An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
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| "Cystic fibrosis is the most frequent substantially lethal inherited disease in the United States." | ( Schuttringer, G; Zinterhofer, L, 1978) |
| "Cystic fibrosis is caused by mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR)." | ( Cheng, SH; Denning, GM; Ostedgaard, LS; Smith, AE; Welsh, MJ, 1992) |
| "Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), which lead to defective Cl- conductance in epithelial cells." | ( Chang, XB; Grinstein, S; Kartner, N; Lukacs, GL; Riordan, JR; Rotstein, OD, 1992) |
| "Cystic fibrosis is a fatal genetic disorder which afflicts 50,000 people worldwide." | ( Alton, EW; Clarke, AR; Dickinson, P; Dorin, JR; Fleming, S; Geddes, DM; Hooper, ML; Kimber, WL; Smith, SN; Stevenson, BJ, 1992) |
| "Cystic fibrosis is thus another disease that may be associated with paucity of interlobular bile ducts presenting as neonatal hepatitis syndrome, and this represents a different pathogenesis of cholestatic jaundice in neonates with cystic fibrosis besides those previously recognized." | ( Canny, GJ; Furuya, KN; Phillips, MJ; Roberts, EA, 1991) |
| "Cystic fibrosis is associated with defective regulation of apical membrane chloride channels in airway epithelial cells." | ( Cozens, AL; Gardner, P; Gruenert, DC; Schulman, H; Stryer, L; Wagner, JA, 1991) |
| "The basic defect in cystic fibrosis is the chloride impermeability of the plasmalemma in different cells." | ( Babel, D; Hein, A; Hilschmann, N; Jürgens, L; König, U; Schmid, A; Thinnes, FP, 1991) |
| "Cystic fibrosis is a disease characterized by a defect in a chloride channel-associated regulatory protein found in epithelial cells." | ( Brubaker, RF; McCannel, CA; Scanlon, PD; Thibodeau, S, 1992) |
| "Cystic fibrosis is characterized by abnormal electrolyte transport across the epithelia of the airways." | ( Boucher, RC; Clarke, LL; Knowles, MR, 1991) |
| "Cystic fibrosis is associated with an cAMP-regulated channel defect, which has been evidenced in many cell types including B lymphocytes." | ( Auffredou, MT; Barot-Ciorbaru, R; Chicheportiche, R; Crevon, MC; Dayer, JM; Emilie, D; Galanaud, P; Lenoir, G, 1990) |
| "Cystic fibrosis is the most common autosomal recessive genetic disorder in the Caucasian population (1:2000-1:4000) (Warwick, W." | ( Bartholomé, K; Driesel, Aj; Grzeschik, KH; Kampmann, W; Mathy, L; Neugebauer, M; Olek, K, 1989) |
| "Adolescents with cystic fibrosis are liable to develop night blindness and conjunctival xerosis, particularly if they have liver disease or fail to take daily vitamin supplements." | ( Brimlow, G; Hiller, EJ; Marenah, C; Neugebauer, MA; Rayner, RJ; Tyrrell, JC; Vernon, SA, 1989) |
| "The hallmark of cystic fibrosis is progressive bronchopulmonary damage associated with chronic infection with Pseudomonas aeruginosa, leading to respiratory failure and, ultimately, death." | ( Ogra, PL; Piedra, P, 1986) |
| "Cystic fibrosis is characterized by an abnormally high electrolyte concentration in exocrine secretions." | ( Baldi, F; Calabria, G; Rolando, M, 1988) |
| "The diagnosis of cystic fibrosis is straight forward if the clinical findings and sweat sodium are considered together." | ( Collins, JE; Rolles, CJ, 1985) |
| "We conclude that cystic fibrosis is unrelated to any abnormality in plasma arginine esterase activity, contrary to some previous reports." | ( Barrett, AJ; Bury, AF, 1982) |
| "Cystic fibrosis is the most common fatal inherited disease of Caucasians." | ( Davis, PB; di Sant'Agnese, PA, 1984) |
| "Cystic fibrosis is the most common fatal genetic disease affecting caucasians and is perhaps best characterized as an exocrinopathy involving a disturbance in fluid and electrolyte transport." | ( Quinton, PM, 1983) |
| "Cystic fibrosis is an autosomal recessive disorder affecting chloride transport in pancreas, lung, and other tissues, which is caused by mutations in the cystic fibrosis transmembrane regulator (CFTR)." | ( Barnoy, S; Cabantchik, ZI; Eidelman, O; Guay-Broder, C; Guggino, WB; Jacobson, KA; Pollard, HB; Turner, RJ; Vergara, L; Zeitlin, PL, 1995) |
| "Cystic fibrosis is a monogenic disease that deranges multiple systems of ion transport in the airways, culminating in chronic infection and destruction of the lung." | ( Edwards, LJ; Engelhardt, JF; Hohneker, KW; Hu, PC; Jones, KR; Knowles, MR; Leigh, MW; Noah, TL; Olsen, JC; Zhou, Z, 1995) |
| "Cystic fibrosis is caused by mutations in the cell membrane protein called CFTR (cystic fibrosis transmembrane conductance regulator) which functions as a regulated Cl- channel." | ( Ko, YH; Pedersen, PL, 1995) |
| "In Japan, cystic fibrosis is rare, and this patient is extremely rare because he has grown up to be a 16-year-old." | ( Kitade, M; Moritaka, T; Nakanishi, N; Ueda, N, 1995) |
| "Cystic fibrosis is a disease characterized by abnormalities in the epithelia of the lungs, intestine, salivary and sweat glands, liver, and reproductive systems, often as a result of inadequate hydration of their secretions." | ( Colledge, WH; Evans, MJ; O'Brien, JA; Ratcliff, RA; Sepúlveda, FV; Valverde, MA, 1995) |
| "Cystic fibrosis is associated with severe abnormalities of the capacities of transport, due to the abnormal mucus and inflammatory changes of the epithelium." | ( Puchelle, E; Zahm, JM, 1995) |
| "The occurrence of cystic fibrosis is very rare in the Asian population." | ( Bhattacharya, SS; Boohene, J; Curtis, A; Jackson, A; Nelson, R; Richardson, RJ, 1993) |
| "Cystic fibrosis is the commonest, fatal, inherited disease of caucasian populations occurring with a frequency of 1 in 2000 live births." | ( Argent, BE; Harris, A, 1993) |
| "Cystic fibrosis is a serious, common genetic condition that causes recurrent pulmonary infections, malabsorption, and increased sweat electrolytes." | ( Fiedler, MA; Wilmott, RW, 1994) |
| "Cystic fibrosis is a recessive, fatal genetic disease involving multiple organ systems, in which patients develop pancreatic insufficiency, malabsorption, and repeated pulmonary infections." | ( Hall, M; Kuhn, RJ; Wallace, CS, 1993) |
| "Cystic fibrosis is a multisystem disease characterised by chronic pulmonary sepsis and malnutrition." | ( Ames, RW; Grey, AB; Matthews, RD; Reid, IR, 1993) |
| "Patients with cystic fibrosis are at risk for vitamin A deficiency because of fat malabsorption as well as for the inflammatory stresses of pulmonary exacerbations of their underlying disease." | ( Agil, A; Colin, AA; Duggan, C; Higgins, L; Rifai, N, 1996) |
| "Cystic fibrosis is associated with an inspissated bile syndrome producing cholestasis secondary to plugging of macroscopically normal bile ducts." | ( Cannon, R; Greenholz, SK; Krishnadasan, B; Marr, C, 1997) |
| "Patients with cystic fibrosis are at risk for impaired vitamin K status due to fat malabsorption from pancreatic insufficiency." | ( Ahrens, RA; Beker, LT; Davidson, KW; Fink, RJ; O'Brien, ME; Sadowski, JA; Sokoll, LJ, 1997) |
| "Cystic fibrosis is associated with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-regulated plasma membrane chloride channel." | ( Harper, GS; Hill, WG; Hopwood, JJ; Rozaklis, T, 1997) |
| "Many patients with cystic fibrosis are malnourished at the time of diagnosis." | ( Bruns, WT; Farrell, PM; Koscik, RE; Kosorok, MR; Laxova, A; Mischler, EH; Shen, G; Splaingard, M, 1997) |
| "Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel, which mediates transepithelial Cl- transport in a variety of epithelia, including airway, intestine, pancreas, and sweat duct." | ( Loffing, J; McCoy, D; Moyer, BD; Stanton, BA, 1998) |
| "Cystic fibrosis is a common disease of the Caucasian population and is associated with significant early mortality." | ( Chambers, SM; Poyser, KH; Wyatt, HA, 1998) |
| "Cystic fibrosis is a catabolic condition, yet the etiology of catabolism is poorly understood." | ( Hardin, DS; LeBlanc, A; Para, L; Seilheimer, DK, 1999) |
| "The genetic disease cystic fibrosis is caused by defects in the chloride channel cystic fibrosis transmembrane conductance regulator (CFTR)." | ( Nagel, G, 1999) |
| "Cystic fibrosis is characterised by oxidative stress in the airways." | ( Barnes, PJ; Ciabattoni, G; Corradi, M; Geddes, DM; Hodson, ME; Kharitonov, SA; Montuschi, P; van Rensen, L, 2000) |
| "Cystic fibrosis is a genetic disease in which persistent respiratory infection, usually due to P." | ( Birnbaum, H; Greenberg, P; LeLorier, J; Perreault, S; Sheehy, O, 2000) |
| "Patients with cystic fibrosis are at risk for malabsorption of fat-soluble vitamins, and those with low 25-OH vitamin D levels have a higher risk of low bone mineral density and long-term skeletal complications." | ( Drury, D; Grey, V; Lands, L; Pall, H, 2000) |
| "Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene." | ( Friedman, KJ; Ganeshananthan, M; Knowles, MR; Noone, PG; Pue, CA; Silverman, LM; Simon, RH; Wakeling, EL; Zhou, Z, 2000) |
| "Cystic fibrosis is a genetic disease occurring more frequently in Caucasians." | ( Clement, A; Fauroux, B; Tamalet, A, 2000) |
| "Cystic fibrosis is a genetic recessive disorder caused by mutations in the gene that encodes the CFTR protein." | ( Chinet, T; Fajac, I; Ferec, C; Garcia Carmona, T; Nguyen-Khoa, T, 2000) |
| "Cystic fibrosis is an autosomal recessive genetic disorder that causes dysfunction of exocrine glands, and has several clinical manifestations." | ( Alothman, GA; Gysin, C; Papsin, BC, 2000) |
| "Cystic fibrosis is a common, heriditary disease resulting from mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene." | ( Bernard, S; Cabrini, G; Diot, P; Faure, L; Le Pape, A; Lerondel, S; Lusky, M; Mehtali, M; Nicolis, E; Pavirani, A; Sené, C, 2001) |
| "Cystic fibrosis is an autosomal recessive disorder affecting the lungs, pancreas, intestines, sweat ducts and liver, due to an abnormal salt transport across the apical border of epithelial cells." | ( Scheffer, H; van den Ouweland, AM; Veeze, HJ, 2001) |
| "Cystic fibrosis is characterized by chronic pulmonary disease, insufficient pancreatic and digestive function, and abnormal sweat concentrations." | ( Caplan, D; Gatti, JM; Kirsch, AJ; Perez-Brayfield, MR; Smith, EA, 2002) |
| "Cystic fibrosis is a life-limiting autosomal recessive disorder with a highly variable clinical presentation." | ( Cutting, GR; Groman, JD; Meyer, ME; Wilmott, RW; Zeitlin, PL, 2002) |
| "Cystic fibrosis is the most common life threatening, inherited disease of the Caucasians." | ( Kumar, L; Prasad, R; Singh, M, 2002) |
| "Cystic fibrosis is a heterogenic disease, in which the phenotype can also vary for patients with the same genotype." | ( Andersson, C; Dragomir, A; Hjelte, L; Roomans, GM, 2002) |
| "Cystic fibrosis is a disease for which a number of Phase I clinical trials of gene therapy have been initiated." | ( Glick, MC; Klink, DT; Scanlin, TF, 2001) |
| "A patient with cystic fibrosis is reported who developed iatrogenic Cushing's syndrome after long-term treatment with daily doses of 800 mg itraconazole and 1,600 microg budesonide." | ( Dige-Petersen, H; Koch, C; Lanng, S; Main, KM; Müller, J; Sillesen, IB; Skov, M, 2002) |
| "Cystic fibrosis is characterised in the lungs by high levels of neutrophil elastase (NE)." | ( Carroll, TP; Devaney, JM; Greene, CM; McElvaney, NG; O'Neill, SJ; Taggart, CC, 2003) |
| "Cystic fibrosis is consistently accompanied by the ability to taste and smell salt, sweet, sour, and bitter substances in solution at concentrations much more dilute than those at which the substances are detectable by normal persons." | ( HENKIN, RI; POWELL, GF, 1962) |
| "Patients with cystic fibrosis are exposed to chronic oxidative stress due to an overproduction of reactive oxygen species as a result of neutrophil-dominated lung inflammation and impaired antioxidant status." | ( Ribalta, J; Rock, E; Roob, JM; Shmerling, DH; Winklhofer-Roob, BM, 2003) |
| "Cystic fibrosis is caused by mutations in the CFTR, resulting in impaired Cl(-) transport and plasma membrane targeting." | ( Thiagarajah, JR; Verkman, AS, 2003) |
| "Cystic fibrosis is caused by mutations in the gene encoding an ion-transport protein, the cystic-fibrosis transmembrane conductance regulator (CFTR)." | ( Chang Chan, H; Chung, YW; Jiang, JL; Lam, SY; Leung, KM; Li, J; Lo, PS; Rowlands, DK; Sha, J; Tsang, LL; Yu, MK; Zhou, Z; Zhu, H; Zhu, JX, 2003) |
| "Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR)." | ( Canny, S; Caplan, MJ; Du, K; Egan, ME; Glöckner-Pagel, J; Lukacs, GL; Pearson, M; Rajendran, V; Rubin, D; Weiner, SA, 2004) |
| "The lung disease of cystic fibrosis is associated with a chronic inflammatory reaction and an over abundance of oxidants relative to antioxidants." | ( Bahlool, R; Grey, V; Lands, LC; Mohammed, SR; Smountas, AA, 2003) |
| "Although cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, the severity of disease is highly variable indicating the influence of modifier genes." | ( De Lisle, RC; Norkina, O, 2005) |
| "Patients with cystic fibrosis are particularly at risk of infection with non-tuberculous mycobacteria (NTM)." | ( Catherinot, E; Gaillard, JL; Le Bourgeois, M; Sermet-Gaudelus, I, 2005) |
| "Cystic fibrosis is caused by dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, leading to altered ion transport, chronic infection, and excessive inflammation." | ( Clancy, JP; Li, Y; Parker, W; Wang, W, 2006) |
| "Cystic fibrosis is usually diagnosed based on suspicion arising from a typical clinical picture and must be confirmed by either a finding of high chloride concentrations in sweat tests on 2 separate days or detection of 2 gene mutations." | ( Bosque-García, M; Domingo-Ribas, C, 2006) |
| "Cystic Fibrosis is the most common lethal genetic disease among Caucasian population." | ( Lallemand, JY; Leclaire, B; Lenoir, G; Sermet, I, 2005) |
| "Cystic fibrosis is a life-threatening autosomal recessive disorder with a highly variable clinical presentation." | ( Soferman, R, 2006) |
| "Cystic fibrosis is the most common autosomal recessive lethal genetic disorder in the Caucasian population." | ( Bán, Z; Lázár, L; Nagy, B; Nagy, GR; Papp, Z, 2006) |
| "Cystic fibrosis is an autosomal recessive disease affecting approximately 1 in 2500 live births." | ( Anklesaria, P; Coates, AL; Dutzar, B; Leung, K; Louca, E; Munson, K, 2007) |
| "Cystic fibrosis is the most common autosomal recessive genetic defect of one gene CFTR, where a variety of mutations were revealed." | ( Iwańczak, F; Mowszet, K; Pawłowicz, J; Sasiadek, M; Smigiel, R; Stawarski, A; Stembalska, A, 2005) |
| "CF (cystic fibrosis) is a disease caused by mutations within the CFTR (CF transmembrane conductance regulator) gene." | ( Cardone, RA; Casavola, V; De Santis, T; Fanelli, T; Favia, M; Guerra, L; Monterisi, S; Reshkin, SJ; Riccardi, SM; Zaccolo, M, 2008) |
| "Cystic fibrosis is the most common incurable hereditary disease in the US." | ( Duh, MS; Sacco, P; Toy, EL; Weiner, JR, 2008) |
| "Cystic fibrosis is the most common lethal genetic disease in Caucasians, manifesting as progressive lung dysfunction, pancreatic insufficiency, and intestinal disease." | ( Kleven, DT; McCudden, CR; Willis, MS, 2008) |
| "Cystic fibrosis is a chronic progressive autosomal recessive disorder caused by the CFTR gene mutations." | ( Ahn, KM; Cha, SI; Ki, CS; Kim, JH; Kim, SJ; Lee, M; Park, HY, 2008) |
| "Cystic fibrosis is characterized by recurring pulmonary exacerbations that lead to the deterioration of lung function and eventual lung failure." | ( Chen, J; Cotton, C; Kelley, TJ; Kinter, M; Shank, S; Ziady, AG, 2008) |
| "CF (cystic fibrosis) is caused by mutations in CFTR (CF transmembrane conductance regulator), which cause its mistrafficking and/or dysfunction as a regulated chloride channel on the apical surface of epithelia." | ( Bear, CE; Li, C; Pasyk, S; Ramjeesingh, M, 2009) |
| "Cystic fibrosis is one of the most common lethal hereditary diseases and is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene." | ( Mall, MA, 2009) |
| "Cystic fibrosis is an inherited condition resulting in thickened, sticky respiratory secretions." | ( Nash, EF; Ratjen, F; Stephenson, A; Tullis, E, 2009) |
| "Cystic fibrosis is usually diagnosed during the first years of life." | ( Bienvenu, T; Burgel, PR; Coman, T; Desmazes-Dufeu, N; Dusser, D; Fajac, I; Hubert, D; Kanaan, R, 2009) |
| "Cystic fibrosis is characterized by relevant cholesterol oxidation that is associated with an abnormal fatty acid profile." | ( Boaz, M; Galli, F; Iuliano, L; Monticolo, R; Quattrucci, S; Straface, G; Zullo, S, 2009) |
| "Patients with cystic fibrosis are frequently affected with pancreatic insufficiency and are predisposed to the development of diabetes mellitus (DM) and bone demineralization." | ( Curran, DR; McArdle, JR; Talwalkar, JS, 2009) |
| "Cystic fibrosis is a lethal autosomal recessive disorder usually associated with lung disease, pancreatic insufficiency and high sweat chloride levels." | ( Conoley, VG; Frederick, CA; Kappes, J; Li, C; Naren, AP; Nekkalapu, S; Penmatsa, H; Stokes, DC; Zhang, W, 2009) |
| "Cystic fibrosis is one of the most common autosomal recessive hereditary diseases in the Caucasian population, with an incidence of 1:2000 to 1:3500 liveborns." | ( Fernandes, MI; Gabetta, CS; Maciel, LM; Magalhaes, PK; Pazin Filho, A; Pedro, KP; Ribeiro, AF; Rodrigues, R; Santos, JL; Souza, RM; Valdetaro, F, 2009) |
| "Cystic fibrosis is an autosomal recessive disorder and it is characterised by chronic bacterial airway infection which leads to progressive lung deterioration, sometimes with fatal outcome." | ( Cescutti, P; Ieranò, T; Lanzetta, R; Leone, MR; Luciani, A; Maiuri, L; Molinaro, A; Parrilli, M; Raia, V; Rizzo, R; Silipo, A, 2010) |
| "Cystic fibrosis is the most common, multiorgan inherited autosomal recessive disorder." | ( Iwańczak, B; Iwańczak, F; Pytrus, T; Ryzko, J; Smigiel, R; Socha, P, 2009) |
| "Cystic fibrosis is caused by mutations in CFTR, the cystic fibrosis transmembrane conductance regulator gene." | ( Camden, JM; Catalán, MA; Clarke, LL; Gonzalez-Begne, M; Melvin, JE; Nakamoto, T; Wall, SM, 2010) |
| "Cystic fibrosis is one of the most common life-shortening, childhood-onset inherited diseases." | ( Beni, V; Nasef, H; O'Sullivan, CK, 2010) |
| "Cystic fibrosis is associated with pancreatic insufficiency and acidic intraluminal conditions that limit the action of pancreatic enzyme replacement therapy, especially that of lipase." | ( Colin, DY; Deprez-Beauclair, P; Infantes, L; Kerfelec, B; Silva, N, 2010) |
| "Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (encoded by Cftr) that impair its role as an apical chloride channel that supports bicarbonate transport." | ( Barrett, KE; Dennis, EA; Dong, H; Dumlao, DS; Glass, CK; Harmon, GS; Ng, DT, 2010) |
| "Cystic fibrosis is one of the most common genetically inherited diseases in Northern Europe, consisting of an inherited defect of chloride transport in the epithelium." | ( Beni, V; Hayes, K; Lerga, TM; O'Sullivan, CK, 2010) |
| "Cystic fibrosis is an inherited, life-threatening disease associated with mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene." | ( Andersen, J; Cotard, S; Lee, M; Lin, S; Staunton, J; Sui, J; Zhu, P, 2010) |
| "Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction." | ( Chang, AB; Fedorowicz, Z; Jagannath, VA; Thaker, V, 2011) |
| "Cystic fibrosis is caused by impaired ion transport due to mutated cystic fibrosis transmembrane conductance regulator, accompanied by elevated activity of the amiloride-sensitive epithelial Na(+) channel (ENaC)." | ( Brose, N; Jiang, C; Kawabe, H; Kimura, T; Lu, C; Lu, WY; Rotin, D; Salter, MW; Xiang, YY; Zhang, W, 2011) |
| "Cystic fibrosis is caused by mutations of the Cystic Fibrosis Transmembrane conductance Regulator gene (CFTR)." | ( Commandeur, D; Corcos, L; Dujardin, G; Ferec, C; Le Jossic-Corcos, C, 2011) |
| "Cystic fibrosis is a hereditary disease caused by a mutation in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene that encodes a chloride (Cl(-)) channel." | ( Clement, A; Guillot, L; Jacquot, J; Rebeyrol, C; Ruffin, M; Saint-Criq, V; Tabary, O, 2012) |
| "Cystic fibrosis is a monogenic recessive disorder found predominantly in Caucasian population." | ( Bazzaz, JT; Douki, MR; Larijani, B; Niaki, HA; Tabaripour, R; Yaghmaei, P, 2012) |
| "Women with cystic fibrosis are at increased risk for mucoid conversion of Pseudomonas aeruginosa, which contributes to a sexual dichotomy in disease severity." | ( Chotirmall, SH; Cosgrove, S; Dimitrov, BD; Greene, CM; Gunaratnam, C; Harvey, BJ; McElvaney, NG; O'Neill, SJ; Smith, SG, 2012) |
| "A hallmark of cystic fibrosis is the massive recruitment of neutrophils into the lung compartment in response to chronic Pseudomonas aeruginosa infection." | ( Bensman, TJ; Beringer, PM; Nguyen, AN; Rao, AP, 2012) |
| "Most cystic fibrosis is caused by the deletion of a single amino acid (F508) from CFTR and the resulting misfolding and destabilization of the protein." | ( Aleksandrov, AA; Cui, L; Dokholyan, NV; He, L; Jensen, T; Kota, P; Riordan, JR, 2013) |
| "Cystic fibrosis is one of the most common autosomal recessive genetic disorders in caucasians of Northern European descent, affecting approximately 30 000 children and adults living in the United States, today." | ( Almgren, MM; Braga, SF, 2013) |
| "Cystic fibrosis is a genetic disorder in which abnormal mucus in the lungs is associated with susceptibility to persistent infection." | ( Jahnke, N; Remmington, T; Ryan, G, 2012) |
| "Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction." | ( Chang, AB; Fedorowicz, Z; Jagannath, VA; Thaker, V, 2013) |
| "Cystic fibrosis is an autosomal recessive genetic disease due to mutations in the (cystic fibrosis transmembrane conductance regulator) CFTR gene." | ( Fajac, I; Sermet-Gaudelus, I, 2013) |
| "Cystic fibrosis is a multi-system disease characterised by the production of thick secretions causing recurrent pulmonary infection, often with unusual bacteria." | ( Forrester, DL; Hurley, MN; Smyth, AR, 2013) |
| "Cystic fibrosis is a common disease which has an associated characteristic symptom of high sweat chloride content." | ( Batchelor-McAuley, C; Compton, RG; Toh, HS; Tschulik, K, 2013) |
| "Cystic fibrosis is an inherited condition resulting in thickened, sticky respiratory secretions." | ( Nash, EF; Ratjen, F; Stephenson, A; Tam, J; Tullis, E, 2013) |
| "Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described." | ( Calero Acuña, C; Delgado-Pecellín, I; Quintana-Gallego, E, 2014) |
| "Cystic fibrosis is a serious genetic disease due to mutations in the gene encoding CFTR (Cystic FibrosisTransmembrane conductance Regulator), a protein involved in cellular transmembrane transport, particularly of chloride ions." | ( , 2013) |
| "Cystic fibrosis is caused by dysfunction or deficiency of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, an epithelial chloride channel that has a key role in maintaining homoeostasis of the airway surface liquid layer in the lungs." | ( Boyle, MP; De Boeck, K, 2013) |
| "Cystic fibrosis care programs were assigned to quartiles based on adjusted mean patient BMI percentiles." | ( Haupt, ME; Kwasny, MJ; McColley, SA; Schechter, MS, 2014) |
| "Cystic fibrosis is a life-threatening genetic disease mainly characterised by accumulation of viscous secretions in the airways." | ( , 2014) |
| "Cystic fibrosis is responsible for severe chronic pulmonary disorders in children." | ( Agarwal, A; Sengar, AS; Singh, MK, 2014) |
| "Because diagnosing cystic fibrosis is a combined effort between local pediatric departments, biochemical and genetic departments and cystic fibrosis centers, a national harmonization is necessary to assure correct clinical use." | ( Christiansen, AL; Nybo, M, 2014) |
| "People with cystic fibrosis are at an increased risk of fat-soluble vitamin deficiency including vitamin E." | ( Barrett, J; Kansra, S; Okebukola, PO, 2014) |
| "Cystic fibrosis is the most common inherited life-shortening illness in Caucasians and caused by a mutation in the gene that codes for the cystic fibrosis transmembrane regulator protein (CFTR), which functions as a salt transporter." | ( Dwan, K; Echevarria, C; Patel, S; Schechter, M; Sinha, IP; Southern, KW, 2015) |
| "Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction." | ( Chang, AB; Fedorowicz, Z; Jagannath, VA; Thaker, V, 2015) |
| "Cystic fibrosis is a life-limiting disease that is caused by defective or deficient cystic fibrosis transmembrane conductance regulator (CFTR) protein activity." | ( Boyle, MP; Cipolli, M; Colombo, C; Davies, JC; De Boeck, K; Elborn, JS; Flume, PA; Huang, X; Konstan, MW; Marigowda, G; McColley, SA; McCoy, K; McKone, EF; Munck, A; Ramsey, BW; Ratjen, F; Rowe, SM; Wainwright, CE; Waltz, D, 2015) |
| "Biomarkers in cystic fibrosis are used i." | ( Ramsey, KA; Schultz, A; Stick, SM, 2015) |
| "Cystic Fibrosis is caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene resulting in abnormal protein function." | ( Marshall, SG; Ramsey, BW; Rowe, SM; Solomon, GM, 2015) |
| "Cystic fibrosis is the most common genetically determined, life-limiting disorder in populations of European ancestry." | ( Brodlie, M; Elborn, JS; Haq, IJ; Roberts, K, 2015) |
| "Cystic fibrosis is an autosomal recessive multisystem disorder with an approximate prevalence of 1 in 3500 live births." | ( Jat, KR; Khairwa, A; Walia, DK, 2015) |
| "Cystic fibrosis is an inherited life-threatening multisystem disorder with lung disease characterized by abnormally thick airway secretions and persistent bacterial infection." | ( Ahmed, MI; Mukherjee, S, 2016) |
| "Depressed MCC in cystic fibrosis is associated with P." | ( Corcoran, TE; Czachowski, MR; Lacy, RT; Locke, LW; Markovetz, MR; Muthukrishnan, A; Myerburg, MM; Parker, RS; Pilewski, JM; Weber, L; Weiner, DJ, 2016) |
| "NBS for cystic fibrosis is a cost-effective strategy and, if standards of care are fully implemented and robust management pathways are in place, has a positive effect on clinical outcomes." | ( Castellani, C; Massie, J; Sontag, M; Southern, KW, 2016) |
| "Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), an epithelial cell anion channel." | ( Linsdell, P, 2017) |
| "Cystic fibrosis is a common life-shortening genetic disorder in the Caucasian population (less common in other ethnic groups) caused by the mutation of a single gene that codes for the production of the cystic fibrosis transmembrane conductance regulator protein." | ( Aslam, AA; Higgins, C; Sinha, IP; Southern, KW, 2017) |
| "Cystic fibrosis is realizing the promise of personalized medicine." | ( Bear, CE; Boëlle, PY; Corvol, H; Gong, J; Gonska, T; He, G; Ip, W; Keenan, K; Li, W; Lin, F; Panjwani, N; Parkins, MD; Price, A; Rabin, H; Ratjen, F; Rommens, JM; Soave, D; Strug, LJ; Sun, L; Tullis, E; Xiao, B; Zuberbuhler, PC, 2016) |
| "Children with cystic fibrosis are commonly colonized with multi-resistant bacteria." | ( Abaji, R; Autmizguine, J; Goyer, I; Iseppon, M; Krajinovic, M; Thibault, C, 2017) |
| "People with cystic fibrosis are at an increased risk of fat-soluble vitamin deficiency including vitamin E." | ( Barrett, J; Kansra, S; Okebukola, PO, 2017) |
| "Cystic fibrosis is the most common genetic disease, in which symptoms may be alleviated but not fully eliminated." | ( Abu-Arish, A; Cupri, S; De Sanctis, JB; Garić, D; Hajduch, M; Hanrahan, JW; Houle, D; Matouk, E; Radzioch, D; Wojewodka, G, 2017) |
| "Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction." | ( Chang, AB; Jagannath, VA; Price, AI; Thaker, V, 2017) |
| "Cystic Fibrosis is an autosomal recessive disorder caused by mutations in the CFTR gene." | ( Harrison, PT; Hollywood, JA; Sanz, DJ; Scallan, MF, 2017) |
| "Cystic fibrosis is an autosomal recessive disorder characterized by chronic progressive multisystem involvement." | ( Bianco, A; Calabrese, C; Carnovale, V; Ferrara, N; Iacotucci, P; Iadevaia, C; Mazzarella, G; Perrotta, F; Rea, G, 2017) |
| "Cystic fibrosis is an autosomal recessive disease caused by mutations in the CFTR gene that lead to progressive respiratory decline." | ( Daines, C; Davies, JC; Han, L; Ingenito, EP; Kerem, E; Lekstrom-Himes, J; McKee, C; Nair, N; Ringshausen, FC; Rowe, SM; Simard, C; Tullis, E; Wilson, J, 2017) |
| "Cystic Fibrosis is the most common recessive autosomal rare disease found in Caucasian." | ( Adolphe, C; El-Seedy, A; Farhat, R; Kitzis, A; Ladevèze, V; Norez, C; Pasquet, MC; Talbot, H, 2017) |
| "Cystic fibrosis is a life-limiting inherited condition which affects one in 2500 newborns in the UK and 70,000 children and adults worldwide." | ( Edwards, CT; Smith, S, 2017) |
| "Cystic fibrosis is the most common genetic disorder in Caucasians." | ( Benden, C; Bürgi, U; Hirt, A; Huber, LC; Kurowski, T; Murer, C; Robinson, CA, 2018) |
| "Cystic fibrosis is an autosomal recessive multisystem disorder with an approximate prevalence of 1 in 3500 live births." | ( Jat, KR; Khairwa, A; Walia, DK, 2018) |
| "Patients with cystic fibrosis are more likely to have glutathione deficiency, and greater susceptibility to liver injury." | ( Cheung, B; Gantier, M; Graudins, A; Nejad, C; Wong, A, 2018) |
| "Cystic fibrosis is a life-threatening genetic disease that causes severe damage to the lungs." | ( Feng, LB; Fink, AK; Green, RF; Grosse, SD; Sawicki, GS, 2018) |
| "Cystic fibrosis is a genetic disease that affects approximately 75,000 individuals around the world." | ( Flume, PA; Jennings, MT, 2018) |
| "Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in sufferers." | ( Lo, DK; Muhlebach, MS; Smyth, AR, 2018) |
| "Cystic fibrosis is an inherited life-threatening multisystem disorder with lung disease characterized by abnormally thick airway secretions and persistent bacterial infection." | ( Ahmed, MI; Mukherjee, S, 2018) |
| "Cystic fibrosis is the most common life-limiting genetic condition in Caucasians caused by Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene mutations." | ( Graff, G; Siddaiah, R; Thau, E, 2018) |
| "Cystic fibrosis is a genetic disorder in which abnormal mucus in the lungs is associated with susceptibility to persistent infection." | ( Charbek, E; Rowbotham, NJ; Smith, S, 2018) |
| "Cystic fibrosis is the most common metabolic chronic disease among European Caucasian children." | ( Acıcan, D; Demir, O; Ercan, Ö; Erdoğan, M; Göçlü, F; Güney, D; Hangül, M; Kendirci, M; Köse, M; Öznavruz, H; Pekcan, S; Şahlar, TE, 2019) |
| "Patients with cystic fibrosis are more susceptible than members of the general population to lung infections." | ( Claude, F; Hafen, GM; Rochat, I, 2019) |
| "Cystic fibrosis is a fatal disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR)." | ( Chen, J; Levit, A; Levring, J; Liu, F; Shoichet, BK; Touhara, KK; Zhang, Z, 2019) |
| "Cystic Fibrosis is a lethal monogenic autosomal recessive disease linked to mutations in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein." | ( Aissat, A; Bizard, L; Decrouy, X; Degrugillier, F; Fanen, P; Jiang, C; Prulière-Escabasse, V; Rotin, D; Simon, S; Simonneau, B, 2020) |
| "Cystic fibrosis is associated with significant morbidity and early mortality due to recurrent acute and chronic lung infections." | ( Biswas, B; Bivens, BN; Brown, DE; Brownstein, MJ; Burch, AK; Daniels, R; Fackler, J; Gainey, AB; Horne, B; Malagon, F, 2020) |
| "Cystic fibrosis is a multi-system disease characterised by the production of thick secretions causing recurrent pulmonary infection, often with unusual bacteria." | ( Forrester, DL; Hurley, MN; Smith, S; Smyth, AR, 2020) |
| "Cystic fibrosis is a genetic disease caused by loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator gene, encoding for CFTR protein." | ( Leoni, A; Locatelli, A; Morigi, R; Pedemonte, N; Pesce, E, 2020) |
| "People with cystic fibrosis are at an increased risk of fat-soluble vitamin deficiency, including vitamin E." | ( Barrett, J; Kansra, S; Okebukola, PO, 2020) |
| "Sufferers of cystic fibrosis are at extremely high risk for contracting chronic lung infections." | ( Ahmadipour, S; Beswick, L; Dimitriou, E; Field, RA; Miller, GJ; Rejzek, M; Reynisson, J; Zafar, A, 2020) |
| "Cystic fibrosis is a genetic disorder that results in a multi-organ disease with progressive respiratory decline which leads to premature death." | ( Grepo, N; Jaffe, A; Morris, KV; Scott, T; Villamizar, O; Waters, SA, 2021) |
| "Cystic fibrosis is the most common life-limiting genetic disease in the Caucasian population, with median predicted survival progressively improving up to 50 years, thanks to highly standardized multidisciplinary approach." | ( Aliberti, S; Bindo, F; Blasi, F; Contarini, M; Gramegna, A, 2021) |
| "Cystic fibrosis is a monogenic, autosomal, recessive disease characterized by an alteration of chloride transport caused by mutations in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene." | ( Franceschelli, S; Pascale, M; Pecoraro, M, 2021) |
| "Cystic fibrosis is a deadly multiorgan disorder caused by loss of function mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator (CFTR) chloride/bicarbonate ion channel." | ( Arora, K; Brewington, J; Clancy, JP; Cortez, AR; Helmrath, M; McPhail, G; Naren, AP; Ogden, H; Ramananda, Y; Sundaram, N; Yang, F, 2021) |
| "Cystic fibrosis is a monogenic disease considered to affect at least 100 000 people worldwide." | ( Davies, JC; Haq, IJ; Polineni, D; Shteinberg, M, 2021) |
| "Cystic fibrosis is the most prevalent inherited disease caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene." | ( Beekman, JM; Bierlaagh, MC; Muilwijk, D; van der Ent, CK, 2021) |
| "Cystic fibrosis is a life-limiting, inherited, multi-organ disease which affects many systems of the body." | ( Blaikie, L; Collins, S; Daniels, T; Edwards, V; Ketchell, I; McCulloch, A; Shaw, N; Smith, T, 2021) |
| "Cystic fibrosis is a genetic disorder in which abnormal mucus in the lungs is associated with susceptibility to persistent infection." | ( Charbek, E; Rowbotham, NJ; Smith, S, 2022) |
| "Cystic fibrosis is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) that can lead to terminal respiratory failure." | ( Hacheney, I; Krutmann, J; Nguyen, T; Rossi, A; Schins, RPF; Stahlmecke, B; Stermann, T; Todea, AM; Unfried, K; Woeste, S, 2022) |
| "Cystic fibrosis is an autosomal-recessive defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene located on chromosome 7 that affects 1 in 2500 live White births." | ( Fialkowski, E; Maloney, L; Tillotson, M; Tobias, J, 2022) |
| "Cystic fibrosis is a hereditary disease mainly caused by the deletion of the Phe 508 (F508del) of the cystic fibrosis transmembrane conductance regulator (CFTR) protein that is thus withheld in the endoplasmic reticulum and rapidly degraded by the ubiquitin/proteasome system." | ( D'Ursi, P; Ford, RC; Fossa, P; Meng, X; Milanesi, M; Orro, A; Padoan, R; Pedemonte, N; Pesce, E; Rondina, A; Rusnati, M; Uggeri, M; Urbinati, C, 2022) |
| "Cystic fibrosis is a genetic disease caused by mutation of the CFTR gene, which encodes a chloride and bicarbonate transporter in epithelial cells." | ( Hafkemeyer, S; Nietert, MM; Stanke, F; Vinhoven, L, 2022) |
| "Cystic fibrosis is a severe monogenic disease that affects around 7400 patients in France." | ( Foucaud, P; Mercier, JC, 2023) |
| "Cystic fibrosis is a severe monogenic disease that affects around 7400 patients in France." | ( Foucaud, P; Mercier, JC, 2023) |
| "Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in sufferers." | ( Lo, DK; Muhlebach, MS; Smyth, AR, 2022) |
| "Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in sufferers." | ( Lo, DK; Muhlebach, MS; Smyth, AR, 2022) |
| "Cystic fibrosis is a severe monogenic disease that affects around 7 300 patients in France." | ( Durieu, I; Durupt, S; Reynaud, Q, 2022) |
| "Cystic fibrosis is a severe monogenic disease that affects around 7 300 patients in France." | ( Durieu, I; Durupt, S; Reynaud, Q, 2022) |
| "Cystic fibrosis is caused by mutations impairing expression, trafficking, stability and/or activity of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel." | ( Bocciardi, R; Capurro, V; Castellani, C; Costa, S; Cresta, F; Cristadoro, S; Di Duca, M; Galietta, LJV; Lena, M; Lucanto, MC; Pastorino, C; Pedemonte, N; Pesce, E; Sondo, E; Tomati, V; Zara, F, 2023) |
| "Cystic fibrosis is an autosomal recessive disease that causes respiratory tract infection." | ( Baniasadi, S; Hassanzad, M; Kouhestani, F, 2024) |
| "Cystic fibrosis is a life-threatening genetic disorder caused by mutations in the CFTR chloride channel." | ( Hu, Y; Jennings, S; Luo, M; Nauseef, WM; Scull, C; Taylor, CM; Wang, G; Wellems, D, 2023) |
| "Cystic fibrosis is a severe, autosomal recessive disease that shortens life expectancy." | ( Kabra, VD; Lahoti, SR, 2023) |
| "Children with cystic fibrosis are at risk of fat-soluble vitamin deficiency." | ( Balfour-Lynn, IM; Bentley, S; Carr, SB; Schembri, L; Warraich, S, 2023) |
| Excerpt | Reference |
|---|
| "Bile is lithogenic in untreated cystic fibrosis and responds to pancreatic enzymes." | ( Combes, JC; Lasalle, R; Mégevand, A; Morin, CL; Nusslé, D; Roy, CC; Weber, AM, 1977) |
| "The effect of chronic treatment with cystic fibrosis (CF) fibroblast medium on rat submandibular gland and pancreas was investigated." | ( Ceder, O; Kollberg, H; Roomans, GM; von Euler, AM, 1985) |
| "Advances in the treatment of cystic fibrosis, as well as a review concerning the pulmonary toxicity of various medications used in the treatment of pediatric illness are discussed." | ( Reisman, JJ, 1993) |
| "Amiloride inhalation as treatment for cystic fibrosis (CF) lung disease has been shown in independent studies to increase mucus clearance by ciliary and/or cough action and to retard the decline in lung function." | ( App, EM; Boucher, RC; Church, N; King, M; Knowles, MR; Ramirez, O; Tomkiewicz, RP; Zayas, JG, 1993) |
| "centers for the treatment of cystic fibrosis and randomly assigned to one of two crossover regimens." | ( Astley, SJ; Dorkin, HL; Eisenberg, JD; Gibson, RL; Harwood, IR; Kravitz, RM; McBurnie, MA; Ramsey, BW; Schidlow, DV; Wilmott, RW, 1993) |
| "The use of inhaled antibiotics in the treatment of cystic fibrosis has become widespread despite controversy in the literature as to the appropriate dosing regimen and its effectiveness." | ( Coates, AL; Kelemen, S; MacDonald, J; MacNeish, CF; Meisner, D; Thibert, R; Vadas, E, 1997) |
| "TH1 reacted strongly with TFMSA treated cystic fibrosis mucin but not with the fully glycosylated mucin, indicating reactivity with a core mucin epitope." | ( Devine, PL; Liew, YW; Quin, RJ; Sheehan, JK; Shield, PW; Thornton, DJ, 1998) |
| "Ursodiol, the drug of choice for the treatment of cystic fibrosis liver disease, may offer a safe method of using high-dose IBU in these patients by ameliorating the enteropathy." | ( Beno, DW; Jiyamapa-Serna, VA; Kimura, RE; Lloyd-Still, JD; Uhing, MR, 2001) |
| "Hence these compounds may be useful to treat cystic fibrosis (CF) airway disease." | ( Forman, HJ; Fuller, CM; Gao, L; Matalon, S; Sorscher, EJ; Venglarik, CJ; Yankaskas, JR, 2001) |
| "As piperacillin is commonly used in the treatment of cystic fibrosis, anti-piperacillin should be considered whenever patients with cystic fibrosis develop haemolytic anaemia and/or positive DATs." | ( Arndt, PA; Chandrasekaran, V; Garratty, G; Hill, J; Kasper, M, 2002) |
| "Azithromycin is used for the treatment of cystic fibrosis lung disease, although its mechanisms of action are not completely understood." | ( Cazzola, G; Cipolli, M; Delmarco, A; Mini, E; Morganti, M; Pradal, U, 2005) |
| "Azithromycin is used for the treatment of cystic fibrosis lung disease, although its mechanisms of action are poorly understood." | ( Delos, M; Huaux, F; Leal, T; Lebacq, J; Lebecque, P; Legssyer, R; Lison, D; Marbaix, E; Scholte, BJ; Wallemacq, P, 2006) |
| "Long-term low dose azithromycin treatment in cystic fibrosis patients with chronic Pseudomonas aeruginosa infection is safe and reduces the decline in lung function, the number of acute exacerbations and improves nutritional status; underlying efficacy mechanisms are multiple and synergistic." | ( Amiour, M; El Hachem, C; Guillot, M; Harchaoui, S; Paris, C; Ribault, V, 2006) |
| "The spectrum of treatments for cystic fibrosis lung disease is rapidly increasing." | ( Ratjen, F, 2007) |
| "Small-scale clinical trials show that treatment of cystic fibrosis (CF) patients with ibuprofen, a nonsteroidal anti-inflammatory drug, improves the symptoms of CF and slows down the decline of lung function." | ( Hu, J; Li, J; Lu, WY; Macdonald, JF; Tsui, LC; Xiang, YY; Ye, L, 2008) |
| "A new approach in the treatment of cystic fibrosis involves improving the function of mutant cystic fibrosis transmembrane conductance regulator (CFTR)." | ( 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, J, 2010) |
| "aeruginosa isolated from polymyxin-treated cystic fibrosis patients harbored mutant alleles of colRS and cprS; when expressed in a ΔphoQ background, these mutant alleles enhanced polymyxin resistance." | ( Brannon, MK; Gutu, AD; Haugen, E; Høiby, N; Jacobs, MA; Johansen, HK; Kaul, RK; Moskowitz, SM; Sgambati, N; Strasbourger, P, 2013) |
| "Lung transplantation is an established treatment for cystic fibrosis (CF) patients with end-stage lung disease." | ( Annesley, T; Bartos, C; Cibrik, DM; Clark Ojo, T; Park, JM; Stuckey, L, 2014) |
| "It is now licensed for the treatment of cystic fibrosis patients 6 years of age and older who carry the CFTR G551D mutation, which is the case for about 4% to 5% of cystic fibrosis patients." | ( , 2013) |
| "We presently have numerous symptomatic treatments for the cystic fibrosis care." | ( Durieu, I; Durupt, S; Nove Josserand, R, 2014) |
| "Powder inhalers for the treatment of cystic fibrosis have recently been made available." | ( Steinkamp, G, 2014) |
| "Significant improvements in the treatment of cystic fibrosis over the last few decades have altered this lethal disease in children to a multisystem disorder with survival into adult life now common." | ( Kumar, S; Shankar, A; Tana, A, 2014) |
| "The best investigational drug to treat cystic fibrosis (CF) patients with the most common CF-causing mutation (F508del) is VX-809 (lumacaftor) which recently succeeded in Phase III clinical trial in combination with ivacaftor." | ( Amaral, MD; Awatade, NT; Clarke, LA; Farinha, CM; Mendes, K; Pastor, J; Ramos, MM; Solé, A; Uliyakina, I, 2015) |
| "Ivacaftor is indicated for treatment of cystic fibrosis (CF) mediated by 10 mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene that causes gating or partial function abnormalities." | ( McColley, SA, 2016) |
| "Ivacaftor was approved in 2012 to treat patients with cystic fibrosis (CF) with specific CFTR gene mutations." | ( Bonafede, M; Limone, B; O'Callaghan, L; Sawicki, GS; Suthoff, ED; Wagener, JS, 2016) |
| "Ivacaftor, a breakthrough treatment for cystic fibrosis (CF) patients with the G551D genetic mutation, lacks long-term clinical and cost projections." | ( Campbell, JD; Dilokthornsakul, P; Hansen, RN, 2016) |
| "Potentiator drugs used in the treatment of cystic fibrosis act on the channel to increase overall channel function, by increasing the stability of its open state and/or decreasing the stability of its closed state." | ( Linsdell, P, 2017) |
| "However, the usefulness of NAC in the treatment of cystic fibrosis and bronchiectasis is still matter of debate." | ( Blasi, F; Cazzola, M; Matera, MG; Page, C; Pallecchi, L; Rogliani, P; Rossolini, GM, 2016) |
| "Lumacaftor-ivacaftor is indicated for treatment of cystic fibrosis (CF) in patients homozygous for the Phe-508del cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations." | ( McColley, SA; Talamo Guevara, M, 2017) |
| "Combination treatment with the cystic fibrosis transmembrane conductance regulator (CFTR) modulators tezacaftor (VX-661) and ivacaftor (VX-770) was designed to target the underlying cause of disease in patients with cystic fibrosis." | ( Elborn, JS; Ingenito, EP; Lekstrom-Himes, J; Lu, Y; McKee, C; McKone, EF; Moeller, A; Munck, A; Simard, C; Taylor-Cousar, JL; van der Ent, CK; Wang, LT, 2017) |
| "Forty one parents of children receiving treatment at the Cystic fibrosis (CF) clinic were approached to be part of the study." | ( Ahangar, AA; Ahmad, QI; Ali, SW; Bhat, JI; Charoo, BA; Wani, WA, 2018) |
| "A new class of drugs in the treatment of cystic fibrosis (CF) includes two agents: lumacaftor, which corrects CFTR channel protein, and ivacaftor, which increases CFTR channel activity." | ( De Biase, RV; Gnessi, L; Lenzi, A; Lubrano, C; Pascucci, C; Quattrucci, S; Savi, D, 2019) |
| "Antibiotic therapy is one of the main treatments for cystic fibrosis (CF)." | ( Jullien, V; Launay, M; Magréault, S; Roy, C; Sermet-Gaudelus, I, 2021) |
| "An attractive approach to treat people with Cystic Fibrosis (CF), a life-shortening disease caused by mutant CFTR, is to compensate for the absence of this chloride/bicarbonate channel by activating alternative (non-CFTR) chloride channels." | ( Amaral, MD; Botelho, HM; Kunzelmann, K; Neumann, B; Pepperkok, R; Pinto, MC; Railean, V; Schreiber, R; Silva, IAL, 2022) |
| "A new treatment for cystic fibrosis combining 3 CFTR modulators-elexacaftor (ELX), tezacaftor (TEZ), and ivacaftor (IVA)-has recently been approved for cystic fibrosis treatment." | ( Burgel, PR; Carlotti, A; Dupin, N; Franck, N; Honoré, I; Kanaan, R; Lheure, C; Martin, C; Okroglic, L; Oulès, B; Sohier, P, 2023) |
| "A new treatment for cystic fibrosis combining 3 CFTR modulators-elexacaftor (ELX), tezacaftor (TEZ), and ivacaftor (IVA)-has recently been approved for cystic fibrosis treatment." | ( Burgel, PR; Carlotti, A; Dupin, N; Franck, N; Honoré, I; Kanaan, R; Lheure, C; Martin, C; Okroglic, L; Oulès, B; Sohier, P, 2023) |