a Bridge to Lung Retransplant in a Recipient With Cystic Fibrosis.
    Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation, 2022, Volume: 20, Issue:4

    Topics: Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Lung; Male; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Treatment Outcome

2022
Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Middle Aged; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sinusitis; Young Adult

2022
CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.
    JCI insight, 2022, 03-22, Volume: 7, Issue:6

    Topics: Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Phenotype; Quinolones

2022
The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs.
    International journal of molecular sciences, 2022, Mar-15, Volume: 23, Issue:6

    Topics: Alleles; Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Current prices versus minimum costs of production for CFTR modulators.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Quinolones

2022
Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated With 1 Month of Lumacaftor/Ivacaftor.
    Journal of pediatric gastroenterology and nutrition, 2022, 07-01, Volume: 75, Issue:1

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Breath Tests; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Glucose; Humans; Hydrogen; Longitudinal Studies; Male; Mutation; Quinolones; Young Adult

2022
Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A case series.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5

    Topics: Adult; Aminophenols; Antihypertensive Agents; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Hypertension; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
At-home compounding preparation of slow desensitization of elexacaftor/tezacaftor/ivacaftor for delayed hypersensitivity rash.
    Pediatric pulmonology, 2022, Volume: 57, Issue:7

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Exanthema; Humans; Hypersensitivity, Delayed; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Glycated Hemoglobin; Humans; Indoles; Lung; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Transaminases; Transplant Recipients

2022
The impact of FDA and EMA regulatory decision-making process on the access to CFTR modulators for the treatment of cystic fibrosis.
    Orphanet journal of rare diseases, 2022, 05-07, Volume: 17, Issue:1

    Topics: Adult; Aminophenols; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2022
Elexacaftor/tezacaftor/ivacaftor in children aged 6-11 years with cystic fibrosis, at least one F508DEL allele, and advanced lung disease: A 24-week observational study.
    Pediatric pulmonology, 2022, Volume: 57, Issue:9

    Topics: Alleles; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
How Should the Effects of CFTR Modulator Therapy on Cystic Fibrosis Lung Disease Be Monitored?
    American journal of respiratory and critical care medicine, 2022, 08-01, Volume: 206, Issue:3

    Topics: Alleles; Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Lung; Magnetic Resonance Imaging; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
The Effect of Elexacaftor/Tezacaftor/Ivacaftor on Hospitalizations and Intravenous Antibiotic Use.
    The Permanente journal, 2022, 04-05, Volume: 26, Issue:1

    Topics: Aminophenols; Anti-Bacterial Agents; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Hospitalization; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Overcoming health disparities in access to CFTR modulator therapies: One child's journey with cystic fibrosis.
    Pediatric pulmonology, 2022, Volume: 57, Issue:9

    Topics: Aminophenols; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2022
Development and Internal Validation of a Prognostic Model of the Probability of Death or Lung Transplantation Within 2 Years for Patients With Cystic Fibrosis and FEV
    Chest, 2022, Volume: 162, Issue:4

    Topics: Aminophenols; Anti-Bacterial Agents; Child; Cystic Fibrosis; Hemoglobins; Humans; Lung Transplantation; Oxygen; Probability; Prognosis; Quinolones

2022
Change in exercise capacity measured by Cardio-pulmonary Exercise Testing (CPET) in Danish people with cystic fibrosis after initiation of treatment with Lumacaftor/Ivacaftor and Tezacaftor/Ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5

    Topics: Aminophenols; Aminopyridines; Bacterial Toxins; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Denmark; Drug Combinations; Exercise Test; Exercise Tolerance; Humans; Indoles; Mutation; Prospective Studies; Quinolones

2022
T-cell-mediated hypersensitivity to lumacaftor and ivacaftor in cystic fibrosis.
    Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology, 2022, Volume: 33, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Humans; Mutation; Quinolones; T-Lymphocytes

2022
Modulation, microbiota and inflammation in the adult CF gut: A prospective study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5

    Topics: Adult; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Inflammation; Lactoferrin; Leukocyte L1 Antigen Complex; Microbiota; Mutation; Prospective Studies; Quinolones

2022
Real Life With Tezacaftor and Ivacaftor in Adult Patients With Cystic Fibrosis: Spanish Multicenter Study.
    Archivos de bronconeumologia, 2022, Volume: 58, Issue:9

    Topics: Adult; Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Quinolones

2022
Ivacaftor, not ivacaftor/lumacaftor, associated with lower pulmonary inflammation in preschool cystic fibrosis.
    Pediatric pulmonology, 2022, Volume: 57, Issue:10

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Pneumonia; Quinolones

2022
Impact of highly effective CFTR modulator therapy on digital clubbing in patients with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5

    Topics: Aminophenols; Benzodioxoles; Calcium Sulfate; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2022
Plasma and cellular ivacaftor concentrations in patients with cystic fibrosis.
    Pediatric pulmonology, 2022, Volume: 57, Issue:11

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2022
Malassezia Folliculitis following Triple Therapy for Cystic Fibrosis.
    Medicina (Kaunas, Lithuania), 2022, Sep-02, Volume: 58, Issue:9

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Folliculitis; Humans; Malassezia; Mutation; Quinolones

2022
Exploring the impact of elexacaftor-tezacaftor-ivacaftor treatment on opinions regarding airway clearance techniques and nebulisers: TEMPO a qualitative study in children with cystic fibrosis, their families and healthcare professionals.
    BMJ open respiratory research, 2022, Volume: 9, Issue:1

    Topics: Aged; Aminophenols; Benzodioxoles; Child; Cyclic N-Oxides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Delivery of Health Care; Humans; Indoles; Mutation; Nebulizers and Vaporizers; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Modulator Combination Improves In Vitro the Microrheological Properties of the Airway Surface Liquid of Cystic Fibrosis Airway Epithelia.
    International journal of molecular sciences, 2022, Sep-27, Volume: 23, Issue:19

    Topics: Aminophenols; Benzodioxoles; Bicarbonates; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Phenylalanine; Quinolones

2022
The Impact of Highly Effective Cystic Fibrosis Transmembrane Conductance Regulator Modulators on the Health of Female Subjects With Cystic Fibrosis.
    Clinical therapeutics, 2023, Volume: 45, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Mutation; Quinolones

2023
A paradigm shift in cystic fibrosis nutritional care: Clinicians' views on the management of patients with overweight and obesity.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2023, Volume: 22, Issue:5

    Topics: Adult; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Obesity; Overweight; Quinolones

2023
Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?
    Pediatric pulmonology, 2023, Volume: 58, Issue:9

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones; Retrospective Studies

2023
Successful Pregnancy of a Patient with Cystic Fibrosis Genotype F508del/ F508del and Progressed Pulmonary Destruction on lumacaftor/ivacaftor.
    Klinische Padiatrie, 2019, Volume: 231, Issue:5

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Lung; Mutation; Pregnancy; Pregnancy Outcome; Quinolones

2019
Patient-reported outcomes in patients with cystic fibrosis with a G551D mutation on ivacaftor treatment: results from a cross-sectional study.
    BMC pulmonary medicine, 2019, Aug-13, Volume: 19, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Cross-Sectional Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Forced Expiratory Volume; Humans; Internationality; Male; Multivariate Analysis; Mutation; Patient Reported Outcome Measures; Quality of Life; Quinolones; Regression Analysis; Surveys and Questionnaires

2019
ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channe
    Molecular pharmacology, 2019, Volume: 96, Issue:4

    Topics: Aminophenols; Aminopyridines; Animals; Arginase; Arginine; Benzodioxoles; Bronchi; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytosol; Drug Combinations; Humans; Intestinal Mucosa; Mice; Mutation; Nitric Oxide; Nose; Quinolones

2019
Effect of Lumacaftor/Ivacaftor on Pulmonary Exacerbation Rates in Members with Cystic Fibrosis in a Medicaid Population.
    Journal of managed care & specialty pharmacy, 2019, Volume: 25, Issue:9

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Lung; Male; Medicaid; Middle Aged; Mutation; Quinolones; United States; Young Adult

2019
Whole-blood transcriptomic responses to lumacaftor/ivacaftor therapy in cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:2

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Biomarkers, Pharmacological; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Homozygote; Humans; Ion Transport; Male; Metabolomics; Mutation; Pharmacogenomic Testing; Pharmacogenomic Variants; Prognosis; Quinolones; Transcriptome

2020
VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.
    Scientific reports, 2019, 09-17, Volume: 9, Issue:1

    Topics: Aminophenols; Animals; Cell Line; Cyclic AMP-Dependent Protein Kinases; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Mutation; Oocytes; Phosphorylation; Quinolones; Rats; Xenopus laevis

2019
Changes in Airway Microbiome and Inflammation with Ivacaftor Treatment in Patients with Cystic Fibrosis and the G551D Mutation.
    Annals of the American Thoracic Society, 2020, Volume: 17, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Longitudinal Studies; Lung; Male; Microbiota; Mutation; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Respiratory System Agents; Sputum; Sweat; Treatment Outcome; Young Adult

2020
Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
    Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.], 2019, Volume: 19, Issue:8

    Topics: Adult; Aged; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Pancreatic Insufficiency; Female; Humans; Indoles; Male; Middle Aged; Quinolones; Retrospective Studies

2019
Orphan Drug Pricing and Costs: A Case Study of Kalydeco and Orkambi.
    Healthcare policy = Politiques de sante, 2019, Volume: 15, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cost-Benefit Analysis; Cystic Fibrosis; Drug Combinations; Drug Costs; Humans; Orphan Drug Production; Quinolones

2019
Combination Therapy with Lumacaftor-Ivacaftor in Cystic Fibrosis. Keeping It Real.
    American journal of respiratory and critical care medicine, 2020, 01-15, Volume: 201, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Humans; Quinolones

2020
Clinical significance of
    Journal of medical microbiology, 2019, Volume: 68, Issue:12

    Topics: Adolescent; Adult; Anti-Bacterial Agents; Cystic Fibrosis; Female; Humans; Male; Middle Aged; Pseudomonas aeruginosa; Quinolones; Quorum Sensing; Young Adult

2019
Does Ivacaftor Taken Twice a Day Keep the
    Annals of the American Thoracic Society, 2019, Volume: 16, Issue:11

    Topics: Aminophenols; Cohort Studies; Cystic Fibrosis; Humans; Infections; Pseudomonas; Quinolones; Registries

2019
Longitudinal changes in lung function following initiation of lumacaftor/ivacaftor combination.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:4

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Monitoring; Duration of Therapy; Female; Forced Expiratory Volume; Humans; Longitudinal Studies; Male; Medication Therapy Management; Quinolones; Respiratory Function Tests; Retrospective Studies; Treatment Outcome; United States

2020
Mutation-specific dual potentiators maximize rescue of CFTR gating mutants.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:2

    Topics: Aminophenols; Cells, Cultured; Chloride Channel Agonists; Cluster Analysis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Drug Therapy, Combination; Humans; Ion Channel Gating; Ion Transport; Mutation, Missense; Nasal Mucosa; Precision Medicine; Pyrans; Pyrazoles; Quinolones

2020
Entering the era of highly effective CFTR modulator therapy.
    Lancet (London, England), 2019, 11-23, Volume: 394, Issue:10212

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Humans; Indoles; Mutation; Quinolones; Safety

2019
Positive clinical outcomes following ivacaftor treatment in a cystic fibrosis patient with the genotype 3272-26A > G/Q493X.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:1

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Eligibility Determination; Female; Humans; Medication Therapy Management; Mutation; Quality of Life; Quinolones; Respiratory Function Tests; Respiratory Tract Infections; Treatment Outcome

2020
New Drug Hailed as Major Breakthrough in Cystic Fibrosis.
    American journal of medical genetics. Part A, 2020, Volume: 182, Issue:1

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Combinations; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolines; Quinolones; United States; United States Food and Drug Administration

2020
Expanding access to CFTR modulators for rare mutations: The utility of n-of-1 trials.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:1

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2020
Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males.
    American journal of respiratory and critical care medicine, 2020, 04-15, Volume: 201, Issue:8

    Topics: Adolescent; Adult; Age Factors; Aminophenols; Body Mass Index; Body Weight; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Disease Progression; Female; Forced Expiratory Volume; Humans; Male; Quinolones; Sex Factors; Sweat; Young Adult

2020
Lumacaftor/ivacaftor reduces exacerbations in adults homozygous for Phe508del mutation with severe lung disease.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:3

    Topics: Administration, Intravenous; Adult; Aminophenols; Aminopyridines; Anti-Bacterial Agents; Australia; Benzodioxoles; Case-Control Studies; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug-Related Side Effects and Adverse Reactions; Female; Forced Expiratory Volume; Humans; Male; Mutation; Quinolones; Respiratory Function Tests; Respiratory Tract Infections; Symptom Flare Up

2020
Suppressing 'nonsense' in cystic fibrosis.
    The Journal of physiology, 2020, Volume: 598, Issue:3

    Topics: Aminophenols; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2020
To be or not to be on CFTR modulators during pregnancy: Risks to be considered.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:2

    Topics: Adult; Aminophenols; Bronchiectasis; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Delivery, Obstetric; Drug Dosage Calculations; Exocrine Pancreatic Insufficiency; Female; Gestational Age; Humans; Infant, Newborn; Lung Diseases; Monitoring, Physiologic; Mutation; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Pseudomonas; Quinolones

2020
Outcomes of a methicillin-resistant Staphylococcus aureus (MRSA) eradication protocol in pediatric cystic fibrosis (CF) patients.
    Pediatric pulmonology, 2020, Volume: 55, Issue:3

    Topics: Adolescent; Aminophenols; Anti-Bacterial Agents; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Male; Methicillin-Resistant Staphylococcus aureus; Quinolones; Retrospective Studies; Rifampin; Staphylococcal Infections

2020
Adherence to lumacaftor-ivacaftor therapy in patients with cystic fibrosis in France.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:3

    Topics: Adult; Age Factors; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cost-Benefit Analysis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Forced Expiratory Volume; France; Homozygote; Humans; Male; Medication Adherence; Quinolones; Respiratory Function Tests; Retrospective Studies; Treatment Outcome

2020
New Treatment for Cystic Fibrosis.
    The American journal of nursing, 2020, Volume: 120, Issue:2

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Drug Approval; Drug Combinations; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolines; Quinolones; United States; United States Food and Drug Administration

2020
Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:4

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Bronchoscopy; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Drug Therapy, Combination; Endometriosis; Female; Fertility Agents, Female; Hemoptysis; Humans; Indoles; Leuprolide; Pyrazoles; Pyridines; Quinolines; Quinolones; Radiography, Thoracic; Thoracic Diseases; Tomography, X-Ray Computed

2020
Elexacaftor/tezacaftor/ivacaftor (Trikafta) for cystic fibrosis.
    The Medical letter on drugs and therapeutics, 2020, Jan-13, Volume: 62, Issue:1589

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Administration Schedule; Drug Combinations; Drug Interactions; Forced Expiratory Volume; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Randomized Controlled Trials as Topic; Treatment Outcome

2020
Variable cellular ivacaftor concentrations in people with cystic fibrosis on modulator therapy.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:5

    Topics: Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Epithelial Cells; Feasibility Studies; Humans; Pilot Projects; Quinolones

2020
Triple Therapy for Cystic Fibrosis with a Phe508del CFTR Mutation.
    The New England journal of medicine, 2020, 02-13, Volume: 382, Issue:7

    Topics: Alleles; Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Mutation; Quinolones

2020
Precise Targeting of miRNA Sites Restores CFTR Activity in CF Bronchial Epithelial Cells.
    Molecular therapy : the journal of the American Society of Gene Therapy, 2020, 04-08, Volume: 28, Issue:4

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cells, Cultured; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Synergism; Epithelial Cells; Female; Humans; Indoles; Infant; Male; MicroRNAs; Middle Aged; Models, Biological; Nanoparticles; Oligonucleotides; Polylactic Acid-Polyglycolic Acid Copolymer; Quinolones

2020
R117H-CFTR function and response to VX-770 correlate with mRNA and protein expression in intestinal organoids.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:5

    Topics: Aminophenols; Cell Culture Techniques; Chloride Channel Agonists; Colon; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Organoids; Quinolones; RNA, Messenger

2020
Anti-Infectives Restore ORKAMBI
    Biomolecules, 2020, 02-19, Volume: 10, Issue:2

    Topics: Aminophenols; Aminopyridines; Anti-Bacterial Agents; Antimicrobial Cationic Peptides; Benzodioxoles; Bronchi; Cell Line; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Tobramycin

2020
Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis.
    eLife, 2020, 03-02, Volume: 9

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytokines; Down-Regulation; Drug Therapy, Combination; Female; Humans; Indoles; Inflammation; Interleukin-18; Interleukin-1beta; Male; Monocytes; Quinolones; Tumor Necrosis Factor-alpha; Young Adult

2020
Genomically-guided therapies: A new era for cystic fibrosis.
    Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, 2020, Volume: 27 Suppl 1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genetic Therapy; Humans; Indoles; Mutation; Precision Medicine; Quinolones; Respiratory System Agents

2020
Effect of one-year lumacaftor-ivacaftor treatment on glucose tolerance abnormalities in cystic fibrosis patients.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:5

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Blood Glucose; Child; Cystic Fibrosis; Diabetes Mellitus; Drug Administration Schedule; Drug Combinations; Female; Glucose Intolerance; Glucose Tolerance Test; Humans; Male; Middle Aged; Prospective Studies; Quinolones; Young Adult

2020
Long-term effects of ivacaftor on nonpulmonary outcomes in individuals with cystic fibrosis, heterozygous for a S1251N mutation.
    Pediatric pulmonology, 2020, Volume: 55, Issue:6

    Topics: Adolescent; Adult; Aminophenols; Body Composition; Body Mass Index; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Energy Metabolism; Female; Heterozygote; Humans; Lung; Male; Mutation; Quinolones; Spirometry; Young Adult

2020
Inhalable nano into micro dry powders for ivacaftor delivery: The role of mannitol and cysteamine as mucus-active agents.
    International journal of pharmaceutics, 2020, May-30, Volume: 582

    Topics: Administration, Inhalation; Aminophenols; Animals; Cells, Cultured; Chloride Channel Agonists; Cysteamine; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Compounding; Drug Liberation; Expectorants; Mannitol; Mutation; Nanoparticles; Peptides; Powders; Quinolones; Rats, Inbred F344

2020
Ataluren/ivacaftor combination therapy: Two N-of-1 trials in cystic fibrosis patients with nonsense mutations.
    Pediatric pulmonology, 2020, Volume: 55, Issue:7

    Topics: Adult; Aminophenols; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Oxadiazoles; Quinolones; Treatment Outcome

2020
Celiac Disease in Patients With Cystic Fibrosis on Ivacaftor: A Case Series.
    Journal of pediatric gastroenterology and nutrition, 2020, Volume: 71, Issue:2

    Topics: Aminophenols; Benzodioxoles; Celiac Disease; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2020
Microbial clues lead to a diagnosis of cystic fibrosis in late adulthood.
    BMJ case reports, 2020, Apr-20, Volume: 13, Issue:4

    Topics: Aged; Aminophenols; Bronchoalveolar Lavage Fluid; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genetic Testing; Humans; Mutation; Quinolones; Scedosporium; Staphylococcus aureus; Stenotrophomonas maltophilia

2020
Structural Consequences of the 1,2,3-Triazole as an Amide Bioisostere in Analogues of the Cystic Fibrosis Drugs VX-809 and VX-770.
    ChemMedChem, 2020, 09-16, Volume: 15, Issue:18

    Topics: Amides; Aminophenols; Aminopyridines; Benzodioxoles; Crystallography, X-Ray; Cystic Fibrosis; Humans; Models, Molecular; Molecular Structure; Quantum Theory; Quinolones; Triazoles

2020
Can lumacaftor-ivacaftor reverse glucose-tolerance abnormalities in cystic fibrosis?
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Glucose; Humans; Quinolones

2020
Morbidity and mortality in carriers of the cystic fibrosis mutation
    The European respiratory journal, 2020, Volume: 56, Issue:3

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Male; Morbidity; Mutation; Quinolones

2020
Correlation between Ivacaftor-induced CFTR Activation in Airway Epithelial Cells and Improved Lung Function: A Proof-of-Concept Study.
    Annals of the American Thoracic Society, 2020, Volume: 17, Issue:8

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Lung; Proof of Concept Study; Quinolones

2020
Females with cystic fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Body Mass Index; Child; Chlorides; Correlation of Data; Cystic Fibrosis; Drug Combinations; Female; Forced Expiratory Volume; Humans; Male; Quinolones; Retrospective Studies; Sex Factors; Sweat; Young Adult

2021
Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.
    Pediatric pulmonology, 2020, Volume: 55, Issue:9

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Child, Preschool; Chloride Channel Agonists; Cross-Sectional Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Indoles; Infant; Male; Mutation; Pyrazoles; Pyridines; Quinolines; Quinolones; Registries; Turkey; Young Adult

2020
Quantitative phase imaging to study transmembrane water fluxes regulated by CFTR and AQP3 in living human airway epithelial CFBE cells and CHO cells.
    PloS one, 2020, Volume: 15, Issue:5

    Topics: Aminophenols; Animals; Aquaporin 3; Biological Transport, Active; Biophysical Phenomena; Bronchi; Cell Line; Chloride Channel Agonists; CHO Cells; Colforsin; Cricetulus; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Microscopy, Interference; Mutant Proteins; Osmosis; Quinolones; Respiratory Mucosa; RNA, Messenger; Water

2020
Comment on "Effect of one-year lumacaftor-ivacaftor treatment on glucose tolerance abnormalities in cystic fibrosis patients".
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Glucose; Humans; Quinolones

2020
Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:6

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Middle Aged; Mutation; Quinolones; Respiratory Function Tests; United States

2020
Antisense oligonucleotide-mediated correction of CFTR splicing improves chloride secretion in cystic fibrosis patient-derived bronchial epithelial cells.
    Nucleic acids research, 2020, 07-27, Volume: 48, Issue:13

    Topics: Aminophenols; Bronchi; Cell Line, Tumor; Cells, Cultured; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Ion Transport; Mutation; Oligodeoxyribonucleotides, Antisense; Quinolones; RNA Splicing

2020
Drug allergy to CFTR modulator therapy associated with lumacaftor-specific CD4
    The Journal of allergy and clinical immunology, 2021, Volume: 147, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; CD4-Positive T-Lymphocytes; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Hypersensitivity; Female; Humans; Quinolones; Young Adult

2021
Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020, Volume: 19, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cell Culture Techniques; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Epithelial Cells; Humans; Indoles; Quinolones; Respiratory Mucosa

2020
Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR.
    American journal of respiratory and critical care medicine, 2020, 11-01, Volume: 202, Issue:9

    Topics: Aminophenols; Animals; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Models, Animal; Mucus; Quinolones; Rats

2020
Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:1

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cholesterol; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Liver; Male; Mutation; Quinolones; Treatment Outcome; Young Adult

2021
Survey of patients with cystic fibrosis and caregivers decisions regarding CFTR modulators.
    Pediatric pulmonology, 2020, Volume: 55, Issue:11

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Caregivers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Substitution; Foundations; Humans; Indoles; Middle Aged; Quinolones; Social Media; Surveys and Questionnaires; Young Adult

2020
Cystic Fibrosis-Related Pancreatic Cysts Decrease in Size and Number Upon Treatment With Cystic Fibrosis Transmembrane Conductance Regulator Modulators.
    Pancreas, 2020, Volume: 49, Issue:6

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Male; Mutation; Pancreas; Pancreatic Cyst; Quinolones; Treatment Outcome; Ultrasonography

2020
Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Disease Progression; Drug Combinations; Female; France; Humans; Male; Quinolones; Respiratory Function Tests

2021
GM1 as Adjuvant of Innovative Therapies for Cystic Fibrosis Disease.
    International journal of molecular sciences, 2020, Jun-24, Volume: 21, Issue:12

    Topics: Adjuvants, Immunologic; Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; G(M1) Ganglioside; Humans; Mutation; Quinolones; Therapies, Investigational

2020
Evidence of Small Airways Disease and the Immediate Effects of Lumacaftor/Ivacaftor in Children with Cystic Fibrosis.
    Irish medical journal, 2020, 05-07, Volume: 113, Issue:5

    Topics: Adolescent; Airway Remodeling; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Drug Combinations; Female; Forced Expiratory Volume; Humans; Male; Negative Results; Pulmonary Disease, Chronic Obstructive; Quinolones; Retrospective Studies; Risk Factors

2020
Altered iron metabolism in cystic fibrosis macrophages: the impact of CFTR modulators and implications for Pseudomonas aeruginosa survival.
    Scientific reports, 2020, 07-02, Volume: 10, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Biofilms; Child; Culture Media, Conditioned; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Gene Expression Profiling; Host-Pathogen Interactions; Humans; Iron; Iron-Regulatory Proteins; Macrophages; Male; Middle Aged; Pseudomonas aeruginosa; Quinolones; Sputum

2020
Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions.
    International journal of molecular sciences, 2020, Jun-25, Volume: 21, Issue:12

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cell Line; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Protein Domains; Quinolones; Regulatory Sequences, Nucleic Acid; Signal Transduction

2020
Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR.
    International journal of molecular sciences, 2020, Jul-08, Volume: 21, Issue:14

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Cell Line; Cell Membrane; Crystallins; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Heat-Shock Proteins; HEK293 Cells; Humans; Male; Mice; Molecular Chaperones; Mutation; Phenylalanine; Phosphorylation; Proteasome Endopeptidase Complex; Protein Transport; Quinolones

2020
Distinctive lipid signatures of bronchial epithelial cells associated with cystic fibrosis drugs, including Trikafta.
    JCI insight, 2020, 08-20, Volume: 5, Issue:16

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cells, Cultured; Ceramides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Epithelial Cells; Humans; Indoles; Lipid Metabolism; Lipidomics; Pyrazoles; Pyridines; Quinolines; Quinolones; Spectrometry, Mass, Electrospray Ionization

2020
CFTR modulator therapy for cystic fibrosis caused by the rare c.3700A>G mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Mutant Proteins; Mutation, Missense; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Sustained recovery of exocrine pancreatic function in a teenager with cystic fibrosis treated with ivacaftor.
    Pediatric pulmonology, 2020, Volume: 55, Issue:10

    Topics: Adolescent; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gastrointestinal Microbiome; Humans; Quinolones

2020
Ivacaftor or lumacaftor/ivacaftor treatment does not alter the core CF airway epithelial gene response to rhinovirus.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cells, Cultured; Common Cold; Cystic Fibrosis; Drug Combinations; Humans; Quinolones; Respiratory Mucosa; Rhinovirus

2021
Comparison of Organoid Swelling and
    American journal of respiratory and critical care medicine, 2020, 12-01, Volume: 202, Issue:11

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Edema; Female; Forced Expiratory Volume; Homozygote; Humans; In Vitro Techniques; Male; Membrane Potentials; Middle Aged; Nasal Mucosa; Organoids; Precision Medicine; Prospective Studies; Quinolones; Rectum; Sweat; Treatment Outcome; Vital Capacity; Young Adult

2020
The impact of ivacaftor on sinonasal pathology in S1251N-mediated cystic fibrosis patients.
    PloS one, 2020, Volume: 15, Issue:7

    Topics: Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Male; Nitric Oxide; Paranasal Sinuses; Polymorphism, Single Nucleotide; Prospective Studies; Quinolones; Tertiary Care Centers; Tomography, X-Ray Computed; Young Adult

2020
Rats Race to Keep Pace in the Growing Cystic Fibrosis Model Space.
    American journal of respiratory and critical care medicine, 2020, 11-01, Volume: 202, Issue:9

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Mucus; Quinolones; Rats

2020
Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:3

    Topics: Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Female; Humans; Indoles; Male; Paranasal Sinus Diseases; Prospective Studies; Pyrazoles; Pyridines; Pyrrolidines; Quality of Life; Quinolones

2021
Organoids as a personalized medicine tool for ultra-rare mutations in cystic fibrosis: The case of S955P and 1717-2A>G.
    Biochimica et biophysica acta. Molecular basis of disease, 2020, 11-01, Volume: 1866, Issue:11

    Topics: Alleles; Aminophenols; Aminopyridines; Benzodioxoles; Blotting, Western; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophysiology; Fluorescent Antibody Technique; Genotype; Humans; Indoles; Mutation; Precision Medicine; Quinolones

2020
Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:3

    Topics: Adult; Aminophenols; Benzodioxoles; Biliary Tract Diseases; Chloride Channel Agonists; Cholecystectomy; Cystic Fibrosis; Drug Combinations; Female; Humans; Indoles; Male; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Reduced Intestinal Inflammation With Lumacaftor/Ivacaftor in Adolescents With Cystic Fibrosis.
    Journal of pediatric gastroenterology and nutrition, 2020, Volume: 71, Issue:6

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Inflammation; Lung; Mutation; Quinolones

2020
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Evaluation, Preclinical; Drug Resistance; Humans; Mutation; Quinolones

2021
Azithromycin and tezacaftor/ivacaftor is associated with first-degree heart block in an adult with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Aminophenols; Anti-Bacterial Agents; Azithromycin; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Electrocardiography; Female; Heart Block; Humans; Indoles; Quinolones; Young Adult

2021
Drug efficacy and toxicity prediction: an innovative application of transcriptomic data.
    Cell biology and toxicology, 2020, Volume: 36, Issue:6

    Topics: Aminophenols; Aminopyridines; Animals; Antineoplastic Agents; Benzodioxoles; Cell Death; Cystic Fibrosis; Dose-Response Relationship, Drug; Drug Combinations; Drug Development; Epithelial Cells; Gene Expression Profiling; Gene Regulatory Networks; Humans; Leukemia, Myeloid, Acute; Pyrazines; Pyrazoles; Quinolones; Toxicity Tests; Transcriptome

2020
Ivacaftor improves lung disease in patients with advanced CF carrying CFTR mutations that confer residual function.
    Respiratory medicine, 2020, Volume: 171

    Topics: Adult; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Lung; Lung Diseases; Male; Middle Aged; Mutation; Quinolones; Respiratory Function Tests; Retrospective Studies; Treatment Outcome; Young Adult

2020
Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Canada; Child; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Health Services Accessibility; Humans; Indoles; Middle Aged; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Registries

2021
Ivacaftor modifies cystic fibrosis neutrophil phenotype in subjects with R117H residual function CFTR mutations.
    The European respiratory journal, 2021, Volume: 57, Issue:1

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Neutrophils; Phenotype; Quinolones

2021
Transparency and diversity in cystic fibrosis research.
    Lancet (London, England), 2020, 08-29, Volume: 396, Issue:10251

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Double-Blind Method; Humans; Indoles; Mutation; Quinolones

2020
Transparency and diversity in cystic fibrosis research.
    Lancet (London, England), 2020, 08-29, Volume: 396, Issue:10251

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Double-Blind Method; Humans; Indoles; Mutation; Quinolones

2020
Persistent recovery of pancreatic function in patients with cystic fibrosis after ivacaftor.
    Pediatric pulmonology, 2020, Volume: 55, Issue:12

    Topics: Aminophenols; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Pancreatic Insufficiency; Female; Humans; Male; Mutation; Pancreas; Quinolones

2020
Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Glucose Tolerance Test; Homozygote; Humans; Insulin Secretion; Longitudinal Studies; Male; Middle Aged; Mutation; Quinolones

2021
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.
    International journal of molecular sciences, 2020, Sep-10, Volume: 21, Issue:18

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Case-Control Studies; Child; Child, Preschool; Cystic Fibrosis; Drug Combinations; Exosomes; Humans; Indoles; Kidney Diseases; Proteome; Quinolones; Young Adult

2020
Appearance of Pancreatic Sufficiency and Discontinuation of Pancreatic Enzyme Replacement Therapy in Children with Cystic Fibrosis on Ivacaftor.
    Annals of the American Thoracic Society, 2021, Volume: 18, Issue:1

    Topics: Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Enzyme Replacement Therapy; Humans; Mutation; Pancreas; Quinolones

2021
Cost-Effectiveness of Ivacaftor Therapy for Treatment of Cystic Fibrosis Patients With the G551D Gating Mutation.
    Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research, 2020, Volume: 23, Issue:10

    Topics: Aminophenols; Chloride Channel Agonists; Cost-Benefit Analysis; Cystic Fibrosis; Drug Costs; Female; Health Care Costs; Humans; Male; Mutation; Quality-Adjusted Life Years; Quinolones; Time Factors

2020
1-BENZYLSPIRO[PIPERIDINE-4,1'-PYRIDO[3,4-b]indole] 'co-potentiators' for minimal function CFTR mutants.
    European journal of medicinal chemistry, 2021, Jan-01, Volume: 209

    Topics: Aminophenols; Animals; Cell Line; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Models, Molecular; Mutation; Piperidines; Quinolones; Rats; Structure-Activity Relationship

2021
Getting Near to "Closing the Gap" in the Pediatric Age Group for the First Personalized Treatment of Cystic Fibrosis.
    American journal of respiratory and critical care medicine, 2021, 03-01, Volume: 203, Issue:5

    Topics: Aminophenols; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Mutation; Precision Medicine; Quinolones

2021
Efficacy of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease.
    The European respiratory journal, 2021, Volume: 57, Issue:2

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
A 48-Year-Old Woman With Chronic Cough, Dyspnea, and Bronchiectasis.
    Chest, 2020, Volume: 158, Issue:5

    Topics: Alveolitis, Extrinsic Allergic; Aminophenols; Anti-Bacterial Agents; Bronchiectasis; Bronchoscopy; Cefazolin; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, Differential; Female; Genetic Testing; Humans; Late Onset Disorders; Middle Aged; Quinolones; Staphylococcal Infections; Staphylococcus aureus; Tomography, X-Ray Computed; Treatment Outcome

2020
Quantitative Method for the Analysis of Ivacaftor, Hydroxymethyl Ivacaftor, Ivacaftor Carboxylate, Lumacaftor, and Tezacaftor in Plasma and Sputum Using Liquid Chromatography With Tandem Mass Spectrometry and Its Clinical Applicability.
    Therapeutic drug monitoring, 2021, 08-01, Volume: 43, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chromatography, Liquid; Cystic Fibrosis; Drug Combinations; Humans; Indoles; Mutation; Plasma; Quinolones; Sputum; Tandem Mass Spectrometry

2021
Targeted deubiquitination rescues distinct trafficking-deficient ion channelopathies.
    Nature methods, 2020, Volume: 17, Issue:12

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Channelopathies; Cystic Fibrosis; Deubiquitinating Enzymes; Drug Combinations; Humans; Indoles; Ion Transport; Long QT Syndrome; Myocytes, Cardiac; Nedd4 Ubiquitin Protein Ligases; Potassium Channels; Pyrazoles; Pyridines; Quinolines; Quinolones

2020
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Longitudinal Studies; Male; Mutant Proteins; Mutation; Prospective Studies; Pseudomonas aeruginosa; Pseudomonas Infections; Quality of Life; Quinolones; Respiratory Function Tests; United States

2021
The Effects of Ivacaftor on Bone Density and Microarchitecture in Children and Adults with Cystic Fibrosis.
    The Journal of clinical endocrinology and metabolism, 2021, 03-08, Volume: 106, Issue:3

    Topics: Adolescent; Adult; Aged; Amino Acid Substitution; Aminophenols; Bone and Bones; Bone Density; Case-Control Studies; Child; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Middle Aged; Mutation, Missense; Quinolones; United States; Young Adult

2021
Rescue of multiple class II CFTR mutations by elexacaftor+tezacaftor+ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator.
    The European respiratory journal, 2021, Volume: 57, Issue:6

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; HEK293 Cells; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolines; Quinolones

2021
'Triple therapy' (elexacaftor, tezacaftor, ivacaftor) skin rash in patients with cystic fibrosis.
    Postgraduate medical journal, 2022, Volume: 98, Issue:1156

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Exanthema; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:3

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Female; Humans; Indoles; Infertility; Pregnancy; Pregnancy Rate; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Retrospective Studies

2021
The PROSPECT Is Bright for CFTR Modulators.
    Annals of the American Thoracic Society, 2021, Volume: 18, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones; Treatment Outcome

2021
Changes in fecal microbiota with CFTR modulator therapy: A pilot study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Adolescent; Adult; Aminophenols; Anti-Bacterial Agents; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Pancreatic Insufficiency; Feces; Humans; Microbiota; Pilot Projects; Quinolones; Young Adult

2021
Tribulations and (clinical) trials in cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cannabinoid Receptor Agonists; Chloride Channel Agonists; Clinical Trials as Topic; Cystic Fibrosis; Dronabinol; Drug Combinations; Humans; Indoles; Quinolones

2021
Modulation of cAMP metabolism for CFTR potentiation in human airway epithelial cells.
    Scientific reports, 2021, 01-13, Volume: 11, Issue:1

    Topics: Aminophenols; Bronchi; Cell Line; Cells, Cultured; Cyclic AMP; Cyclic Nucleotide Phosphodiesterases, Type 4; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Epithelial Cells; Humans; Multidrug Resistance-Associated Proteins; Phosphodiesterase 4 Inhibitors; Quinolones

2021
Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Clinical Trial Study Design.
    Annals of the American Thoracic Society, 2021, Volume: 18, Issue:8

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2021
The march towards CFTR modulator access for all people with CF: The end of the beginning.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Precision Medicine; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
CFTR modulators: transformative therapies for cystic fibrosis.
    Journal of managed care & specialty pharmacy, 2021, Volume: 27, Issue:2

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Combinations; Drug Costs; Humans; Indoles; Medical Assistance; Mutation; Pyrazoles; Pyridines; Quinolines; Quinolones; Treatment Outcome; United States; United States Food and Drug Administration

2021
The effectiveness and value of novel treatments for cystic fibrosis.
    Journal of managed care & specialty pharmacy, 2021, Volume: 27, Issue:2

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cost-Benefit Analysis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Combinations; Drug Costs; Health Policy; Humans; Indoles; Models, Economic; Mutation; Pyrazoles; Pyridines; Quinolines; Quinolones; Treatment Outcome; United States; United States Food and Drug Administration

2021
Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Microbiota; Quinolones; Respiratory Function Tests; Sputum; Young Adult

2021
Correction of the Gene Defect in Cystic Fibrosis: Is It Too Late for Bone?
    The Journal of clinical endocrinology and metabolism, 2021, 04-23, Volume: 106, Issue:5

    Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2021
Effect of CFTR modulator therapy on cystic fibrosis-related diabetes.
    Journal of diabetes and its complications, 2021, Volume: 35, Issue:6

    Topics: Adult; Aminophenols; Blood Glucose; Blood Glucose Self-Monitoring; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diabetes Mellitus; Humans; Insulin; Quinolones

2021
Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:6

    Topics: Adolescent; Adult; Aged; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Male; Middle Aged; Prospective Studies; Quinolones; Sweat

2021
Longitudinal effects of ivacaftor and medicine possession ratio in people with the
    Thorax, 2021, Volume: 76, Issue:9

    Topics: Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Mutation; Quinolones; Retrospective Studies

2021
Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
    The Lancet. Respiratory medicine, 2021, Volume: 9, Issue:7

    Topics: Adult; Aminophenols; Australia; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Europe; Female; Humans; Indoles; Israel; Male; Mutation; North America; Quinolones; Time; Treatment Outcome

2021
Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion.
    Journal of endocrinological investigation, 2021, Volume: 44, Issue:10

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Blood Glucose; Case-Control Studies; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Follow-Up Studies; Homozygote; Humans; Insulin Secretion; Male; Mutation; Prognosis; Quinolones; Retrospective Studies; Young Adult

2021
Novel Correctors and Potentiators Enhance Translational Readthrough in CFTR Nonsense Mutations.
    American journal of respiratory cell and molecular biology, 2021, Volume: 64, Issue:5

    Topics: Aminophenols; Aminopyridines; Animals; Benzoates; Benzodioxoles; Benzopyrans; Cell Line; Chlorides; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Ion Transport; Protein Biosynthesis; Pyrans; Pyrazoles; Quinolones; Rats; Recovery of Function; Thyroid Epithelial Cells

2021
SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Aminophenols; Antiporters; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Male; Polymorphism, Single Nucleotide; Prospective Studies; Quinolones; Respiratory Function Tests; Severity of Illness Index; Sulfate Transporters; Young Adult

2021
[Cystic fibrosis prognosis in Europa : chronicle of an announced dilemma].
    Revue medicale de Liege, 2021, Volume: 76, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Europe; Humans; Mutation; Prognosis; Quinolones

2021
Ex vivo model predicted in vivo efficacy of CFTR modulator therapy in a child with rare genotype.
    Molecular genetics & genomic medicine, 2021, Volume: 9, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cells, Cultured; Child, Preschool; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Female; Genotype; Humans; Mutation, Missense; Nasal Mucosa; Pancreatic Elastase; Quinolones

2021
To treat or not to treat: CFTR modulators after lung transplantation.
    Pediatric transplantation, 2021, Volume: 25, Issue:4

    Topics: Adolescent; Aminophenols; Benzodioxoles; Biomarkers; Combined Modality Therapy; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Lung Transplantation; Postoperative Care; Pyrazoles; Pyridines; Quinolines; Quinolones; Treatment Outcome; Young Adult

2021
Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:3

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Female; Humans; Indoles; Lactation; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Pregnancy Rate; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Surveys and Questionnaires

2021
CFTR modulator therapy improves cystic fibrosis-related diabetes. But how?
    Journal of diabetes and its complications, 2021, Volume: 35, Issue:6

    Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diabetes Mellitus; Humans; Indoles; Quinolones

2021
Real-world use of ivacaftor in Canada: A retrospective analysis using the Canadian Cystic Fibrosis Registry.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:6

    Topics: Adolescent; Adult; Aminophenols; Canada; Child; Chloride Channel Agonists; Cystic Fibrosis; Female; Humans; Longitudinal Studies; Male; Middle Aged; Pseudomonas Infections; Quinolones; Registries; Retrospective Studies

2021
Optical Measurements of Sweat for in Vivo Quantification of CFTR Function in Individual Sweat Glands.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Optics and Photonics; Quinolones; Sweat; Sweat Glands

2021
Ivacaftor partially corrects airway inflammation in a humanized G551D rat.
    American journal of physiology. Lung cellular and molecular physiology, 2021, 06-01, Volume: 320, Issue:6

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Inflammation; Ion Transport; Lung; Molecular Targeted Therapy; Mucociliary Clearance; Quinolones; Rats, Transgenic

2021
Identification of Potential Leukocyte Biomarkers Related to Drug Recovery of CFTR: Clinical Applications in Cystic Fibrosis.
    International journal of molecular sciences, 2021, Apr-10, Volume: 22, Issue:8

    Topics: Actin Cytoskeleton; Adult; Aminophenols; Biomarkers; Cell Movement; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Gene Expression Regulation; Humans; Leukocytes, Mononuclear; Male; Matrix Metalloproteinase 9; Proteome; Quinolones

2021
Patient personalized translational tools in cystic fibrosis to transform data from bench to bed-side and back.
    American journal of physiology. Gastrointestinal and liver physiology, 2021, 06-01, Volume: 320, Issue:6

    Topics: Aminophenols; Animals; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mice; Mutation; Organoids; Precision Medicine; Quinolones

2021
Long-term safety of lumacaftor-ivacaftor in children aged 2-5 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a multicentre, phase 3, open-label, extension study.
    The Lancet. Respiratory medicine, 2021, Volume: 9, Issue:9

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2021
Combination CFTR modulator therapy in children and adults with cystic fibrosis.
    The Lancet. Respiratory medicine, 2021, Volume: 9, Issue:7

    Topics: Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2021
Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Advanced Cystic Fibrosis Lung Disease.
    Annals of the American Thoracic Society, 2021, Volume: 18, Issue:11

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Pharmacokinetic interactions between ivacaftor and cytochrome P450 3A4 inhibitors in people with cystic fibrosis and healthy controls.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Adult; Aminophenols; Azithromycin; Case-Control Studies; Chloride Channel Agonists; Cystic Fibrosis; Cytochrome P-450 CYP3A Inhibitors; Drug Interactions; Drug Therapy, Combination; Female; Humans; Male; Middle Aged; Quinolones; Ritonavir

2021
CFTR function and clinical response to modulators parallel nasal epithelial organoid swelling.
    American journal of physiology. Lung cellular and molecular physiology, 2021, 07-01, Volume: 321, Issue:1

    Topics: Aminophenols; Benzodioxoles; Case-Control Studies; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Ion Transport; Mutation; Nasal Mucosa; Organoids; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2021
Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Aminophenols; Animals; Benzodioxoles; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Indoles; Ion Transport; Phosphates; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Rats; Rats, Inbred F344; Sodium-Phosphate Cotransporter Proteins, Type IIb

2021
Papilledema and hypervitaminosis A after elexacaftor/tezacaftor/ivacaftor for cystic fibrosis.
    Canadian journal of ophthalmology. Journal canadien d'ophtalmologie, 2022, Volume: 57, Issue:1

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Humans; Hypervitaminosis A; Indoles; Mutation; Papilledema; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Ivacaftor and Airway Inflammation in Preschool Children with Cystic Fibrosis.
    American journal of respiratory and critical care medicine, 2021, 09-01, Volume: 204, Issue:5

    Topics: Age Factors; Airway Obstruction; Aminophenols; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Infant; Inflammation; Male; Mutation; Quinolones

2021
Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Longitudinal Studies; Male; Middle Aged; Mucociliary Clearance; Prospective Studies; Quinolones; Young Adult

2022
Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.
    Respiratory medicine and research, 2021, Volume: 80

    Topics: Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Humans; Indoles; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quality of Life; Quinolones; Sleep Quality

2021
Ivacaftor-elexacaftor-tezacaftor and tacrolimus combination in cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:1

    Topics: Adolescent; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Indoles; Liver Transplantation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Tacrolimus

2022
Short-term effect of elexacaftor-tezacaftor-ivacaftor on lung function and transplant planning in cystic fibrosis patients with advanced lung disease.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Drug Therapy, Combination; Female; Humans; Indoles; Lung Transplantation; Male; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Respiratory Function Tests; Retrospective Studies

2021
Breast development in a 7 year old girl with CF treated with ivacaftor: An indication for personalized dosing?
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Aminophenols; Breast; Child; Chloride Channel Agonists; Cystic Fibrosis; Female; Humans; Puberty, Precocious; Quinolones

2021
Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:1

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Quinolones; Respiratory Function Tests

2022
Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021, Volume: 20, Issue:5

    Topics: Aminophenols; Animals; Brain; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Lung; Milk; Placenta; Pregnancy; Quinolones; Rats; Rats, Sprague-Dawley

2021
Drug-induced liver injury from elexacaftor/ivacaftor/tezacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Aminophenols; Benzodioxoles; Chemical and Drug Induced Liver Injury; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Response to elexacaftor/tezacaftor/ivacaftor in intestinal organoids derived from people with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Mutation; Organoids; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Elexacaftor/Tezacaftor/Ivacaftor Improved Clinical Outcomes in a Patient with N1303K-CFTR Based on
    American journal of respiratory and critical care medicine, 2021, 11-15, Volume: 204, Issue:10

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Aminophenols; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Gene Expression Regulation; Humans; Indoles; Male; Middle Aged; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Young Adult

2021
Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Liver Transplantation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones

2022
Ivacaftor withdrawal syndrome: A potentially life-threatening consequence from a life-saving medication.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2022
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:2

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Liver; Mutation; Prospective Studies; Quinolones

2022
Relationship of Antibiotic Treatment to Recovery after Acute FEV
    Annals of the American Thoracic Society, 2017, Volume: 14, Issue:6

    Topics: Adolescent; Ambulatory Care; Anti-Bacterial Agents; Child; Cystic Fibrosis; Disease Progression; Female; Forced Expiratory Volume; Humans; Logistic Models; Lung; Male; North America; Prospective Studies; Pseudomonas Infections; Quinolones

2017
Effects of Lumacaftor/Ivacaftor in a Pediatric Cohort Homozygous for F508del-CFTR.
    American journal of respiratory and critical care medicine, 2017, 04-01, Volume: 195, Issue:7

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2017
Pseudomonas eradication and clinical effectivness of Ivacaftor in four Hispanic patients with S549N.
    Pediatric pulmonology, 2017, Volume: 52, Issue:7

    Topics: Adolescent; Adolescent Development; Aminophenols; Child; Child Development; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Hispanic or Latino; Humans; Mutation; Pseudomonas; Pseudomonas Infections; Quinolones; Treatment Outcome

2017
An Observational Study of Outcomes and Tolerances in Patients with Cystic Fibrosis Initiated on Lumacaftor/Ivacaftor.
    Annals of the American Thoracic Society, 2017, Volume: 14, Issue:11

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Dyspnea; Female; Forced Expiratory Volume; Humans; Logistic Models; Lung; Male; Middle Aged; Mutation; Quinolones; Retrospective Studies; Young Adult

2017
Orkambi in patients with severe disease - Bumps in the road to CFTR modulation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Quinolones

2017
AJRCCM: 100-Year Anniversary. Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis.
    American journal of respiratory and critical care medicine, 2017, 05-01, Volume: 195, Issue:9

    Topics: Aminophenols; Aminopyridines; Anniversaries and Special Events; Anti-Bacterial Agents; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Enzyme Replacement Therapy; History, 20th Century; History, 21st Century; Mutation; Periodicals as Topic; Pulmonary Medicine; Quinolones

2017
Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:4

    Topics: Adolescent; Aminophenols; Aminopyridines; Antidepressive Agents; Anxiety; Benzodioxoles; Child; Cognitive Behavioral Therapy; Cystic Fibrosis; Depression; Drug Combinations; Female; Humans; Membrane Transport Modulators; Quinolones; Suicidal Ideation; Suicide, Attempted; Withholding Treatment

2017
Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Female; Humans; Male; Mucociliary Clearance; Mutation; Outcome Assessment, Health Care; Quinolones; Recovery of Function; Respiratory Function Tests

2018
FDA deems in vitro data on mutations sufficient to expand cystic fibrosis drug label.
    Nature biotechnology, 2017, 07-12, Volume: 35, Issue:7

    Topics: Aminophenols; Cystic Fibrosis; Drug Approval; Drug Labeling; Humans; Quinolones; United States; United States Food and Drug Administration

2017
Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2years of treatment with ivacaftor in a real-world setting.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Anti-Bacterial Agents; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; France; Humans; Male; Pseudomonas aeruginosa; Quinolones; Respiratory Function Tests; Respiratory System; Staphylococcus aureus; Time

2018
Comment on data sparsity - Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: A longitudinal study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:6

    Topics: Cystic Fibrosis; Humans; Longitudinal Studies; Prognosis; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones

2017
Lumacaftor/ivacaftor combination therapy for cystic fibrosis: A nationwide survey among clinicians.
    The clinical respiratory journal, 2018, Volume: 12, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Drug Combinations; Drug Therapy, Combination; Forced Expiratory Volume; Homozygote; Humans; Italy; Mutation; Quinolones; Surveys and Questionnaires; Treatment Outcome

2018
Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy.
    Hepatology (Baltimore, Md.), 2018, Volume: 67, Issue:3

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Biliary Tract; Cell Culture Techniques; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytokines; Cytoskeleton; Epithelial Cells; Fluorescent Antibody Technique; Humans; Induced Pluripotent Stem Cells; Inflammation; Mice; Microscopy, Confocal; Pyrimidines; Quinolones; Signal Transduction; src-Family Kinases

2018
The organoid architect.
    Science (New York, N.Y.), 2017, Aug-25, Volume: 357, Issue:6353

    Topics: Aminophenols; Cystic Fibrosis; Drug Evaluation, Preclinical; History, 20th Century; History, 21st Century; Humans; Intestines; Organoids; Quinolones

2017
Clinical trial research in focus: ensuring new cystic fibrosis drugs fulfil their potential.
    The Lancet. Respiratory medicine, 2017, Volume: 5, Issue:9

    Topics: Aminophenols; Aminopyridines; Anti-Bacterial Agents; Benzodioxoles; Chloride Channel Agonists; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Expectorants; Genetic Therapy; Humans; Indoles; Mutation; Patient Selection; Quality of Life; Quinolones

2017
FDA OKs first in vitro route to expanded approval.
    Nature reviews. Drug discovery, 2017, 09-01, Volume: 16, Issue:9

    Topics: Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Humans; Mutation; Quinolones; United States; United States Food and Drug Administration

2017
Vertex CF data wow Wall Street.
    Nature biotechnology, 2017, 09-11, Volume: 35, Issue:9

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Humans; Quinolones

2017
Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience.
    Respiratory medicine, 2017, Volume: 131

    Topics: Adolescent; Adult; Aminophenols; Blood Glucose; Body Mass Index; Child; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Glucose Tolerance Test; Humans; Israel; Male; Mutation; Quinolones; Retrospective Studies; Sweat; Treatment Outcome; Vital Capacity; Young Adult

2017
Cigarette smoke activates CFTR through ROS-stimulated cAMP signaling in human bronchial epithelial cells.
    American journal of physiology. Cell physiology, 2018, 01-01, Volume: 314, Issue:1

    Topics: Aminophenols; Aminopyridines; Autocrine Communication; Benzodioxoles; Bronchi; Calcium Signaling; Cell Line; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Mutation; Oxidative Stress; Quinolones; Reactive Oxygen Species; Receptors, Prostaglandin E, EP4 Subtype; Second Messenger Systems; Secretory Pathway; Tobacco Smoke Pollution

2018
The U.S. Food and Drug Administration's Experience with Ivacaftor in Cystic Fibrosis. Establishing Efficacy Using In Vitro Data in Lieu of a Clinical Trial.
    Annals of the American Thoracic Society, 2018, Volume: 15, Issue:1

    Topics: Aminophenols; Chloride Channel Agonists; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Development; Humans; Mutation; Quinolones; Sweat; United States; United States Food and Drug Administration

2018
Homogeneity of Aerosol Deposition and Mucociliary Clearance are Improved Following Ivacaftor Treatment in Cystic Fibrosis.
    Journal of aerosol medicine and pulmonary drug delivery, 2018, Volume: 31, Issue:4

    Topics: Administration, Inhalation; Adolescent; Adult; Aerosols; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Mucociliary Clearance; Quinolones; Young Adult

2018
CORK Study in Cystic Fibrosis: Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor.
    Chest, 2018, Volume: 153, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Male; Prospective Studies; Quinolones; Radiography, Thoracic; Saliva; Tomography, X-Ray Computed; Young Adult

2018
Mutation-specific downregulation of CFTR2 variants by gating potentiators.
    Human molecular genetics, 2017, 12-15, Volume: 26, Issue:24

    Topics: Aminophenols; Bronchi; Cell Line; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Down-Regulation; Drug Synergism; Epithelial Cells; Humans; Ion Channel Gating; Lung; Models, Molecular; Mutation; Quinolones; Respiratory Mucosa

2017
Reduction of Recurrence Risk of Pancreatitis in Cystic Fibrosis With Ivacaftor: Case Series.
    Journal of pediatric gastroenterology and nutrition, 2018, Volume: 66, Issue:3

    Topics: Adolescent; Adult; Aminophenols; Child; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Male; Middle Aged; Pancreatitis, Chronic; Quinolones; Recurrence; Retrospective Studies; Secondary Prevention; Treatment Outcome; Young Adult

2018
Breathing Easier: A Well-tolerated Corrector for F508del.
    American journal of respiratory and critical care medicine, 2018, 01-15, Volume: 197, Issue:2

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Quinolones

2018
Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:2

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Male; Mutation; Quinolones; Substance Withdrawal Syndrome; Young Adult

2018
Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: A longitudinal study; response to comments.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:6

    Topics: Cystic Fibrosis; Humans; Longitudinal Studies; Prognosis; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones

2017
Effects of Ivacaftor in Three Pediatric Siblings With Cystic Fibrosis Carrying the Mutations G551D And F508del.
    Archivos de bronconeumologia, 2018, Volume: 54, Issue:4

    Topics: Adolescent; Aminophenols; Child, Preschool; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Ion Transport; Male; Mutation, Missense; Pancreas; Quinolones; Sequence Deletion; Treatment Outcome

2018
Lumacaftor (VX-809) restores the ability of CF macrophages to phagocytose and kill Pseudomonas aeruginosa.
    American journal of physiology. Lung cellular and molecular physiology, 2018, 03-01, Volume: 314, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Forced Expiratory Volume; Humans; Macrophages; Mutation; Phagocytosis; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones

2018
Discovery of N-(3-Carbamoyl-5,5,7,7-tetramethyl-5,7-dihydro-4H-thieno[2,3-c]pyran-2-yl)-lH-pyrazole-5-carboxamide (GLPG1837), a Novel Potentiator Which Can Open Class III Mutant Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channels to a High
    Journal of medicinal chemistry, 2018, 02-22, Volume: 61, Issue:4

    Topics: Aminophenols; Animals; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Discovery; Humans; Mutant Proteins; Mutation; Pyrazoles; Quinolones; Rats; Structure-Activity Relationship

2018
Optimized LC-MS/MS Method for the High-throughput Analysis of Clinical Samples of Ivacaftor, Its Major Metabolites, and Lumacaftor in Biological Fluids of Cystic Fibrosis Patients.
    Journal of visualized experiments : JoVE, 2017, 10-15, Issue:128

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Case-Control Studies; Chromatography, Liquid; Cystic Fibrosis; High-Throughput Screening Assays; Humans; Quinolones; Tandem Mass Spectrometry

2017
Ivacaftor for cystic fibrosis: An evaluation of real world utilisation and expenditure in the Irish Healthcare Setting.
    Irish medical journal, 2017, Aug-12, Volume: 110, Issue:7

    Topics: Adolescent; Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Health Expenditures; Humans; Ireland; Male; Mutation; Quinolones; Young Adult

2017
Nasospheroids permit measurements of CFTR-dependent fluid transport.
    JCI insight, 2017, 11-16, Volume: 2, Issue:22

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biological Transport; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Nasal Mucosa; Particle Size; Precision Medicine; Quinolones; Spheroids, Cellular

2017
Treating Specific Variants Causing Cystic Fibrosis.
    JAMA, 2017, 12-05, Volume: 318, Issue:21

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genetic Variation; Humans; Quinolones

2017
Mucus and Cell-Penetrating Nanoparticles Embedded in Nano-into-Micro Formulations for Pulmonary Delivery of Ivacaftor in Patients with Cystic Fibrosis.
    ACS applied materials & interfaces, 2018, Jan-10, Volume: 10, Issue:1

    Topics: Aminophenols; Cystic Fibrosis; Drug Carriers; Humans; Mucus; Nanoparticles; Quinolones

2018
The expression of Mirc1/Mir17-92 cluster in sputum samples correlates with pulmonary exacerbations in cystic fibrosis patients.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:4

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Chloride Channel Agonists; Correlation of Data; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Drug Combinations; Drug Monitoring; Female; Gene Expression Profiling; Humans; Male; MicroRNAs; Nerve Tissue Proteins; Quinolones; Respiratory Function Tests; Respiratory System; RNA, Long Noncoding; Sputum

2018
Forecasting the Long-Term Clinical and Economic Outcomes of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients with Homozygous phe508del Mutation.
    Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research, 2017, Volume: 20, Issue:10

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Forced Expiratory Volume; Homozygote; Humans; Markov Chains; Mutation; Quality-Adjusted Life Years; Quinolones; Severity of Illness Index; Treatment Outcome; United States

2017
Cystic Fibrosis Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity.
    Immunity, 2017, 12-19, Volume: 47, Issue:6

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gene Expression Regulation; Humans; Mice; Mice, Inbred C57BL; Mice, Knockout; Models, Molecular; Monocytes; Phosphatidylinositol 3-Kinases; Protein Binding; Protein Conformation; Protein Transport; Proto-Oncogene Proteins c-akt; Pseudomonas aeruginosa; Pseudomonas Infections; PTEN Phosphohydrolase; Quinolones; Signal Transduction

2017
Tezacaftor-ivacaftor is safe and efficacious in patients with cystic fibrosis with Phe508del mutations.
    The Lancet. Respiratory medicine, 2018, Volume: 6, Issue:1

    Topics: Aminophenols; Benzodioxoles; Cystic Fibrosis; Heterozygote; Humans; Indoles; Mutation; Quinolones

2018
Effect of Lumacaftor/Ivacaftor on glucose metabolism and insulin secretion in Phe508del homozygous cystic fibrosis patients.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Blood Glucose; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Glucose Tolerance Test; Homozygote; Humans; Insulin Secretion; Male; Quinolones; Young Adult

2018
Ivacaftor-treated Patients with Cystic Fibrosis Derive Long-Term Benefit Despite No Short-Term Clinical Improvement.
    American journal of respiratory and critical care medicine, 2018, 06-01, Volume: 197, Issue:11

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Aminophenols; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Follow-Up Studies; Humans; Male; Middle Aged; Quinolones; Time Factors; Treatment Outcome; Young Adult

2018
CFTR Protein Function Modulation Therapy Is Finally Targeting Cystic Fibrosis-related Gastrointestinal Disease.
    Journal of pediatric gastroenterology and nutrition, 2018, Volume: 66, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gastrointestinal Diseases; Humans; Pancreatitis; Quinolones

2018
Cis variants identified in F508del complex alleles modulate CFTR channel rescue by small molecules.
    Human mutation, 2018, Volume: 39, Issue:4

    Topics: Alleles; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Phenylalanine; Quinolones

2018
Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:2

    Topics: Adult; Alleles; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Male; Mutation; Quinolones; Sweat Glands; Sweating

2018
Pseudomonas aeruginosa in cystic fibrosis patients with c.1652G›A (G551D)-CFTR treated with ivacaftor-Changes in microbiological parameters.
    Journal of clinical pharmacy and therapeutics, 2018, Volume: 43, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Anti-Bacterial Agents; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Microbial Sensitivity Tests; Mutation; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Retrospective Studies; Young Adult

2018
Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis.
    American journal of respiratory and critical care medicine, 2018, 06-01, Volume: 197, Issue:11

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Germany; Homozygote; Humans; Male; Prospective Studies; Quinolones; Young Adult

2018
Vx-809/Vx-770 treatment reduces inflammatory response to Pseudomonas aeruginosa in primary differentiated cystic fibrosis bronchial epithelial cells.
    American journal of physiology. Lung cellular and molecular physiology, 2018, 04-01, Volume: 314, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cells, Cultured; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Interleukin-8; Mutation; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones

2018
Nucleic acid based therapies: developing frontier for precision medicine.
    BMJ (Clinical research ed.), 2018, 01-23, Volume: 360

    Topics: Adenoviridae; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Genomics; Hemophilia A; Humans; Molecular Targeted Therapy; Motor Neuron Disease; Mutation; Nucleic Acids; Oligonucleotides, Antisense; Precision Medicine; Quinolones; United Kingdom

2018
Biomarkers: Their Role in CFTR Modulator Therapies from Early Development to the Clinic.
    American journal of respiratory and critical care medicine, 2018, 06-01, Volume: 197, Issue:11

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2018
First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial - preliminary results.
    Swiss medical weekly, 2018, Volume: 148

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Genotype; Homozygote; Humans; Lung Transplantation; Male; Mutation; Prospective Studies; Quinolones; Severity of Illness Index; Switzerland

2018
Metabolomic responses to lumacaftor/ivacaftor in cystic fibrosis.
    Pediatric pulmonology, 2018, Volume: 53, Issue:5

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Chromatography, Liquid; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Male; Metabolomics; Middle Aged; Quinolones; Treatment Outcome; Young Adult

2018
Chronic β2AR stimulation limits CFTR activation in human airway epithelia.
    JCI insight, 2018, 02-22, Volume: 3, Issue:4

    Topics: Adrenergic beta-2 Receptor Agonists; Albuterol; Aminophenols; Aminopyridines; Benzodioxoles; Cell Line; Cilia; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Interactions; Epithelial Cells; Humans; Mutation; Quinolones; Respiratory Mucosa; Time Factors

2018
Miniaturized dispersive liquid-liquid microextraction and MALDI MS using ionic liquid matrices for the detection of bacterial communication molecules and virulence factors.
    Analytical and bioanalytical chemistry, 2018, Volume: 410, Issue:19

    Topics: 4-Quinolones; Cystic Fibrosis; Humans; Ionic Liquids; Isotope Labeling; Liquid Phase Microextraction; Pseudomonas aeruginosa; Pyocyanine; Quinolones; Quorum Sensing; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Virulence Factors

2018
Sensitivity of ivacaftor to drug-drug interactions with rifampin, a cytochrome P450 3A4 inducer.
    Pediatric pulmonology, 2018, Volume: 53, Issue:5

    Topics: Adult; Aminophenols; Anti-Bacterial Agents; Chloride Channel Agonists; Cystic Fibrosis; Cytochrome P-450 CYP3A; Drug Interactions; Female; Humans; Medication Adherence; Methicillin-Resistant Staphylococcus aureus; Quinolones; Rifampin; Staphylococcal Infections; Treatment Outcome

2018
CFTR Modulators: Deciding What Is Best for Individuals in an Era of Precision Medicine.
    Annals of the American Thoracic Society, 2018, Volume: 15, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Precision Medicine; Quinolones

2018
Personalised CFTR pharmacotherapeutic response testing and therapy of cystic fibrosis.
    The European respiratory journal, 2018, Volume: 51, Issue:6

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Male; Mutation; Precision Medicine; Quinolones

2018
Cytochrome P450 3A4 Induction: Lumacaftor versus Ivacaftor Potentially Resulting in Significantly Reduced Plasma Concentration of Ivacaftor.
    Drug metabolism letters, 2018, Volume: 12, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytochrome P-450 CYP1A2; Cytochrome P-450 CYP2C9; Cytochrome P-450 CYP3A; Drug Combinations; Drug Interactions; Enzyme Assays; Enzyme Induction; Female; Humans; Indoles; Quinolones

2018
Changes in Lung Clearance Index in Preschool-aged Patients with Cystic Fibrosis Treated with Ivacaftor (GOAL): A Clinical Trial.
    American journal of respiratory and critical care medicine, 2018, 08-15, Volume: 198, Issue:4

    Topics: Aminophenols; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Female; Humans; Lung; Male; Quinolones; Treatment Outcome

2018
CFTR rescue with VX-809 and VX-770 favors the repair of primary airway epithelial cell cultures from patients with class II mutations in the presence of Pseudomonas aeruginosa exoproducts.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Exotoxins; Glycine; Humans; Hydrazines; Mutation; Pseudomonas aeruginosa; Quinolones; Regeneration; Respiratory Mucosa

2018
Impact of a cystic fibrosis transmembrane conductance regulator (CFTR) modulator on high-dose ibuprofen therapy in pediatric cystic fibrosis patients.
    Pediatric pulmonology, 2018, Volume: 53, Issue:8

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Interactions; Drug Therapy, Combination; Female; Humans; Ibuprofen; Male; Quinolones

2018
Precision Medicine In Action: The Impact Of Ivacaftor On Cystic Fibrosis-Related Hospitalizations.
    Health affairs (Project Hope), 2018, Volume: 37, Issue:5

    Topics: Adolescent; Adult; Age Factors; Aminophenols; Child; Cohort Studies; Cost Savings; Cystic Fibrosis; Databases, Factual; Female; Hospitalization; Humans; Male; Middle Aged; Precision Medicine; Prescriptions; Quinolones; Retrospective Studies; Risk Assessment; Sex Factors; Treatment Outcome; United States; United States Food and Drug Administration; Young Adult

2018
Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor.
    Thorax, 2018, Volume: 73, Issue:8

    Topics: Adolescent; Adult; Aminophenols; Child; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Disease Progression; Female; Humans; Infant; Male; Quinolones; Registries; Respiratory Function Tests; Treatment Outcome; United Kingdom; United States

2018
Ivacaftor for cystic fibrosis.
    BMJ (Clinical research ed.), 2018, 05-17, Volume: 361

    Topics: Aminophenols; Chloride Channel Agonists; Contraindications, Drug; Cost-Benefit Analysis; Cystic Fibrosis; Dose-Response Relationship, Drug; Double-Blind Method; Evidence-Based Medicine; Forced Expiratory Volume; Humans; Mutation; Patient Education as Topic; Practice Guidelines as Topic; Quinolones; Randomized Controlled Trials as Topic; Respiratory Function Tests; Treatment Outcome

2018
Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:6

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Breast Feeding; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Monitoring; Female; Fetal Blood; Humans; Infant, Newborn; Liver Function Tests; Milk, Human; Pregnancy; Pregnancy Complications; Prenatal Exposure Delayed Effects; Quinolones; Treatment Outcome

2018
An automated computed tomography score for the cystic fibrosis lung.
    European radiology, 2018, Volume: 28, Issue:12

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cross-Sectional Studies; Cystic Fibrosis; Female; Forced Expiratory Volume; Humans; Lung; Male; Observer Variation; Quinolones; Reproducibility of Results; Respiratory Function Tests; Retrospective Studies; Tomography, Spiral Computed; Young Adult

2018
Treatment of cystic fibrosis in infants.
    The Lancet. Respiratory medicine, 2018, Volume: 6, Issue:7

    Topics: Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant; Mutation; Quinolones

2018
Ivacaftor CFTR Potentiator Therapy is Efficient for Pancreatic Manifestations in Cystic Fibrosis.
    The American journal of gastroenterology, 2018, Volume: 113, Issue:7

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, Differential; Humans; Male; Middle Aged; Pancreatitis; Quinolones; Recurrence

2018
Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:5

    Topics: Aminophenols; Animals; Cell Line; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Humans; Ion Channel Gating; Mutant Proteins; Mutation; Quinolones; Structure-Activity Relationship; Sulfonamides

2018
Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.
    Molecular pharmacology, 2018, Volume: 94, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cell Line; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Freeze Drying; HEK293 Cells; Humans; Microscopy, Fluorescence; Mutation; Protein Stability; Quinolones; Sulfate Transporters

2018
The use of lumacaftor/ivacaftor to treat acute deterioration in paediatric cystic fibrosis.
    Paediatric respiratory reviews, 2018, Volume: 27

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Drug Combinations; Female; Homozygote; Humans; Lung; Membrane Transport Modulators; Mutation; Patient Care Management; Quinolones; Respiratory Function Tests; Symptom Flare Up; Treatment Outcome

2018
Overexpression of RANK and M-CSFR in Monocytes of G551D-Bearing Patients with Cystic Fibrosis.
    American journal of respiratory and critical care medicine, 2018, 10-01, Volume: 198, Issue:7

    Topics: Aminophenols; Cells, Cultured; Chloride Channel Agonists; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Gene Expression Regulation; Humans; Male; Monocytes; Mutation; Prognosis; Quinolones; Receptor Activator of Nuclear Factor-kappa B; Receptor, Macrophage Colony-Stimulating Factor

2018
Does ivacaftor interfere with the antimicrobial activity of commonly used antibiotics against Pseudomonas aeruginosa?-Results of an in vitro study.
    Journal of clinical pharmacy and therapeutics, 2018, Volume: 43, Issue:6

    Topics: Adult; Aminophenols; Anti-Bacterial Agents; Case-Control Studies; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Interactions; Humans; In Vitro Techniques; Microbial Sensitivity Tests; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones

2018
Italian and North American dietary intake after ivacaftor treatment for Cystic Fibrosis Gating Mutations.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Child; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diet; Dietary Supplements; DNA; DNA Mutational Analysis; Energy Intake; Energy Metabolism; Female; Humans; Italy; Male; Middle Aged; Morbidity; Mutation; North America; Quinolones; Young Adult

2019
Energy Balance and Mechanisms of Weight Gain with Ivacaftor Treatment of Cystic Fibrosis Gating Mutations.
    The Journal of pediatrics, 2018, Volume: 201

    Topics: Adolescent; Adult; Aminophenols; Child; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; DNA Mutational Analysis; Energy Metabolism; Female; Humans; Male; Middle Aged; Mutation; Quality of Life; Quinolones; Treatment Outcome; Weight Gain; Young Adult

2018
Residual function of cystic fibrosis mutants predicts response to small molecule CFTR modulators.
    JCI insight, 2018, 07-26, Volume: 3, Issue:14

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Therapy, Combination; HEK293 Cells; Humans; Mutation; Quinolones

2018
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.
    JCI insight, 2018, 08-09, Volume: 3, Issue:15

    Topics: Adult; Aminophenols; Animals; Bicarbonates; Biological Transport, Active; Bronchoalveolar Lavage Fluid; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Female; Humans; Hydrogen-Ion Concentration; Infant; Infant, Newborn; Ion Transport; Longitudinal Studies; Lung; Male; Middle Aged; Mutation; Quinolones; Respiratory Mucosa; Sweat; Young Adult

2018
[Real-world effectiveness of ivacaftor in children with cystic fibrosis and the G551D mutation].
    Anales de pediatria, 2019, Volume: 90, Issue:3

    Topics: Adolescent; Aminophenols; Aspergillosis, Allergic Bronchopulmonary; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Male; Mutation; Mycobacterium abscessus; Mycobacterium Infections, Nontuberculous; Nutritional Status; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Treatment Outcome

2019
Continuous glucose monitoring in youth with cystic fibrosis treated with lumacaftor-ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:1

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Blood Glucose; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; Drug Combinations; Female; Forced Expiratory Volume; Glucose Tolerance Test; Glycated Hemoglobin; Homozygote; Humans; Male; Mutation; Prognosis; Quinolones; Treatment Outcome

2019
Islet Hormone and Incretin Secretion in Cystic Fibrosis after Four Months of Ivacaftor Therapy.
    American journal of respiratory and critical care medicine, 2019, 02-01, Volume: 199, Issue:3

    Topics: Adolescent; Adult; Aminophenols; Blood Glucose; C-Peptide; Child; Cystic Fibrosis; Female; Follow-Up Studies; Glucose Tolerance Test; Humans; Incretins; Insulin; Male; Quinolones; Young Adult

2019
Potentiation of the cystic fibrosis transmembrane conductance regulator Cl
    American journal of physiology. Lung cellular and molecular physiology, 2018, 11-01, Volume: 315, Issue:5

    Topics: Aminophenols; Animals; Benzodioxoles; Cell Line; Cricetinae; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Ion Channel Gating; Ion Transport; Mice; Mice, Inbred CFTR; Mutation; Quinolones; Temperature

2018
Prolonged co-treatment with HGF sustains epithelial integrity and improves pharmacological rescue of Phe508del-CFTR.
    Scientific reports, 2018, 08-29, Volume: 8, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cell Line; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Hepatocyte Growth Factor; Humans; Models, Biological; Mutant Proteins; Quinolones; Treatment Outcome

2018
Is Cystic Fibrosis-related Diabetes Reversible? New Data on CFTR Potentiation and Insulin Secretion.
    American journal of respiratory and critical care medicine, 2019, 02-01, Volume: 199, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diabetes Mellitus; Humans; Incretins; Insulin Secretion; Quinolones

2019
Rate and predictors of prescription of lumacaftor - Ivacaftor in the 18 months following approval in the United States.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:6

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Drug Prescriptions; Eligibility Determination; Female; Humans; Insurance Claim Review; Male; Product Surveillance, Postmarketing; Quinolones; Registries; Severity of Illness Index; United States

2018
False-positive cannabinoid screens in adult cystic fibrosis patients treated with lumacaftor/ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:6

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cannabinoids; Chloride Channel Agonists; Cystic Fibrosis; Diagnostic Errors; Drug Combinations; False Positive Reactions; Female; Humans; Male; Middle Aged; Quinolones; Substance Abuse Detection

2018
Cost-Effectiveness and Budget Impact of Lumacaftor/Ivacaftor in the Treatment of Cystic Fibrosis.
    Journal of managed care & specialty pharmacy, 2018, Volume: 24, Issue:10

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Budgets; Clinical Decision-Making; Cost-Benefit Analysis; Cystic Fibrosis; Decision Support Techniques; Drug Combinations; Drug Costs; Forced Expiratory Volume; Humans; Lung; Models, Economic; Quality-Adjusted Life Years; Quinolones; Respiratory System Agents; Time Factors; Treatment Outcome

2018
Cost-effectiveness analysis of lumacaftor and ivacaftor combination for the treatment of patients with cystic fibrosis in the United States.
    Orphanet journal of rare diseases, 2018, 09-29, Volume: 13, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Cost-Benefit Analysis; Cystic Fibrosis; Female; Humans; Male; Quality-Adjusted Life Years; Quinolones; United States

2018
Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:2

    Topics: Adult; Aminophenols; Biomarkers; Body Mass Index; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Female; Forced Expiratory Volume; Humans; Male; Mutation; Quinolones; Respiratory Function Tests; Retrospective Studies; Sweat; WAP Four-Disulfide Core Domain Protein 2

2019
IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Bile Acids and Salts; Biological Availability; Child; Chloride Channel Agonists; Cholestenones; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Enterohepatic Circulation; Female; Fibroblast Growth Factors; Homeostasis; Humans; Male; Mutation; Netherlands; Quinolones

2019
The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model.
    The European respiratory journal, 2018, Volume: 52, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cell Line; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Inflammation; Mutation; Quinolones; Respiratory System

2018
Cystic Fibrosis: Emerging Understanding and Therapies.
    Annual review of medicine, 2019, 01-27, Volume: 70

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chronic Disease; Combined Modality Therapy; Comprehension; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Management; Disease Progression; Drug Approval; Female; Genetic Predisposition to Disease; Humans; Lung Transplantation; Male; Prognosis; Quinolones; Risk Assessment; Severity of Illness Index; Survival Analysis; United States; United States Food and Drug Administration

2019
Triple CFTR Modulator Therapy for Cystic Fibrosis.
    The New England journal of medicine, 2018, 10-25, Volume: 379, Issue:17

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2018
Tezacaftor/Ivacaftor (Symdeko) for cystic fibrosis.
    The Medical letter on drugs and therapeutics, 2018, Oct-22, Volume: 60, Issue:1558

    Topics: Administration, Oral; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Administration Schedule; Drug Combinations; Drug Interactions; Humans; Indoles; Lung; Membrane Transport Modulators; Mutation; Quinolones; Treatment Outcome

2018
Positive clinical response to ivacaftor treatment in an individual with the CFTR genotype F508del/V456A.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:2

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Humans; Male; Mutation; Quinolones; Respiratory Function Tests; Sweat; Treatment Outcome

2019
Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis.
    The Journal of physiology, 2019, Volume: 597, Issue:2

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Chloride Channel Agonists; CHO Cells; Cricetulus; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Ion Channel Gating; Mutation; Protein Conformation; Quinolones

2019
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function.
    Scientific reports, 2018, 11-20, Volume: 8, Issue:1

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Case-Control Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Cytokines; Drug Combinations; Female; Humans; Ion Transport; Macrophages; Male; Mutation; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Signal Transduction

2018
Ivacaftor improves rhinologic, psychologic, and sleep-related quality of life in G551D cystic fibrosis patients.
    International forum of allergy & rhinology, 2019, Volume: 9, Issue:3

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Aminophenols; Child; Chronic Disease; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Mental Health; Middle Aged; Mutation; Prospective Studies; Quality of Life; Quinolones; Rhinitis; Sinusitis; Sleep; Surveys and Questionnaires; Young Adult

2019
Compassionate Use of Lumacaftor/Ivacaftor in Cystic Fibrosis: Spanish Experience.
    Archivos de bronconeumologia, 2018, Volume: 54, Issue:12

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonists; Compassionate Use Trials; Cystic Fibrosis; Drug Combinations; Female; Humans; Male; Middle Aged; Quinolones; Retrospective Studies; Spain; Young Adult

2018
Corticosteroid use and increased CXCR2 levels on leukocytes are associated with lumacaftor/ivacaftor discontinuation in cystic fibrosis patients homozygous for the F508del CFTR mutation.
    PloS one, 2018, Volume: 13, Issue:12

    Topics: Adrenal Cortex Hormones; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Forced Expiratory Volume; Gene Deletion; Homozygote; Humans; Leukocytes; Logistic Models; Male; Medication Adherence; Quinolones; Receptors, Interleukin-8B; Tetraspanin 30

2018
Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR.
    JCI insight, 2018, 12-20, Volume: 3, Issue:24

    Topics: Adolescent; Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Longitudinal Studies; Male; Mucociliary Clearance; Mutation; Prospective Studies; Quinolones; Respiratory Function Tests; Treatment Outcome; Young Adult

2018
Predictive factors for lumacaftor/ivacaftor clinical response.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers, Pharmacological; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Monitoring; Female; Humans; Male; Mutation; Pharmacogenomic Testing; Quinolones; Respiratory Function Tests; Sweat; Treatment Outcome; Young Adult

2019
Cystic fibrosis: What's new in South Africa in 2019.
    South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde, 2018, Dec-13, Volume: 109, Issue:1

    Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Testing; Humans; Indoles; Molecular Diagnostic Techniques; Mutation; Practice Guidelines as Topic; Prospective Studies; Public-Private Sector Partnerships; Quinolones; Registries; South Africa

2018
Combination therapy.
    Nature medicine, 2019, Volume: 25, Issue:1

    Topics: Alleles; Aminophenols; Benzodioxoles; Cystic Fibrosis; Humans; Indoles; Organothiophosphorus Compounds; Quinolones

2019
Folding-function relationship of the most common cystic fibrosis-causing CFTR conductance mutants.
    Life science alliance, 2019, Volume: 2, Issue:1

    Topics: Alleles; Aminophenols; Aminopyridines; Benzodioxoles; Biopsy; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; HEK293 Cells; Humans; Mutation; Organoids; Protein Folding; Protein Structure, Tertiary; Quinolones; Rectum; Transfection

2019
Inhalation treatment of cystic fibrosis with lumacaftor and ivacaftor co-delivered by nanostructured lipid carriers.
    Journal of controlled release : official journal of the Controlled Release Society, 2019, 02-28, Volume: 296

    Topics: Administration, Inhalation; Aminophenols; Aminopyridines; Animals; Benzodioxoles; Cell Line; Chloride Channel Agonists; Cystic Fibrosis; Drug Carriers; Drug Combinations; Humans; Lipids; Lung; Mice, Transgenic; Nanostructures; Quinolones

2019
The study of CFTR modulators in the very young.
    The Lancet. Respiratory medicine, 2019, Volume: 7, Issue:4

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2019
After the Celebrations: Lessons from the New Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy.
    Annals of the American Thoracic Society, 2019, Volume: 16, Issue:2

    Topics: Aminophenols; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Ireland; Quinolones; Registries

2019
Immeasurable Time Bias In Exposure to Ivacaftor.
    Health affairs (Project Hope), 2019, Volume: 38, Issue:2

    Topics: Adult; Aminophenols; Bias; Chloride Channel Agonists; Cystic Fibrosis; Humans; Quinolones; Research Design; Time Factors

2019
Cystic Fibrosis And Ivacaftor Use: The Authors Reply.
    Health affairs (Project Hope), 2019, Volume: 38, Issue:2

    Topics: Aminophenols; Bias; Cystic Fibrosis; Humans; Quinolones

2019
Extent of rescue of F508del-CFTR function by VX-809 and VX-770 in human nasal epithelial cells correlates with SNP rs7512462 in SLC26A9 gene in F508del/F508del Cystic Fibrosis patients.
    Biochimica et biophysica acta. Molecular basis of disease, 2019, 06-01, Volume: 1865, Issue:6

    Topics: Alleles; Aminophenols; Aminopyridines; Antiporters; Base Sequence; Benzodioxoles; Body Mass Index; Case-Control Studies; Cellular Reprogramming; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diffusion Chambers, Culture; Epithelial Cells; Gene Expression; Genotype; Humans; Models, Biological; Nasal Mucosa; Polymorphism, Single Nucleotide; Primary Cell Culture; Quinolones; Sequence Deletion; Sulfate Transporters; Sweat

2019
Exhaled nitric oxide in stable adult cystic fibrosis patients, during exacerbation and following CFTR-modifying treatment.
    The European respiratory journal, 2019, Volume: 53, Issue:5

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Breath Tests; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Drug Combinations; Exhalation; Female; Humans; Male; Nitric Oxide; Prospective Studies; Quinolones; Young Adult

2019
Nonsense-mediated RNA Decay Pathway Inhibition Restores Expression and Function of W1282X CFTR.
    American journal of respiratory cell and molecular biology, 2019, Volume: 61, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Homozygote; Humans; Mutation; Nonsense Mediated mRNA Decay; Quinolones; RNA

2019
Effects of Lumacaftor/Ivacaftor on physical activity and exercise tolerance in three adults with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2019, Volume: 18, Issue:3

    Topics: Activities of Daily Living; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Monitoring; Exercise; Exercise Tolerance; Forced Expiratory Volume; Humans; Male; Middle Aged; Mutation; Oxygen Consumption; Quinolones; Treatment Outcome

2019
In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis.
    Science translational medicine, 2019, 03-27, Volume: 11, Issue:485

    Topics: Aminophenols; Animals; Animals, Genetically Modified; Animals, Newborn; Blood Glucose; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Disease Progression; Female; Ferrets; Gene Knock-In Techniques; Genitalia, Male; Gestational Age; Humans; Male; Mutation; Pancreas, Exocrine; Pregnancy; Quinolones; Respiratory Tract Infections; Translational Research, Biomedical

2019
Use of ivacaftor in late diagnosed cystic fibrosis monozygotic twins heterozygous for F508del and R117H-7T - a case report.
    BMC pulmonary medicine, 2019, Apr-11, Volume: 19, Issue:1

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Heterozygote; Humans; Lung; Middle Aged; Mutation; Quinolones; Twins, Monozygotic

2019
Effectiveness of ivacaftor in cystic fibrosis: Improvement of liver cirrhosis, nutritional status and respiratory function.
    Medicina clinica, 2020, 07-24, Volume: 155, Issue:2

    Topics: Aminophenols; Cystic Fibrosis; Forced Expiratory Volume; Humans; Liver Cirrhosis; Mutation; Nutritional Status; Quinolones

2020
Phenotyping ciliary dynamics and coordination in response to CFTR-modulators in Cystic Fibrosis respiratory epithelial cells.
    Nature communications, 2019, 04-16, Volume: 10, Issue:1

    Topics: Algorithms; Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cilia; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Epithelial Cells; Genotype; Humans; Microscopy; Oscillometry; Phenotype; Quinolones; Video Recording

2019
Transcriptomic Responses to Ivacaftor and Prediction of Ivacaftor Clinical Responsiveness.
    American journal of respiratory cell and molecular biology, 2019, Volume: 61, Issue:5

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Leukocytes, Mononuclear; Mucociliary Clearance; Mutation; Quinolones; Transcriptome

2019
Impact of CFTR-modulating drugs on GH-IGF-1 axis impairment in adult patients with cystic fibrosis.
    Journal of endocrinological investigation, 2019, Volume: 42, Issue:11

    Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Follow-Up Studies; Growth Disorders; Human Growth Hormone; Humans; Insulin-Like Growth Factor I; Male; Middle Aged; Mutation; Prognosis; Quinolones

2019
Pathogen acquisition in patients with cystic fibrosis receiving ivacaftor or lumacaftor/ivacaftor.
    Pediatric pulmonology, 2019, Volume: 54, Issue:8

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Female; Humans; Male; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Retrospective Studies; Staphylococcal Infections; Staphylococcus aureus; Young Adult

2019
Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use.
    Pediatric pulmonology, 2019, Volume: 54, Issue:8

    Topics: Aminophenols; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Pancreatic Insufficiency; Humans; Male; Quinolones; Treatment Outcome

2019
Unexpected analytical interference in isavuconazole UV determination in a child in therapy with lumacaftor/ivacaftor for cystic fibrosis.
    Clinical chemistry and laboratory medicine, 2019, Oct-25, Volume: 57, Issue:11

    Topics: Aminophenols; Aminopyridines; Antifungal Agents; Antineoplastic Combined Chemotherapy Protocols; Benzodioxoles; Cystic Fibrosis; Female; Humans; Male; Nitriles; Pyridines; Quinolones; Triazoles

2019
Cystic fibrosis drug ivacaftor stimulates CFTR channels at picomolar concentrations.
    eLife, 2019, 06-17, Volume: 8

    Topics: Aminophenols; Animals; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Ion Channel Gating; Ion Transport; Mutation; Oocytes; Quinolones; Solubility; Solvents; Xenopus laevis

2019
Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction.
    BMC pulmonary medicine, 2019, Jun-17, Volume: 19, Issue:1

    Topics: Adult; Aminophenols; Aminopyridines; Australia; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Exercise Tolerance; Female; Genotype; Homozygote; Humans; Male; Middle Aged; Mutation; Quinolones; Respiratory Function Tests; Walk Test; Young Adult

2019
Structural identification of a hotspot on CFTR for potentiation.
    Science (New York, N.Y.), 2019, 06-21, Volume: 364, Issue:6446

    Topics: Aminophenols; Binding Sites; Chloride Channel Agonists; Cryoelectron Microscopy; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drugs, Investigational; HEK293 Cells; Humans; Hydrogen Bonding; Mutagenesis; Protein Domains; Protein Folding; Pyrans; Pyrazoles; Quinolones

2019
Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations.
    Pediatric pulmonology, 2019, Volume: 54, Issue:9

    Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Chlorides; Compassionate Use Trials; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Ion Channel Gating; Lung; Male; Middle Aged; Mutation; Quinolones; Respiratory Function Tests; Retrospective Studies; Sweat

2019
The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report.
    BMC gastroenterology, 2019, Jul-11, Volume: 19, Issue:1

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Male; Pancreatitis, Chronic; Quinolones; Recurrence; Treatment Outcome; Young Adult

2019
Negotiations between the NHS and Vertex on access to lumacaftor and ivacaftor become protracted.
    The Lancet. Respiratory medicine, 2019, Volume: 7, Issue:9

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Drug Combinations; Drug Industry; England; Humans; Negotiating; Quinolones; State Medicine

2019
Pulmonology: CFTR modulators for cystic fibrosis.
    JAAPA : official journal of the American Academy of Physician Assistants, 2013, Volume: 26, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Lung; Mutation; Oxadiazoles; Quinolones

2013
The impact of personalised therapies on respiratory medicine.
    European respiratory review : an official journal of the European Respiratory Society, 2013, Mar-01, Volume: 22, Issue:127

    Topics: Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Predisposition to Disease; Humans; Lung; Molecular Targeted Therapy; Mutation; Patient Selection; Phenotype; Precision Medicine; Pulmonary Medicine; Quinolones; Respiratory System Agents; Sweat; Treatment Outcome

2013
Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis.
    Drugs of today (Barcelona, Spain : 1998), 2013, Volume: 49, Issue:4

    Topics: Aminophenols; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Mutation; Quality of Life; Quinolones

2013
The predictive potential of the sweat chloride test in cystic fibrosis patients with the G551D mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2013, Volume: 12, Issue:6

    Topics: Administration, Oral; Adolescent; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Male; Mutation; Quinolones; Sensitivity and Specificity; Sweat; Weight Gain

2013
Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2013, Volume: 12, Issue:6

    Topics: Adult; Aminophenols; Anti-Bacterial Agents; Bronchi; Cystic Fibrosis; Female; Forced Expiratory Volume; Humans; Male; Pregnancy; Pregnancy Complications; Quinolones; Retrospective Studies; Treatment Outcome; Young Adult

2013
[News in cystic fibrosis].
    Revue de pneumologie clinique, 2013, Volume: 69, Issue:4

    Topics: Adult; Aminophenols; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; France; Humans; Infant, Newborn; Molecular Targeted Therapy; Neonatal Screening; Quinolones

2013
Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:1

    Topics: Aminophenols; Animals; Cell Line; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; In Vitro Techniques; Ion Channel Gating; Mutation, Missense; Quinolones; Rats; Rats, Inbred F344; Thyroid Gland

2014
Evaluating the predictive ability of sweat chloride.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:1

    Topics: Aminophenols; Chlorides; Cystic Fibrosis; Female; Humans; Male; Quinolones; Sweat

2014
Ivacaftor in a G551D homozygote with cystic fibrosis.
    The New England journal of medicine, 2013, Sep-26, Volume: 369, Issue:13

    Topics: Adolescent; Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Homozygote; Humans; Mutation; Quinolones; Sweat

2013
Normalization of sweat chloride concentration and clinical improvement with ivacaftor in a patient with cystic fibrosis with mutation S549N.
    Chest, 2013, Volume: 144, Issue:4

    Topics: Aminophenols; Child; Chlorides; Cystic Fibrosis; Female; Humans; Mutation; Quinolones; Sweat

2013
Response.
    Chest, 2013, Volume: 144, Issue:4

    Topics: Aminophenols; Chlorides; Cystic Fibrosis; Humans; Outcome Assessment, Health Care; Quinolones; Sweat

2013
Understanding the relationship between sweat chloride and lung function in cystic fibrosis.
    Chest, 2013, Volume: 144, Issue:4

    Topics: Aminophenols; Chlorides; Cystic Fibrosis; Humans; Outcome Assessment, Health Care; Quinolones; Sweat

2013
Pricing for orphan drugs: will the market bear what society cannot?
    JAMA, 2013, Oct-02, Volume: 310, Issue:13

    Topics: Aminophenols; Cost Sharing; Cystic Fibrosis; Drug Costs; Drug Discovery; Drug Industry; Health Expenditures; Humans; Orphan Drug Production; Quinolones; Social Justice; United States

2013
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.
    American journal of respiratory cell and molecular biology, 2014, Volume: 50, Issue:4

    Topics: Aminoglycosides; Aminophenols; Animals; Biological Transport; Cell Line; Chlorides; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Drug Synergism; Genes, Reporter; Humans; Luciferases; Mice; Mice, Inbred CFTR; Mice, Transgenic; Organ of Corti; Quinolones; Rats; Rats, Inbred F344; Time Factors; Transfection

2014
Sweat chloride is not a useful marker of clinical response to Ivacaftor.
    Thorax, 2014, Volume: 69, Issue:6

    Topics: Aminophenols; Biomarkers; Body Height; Body Weight; Chlorides; Cohort Studies; Cystic Fibrosis; Forced Expiratory Flow Rates; Humans; Lung; Prospective Studies; Quinolones; Spirometry; Sweat

2014
Basic research funding by philanthropic organizations: a case in point.
    American journal of respiratory and critical care medicine, 2013, Dec-01, Volume: 188, Issue:11

    Topics: Aminophenols; Cystic Fibrosis; Fund Raising; Humans; Molecular Targeted Therapy; Patient Participation; Private Sector; Quinolones; Research Support as Topic; Translational Research, Biomedical

2013
Ivacaftor. Uncertain harm-benefit balance.
    Prescrire international, 2013, Volume: 22, Issue:142

    Topics: Aminophenols; Body Weight; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Interactions; Forced Expiratory Volume; Humans; Quinolones; Randomized Controlled Trials as Topic; Uncertainty

2013
CFTR mutations and cystic fibrosis.
    Prescrire international, 2013, Volume: 22, Issue:142

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2013
Aquagenic wrinkling of the palms in a patient with cystic fibrosis.
    The New England journal of medicine, 2013, Dec-12, Volume: 369, Issue:24

    Topics: Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Hand; Humans; Male; Quinolones; Skin Aging; Sweat; Water

2013
New cystic fibrosis drug paves the way for orphan diseases.
    The Lancet. Respiratory medicine, 2013, Volume: 1, Issue:2

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Costs; Humans; Mutation; Precision Medicine; Quinolones; Rare Diseases; Respiratory System Agents; United Kingdom

2013
Cystic fibrosis: bridging the treatment gap in early childhood.
    The Lancet. Respiratory medicine, 2013, Volume: 1, Issue:6

    Topics: Aminophenols; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Infant; Infant, Newborn; Quinolones

2013
Mutation-specific therapy in cystic fibrosis: the earlier, the better.
    The Lancet. Respiratory medicine, 2013, Volume: 1, Issue:8

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones; Respiratory System Agents

2013
Interspecific small molecule interactions between clinical isolates of Pseudomonas aeruginosa and Staphylococcus aureus from adult cystic fibrosis patients.
    PloS one, 2014, Volume: 9, Issue:1

    Topics: Adolescent; Adult; Biofilms; Chromatography, Liquid; Cystic Fibrosis; Female; Forced Expiratory Volume; Humans; Male; Microbial Interactions; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Staphylococcal Infections; Staphylococcus aureus; Young Adult

2014
A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor.
    PloS one, 2014, Volume: 9, Issue:2

    Topics: Amino Acid Substitution; Aminophenols; Case-Control Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones; Sweat; Sweat Glands

2014
Effects of ivacaftor in patients with cystic fibrosis who carry the G551D mutation and have severe lung disease.
    Chest, 2014, Volume: 146, Issue:1

    Topics: Administration, Oral; Adult; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; DNA Mutational Analysis; Female; Follow-Up Studies; Forced Expiratory Volume; Genotype; Humans; Lung Diseases; Male; Mutation; Quinolones; Retrospective Studies; Severity of Illness Index; Young Adult

2014
Paying twice: questions over high cost of cystic fibrosis drug developed with charitable funding.
    BMJ (Clinical research ed.), 2014, Feb-12, Volume: 348

    Topics: Aminophenols; Cystic Fibrosis; Drug Discovery; Drug Industry; Foundations; Humans; Quinolones; Research Support as Topic; United Kingdom; United States

2014
Ivacaftor imaging response in cystic fibrosis.
    American journal of respiratory and critical care medicine, 2014, Feb-15, Volume: 189, Issue:4

    Topics: Adult; Aminophenols; Cystic Fibrosis; Humans; Male; Quinolones; Respiratory System Agents; Tomography, X-Ray Computed

2014
A new era in the treatment of cystic fibrosis.
    Clinical medicine (London, England), 2014, Volume: 14, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Molecular Targeted Therapy; Mutation; Oxadiazoles; Quinolones

2014
Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotype.
    Clinical pharmacology and therapeutics, 2014, Volume: 95, Issue:6

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Testing; Humans; Pharmacogenetics; Quinolones; Risk Assessment

2014
Cystic Fibrosis Trust's clarification of Cohen and Raftery's article on cystic fibrosis drug development.
    BMJ (Clinical research ed.), 2014, Mar-06, Volume: 348

    Topics: Aminophenols; Cystic Fibrosis; Drug Discovery; Drug Industry; Foundations; Humans; Quinolones

2014
A new model for drug development using a multi-stakeholder consortium.
    BMJ (Clinical research ed.), 2014, Mar-06, Volume: 348

    Topics: Aminophenols; Cystic Fibrosis; Drug Discovery; Drug Industry; Foundations; Humans; Quinolones

2014
Combined effects of VX-770 and VX-809 on several functional abnormalities of F508del-CFTR channels.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:5

    Topics: Adenosine Triphosphate; Aminophenols; Aminopyridines; Benzodioxoles; Carrier Proteins; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; In Vitro Techniques; Intracellular Signaling Peptides and Proteins; Patch-Clamp Techniques; Peptide Fragments; Quinolones

2014
Update in cystic fibrosis 2013.
    American journal of respiratory and critical care medicine, 2014, May-15, Volume: 189, Issue:10

    Topics: Aminophenols; Bronchiectasis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Evidence-Based Medicine; Humans; Life Expectancy; Mutation; Oxidative Stress; Prevalence; Quinolones; Risk Assessment; Risk Factors; Smoking

2014
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.
    British journal of pharmacology, 2014, Volume: 171, Issue:19

    Topics: Aminophenols; Animals; Biotinylation; Cell Line; Cricetinae; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genistein; Iodides; Ion Channel Gating; Mutation; Quinolones; Temperature

2014
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.
    American journal of respiratory and critical care medicine, 2014, Jul-15, Volume: 190, Issue:2

    Topics: Adolescent; Adult; Aminophenols; Biomarkers; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Follow-Up Studies; Forced Expiratory Volume; Genetic Markers; Hospitalization; Humans; Hydrogen-Ion Concentration; Intestine, Small; Lung; Male; Microbiota; Mucociliary Clearance; Mutation; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Respiratory System Agents; Sputum; Sweat; Treatment Outcome; Young Adult

2014
A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy.
    Blood, 2014, Aug-14, Volume: 124, Issue:7

    Topics: Adult; Aminophenols; Cell Degranulation; Cells, Cultured; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophoresis, Gel, Two-Dimensional; Female; Homeostasis; Humans; Immunoblotting; Magnesium; Male; Mutation; Neutrophils; Protein Transport; Proteome; Proteomics; Quinolones; rab GTP-Binding Proteins; rab27 GTP-Binding Proteins; Sodium; Tumor Necrosis Factor-alpha; Young Adult

2014
The stony road to phe508del CFTR pharmacotherapy: smoothing the first rock.
    The Lancet. Respiratory medicine, 2014, Volume: 2, Issue:7

    Topics: Aminophenols; Aminopyridines; Base Sequence; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones; Sequence Deletion

2014
Ivacaftor: from bench to bedside... and back again.
    American journal of respiratory and critical care medicine, 2014, Jul-15, Volume: 190, Issue:2

    Topics: Aminophenols; Cystic Fibrosis; Female; Humans; Intestine, Small; Lung; Male; Quinolones; Respiratory System Agents

2014
Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:1

    Topics: Administration, Oral; Adolescent; Adult; Aminophenols; Child; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Follow-Up Studies; Humans; Male; Molecular Targeted Therapy; Mutation; Prospective Studies; Quinolones; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Young Adult

2015
A cocktail drug therapy for patients with cystic fibrosis?
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2014
CT imaging of pediatric patients with cystic fibrosis on ivacaftor therapy.
    Lung, 2014, Volume: 192, Issue:5

    Topics: Adolescent; Age Factors; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Genetic Predisposition to Disease; Humans; Lung; Male; Mutation; Phenotype; Predictive Value of Tests; Quinolones; Recovery of Function; Respiratory System Agents; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Vital Capacity

2014
CFTR potentiators: not an open and shut case.
    Science translational medicine, 2014, Jul-23, Volume: 6, Issue:246

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Mutation; Quinolones

2014
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.
    Science translational medicine, 2014, Jul-23, Volume: 6, Issue:246

    Topics: Aminophenols; Bronchi; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Epithelial Cells; Humans; Models, Molecular; Mutant Proteins; Protein Stability; Quinolones

2014
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.
    Science translational medicine, 2014, Jul-23, Volume: 6, Issue:246

    Topics: Aminophenols; Bronchi; Cell Membrane; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Down-Regulation; Endocytosis; Epithelial Cells; Humans; Ion Channel Gating; Mutation; Quinolones; Suppression, Genetic; Time Factors

2014
Improvement of sinus disease in cystic fibrosis with ivacaftor therapy.
    American journal of respiratory and critical care medicine, 2014, Aug-15, Volume: 190, Issue:4

    Topics: Acetates; Adult; Aminophenols; Androstadienes; Anti-Allergic Agents; Cyclopropanes; Cystic Fibrosis; Female; Fluticasone; Humans; Leukotriene Antagonists; Paranasal Sinus Diseases; Quinolines; Quinolones; Rhinitis, Allergic, Perennial; Sulfides; Treatment Outcome; Young Adult

2014
Ivacaftor improves appearance of sinus disease on computerised tomography in cystic fibrosis patients with G551D mutation.
    Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery, 2015, Volume: 40, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Child; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Male; Mutation; Paranasal Sinus Diseases; Quinolones; Tomography, X-Ray Computed; Treatment Outcome; Young Adult

2015
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.
    Biochemistry, 2014, Sep-09, Volume: 53, Issue:35

    Topics: Amino Acid Substitution; Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Mutagenesis, Site-Directed; Mutant Proteins; Oocytes; Protein Stability; Quinolones; Recombinant Proteins; Temperature; Xenopus laevis

2014
Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:3

    Topics: Adolescent; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; Female; Forced Expiratory Volume; Genotype; Humans; Ion Transport; Mutation; Paranasal Sinus Diseases; Paranasal Sinuses; Quinolones; Tomography, X-Ray Computed

2015
Resolution of cystic fibrosis-related diabetes with ivacaftor therapy.
    American journal of respiratory and critical care medicine, 2014, Sep-01, Volume: 190, Issue:5

    Topics: Adult; Aminophenols; Cystic Fibrosis; Diabetes Mellitus; Humans; Hypoglycemic Agents; Male; Quinolones

2014
Improvement in bronchiectasis on CT imaging in a pediatric patient with cystic fibrosis on ivacaftor therapy.
    Respiration; international review of thoracic diseases, 2014, Volume: 88, Issue:4

    Topics: Aminophenols; Bronchiectasis; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Female; Humans; Membrane Transport Modulators; Quinolones; Spirometry; Tomography, X-Ray Computed; Treatment Outcome

2014
The effect of ivacaftor in adolescents with cystic fibrosis (G551D mutation): an exercise physiology perspective.
    Pediatric physical therapy : the official publication of the Section on Pediatrics of the American Physical Therapy Association, 2014,Winter, Volume: 26, Issue:4

    Topics: Adolescent; Aminophenols; Body Weights and Measures; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exercise Test; Female; Humans; Male; Quinolones; Respiratory Function Tests

2014
50 years ago in The Journal of Pediatrics: The use of N-acetylcysteine in the treatment of cystic fibrosis.
    The Journal of pediatrics, 2014, Volume: 165, Issue:4

    Topics: Acetylcysteine; Administration, Oral; Aminophenols; Cell Membrane; Cystic Fibrosis; Deoxyribonuclease I; DNA; Female; History, 20th Century; Humans; Lung; Mutation; Pediatrics; Quality of Life; Quinolones; Recombinant Proteins

2014
False dawn for cystic fibrosis disease modifiers?
    Nature reviews. Drug discovery, 2014, Volume: 13, Issue:10

    Topics: Aminophenols; Cystic Fibrosis; Drug Approval; Drug Design; Humans; Quinolones; United States

2014
Mutation-targeted personalised medicine for cystic fibrosis.
    The Lancet. Respiratory medicine, 2014, Volume: 2, Issue:11

    Topics: Aminophenols; Cough; Cystic Fibrosis; Female; Humans; Male; Quinolones; Respiratory Tract Infections

2014
Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor.
    International forum of allergy & rhinology, 2015, Volume: 5, Issue:2

    Topics: Aminophenols; Anti-Inflammatory Agents; Cells, Cultured; Child; Chronic Disease; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Hydrogen-Ion Concentration; Ion Transport; Mutation; Quinolones; Respiratory Mucosa; Sinusitis; Treatment Outcome; Viscosity

2015
Discovery heralds new approach to the treatment of cystic fibrosis.
    Journal of medicinal chemistry, 2014, Dec-11, Volume: 57, Issue:23

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Male; Quinolones

2014
Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor.
    Clinical infectious diseases : an official publication of the Infectious Diseases Society of America, 2015, Mar-01, Volume: 60, Issue:5

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Amino Acid Substitution; Aminophenols; Aspergillus; Child; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Enzyme Activators; Female; Humans; Longitudinal Studies; Male; Middle Aged; Mutant Proteins; Mutation, Missense; Prevalence; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Staphylococcus aureus; Treatment Outcome; United States; Young Adult

2015
Personalized Drug Therapy in Cystic Fibrosis: From Fiction to Reality.
    Current drug targets, 2015, Volume: 16, Issue:9

    Topics: Aminophenols; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Precision Medicine; Quinolones

2015
Discovery of N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide (VX-770, ivacaftor), a potent and orally bioavailable CFTR potentiator.
    Journal of medicinal chemistry, 2014, Dec-11, Volume: 57, Issue:23

    Topics: Aminophenols; Animals; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dogs; Humans; Macaca fascicularis; Male; Mice; NIH 3T3 Cells; Quinolones; Rats, Sprague-Dawley; Structure-Activity Relationship

2014
Physiological mechanisms of dyspnea relief following ivacaftor in cystic fibrosis: a case report.
    Respiratory physiology & neurobiology, 2015, Jan-01, Volume: 205

    Topics: Adult; Aminophenols; Cystic Fibrosis; Dyspnea; Exercise Tolerance; Humans; Male; Quinolones; Respiratory Function Tests; Respiratory Physiological Phenomena

2015
Chronic ivacaftor treatment: getting F508del-CFTR into more trouble?
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:6

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2014
Remarkable progress toward new treatments for cystic fibrosis.
    The Lancet. Respiratory medicine, 2014, Volume: 2, Issue:12

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Forced Expiratory Volume; Humans; Mutation; Quinolones

2014
A bioassay using intestinal organoids to measure CFTR modulators in human plasma.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:2

    Topics: Aminophenols; Antimutagenic Agents; Biological Assay; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Humans; Intestinal Mucosa; Intestines; Mutation; Organoids; Quinolones; Treatment Outcome

2015
PharmGKB summary: very important pharmacogene information for CFTR.
    Pharmacogenetics and genomics, 2015, Volume: 25, Issue:3

    Topics: Aminophenols; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Molecular Targeted Therapy; Polymorphism, Single Nucleotide; Quinolones

2015
Foundation receives $3.3-billion windfall for Kalydeco.
    Nature biotechnology, 2015, Volume: 33, Issue:1

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Financing, Organized; Foundations; Humans; Quinolones

2015
Ivacaftor therapy in siblings with cystic fibrosis-the potential implications of Itraconazole in dosage and efficacy.
    Pulmonary pharmacology & therapeutics, 2015, Volume: 31

    Topics: Adult; Aminophenols; Antifungal Agents; Cystic Fibrosis; Cytochrome P-450 CYP3A Inhibitors; Dose-Response Relationship, Drug; Drug Antagonism; Enzyme Inhibitors; Humans; Itraconazole; Male; Mutation; Quinolones; Siblings

2015
Improvement of hepatic steatosis in cystic fibrosis with ivacaftor therapy.
    Journal of pediatric gastroenterology and nutrition, 2015, Volume: 60, Issue:5

    Topics: Adolescent; Aminophenols; Cystic Fibrosis; Fatty Liver; Female; Humans; Quinolones; Radiography

2015
Ivacaftor as salvage therapy in a patient with cystic fibrosis genotype F508del/R117H/IVS8-5T.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:4

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; Male; Middle Aged; Quinolones; Salvage Therapy

2015
Improved clinical and radiographic outcomes after treatment with ivacaftor in a young adult with cystic fibrosis with the P67L CFTR mutation.
    Chest, 2015, Volume: 147, Issue:3

    Topics: Adolescent; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Lung; Mutation; Quinolones; Radiography, Thoracic; Spirometry; Tomography, X-Ray Computed; Treatment Outcome

2015
Alterations in blood leukocytes of G551D-bearing cystic fibrosis patients undergoing treatment with ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016, Volume: 15, Issue:1

    Topics: Adult; Aminophenols; CD11b Antigen; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Monocytes; Mutation; Neutrophils; Quinolones; Statistics as Topic; Tetraspanin 30

2016
Settlement reached over medicaid coverage of cystic fibrosis drug: Arkansas federal court case highlights medical necessity, high cost of targeted therapies.
    American journal of medical genetics. Part A, 2015, Volume: 167A, Issue:4

    Topics: Aminophenols; Arkansas; Cystic Fibrosis; Health Services Accessibility; Humans; Medicaid; Patient Rights; Poverty; Quinolones; United States

2015
Impact of the CFTR-potentiator ivacaftor on airway microbiota in cystic fibrosis patients carrying a G551D mutation.
    PloS one, 2015, Volume: 10, Issue:4

    Topics: Adolescent; Amino Acid Substitution; Aminophenols; Anaerobiosis; Bacterial Typing Techniques; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Longitudinal Studies; Lung; Microbiota; Molecular Targeted Therapy; Mutation; Porphyromonas; Prevotella; Quinolones; Respiratory Function Tests; RNA, Ribosomal, 16S; Streptococcus; Treatment Outcome

2015
Iron Depletion Enhances Production of Antimicrobials by Pseudomonas aeruginosa.
    Journal of bacteriology, 2015, Volume: 197, Issue:14

    Topics: Anti-Infective Agents; Coculture Techniques; Cystic Fibrosis; Gene Expression Regulation, Bacterial; Humans; Iron; Mutation; Pseudomonas aeruginosa; Quinolones; Staphylococcus aureus

2015
Another Beginning for Cystic Fibrosis Therapy.
    The New England journal of medicine, 2015, Jul-16, Volume: 373, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones

2015
Drug combination that corrects deficient protein in cystic fibrosis improves lung function.
    BMJ (Clinical research ed.), 2015, May-19, Volume: 350

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Peptide Fragments; Quinolones; Randomized Controlled Trials as Topic; Respiratory Function Tests

2015
Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: should we curb our enthusiasm?
    Thorax, 2015, Volume: 70, Issue:7

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Gene Deletion; Homozygote; Humans; Molecular Targeted Therapy; Quinolones

2015
Rate of Uptake of Ivacaftor Use after U.S. Food and Drug Administration Approval among Patients Enrolled in the U.S. Cystic Fibrosis Foundation Patient Registry.
    Annals of the American Thoracic Society, 2015, Volume: 12, Issue:8

    Topics: Adolescent; Adult; Aminophenols; Child; Cross-Sectional Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Logistic Models; Male; Multivariate Analysis; Mutation; Quinolones; Registries; United States; United States Food and Drug Administration; Young Adult

2015
Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:5

    Topics: Adolescent; Adult; Aminophenols; Child; Clinical Pharmacy Information Systems; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Drug Prescriptions; Electronic Data Processing; Female; Humans; Male; Medication Adherence; Middle Aged; Prospective Studies; Quinolones; Young Adult

2015
Radical new treatments for cystic fibrosis.
    Archives of disease in childhood, 2015, Volume: 100, Issue:8

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Quinolones; Randomized Controlled Trials as Topic

2015
A new chapter in therapy for cystic fibrosis.
    The Lancet. Respiratory medicine, 2015, Volume: 3, Issue:7

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones

2015
Sustained Benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data.
    American journal of respiratory and critical care medicine, 2015, Oct-01, Volume: 192, Issue:7

    Topics: Aminophenols; Body Mass Index; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Lung; Nutritional Status; Propensity Score; Quinolones; Registries; Respiratory Function Tests

2015
Effect of ivacaftor therapy on exhaled nitric oxide in patients with cystic fibrosis.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:6

    Topics: Adult; Aminophenols; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exhalation; Female; Humans; Male; Mutation; Nitric Oxide; Quinolones; Young Adult

2015
Cystic fibrosis, ivacaftor, and the Arg117His-CFTR mutation.
    The Lancet. Respiratory medicine, 2015, Volume: 3, Issue:7

    Topics: Aminophenols; Cystic Fibrosis; Female; Humans; Male; Quinolones; Respiratory System Agents

2015
Cystic fibrosis transmembrane conductance regulator biomarkers in 'real life': can we evaluate individual efficacy of cystic fibrosis transmembrane conductance regulator therapy?
    Therapeutic advances in respiratory disease, 2015, Volume: 9, Issue:4

    Topics: Adolescent; Adult; Aminophenols; Biomarkers; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Mutation; Quinolones; Treatment Outcome; Young Adult

2015
[Cystic Fibrosis: toward a genetic treatment; Clostridium difficile versus Clostridium difficile].
    Revue medicale suisse, 2015, May-27, Volume: 11, Issue:476

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers; Clostridioides difficile; Clostridium Infections; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Mutation; Quinolones; Spores, Bacterial; Treatment Outcome

2015
Precision Medicine: At What Price?
    American journal of respiratory and critical care medicine, 2015, Sep-15, Volume: 192, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cost-Benefit Analysis; Cystic Fibrosis; Drug Combinations; Humans; Precision Medicine; Quinolones; Respiratory System Agents; United States

2015
Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:6

    Topics: Adolescent; Aminophenols; Biomarkers; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Nitric Oxide; Quinolones; Young Adult

2015
Adherence to Ivacaftor is suboptimal.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2015, Volume: 14, Issue:5

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Humans; Medication Adherence; Quinolones; Treatment Outcome

2015
The Role of Ivacaftor in Severe Cystic Fibrosis in a Patient With the R117H Mutation.
    Chest, 2015, Volume: 148, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Middle Aged; Mutation; Quinolones; Tomography, X-Ray Computed

2015
Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening.
    Journal of biomolecular screening, 2015, Volume: 20, Issue:10

    Topics: Aminophenols; Base Sequence; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Discovery; HEK293 Cells; High-Throughput Screening Assays; Humans; Models, Molecular; Molecular Sequence Data; Mutagenesis, Site-Directed; Polymerase Chain Reaction; Quinolones; Sequence Alignment

2015
Orkambi's Slick Unveiling Puts Insurers in a Bind.
    Managed care (Langhorne, Pa.), 2015, Volume: 24, Issue:8

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Drug Costs; Drug Industry; Humans; Insurance Coverage; Insurance, Pharmaceutical Services; Quinolones; United States

2015
A combination therapy for cystic fibrosis.
    Cell, 2015, Sep-24, Volume: 163, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Gene Deletion; History, 20th Century; History, 21st Century; Humans; Quinolones

2015
The evidence for long-term benefits of restoration of CFTR function continues to grow.
    American journal of respiratory and critical care medicine, 2015, Oct-01, Volume: 192, Issue:7

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2015
Ivacaftor in a young boy with the rare gating mutation S549R--use of lung clearance index to track progress: a case report.
    BMC pulmonary medicine, 2015, Oct-16, Volume: 15

    Topics: Aminophenols; Breath Tests; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Humans; Male; Mutation; Nitrogen; Quinolones; Treatment Outcome

2015
Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor).
    British journal of pharmacology, 2016, Volume: 173, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Bronchi; Cell Line; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Interactions; Epithelial Cells; Humans; Mutation; Quinolones

2016
Response to: 'Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: should we curb our enthusiasm?' by Jones and Barry.
    Thorax, 2016, Volume: 71, Issue:2

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gene Deletion; Humans; Quinolones

2016
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
    The New England journal of medicine, 2015, 10-29, Volume: 373, Issue:18

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones

2015
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
    The New England journal of medicine, 2015, 10-29, Volume: 373, Issue:18

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones

2015
Breathing easier with combinations.
    Nature biotechnology, 2015, Volume: 33, Issue:11

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomedical Research; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Industry; Drug Therapy, Combination; Humans; Mutation; Quinolones

2015
CFTR potentiator therapy ameliorates impaired insulin secretion in CF patients with a gating mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016, Volume: 15, Issue:3

    Topics: Aminophenols; Blood Glucose; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Insulin; Insulin Secretion; Male; Mutation; Quinolones; Siblings; Treatment Outcome; Young Adult

2016
Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor.
    American journal of respiratory and critical care medicine, 2015, Nov-15, Volume: 192, Issue:10

    Topics: Adolescent; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Mutation; Precision Medicine; Quinolones

2015
Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.
    Expert review of respiratory medicine, 2016, Volume: 10, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones; Randomized Controlled Trials as Topic

2016
Microbial, host and xenobiotic diversity in the cystic fibrosis sputum metabolome.
    The ISME journal, 2016, Volume: 10, Issue:6

    Topics: Adolescent; Ceramides; Cystic Fibrosis; Humans; Lung; Metabolome; Microbiota; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; RNA, Ribosomal, 16S; Sputum; Xenobiotics

2016
Game Changers.
    The Ulster medical journal, 2015, Volume: 84, Issue:3

    Topics: Acute Disease; Aminophenols; Aminopyridines; Benzodioxoles; Brain Ischemia; Cystic Fibrosis; Drug Combinations; Endovascular Procedures; Humans; Quinolones; Rectal Neoplasms; Stroke; Transanal Endoscopic Microsurgery

2015
A successful uncomplicated CF pregnancy while remaining on Ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016, Volume: 15, Issue:1

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Female; Humans; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Quinolones; Respiratory Function Tests

2016
Lumacaftor-ivacaftor (Orkambi) for cystic fibrosis: behind the 'breakthrough'.
    Evidence-based medicine, 2016, Volume: 21, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Body Mass Index; Cystic Fibrosis; Data Interpretation, Statistical; Disease Progression; Drug Approval; Drug Combinations; Drug Costs; Evidence-Based Medicine; Humans; Lung; Quinolones; Surveys and Questionnaires; Treatment Outcome; United States; United States Food and Drug Administration

2016
[FUTURE THERAPIES FOR CYSTIC FIBROSIS].
    La Revue du praticien, 2015, Volume: 65, Issue:8

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones; Therapies, Investigational

2015
Resolution of Intestinal Histopathology Changes in Cystic Fibrosis after Treatment with Ivacaftor.
    Annals of the American Thoracic Society, 2016, Volume: 13, Issue:2

    Topics: Aminophenols; Child; Cystic Fibrosis; Duodenum; Humans; Mucus; Quinolones; Treatment Outcome

2016
Lung clearance index response in patients with CF with class III CFTR mutations.
    Thorax, 2016, Volume: 71, Issue:5

    Topics: Adult; Alleles; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Humans; Maximal Midexpiratory Flow Rate; Mutation; Quinolones; Saline Solution, Hypertonic; Severity of Illness Index; Spirometry; Treatment Outcome

2016
[A milestone in cystic fibrosis therapy].
    MMW Fortschritte der Medizin, 2015, Nov-05, Volume: 157, Issue:19

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones

2015
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.
    American journal of physiology. Lung cellular and molecular physiology, 2016, 05-15, Volume: 310, Issue:10

    Topics: Amiloride; Aminophenols; Animals; Cells, Cultured; Chloride Channel Agonists; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Evaluation, Preclinical; Drug Therapy, Combination; Humans; Membrane Potentials; Mice; Mutation, Missense; NIH 3T3 Cells; Quinolones

2016
[Combined administration of lumacaftor and ivacaftor as a causal therapeutic approach].
    Medizinische Monatsschrift fur Pharmazeuten, 2016, Volume: 39, Issue:1

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Drug Combinations; Humans; Quinolones; Randomized Controlled Trials as Topic

2016
Lumacaftor/ivacaftor (Orkambi) for cystic fibrosis.
    The Medical letter on drugs and therapeutics, 2016, Mar-28, Volume: 58, Issue:1491

    Topics: Administration, Oral; Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Drug Costs; Drug Interactions; Humans; Membrane Transport Modulators; Mutation; Quinolones; Risk Factors; Treatment Outcome

2016
Ivacaftor-Induced Proteomic Changes Suggest Monocyte Defects May Contribute to the Pathogenesis of Cystic Fibrosis.
    American journal of respiratory cell and molecular biology, 2016, Volume: 54, Issue:4

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Monocytes; Proteome; Quinolones

2016
Healthcare resource utilization associated with ivacaftor use in patients with cystic fibrosis.
    Journal of medical economics, 2016, Volume: 19, Issue:9

    Topics: Adolescent; Adult; Age Factors; Aminophenols; Child; Chloride Channel Agonists; Costs and Cost Analysis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Health Expenditures; Health Services; Humans; Insurance, Health; Male; Middle Aged; Mutation; Quinolones; Retrospective Studies; Sex Factors; Young Adult

2016
Forecasting US ivacaftor outcomes and cost in cystic fibrosis patients with the G551D mutation.
    The European respiratory journal, 2016, Volume: 47, Issue:6

    Topics: Aminophenols; Cohort Studies; Computer Simulation; Cost-Benefit Analysis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy; Forced Expiratory Volume; Forecasting; Health Status; Humans; Lung Transplantation; Markov Chains; Monte Carlo Method; Mutation; Quality-Adjusted Life Years; Quinolones; Respiratory Function Tests; Treatment Outcome; United States

2016
Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016, Volume: 15, Issue:5

    Topics: Aminophenols; Chloride Channel Agonists; Curcumin; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Drug Therapy, Combination; Enzyme Inhibitors; Genistein; Humans; Models, Theoretical; Molecular Targeted Therapy; Mutation; Organoids; Quinolones; Rectum

2016
Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.
    PloS one, 2016, Volume: 11, Issue:5

    Topics: Aminophenols; Aminopyridines; Benzamides; Benzodioxoles; Cell Line; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endoplasmic Reticulum; Gene Expression Regulation; Half-Life; HEK293 Cells; Humans; Mutation; Quinolones; Thiazoles

2016
Ivacaftor in cystic fibrosis adults: Czech experience with six years of follow-up.
    Biomedical papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia, 2016, Volume: 160, Issue:2

    Topics: Adult; Aftercare; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Female; Forced Expiratory Volume; Humans; Male; Quinolones; Treatment Outcome; Young Adult

2016
Long-term computed tomographic changes in cystic fibrosis patients treated with ivacaftor.
    The European respiratory journal, 2016, Volume: 48, Issue:1

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Female; France; Humans; Lung; Male; Middle Aged; Quinolones; Retrospective Studies; Time; Tomography, X-Ray Computed

2016
Attitudes Toward Discussing Approved and Investigational Treatments for Cystic Fibrosis in Prenatal Genetic Counseling Practice.
    Journal of genetic counseling, 2017, Volume: 26, Issue:1

    Topics: Aminophenols; Attitude to Health; Chloride Channel Agonists; Counselors; Cystic Fibrosis; Decision Making; Female; Genetic Counseling; Genetic Testing; Humans; Mutation; Parents; Precision Medicine; Pregnancy; Prenatal Diagnosis; Quality of Life; Quinolones; Surveys and Questionnaires; Therapies, Investigational

2017
Potentiators exert distinct effects on human, murine, and Xenopus CFTR.
    American journal of physiology. Lung cellular and molecular physiology, 2016, 08-01, Volume: 311, Issue:2

    Topics: Amino Acid Sequence; Aminophenols; Animals; Cells, Cultured; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Evaluation, Preclinical; Glycine; Hydrazines; Membrane Potentials; Mice; Nitrobenzoates; Patch-Clamp Techniques; Quinolones; Sequence Deletion; Xenopus laevis

2016
Promising gene therapies pose million-dollar conundrum.
    Nature, 2016, 06-16, Volume: 534, Issue:7607

    Topics: Aminophenols; Anemia, Sickle Cell; Antibodies, Monoclonal; Antibodies, Monoclonal, Humanized; Biotechnology; Cystic Fibrosis; Drug Costs; Drug Industry; Genetic Therapy; Humans; Insurance, Health; Neoplasms; Quinolones; Sofosbuvir

2016
[Causal therapy is available].
    MMW Fortschritte der Medizin, 2016, Jun-23, Volume: 158, Issue:12

    Topics: Adolescent; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Drug Combinations; Humans; Quinolones

2016
Cystic fibrosis drug is not cost effective, says NICE.
    BMJ (Clinical research ed.), 2016, Jun-19, Volume: 353

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cost-Benefit Analysis; Cystic Fibrosis; Humans; Quinolones; Respiratory System Agents

2016
Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis.
    Science translational medicine, 2016, 06-22, Volume: 8, Issue:344

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biological Assay; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; In Vitro Techniques; Mutation; Organoids; Quinolones

2016
Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770.
    American journal of physiology. Lung cellular and molecular physiology, 2016, 09-01, Volume: 311, Issue:3

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cell Line; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Evaluation, Preclinical; Humans; Mutation, Missense; Protein Folding; Quinolones; Sequence Deletion

2016
The effects of ivacaftor on CF fatty acid metabolism: An analysis from the GOAL study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1

    Topics: Adult; Aminophenols; Arachidonic Acid; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Fatty Acids, Monounsaturated; Female; Humans; Inflammation Mediators; Linoleic Acid; Lipid Metabolism; Male; Mutation; Pilot Projects; Prostaglandins E; Quinolones

2017
Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Monitoring; Female; Gastroesophageal Reflux; Humans; Lung; Male; Mutation; Quinolones; Respiratory Aspiration; Respiratory Function Tests; Treatment Outcome; United Kingdom

2017
Potential for Therapeutic Benefit among Cystic Fibrosis Populations Excluded from Clinical Trials or Labeling of Marketed Therapies.
    Annals of the American Thoracic Society, 2016, Volume: 13, Issue:11

    Topics: Aminophenols; Clinical Trials as Topic; Cystic Fibrosis; Health Care Costs; Humans; Off-Label Use; Patient Selection; Quinolones; Risk Assessment

2016
An "Unlikely" Pair: The Antimicrobial Synergy of Polymyxin B in Combination with the Cystic Fibrosis Transmembrane Conductance Regulator Drugs KALYDECO and ORKAMBI.
    ACS infectious diseases, 2016, 07-08, Volume: 2, Issue:7

    Topics: Aminophenols; Aminopyridines; Anti-Bacterial Agents; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Drug Therapy, Combination; Humans; Polymyxin B; Pseudomonas aeruginosa; Quinolones

2016
PharmGKB summary: ivacaftor pathway, pharmacokinetics/pharmacodynamics.
    Pharmacogenetics and genomics, 2017, Volume: 27, Issue:1

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Quinolones

2017
Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1

    Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Membrane Potentials; Middle Aged; Mutation; Nasal Mucosa; Outcome Assessment, Health Care; Quinolones; Sweat; Treatment Outcome

2017
Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1

    Topics: Aminophenols; Chloride Channel Agonists; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Homozygote; Humans; Ion Transport; Quinolones; Treatment Outcome

2017
Cystic Fibrosis: The Dawn of a New Therapeutic Era.
    American journal of respiratory and critical care medicine, 2017, 04-15, Volume: 195, Issue:8

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Early Diagnosis; Genetic Therapy; Humans; Quinolones; Survival Rate

2017
Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016, Volume: 15, Issue:6

    Topics: Adult; Aminophenols; Bone Demineralization, Pathologic; Bone Density; Cells, Cultured; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Middle Aged; Mutation; Osteoblasts; Quinolones; Statistics as Topic

2016
Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: A longitudinal study.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:2

    Topics: Adult; Biomarkers; Child; Cross-Sectional Studies; Cystic Fibrosis; Early Diagnosis; Female; Humans; Male; Predictive Value of Tests; Prognosis; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Reproducibility of Results; Respiratory Tract Infections; United Kingdom

2017
Correlation of sweat chloride and percent predicted FEV
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1

    Topics: Aminophenols; Biomarkers; Chloride Channel Agonists; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Humans; Mutation; Predictive Value of Tests; Quinolones; Sweat; Treatment Outcome

2017
Development of HPLC and LC-MS/MS methods for the analysis of ivacaftor, its major metabolites and lumacaftor in plasma and sputum of cystic fibrosis patients treated with ORKAMBI or KALYDECO.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2016, Dec-01, Volume: 1038

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Chromatography, High Pressure Liquid; Cystic Fibrosis; Humans; Limit of Detection; Quinolones; Sputum; Tandem Mass Spectrometry

2016
Therapeutic benefit of ivacaftor in late cystic fibrosis caused by homozygous IVS8-5T CFTR polymorphism.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1

    Topics: Aged; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Male; Polymorphism, Genetic; Quinolones

2017
Cystic fibrosis in 2016: considerable progress, but much more to do.
    The Lancet. Respiratory medicine, 2016, Volume: 4, Issue:12

    Topics: Aminophenols; Aspergillosis, Allergic Bronchopulmonary; Chloride Channel Agonists; Comorbidity; Cystic Fibrosis; Humans; Pseudomonas Infections; Quinolones

2016
MRI accelerating progress in functional assessment of cystic fibrosis lung disease.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:2

    Topics: Aminophenols; Cystic Fibrosis; Helium; Humans; Isotopes; Magnetic Resonance Imaging; Quinolones

2017
A little CFTR can change a lot: slowing cystic fibrosis progression.
    The Lancet. Respiratory medicine, 2017, Volume: 5, Issue:2

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Quinolones

2017
Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770).
    Hepatology (Baltimore, Md.), 2017, Volume: 65, Issue:2

    Topics: Adenosine Triphosphate; Adolescent; Aminophenols; ATP Binding Cassette Transporter, Subfamily B; Binding Sites; Cells, Cultured; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Hep G2 Cells; Humans; Male; Mutagenesis; Mutation, Missense; Phosphatidylcholines; Quinolones; Sampling Studies; Transfection; Young Adult

2017
Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.
    The Journal of biological chemistry, 2017, 03-03, Volume: 292, Issue:9

    Topics: Aminophenols; Aminopyridines; Animals; Benzodioxoles; Cell Line; Cell Membrane; Cell-Free System; Chromatography; Cricetinae; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Hot Temperature; Humans; Mutation; Patch-Clamp Techniques; Protein Denaturation; Quinolones

2017
Cystic fibrosis gene modifier SLC26A9 modulates airway response to CFTR-directed therapeutics.
    Human molecular genetics, 2016, 10-15, Volume: 25, Issue:20

    Topics: Aminophenols; Antiporters; Canada; Cells, Cultured; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; France; Genes, Modifier; Genetic Association Studies; Humans; Lung; Male; Models, Genetic; Patient Acuity; Pharmacogenomic Variants; Polymorphism, Single Nucleotide; Precision Medicine; Quinolones; Sulfate Transporters

2016
Pregnancy among cystic fibrosis women in the era of CFTR modulators.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:6

    Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Pregnancy Rate; Pregnancy, High-Risk; Quinolones; Risk Adjustment; United States

2017
Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections.
    American journal of respiratory and critical care medicine, 2017, 06-15, Volume: 195, Issue:12

    Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Inflammation; Lung; Male; Quinolones; Respiratory Tract Infections; Sputum; Tomography, X-Ray Computed

2017
Within-host whole genome analysis of an antibiotic resistant Pseudomonas aeruginosa strain sub-type in cystic fibrosis.
    PloS one, 2017, Volume: 12, Issue:3

    Topics: Adult; Anti-Bacterial Agents; Chromosome Mapping; Cystic Fibrosis; Drug Resistance, Microbial; Frameshift Mutation; Genome, Bacterial; High-Throughput Nucleotide Sequencing; Humans; Male; Microbial Sensitivity Tests; Phylogeny; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Sequence Analysis, DNA

2017
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.
    Proceedings of the National Academy of Sciences of the United States of America, 2009, Nov-03, Volume: 106, Issue:44

    Topics: Absorption; Amino Acid Substitution; Aminophenols; Animals; Bronchi; Cells, Cultured; Chlorides; Cilia; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Epithelial Cells; Epithelial Sodium Channels; Humans; Ion Channel Gating; Mice; Mutation; NIH 3T3 Cells; Quinolines; Quinolones; Sodium

2009
Targeting the basic defect in cystic fibrosis.
    The New England journal of medicine, 2010, Nov-18, Volume: 363, Issue:21

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant, Newborn; Mutation; Neonatal Screening; Quinolones

2010
Molecular characterization and phylogenetic analysis of quinolone resistance-determining regions (QRDRs) of gyrA, gyrB, parC and parE gene loci in viridans group streptococci isolated from adult patients with cystic fibrosis.
    The Journal of antimicrobial chemotherapy, 2011, Volume: 66, Issue:3

    Topics: Adult; Anti-Bacterial Agents; Ciprofloxacin; Cystic Fibrosis; DNA Gyrase; DNA Topoisomerase IV; DNA, Bacterial; Drug Resistance, Bacterial; Drug Utilization; Female; Humans; Male; Microbial Sensitivity Tests; Middle Aged; Quinolones; Sequence Analysis, DNA; Streptococcal Infections; Viridans Streptococci

2011
Chronic desensitization to quinolones in fixed drug eruption.
    Journal of investigational allergology & clinical immunology, 2011, Volume: 21, Issue:1

    Topics: Adult; Ciprofloxacin; Cystic Fibrosis; Desensitization, Immunologic; Drug Eruptions; Erythema; Female; Humans; Pipemidic Acid; Quinolones

2011
Mutation-specific cystic fibrosis treatments on verge of approval.
    Nature medicine, 2011, Volume: 17, Issue:4

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Discovery; Humans; Models, Molecular; Mutant Proteins; Mutation; Quinolones

2011
Orphan cystic fibrosis drugs find sister diseases.
    Nature medicine, 2011, Volume: 17, Issue:4

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Discovery; Humans; Mutant Proteins; Orphan Drug Production; Quinolones

2011
Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.
    The Journal of biological chemistry, 2011, Jul-15, Volume: 286, Issue:28

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endoplasmic Reticulum; HEK293 Cells; Humans; Mutation; Piperazines; Protein Binding; Protein Folding; Protein Structure, Tertiary; Quinazolines; Quinolones

2011
First cystic fibrosis drug advances towards approval.
    Nature biotechnology, 2011, Jun-07, Volume: 29, Issue:6

    Topics: Aminophenols; Anti-Bacterial Agents; Anti-Inflammatory Agents; Clinical Trials, Phase III as Topic; Cloning, Molecular; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Industry; Humans; Pharmacogenetics; Quinolones

2011
Excitement mounts for first disease-modifying cystic fibrosis drugs.
    Nature reviews. Drug discovery, 2011, Jul-01, Volume: 10, Issue:7

    Topics: Aminophenols; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Discovery; Humans; Mutation; Quinolones

2011
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.
    American journal of physiology. Lung cellular and molecular physiology, 2011, Volume: 301, Issue:4

    Topics: Aminophenols; Animals; Blotting, Western; Cell Line; Chlorocebus aethiops; Colforsin; Cresols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diffusion Chambers, Culture; Dimerization; Enzyme-Linked Immunosorbent Assay; Gene Expression; High-Throughput Screening Assays; Humans; Ion Channel Gating; Ion Transport; Lentivirus; Membrane Transport Modulators; Mice; Mutation; Phosphorylation; Protein Structure, Tertiary; Pyrazoles; Quinolones; Retroviridae; Transduction, Genetic

2011
Therapy for cystic fibrosis--the end of the beginning?
    The New England journal of medicine, 2011, Nov-03, Volume: 365, Issue:18

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Quinolones

2011
[Investigation of plasmid-mediated quinolone resistance in Pseudomonas aeruginosa strains isolated from cystic fibrosis patients].
    Mikrobiyoloji bulteni, 2011, Volume: 45, Issue:4

    Topics: Anti-Infective Agents; Ciprofloxacin; Cystic Fibrosis; Drug Resistance, Bacterial; Humans; Microbial Sensitivity Tests; Multiplex Polymerase Chain Reaction; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; R Factors; Respiratory System

2011
Transformative mutation specific pharmacotherapy for cystic fibrosis.
    BMJ (Clinical research ed.), 2012, Jan-09, Volume: 344

    Topics: Aminophenols; Cystic Fibrosis; Forced Expiratory Volume; Humans; Mutation; Quinolones

2012
Personalized medicine comes to cystic fibrosis.
    American journal of medical genetics. Part A, 2012, Volume: 158A, Issue:2

    Topics: Aminophenols; Clinical Trials, Phase III as Topic; Cystic Fibrosis; Humans; Molecular Targeted Therapy; Mutation; Oxadiazoles; Precision Medicine; Quinolones

2012
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2012, Volume: 11, Issue:3

    Topics: Aminophenols; Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; DNA; DNA Mutational Analysis; Ion Channel Gating; Ion Transport; Mutation; Prognosis; Quinolones; Rats; Rats, Inbred F344; Thyroid Gland

2012
Personalized medicine. New cystic fibrosis drug offers hope, at a price.
    Science (New York, N.Y.), 2012, Feb-10, Volume: 335, Issue:6069

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Costs; Humans; Molecular Targeted Therapy; Mutation; Precision Medicine; Quinolones; Small Molecule Libraries; United States; United States Food and Drug Administration

2012
Straight talk with... Robert Beall. Interviewed by Elie Dolgin.
    Nature medicine, 2012, Mar-06, Volume: 18, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Drug Approval; Fund Raising; Humans; Quinolones

2012
Cystic fibrosis drug Vertex's latest triumph.
    Nature biotechnology, 2012, Mar-07, Volume: 30, Issue:3

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Drug Industry; Humans; Mutation; Quinolones; Respiratory System Agents

2012
Hot off the breath: 'I've a cost for'--the 64 million dollar question.
    Thorax, 2012, Volume: 67, Issue:5

    Topics: Aged; Aged, 80 and over; Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Middle Aged; Quinolones

2012
Ivacaftor (Kalydeco) for cystic fibrosis.
    The Medical letter on drugs and therapeutics, 2012, Apr-16, Volume: 54, Issue:1388

    Topics: Aminophenols; Cystic Fibrosis; Humans; Quinolones

2012
Promising new era dawns for cystic fibrosis treatment.
    Lancet (London, England), 2012, Apr-21, Volume: 379, Issue:9825

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Mutation; Quinolones

2012
Ivacaftor.
    Nature reviews. Drug discovery, 2012, Apr-30, Volume: 11, Issue:5

    Topics: Aminophenols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Approval; Humans; Mutation; Quinolones; United States; United States Food and Drug Administration

2012
Targeting a cystic fibrosis mutation opens door for personalized treatment.
    Managed care (Langhorne, Pa.), 2012, Volume: 21, Issue:4

    Topics: Aminophenols; Cystic Fibrosis; Humans; Polymorphism, Genetic; Precision Medicine; Quinolones; United States

2012
Highlights of the North American Cystic Fibrosis Conference 2011.
    Journal of the Royal Society of Medicine, 2012, Volume: 105 Suppl 2

    Topics: Adolescent; Adult; Aminophenols; Animals; Anti-Bacterial Agents; Child; Child, Preschool; Congresses as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Drugs, Investigational; Female; Genetic Therapy; Humans; Infant; Infant, Newborn; Male; Mice; Mice, Transgenic; Molecular Targeted Therapy; Mutation; Neonatal Screening; Oxadiazoles; Practice Guidelines as Topic; Prognosis; Quinolones; Respiratory Function Tests; Treatment Outcome; Young Adult

2012
A young Hispanic with c.1646G>A mutation exhibits severe cystic fibrosis lung disease: is ivacaftor an option for therapy?
    American journal of respiratory and critical care medicine, 2012, Oct-01, Volume: 186, Issue:7

    Topics: Aminophenols; Child, Preschool; Cystic Fibrosis; Female; Hispanic or Latino; Humans; Quinolones

2012
Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience.
    Chest, 2013, Volume: 143, Issue:1

    Topics: Aminophenols; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Forced Expiratory Volume; Humans; Outcome Assessment, Health Care; Quinolones; Sweat; Treatment Outcome

2013
The use of ivacaftor in an adult with severe lung disease due to cystic fibrosis (ΔF508/G551D).
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2013, Volume: 12, Issue:5

    Topics: Adult; Aminophenols; Cystic Fibrosis; Forced Expiratory Volume; Humans; Lung Diseases; Male; Quinolones; Severity of Illness Index

2013
A bacterial cell to cell signal in the lungs of cystic fibrosis patients.
    FEMS microbiology letters, 2002, Sep-24, Volume: 215, Issue:1

    Topics: Bronchoalveolar Lavage Fluid; Cystic Fibrosis; Humans; Pneumonia, Bacterial; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolones; Signal Transduction; Sputum

2002
Quantitative proteomic analysis indicates increased synthesis of a quinolone by Pseudomonas aeruginosa isolates from cystic fibrosis airways.
    Proceedings of the National Academy of Sciences of the United States of America, 2003, Mar-04, Volume: 100, Issue:5

    Topics: Anti-Bacterial Agents; Child, Preschool; Cystic Fibrosis; Humans; Lipopolysaccharides; Magnesium; Models, Biological; Peptides; Proteome; Pseudomonas aeruginosa; Quinolones; Signal Transduction; Sputum; Temperature

2003
Microbiological rationale for the utilisation of prulifloxacin, a new fluoroquinolone, in the eradication of serious infections caused by Pseudomonas aeruginosa.
    International journal of antimicrobial agents, 2005, Volume: 26, Issue:5

    Topics: Anti-Bacterial Agents; Aza Compounds; Ciprofloxacin; Cystic Fibrosis; Dioxolanes; Drug Resistance, Bacterial; Fluoroquinolones; Humans; In Vitro Techniques; Levofloxacin; Microbial Sensitivity Tests; Moxifloxacin; Ofloxacin; Opportunistic Infections; Piperazines; Pseudomonas aeruginosa; Pseudomonas Infections; Quinolines; Quinolones

2005
[A case of cystic fibrosis in a Japanese student].
    Nihon Kyobu Shikkan Gakkai zasshi, 1995, Volume: 33, Issue:7

    Topics: Administration, Inhalation; Administration, Oral; Adolescent; Amikacin; Anti-Bacterial Agents; Anti-Infective Agents; Cystic Fibrosis; Drug Therapy, Combination; Erythromycin; Fluoroquinolones; Humans; Japan; Male; Pseudomonas Infections; Quinolones; Respiratory Tract Infections

1995
Maintenance treatment with antibiotics in cystic fibrosis patients. Sense or nonsense?
    The Netherlands journal of medicine, 1995, Volume: 46, Issue:6

    Topics: Aerosols; Anti-Bacterial Agents; Bacterial Infections; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Lung Diseases; Quinolones

1995