iodine has been researched along with Cystic Fibrosis in 1143 studies
Iodine: A nonmetallic element of the halogen group that is represented by the atomic symbol I, atomic number 53, and atomic weight of 126.90. It is a nutritionally essential element, especially important in thyroid hormone synthesis. In solution, it has anti-infective properties and is used topically.
diiodine : Molecule comprising two covalently bonded iodine atoms with overall zero charge..
Cystic Fibrosis: 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.
| Excerpt | Relevance | Reference |
|---|---|---|
| "We provide evidence that combination lumacaftor and ivacaftor improves FEV1 for patients with cystic fibrosis who are homozygous for phe508del CFTR, with a modest effect on sweat chloride concentration." | 9.19 | 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. ( Bell, SC; Boyle, MP; Huang, X; Konstan, MW; McColley, SA; Patel, NR; Rietschel, E; Rodman, D; Rowe, SM; Waltz, D, 2014) |
| "We randomly assigned 39 adults with cystic fibrosis and at least one G551D-CFTR allele to receive oral VX-770 every 12 hours at a dose of 25, 75, or 150 mg or placebo for 14 days (in part 1 of the study) or VX-770 every 12 hours at a dose of 150 or 250 mg or placebo for 28 days (in part 2 of the study)." | 9.14 | Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. ( 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) |
| "Twenty-one children receiving topiramate and 20 healthy controls with no signs or symptoms of pulmonary or gastrointestinal disease and a negative family history for cystic fibrosis (CF) underwent bilateral pilocarpine iontophoresis and sweat collection via Macroduct® system." | 7.78 | Elevated sweat chloride concentration in children without cystic fibrosis who are receiving topiramate therapy. ( Guglani, L; Kurland, G; Lower, D; Sitwat, B; Weiner, DJ, 2012) |
| "To examine immunoreactive trypsinogen (IRT)-based screening for cystic fibrosis (CF) for recall rate, genotype distribution, and "borderline" sweat test results." | 7.73 | Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes. ( Accurso, FJ; Hammond, KB; Sontag, MK; Wagener, JS; Zielenski, J, 2005) |
| "To quantitate the proportion of infants identified through cystic fibrosis (CF) newborn screening (NBS) by an immunoreactive trypsinogen (IRT)/DNA screening algorithm who have an unclear diagnosis as defined by the findings of an elevated IRT level and either 1) 2 CF gene (CFTR) mutations detected and sweat chloride level <60 mEq/L; or 2) 0 or 1 CFTR mutations and a "borderline" sweat chloride level >or=30 and <60 mEq/L." | 7.73 | Diagnostic dilemmas resulting from the immunoreactive trypsinogen/DNA cystic fibrosis newborn screening algorithm. ( Comeau, AM; Parad, RB, 2005) |
| " Chest computed tomography showed the diffuse bronchiectasis in both lungs, and their diagnosis was confirmed by the repeated analysis of a quantitative pilocarpine iontophoresis test (QPIT)." | 7.73 | Cystic fibrosis in Korean children:a case report identified by a quantitative pilocarpine iontophoresis sweat test and genetic analysis. ( Ahn, KM; Kang, IJ; Kim, JH; Lee, JH; Lee, MG; Lee, SI; Park, HY, 2005) |
| "To characterize the time course and physiologic significance of decline in serum immunoreactive trypsinogen (IRT) levels in infants with cystic fibrosis (CF) by mode of diagnosis and genotype, and to examine IRT heritability." | 7.73 | Genetic and physiologic correlates of longitudinal immunoreactive trypsinogen decline in infants with cystic fibrosis identified through newborn screening. ( Accurso, FJ; Corey, M; Hokanson, JE; Marshall, JA; Sommer, SS; Sontag, MK; Zerbe, GO, 2006) |
| "Statewide screening for cystic fibrosis based on measurements of immunoreactive trypsinogen in dried blood spots is feasible and can be implemented with acceptable rates of repeat testing and false positive and false negative results." | 7.68 | Efficacy of statewide neonatal screening for cystic fibrosis by assay of trypsinogen concentrations. ( Abman, SH; Accurso, FJ; Hammond, KB; Sokol, RJ, 1991) |
| "A three day loading with sodium bromide was performed in 19 healthy controls, 16 definite cystic fibrosis heterozygotes, and 14 homozygote patients with cystic fibrosis." | 7.67 | Detection of cystic fibrosis heterozygotes using a modified loading with bromide. ( Gressmann, HW; Theile, H; Winiecki, P, 1985) |
| "Cystic fibrosis is the most common life-limiting genetic condition in Caucasians caused by Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene mutations." | 5.48 | Effect of topiramate on sweat chloride level while screening for cystic fibrosis. ( Graff, G; Siddaiah, R; Thau, E, 2018) |
| " Methods An open-label, prospective pharmacokinetic study was performed." | 5.46 | Individual pharmacokinetic variation leads to underdosing of ciprofloxacin in some cystic fibrosis patients. ( Buchard, A; Dalbøge, CS; Dalhoff, K; Duno, M; Høiby, N; Johansen, HK; Nielsen, XC; Pressler, T; Schultz, AN; Wang, H, 2017) |
| "Cystic fibrosis is a common disease which has an associated characteristic symptom of high sweat chloride content." | 5.39 | Electrochemical detection of chloride levels in sweat using silver nanoparticles: a basis for the preliminary screening for cystic fibrosis. ( Batchelor-McAuley, C; Compton, RG; Toh, HS; Tschulik, K, 2013) |
| "Although ataluren did not improve lung function in the overall population of nonsense-mutation cystic fibrosis patients who received this treatment, it might be beneficial for patients not taking chronic inhaled tobramycin." | 5.19 | Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial. ( Accurso, FJ; Ajayi, T; Barth, J; Branstrom, A; Bronsveld, I; De Boeck, K; Elborn, JS; Elfring, GL; Fajac, I; Kerem, E; Knoop, C; Konstan, MW; Malfroot, A; McColley, SA; Melotti, P; Peltz, SW; Quattrucci, S; Rietschel, E; Rosenbluth, DB; Rowe, SM; Sermet-Gaudelus, I; Spiegel, RJ; Walker, PA; Welch, EM; Wilschanski, M; Zeitlin, PL, 2014) |
| "We provide evidence that combination lumacaftor and ivacaftor improves FEV1 for patients with cystic fibrosis who are homozygous for phe508del CFTR, with a modest effect on sweat chloride concentration." | 5.19 | 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. ( Bell, SC; Boyle, MP; Huang, X; Konstan, MW; McColley, SA; Patel, NR; Rietschel, E; Rodman, D; Rowe, SM; Waltz, D, 2014) |
| "Collection of sweat via pilocarpine iontophoresis is commonly used to diagnose cystic fibrosis (CF), with thousands of tests performed each day." | 5.17 | Skin pretreatment with microneedles prior to pilocarpine iontophoresis increases sweat production. ( Buono, MJ; Prausnitz, MR; Wing, D, 2013) |
| "Ivacaftor (VX-770) is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that was approved in the United States for the treatment of cystic fibrosis (CF) in patients ≥ 6 years of age who have a G551D mutation; however, the most prevalent disease-causing CFTR mutation, F508del, causes a different functional defect." | 5.16 | Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation. ( Borowitz, DS; Flume, PA; Geller, DE; Li, H; Liou, TG; Ordoñez, CL; Yen, K, 2012) |
| "We randomly assigned 39 adults with cystic fibrosis and at least one G551D-CFTR allele to receive oral VX-770 every 12 hours at a dose of 25, 75, or 150 mg or placebo for 14 days (in part 1 of the study) or VX-770 every 12 hours at a dose of 150 or 250 mg or placebo for 28 days (in part 2 of the study)." | 5.14 | Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. ( 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) |
| " The sweat obtained from patients with cystic fibrosis has been compared with that from normal children: the concentrations of cyclic AMP and cyclic GMP are the same." | 5.05 | Is salt reabsorption in the human sweat duct subject to control? ( Schwarz, V; Simpson, IM, 1985) |
| "The administration of pharmacologic quantities of iodine such as iodides for the treatment of pulmonary disease, organic iodine present in medications and x-ray contrast dyes, and the ingestion of iodine-rich natural foods, may result in goiter, hypothyroidism, or hyperthyroidism, especially in patients with underlying thyroid disease." | 4.75 | Adverse effects of iodides on thyroid function. ( Braverman, LE; Vagenakis, AG, 1975) |
| "We examined the relation between the number of (TG) repeats at the (IVS8)-(TG)m(T)5 locus of the CFTR gene with neonatal serum immunoreactive trypsinogen (IRT) and sweat chloride (SC) concentrations in hypertrypsinogenemic infants with genotype ΔF508-9T/5T identified by California cystic fibrosis newborn screening." | 3.78 | Impact of IVS8-(TG)m(T)n on IRT and sweat chloride levels in newborns identified by California CF newborn screening. ( Keiles, S; Kharrazi, M; Koepke, R; Parad, R, 2012) |
| "Twenty-one children receiving topiramate and 20 healthy controls with no signs or symptoms of pulmonary or gastrointestinal disease and a negative family history for cystic fibrosis (CF) underwent bilateral pilocarpine iontophoresis and sweat collection via Macroduct® system." | 3.78 | Elevated sweat chloride concentration in children without cystic fibrosis who are receiving topiramate therapy. ( Guglani, L; Kurland, G; Lower, D; Sitwat, B; Weiner, DJ, 2012) |
| " The United States CF Foundation recently proposed the term cystic fibrosis transmembrane conductance regulator related metabolic syndrome (CRMS) to describe infants with elevated immunoreactive trypsinogen (IRT) on NBS who do not meet diagnostic criteria for CF." | 3.77 | Clinical outcomes in infants with cystic fibrosis transmembrane conductance regulator (CFTR) related metabolic syndrome. ( Desai, H; Dixon, M; Platt, M; Ren, CL, 2011) |
| "Newborn screening (NBS) for cystic fibrosis (CF) offers the opportunity for early diagnosis and improved outcomes in patients with CF and has been universally available in the state of Massachusetts since 1999 using an immunoreactive trypsinogen (IRT)-DNA algorithm." | 3.76 | Cystic fibrosis newborn screening: using experience to optimize the screening algorithm. ( Comeau, AM; Dorkin, HL; Gerstle, R; Hale, JE; Lapey, A; O'Sullivan, BP; Parad, RB; Spencer, T; Yee, W, 2010) |
| "To examine immunoreactive trypsinogen (IRT)-based screening for cystic fibrosis (CF) for recall rate, genotype distribution, and "borderline" sweat test results." | 3.73 | Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes. ( Accurso, FJ; Hammond, KB; Sontag, MK; Wagener, JS; Zielenski, J, 2005) |
| "To characterize the time course and physiologic significance of decline in serum immunoreactive trypsinogen (IRT) levels in infants with cystic fibrosis (CF) by mode of diagnosis and genotype, and to examine IRT heritability." | 3.73 | Genetic and physiologic correlates of longitudinal immunoreactive trypsinogen decline in infants with cystic fibrosis identified through newborn screening. ( Accurso, FJ; Corey, M; Hokanson, JE; Marshall, JA; Sommer, SS; Sontag, MK; Zerbe, GO, 2006) |
| " Chest computed tomography showed the diffuse bronchiectasis in both lungs, and their diagnosis was confirmed by the repeated analysis of a quantitative pilocarpine iontophoresis test (QPIT)." | 3.73 | Cystic fibrosis in Korean children:a case report identified by a quantitative pilocarpine iontophoresis sweat test and genetic analysis. ( Ahn, KM; Kang, IJ; Kim, JH; Lee, JH; Lee, MG; Lee, SI; Park, HY, 2005) |
| "Describe and define limitations of early pilocarpine iontophoresis (sweat testing) for cystic fibrosis (CF) newborn screening (NBS)." | 3.73 | Sweat testing infants detected by cystic fibrosis newborn screening. ( Comeau, AM; Dorkin, HL; Dovey, M; Gerstle, R; Martin, T; O'Sullivan, BP; Parad, RB, 2005) |
| "To quantitate the proportion of infants identified through cystic fibrosis (CF) newborn screening (NBS) by an immunoreactive trypsinogen (IRT)/DNA screening algorithm who have an unclear diagnosis as defined by the findings of an elevated IRT level and either 1) 2 CF gene (CFTR) mutations detected and sweat chloride level <60 mEq/L; or 2) 0 or 1 CFTR mutations and a "borderline" sweat chloride level >or=30 and <60 mEq/L." | 3.73 | Diagnostic dilemmas resulting from the immunoreactive trypsinogen/DNA cystic fibrosis newborn screening algorithm. ( Comeau, AM; Parad, RB, 2005) |
| "We tested 39 patients with idiopathic chronic pancreatitis (mean age at diagnosis, 33 years) for common mutations of CFTR and of genes encoding a trypsin inhibitor (PSTI) and trypsinogen (PRSS1)." | 3.71 | Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations. ( Cohn, JA; Jowell, PS; Knowles, MR; Noone, PG; Silverman, LM; Zhou, Z, 2001) |
| "To assess the performance of a two tier neonatal screening programme (IRT/DNA/IRT) for cystic fibrosis, based on immunoreactive trypsinogen (IRT) followed by direct cystic fibrosis transmembrane conductance regulator (CFTR) gene analysis (based on a panel of up to 31 mutations) in hypertrypsinaemic newborn infants and to compare it with a previous screening protocol." | 3.71 | Screening for cystic fibrosis in newborn infants: results of a pilot programme based on a two tier protocol (IRT/DNA/IRT) in the Italian population. ( Ambrosioni, A; Bassotti, A; Corbetta, C; Giunta, A; Padoan, R; Seia, M, 2002) |
| "Statewide screening for cystic fibrosis based on measurements of immunoreactive trypsinogen in dried blood spots is feasible and can be implemented with acceptable rates of repeat testing and false positive and false negative results." | 3.68 | Efficacy of statewide neonatal screening for cystic fibrosis by assay of trypsinogen concentrations. ( Abman, SH; Accurso, FJ; Hammond, KB; Sokol, RJ, 1991) |
| "Two methods for the determination of sodium in the sweat after stimulation by pilocarpine-iontophoresis were compared in 227 patients, among them 27 with a known cystic fibrosis." | 3.67 | [Diagnosis of mucoviscidosis by examination of sweat: value of conductometry]. ( Bergmann, S; Hoffmann, C; Sprössig, C; Wunderlich, P, 1989) |
| "Because patients with cystic fibrosis (CF) consistently lack sweating response to isoproterenol (ISO) in vivo and in vitro, we studied to what extent beta-adrenergic defect is expressed in CF heterozygotes." | 3.67 | Variable reduction in beta-adrenergic sweat secretion in cystic fibrosis heterozygotes. ( Sato, F; Sato, K, 1988) |
| "A four year regional screening programme to detect cystic fibrosis using measurement of immunoreactive trypsinogen is described." | 3.67 | Screening for cystic fibrosis: a four year regional experience. ( Black, A; Redmond, A; Roberts, G; Stanfield, M, 1988) |
| "Sweat tests were carried out on 14 patients with cystic fibrosis and 14 controls when on no antibiotics and when taking oral flucloxacillin." | 3.67 | Sweat tests and flucloxacillin. ( Green, A; Griffiths, PD; Weller, PH; Williams, J, 1988) |
| "A three day loading with sodium bromide was performed in 19 healthy controls, 16 definite cystic fibrosis heterozygotes, and 14 homozygote patients with cystic fibrosis." | 3.67 | Detection of cystic fibrosis heterozygotes using a modified loading with bromide. ( Gressmann, HW; Theile, H; Winiecki, P, 1985) |
| "In order to study the disposition which is thought to be latent in chronic pancreatitis, we investigated the sweat chloride concentration of 95 normal subjects, 43 cases of chronic pancreatitis, 12 cases of cholelithiasis, 15 cases of peptic ulcers, 16 cases of hepatic diseases and 23 cases of diabetes mellitus with the sweat test, using the method of pilocarpine iontophoresis." | 3.66 | The significance of the sweat test in chronic pancreatitis. ( Endo, K; Hanawa, M; Koizumi, M; Takahashi, S; Takebe, T, 1978) |
| " Observed safety results support further evaluation of GLPG2222, including in combination with other CFTR modulators." | 2.90 | CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials. ( Barry, PJ; Bell, SC; Bellaire, S; Conrath, K; De Boeck, K; de Kock, H; Drevinek, P; Elborn, JS; Geller, DE; Kanters, D; Minić, P; Muller, K; Plant, BJ; Van Braeckel, E; Van de Steen, O; van der Ent, K; Verhulst, S, 2019) |
| "Cystic fibrosis is a severe monogenic disease that affects around 7 300 patients in France." | 2.82 | [Therapeutic advances in cystic fibrosis: from genetics to treatment personalized]. ( Durieu, I; Durupt, S; Reynaud, Q, 2022) |
| " Results of ivacaftor pharmacokinetics suggested that exposure was similar to that reported in adults (median Cmin were 536 ng/mL for the 50 mg dose; 580 ng/mL for the 75 mg dose; median ivacaftor AUC values were 9840 ng × h/mL and 10 200 ng × h/mL, respectively)." | 2.82 | 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. ( Cooke, J; Cunningham, S; Davies, JC; Green, Y; Harris, WT; Lapey, A; Regelmann, WE; Robertson, S; Rosenfeld, M; Sawicki, GS; Southern, KW, 2016) |
| " Efficacy was determined using nasal transepithelial potential difference and sweat chloride measurements prior to dosing and at 1, 2, and 4 hr postdose." | 2.70 | A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis. ( Ahrens, RC; Aitken, ML; Hilliard, KA; Kelley, TJ; Konstan, MW; Launspach, J; McCarty, NA; Milgram, LJ; Regelmann, WE; Standaert, TA; Teresi, M; Tuthill, C; Weatherly, MR, 2002) |
| "Biomarkers in cystic fibrosis are used i." | 2.52 | Biomarkers in Paediatric Cystic Fibrosis Lung Disease. ( Ramsey, KA; Schultz, A; Stick, SM, 2015) |
| "Cystic fibrosis is responsible for severe chronic pulmonary disorders in children." | 2.50 | Cystic fibrosis: need for mass deployable screening methods. ( Agarwal, A; Sengar, AS; Singh, MK, 2014) |
| "Cystic fibrosis is the most common lethal genetic disease in Caucasians, manifesting as progressive lung dysfunction, pancreatic insufficiency, and intestinal disease." | 2.44 | Cystic fibrosis: newborn screening in America. ( Kleven, DT; McCudden, CR; Willis, MS, 2008) |
| "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." | 2.41 | [From gene to disease; from defective chloride ion transport to cystic fibrosis]. ( Scheffer, H; van den Ouweland, AM; Veeze, HJ, 2001) |
| "Exercise-related hyponatremia is an infrequent but potentially life-threatening accompaniment of prolonged exercise." | 2.41 | Hyponatremia associated with exercise: risk factors and pathogenesis. ( Montain, SJ; Sawka, MN; Wenger, CB, 2001) |
| "Cystic fibrosis is a genetic recessive disorder caused by mutations in the gene that encodes the CFTR protein." | 2.41 | [Diagnosis of cystic fibrosis in adults]. ( Chinet, T; Fajac, I; Ferec, C; Garcia Carmona, T; Nguyen-Khoa, T, 2000) |
| "Cystic fibrosis is a genetic disease occurring more frequently in Caucasians." | 2.41 | [Diagnosis and management of cystic fibrosis in children]. ( Clement, A; Fauroux, B; Tamalet, A, 2000) |
| "In young adults with acute pancreatitis a wide etiologic spectrum has to be considered." | 2.38 | Relapsing pancreatitis as initial manifestation of cystic fibrosis in a young man without pulmonary disease. ( Denzel, K; Gerok, W; Gross, V; Schoelmerich, J, 1989) |
| "Sinusitis is almost universal, and nasal polyposis is frequently present." | 2.37 | Diagnosis and treatment of cystic fibrosis. An update. ( Davis, PB; di Sant'Agnese, PA, 1984) |
| "Pilocarpine was used at a concentration ∼130-times lower than that used in the classical Gibson and Cooke sweat test." | 1.72 | Needle-free iontophoresis-driven β-adrenergic sweat rate test. ( Frédérick, R; Gohy, S; Leal, T; Lebecque, P; Mottais, A; Reynaerts, A; Vanbever, R; Vermeulen, F, 2022) |
| "Cystic fibrosis is the most common metabolic chronic disease among European Caucasian children." | 1.51 | The Incidence of Cystic Fibrosis in the Central Region of Anatolia in Turkey Between 2015 and 2016 ( 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) |
| "Cystic fibrosis is the most common life-limiting genetic condition in Caucasians caused by Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene mutations." | 1.48 | Effect of topiramate on sweat chloride level while screening for cystic fibrosis. ( Graff, G; Siddaiah, R; Thau, E, 2018) |
| " Methods An open-label, prospective pharmacokinetic study was performed." | 1.46 | Individual pharmacokinetic variation leads to underdosing of ciprofloxacin in some cystic fibrosis patients. ( Buchard, A; Dalbøge, CS; Dalhoff, K; Duno, M; Høiby, N; Johansen, HK; Nielsen, XC; Pressler, T; Schultz, AN; Wang, H, 2017) |
| "AH1N1 virus infections caused classic influenza symptoms in the majority of cystic fibrosis patients while others experienced severe outcomes." | 1.46 | Incidental late diagnosis of cystic fibrosis following AH1N1 influenza virus pneumonia: a case report. ( Bianco, A; Calabrese, C; Carnovale, V; Ferrara, N; Iacotucci, P; Iadevaia, C; Mazzarella, G; Perrotta, F; Rea, G, 2017) |
| "Cases of bronchial asthma, diagnosed by GINA guideline 2008, and age matched healthy controls were included." | 1.42 | Higher sweat chloride levels in patients with asthma: a case-control study. ( Awasthi, S; Dixit, P; Maurya, N, 2015) |
| "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." | 1.40 | Lack of harmonization in sweat testing for cystic fibrosis - a national survey. ( Christiansen, AL; Nybo, M, 2014) |
| "To determine if oral dosing with the CFTR-potentiator ivacaftor (VX-770, Kalydeco) improves CFTR-dependent sweating in CF subjects carrying G551D or R117H-5T mutations, we optically measured sweat secretion from 32-143 individually identified glands in each of 8 CF subjects; 6 F508del/G551D, one G551D/R117H-5T, and one I507del/R117H-5T." | 1.40 | A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor. ( Char, JE; Cho, HJ; Davies, Z; Dunn, C; Frisbee, E; Jeong, JH; Milla, C; Moss, RB; Park, IH; Thomas, EA; Wine, JJ; Wolfe, MH, 2014) |
| "Exercise-associated hyponatremia is known to be a complex process resulting from the interplay of hydration, arginine vasopressin, and sodium balance." | 1.40 | The need for salt: does a relationship exist between cystic fibrosis and exercise-associated hyponatremia? ( Hew-Butler, TD; Hoffman, MD; Lewis, DP; Owen, BE; Rogers, IR; Stuempfle, KJ; Verbalis, JG, 2014) |
| "Sensitivity and specificity were calculated as well as the means and coefficient of variation by test and by extremity." | 1.40 | A new method of sweat testing: the CF Quantum®sweat test. ( Eickhoff, J; Makholm, L; Rock, MJ, 2014) |
| " A clinical trial performed to support ivacaftor dose selection demonstrated a dose-response relationship between improvement in FEV(1) and decrease in sweat chloride, a measure of CFTR function." | 1.39 | Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience. ( Chowdhury, BA; Durmowicz, AG; Rosebraugh, CJ; Witzmann, KA, 2013) |
| "Aquagenic palmoplantar keratoderma (APK) is a cutaneous phenomenon marked by the formation of edematous, translucent papules and plaques on the palms after water immersion." | 1.39 | [Aquagenic palmoplantar keratoderma in children with cystic fibrosis]. ( Chaillou, E; Chevalier, MC; Chiffoleau, M; Darviot, E; Giniès, JL; Martin, L; Pelatan, C; Troussier, F; Weil, B, 2013) |
| "Cystic fibrosis is a common disease which has an associated characteristic symptom of high sweat chloride content." | 1.39 | Electrochemical detection of chloride levels in sweat using silver nanoparticles: a basis for the preliminary screening for cystic fibrosis. ( Batchelor-McAuley, C; Compton, RG; Toh, HS; Tschulik, K, 2013) |
| "Cystic fibrosis is a chronic progressive autosomal recessive disorder caused by the CFTR gene mutations." | 1.35 | [Standardized sweat chloride analysis for the diagnosis of cystic fibrosis in Korea]. ( Ahn, KM; Cha, SI; Ki, CS; Kim, JH; Kim, SJ; Lee, M; Park, HY, 2008) |
| "Cystic fibrosis is a lethal autosomal recessive disorder usually associated with lung disease, pancreatic insufficiency and high sweat chloride levels." | 1.35 | Clinical and molecular characterization of S1118F-CFTR. ( Conoley, VG; Frederick, CA; Kappes, J; Li, C; Naren, AP; Nekkalapu, S; Penmatsa, H; Stokes, DC; Zhang, W, 2009) |
| "Cystic fibrosis is the most common, multiorgan inherited autosomal recessive disorder." | 1.35 | [Ascites and extreme lipid abnormalities as initial symptoms of cystic fibrosis in a 5-years-old girl--case report]. ( Iwańczak, B; Iwańczak, F; Pytrus, T; Ryzko, J; Smigiel, R; Socha, P, 2009) |
| "In sweat of 11 patients with G6PD deficiency we did not find any abnormality." | 1.35 | Sweat test in patients with glucose-6-phosphate-1-dehydrogenase deficiency. ( Barben, J; Casaulta, C; Schoeni, MH; Stirnimann, A, 2008) |
| "Cystic fibrosis is usually diagnosed during the first years of life." | 1.35 | [The diagnosis of cystic fibrosis in adults: lessons from a family story]. ( Bienvenu, T; Burgel, PR; Coman, T; Desmazes-Dufeu, N; Dusser, D; Fajac, I; Hubert, D; Kanaan, R, 2009) |
| " From a panel of short chain fatty acid derivatives, we showed that 2,2-dimethyl-butyrate (ST20) and alpha-methylhydrocinnamic acid (ST7), exhibiting high oral bioavailability and sustained plasma levels, correct the DeltaF508-CFTR defect." | 1.33 | Novel short chain fatty acids restore chloride secretion in cystic fibrosis. ( Kim, US; Nguyen, TD; Perrine, SP, 2006) |
| "Cystic fibrosis is the most common autosomal recessive genetic defect of one gene CFTR, where a variety of mutations were revealed." | 1.33 | [Genotype and phenotype of gastrointestinal symptoms analysis in children with cystic fibrosis]. ( Iwańczak, F; Mowszet, K; Pawłowicz, J; Sasiadek, M; Smigiel, R; Stawarski, A; Stembalska, A, 2005) |
| "Failure to thrive was was defined by either a weight below the 5th precentile or crossing of 2 major precentiles in 1 year." | 1.32 | Diagnosing cystic fibrosis--asthma and failure to thrive as indications for a sweat test. ( Bar-Zohar, D; Belson, A; Reif, S; Segal-Algranati, D, 2004) |
| "Primary sclerosing cholangitis (PSC) and cystic fibrosis (CF) are both slowly progressive cholestatic liver diseases characterized by fibro-obliterative inflammation of the biliary tract." | 1.32 | Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis. ( Bishop, MD; Chopra, S; Durie, PR; Freedman, SD; Malmberg, E; Regan, MM; Ricci, R; Shea, JC; Sheth, S; Tsui, LC; Walker, C; Zielenski, J, 2003) |
| "This study was to ascertain the reliability of sweat-testing by the Macroduct collection method combined with conductivity analysis (MCS) compared with the Gibson and Cooke technique (GCT)." | 1.31 | Reliability of sweat-testing by the Macroduct collection method combined with conductivity analysis in comparison with the classic Gibson and Cooke technique. ( Borruso, A; Di Cesare, G; Mastella, G; Menin, L; Zanolla, L, 2000) |
| "Chronic rhinosinusitis is a consistent feature of the autosomal recessive disorder cystic fibrosis (CF)." | 1.31 | Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population. ( Cutting, GR; Kim, J; Leopold, DA; Moylan, B; Proud, D; Rubenstein, RC; Togias, A; Wang, X; Zeitlin, PL, 2000) |
| "Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene." | 1.31 | Lung disease associated with the IVS8 5T allele of the CFTR gene. ( Friedman, KJ; Ganeshananthan, M; Knowles, MR; Noone, PG; Pue, CA; Silverman, LM; Simon, RH; Wakeling, EL; Zhou, Z, 2000) |
| "Nonclassic forms of cystic fibrosis have been associated with mutations that reduce but do not eliminate the function of the CFTR protein." | 1.31 | Variant cystic fibrosis phenotypes in the absence of CFTR mutations. ( Cutting, GR; Groman, JD; Meyer, ME; Wilmott, RW; Zeitlin, PL, 2002) |
| "We found no patients with severe acute pancreatitis who had CFTR gene mutations, suggesting that these alterations are not a risk factor for the disease in our population." | 1.31 | CFTR gene mutations in patients suffering from acute pancreatitis. ( Kostuch, M; Kulczycki, L; Rudzki, S; Semczuk, A, 2002) |
| " Age-corrected values for sweat electrolytes, rate of progression of lung disease as assessed by Brasfield chest x-ray scores, and severity of pancreatic insufficiency as assessed by daily supplemented enzyme dosage were obtained for 55, 59, and 59 patients, respectively." | 1.28 | Correlation of phenotypic and genetic heterogeneity in cystic fibrosis: variability in sweat electrolyte levels contributes to heterogeneity and is increased with the XV-2c/KM19 B haplotype. ( Erickson, RP; Farber, R; Howatt, WF; Ober, C; Witt, M, 1991) |
| "Adults with cystic fibrosis have significantly increased sweat electrolyte concentrations (90-120 mmol/l)." | 1.28 | [Reference values of Na(+) and Cl(-) concentrations in adult sweat]. ( Ferner, S; Heilmann, W; Koszmagk, R; Lehmann, A, 1990) |
| "These findings suggested Bartter's syndrome." | 1.27 | Cystic fibrosis mimicking Bartter's syndrome. ( Davison, AG; Snodgrass, GJ, 1983) |
| "A higher incidence of premature labor is noted and all patients are best managed in tertiary referral centers." | 1.27 | Diagnosis of maternal cystic fibrosis during pregnancy. ( Hanson, R; Johnson, SR; Varner, MW; Yates, SJ, 1983) |
| "Pancreatitis is a known complication of cystic fibrosis and may occur at any time in the course of the disease; in rare instances, pancreatitis is the first presenting manifestation." | 1.27 | Pancreatitis as initial presentation of cystic fibrosis in young adults. A report of two cases. ( Achkar, E; Masaryk, TJ, 1983) |
| "This case of Munchausen syndrome by proxy highlights the extent to which the diagnosis of cystic fibrosis rests on reliable history and laboratory data and emphasizes the extremes to which perpetrators of this form of child abuse may go to make their case." | 1.27 | Munchausen syndrome by proxy simulating cystic fibrosis. ( Orenstein, DM; Wasserman, AL, 1986) |
| "Sweat electrolytes were initially elevated in a child who was diagnosed as having celiac disease and also in one with psychosocial failure to thrive." | 1.27 | Abnormal sweat electrolytes in a case of celiac disease and a case of psychosocial failure to thrive. Review of other reported causes. ( Ruddy, RM; Scanlin, TF, 1987) |
| "Patients with cystic fibrosis have characteristic disturbances in the electrolyte composition of their sweat, saliva, and pancreatic secretions." | 1.27 | Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis. ( Bijman, J; Quinton, PM, 1983) |
| "The diagnosis of cystic fibrosis is straight forward if the clinical findings and sweat sodium are considered together." | 1.27 | Is the sweat test infallible in cystic fibrosis? ( Collins, JE; Rolles, CJ, 1985) |
| "No known cases of cystic fibrosis have been missed." | 1.26 | Cystic fibrosis screening in the newborn. ( Elliot, RB; Robinson, PG, 1976) |
| "Cystic fibrosis has been diagnosed during life in three South Indian infants on the basis of characteristic clinical features and a positive sweat test." | 1.26 | Cystic fibrosis in South India. ( Hill, PG; Jadhav, M; Maya, PP; Sudarsanam, D, 1980) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 582 (50.92) | 18.7374 |
| 1990's | 113 (9.89) | 18.2507 |
| 2000's | 172 (15.05) | 29.6817 |
| 2010's | 212 (18.55) | 24.3611 |
| 2020's | 64 (5.60) | 2.80 |
| Authors | Studies |
|---|---|
| Ramsey, B | 1 |
| Bush, A | 7 |
| Naehrlich, L | 4 |
| Dörr, HG | 1 |
| Bagheri-Behrouzi, A | 2 |
| Rauh, M | 1 |
| SILVERMAN, FN | 1 |
| SHIRKEY, HC | 1 |
| REEMTSMA, K | 2 |
| DI SANT'AGNESE, PA | 11 |
| MALM, JR | 1 |
| BARKER, HG | 2 |
| MACDOUGALL, LG | 1 |
| Nguyen, TD | 1 |
| Kim, US | 1 |
| Perrine, SP | 1 |
| Santos, GF | 1 |
| Reenstra, WW | 1 |
| Chao, AC | 1 |
| Zifferblatt, JB | 1 |
| Wagner, JA | 1 |
| Dong, YJ | 1 |
| Gruenert, DC | 1 |
| Gardner, P | 2 |
| Matson, RS | 1 |
| Rampal, JB | 1 |
| Coassin, PJ | 1 |
| Gabriel, SE | 1 |
| Makhlina, M | 1 |
| Martsen, E | 1 |
| Thomas, EJ | 1 |
| Lethem, MI | 1 |
| Boucher, RC | 5 |
| Vagenakis, AG | 1 |
| Braverman, LE | 1 |
| Mason, DK | 1 |
| Harden, RM | 1 |
| Alexander, WD | 1 |
| Reynaerts, A | 2 |
| Vermeulen, F | 5 |
| Mottais, A | 1 |
| Gohy, S | 2 |
| Lebecque, P | 7 |
| Frédérick, R | 1 |
| Vanbever, R | 1 |
| Leal, T | 8 |
| Terlizzi, V | 4 |
| Claut, L | 2 |
| Colombo, C | 4 |
| Tosco, A | 4 |
| Castaldo, A | 2 |
| Fabrizzi, B | 1 |
| Lucarelli, M | 1 |
| Cimino, G | 2 |
| Carducci, C | 1 |
| Dolce, D | 2 |
| Biffi, A | 1 |
| Bonomi, P | 1 |
| Timpano, S | 1 |
| Padoan, R | 9 |
| Maguire, B | 1 |
| Blake, O | 1 |
| Boran, G | 1 |
| Borovickova, I | 1 |
| Abdelfadil, S | 1 |
| Murray, C | 1 |
| Elnazir, B | 1 |
| Linnane, B | 2 |
| Pallenberg, ST | 1 |
| Junge, S | 1 |
| Ringshausen, FC | 1 |
| Sauer-Heilborn, A | 1 |
| Hansen, G | 1 |
| Dittrich, AM | 1 |
| Tümmler, B | 10 |
| Nietert, M | 1 |
| Gonska, T | 12 |
| Keenan, K | 3 |
| Au, J | 1 |
| Dupuis, A | 5 |
| Chilvers, MA | 1 |
| Burgess, C | 1 |
| Bjornson, C | 1 |
| Fairservice, L | 1 |
| Brusky, J | 1 |
| Kherani, T | 1 |
| Jober, A | 1 |
| Kosteniuk, L | 1 |
| Price, A | 1 |
| Itterman, J | 1 |
| Morgan, L | 1 |
| Mateos-Corral, D | 1 |
| Hughes, D | 1 |
| Donnelly, C | 1 |
| Smith, MJ | 2 |
| Iqbal, S | 1 |
| Arpin, J | 1 |
| Reisman, J | 1 |
| Hammel, J | 1 |
| van Wylick, R | 1 |
| Derynck, M | 1 |
| Henderson, N | 1 |
| Solomon, M | 6 |
| Ratjen, F | 2 |
| Woodley, FW | 1 |
| Gecili, E | 1 |
| Szczesniak, RD | 1 |
| Shrestha, CL | 1 |
| Nemastil, CJ | 1 |
| Kopp, BT | 1 |
| Hayes, D | 3 |
| Cirilli, N | 6 |
| Southern, KW | 8 |
| Barben, J | 10 |
| Munck, A | 15 |
| Wilschanski, M | 11 |
| Nguyen-Khoa, T | 8 |
| Aralica, M | 3 |
| Simmonds, NJ | 5 |
| De Wachter, E | 3 |
| Leniček Krleža, J | 1 |
| Tješić-Drinković, D | 1 |
| Crneković, K | 1 |
| Culej, J | 1 |
| Fressl Juroš, G | 1 |
| Horvat, V | 1 |
| Metzner, D | 1 |
| Pamuković Jaram, D | 1 |
| Pipić Kitter, A | 1 |
| Smaić, F | 1 |
| Šimić Vojak, S | 1 |
| Šimičević, L | 1 |
| Verić, V | 1 |
| Gallagher, A | 1 |
| Hooley, K | 1 |
| Costello, S | 1 |
| Felsenstein, S | 1 |
| Mullane, D | 1 |
| Dempsey, E | 1 |
| Ní Chróinín, M | 1 |
| DeRose, L | 1 |
| Kim, J | 2 |
| Farahmand, M | 1 |
| Shinbashi, MY | 1 |
| Joo, NS | 2 |
| Wine, JJ | 4 |
| Mathiaparanam, S | 1 |
| de Macedo, AN | 1 |
| Gill, B | 1 |
| Pedder, L | 1 |
| Hill, S | 1 |
| Britz-McKibbin, P | 1 |
| Hatton, A | 3 |
| Drummond, D | 1 |
| Aoust, L | 1 |
| Schlatter, J | 1 |
| Martin, C | 1 |
| Ramel, S | 1 |
| Kiefer, S | 1 |
| Gachelin, E | 1 |
| Stremler, N | 2 |
| Cosson, L | 1 |
| Gabsi, A | 1 |
| Remus, N | 3 |
| Benhamida, M | 1 |
| Hadchouel, A | 1 |
| Fajac, I | 12 |
| Girodon, E | 10 |
| Sermet-Gaudelus, I | 15 |
| Mayer-Hamblett, N | 7 |
| Zemanick, ET | 4 |
| Odem-Davis, K | 2 |
| VanDevanter, D | 1 |
| Warden, M | 1 |
| Rowe, SM | 19 |
| Young, J | 1 |
| Konstan, MW | 11 |
| Birket, SE | 1 |
| Burat, B | 1 |
| Baiwir, D | 1 |
| Fléron, M | 1 |
| Eppe, G | 1 |
| Mazzucchelli, G | 1 |
| Sahoo, N | 1 |
| Dhochak, N | 1 |
| Jat, KR | 1 |
| Sankar, J | 1 |
| Lodha, R | 3 |
| Sethuraman, G | 2 |
| Kabra, M | 3 |
| Kabra, SK | 4 |
| Solomon, GM | 1 |
| Melotti, P | 6 |
| Graeber, SY | 2 |
| Bronsveld, I | 7 |
| Cutting, GR | 10 |
| Reynaud, Q | 2 |
| Durupt, S | 3 |
| Durieu, I | 5 |
| Salinas, DB | 1 |
| Ginsburg, DK | 1 |
| Wee, CP | 1 |
| Saeed, MM | 1 |
| Brewington, JJ | 1 |
| Bedran, RM | 2 |
| Alvim, CG | 3 |
| Sader, OG | 1 |
| Alves Júnior, JV | 1 |
| Pereira, FH | 1 |
| Nolasco, DM | 1 |
| Zhang, L | 2 |
| Camargos, P | 3 |
| Ha, JH | 1 |
| Jeong, Y | 1 |
| Ahn, J | 1 |
| Hwang, S | 1 |
| Jeon, S | 1 |
| Kim, D | 1 |
| Ko, J | 1 |
| Kang, B | 1 |
| Jung, Y | 1 |
| Choi, J | 2 |
| Han, H | 1 |
| Gu, J | 1 |
| Cho, S | 1 |
| Kim, H | 1 |
| Bok, M | 1 |
| Park, SA | 1 |
| Jeong, JH | 2 |
| Park, I | 1 |
| Alexopoulos, A | 1 |
| Chouliaras, G | 1 |
| Kakourou, T | 1 |
| Dakoutrou, M | 1 |
| Petrocheilou, A | 1 |
| Nasi, L | 1 |
| Thanopoulou, I | 1 |
| Siahanidou, S | 1 |
| Kanaka-Gantenbein, C | 1 |
| Kontara, L | 1 |
| Michos, A | 1 |
| Loukou, I | 1 |
| Nomiyama, S | 1 |
| Ribeiro, JD | 6 |
| Rock, MJ | 10 |
| Baker, M | 2 |
| Farrell, PM | 22 |
| Omosule, CL | 1 |
| Dietzen, DJ | 2 |
| Roper, SM | 1 |
| Taghizadeh-Behbahani, M | 1 |
| Hemmateenejad, B | 1 |
| Shamsipur, M | 1 |
| Tavassoli, A | 1 |
| van Koningsbruggen-Rietschel, S | 2 |
| Conrath, K | 3 |
| Fischer, R | 1 |
| Sutharsan, S | 2 |
| Kempa, A | 1 |
| Gleiber, W | 1 |
| Schwarz, C | 1 |
| Hector, A | 1 |
| Van Osselaer, N | 1 |
| Pano, A | 1 |
| Corveleyn, S | 1 |
| Bwirire, D | 1 |
| Santermans, E | 1 |
| Muller, K | 2 |
| Bellaire, S | 2 |
| Van de Steen, O | 3 |
| Harris, JK | 1 |
| Wagner, BD | 1 |
| Robertson, CE | 1 |
| Stevens, MJ | 1 |
| Heltshe, SL | 7 |
| Sagel, SD | 4 |
| Heijerman, HGM | 2 |
| McKone, EF | 8 |
| Downey, DG | 1 |
| Van Braeckel, E | 3 |
| Tullis, E | 12 |
| Mall, MA | 7 |
| Welter, JJ | 1 |
| Ramsey, BW | 8 |
| McKee, CM | 4 |
| Marigowda, G | 3 |
| Moskowitz, SM | 5 |
| Waltz, D | 6 |
| Sosnay, PR | 4 |
| Simard, C | 4 |
| Ahluwalia, N | 3 |
| Xuan, F | 5 |
| Zhang, Y | 2 |
| Taylor-Cousar, JL | 7 |
| McCoy, KS | 3 |
| Middleton, PG | 4 |
| Dřevínek, P | 3 |
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| Polineni, D | 2 |
| Nair, N | 1 |
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| Jain, R | 1 |
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| Zare, RN | 1 |
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| Secunda, KE | 1 |
| Guimbellot, JS | 1 |
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| Bensliman, S | 1 |
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| van Oirschot-van de Ven, MMM | 1 |
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| van der Ent, CK | 2 |
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| Van Daele, S | 1 |
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| Kruisselbrink, E | 1 |
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| Ren, CL | 5 |
| Sontag, MK | 6 |
| LeGrys, VA | 18 |
| Doull, I | 3 |
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| Hanks, RE | 1 |
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| Vanden Broeck, SMP | 1 |
| Nelson, DJ | 1 |
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| Ren, L | 1 |
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| Shu, X | 1 |
| Lin, W | 1 |
| Yang, P | 1 |
| Chen, J | 2 |
| Teo, KL | 1 |
| Xiao, F | 1 |
| Wang, H | 2 |
| Yao, T | 1 |
| Zhao, X | 1 |
| Yang, X | 1 |
| Yu, DYW | 1 |
| Rogach, AL | 1 |
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| Leitner, W | 1 |
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| Xing, H | 1 |
| Xu, Z | 1 |
| Zhu, X | 1 |
| Yuan, M | 1 |
| Wang, L | 3 |
| Yu, J | 1 |
| Li, Y | 1 |
| Yang, L | 1 |
| Liu, H | 1 |
| Cao, L | 1 |
| Zhang, S | 1 |
| Zhao, D | 1 |
| Yan, T | 1 |
| Yang, G | 1 |
| Lin, Z | 1 |
| Luo, M | 1 |
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| Lee, SW | 1 |
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| Phillips, KS | 1 |
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| He, ZZ | 1 |
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| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| A Phase IIa, Randomized, Double-blind, Placebo-controlled Study to Evaluate GLPG2737 in Orkambi-treated Subjects With Cystic Fibrosis Homozygous for the F508del Mutation[NCT03474042] | Phase 2 | 22 participants (Actual) | Interventional | 2017-11-29 | Completed | ||
| Functional and Phenotypic Characteristics of Blood Neutrophils in Cystic Fibrosis[NCT04970225] | 130 participants (Anticipated) | Interventional | 2021-07-08 | Recruiting | |||
| A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del Mutation (F/F)[NCT03525548] | Phase 3 | 113 participants (Actual) | Interventional | 2018-08-03 | Completed | ||
| TAAI Erasmus Research Initiative to Fight CF: Monitoring Inflammation in CF Lung Disease Into a New Era[NCT05752019] | 100 participants (Anticipated) | Observational | 2022-03-21 | Active, not recruiting | |||
| Symptom Based Performance of Airway Clearance Therapy After Starting Highly Effective CFTR Modulator Therapy for Cystic Fibrosis[NCT05392855] | 30 participants (Anticipated) | Interventional | 2023-09-05 | Recruiting | |||
| A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Minimal Function Mutation (F/MF)[NCT03525444] | Phase 3 | 405 participants (Actual) | Interventional | 2018-06-15 | Completed | ||
| Prospective Study of the Phenotypic Expression of Cystic Fibrosis (CF) Screened Positive Newborns With an Atypical Form of CF (DPAM)[NCT02869932] | 130 participants (Actual) | Interventional | 2012-03-31 | Completed | |||
| A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Gating or Residual Function Mutation (F/G and F/RF [NCT04058353] | Phase 3 | 271 participants (Actual) | Interventional | 2019-08-28 | Completed | ||
| A New Optical Sweat Test Method Based on a Citric Acid-derived Multi-halide Sensor[NCT03367494] | 25 participants (Actual) | Interventional | 2018-03-01 | Terminated (stopped due to Investigator Decision) | |||
| Effect of the CFTR-modulating Triple Therapy Elexacaftor - Tezacaftor - Ivacaftor on Numerical Distribution in Peripheral Mononuclear Immune Cells Derived From Patients With Cystic Fibrosis[NCT05576324] | 130 participants (Anticipated) | Observational | 2020-12-30 | Recruiting | |||
| A Phase 1/2 Study of VX-445 in Healthy Subjects and Subjects With Cystic Fibrosis[NCT03227471] | Phase 1/Phase 2 | 225 participants (Actual) | Interventional | 2017-01-23 | Completed | ||
| A Phase 1, Randomized, Double Blind, Placebo Controlled, Dose Escalation, and Bioavailability Study Evaluating the Safety and Pharmacokinetics of VX-659 in Healthy Subjects and in Subjects With Cystic Fibrosis[NCT03029455] | Phase 1 | 163 participants (Actual) | Interventional | 2016-11-30 | Completed | ||
| A Phase 2, Randomized, Double-blind, Controlled Study to Evaluate the Safety and Efficacy of VX-659 Combination Therapy in Subjects Aged 18 Years and Older With Cystic Fibrosis[NCT03224351] | Phase 2 | 124 participants (Actual) | Interventional | 2017-08-08 | Completed | ||
| Baby Observational and Nutritional Study (BONUS)[NCT01424696] | 231 participants (Actual) | Observational | 2011-12-31 | Completed | |||
| A Phase 3, 2-Arm, Roll-Over Study to Evaluate the Long-term Safety and Pharmacodynamics of Ivacaftor Treatment in Pediatric Subjects With Cystic Fibrosis and a CFTR Gating Mutation[NCT01946412] | Phase 3 | 33 participants (Actual) | Interventional | 2013-12-31 | Completed | ||
| A Phase IIa, Randomized, Double-blind, Placebo-controlled Study to Evaluate Multiple Doses of GLPG2222 in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del Mutation[NCT03119649] | Phase 2 | 59 participants (Actual) | Interventional | 2017-03-18 | Completed | ||
| A Phase IIa, Randomized, Double-blind, Placebo-controlled Study to Evaluate GLPG2222 in Ivacaftor-treated Subjects With Cystic Fibrosis Harbouring One F508del CFTR Mutation and a Second Gating (Class III) Mutation[NCT03045523] | Phase 2 | 37 participants (Actual) | Interventional | 2017-01-31 | Completed | ||
| A Phase IIa, Open-label Study of Multiple Doses of GLPG1837 in Subjects With Cystic Fibrosis and the G551D Mutation[NCT02707562] | Phase 2 | 26 participants (Actual) | Interventional | 2016-02-29 | Completed | ||
| A Phase 3, Open Label Study to Evaluate the Pharmacokinetics, Safety, and Tolerability of VX-661 in Combination With Ivacaftor in Subjects 6 Through 11 Years of Age With Cystic Fibrosis, Homozygous or Heterozygous for the F508del CFTR Mutation[NCT02953314] | Phase 3 | 83 participants (Actual) | Interventional | 2016-11-30 | Completed | ||
| A Phase 2a, Randomized, Double-Blind, Placebo-Controlled Study of VX-770 to Evaluate Safety, Pharmacokinetics, and Biomarkers of CFTR Activity in Cystic Fibrosis (CF) Subjects With Genotype G551D[NCT00457821] | Phase 2 | 39 participants (Actual) | Interventional | 2007-05-31 | Completed | ||
| A Phase 3 Efficacy and Safety Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis[NCT00803205] | Phase 3 | 238 participants (Actual) | Interventional | 2009-09-08 | Completed | ||
| Personalized Therapy of Cystic Fibrosis: Set-up of Response Markers[NCT02965326] | 75 participants (Anticipated) | Interventional | 2016-05-31 | Recruiting | |||
| A Phase 2, Multicenter, Double-Blinded, Placebo-Controlled, Multiple-Dose Study to Evaluate Safety, Tolerability, Efficacy, Pharmacokinetics, and Pharmacodynamics of Lumacaftor Monotherapy, and Lumacaftor and Ivacaftor Combination Therapy in Subjects With[NCT01225211] | Phase 2 | 312 participants (Actual) | Interventional | 2010-10-31 | Completed | ||
| Registry Study on Primary Ciliary Dyskinesia in Chinese children-a Multicenter, Prospective Cohort Study[NCT02704455] | 100 participants (Anticipated) | Observational | 2016-05-31 | Not yet recruiting | |||
| A Phase 3, 2-Part, Open-Label Study to Evaluate the Safety, Pharmacokinetics and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are 2 Through 5 Years of Age and Have a CFTR Gating Mutation[NCT01705145] | Phase 3 | 35 participants (Actual) | Interventional | 2013-01-31 | Completed | ||
| Gene Modifiers of Cystic Fibrosis Lung Disease[NCT00037765] | 600 participants (Anticipated) | Observational | 2001-09-30 | Active, not recruiting | |||
| Short Term Effects of Ivacaftor in Non-G551D Cystic Fibrosis Patients[NCT01784419] | 10 participants (Actual) | Interventional | 2013-10-31 | Completed | |||
| COVID-19 Antibody Responses In Cystic Fibrosis[NCT04904445] | 40 participants (Anticipated) | Observational | 2021-05-21 | Active, not recruiting | |||
| A Phase II Study to Establish the Efficacy of Synthetic Human SecretiN in Human Acute Pancreatitis (SNAP) Study[NCT03686618] | Phase 2 | 40 participants (Anticipated) | Interventional | 2018-10-01 | Recruiting | ||
| Nutritional Impact of Ivacaftor Treatment in 4 Month to 2 Year Old Children With CF Gating Mutations[NCT03783286] | 15 participants (Actual) | Observational | 2019-02-06 | Completed | |||
| Cystic Fibrosis Related Bone Disease: the Role of CFTR[NCT01549314] | 79 participants (Actual) | Observational | 2012-04-30 | Completed | |||
| A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Safety and Efficacy of VX-770 in Subjects Aged 12 Years and Older With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation[NCT00953706] | Phase 2 | 140 participants (Actual) | Interventional | 2009-09-30 | Terminated (stopped due to Following review of results obtained from a pre-specified 6-month analysis of Part B data the study was terminated on the basis of futility.) | ||
| Nutritional Impact of Orkambi Treatment in 2 to 5 Year Old Children Homozygous for F508del Mutations[NCT03795363] | 28 participants (Actual) | Observational | 2019-04-10 | Completed | |||
| Phase II Study: LYM-X-SORB™, an Organized Lipid Matrix: Fatty Acids and Choline in CF[NCT00406536] | Phase 2 | 110 participants (Actual) | Interventional | 2007-01-31 | Completed | ||
| Sustained Impact of Hypertonic Saline on Mucociliary Clearance in Young Children With Cystic Fibrosis[NCT01031706] | 23 participants (Actual) | Interventional | 2009-09-30 | Completed | |||
| Pragmatic Implementation Trial of a CF Primary Palliative Care Intervention[NCT04923880] | 643 participants (Actual) | Interventional | 2021-06-25 | Active, not recruiting | |||
| Characterization of β-cell Function and Insulin Sensitivity in Pre-transplant Patients With Cystic Fibrosis[NCT04379726] | 150 participants (Anticipated) | Observational | 2020-07-01 | Not yet recruiting | |||
| Randomized Cross Over Study of Inhaled Hypertonic Xylitol Versus Hypertonic Saline in Stable Subjects With Cystic Fibrosis[NCT01355796] | Phase 1/Phase 2 | 30 participants (Actual) | Interventional | 2011-05-31 | Completed | ||
| Randomized Cross-over Physiologic Study of High Flow Nasal Oxygen Cannula Versus Non-invasive Ventilation in Cystic Fibrosis. The HIFEN Study[NCT02262871] | 15 participants (Actual) | Interventional | 2014-12-31 | Completed | |||
| A Randomized, Double-Blind, Placebo-Controlled, Multi-Center, Crossover Study to Evaluate the Effectiveness and Safety of PANCRECARB® MS-16 (Pancrelipase) in Reducing Steatorrhea in Children and Adults With Cystic Fibrosis[NCT00432861] | Phase 3 | 29 participants (Actual) | Interventional | 2007-01-31 | Completed | ||
| A Single Arm 48-Week Follow-on Safety Study to the Core Study (A Multicentre, Multinational, Open-Label, Randomised, Parallel Group Clinical Trial of Tobrineb®/Actitob®/Bramitob® (Tobramycin Solution for Nebulisation, 300mg Twice Daily in 4mL Unit Dose Vi[NCT01111383] | Phase 3 | 209 participants (Actual) | Interventional | 2009-09-30 | Completed | ||
| Fluid Balance During Exercise in the Heat With Water, Flavored Placebo, or a Carbohydrate-electrolyte Beverage Intake (The APEX Study)[NCT01893853] | 24 participants (Actual) | Interventional | 2012-10-31 | Completed | |||
| Development a Core Set for Adults With Cystic Fibrosis Based on the International Classification of Functioning, Disability, and Health (ICF)[NCT06128499] | 30 participants (Anticipated) | Observational | 2023-12-20 | Not yet recruiting | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicate fewer symptoms and better health-related quality of life. (NCT03525548)
Timeframe: From Baseline at Week 4
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| TEZ/IVA | -1.4 |
| VX-445/TEZ/IVA TC | 16.0 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03525548)
Timeframe: From Baseline at Week 4
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| TEZ/IVA | 0.4 |
| VX-445/TEZ/IVA TC | 10.4 |
Sweat samples were collected using an approved collection device. (NCT03525548)
Timeframe: From Baseline at Week 4
| Intervention | millimole per liter (mmol/L) (Least Squares Mean) |
|---|---|
| TEZ/IVA | 1.7 |
| VX-445/TEZ/IVA TC | -43.4 |
(NCT03525548)
Timeframe: Day 1 and Week 4
| Intervention | microgram per milliliter (mcg/mL) (Mean) | |||||
|---|---|---|---|---|---|---|
| TEZ: Day 1 | TEZ: Week 4 | M1-TEZ: Day 1 | M1-TEZ: Week 4 | IVA: Day 1 | IVA: Week 4 | |
| TEZ/IVA | 1.71 | 1.48 | 4.84 | 4.86 | 0.652 | 0.530 |
(NCT03525548)
Timeframe: Day 1 and Week 4
| Intervention | microgram per milliliter (mcg/mL) (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| VX-445: Day 1 | VX-445: Week 4 | TEZ: Day 1 | TEZ: Week 4 | M1-TEZ: Day 1 | M1-TEZ: Week 4 | IVA: Day 1 | IVA: Week 4 | |
| VX-445/TEZ/IVA TC | NA | 4.84 | 1.60 | 1.89 | 4.76 | 5.28 | 0.598 | 0.652 |
(NCT03525548)
Timeframe: From first dose of study drug in TC treatment period up to 28 days after last dose of study drug or to the completion of study participation date, whichever occurs first (up to Week 8)
| Intervention | participants (Number) | |
|---|---|---|
| Participants with AEs | Participants with SAEs | |
| TEZ/IVA | 33 | 1 |
| VX-445/TEZ/IVA TC | 32 | 2 |
BMI was defined as weight in kg divided by height in m^2. Z-score is a statistical measure to describe whether a mean was above or below the standard. BMI, adjusted for age and sex, was analyzed as BMI-for-age z-score. A z-score of 0 is equal to the mean and is considered normal. Lower numbers indicate values lower than the mean and higher numbers indicate values higher than the mean. Higher values are indicative of higher BMI. (NCT03525444)
Timeframe: From Baseline at Week 24
| Intervention | z-score (Least Squares Mean) |
|---|---|
| Placebo | 0.04 |
| VX-445/TEZ/IVA TC | 0.34 |
BMI was defined as weight in kilogram (kg) divided by height in square meter (m^2). (NCT03525444)
Timeframe: From Baseline at Week 24
| Intervention | kilogram per meter square (kg/m^2) (Least Squares Mean) |
|---|---|
| Placebo | 0.09 |
| VX-445/TEZ/IVA TC | 1.13 |
(NCT03525444)
Timeframe: From Baseline at Week 24
| Intervention | kg (Least Squares Mean) |
|---|---|
| Placebo | 0.5 |
| VX-445/TEZ/IVA TC | 3.4 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT03525444)
Timeframe: From Baseline at Week 4
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Placebo | -1.9 |
| VX-445/TEZ/IVA TC | 18.1 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT03525444)
Timeframe: From Baseline through Week 24
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Placebo | -2.7 |
| VX-445/TEZ/IVA TC | 17.5 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03525444)
Timeframe: From Baseline at Week 4
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Placebo | -0.2 |
| VX-445/TEZ/IVA TC | 13.6 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03525444)
Timeframe: From Baseline through Week 24
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Placebo | -0.4 |
| VX-445/TEZ/IVA TC | 13.9 |
Sweat samples were collected using an approved collection device. (NCT03525444)
Timeframe: From Baseline at Week 4
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Placebo | 0.1 |
| VX-445/TEZ/IVA TC | -41.2 |
Sweat samples were collected using an approved collection device. (NCT03525444)
Timeframe: From Baseline through Week 24
| Intervention | millimole per liter (mmol/L) (Least Squares Mean) |
|---|---|
| Placebo | -0.4 |
| VX-445/TEZ/IVA TC | -42.2 |
Pulmonary exacerbation was defined as the treatment with new or changed antibiotic therapy (intravenous, inhaled, or oral) for greater than or equal to 4 sinopulmonary signs/symptoms. (NCT03525444)
Timeframe: From Baseline through Week 24
| Intervention | pulmonary exacerbation events (Number) |
|---|---|
| Placebo | 113 |
| VX-445/TEZ/IVA TC | 41 |
Pulmonary exacerbation was defined as the treatment with new or changed antibiotic therapy (intravenous, inhaled, or oral) for greater than or equal to 4 sinopulmonary signs/symptoms. (NCT03525444)
Timeframe: From Baseline through Week 24
| Intervention | days (Median) |
|---|---|
| Placebo | NA |
| VX-445/TEZ/IVA TC | NA |
(NCT03525444)
Timeframe: Pre-dose on Week 4, 8, 12, and 16
| Intervention | microgram per milliliter (mcg/mL) (Mean) | |||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| VX-445 (Week 4) | VX-445 (Week 8) | VX-445 (Week 12) | VX-445 (Week 16) | TEZ (Week 4) | TEZ (Week 8) | TEZ (Week 12) | TEZ (Week 16) | M1-TEZ (Week 4) | M1-TEZ (Week 8) | M1-TEZ (Week 12) | M1-TEZ (Week 16) | IVA (Week 4) | IVA (Week 8) | IVA (Week 12) | IVA (Week 16) | |
| VX-445/TEZ/IVA TC | 5.02 | 4.90 | 4.99 | 4.75 | 2.16 | 2.14 | 2.22 | 2.32 | 5.18 | 5.09 | 5.06 | 5.29 | 0.748 | 0.738 | 0.758 | 0.778 |
(NCT03525444)
Timeframe: From first dose of study drug in TC treatment period up to 28 days after last dose of study drug or to the completion of study participation date, whichever occurs first (up to 28 weeks)
| Intervention | Participants (Count of Participants) | |
|---|---|---|
| Participants with TEAEs | Participants with Serious TEAEs | |
| Placebo | 193 | 42 |
| VX-445/TEZ/IVA TC | 188 | 28 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT04058353)
Timeframe: From Baseline Through Week 8
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Control: IVA or TEZ/IVA | 1.6 |
| TC: ELX/TEZ/IVA | 10.3 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT04058353)
Timeframe: From Baseline Through Week 8
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| TC: ELX/TEZ/IVA | 10.3 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT04058353)
Timeframe: From Baseline Through Week 8
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| TC: ELX/TEZ/IVA | 3.7 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT04058353)
Timeframe: From Baseline Through Week 8
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Control: IVA or TEZ/IVA | 0.2 |
| TC: ELX/TEZ/IVA | 3.7 |
Sweat samples were collected using an approved collection device. (NCT04058353)
Timeframe: From Baseline Through Week 8
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Control: IVA or TEZ/IVA | 0.7 |
| TC: ELX/TEZ/IVA | -22.3 |
Sweat samples were collected using an approved collection device. (NCT04058353)
Timeframe: From Baseline Through Week 8
| Intervention | millimole per Liter (mmol/L) (Least Squares Mean) |
|---|---|
| TC: ELX/TEZ/IVA | -22.3 |
(NCT04058353)
Timeframe: Day 1 up to Week 12
| Intervention | participants (Number) | |
|---|---|---|
| Participants With TEAEs | Participants With SAEs | |
| Control: IVA or TEZ/IVA | 83 | 11 |
| TC: ELX/TEZ/IVA | 88 | 5 |
(NCT03227471)
Timeframe: Cohort A1-5: Pre-dose to 96 hours post-dose; Cohort A7: Pre-dose to 120 hours post-dose
| Intervention | microgram*hour per milliliter (Geometric Mean) |
|---|---|
| Part A: VX-445 (Cohort A1) | 11.4 |
| Part A: VX-445 (Cohort A2) | 30.8 |
| Part A: VX-445 (Cohort A3) | 87.1 |
| Part A: VX-445 (Cohort A4) | 125 |
| Part A: VX-445 (Cohort A5) | 286 |
| Part A: VX-445 (Cohort A7-Fasted) | 21.8 |
| Part A: VX-445 (Cohort A7-Fed) | 55.1 |
| Part A: VX-445 (Cohort A7-IV) | 13.6 |
(NCT03227471)
Timeframe: Cohort A1-A5: Pre-dose to 96 hours post-dose; Cohort A7: Pre-dose to 120 hours post-dose
| Intervention | microgram per milliliter (Geometric Mean) |
|---|---|
| Part A: VX-445 (Cohort A1) | 0.398 |
| Part A: VX-445 (Cohort A2) | 0.989 |
| Part A: VX-445 (Cohort A3) | 2.52 |
| Part A: VX-445 (Cohort A4) | 4.56 |
| Part A: VX-445 (Cohort A5) | 7.07 |
| Part A: VX-445 (Cohort A7-Fasted) | 0.486 |
| Part A: VX-445 (Cohort A7-Fed) | 1.76 |
| Part A: VX-445 (Cohort A7-IV) | 0.740 |
(NCT03227471)
Timeframe: Pre-dose on Day 10
| Intervention | microgram per milliliter (Geometric Mean) |
|---|---|
| Part B: VX-445 (Cohort B1) | 1.05 |
| Part B: VX-445 (Cohort B2) | 2.81 |
| Part B: VX-445 (Cohort B3) | 7.10 |
| Part B: VX-445 (Cohort B4) | 11.5 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Part D: Placebo | 4.2 |
| Part D: VX-445/TEZ/IVA TC - Low Dose | 20.8 |
| Part D: VX-445/TEZ/IVA TC - Medium Dose | 15.4 |
| Part D: VX-445/TEZ/IVA TC - High Dose | 25.7 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Part D: Placebo | 0.0 |
| Part D: VX-445/TEZ/IVA TC - Low Dose | 11.1 |
| Part D: VX-445/TEZ/IVA TC - Medium Dose | 7.9 |
| Part D: VX-445/TEZ/IVA TC - High Dose | 13.8 |
Sweat samples were collected using an approved collection device. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | millimole per liter (mmol/L) (Least Squares Mean) |
|---|---|
| Part D: Placebo | -2.2 |
| Part D: VX-445/TEZ/IVA TC - Low Dose | -38.2 |
| Part D: VX-445/TEZ/IVA TC - Medium Dose | -33.2 |
| Part D: VX-445/TEZ/IVA TC - High Dose | -39.1 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | percent change (Least Squares Mean) |
|---|---|
| Part D: Placebo | 0.3 |
| Part D: VX-445/TEZ/IVA TC - Low Dose | 19.3 |
| Part D: VX-445/TEZ/IVA TC - Medium Dose | 13.8 |
| Part D: VX-445/TEZ/IVA TC - High Dose | 26.2 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Part E: TEZ/IVA | 5.2 |
| Part E: VX-445/TEZ/IVA TC | 20.7 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Part E: TEZ/IVA | 0.4 |
| Part E: VX-445/TEZ/IVA TC | 11.0 |
Sweat samples were collected using an approved collection device. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Part E: TEZ/IVA | 0.8 |
| Part E: VX-445/TEZ/IVA TC | -39.6 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | percent change (Least Squares Mean) |
|---|---|
| Part E: TEZ/IVA | 1.4 |
| Part E: VX-445/TEZ/IVA TC | 19.2 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Part F: Placebo | 20.2 |
| Part F: VX-445/TEZ/VX-561 TC | 20.2 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Part F: Placebo | 1.2 |
| Part F: VX-445/TEZ/VX-561 TC | 11.7 |
Sweat samples were collected using an approved collection device. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Part F: Placebo | 1.0 |
| Part F: VX-445/TEZ/VX-561 TC | -33.6 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03227471)
Timeframe: From Baseline through Day 29
| Intervention | percent change (Least Squares Mean) |
|---|---|
| Part F: Placebo | 1.6 |
| Part F: VX-445/TEZ/VX-561 TC | 19.9 |
(NCT03227471)
Timeframe: Pre-dose to 96 hours post-dose on Day 1 and Day 10
| Intervention | microgram*hour per milliliter (Geometric Mean) | |
|---|---|---|
| Day 1 | Day 10 | |
| Part B: VX-445 (Cohort B1) | 17.9 | 61.4 |
| Part B: VX-445 (Cohort B2) | 42.2 | 183 |
| Part B: VX-445 (Cohort B3) | 57.9 | 452 |
| Part B: VX-445 (Cohort B4) | 119 | 692 |
(NCT03227471)
Timeframe: Pre-dose to 96 hours post-dose on Day 1 and Day 10
| Intervention | microgram per milliliter (Geometric Mean) | |
|---|---|---|
| Day 1 | Day 10 | |
| Part B: VX-445 (Cohort B1) | 1.18 | 2.13 |
| Part B: VX-445 (Cohort B2) | 2.82 | 5.83 |
| Part B: VX-445 (Cohort B3) | 3.47 | 11.4 |
| Part B: VX-445 (Cohort B4) | 9.56 | 18.2 |
(NCT03227471)
Timeframe: Pre-dose to 96 hours post-dose on Day 1, Day 7 and Day 14
| Intervention | nanogram*hour per milliliter (Geometric Mean) | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| IVA: Day 1 | IVA: Day 7 | IVA: Day 14 | M1-IVA: Day 1 | M1-IVA: Day 7 | M1-IVA: Day 14 | M6-IVA: Day 1 | M6-IVA: Day 7 | M6-IVA: Day 14 | |
| Part C: VX-445/TEZ/IVA TC (Cohort C1) | 6350 | 21600 | 27900 | 16900 | 47500 | 75300 | 11100 | 39700 | 66300 |
| Part C: VX-445/TEZ/IVA TC (Cohort C2) | 6210 | 18800 | 35000 | 14800 | 46300 | 97000 | 14100 | 51400 | 123000 |
| Part C: VX-445/TEZ/IVA TC (Cohort C3) | 5480 | 16900 | 32600 | 13300 | 36400 | 86100 | 9050 | 29100 | 67100 |
(NCT03227471)
Timeframe: Pre-dose to 96 hours post-dose on Day 1, Day 7 and Day 14
| Intervention | microgram*hour per milliliter (Geometric Mean) | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| VX-445: Day 1 | VX-445: Day 7 | VX-445: Day 14 | TEZ: Day 1 | TEZ: Day 7 | TEZ: Day 14 | M1-TEZ: Day 1 | M1-TEZ: Day 7 | M1-TEZ: Day 14 | M2-TEZ: Day 1 | M2-TEZ: Day 7 | M2-TEZ: Day 14 | |
| Part C: VX-445/TEZ/IVA TC (Cohort C1) | 48.6 | 156 | 345 | 41.4 | 103 | 252 | 17.7 | 90.9 | 380 | 6.65 | 108 | 617 |
| Part C: VX-445/TEZ/IVA TC (Cohort C2) | 72.5 | 177 | 506 | 43.8 | 105 | 288 | 20.1 | 110 | 477 | 8.19 | 143 | 831 |
| Part C: VX-445/TEZ/IVA TC (Cohort C3) | 26.4 | 65.4 | 183 | 42.7 | 96.9 | 275 | 25.5 | 125 | 512 | 9.00 | 123 | 652 |
(NCT03227471)
Timeframe: Pre-dose to 96 hours post-dose on Day 1, Day 7 and Day 14
| Intervention | nanogram per milliliter (Geometric Mean) | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| IVA: Day 1 | IVA: Day 7 | IVA: Day 14 | M1-IVA: Day 1 | M1-IVA: Day 7 | M1-IVA: Day 14 | M6-IVA: Day 1 | M6-IVA: Day 7 | M6-IVA: Day 14 | |
| Part C: VX-445/TEZ/IVA TC (Cohort C1) | 425 | 1290 | 1360 | 1250 | 2460 | 2680 | 901 | 1960 | 2060 |
| Part C: VX-445/TEZ/IVA TC (Cohort C2) | 461 | 1190 | 1920 | 1190 | 2590 | 3900 | 1180 | 2590 | 3350 |
| Part C: VX-445/TEZ/IVA TC (Cohort C3) | 437 | 1070 | 1410 | 1070 | 2070 | 2990 | 631 | 1400 | 1980 |
(NCT03227471)
Timeframe: Pre-dose to 96 hours post-dose on Day 1, Day 7 and Day 14
| Intervention | microgram per milliliter (Geometric Mean) | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| VX-445: Day 1 | VX-445: Day 7 | VX-445: Day 14 | TEZ: Day 1 | TEZ: Day 7 | TEZ: Day 14 | M1-TEZ: Day 1 | M1-TEZ: Day 7 | M1-TEZ: Day 14 | M2-TEZ: Day 1 | M2-TEZ: Day 7 | M2-TEZ: Day 14 | |
| Part C: VX-445/TEZ/IVA TC (Cohort C1) | 3.04 | 8.29 | 8.71 | 3.81 | 7.09 | 8.11 | 0.929 | 4.34 | 5.77 | 0.553 | 4.72 | 7.69 |
| Part C: VX-445/TEZ/IVA TC (Cohort C2) | 4.86 | 10.3 | 14.0 | 5.67 | 8.29 | 9.07 | 0.988 | 5.19 | 6.79 | 0.667 | 6.41 | 10.1 |
| Part C: VX-445/TEZ/IVA TC (Cohort C3) | 1.72 | 3.58 | 4.73 | 5.38 | 7.01 | 8.60 | 1.30 | 5.86 | 7.54 | 0.716 | 5.46 | 7.93 |
(NCT03227471)
Timeframe: Pre-dose on Day 7 and Day 14
| Intervention | nanogram per milliliter (Geometric Mean) | |||||
|---|---|---|---|---|---|---|
| IVA: Day 7 | IVA: Day 14 | M1-IVA: Day 7 | M1-IVA: Day 14 | M6-IVA: Day 7 | M6-IVA: Day 14 | |
| Part C: VX-445/TEZ/IVA TC (Cohort C1) | 753 | 610 | 1790 | 1600 | 1530 | 1360 |
| Part C: VX-445/TEZ/IVA TC (Cohort C2) | 759 | 990 | 1960 | 2210 | 2260 | 2600 |
| Part C: VX-445/TEZ/IVA TC (Cohort C3) | 604 | 881 | 1400 | 1930 | 1270 | 1640 |
(NCT03227471)
Timeframe: Pre-dose on Day 7 and Day 14
| Intervention | microgram per milliliter (Geometric Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| VX-445: Day 7 | VX-445: Day 14 | TEZ: Day 7 | TEZ: Day 14 | M1-TEZ: Day 7 | M1-TEZ: Day 14 | M2-TEZ: Day 7 | M2-TEZ: Day 14 | |
| Part C: VX-445/TEZ/IVA TC (Cohort C1) | 4.63 | 4.45 | 2.73 | 3.10 | 3.36 | 4.38 | 4.06 | 6.46 |
| Part C: VX-445/TEZ/IVA TC (Cohort C2) | 5.91 | 7.60 | 2.77 | 3.70 | 3.98 | 5.42 | 5.63 | 8.85 |
| Part C: VX-445/TEZ/IVA TC (Cohort C3) | 2.09 | 2.92 | 2.52 | 3.62 | 4.71 | 5.52 | 4.92 | 7.38 |
(NCT03227471)
Timeframe: Pre-dose on Day 15 and Day 29
| Intervention | microgram per milliliter (Mean) | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| VX-445: Day 15 | VX-445: Day 29 | TEZ: Day 15 | TEZ: Day 29 | M1-TEZ: Day 15 | M1-TEZ: Day 29 | IVA: Day 15 | IVA: Day 29 | M1-IVA: Day 15 | M1-IVA: Day 29 | |
| Part D: VX-445/TEZ/IVA TC - High Dose | 5.77 | 5.57 | 1.76 | 2.22 | 4.43 | 4.74 | 0.701 | 0.658 | 1.45 | 1.29 |
| Part D: VX-445/TEZ/IVA TC - Low Dose | 1.04 | 1.27 | 1.85 | 2.16 | 4.46 | 4.77 | 0.720 | 0.753 | 1.29 | 1.57 |
| Part D: VX-445/TEZ/IVA TC - Medium Dose | 2.15 | 2.18 | 1.68 | 1.77 | 4.11 | 4.30 | 0.665 | 0.704 | 1.21 | 1.20 |
(NCT03227471)
Timeframe: Pre-dose on Day 15 and Day 29
| Intervention | microgram per milliliter (Mean) | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| VX-445: Day 15 | VX-445: Day 29 | TEZ: Day 15 | TEZ: Day 29 | M1-TEZ: Day 15 | M1-TEZ: Day 29 | IVA: Day 15 | IVA: Day 29 | M1-IVA: Day 15 | M1-IVA: Day 29 | |
| Part E: VX-445/TEZ/IVA TC | 5.07 | 5.35 | 1.86 | 1.99 | 4.57 | 4.71 | 0.659 | 0.798 | 1.09 | 1.43 |
(NCT03227471)
Timeframe: Pre-dose on Day 15 and Day 29
| Intervention | microgram per milliliter (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| TEZ: Day 15 | TEZ: Day 29 | M1-TEZ: Day 15 | M1-TEZ: Day 29 | IVA: Day 15 | IVA: Day 29 | M1-IVA: Day 15 | M1-IVA: Day 29 | |
| Part E: TEZ/IVA | 1.85 | 1.84 | 3.96 | 3.73 | 0.766 | 0.595 | 1.22 | 0.943 |
(NCT03227471)
Timeframe: Pre-dose on Day 15 and Day 29
| Intervention | microgram per milliliter (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| VX-445: Day 15 | VX-445: Day 29 | TEZ: Day 15 | TEZ: Day 29 | M1-TEZ: Day 15 | M1-TEZ: Day 29 | VX-561: Day 15 | VX-561: Day 29 | |
| Part F: VX-445/TEZ/VX-561 TC | 4.40 | 5.25 | 1.80 | 2.22 | 4.99 | 5.09 | 0.441 | 0.597 |
(NCT03227471)
Timeframe: From first dose of study drug in treatment period up to safety follow-up (up to 28 days)
| Intervention | Participants (Count of Participants) | |
|---|---|---|
| Participants with AEs | Participants with SAEs | |
| Part A: Pooled Placebo (Except Cohort A7) | 0 | 0 |
| Part A: VX-445 (Cohort A7) | 1 | 0 |
| Part A: VX-445 (Except Cohort A7) | 3 | 0 |
| Part B: Pooled Placebo (Cohort B1 to B4) | 2 | 0 |
| Part B: VX-445 (Cohort B1 to B4) | 2 | 0 |
| Part C: Pooled Placebo (Cohort C1 to C3) | 2 | 0 |
| Part C: VX-445/TEZ/IVA TC (Cohort C1 to C3) | 5 | 0 |
(NCT03227471)
Timeframe: From first dose of study drug in TC treatment period up to 28 days after last dose of study drug (up to 5 weeks)
| Intervention | Participants (Count of Participants) | |
|---|---|---|
| Participants with AEs | Participants with SAEs | |
| Part D: Placebo | 12 | 2 |
| Part D: VX-445/TEZ/IVA TC | 49 | 3 |
| Part E: TEZ/IVA | 5 | 1 |
| Part E: VX-445/TEZ/IVA TC | 19 | 0 |
| Part F: Placebo | 7 | 1 |
| Part F: VX-445/TEZ/VX-561 TC | 19 | 0 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT03224351)
Timeframe: From Baseline at Day 29
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Part 1: Placebo | 4.7 |
| Part 1: VX-659/TEZ/IVA TC - Low Dose | 24.6 |
| Part 1: VX-659/TEZ/IVA TC - Medium Dose | 19.8 |
| Part 1: VX-659/TEZ/IVA TC - High Dose | 21.8 |
| Part 2: TEZ/IVA | 2.9 |
| Part 2: VX-659/TEZ/IVA TC | 19.5 |
| Part 3: Placebo | -4.1 |
| Part 3: VX-659/TEZ/VX-561 TC | 14.7 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03224351)
Timeframe: From Baseline Through Day 29
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Part 1: Placebo | 0.4 |
| Part 1: VX-659/TEZ/IVA TC - Low Dose | 10.2 |
| Part 1: VX-659/TEZ/IVA TC - Medium Dose | 12.0 |
| Part 1: VX-659/TEZ/IVA TC - High Dose | 13.3 |
| Part 2: TEZ/IVA | 0.0 |
| Part 2: VX-659/TEZ/IVA TC | 9.7 |
| Part 3: Placebo | -5.0 |
| Part 3: VX-659/TEZ/VX-561 TC | 12.2 |
Sweat samples were collected using an approved collection device. (NCT03224351)
Timeframe: From Baseline Through Day 29
| Intervention | millimole per liter (mmol/L) (Least Squares Mean) |
|---|---|
| Part 1: Placebo | 2.9 |
| Part 1: VX-659/TEZ/IVA TC - Low Dose | -45.7 |
| Part 1: VX-659/TEZ/IVA TC - Medium Dose | -43.8 |
| Part 1: VX-659/TEZ/IVA TC - High Dose | -51.4 |
| Part 2: TEZ/IVA | 3.0 |
| Part 2: VX-659/TEZ/IVA TC | -42.2 |
| Part 3: Placebo | -1.3 |
| Part 3: VX-659/TEZ/VX-561 TC | -38.1 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT03224351)
Timeframe: From Baseline Through Day 29
| Intervention | percent change (Least Squares Mean) |
|---|---|
| Part 1: Placebo | 0.0 |
| Part 1: VX-659/TEZ/IVA TC - Low Dose | 18.8 |
| Part 1: VX-659/TEZ/IVA TC - Medium Dose | 21.1 |
| Part 1: VX-659/TEZ/IVA TC - High Dose | 24.6 |
| Part 2: TEZ/IVA | 0.1 |
| Part 2: VX-659/TEZ/IVA TC | 17.3 |
| Part 3: Placebo | -11.3 |
| Part 3: VX-659/TEZ/VX-561 TC | 21.5 |
(NCT03224351)
Timeframe: Pre-dose at Day 15 and Day 29
| Intervention | nanogram per milliliter (ng/mL) (Mean) | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| VX-659: Day 15 | VX-659: Day 29 | TEZ: Day 15 | TEZ: Day 29 | M1-TEZ: Day 15 | M1-TEZ: Day 29 | IVA: Day 15 | IVA: Day 29 | M1-IVA: Day 15 | M1-IVA Day 29 | |
| Part 1: VX-659/TEZ/IVA TC - High Dose | 1100 | 1080 | 1110 | 1010 | 4280 | 3870 | 423 | 371 | 1170 | 1030 |
| Part 1: VX-659/TEZ/IVA TC - Low Dose | 393 | 566 | 1910 | 1910 | 4390 | 4300 | 824 | 719 | 1200 | 1130 |
| Part 1: VX-659/TEZ/IVA TC - Medium Dose | 622 | 699 | 1250 | 1050 | 3710 | 3650 | 522 | 443 | 1050 | 1140 |
| Part 2: VX-659/TEZ/IVA TC | 835 | 1070 | 1350 | 955 | 4010 | 3810 | 313 | 296 | 844 | 866 |
(NCT03224351)
Timeframe: Pre-dose at Day 15 and Day 29
| Intervention | nanogram per milliliter (ng/mL) (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| TEZ: Day 15 | TEZ: Day 29 | M1-TEZ: Day 15 | M1-TEZ: Day 29 | IVA: Day 15 | IVA: Day 29 | M1-IVA: Day 15 | M1-IVA Day 29 | |
| Part 2: TEZ/IVA | 1050 | 1150 | 4160 | 3790 | 458 | 490 | 1310 | 1240 |
(NCT03224351)
Timeframe: Pre-dose at Day 15 and Day 29
| Intervention | nanogram per milliliter (ng/mL) (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| VX-659: Day 15 | VX-659: Day 29 | TEZ: Day 15 | TEZ: Day 29 | M1-TEZ: Day 15 | M1-TEZ: Day 29 | VX-561: Day 15 | VX-561: Day 29 | |
| Part 3: VX-659/TEZ/VX-561 TC | 1140 | 923 | 1000 | 776 | 4060 | 3660 | 380 | 288 |
(NCT03224351)
Timeframe: Day 1 Through Safety Follow-up (up to Day 61 for Part 1, Day 85 for Part 2 and Day 57 for Part 3)
| Intervention | participants (Number) | |
|---|---|---|
| Participants with TEAEs | Participants with SAEs | |
| Part 1: Placebo | 9 | 3 |
| Part 1: VX-659/TEZ/IVA TC - High Dose | 17 | 1 |
| Part 1: VX-659/TEZ/IVA TC - Low Dose | 10 | 1 |
| Part 1: VX-659/TEZ/IVA TC - Medium Dose | 15 | 4 |
| Part 2: TEZ/IVA | 9 | 2 |
| Part 2: VX-659/TEZ/IVA TC | 15 | 1 |
| Part 3: Placebo | 6 | 3 |
| Part 3: VX-659/TEZ/VX-561 TC | 18 | 2 |
BMI = (Weight [in kg]) divided by (Stature [in meters]) ^2. Baseline was defined as the most recent measurement prior to intake of the first dose of study drug in study 108 Part B (NCT01705145). (NCT01946412)
Timeframe: Baseline (study 108), Week 12, 24, 36, 48, 60, 72 and 84 (study 109)
| Intervention | Kilogram per square meter (kg/m^2) (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| Baseline (n=9, 24) | Absolute Change at Week 12 (n=9, 23) | Absolute Change at Week 24 (n=9, 23) | Absolute Change at Week 36 (n=9, 23) | Absolute Change at Week 48 (n=9, 22) | Absolute Change at Week 60 (n=9, 22) | Absolute Change at Week 72 (n=9, 20) | Absolute Change at Week 84 (n=9, 19) | |
| Ivacaftor 50 mg | 15.74 | 0.03 | 0.31 | 0.15 | -0.31 | -0.12 | -0.38 | -0.16 |
| Ivacaftor 75 mg | 16.06 | 0.09 | -0.12 | 0.09 | -0.13 | -0.06 | 0.09 | 0.28 |
Stature was measured as height if children could stand unassisted and follow directions; otherwise, stature was measured as length. Baseline was defined as the most recent measurement prior to intake of the first dose of study drug in study 108 Part B (NCT01705145). (NCT01946412)
Timeframe: Baseline (study 108), Week 12, 24, 36, 48, 60, 72 and 84 (study 109)
| Intervention | Centimeters (cm) (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| Baseline (n=9, 24) | Absolute Change in Week 12 (n=9, 23) | Absolute Change in Week 24 (n=9, 23) | Absolute Change in Week 36 (n=9, 23) | Absolute Change in Week 48 (n=9, 21) | Absolute Change in Week 60 (n=9, 22) | Absolute Change in Week 72 (n=9, 20) | Absolute Change in Week 84 (n=9, 19) | |
| Ivacaftor 50 mg | 89.1 | 4.6 | 6.0 | 7.8 | 9.7 | 11.0 | 12.5 | 13.6 |
| Ivacaftor 75 mg | 102.3 | 5.4 | 7.7 | 8.8 | 10.4 | 11.6 | 13.4 | 15.0 |
Sweat samples were collected using an approved Macroduct (Wescor, Logan, Utah) collection device. A volume of greater than or equal to (>=) 15 microliter was required for determination of sweat chloride. Baseline was defined as the most recent measurement prior to intake of the first dose of study drug in study 108 Part B (NCT01705145). (NCT01946412)
Timeframe: Baseline (study 108), Week 24, 48, 72 and 84 (study 109)
| Intervention | millimole per liter (mmol/L) (Mean) | ||||
|---|---|---|---|---|---|
| Baseline (n=7,22) | Absolute Change at Week 24 (n=6,18) | Absolute Change at Week 48 (n=6,15) | Absolute Change at Week 72 (n=7,14) | Absolute Change at Week 84 (n=6,14) | |
| Ivacaftor 50 mg | 93.1 | -62.1 | -29.3 | -46.4 | -46.5 |
| Ivacaftor 75 mg | 99.6 | -48.5 | -51.8 | -52.9 | -58.1 |
Baseline was defined as the most recent measurement prior to intake of the first dose of study drug in study 108 Part B (NCT01705145) (NCT01946412)
Timeframe: Baseline (study 108), Week 12, 24, 36, 48, 60, 72 and 84 (study 109)
| Intervention | kilogram (kg) (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| Baseline (n=9, 24) | Absolute Change at Week 12 (n= 9, 23) | Absolute Change at Week 24 (n=9, 23) | Absolute Change at Week 36 (n=9, 23) | Absolute Change at Week 48 (n=9, 22) | Absolute Change at Week 60 (n=9, 22) | Absolute Change at Week 72 (n=9, 20) | Absolute Change at Week 84 (n=9, 19) | |
| Ivacaftor 50 mg | 12.5 | 1.3 | 2.0 | 2.4 | 2.6 | 3.2 | 3.4 | 4.0 |
| Ivacaftor 75 mg | 16.8 | 1.9 | 2.5 | 3.1 | 3.4 | 4.0 | 4.8 | 5.7 |
BMI = (Weight [in kg]) divided by (Stature [in meters]) ^2. Baseline is defined as the most recent measurement prior to intake of the first dose of study drug in study 109 (NCT01946412). (NCT01946412)
Timeframe: Baseline (study 109), Week 12, 24, 36, 48, 60, 72 and 84 (study 109)
| Intervention | kg/m^2 (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| Baseline (n= 9, 24) | Absolute Change at Week 12 (n= 9, 23) | Absolute Change at Week 24 (n= 9, 23) | Absolute Change at Week 36 (n= 9, 23) | Absolute Change at Week 48 (n= 9, 22) | Absolute Change at Week 60 (n= 9, 22) | Absolute Change at Week 72 (n= 9, 20) | Absolute Change at Week 84 (n= 9, 19) | |
| Ivacaftor 50 mg | 16.07 | -0.30 | -0.02 | -0.18 | -0.64 | -0.45 | -0.71 | -0.49 |
| Ivacaftor 75 mg | 16.33 | -0.16 | -0.36 | -0.16 | -0.35 | -0.29 | -0.21 | -0.01 |
Stature was measured as height if children could stand unassisted and follow directions; otherwise, stature was measured as length. Baseline is defined as the most recent measurement prior to intake of the first dose of study drug in study 109 (NCT01946412). (NCT01946412)
Timeframe: Baseline (study 109), Week 12, 24, 36, 48, 60, 72 and 84 (study 109)
| Intervention | cm (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| Baseline (n=9, 24) | Absolute Change at Week 12 (n=9, 23) | Absolute Change at Week 24 (n=9, 23) | Absolute Change at Week 36 (n=9, 23) | Absolute Change at Week 48 (n=9, 21) | Absolute Change at Week 60 (n=9, 22) | Absolute Change at Week 72 (n=9, 20) | Absolute Change at Week 84 (n=9, 19) | |
| Ivacaftor 50 mg | 91.7 | 2.0 | 3.4 | 5.2 | 7.2 | 8.4 | 10.0 | 11.1 |
| Ivacaftor 75 mg | 105.8 | 1.8 | 4.1 | 5.2 | 6.8 | 8.0 | 9.8 | 11.4 |
Sweat samples were collected using an approved Macroduct (Wescor, Logan, Utah) collection device. A volume of >=15 microliter was required for determination of sweat chloride. Baseline is defined as the most recent measurement prior to intake of the first dose of study drug in study 109 (NCT01946412). (NCT01946412)
Timeframe: Baseline (study 109), Week 24, 48, 72 and 84 (study 109)
| Intervention | mmol/L (Mean) | ||||
|---|---|---|---|---|---|
| Baseline (n= 8,23) | Absolute Change at Week 24 (n=7,18) | Absolute Change at Week 48 (n=7,17) | Absolute Change at Week 72 (n=8,15) | Absolute Change at Week 84 (n=7,16) | |
| Ivacaftor 50 mg | 47.8 | -4.3 | 18.1 | -1.5 | -2.4 |
| Ivacaftor 75 mg | 52.9 | 3.4 | -4.5 | -6.0 | -11.2 |
Baseline is defined as the most recent measurement prior to intake of the first dose of study drug in study 109 (NCT01946412). (NCT01946412)
Timeframe: Baseline (study 109), Week 12, 24, 36, 48, 60, 72 and 84 (study 109)
| Intervention | Kg (Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| Baseline (n= 9, 24) | Absolute Change at Week 12 (n=9, 23) | Absolute Change at Week 24 (n=9, 23) | Absolute Change at Week 36 (n=9, 23) | Absolute Change at Week 48 (n=9, 22) | Absolute Change at Week 60 (n=9, 22) | Absolute Change at Week 72 (n=9, 20) | Absolute Change at Week 84 (n=9, 19) | |
| Ivacaftor 50 mg | 13.5 | 0.3 | 1.0 | 1.4 | 1.6 | 2.2 | 2.4 | 3.0 |
| Ivacaftor 75 mg | 18.3 | 0.4 | 1.0 | 1.6 | 1.9 | 2.5 | 3.3 | 4.2 |
AE: any untoward medical occurrence in a participant during the study; the event does not necessarily have a causal relationship with the treatment. This includes any newly occurring event or previous condition that has increased in severity or frequency after the informed consent form is signed. AE includes serious as well as Non-serious AEs. SAE (subset of AE): medical event or condition, which falls into any of the following categories, regardless of its relationship to the study drug: death, life threatening adverse experience, Inpatient hospitalization/prolongation of hospitalization, persistent/significant disability or incapacity, congenital anomaly/birth defect, important medical event. AEs with start date or increased severity on or after the first dose of study drug through the end of study participation was considered treatment-emergent. (NCT01946412)
Timeframe: Day 1 up to Week 97 (for participants who completed study drug dosing); Day 1 up to 24 weeks after the last dose (up to Week 108, for participants who prematurely discontinued study drug dosing)
| Intervention | participants (Number) | |
|---|---|---|
| AEs | SAEs | |
| Ivacaftor 50 mg | 9 | 6 |
| Ivacaftor 75 mg | 24 | 5 |
Area under the concentration-time curve from time 0 up to 24 hours following multiple dosing (ng.h/mL), calculated by linear up/log down trapezoidal summation. All PK parameters were determined from Day 15; Day 29 data were determined if the participant was not available for full PK profiling on Day 15. (NCT03119649)
Timeframe: Day 15 (predose and 0.5, 1, 2, 3, 4, 6, and 8 hours postdose) and prior to dosing on Day 29
| Intervention | ng.h/mL (Mean) |
|---|---|
| Cohort A: GLPG2222 50 mg QD | 3850 |
| Cohort A: GLPG2222 100 mg QD | 9670 |
| Cohort B: GLPG2222 200 mg QD | 22900 |
| Cohort B: GLPG2222 400 mg QD | 46400 |
Percent predicted FEV1 for age, gender, and height was determined from standardized spirometry assessments and estimated using the 2012 Global Lungs Initiative equation. Baseline was defined as the last non-missing predose assessment on Day 1. (NCT03119649)
Timeframe: Predose and between 1 and 2 hours postdose on Days 1 and 29, or at early discontinuation
| Intervention | % predicted FEV1 (Least Squares Mean) |
|---|---|
| Pooled Placebo | -1.0 |
| Cohort A: GLPG2222 50 mg QD | 0.1 |
| Cohort A: GLPG2222 100 mg QD | -0.3 |
| Cohort B: GLPG2222 200 mg QD | 0.0 |
| Cohort B: GLPG2222 400 mg QD | 1.3 |
Two sweat collections, one from each arm, were obtained. Mean sweat chloride concentration was determined from both arms and measured as millimoles per liter (mmol/L). Baseline was defined as the predose value on Day 1 (or the last non-missing predose measurement). (NCT03119649)
Timeframe: Prior to dosing on Days 1 and 29, or at early discontinuation
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Pooled Placebo | -2.5 |
| Cohort A: GLPG2222 50 mg QD | -5.8 |
| Cohort A: GLPG2222 100 mg QD | -6.6 |
| Cohort B: GLPG2222 200 mg QD | -18.3 |
| Cohort B: GLPG2222 400 mg QD | -8.8 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. The respiratory domain assessed respiratory symptoms (for example, coughing, congestion, wheezing), derived from Questions 40, 41, 42, 45, and 46 if at least 50% of the questions had non-missing data. The scale score ranged from 0-100; higher scores indicated fewer symptoms and better health-related quality of life with a negative change indicating a worsening of symptoms. A change of 4 is considered clinically relevant. (NCT03119649)
Timeframe: Prior to dosing on Days 1 and 29, or at early discontinuation
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Pooled Placebo | -2.4 |
| Cohort A: GLPG2222 50 mg QD | 0.4 |
| Cohort A: GLPG2222 100 mg QD | -0.7 |
| Cohort B: GLPG2222 200 mg QD | 4.5 |
| Cohort B: GLPG2222 400 mg QD | -0.8 |
Plasma concentration of GLPG2222 observed at pre-dose (ng/mL), obtained directly from the observed concentration versus time data. Ctrough was calculated using both Day 15 and Day 29 PK data. (NCT03119649)
Timeframe: Days 15 and 29 (predose)
| Intervention | ng/mL (Mean) |
|---|---|
| Cohort A: GLPG2222 50 mg QD | 48.1 |
| Cohort A: GLPG2222 100 mg QD | 132 |
| Cohort B: GLPG2222 200 mg QD | 343 |
| Cohort B: GLPG2222 400 mg QD | 677 |
Maximum concentration of GLPG2222 after multiple dosing (ng/ML), obtained directly from the observed concentration versus time data. All pharmacokinetic (PK) parameters were determined from Day 15; Day 29 data were determined if the participant was not available for full PK profiling on Day 15. (NCT03119649)
Timeframe: Day 15 (predose and 0.5, 1, 2, 3, 4, 6, and 8 hours postdose) and prior to dosing on Day 29
| Intervention | ng/mL (Mean) |
|---|---|
| Cohort A: GLPG2222 50 mg QD | 478 |
| Cohort A: GLPG2222 100 mg QD | 1170 |
| Cohort B: GLPG2222 200 mg QD | 2490 |
| Cohort B: GLPG2222 400 mg QD | 5330 |
Time of occurrence of maximum concentration of GLPG2222 after multiple dosing (h), obtained directly from the observed concentration versus time data. All PK parameters were determined from Day 15; Day 29 data were determined if the participant was not available for full PK profiling on Day 15. (NCT03119649)
Timeframe: Day 15 (predose and 0.5, 1, 2, 3, 4, 6, and 8 hours postdose) and prior to dosing on Day 29
| Intervention | hours (Median) |
|---|---|
| Cohort A: GLPG2222 50 mg QD | 2.0 |
| Cohort A: GLPG2222 100 mg QD | 2.0 |
| Cohort B: GLPG2222 200 mg QD | 3.0 |
| Cohort B: GLPG2222 400 mg QD | 2.0 |
Number of participants with any treatment-emergent adverse events (TEAEs) and serious or treatment-related TEAEs, as well as number of patients with TEAEs by worst intensity reported (mild, moderate, or severe). (NCT03119649)
Timeframe: First administration (Day 1) through Follow-up (Day 43)
| Intervention | Participants (Count of Participants) | |||||
|---|---|---|---|---|---|---|
| Any TEAE | Any Serious TEAE | Worst TEAE Intensity=Mild | Worst TEAE Intensity=Moderate | Worst TEAE Intensity=Severe | Any Treatment-related TEAE | |
| Cohort A: GLPG2222 100 mg QD | 10 | 1 | 7 | 2 | 1 | 6 |
| Cohort A: GLPG2222 50 mg QD | 8 | 0 | 4 | 4 | 0 | 2 |
| Cohort B: GLPG2222 200 mg QD | 11 | 0 | 8 | 2 | 1 | 5 |
| Cohort B: GLPG2222 400 mg QD | 9 | 0 | 9 | 0 | 0 | 1 |
| Pooled Placebo | 9 | 2 | 6 | 2 | 1 | 2 |
BMI was defined as weight in kg divided by height in m^2. z-score is a statistical measure to describe whether a mean was above or below the standard. BMI, adjusted for age and sex, was analyzed as BMI-for-age z-score. A z-score of 0 is equal to the mean and is considered normal. Lower numbers indicate values lower than the mean and higher numbers indicate values higher than the mean. Higher values are indicative of higher BMI. (NCT02953314)
Timeframe: From Baseline at Week 24
| Intervention | z-score (Least Squares Mean) |
|---|---|
| Part B | -0.03 |
BMI was defined as weight in kg divided by height in square meter (m^2). (NCT02953314)
Timeframe: From Baseline at Week 24
| Intervention | kg/m^2 (Least Squares Mean) |
|---|---|
| Part B | 0.23 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT02953314)
Timeframe: From Baseline through Week 24
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Part B | 3.4 |
(NCT02953314)
Timeframe: From Baseline at Week 24
| Intervention | centimeter (cm) (Least Squares Mean) |
|---|---|
| Part B | 2.7 |
z-score is a statistical measure to describe whether a mean was above or below the standard. Height, adjusted for age and sex, was analyzed as height-for-age z-score. A z-score of 0 is equal to the mean and is considered normal. Lower numbers indicate values lower than the mean and higher numbers indicate values higher than the mean. Higher values are indicative of higher height. (NCT02953314)
Timeframe: From Baseline at Week 24
| Intervention | z-score (Least Squares Mean) |
|---|---|
| Part B | 0.00 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT02953314)
Timeframe: From Baseline through Week 24
| Intervention | percentage points (Least Squares Mean) |
|---|---|
| Part B | 0.9 |
Sweat samples were collected using an approved collection device. (NCT02953314)
Timeframe: From Baseline through Week 24
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Part B | -14.5 |
Sweat samples were collected using an approved collection device. (NCT02953314)
Timeframe: From Baseline through Week 4
| Intervention | millimole per liter (mmol/L) (Least Squares Mean) |
|---|---|
| Part B | -13.0 |
(NCT02953314)
Timeframe: From Baseline at Week 24
| Intervention | kg (Least Squares Mean) |
|---|---|
| Part B | 1.7 |
z-score is a statistical measure to describe whether a mean was above or below the standard. Weight, adjusted for age and sex, was analyzed as weight-for-age z-score. A z-score of 0 is equal to the mean and is considered normal. Lower numbers indicate values lower than the mean and higher numbers indicate values higher than the mean. Higher values are indicative of higher weight. (NCT02953314)
Timeframe: From Baseline at Week 24
| Intervention | z-score (Least Squares Mean) |
|---|---|
| Part B | 0.00 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT02953314)
Timeframe: From Baseline through Week 24
| Intervention | percent change (Least Squares Mean) |
|---|---|
| Part B | 1.4 |
(NCT02953314)
Timeframe: Day 1 and Day 14
| Intervention | hour*nanogram per milliliter (hr*ng/mL) (Geometric Mean) | |||||
|---|---|---|---|---|---|---|
| Day 1: TEZ (<25 Kg) | Day 1: TEZ (≥25 Kg) | Day 14: TEZ (<25 Kg) | Day 14: TEZ (≥25 Kg) | Day 14: IVA (<25 Kg) | Day 14: IVA (≥25 Kg) | |
| Part A | 54300 | 41600 | 66500 | 71600 | 5050 | 12400 |
(NCT02953314)
Timeframe: Day 1 and Day 14
| Intervention | hr*ng/mL (Geometric Mean) | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Day 1: M1-TEZ (<25 kg) | Day 1: M1-TEZ (≥25 kg) | Day 14: M1-TEZ (<25 kg) | Day 14: M1-TEZ (≥25 kg) | Day 1: M2-TEZ (<25 kg) | Day 1: M2-TEZ (≥25 kg) | Day 14: M2-TEZ (<25 kg) | Day 14: M2-TEZ (≥25 kg) | Day 14: M1-IVA (<25 kg) | Day 14: M1-IVA (≥25 kg) | Day 14: M6-IVA (<25 kg) | Day 14: M6-IVA (≥25 kg) | |
| Part A | 36500 | 27400 | 160000 | 121000 | 14200 | 11100 | 137000 | 119000 | 13700 | 30300 | 10200 | 26000 |
(NCT02953314)
Timeframe: Day 1 and Day 14
| Intervention | ng/mL (Geometric Mean) | |||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Day 1: M1-TEZ (<25 kg) | Day 1: M1-TEZ (≥25 kg) | Day 14: M1-TEZ (<25 kg) | Day 14: M1-TEZ (≥25 kg) | Day 1: M2-TEZ (<25 kg) | Day 1: M2-TEZ (≥25 kg) | Day 14: M2-TEZ (<25 kg) | Day 14: M2-TEZ (≥25 kg) | Day 1: M1-IVA (<25 kg) | Day 1: M1-IVA (≥25 kg) | Day 14: M1-IVA (<25 kg) | Day 14: M1-IVA (≥25 kg) | Day 1: M6-IVA (<25 kg) | Day 1: M6-IVA (≥25 kg) | Day 14: M6-IVA (<25 kg) | Day 14: M6-IVA (≥25 kg) | |
| Part A | 1720 | 1530 | 8360 | 5930 | 1130 | 922 | 6180 | 5350 | 2320 | 2430 | 1460 | 3420 | 849 | 1070 | 1090 | 2720 |
(NCT02953314)
Timeframe: Day 1 and Day 14
| Intervention | nanogram per milliliter (ng/mL) (Geometric Mean) | |||||||
|---|---|---|---|---|---|---|---|---|
| Day 1: TEZ (<25 Kg) | Day 1: TEZ (≥25 Kg) | Day 14: TEZ (<25 Kg) | Day 14: TEZ (≥25 Kg) | Day 1: IVA (<25 Kg) | Day 1: IVA (≥25 Kg) | Day 14: IVA (<25 Kg) | Day 14: IVA (≥25 Kg) | |
| Part A | 6630 | 4310 | 6300 | 5340 | 656 | 1010 | 578 | 1490 |
(NCT02953314)
Timeframe: Day 1 up to Day 28
| Intervention | participants (Number) | |
|---|---|---|
| Participants with AEs | Participants with SAEs | |
| Part A | 12 | 0 |
(NCT02953314)
Timeframe: Week 16
| Intervention | hr*ng/mL (Geometric Mean) | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| TEZ (<40 kg) | TEZ (≥40 kg) | M1-TEZ (<40 kg) | M1-TEZ (≥40 kg) | M2-TEZ (<40 kg) | M2-TEZ (≥40 kg) | IVA (<40 kg) | IVA (≥40 kg) | M1-IVA (<40 kg) | M1-IVA (≥40 kg) | M6-IVA (<40 kg) | M6-IVA (≥40 kg) | |
| Part B | 50300 | 60900 | 104000 | 100000 | 88400 | 93600 | 5330 | 7410 | 12700 | 17200 | 8140 | 11100 |
(NCT02953314)
Timeframe: Week 16
| Intervention | ng/mL (Geometric Mean) | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| TEZ (<40 kg) | TEZ (≥40 kg) | M1-TEZ (<40 kg) | M1-TEZ (≥40 kg) | M2-TEZ (<40 kg) | M2-TEZ (≥40 kg) | IVA (<40 kg) | IVA (≥40 kg) | M1-IVA (<40 kg) | M1-IVA (≥40 kg) | M6-IVA (<40 kg) | M6-IVA (≥40 kg) | |
| Part B | 4800 | 5870 | 5310 | 5440 | 4170 | 5210 | 725 | 886 | 1560 | 1870 | 870 | 1120 |
(NCT02953314)
Timeframe: Day 1 up to Week 28
| Intervention | participants (Number) | |
|---|---|---|
| Participants with AEs | Participants with SAEs | |
| Part B | 65 | 6 |
The sweat chloride (quantitative pilocarpine iontophoresis) test is a standard diagnostic tool for cystic fibrosis (CF), serving as an indicator of cystic fibrosis transmembrane conductance regulator (CFTR) activity. (NCT00457821)
Timeframe: 14 days and 28 days
| Intervention | millimoles per liter (Least Squares Mean) | |
|---|---|---|
| Change from Baseline in Sweat Chloride, Day 14 | Change from Baseline in Sweat Chloride, Day ≥14 | |
| 150 mg Ivacaftor q12h | -46.0 | -44.2 |
| 25 mg Ivacaftor q12h | -33.8 | -32.9 |
| 250 mg Ivacaftor q12h | -27.1 | -28.2 |
| 75 mg Ivacaftor q12h | -42.0 | -40.8 |
| Placebo | 2.0 | 4.9 |
The transepithelial nasal potential difference (NPD) is a direct measure of transepithelial ion transport. NPD under conditions of zero chloride concentration perfusion solution in the presence of isoproterenol was of primary interest. (NCT00457821)
Timeframe: 14 days and 28 days
| Intervention | millivolts (Least Squares Mean) | |||
|---|---|---|---|---|
| Zero Chloride + Isoproterenol, Day 14 | Zero Chloride + Isoproterenol, Day ≥ 14 | Amiloride, Day 14 | Amiloride, Day ≥ 14 | |
| 150 mg Ivacaftor q12h | -4.6 | -5.3 | -9.9 | -8.8 |
| 25 mg Ivacaftor q12h | -1.4 | -1.3 | -0.1 | -0.3 |
| 250 mg Ivacaftor q12h | -7.6 | -8.4 | -5.5 | -4.0 |
| 75 mg Ivacaftor q12h | -4.4 | -4.5 | -4.2 | -3.7 |
| Placebo | -0.3 | -0.5 | 0.3 | -0.7 |
"Spirometry is a standardized assessment to evaluate lung function that is the most widely used endpoint in cystic fibrosis studies.~Relative change reflects the percent change from the baseline values [100% * (X-Y)/Y], where X and Y are post-baseline and baseline values, respectively." (NCT00457821)
Timeframe: 14 days and 28 days
| Intervention | percent predicted (%) (Least Squares Mean) | |||
|---|---|---|---|---|
| Absolute Change from Baseline, Day 14 | Absolute Change from Baseline, Day ≥ 14 | Relative Change from Baseline, Day 14 | Relative Change from Baseline, Day ≥ 14 | |
| 150 mg Ivacaftor q12h | 6.9 | 6.9 | 10.8 | 10.6 |
| 25 mg Ivacaftor q12h | 2.7 | 2.5 | 4.7 | 4.1 |
| 250 mg Ivacaftor q12h | 8.4 | 6.7 | 12.0 | 9.4 |
| 75 mg Ivacaftor q12h | 5.1 | 5.3 | 9.5 | 9.3 |
| Placebo | 0.5 | 2.1 | 2.0 | 3.3 |
The CFQ-R is a health-related quality of life measure for subjects with cystic fibrosis. Each domain is scored from 0 (worst) to 100 (best). A difference of at least 4 points in the respiratory domain score of the CFQ-R is considered a minimal clinically important difference (MCID). (NCT00457821)
Timeframe: 14 days and 28 days
| Intervention | score on a scale (Mean) | ||
|---|---|---|---|
| Baseline Respiratory Domain Score | Change from Baseline in Respiratory Score, Day 14 | Change from Baseline in Respiratory Score, Day 28 | |
| 150 mg Ivacaftor q12h | 68.8 | 6.3 | 6.9 |
| 250 mg Ivacaftor q12h | 73.0 | 5.6 | 11.9 |
| Placebo | 70.8 | 2.8 | 2.8 |
Adverse event data were collected up to the follow-up visit (5 to 9 days after last dose of study drug). Serious adverse events that were ongoing at the follow-up visit were followed until the event resolved, returned to baseline, or was determined to be a stable or chronic condition. (NCT00457821)
Timeframe: Baseline to Follow-up
| Intervention | events (Number) | |||
|---|---|---|---|---|
| Number of Adverse Events (AEs) | Number of Related or Possibly Related AEs | Number of Serious Adverse Events (SAEs) | Number of Related or Possibly Related SAEs | |
| Ivacaftor | 179 | 40 | 2 | 0 |
| Placebo | 32 | 11 | 0 | 0 |
Adverse event data were collected up to the follow-up visit (5 to 9 days after last dose of study drug). Serious adverse events that were ongoing at the follow-up visit were followed until the event resolved, returned to baseline, or was determined to be a stable or chronic condition. (NCT00457821)
Timeframe: Baseline to Follow-up
| Intervention | participants (Number) | |||
|---|---|---|---|---|
| Subjects with AEs | Subjects with Related or Possibly Related AEs | Subjects with SAEs | Subjects with Related or Possibly Related SAEs | |
| Ivacaftor | 26 | 13 | 1 | 0 |
| Placebo | 7 | 3 | 0 | 0 |
Spirometry was used to assess pulmonary function by measuring the percentage of predicted function, which was determined on the basis of the height value obtained at the same study visit, for FVC (the amount of air that can be exhaled after taking a deep breath). Spirometry was assessed by using current guidelines of the ATS and ERS. Baseline was the average of percent-predicted FVC at screening and randomization. (NCT00803205)
Timeframe: Baseline (Week 1)
| Intervention | percentage of predicted FVC (Mean) |
|---|---|
| Ataluren | 78.332 |
| Placebo | 76.609 |
Spirometry was used to assess pulmonary function by measuring the percentage of predicted function, which was determined on the basis of the height value obtained at the same study visit, for FEV1 (the amount of air that can be exhaled in 1 second). Spirometry was assessed by using current guidelines of the American Thoracic Society (ATS) and European Respiratory Society (ERS). The percentage of change in percent-predicted of FEV1 was calculated as follows: ([percent-predicted FEV1-Baseline percent-predicted FEV1]/Baseline percent-predicted FEV1)*100. Baseline was the average of percent-predicted FEV1 at screening and randomization. A negative change from Baseline indicates that percent-predicted of FEV1 decreased. (NCT00803205)
Timeframe: End of Treatment (EOT) (Week 48)
| Intervention | percent change (Mean) |
|---|---|
| Ataluren | -2.534 |
| Placebo | -5.500 |
Spirometry was used to assess pulmonary function by measuring the percentage of predicted function, which was determined on the basis of the height value obtained at the same study visit, for FVC (the amount of air that can be exhaled after taking a deep breath). Spirometry was assessed by using current guidelines of the ATS and ERS. The percentage of change in percent-predicted of FVC was calculated as follows: ((percent-predicted FVC-Baseline percent-predicted FVC)/Baseline percent-predicted FVC)*100. Baseline was the average of percent-predicted FVC at screening and randomization. A negative change from Baseline indicates that percent-predicted of FVC decreased. (NCT00803205)
Timeframe: EOT (Week 48)
| Intervention | percent change (Mean) |
|---|---|
| Ataluren | -2.139 |
| Placebo | -3.484 |
Spirometry was used to assess pulmonary function by measuring the percentage of predicted function, which was determined on the basis of the height value obtained at the same study visit, for FEV1 (the amount of air that can be exhaled in 1 second). Spirometry was assessed by using current guidelines of the American Thoracic Society (ATS) and European Respiratory Society (ERS). Baseline was the average of percent-predicted FEV1 at screening and randomization. (NCT00803205)
Timeframe: Baseline (Week 1)
| Intervention | percentage of predicted FEV1 (Mean) |
|---|---|
| Ataluren | 62.092 |
| Placebo | 60.232 |
During treatment, any disruption in the activities of daily living, such as missed school or work, was documented if it was due to an exacerbation-like episode. Participants and caregivers recorded all disruptions in an electronic diary. The rate of disruptions was defined as the total days with disruptions to daily living divided by the total study duration. (NCT00803205)
Timeframe: Baseline up to EOT (Week 48)
| Intervention | days with disruptions per study (Mean) |
|---|---|
| Ataluren | 0.037 |
| Placebo | 0.047 |
A Respiratory Event Form, which collected data on various signs, symptoms, and effects for each event, was completed by the Investigator when informed by the participant of a respiratory event. Pulmonary exacerbations were assessed by using the modified Fuchs' criteria, which defines an exacerbation as a respiratory event requiring treatment with parenteral antibiotics for any 4 of the following 12 symptoms, with or without intravenous antibiotics: change in sputum; new or increased hemoptysis; increased cough; increased dyspnea; fatigue; temperature >38°C; anorexia; sinus pain; change in sinus discharge; change in physical examination of the chest; decrease in pulmonary function by 10% or more from a previously recorded value; or radiographic changes indicative of pulmonary function. The 48-week exacerbation rate was determined by adding the weekly rates for each arm and dividing the sum by 48. (NCT00803205)
Timeframe: Baseline to EOT (Week 48)
| Intervention | exacerbations (Mean) |
|---|---|
| Ataluren | 1.42 |
| Placebo | 1.78 |
"Patient-reported data were obtained from the participant's electronic daily diary, which was completed by the participant or the caregiver. During study treatment, the electronic daily diary was to be completed by the participant or caregiver each day for each dose. For each participant, compliance is described in terms of the percentage of study drug actually taken. All calculations were based on the records of the first dose date to the last dose date. To differentiate dose strengths while maintaining the blind, each kit had a unique kit number and had prominent lettering A and B. Each kit contained 65 packets of 1 of the dose strengths (125, 250, or 1000 mg or matching placebo). Labeling for active drug and placebo was identical." (NCT00803205)
Timeframe: Baseline up to EOT (Week 48)
| Intervention | percent of doses taken (Median) |
|---|---|
| Ataluren | 71.48 |
| Placebo | 69.27 |
The frequency of awake cough was measured using the LifeShirt, which incorporates motion-sensing transducers, electrodes, a microphone, and a 3-axis accelerometer into a lightweight vest. The rate was determined by dividing the total number of coughs by 24 (the number of hours of the observation period). Baseline was the latest, valid assessment prior to the treatment. A negative change from Baseline indicates that coughing decreased. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | coughs/hour (Mean) | |
|---|---|---|
| Baseline | Change From Baseline | |
| Ataluren | 28.218 | -0.595 |
| Placebo | 24.472 | 0.882 |
Participants were weighed, and the weight was recorded at Baseline and then every 8 weeks during the treatment period. Baseline was the latest valid assessment prior to the treatment. A positive change from Baseline indicates that weight increased. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | kg (Mean) | |
|---|---|---|
| Baseline | Change From Baseline | |
| Ataluren | 53.46 | 0.87 |
| Placebo | 56.01 | 0.83 |
Sweat was collected, from each arm, by using pilocarpine iontophoresis. The chloride concentration in the sweat was quantified for each arm by using standard laboratory methods. Tests were also considered valid if the sweat collection time was ≤35 minutes; tests with longer collection times were also considered valid if extra time was needed to obtain sufficient volume (≥15uL) for analysis. For analysis purposes, the average of the values from each arm were computed. If the assessment was valid and/or available in only 1 arm, this value was used as if it were the average of both arms. The method used was consistent with the CFFT-TDN guidelines. Baseline was the latest, valid assessment prior to the treatment. A negative change from Baseline indicates that sweat chloride concentration decreased. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | millimoles/L (Mean) | |
|---|---|---|
| Baseline | Change From Baseline | |
| Ataluren | 100.140 | -1.325 |
| Placebo | 96.586 | -0.619 |
Expression of CRP was measured in serum. Baseline was the latest valid assessment prior to the treatment. A positive change from Baseline indicates that CRP concentration increased. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | mg/liter (L) (Mean) | |
|---|---|---|
| Baseline | Change From Baseline | |
| Ataluren | 6.899 | 2.420 |
| Placebo | 7.037 | 2.031 |
Expression of IL-8 was measured in serum and in sputum. Sputum was spontaneously produced and tested by using standardized procedures developed by the Cystic Fibrosis Foundation Therapeutics, Inc. Therapeutics Development Network (CFFT-TDN). Baseline was the latest valid assessment prior to the treatment. A negative change from Baseline indicates that the concentration of IL-8 decreased. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | picograms/mL (Mean) | |||
|---|---|---|---|---|
| Serum, Baseline | Serum, Change From Baseline | Sputum, Baseline | Sputum, Change From Baseline | |
| Ataluren | 39.537 | -2.334 | 267629.93 | 28882.79 |
| Placebo | 55.845 | -16.197 | 250170.95 | 9957.24 |
Expression of neutrophil elastase was measured in sputum. Sputum was spontaneously produced and tested by using standardized procedures developed by the CFFT-TDN. Baseline was the latest valid assessment prior to the treatment. A positive change from Baseline indicates that the concentration of neutrophil elastase increased. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | ug/mL (Mean) | |
|---|---|---|
| Baseline | Change From Baseline | |
| Ataluren | 183.64 | 5.45 |
| Placebo | 227.35 | -8.67 |
The CFQ-R consists of 44 items, including generic scales of physical functioning, role functioning, vitality, health perceptions, emotional functioning, and social functioning, and CF-specific scales of respiratory and digestive symptoms, body image, eating disturbances, and treatment burden. Each domain score ranges from 1 to 4. Scores were linearly transformed to a 0 to 100 scale, with higher scores indicating better health. Domain scores were calculated by using the following formula: 100 * (sum of responses - minimum possible sum)/ (maximum possible sum - minimum possible sum). The minimum possible sum = number of questions * 1; the maximum possible = the number of questions * 4. Baseline was the latest, valid assessment prior to the treatment. A negative change from Baseline indicates that health has worsened. Participants may have switched age groups during the study. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | units on a scale (Mean) | |||
|---|---|---|---|---|
| Aged 6-13 years, Baseline | Aged 6-13 years, Change From Baseline | Age ≥14 years , Baseline | Age ≥14 years, Change From Baseline | |
| Ataluren | 77.78 | -0.69 | 70.06 | -2.81 |
| Placebo | 79.49 | -3.57 | 65.95 | -3.32 |
Lungs were imaged by using non-contrast, spiral CT. The total lung score for each CT scan was established by the sum of 5 characteristics from the Brody scoring system, with scores ranging from 0 to 40.5, with lower scores indicating better lung function. The characteristics scored were bronchiectasis (score range 0 - 12), mucus plugging (score range 0- 6), peribronchial thickening (score range 0 - 9), parenchyma (score range 0 - 9), and hyperinflation (score range 0 - 4.5). Baseline was the latest valid assessment prior to the treatment. A positive change from Baseline indicates that lung function worsened. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | units on a scale (Mean) | |
|---|---|---|
| Baseline | Change From Baseline | |
| Ataluren | 9.531 | 0.282 |
| Placebo | 9.619 | 0.560 |
TEPD was assessed in each nostril using standardized equipment, techniques, and solutions. Assessments were made on the nasal epithelium cells lining the inferior turbinate. Warmed solutions of Ringer's solution, amiloride, chloride-free gluconate, isoproterenol, and adenosine triphosphate (ATP) were perfused for ≥3-minute sequentially through a nasal catheter while a voltage tracing was recorded. Total chloride transport was computed for each nostril. The total chloride transport values were calculated by subtracting the voltages at the end of a perfusion from the voltage at the end of an earlier perfusion (isoproterenol - amiloride). The average of the values for each nostril was computed. If the assessment was available in only 1 nostril, this value was used as if it were the average of both nostrils. Baseline was the latest, valid assessment prior to the treatment. A positive change from Baseline indicates that nasal chloride transport increased. (NCT00803205)
Timeframe: Baseline, EOT (Week 48)
| Intervention | millivolts (Mean) | |
|---|---|---|
| Baseline | Change From Baseline | |
| Ataluren | 1.578 | 0.312 |
| Placebo | 1.950 | 0.139 |
Blood samples were drawn immediately before administration of the first daily dose (dose taken with breakfast) of study drug and 2 hours after the first daily dose. Whenever possible, the pre-dose sample was to be obtained within 15 minutes of drug administration. Participants in the Placebo arm did not receive Ataluren and are not included in this Outcome Measure. (NCT00803205)
Timeframe: Predose and 2 Hours Postdose at Week 1, Week 16, Week 32, EOT (Week 48)
| Intervention | micrograms/milliliter (ug/mL) (Median) | |||||||
|---|---|---|---|---|---|---|---|---|
| Week 1 Predose | Week 1 Postdose | Week 16 Predose | Week 16 Postdose | Week 32 Predose | Week 32 Postdose | Week 48 Predose | Week 48 Postdose | |
| Ataluren | 0 | 14.100 | 4.350 | 11.900 | 4.630 | 13.400 | 3.970 | 10.500 |
A TEAE was any untoward medical occurrence in a participant who received study drug without regard to possibility of causal relationship that occurred or worsened in the period extending from first dose of study drug to 4 weeks after the last dose of study drug. A serious adverse event (SAE) was an AE resulting in any of the following outcomes or deemed significant for any other reason: death; initial or prolonged inpatient hospitalization; life-threatening experience (immediate risk of dying); persistent or significant disability/incapacity; congenital anomaly. AEs included both SAEs and non-serious AEs. AE severity was graded as follows: Grade 1: mild; Grade 2: moderate; Grade 3: severe; Grade 4: life-threatening; Grade 5: fatal. A TEAE was considered related if in the opinion of the Investigator it was possibly or probably caused by the study drug. A summary of other non-serious AEs and all SAEs, regardless of causality is located in the Adverse Events module. (NCT00803205)
Timeframe: Baseline up to 4 Weeks Post-Treatment (Week 52) or Premature Discontinuation (PD)
| Intervention | percent of participants (Number) | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| At least 1 TEAE | Grade 1 TEAE | Grade 2 TEAE | Grade 3 TEAE | Grade 4 TEAE | Grade 5 TEAE | Unrelated TEAE | Unlikely related TEAE | Possibly related TEAE | Probably related TEAE | Discontinuation due to TEAE | Serious TEAE | |
| Ataluren | 98.3 | 15.0 | 67.5 | 15.8 | 0 | 0 | 25.0 | 32.5 | 28.3 | 12.5 | 6.7 | 37.5 |
| Placebo | 97.5 | 16.9 | 55.1 | 25.4 | 0 | 0 | 35.6 | 26.3 | 29.7 | 5.9 | 2.5 | 40.7 |
During treatment, any intervention including hospitalization or use of oral, inhaled, or intravenous antibiotics was documented if it was due to an exacerbation-like episode. Participants and caregivers recorded interventions in an electronic diary. The rate of interventions was defined as the total days with interventions divided by the total study duration. (NCT00803205)
Timeframe: Baseline up to EOT (Week 48)
| Intervention | days with interventions per study (Mean) | |
|---|---|---|
| Hospitalization | Use of Antibiotics | |
| Ataluren | 0.010 | 0.220 |
| Placebo | 0.021 | 0.245 |
"Study drug compliance was assessed by using a Pharmacy Subject Study Drug Accountability Log (completed by the investigational site personnel). The rate of compliance was defined as 100 * (number of sachets taken/number of planned sachets) during the study. All calculations were based on the records of the first dose date to the last dose date. To differentiate dose strengths while maintaining the blind, each kit had a unique kit number and had prominent lettering A and B. Each kit contained 65 packets of 1 of the dose strengths (125, 250, or 1000 mg or matching placebo). Labeling for active drug and placebo was identical." (NCT00803205)
Timeframe: Baseline up to EOT (Week 48)
| Intervention | percent of doses taken (Median) | |
|---|---|---|
| Drug Kit A | Drug Kit B | |
| Ataluren | 90.149 | 90.830 |
| Placebo | 85.119 | 86.614 |
(NCT01225211)
Timeframe: Cohort 1: Baseline, Day 14
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Cohort 1: LUM 200 mg qd - Period 1 | -4.442 |
| Cohort 1: Placebo - Period 1 | -1.668 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. (NCT01225211)
Timeframe: Cohort 1: Day 14, Day 21
| Intervention | liters (Least Squares Mean) |
|---|---|
| Cohort 1: LUM 200 mg qd+IVA 150 mg q12h - Period 2 | 0.128 |
| Cohort 1: LUM 200 mg qd+IVA 250 mg q12h - Period 2 | 0.015 |
| Cohort 1: Placebo - Period 2 | -0.046 |
FEV1 and ppFEV1 are defined in Outcome Measure 6. (NCT01225211)
Timeframe: Cohort 1: Day 14, Day 21
| Intervention | percent predicted of FEV1 (Least Squares Mean) |
|---|---|
| Cohort 1: LUM 200 mg qd+IVA 150 mg q12h - Period 2 | 3.46 |
| Cohort 1: LUM 200 mg qd+IVA 250 mg q12h - Period 2 | 0.63 |
| Cohort 1: Placebo - Period 2 | -1.44 |
(NCT01225211)
Timeframe: Cohort 1: Day 14, Day 21
| Intervention | millimole per liter (mmol/L) (Least Squares Mean) |
|---|---|
| Cohort 1: LUM 200 mg qd+IVA 150 mg q12h - Period 2 | -2.131 |
| Cohort 1: LUM 200 mg qd+IVA 250 mg q12h - Period 2 | -9.128 |
| Cohort 1: Placebo - Period 2 | 0.548 |
(NCT01225211)
Timeframe: Cohort 2: Baseline, Day 14
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Cohort 2: LUM 200 mg qd - Period 1 | -6.490 |
| Cohort 2: LUM 400 mg qd - Period 1 | -5.901 |
| Cohort 2: LUM 600 mg qd (HO) - Period 1 | -9.442 |
| Cohort 2: LUM 600 mg qd (HE) - Period 1 | -3.137 |
| Cohort 3: LUM 400 mg q12h - Period 1 | -9.179 |
| Cohort 2 and 3: Placebo (HO and HE) - Period 1 | 0.048 |
The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for subjects with cystic fibrosis. Respiratory domain assessed respiratory symptoms (for example, coughing, congestion, wheezing), score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. (NCT01225211)
Timeframe: Cohort 2 and 3: Day 28, Day 56
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Cohort 2: LUM 200 mg qd+IVA 250 mg q12h (HO) - Period 2 | 3.3 |
| Cohort 2: LUM 400 mg qd+IVA 250 mg q12h (HO) - Period 2 | 7.9 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HO) - Period 2 | 8.9 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HE) - Period 2 | 5.5 |
| Cohort 3: LUM 400 mg q12h+IVA 250 mg q12h (HO) - Period 2 | 11.2 |
| Cohort 2 and 3: Placebo (HO and HE) - Period 2 | -8.6 |
FEV1 and ppFEV1 are defined in Outcome Measure 6. (NCT01225211)
Timeframe: Cohort 2 and 3: Day 28, Day 56
| Intervention | percent predicted of FEV1 (Least Squares Mean) |
|---|---|
| Cohort 2: LUM 200 mg qd+IVA 250 mg q12h (HO) - Period 2 | 1.96 |
| Cohort 2: LUM 400 mg qd+IVA 250 mg q12h (HO) - Period 2 | 1.99 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HO) - Period 2 | 6.15 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HE) - Period 2 | 2.29 |
| Cohort 3: LUM 400 mg q12h+IVA 250 mg q12h (HO) - Period 2 | 6.09 |
| Cohort 2 and 3: Placebo (HO and HE) - Period 2 | -1.57 |
(NCT01225211)
Timeframe: Cohort 2 and 3: Day 28, Day 56
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Cohort 2: LUM 200 mg qd+IVA 250 mg q12h (HO) - Period 2 | 0.321 |
| Cohort 2: LUM 400 mg qd+IVA 250 mg q12h (HO) - Period 2 | -1.043 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HO) - Period 2 | -2.900 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HE) - Period 2 | -1.240 |
| Cohort 3: LUM 400 mg q12h+IVA 250 mg q12h (HO) - Period 2 | -2.154 |
| Cohort 2 and 3: Placebo (HO and HE) - Period 2 | 1.627 |
FEV1 and ppFEV1 are defined in Outcome Measure 6. (NCT01225211)
Timeframe: Cohort 2 and 3: Day 28, Day 56
| Intervention | percent change (Least Squares Mean) |
|---|---|
| Cohort 2: LUM 200 mg qd+IVA 250 mg q12h (HO) - Period 2 | 3.13 |
| Cohort 2: LUM 400 mg qd+IVA 250 mg q12h (HO) - Period 2 | 2.98 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HO) - Period 2 | 9.70 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HE) - Period 2 | 4.30 |
| Cohort 3: LUM 400 mg q12h+IVA 250 mg q12h (HO) - Period 2 | 8.24 |
| Cohort 2 and 3: Placebo (HO and HE) - Period 2 | -2.05 |
BMI was defined as weight in kilogram (kg) divided by height*height in square meter (m^2). (NCT01225211)
Timeframe: Cohort 4: Baseline, Day 56
| Intervention | kg/m^2 (Least Squares Mean) |
|---|---|
| Cohort 4: Placebo | 0.08 |
| Cohort 4: LUM 400 mg q12h+IVA 250 mg q12h | -0.04 |
CFQ-R respiratory domain is defined in Outcome Measure 17. (NCT01225211)
Timeframe: Cohort 4: Baseline, Day 56
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Cohort 4: Placebo | -0.82 |
| Cohort 4: LUM 400 mg q12h+IVA 250 mg q12h | 5.66 |
FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. ppFEV1 (predicted for age, gender, and height) was calculated using the Hankinson method. (NCT01225211)
Timeframe: Cohort 4: Baseline, Day 56
| Intervention | percent predicted of FEV1 (Least Squares Mean) |
|---|---|
| Cohort 4: Placebo | -1.23 |
| Cohort 4: LUM 400 mg q12h+IVA 250 mg q12h | -0.62 |
(NCT01225211)
Timeframe: Cohort 4: Baseline, Day 56
| Intervention | mmol/L (Least Squares Mean) |
|---|---|
| Cohort 4: Placebo | -0.78 |
| Cohort 4: LUM 400 mg q12h+IVA 250 mg q12h | -11.82 |
(NCT01225211)
Timeframe: Cohort 4: Baseline, Day 56
| Intervention | kg (Least Squares Mean) |
|---|---|
| Cohort 4: Placebo | 0.16 |
| Cohort 4: LUM 400 mg q12h+IVA 250 mg q12h | -0.11 |
FEV1 and ppFEV1 are defined in Outcome Measure 6. (NCT01225211)
Timeframe: Cohort 4: Baseline, Day 56
| Intervention | percent change (Least Squares Mean) |
|---|---|
| Cohort 4: Placebo | -2.20 |
| Cohort 4: LUM 400 mg q12h+IVA 250 mg q12h | -0.69 |
AE: any untoward medical occurrence in a participant during study; irrespective of relationship with treatment. This includes any newly occurring event or previous condition that has increased in severity or frequency after informed consent. AE includes serious AEs (SAEs) as well as Non-SAEs. SAE (subset of AE): medical event or condition, which falls into any of the following categories, regardless of its relationship to study drug: death, life threatening adverse experience, in-patient hospitalization/prolongation of hospitalization, persistent/significant disability or incapacity, congenital anomaly/birth defect, important medical event. Number of participants with AEs and SAEs are reported. AE that started at/after initial dosing of study drug, or increased in severity after initial dosing of study drug is considered treatment-emergent. Results are reported separately for monotherapy period (Period 1: Day 1 to Day 14) and combination therapy period (Period 2: Day 15 to Day 21). (NCT01225211)
Timeframe: Cohort 1: Day 1 up to 28 days after last dose (Last dose = Day 21)
| Intervention | participants (Number) | |
|---|---|---|
| Participants with any AEs | Participants with SAEs | |
| Cohort 1: LUM 200 mg qd - Period 1 | 29 | 0 |
| Cohort 1: LUM 200 mg qd+IVA 150 mg q12h - Period 2 | 14 | 0 |
| Cohort 1: LUM 200 mg qd+IVA 250 mg q12h - Period 2 | 12 | 0 |
| Cohort 1: Placebo - Period 1 | 12 | 0 |
| Cohort 1: Placebo - Period 2 | 15 | 0 |
FEV1 and ppFEV1 are defined in Outcome Measure 6. (NCT01225211)
Timeframe: Cohort 2 and 3: Baseline, Day 28 and 56
| Intervention | percent predicted of FEV1 (Least Squares Mean) | |
|---|---|---|
| Day 28: (n= 21, 20, 20, 18, 11, 27) | Day 56: (n= 21, 20, 20, 17, 10, 24) | |
| Cohort 2 and 3: Placebo (HO and HE) | -0.03 | -2.02 |
| Cohort 2: LUM 200 mg qd/LUM 200 mg qd+IVA 250 mg q12h (HO) | 0.21 | 1.82 |
| Cohort 2: LUM 400 mg qd/LUM 400 mg qd+IVA 250 mg q12h (HO) | -1.35 | 0.64 |
| Cohort 2: LUM 600 mg qd/LUM 600 mg qd+IVA 250 mg q12h (HE) | -3.82 | -1.68 |
| Cohort 2: LUM 600 mg qd/LUM 600 mg qd+IVA 250 mg q12h (HO) | -2.62 | 3.59 |
| Cohort 3: LUM 400 mg q12h/LUM 400 mg q12h+IVA 250 mg q12h (HO) | -4.52 | 2.16 |
FEV1 and ppFEV1 are defined in Outcome Measure 6. (NCT01225211)
Timeframe: Cohort 2 and 3: Baseline, Day 28 and 56
| Intervention | percent change (Least Squares Mean) | |
|---|---|---|
| Day 28: (n= 21, 20, 20, 18, 11, 27) | Day 56: (n= 21, 20, 20, 17, 10, 24) | |
| Cohort 2 and 3: Placebo (HO and HE) | 1.89 | -2.42 |
| Cohort 2: LUM 200 mg qd/LUM 200 mg qd+IVA 250 mg q12h (HO) | 0.24 | 2.51 |
| Cohort 2: LUM 400 mg qd/LUM 400 mg qd+IVA 250 mg q12h (HO) | -1.15 | 1.72 |
| Cohort 2: LUM 600 mg qd/LUM 600 mg qd+IVA 250 mg q12h (HE) | -5.46 | -2.34 |
| Cohort 2: LUM 600 mg qd/LUM 600 mg qd+IVA 250 mg q12h (HO) | -3.13 | 5.55 |
| Cohort 3: LUM 400 mg q12h/LUM 400 mg q12h+IVA 250 mg q12h (HO) | -6.39 | 2.96 |
Detailed description is provided in Outcome Measure 1. Results are reported separately for monotherapy period (Period 1: Day 1 to Day 28) and combination therapy period (Period 2: Day 29 to Day 56). (NCT01225211)
Timeframe: Cohort 2 and 3: Day 1 up to 28 days after last dose (Last dose = Day 56)
| Intervention | participants (Number) | |
|---|---|---|
| Participants with any AEs | Participants with SAEs | |
| Cohort 2 and 3: Placebo (HO and HE) - Period 1 | 23 | 1 |
| Cohort 2 and 3: Placebo (HO and HE) - Period 2 | 20 | 4 |
| Cohort 2: LUM 200 mg qd - Period 1 | 18 | 2 |
| Cohort 2: LUM 200 mg qd+IVA 250 mg q12h (HO) - Period 2 | 12 | 0 |
| Cohort 2: LUM 400 mg qd - Period 1 | 18 | 0 |
| Cohort 2: LUM 400 mg qd+IVA 250 mg q12h (HO) - Period 2 | 15 | 1 |
| Cohort 2: LUM 600 mg qd - Period 1 | 37 | 3 |
| Cohort 2: LUM 600 mg qd+IVA 250 mg q12h (HO&HE) - Period 2 | 26 | 4 |
| Cohort 3: LUM 400 mg q12h - Period 1 | 7 | 2 |
| Cohort 3: LUM 400 mg q12h+IVA 250 mg q12h (HO) - Period 2 | 10 | 1 |
AEs and SAEs are defined in Outcome Measure 1. (NCT01225211)
Timeframe: Cohort 4: Day 1 up to 28 days after last dose (Last dose = Day 56)
| Intervention | participants (Number) | |
|---|---|---|
| Participants with any AEs | Participants with SAEs | |
| Cohort 4: Active Study Drug | 52 | 9 |
| Cohort 4: Placebo | 53 | 5 |
BMI = (Weight [in kg]) divided by (Stature [in meters])^2. Data was reported as per the dose received and for overall participants. (NCT01705145)
Timeframe: Baseline, Week 24
| Intervention | kilogram per square meter (kg/m^2) (Mean) |
|---|---|
| Part B: Ivacaftor 50 mg | 0.332 |
| Part B: Ivacaftor 75 mg | 0.314 |
| Part B: Overall Ivacaftor | 0.319 |
Stature was measured as height if children could stand unassisted and follow directions; otherwise, stature was measured as length. Data was reported as per the dose received and for overall participants. (NCT01705145)
Timeframe: Part B: Baseline, Week 24
| Intervention | centimeters (cm) (Mean) |
|---|---|
| Part B: Ivacaftor 50 mg | 2.5 |
| Part B: Ivacaftor 75 mg | 3.5 |
| Part B: Overall Ivacaftor | 3.3 |
Sweat samples were collected using an approved Macroduct (Wescor, Logan, Utah) collection device. A volume of greater than or equal to (>=) 15 microliter was required for determination of sweat chloride. Data was reported as per the dose received and for overall participants. (NCT01705145)
Timeframe: Part B: Baseline, Week 24
| Intervention | millimole per liter (mmol/L) (Mean) |
|---|---|
| Part B: Ivacaftor 50 mg | -47.07 |
| Part B: Ivacaftor 75 mg | -46.78 |
| Part B: Overall Ivacaftor | -46.86 |
Data was reported as per the dose received and for overall participants. (NCT01705145)
Timeframe: Part B: Baseline, Week 24
| Intervention | kilograms (kg) (Mean) |
|---|---|
| Part B: Ivacaftor 50 mg | 1.00 |
| Part B: Ivacaftor 75 mg | 1.50 |
| Part B: Overall Ivacaftor | 1.36 |
"AE: any adverse change from participant's baseline (pre-treatment) condition, including any adverse experience, abnormal recording/clinical laboratory assessment which occurs during course of study, whether it is considered related to study drug or not. SAE: medical event or condition, which falls into any of following categories, regardless of its relationship to the study drug: death, life threatening adverse experience, in-patient hospitalization/prolonged hospitalization, persistent/significant disability/incapacity, congenital anomaly/birth defect, important medical event.~Related AEs includes all AEs for which the causality was either related to study drug or possibly related to study drug. Data was reported as per the dose received and for overall participants." (NCT01705145)
Timeframe: Part A: Up to 93 Days
| Intervention | participants (Number) | ||
|---|---|---|---|
| AEs | SAEs | Related AEs | |
| Part A: Ivacaftor 50 mg | 3 | 0 | 1 |
| Part A: Ivacaftor 75 mg | 5 | 0 | 3 |
| Part A: Overall Ivacaftor | 8 | 0 | 4 |
Plasma concentration was reported for ivacaftor and its metabolites (hydroxymethyl ivacaftor [M1] and ivacaftor carboxylate [M6]) up to 24 hours post-dose on Day 4 (Hour 0 [pre-dose] on Day 1 and Day 4; 2, 3, 6, 24 hours post-dose on Day 4). Data was planned to be reported for overall participants in the period. (NCT01705145)
Timeframe: Part A: up to 24 hours post-dose on Day 4
| Intervention | nanogram per milliliter (ng/mL) (Mean) | |||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Ivacaftor: Hour 0 on Day 1 | Ivacaftor: Hour 0 on Day 4 | Ivacaftor: 2 Hours Post-Dose on Day 4 | Ivacaftor: 3 Hours Post-Dose on Day 4 | Ivacaftor: 6 Hours Post-Dose on Day 4 | Ivacaftor: 24 Hours Post-Dose on Day 4 | M1: Hour 0 on Day 1 | M1: Hour 0 on Day 4 | M1: 2 Hours Post-Dose on Day 4 | M1: 3 Hours Post-Dose on Day 4 | M1: 6 Hours Post-Dose on Day 4 | M1: 24 Hours Post-Dose on Day 4 | M6: Hour 0 on Day 1 | M6: Hour 0 on Day 4 | M6: 2 Hours Post-Dose on Day 4 | M6: 3 Hours Post-Dose on Day 4 | M6: 6 Hours Post-Dose on Day 4 | M6: 24 Hours Post-Dose on Day 4 | |
| Part A: Overall Ivacaftor | 0.00 | 396 | 726 | 957 | 542 | 124 | 0.00 | 1240 | 1540 | 2310 | 1580 | 389 | 0.00 | 1150 | 1050 | 1300 | 1390 | 439 |
"AE: any adverse change from participant's baseline (pre-treatment) condition, including any adverse experience, abnormal recording/clinical laboratory assessment which occurs during course of study, whether it is considered related to study drug or not. AE includes both serious and non-serious AE. SAE: medical event or condition, which falls into any of following categories, regardless of its relationship to the study drug: death, life threatening adverse experience, in-patient hospitalization/prolonged hospitalization, persistent/significant disability/incapacity, congenital anomaly/birth defect, important medical event.~Related AEs includes all AEs for which the causality was either related to study drug or possibly related to study drug. Data was reported as per the dose received." (NCT01705145)
Timeframe: Part B: Up to 28 Weeks
| Intervention | participants (Number) | ||
|---|---|---|---|
| AEs | SAEs | Related AEs | |
| Part B: Ivacaftor 50 mg | 10 | 3 | 3 |
| Part B: Ivacaftor 75 mg | 23 | 3 | 8 |
| Part B: Overall Ivacaftor | 33 | 6 | 11 |
Plasma concentration was reported for ivacaftor and its metabolites (M1 and M6) up to 24 hours post-dose on Day 168 (Hour 0 [predose] on Day 1, 14, 56, 112, and 168; 2, 3, 6 hours post-dose on Day 14; 1 hour post-dose on Day 56; 4, 6 hours post-dose on Day 112; 24 hours post-dose on Day 168). Data was planned to be reported for overall participants in the period. (NCT01705145)
Timeframe: Part B: up to 24 hours post-dose on Day 168
| Intervention | ng/mL (Mean) | |||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Ivacaftor: Hour 0 on Day 1 | Ivacaftor: Hour 0 on Day 14 | Ivacaftor: 2 Hours Post-Dose on Day 14 | Ivacaftor: 3 Hours Post-Dose on Day 14 | Ivacaftor: 6 Hours Post-Dose on Day 14 | Ivacaftor: Hour 0 on Day 56 | Ivacaftor: 1 Hour Post-Dose on Day 56 | Ivacaftor: Hour 0 on Day 112 | Ivacaftor: 4 Hours Post-Dose on Day 112 | Ivacaftor: 6 Hours Post-Dose on Day 112 | Ivacaftor: Hour 0 on Day 168 | Ivacaftor: 24 Hours Post-Dose on Day 168 | M1: Hour 0 on Day 1 | M1: Hour 0 on Day 14 | M1: 2 Hours Post-Dose on Day 14 | M1: 3 Hours Post-Dose on Day 14 | M1: 6 Hours Post-Dose on Day 14 | M1: Hour 0 on Day 56 | M1: 1 Hour Post-Dose on Day 56 | M1: Hour 0 on Day 112 | M1: 4 Hours Post-Dose on Day 112 | M1: 6 Hours Post-Dose on Day 112 | M1: Hour 0 on Day 168 | M1: 24 Hours Post-Dose on Day 168 | M6: Hour 0 on Day 1 | M6: Hour 0 on Day 14 | M6: 2 Hours Post-Dose on Day 14 | M6: 3 Hours Post-Dose on Day 14 | M6: 6 Hours Post-Dose on Day 14 | M6: Hour 0 on Day 56 | M6: 1 Hour Post-Dose on Day 56 | M6: Hour 0 on Day 112 | M6: 4 Hours Post-Dose on Day 112 | M6: 6 Hours Post-Dose on Day 112 | M6: Hour 0 on Day 168 | M6: 24 Hours Post-Dose on Day 168 | |
| Part B: Overall Ivacaftor | 0.00 | 614 | 932 | 1080 | 1140 | 448 | 514 | 596 | 1080 | 1010 | 500 | 207 | 0.00 | 1580 | 1870 | 2280 | 2670 | 1340 | 1170 | 1680 | 2450 | 2500 | 1460 | 602 | 0.00 | 1520 | 1430 | 1630 | 2090 | 1510 | 1310 | 1660 | 1810 | 2130 | 1520 | 632 |
Subjects will perform multibreath washout testing using standard techniques to measure functional residual capacity and lung clearance index at the beginning and end of each study period. (NCT01784419)
Timeframe: 14 +/- 2 days
| Intervention | Participants (Count of Participants) |
|---|---|
| All Study Participants | NA |
Standard spirometry will be performed at the start and end of each 2 week study period. Subjects will take study drug (ivacaftor or placebo) during each study period. (NCT01784419)
Timeframe: 14 +/- 2 days
| Intervention | Participants (Count of Participants) |
|---|---|
| All Study Participants | NA |
Sweat chloride concentration measured by pilocarpine iontophoresis, a standard clinical laboratory technique. Sweat collection accomplished with the Wescor Macroduct system. Sweat chloride is measured at the start and end of each study period. There are two study periods during which subjects take either ivacaftor or placebo. (NCT01784419)
Timeframe: 14 +/- 2 days
| Intervention | mmol/L (Mean) |
|---|---|
| All Study Participants | NA |
Change in PA spine bone mineral density (NCT01549314)
Timeframe: Baseline and 24 months
| Intervention | g/cm2 (Mean) |
|---|---|
| Subjects With CF Taking Ivacaftor | 0.032 |
| Subjects With CF Not Taking Ivacaftor | 0.024 |
| Healthy Subjects | 0.029 |
Change in cortical volumetric bone mineral density at the radius (NCT01549314)
Timeframe: Baseline and 24 months
| Intervention | mgHA/cm3 (Mean) |
|---|---|
| Subjects With CF Taking Ivacaftor | 44.6 |
| Subjects With CF Not Taking Ivacaftor | 40.1 |
| Healthy Subjects | 43.7 |
Change in osteocalcin (NCT01549314)
Timeframe: Baseline and 24 months
| Intervention | ng/ml (Mean) |
|---|---|
| Subjects With CF Taking Ivacaftor | -5.36 |
| Subjects With CF Not Taking Ivacaftor | -4.51 |
| Healthy Subjects | -4.5 |
The CFQ-R is a validated patient-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms (for example, coughing, congestion, wheezing), score range: 0-100; Higher scores indicating fewer symptoms and better health-related quality of life. (NCT00953706)
Timeframe: Part A baseline through Week 16
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| Placebo - Part A | -1.4 |
| Ivacaftor - Part A | -0.1 |
Spirometry (as measured by ppFEV1) is a standardized assessment to evaluate lung function that is the most widely used endpoint in cystic fibrosis studies. FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. ppFEV1 (predicted for age, gender, and height) was calculated using the Knudson method. (NCT00953706)
Timeframe: Part A baseline through Week 16
| Intervention | percent predicted of FEV1 (Least Squares Mean) |
|---|---|
| Placebo - Part A | -0.2 |
| Ivacaftor - Part A | 1.5 |
The sweat chloride (quantitative pilocarpine iontophoresis) test is a standard diagnostic tool for cystic fibrosis (CF), serving as an indicator of cystic fibrosis transmembrane conductance regulator (CFTR) activity. (NCT00953706)
Timeframe: Part A baseline through Week 16
| Intervention | millimole per liter (mmol/L) (Least Squares Mean) |
|---|---|
| Placebo - Part A | 0.1 |
| Ivacaftor - Part A | -2.7 |
As malnutrition is common in participants with cystic fibrosis (CF) because of increased energy expenditures due to lung disease and fat malabsorption, body weight is an important clinical measure of nutritional status. (NCT00953706)
Timeframe: Part A baseline through Week 16
| Intervention | kilograms per 112 days (Least Squares Mean) |
|---|---|
| Placebo - Part A | 0.9 |
| Ivacaftor - Part A | 0.8 |
Pulmonary exacerbation was defined as new, or changed, antibiotic therapy (intravenous, inhaled, or oral) for any 4 or more of the following signs/symptoms: change in sputum; new or increased hemoptysis; increased cough; increased dyspnea; malaise, fatigue, or lethargy; temperature above 38 degrees Celsius; anorexia or weight loss; sinus pain or tenderness; change in sinus discharge; change in physical examination of the chest; decrease in pulmonary function by 10 percent (%); and radiographic changes indicative of pulmonary infection. (NCT00953706)
Timeframe: Part B baseline through Week 64
| Intervention | participants (Number) |
|---|---|
| Placebo/Ivacaftor - Part B | 4 |
| Ivacaftor/Ivacaftor - Part B | 16 |
Pulmonary exacerbation was defined as new, or changed, antibiotic therapy (intravenous, inhaled, or oral) for any 4 or more of the following signs/symptoms: change in sputum; new or increased hemoptysis; increased cough; increased dyspnea; malaise, fatigue, or lethargy; temperature above 38 degrees Celsius; anorexia or weight loss; sinus pain or tenderness; change in sinus discharge; change in physical examination of the chest; decrease in pulmonary function by 10 percent (%); and radiographic changes indicative of pulmonary infection. (NCT00953706)
Timeframe: Part B baseline through Week 64
| Intervention | events (Number) |
|---|---|
| Placebo/Ivacaftor - Part B | 6 |
| Ivacaftor/Ivacaftor - Part B | 26 |
Pulmonary exacerbation was defined as new, or changed, antibiotic therapy (intravenous, inhaled, or oral) for any 4 or more of the following signs/symptoms: change in sputum; new or increased hemoptysis; increased cough; increased dyspnea; malaise, fatigue, or lethargy; temperature above 38 degrees Celsius; anorexia or weight loss; sinus pain or tenderness; change in sinus discharge; change in physical examination of the chest; decrease in pulmonary function by 10 percent (%); and radiographic changes indicative of pulmonary infection. (NCT00953706)
Timeframe: Part B baseline through Week 64
| Intervention | events per participant per year (Number) |
|---|---|
| Placebo/Ivacaftor - Part B | 1.10 |
| Ivacaftor/Ivacaftor - Part B | 0.82 |
ppFEV1 is defined in Outcome Measure 1. (NCT00953706)
Timeframe: Part A baseline through Week 64
| Intervention | percent predicted of FEV1 per 448 days (Least Squares Mean) |
|---|---|
| Placebo/Ivacaftor - Part B | 5.7445 |
| Ivacaftor/Ivacaftor - Part B | -1.0738 |
ppFEV1 is defined in Outcome Measure 1. (NCT00953706)
Timeframe: Part B baseline through Week 64
| Intervention | percent predicted of FEV1 per 336 days (Least Squares Mean) |
|---|---|
| Placebo/Ivacaftor - Part B | 5.3409 |
| Ivacaftor/Ivacaftor - Part B | -5.2994 |
The CFQ-R is a validated patient-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms (for example, coughing, congestion, wheezing), score range: 0-100; Higher scores indicating fewer symptoms and better health-related quality of life. (NCT00953706)
Timeframe: Change from Part A baseline: Part A Baseline, Week 64; Change from Part B baseline: Part B Baseline (Week 16), Week 64
| Intervention | units on a scale (Mean) | |
|---|---|---|
| Change From Part A Baseline at Week 64 | Change From Part B Baseline at Week 64 | |
| Ivacaftor/Ivacaftor - Part B | 1.50 | 2.62 |
| Placebo/Ivacaftor - Part B | 2.10 | 2.08 |
ppFEV1 is defined in Outcome Measure 1. (NCT00953706)
Timeframe: Change from Part A baseline: Part A Baseline, Week 64; Change from Part B baseline: Part B Baseline (Week 16), Week 64
| Intervention | percent predicted of FEV1 (Mean) | |
|---|---|---|
| Change From Part A Baseline at Week 64 | Change From Part B Baseline at Week 64 | |
| Ivacaftor/Ivacaftor - Part B | 2.7233 | -5.0565 |
| Placebo/Ivacaftor - Part B | 8.9398 | 3.5593 |
The sweat chloride (quantitative pilocarpine iontophoresis) test is a standard diagnostic tool for cystic fibrosis (CF), serving as an indicator of cystic fibrosis transmembrane conductance regulator (CFTR) activity. (NCT00953706)
Timeframe: Change from Part A baseline: Part A Baseline, Week 64; Change from Part B baseline: Part B Baseline (Week 16), Week 64
| Intervention | mmol/L (Mean) | |
|---|---|---|
| Change From Part A Baseline at Week 64 | Change From Part B Baseline at Week 64 | |
| Ivacaftor/Ivacaftor - Part B | -3.65 | -2.44 |
| Placebo/Ivacaftor - Part B | -7.13 | -3.88 |
As malnutrition is common in patients with cystic fibrosis (CF) because of increased energy expenditures due to lung disease and fat malabsorption, body weight is an important clinical measure of nutritional status. (NCT00953706)
Timeframe: Change from Part A baseline: Part A Baseline, Week 64; Change from Part B baseline: Part B Baseline (Week 16), Week 64
| Intervention | kilograms (kg) (Mean) | |
|---|---|---|
| Change From Part A Baseline at Week 64 | Change From Part B Baseline at Week 64 | |
| Ivacaftor/Ivacaftor - Part B | 2.35 | 1.45 |
| Placebo/Ivacaftor - Part B | 3.00 | 1.28 |
"Average radio tracer clearance through 90 minutes (MCC90) is primary index of mucociliary clearance at each study.~Primary study outcome: is absolute change in MCC90 between baseline and at end of treatment (where MCC measured 8-12 hours after final dose of study drug) - reflects sustained impact on MCC" (NCT01031706)
Timeframe: Baseline versus after completion of 4 week treatment period
| Intervention | percent clearance (Mean) |
|---|---|
| Hypertonic Saline | 2.77 |
| Placebo | -2.35 |
Absolute change in % predicted FEV1 between baseline and after 4 weeks of treatment calculated (NCT01031706)
Timeframe: Baseline and after 4 weeks of treatment
| Intervention | Percentage of predicted FEV1 (Mean) |
|---|---|
| Hypertonic Saline | 3.38 |
| Placebo | 1.09 |
Change from baseline in FEV1(maximal amount of air you can forcefully exhale in one second) % predicted (NCT01355796)
Timeframe: Baseline and 14 days
| Intervention | percentage of predicted (Mean) |
|---|---|
| Xylitol | -0.1 |
| Saline | 1.4 |
Difference from baseline in density of Pseudomonas aeruginosa colonization per gram of sputum, (NCT01355796)
Timeframe: baseline and 14 days
| Intervention | Log colony forming units (Mean) |
|---|---|
| Xylitol | -1.98 |
| Saline | 0.93 |
90 reviews available for iodine and Cystic Fibrosis
| Article | Year |
|---|---|
Adverse effects of iodides on thyroid function.
Topics: Cystic Fibrosis; Drug Synergism; Female; Goiter; Graves Disease; Humans; Hyperthyroidism; Hypothyroi | 1975 |
National Guidelines for the Performance of the Sweat Test in Diagnosis of Cystic Fibrosis on behalf of the Croatian Society of Medical Biochemistry and Laboratory Medicine and the Cystic Fibrosis Centre - Paediatrics and adults, University Hospital Centre
Topics: Child; Cystic Fibrosis; Hospitals, University; Humans; Infant, Newborn; Laboratories; Pediatrics; Sw | 2022 |
[Therapeutic advances in cystic fibrosis: from genetics to treatment personalized].
Topics: Aminophenols; Anti-Bacterial Agents; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic F | 2022 |
[Therapeutic advances in cystic fibrosis: from genetics to treatment personalized].
Topics: Aminophenols; Anti-Bacterial Agents; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic F | 2022 |
[Therapeutic advances in cystic fibrosis: from genetics to treatment personalized].
Topics: Aminophenols; Anti-Bacterial Agents; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic F | 2022 |
[Therapeutic advances in cystic fibrosis: from genetics to treatment personalized].
Topics: Aminophenols; Anti-Bacterial Agents; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic F | 2022 |
Sweat conductivity diagnostic accuracy for cystic fibrosis: a systematic review and meta-analysis.
Topics: Cystic Fibrosis; Humans; Prospective Studies; Retrospective Studies; Sensitivity and Specificity; Sw | 2023 |
Current and future diagnosis of cystic fibrosis: Performance and limitations.
Topics: Computational Biology; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Databas | 2020 |
Genetic diagnosis in practice: From cystic fibrosis to CFTR-related disorders.
Topics: Algorithms; Bronchiectasis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Ex | 2020 |
Sodium Status and Replacement in Children and Adults Living with Cystic Fibrosis: A Narrative Review.
Topics: Adult; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator | 2020 |
Diagnostic tools and CFTR functional assays in cystic fibrosis: utility and availability in Switzerland.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; S | 2021 |
Advances in the Diagnosis and Management of Cystic Fibrosis in the Genomic Era.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Databases, Genetic; | 2018 |
Newborn screening for cystic fibrosis: Is there benefit for everyone?
Topics: Aminophenols; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2019 |
Is it cystic fibrosis? The challenges of diagnosing cystic fibrosis.
Topics: Algorithms; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced | 2019 |
The implications and management of cystic fibrosis screen positive, inconclusive diagnosis patients.
Topics: Chlorides; Cost of Illness; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Di | 2019 |
Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adolescent; Adult; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Co | 2013 |
Sixty-five years since the New York heat wave: advances in sweat testing for cystic fibrosis.
Topics: Biomarkers; Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Cystic Fibrosis Transmembrane Cond | 2014 |
Sweat: a sample with limited present applications and promising future in metabolomics.
Topics: Biomarkers; Cystic Fibrosis; Doping in Sports; Humans; Metabolomics; Substance Abuse Detection; Swea | 2014 |
Cystic fibrosis: need for mass deployable screening methods.
Topics: Adenosine Triphosphate; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regula | 2014 |
Biomarkers in Paediatric Cystic Fibrosis Lung Disease.
Topics: Antibodies, Bacterial; Biomarkers; Breath Tests; Bronchoalveolar Lavage Fluid; Child; Cystic Fibrosi | 2015 |
Question 7: For an infant with an equivocal sweat chloride following newborn screening, how likely is a diagnosis of cystic fibrosis?
Topics: Chlorides; Cystic Fibrosis; Humans; Infant, Newborn; Neonatal Screening; Sweat | 2016 |
Cystic fibrosis: newborn screening in America.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Exocr | 2008 |
Newborn screening for cystic fibrosis.
Topics: Algorithms; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Ana | 2008 |
Emerging issues in cystic fibrosis newborn screening.
Topics: Algorithms; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Testing; H | 2010 |
Newborn screening for cystic fibrosis.
Topics: Biomarkers; Cystic Fibrosis; False Positive Reactions; Genetic Carrier Screening; Humans; Infant, Ne | 2012 |
[Mucoviscidosis: CFTR mutation-specific therapy: a ray of sunshine in a cloudy sky].
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; E | 2013 |
Of sweat and bile.
Topics: Antiporters; Bile; Cystic Fibrosis; Saccharomyces cerevisiae Proteins; Sweat | 2002 |
[Genetics and cellular biology of cystic fibrosis].
Topics: Blood Proteins; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Hum | 2003 |
[Diagnosis of cystic fibrosis; simple genotyping to rule out the disease preferable to starting with the sweat test].
Topics: Cystic Fibrosis; DNA; Genotype; Humans; Iontophoresis; Mutation; Pancreatic Function Tests; Pilocarp | 2003 |
[MUCOVISCIDOSIS IN ADULTS].
Topics: Adult; Bronchitis; Cystic Fibrosis; Electroencephalography; Humans; Pancreas; Pathology; Respiratory | 1964 |
Laboratory tests for the diagnosis of cystic fibrosis.
Topics: Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, D | 2002 |
Evidence based guidelines for the performance of the sweat test for the investigation of cystic fibrosis in the UK.
Topics: Chlorides; Cystic Fibrosis; Evidence-Based Medicine; Humans; Infant, Newborn; Neonatal Screening; Pr | 2003 |
Diagnosis of CF despite normal or borderline sweat chloride.
Topics: Child; Chlorides; Cystic Fibrosis; DNA Mutational Analysis; Humans; Nasal Mucosa; Sweat | 2004 |
Diagnosis of cystic fibrosis after newborn screening: the Australasian experience--twenty years and five million babies later: a consensus statement from the Australasian Paediatric Respiratory Group.
Topics: Australasia; Australia; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regula | 2005 |
An unusual presentation of cystic fibrosis in an adult.
Topics: Acid-Base Equilibrium; Adult; Alkalosis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance | 2005 |
Sweat-testing: a review of current technical requirements.
Topics: Chlorides; Cystic Fibrosis; Electrolytes; False Positive Reactions; Humans; Infant, Newborn; Iontoph | 2005 |
Information flow after a positive newborn screening for cystic fibrosis.
Topics: Adult; Anxiety; Chlorides; Communication; Cystic Fibrosis; Female; Friends; Genetic Counseling; Huma | 2005 |
Cystic fibrosis: terminology and diagnostic algorithms.
Topics: Action Potentials; Algorithms; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance | 2006 |
New tests for cystic fibrosis.
Topics: Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, Differe | 2006 |
Guidelines for the performance of the sweat test for the diagnosis of cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Infant, Newborn; Neonatal Screening; Practice Guidelines as Topic; Quality | 2007 |
Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials.
Topics: Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Feasibility Studie | 2007 |
Cystic fibrosis.
Topics: Cost of Illness; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrolytes; | 2007 |
Painful wrinkles in the bathtub: association with hyperhidrosis and cystic fibrosis.
Topics: Adolescent; Adult; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA | 2008 |
Cystic fibrosis in adults: diagnostic and therapeutic aspects.
Topics: Adult; Anti-Bacterial Agents; Anti-Inflammatory Agents; Cystic Fibrosis; Diagnosis, Differential; Dr | 2008 |
Atypical cystic fibrosis and CFTR-related diseases.
Topics: Adolescent; Adult; Airway Obstruction; Aspergillosis, Allergic Bronchopulmonary; Aspergillus fumigat | 2008 |
Microprobe analysis in studies and diagnosis of cystic fibrosis.
Topics: Animals; Blood Proteins; Calcium; Calgranulin A; Child; Cyclic AMP; Cystic Fibrosis; Disease Models, | 1984 |
Laboratory diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Aging; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Disease; Electric Con | 1983 |
Development of a screening system for cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Hu | 1983 |
Cystic fibrosis--its biochemical detection.
Topics: Age Factors; Blood Proteins; Calgranulin A; Chemistry, Clinical; Clinical Enzyme Tests; Cystic Fibro | 1983 |
Diagnosis and treatment of cystic fibrosis. An update.
Topics: Adult; Child; Chlorides; Cystic Fibrosis; Female; Hemoptysis; Humans; Infant, Newborn; Liver Cirrhos | 1984 |
Advances in the diagnosis and management of cystic fibrosis.
Topics: Adolescent; Anti-Bacterial Agents; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infa | 1984 |
Cystic fibrosis: diagnostic considerations.
Topics: Cystic Fibrosis; Diagnosis, Differential; Electrolytes; Gastrointestinal Diseases; Humans; Liver; Re | 1982 |
Four clinical chemistry analyses for pediatric patients: glycosylated hemoglobin, free bilirubin, sweat electrolytes, neonatal thyroxine.
Topics: Bilirubin; Congenital Hypothyroidism; Cystic Fibrosis; Diabetes Mellitus, Type 1; Electrolytes; Glyc | 1982 |
Diagnosis of cystic fibrosis.
Topics: Chlorine; Cystic Fibrosis; Humans; Intestines; Medical History Taking; Phenotype; Sodium; Sweat | 1995 |
Delayed diagnosis of cystic fibrosis in children with a rare genotype (delta F508/R117H).
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Female; Gene Frequency; Genetic Carrier Screening; Gen | 1995 |
[Cystic fibrosis of the pancreas. Historical notes, sweat test, and neonatal screening].
Topics: Cystic Fibrosis; History, 18th Century; History, 19th Century; History, 20th Century; Humans; Infant | 1995 |
Cystic fibrosis for the primary care pediatrician.
Topics: Adolescent; Adult; Anti-Bacterial Agents; Anti-Inflammatory Agents; Child; Cystic Fibrosis; Female; | 1993 |
The application of saliva, sweat and tear fluid for diagnostic purposes.
Topics: Adrenal Gland Diseases; Cystic Fibrosis; Electrolytes; Humans; Lysosomal Storage Diseases; Saliva; S | 1993 |
Primary pulmonary care of the patient with cystic fibrosis.
Topics: Anti-Bacterial Agents; Child; Child Nutritional Physiological Phenomena; Child, Preschool; Cystic Fi | 1993 |
The diagnosis of cystic fibrosis.
Topics: Action Potentials; Bronchoalveolar Lavage; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Geno | 1997 |
Diagnosing cystic fibrosis: blood, sweat, and tears.
Topics: Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; Mutat | 1997 |
Nonneoplastic disorders of the eccrine glands.
Topics: Adult; Blister; Child; Coma; Cystic Fibrosis; Disease Susceptibility; Drug-Related Side Effects and | 1998 |
The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel.
Topics: Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Infant, | 1998 |
[Diagnostic difficulties in cystic fibrosis].
Topics: Cystic Fibrosis; Genotype; Humans; Sweat | 1999 |
[The diagnosis of cystic fibrosis in the adult].
Topics: Adolescent; Adult; Biological Transport, Active; Cystic Fibrosis; Cystic Fibrosis Transmembrane Cond | 2000 |
Fluid and electrolyte supplementation for exercise heat stress.
Topics: Adult; Aged; Body Temperature Regulation; Calcium Chloride; Climate; Cystic Fibrosis; Dehydration; D | 2000 |
[Diagnosis and management of cystic fibrosis in children].
Topics: Adult; Age Factors; Anti-Bacterial Agents; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transm | 2000 |
[Diagnosis of cystic fibrosis in adults].
Topics: Adolescent; Adult; Age Factors; Chlorides; Chronic Disease; Cystic Fibrosis; Cystic Fibrosis Transme | 2000 |
Hyponatremia associated with exercise: risk factors and pathogenesis.
Topics: Cystic Fibrosis; Digestive System; Drinking; Exercise; Extracellular Space; Female; Humans; Hyponatr | 2001 |
Cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembran | 2001 |
[From gene to disease; from defective chloride ion transport to cystic fibrosis].
Topics: Adult; Child; Chloride Channels; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulato | 2001 |
Recent advances in cystic fibrosis research.
Topics: Adolescent; Adult; Agglutination; Biological Transport; Child; Child, Preschool; Chlorides; Cilia; C | 1976 |
Cystic fibrosis. A dilemma in the metabolic pathogenesis of genetic disease.
Topics: Animals; Cells, Cultured; Cystic Fibrosis; Cytoplasm; Fibroblasts; Glycosaminoglycans; Humans; Immun | 1976 |
[Methods of electrolyte determination in sweat for diagnosis of mucoviscidosis].
Topics: Child; Cystic Fibrosis; Electric Conductivity; Electrolytes; Humans; Methods; Sweat | 1978 |
Cystic fibrosis: diagnosis, treatment, and prognosis.
Topics: Aerosols; Anti-Bacterial Agents; Cough; Cystic Fibrosis; Diet; Female; Humans; Infant, Newborn; Infa | 1979 |
Report of the committe for a study for evaluation of testing for cystic fibrosis.
Topics: Adult; Child; Cystic Fibrosis; Duodenum; Female; Financing, Government; Financing, Organized; Geneti | 1976 |
Cystic fibrosis.
Topics: Biological Transport; Cystic Fibrosis; Exocrine Glands; Female; Gastrointestinal Diseases; Genital D | 1976 |
Current biochemical approaches in cystic fibrosis research.
Topics: Biological Transport; Cilia; Cystic Fibrosis; Gastrointestinal Diseases; Glycoproteins; Heterozygote | 1976 |
Screening for cystic fibrosis.
Topics: Cystic Fibrosis; Genetic Carrier Screening; Genetic Testing; Humans; Infant, Newborn; Neonatal Scree | 1992 |
[Current methods in the diagnosis of adult-age cystic fibrosis].
Topics: Adult; Age Factors; Cystic Fibrosis; Humans; Male; Sweat; Tomography, X-Ray Computed | 1992 |
[The sweat test in cystic fibrosis].
Topics: Child; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Sodium; Sweat | 1990 |
Relapsing pancreatitis as initial manifestation of cystic fibrosis in a young man without pulmonary disease.
Topics: Adult; Chlorides; Chymotrypsin; Cystic Fibrosis; Duodenoscopy; Feces; Humans; Male; Oligospermia; Pa | 1989 |
Transport processes in the eccrine sweat gland.
Topics: Biological Transport; Cystic Fibrosis; Eccrine Glands; Electrolytes; Humans; Osmolar Concentration; | 1987 |
An overview of the management of cystic fibrosis.
Topics: Adolescent; Adult; Anti-Bacterial Agents; Child; Child, Preschool; Cystic Fibrosis; Diet; Humans; In | 1986 |
Hereditary aspects of COPD.
Topics: Adult; Alpha-Globulins; Cystic Fibrosis; Cytoplasm; Female; Heterozygote; Humans; Lung; Lung Disease | 1973 |
Research in cystic fibrosis: a review.
Topics: Biological Transport; Calcium; Cells, Cultured; Child; Chlorides; Cough; Cystic Fibrosis; Digestive | 1973 |
Cystic fibrosis.
Topics: Adolescent; Adult; Autopsy; Child; Child, Preschool; Cystic Fibrosis; Diabetes Mellitus, Type 1; Fem | 1972 |
[Mucoviscidosis (Cystic fibrosis)].
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Ob | 1967 |
Cystic fibrosis. Recent advances in understanding of its pathogenesis, detection of carriers and improved prognosis.
Topics: Adolescent; Calcium; Child; Cystic Fibrosis; Glycoproteins; Humans; Metabolism, Inborn Errors; Progn | 1969 |
[Review of the progress in studies on mucoviscidosis in Poland].
Topics: Adolescent; Asthma; Bronchitis; Child; Child, Preschool; Cystic Fibrosis; Diabetes Mellitus, Type 1; | 1968 |
Cystic fibrosis in adults.
Topics: Cystic Fibrosis; Female; Humans; Male; Sweat | 1970 |
[Gastric, intestinal and pancreatic diseases and pulmonary findings (including esophageal diseases)].
Topics: Adult; Celiac Disease; Chlorides; Cystic Fibrosis; Esophageal Neoplasms; Female; Gastrointestinal Di | 1971 |
[Secretion of eccrine sweat].
Topics: Adenosine Triphosphate; Adrenal Glands; Cell Membrane Permeability; Cystic Fibrosis; Emotions; Glyco | 1971 |
38 trials available for iodine and Cystic Fibrosis
| Article | Year |
|---|---|
GLPG2737 in lumacaftor/ivacaftor-treated CF subjects homozygous for the F508del mutation: A randomized phase 2A trial (PELICAN).
Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Chloride Channel Agonists; Cystic Fibrosis; Cyst | 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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
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.
Topics: Adolescent; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Cystic Fibrosis; Cystic F | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2019 |
Triple Therapy for Cystic Fibrosis
Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic | 2021 |
CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy.
Topics: Adolescent; Adult; Aminophenols; C-Reactive Protein; Child; Chloride Channel Agonists; Chlorides; Cy | 2017 |
Analytical and biological variation in repeated sweat chloride concentrations in clinical trials for CFTR modulator therapy.
Topics: Adult; Biological Variation, Individual; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cyst | 2018 |
Psychosocial Distress and Knowledge Deficiencies in Parents of Children in Ireland Who Carry an Altered Cystic Fibrosis Gene.
Topics: Adaptation, Psychological; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulat | 2018 |
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cysti | 2018 |
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cysti | 2018 |
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cysti | 2018 |
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cysti | 2018 |
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; | 2018 |
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; | 2018 |
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; | 2018 |
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; | 2018 |
A multicenter evaluation of sweat chloride concentration and variation in infants with cystic fibrosis.
Topics: Age Factors; Analysis of Variance; Biological Variation, Population; Chlorides; Cystic Fibrosis; Cys | 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).
Topics: Aminophenols; Body Mass Index; Child, Preschool; Chloride Channel Agonists; Cystic Fibrosis; Cystic | 2019 |
CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials.
Topics: Administration, Oral; Adult; Aminophenols; Benzoates; Benzopyrans; Biological Availability; Chloride | 2019 |
CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials.
Topics: Administration, Oral; Adult; Aminophenols; Benzoates; Benzopyrans; Biological Availability; Chloride | 2019 |
CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials.
Topics: Administration, Oral; Adult; Aminophenols; Benzoates; Benzopyrans; Biological Availability; Chloride | 2019 |
CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials.
Topics: Administration, Oral; Adult; Aminophenols; Benzoates; Benzopyrans; Biological Availability; Chloride | 2019 |
GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1).
Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 2019 |
A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11 years with cystic fibrosis.
Topics: Aminophenols; Benzodioxoles; Biological Availability; Child; Child, Preschool; Chloride Channel Agon | 2019 |
Skin pretreatment with microneedles prior to pilocarpine iontophoresis increases sweat production.
Topics: Administration, Cutaneous; Adult; California; Cystic Fibrosis; Electric Impedance; Equipment Design; | 2013 |
Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data.
Topics: Adult; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Co | 2014 |
Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.
Topics: Acute Kidney Injury; Adolescent; Adult; Anti-Bacterial Agents; Child; Chlorides; Codon, Nonsense; Cy | 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.
Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Base Sequence; Benzodioxoles; Chlorides; Cystic Fib | 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.
Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Base Sequence; Benzodioxoles; Chlorides; Cystic Fib | 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.
Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Base Sequence; Benzodioxoles; Chlorides; Cystic Fib | 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.
Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Base Sequence; Benzodioxoles; Chlorides; Cystic Fib | 2014 |
Efficacy response in CF patients treated with ivacaftor: post-hoc analysis.
Topics: Adolescent; Adult; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Co | 2015 |
Effect of salt supplementation on the rate of inadequate sweat collection for infants less than 3 months of age referred for the sweat test.
Topics: Administration, Oral; Cystic Fibrosis; Female; Humans; Infant, Newborn; Male; Neonatal Screening; Re | 2015 |
A semi-blinded study comparing 2 methods of measuring nasal potential difference: Subcutaneous needle versus dermal abrasion.
Topics: Adult; Chlorides; Cystic Fibrosis; Electrodiagnosis; Female; Humans; Male; Membrane Potentials; Nasa | 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.
Topics: Aminophenols; Child, Preschool; Chloride Channel Agonists; Chlorides; Cough; Cystic Fibrosis; Cystic | 2016 |
Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations.
Topics: Adolescent; Adult; Aminophenols; Biological Variation, Population; Biomarkers; Child; Chloride Chann | 2017 |
In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.
Topics: Administration, Oral; Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Chlorides; Cross-O | 2017 |
Diagnosis of cystic fibrosis by sweat testing: age-specific reference intervals.
Topics: Adolescent; Adult; Aged; Aging; Child; Child, Preschool; Chlorides; Cohort Studies; Cystic Fibrosis; | 2008 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2010 |
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.
Topics: Adolescent; Adult; Alleles; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibr | 2012 |
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.
Topics: Adolescent; Adult; Alleles; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibr | 2012 |
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.
Topics: Adolescent; Adult; Alleles; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibr | 2012 |
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.
Topics: Adolescent; Adult; Alleles; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibr | 2012 |
Prospective and parallel assessments of cystic fibrosis newborn screening protocols in the Czech Republic: IRT/DNA/IRT versus IRT/PAP and IRT/PAP/DNA.
Topics: Antigens, Neoplasm; Biomarkers; Biomarkers, Tumor; Clinical Protocols; Cystic Fibrosis; Cystic Fibro | 2012 |
Conductivity determined by a new sweat analyzer compared with chloride concentrations for the diagnosis of cystic fibrosis.
Topics: Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnostic Tests, Routine; Female; Human | 2005 |
A sweat test centered protocol for the disclosure and diagnosis of cystic fibrosis in a newborn screening program.
Topics: Clinical Protocols; Cystic Fibrosis; Female; Humans; Infant, Newborn; Male; Neonatal Screening; Pred | 2007 |
Development of a screening system for cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Hu | 1983 |
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function.
Topics: Administration, Oral; Adolescent; Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 1998 |
A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis.
Topics: Adolescent; Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; | 2002 |
Intravenous linoleic acid supplementation in children with cystic fibrosis.
Topics: Arachidonic Acids; Body Height; Body Weight; Child; Child, Preschool; Clinical Trials as Topic; Cyst | 1979 |
Negative effects of oral fatty acid supplementation on sweat chloride in cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Male; Oils; Prostaglan | 1979 |
Paradoxical effects of essential fatty acid supplementation on lipid profiles and sweat electrolytes in cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Di | 1990 |
Does lithium carbonate affect the ion transport abnormality in cystic fibrosis?
Topics: Adolescent; Adult; Biological Transport; Child; Chlorides; Cystic Fibrosis; Double-Blind Method; Dru | 1990 |
Is salt reabsorption in the human sweat duct subject to control?
Topics: Absorption; Adolescent; Child; Cyclic AMP; Cyclic GMP; Cystic Fibrosis; Humans; Pilocarpine; Potassi | 1985 |
1016 other studies available for iodine and Cystic Fibrosis
| Article | Year |
|---|---|
Cystic Fibrosis: From Tragedy to Triumph.
Topics: Alleles; Child; Cystic Fibrosis; Humans; Iodine | 2023 |
Iodine deficiency and subclinical hypothyroidism are common in cystic fibrosis patients.
Topics: Adolescent; Adult; Age Distribution; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Hypot | 2013 |
A fat absorption test using iodized oil, with particular application as a screening test in the diagnosis of fibrocystic disease of the pancreas.
Topics: Cystic Fibrosis; Disease; Fats; Female; Fibrocystic Breast Disease; Humans; Iodine; Iodized Oil; Oil | 1955 |
Cystic fibrosis of the pancreas: intestinal absorption of fat and fatty acid labeled with I 131.
Topics: Cystic Fibrosis; Fats; Fatty Acids; Intestinal Absorption; Intestines; Iodine | 1958 |
ESTIMATION OF FAT ABSORPTION FROM RANDOM STOOL SPECIMENS. MEASUREMENT BY ZIRCONIUM 95 AND IODINE 131.
Topics: Absorption; Adolescent; Biomedical Research; Celiac Disease; Child; Cystic Fibrosis; Dogs; Feces; Io | 1964 |
Novel short chain fatty acids restore chloride secretion in cystic fibrosis.
Topics: Cell Line; Chlorides; Colforsin; Cystic Fibrosis; Fatty Acids, Volatile; Humans; Iodine; Molecular S | 2006 |
Activation of the cystic fibrosis transmembrane regulator by cyclic AMP is not correlated with inhibition of endocytosis.
Topics: Cell Line; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane | 1994 |
Stimulation of chloride secretion by P1 purinoceptor agonists in cystic fibrosis phenotype airway epithelial cell line CFPEo-.
Topics: Adenine Nucleotides; Adenosine; Calcium; Cell Line; Chloride Channels; Chlorides; Cystic Fibrosis; E | 1994 |
Biopolymer synthesis on polypropylene supports. I. Oligonucleotides.
Topics: Base Sequence; Cystic Fibrosis; DNA; DNA, Complementary; Exons; Humans; Iodine; Molecular Sequence D | 1994 |
Permeabilization via the P2X7 purinoreceptor reveals the presence of a Ca2+-activated Cl- conductance in the apical membrane of murine tracheal epithelial cells.
Topics: 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Animals; Bromine; Calcium; Calcium Channel Blocker | 2000 |
The salivary and thyroid glands. A comparative study in man.
Topics: Cystic Fibrosis; Humans; Iodine; Salivary Gland Diseases; Salivary Glands; Sjogren's Syndrome; Thyro | 1967 |
Needle-free iontophoresis-driven β-adrenergic sweat rate test.
Topics: Adrenergic Agents; Aminophylline; Ascorbic Acid; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis | 2022 |
Outcomes of early repeat sweat testing in infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/CF screen-positive, inconclusive diagnosis.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant; Infant, Newbor | 2021 |
Sweat testing in the modern era: A national survey of sweat testing practice in the Republic of Ireland.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant, New | 2022 |
CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay.
Topics: Adrenergic Agents; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosi | 2022 |
Outcomes of Cystic Fibrosis Screening-Positive Infants With Inconclusive Diagnosis at School Age.
Topics: Age Factors; Biomarkers; Canada; Child; Chlorides; Cohort Studies; Confidence Intervals; Cystic Fibr | 2021 |
Sweat metabolomics before and after intravenous antibiotics for pulmonary exacerbation in people with cystic fibrosis.
Topics: Anti-Bacterial Agents; Cystic Fibrosis; Humans; Metabolomics; Prospective Studies; Sweat | 2022 |
Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme.
Topics: Chlorides; Cystic Fibrosis; Humans; Infant, Newborn; Quality Improvement; Standard of Care; Sweat | 2022 |
Impact of Gestational Age on Sweat Testing.
Topics: Cystic Fibrosis; Gestational Age; Humans; Sweat | 2022 |
Variation in CFTR-dependent 'β-sweating' among healthy adults.
Topics: Adult; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Sweat; Sweat Gl | 2022 |
Rapid chloride and bicarbonate determination by capillary electrophoresis for confirmatory testing of cystic fibrosis infants with volume-limited sweat specimens.
Topics: Bicarbonates; Chlorides; Cystic Fibrosis; Electrophoresis, Capillary; Humans; Infant; Infant, Newbor | 2023 |
The sweat chloride test has lived up to the changes in CF care.
Topics: Chlorides; Cystic Fibrosis; Electric Conductivity; Humans; Sweat | 2022 |
Reclassifying inconclusive diagnosis for cystic fibrosis with new generation sweat test.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Sweat | 2022 |
Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study.
Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis | 2023 |
A step forward for an intermediate cystic fibrosis population.
Topics: Cystic Fibrosis; Humans; Sweat | 2022 |
Sweat Proteomics in Cystic Fibrosis: Discovering Companion Biomarkers for Precision Medicine and Therapeutic Development.
Topics: Biomarkers; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Glycosy | 2022 |
Development of algorithm for diagnosis of cystic fibrosis in absence of sweat chloride testing in resource-limited setting.
Topics: Algorithms; Alkalosis; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance | 2022 |
ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Ion Transport; Mutatio | 2022 |
Gradual increase in sweat chloride concentration is associated with a higher risk of CRMS/CFSPID to CF reclassification.
Topics: Child; Chlorides; Cross-Sectional Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductanc | 2023 |
Sweat conductivity for diagnosing cystic fibrosis after positive newborn screening: prospective, diagnostic test accuracy study.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnostic Tests, R | 2023 |
Variability of the sweat test in children with Cystic Fibrosis previously CRMS/CFSPID: A retrospective monocenter experience.
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regul | 2023 |
A wearable colorimetric sweat pH sensor-based smart textile for health state diagnosis.
Topics: Colorimetry; Curcumin; Cystic Fibrosis; Humans; Hydrogen-Ion Concentration; Sweat; Textiles; Wearabl | 2023 |
Aquagenic wrinkling of the palms in a cohort of Greek children diagnosed with cystic fibrosis: Associated clinical parameters.
Topics: Child; Chlorides; Cystic Fibrosis; Edema; Greece; Humans; Hyperhidrosis; Keratosis; Pruritus; Sweat; | 2023 |
Cystic fibrosis screen-positive neonates with one pathogenic variant still warrant sweat testing.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant, New | 2023 |
Reexamining the Minimum Sweat Rate Requirement for Sweat Chloride Testing.
Topics: Chlorides; Cystic Fibrosis; Humans; Nonoxynol; Sweat | 2023 |
A paper-based length of stain analytical device for naked eye (readout-free) detection of cystic fibrosis.
Topics: Calorimetry; Chlorides; Chromates; Cystic Fibrosis; Humans; Infant, Newborn; Limit of Detection; Pap | 2019 |
Changes in Airway Microbiome and Inflammation with Ivacaftor Treatment in Patients with Cystic Fibrosis and the G551D Mutation.
Topics: Adolescent; Adult; Aminophenols; Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conducta | 2020 |
Proof of concept for identifying cystic fibrosis from perspiration samples.
Topics: Algorithms; Case-Control Studies; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conducta | 2019 |
How to perform and interpret the sweat test.
Topics: Cystic Fibrosis; Diagnostic Tests, Routine; Female; Humans; Infant, Newborn; Male; Neonatal Screenin | 2020 |
Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males.
Topics: Adolescent; Adult; Age Factors; Aminophenols; Body Mass Index; Body Weight; Child; Chloride Channel | 2020 |
[Review of the sweat test indications in a Brussels' cystic fibrosis reference center].
Topics: Adolescent; Adult; Aged; Belgium; Child; Child, Preschool; Chlorine; Cystic Fibrosis; Diagnostic Tes | 2019 |
Phenotype of children with inconclusive cystic fibrosis diagnosis after newborn screening.
Topics: Child; Chlorides; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulat | 2020 |
Identification of a novel cystic fibrosis mutation in three patients of South Asian descent.
Topics: Adolescent; Adult; Asian People; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulato | 2020 |
Newborn screening for CF in France: An exemplary national experience.
Topics: Algorithms; Biomarkers; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; France | 2020 |
The Italian External Quality Assessment Program for Cystic Fibrosis Sweat Chloride Test: Does Active Participation Improve the Quality?
Topics: Chlorides; Cystic Fibrosis; Humans; Italy; Prospective Studies; Quality Assurance, Health Care; Swea | 2020 |
Females with cystic fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males.
Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Body Mass Index; Child; Chlorides; C | 2021 |
Have courage in using nasal potential difference for diagnostic decisions and clinical research.
Topics: Courage; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Nose; Sweat | 2020 |
Comparison of Organoid Swelling and
Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chlorides; Colforsin; Cystic | 2020 |
Sweat chloride assay by inductively coupled plasma mass spectrometry: a confirmation test for cystic fibrosis diagnosis.
Topics: Adult; Case-Control Studies; Chlorides; Cystic Fibrosis; Humans; Limit of Detection; Mass Spectromet | 2020 |
Quantity not sufficient rates and delays in sweat testing in US infants with cystic fibrosis.
Topics: Cystic Fibrosis; Gestational Age; Humans; Infant; Infant, Newborn; Neonatal Screening; Sweat | 2020 |
Cystic fibrosis newborn screening: the importance of bloodspot sample quality.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dried Blood Spot Te | 2021 |
The role of biochemical testing in cystic fibrosis.
Topics: Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnostic T | 2020 |
Screen-Printed Sensor for Low-Cost Chloride Analysis in Sweat for Rapid Diagnosis and Monitoring of Cystic Fibrosis.
Topics: Biosensing Techniques; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulat | 2020 |
Outcomes of repeat sweat testing in cystic fibrosis newborn screen positive infants.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant; Inf | 2021 |
Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.
Topics: Adolescent; Adult; Aged; Aminophenols; Aminopyridines; Benzodioxoles; Child; Chloride Channel Agonis | 2021 |
A Young Girl With Bronchiectasis and Elevated Sweat Chloride.
Topics: Adolescent; Axonemal Dyneins; Bronchiectasis; Chronic Disease; Ciliary Motility Disorders; Cystic Fi | 2021 |
Soft, skin-interfaced sweat stickers for cystic fibrosis diagnosis and management.
Topics: Chlorides; Cystic Fibrosis; Humans; Infant; Quality of Life; Smartphone; Sweat | 2021 |
Optical Measurements of Sweat for in Vivo Quantification of CFTR Function in Individual Sweat Glands.
Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conducta | 2021 |
Topics: Adult; Aged; Aged, 80 and over; Air Pollutants; Air Pollution; Animals; Anti-Bacterial Agents; Anti- | 2021 |
Pseudo-Bartter Syndrome and Intermediate Sweat Chloride Levels-It Could Still be Cystic Fibrosis!
Topics: Bartter Syndrome; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; H | 2021 |
Audit of sweat chloride testing reveals analytical errors.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnostic Tests, R | 2021 |
Elevated sweat chloride test: is it always cystic fibrosis?
Topics: Celiac Disease; Chlorides; Constipation; Cystic Fibrosis; Diagnosis, Differential; Female; Humans; I | 2021 |
Towards harmonization of solutions used for cystic fibrosis diagnosis by nasal potential difference measurements: A formulation approach with CHESS® software.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Nasal Mucosa; Software | 2022 |
Aquagenic wrinkling in children under two years of age: Could this be a potential clinical referral tool for cystic fibrosis among non-screened populations?
Topics: Cystic Fibrosis; Female; Hand Dermatoses; Humans; Immersion; Infant; Infant, Newborn; Male; Mass Scr | 2021 |
Health Characteristics of Patients with Cystic Fibrosis whose Genotype Includes a Variant of the Nucleotide Sequence c.3140-16T>A and Functional Analysis of this Variant.
Topics: Adolescent; Adult; Cells, Cultured; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductanc | 2021 |
An Unexpectedly Normal Sweat Chloride.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Sweat | 2021 |
Infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis and acute recurrent pancreatitis: what definition?
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant; Metabolic Synd | 2022 |
Evaluating performance in sweat testing in medical biochemistry laboratories in Croatia.
Topics: Biochemistry; Clinical Laboratory Techniques; Croatia; Cystic Fibrosis; Humans; Medical Laboratory S | 2017 |
Autonomous sweat extraction and analysis applied to cystic fibrosis and glucose monitoring using a fully integrated wearable platform.
Topics: Cystic Fibrosis; Glucose; Humans; Iontophoresis; Monitoring, Physiologic; Sweat; Wearable Electronic | 2017 |
Feasibility and normal values of an integrated conductivity (Nanoduct™) sweat test system in healthy newborns.
Topics: Cystic Fibrosis; Electric Conductivity; Female; Humans; Infant, Newborn; Male; Neonatal Screening; R | 2017 |
Optimization of the French cystic fibrosis newborn screening programme by a centralized tracking process.
Topics: Algorithms; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; France; Hu | 2018 |
Is sweat chloride predictive of severity of cystic fibrosis lung disease assessed by chest computed tomography?
Topics: Adolescent; Bronchiectasis; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Inf | 2017 |
A smartphone-based chloridometer for point-of-care diagnostics of cystic fibrosis.
Topics: Biosensing Techniques; Chlorides; Cystic Fibrosis; Equipment Design; Humans; Limit of Detection; Poi | 2017 |
Sweat travels: the issue of sweat chloride transportation.
Topics: Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Humans; Specimen Handling; Sweat; Transportati | 2018 |
Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience.
Topics: Adolescent; Adult; Aminophenols; Blood Glucose; Body Mass Index; Child; Chloride Channel Agonists; C | 2017 |
Incidental late diagnosis of cystic fibrosis following AH1N1 influenza virus pneumonia: a case report.
Topics: Anti-Bacterial Agents; Ceftazidime; Colistin; Cystic Fibrosis; Delayed Diagnosis; Drainage, Postural | 2017 |
Laboratory performance of sweat conductivity for the screening of cystic fibrosis.
Topics: Chlorides; Clinical Laboratory Techniques; Cystic Fibrosis; Electric Conductivity; Humans; Quality C | 2018 |
Newborn cystic fibrosis screening in southeastern Mexico: Birth prevalence and novel CFTR gene variants.
Topics: Alleles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Infan | 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.
Topics: Aminophenols; Chloride Channel Agonists; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis | 2018 |
Cystic fibrosis newborn screening: outcome of infants with normal sweat tests.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant, Newborn; Londo | 2018 |
Normal sweat chloride test does not rule out cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Anal | 2017 |
Another step in the journey: From CFTR mutation to sweat chloride concentration to survival.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; M | 2018 |
The relationship between sweat chloride levels and mortality in cystic fibrosis varies by individual genotype.
Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Geno | 2018 |
Is sweat testing for cystic fibrosis feasible in patients with down syndrome?
Topics: Adolescent; Adult; Age Factors; Case-Control Studies; Child; Child, Preschool; Chlorides; Cystic Fib | 2018 |
May the new suggested lower borderline limit of sweat chloride impact the diagnostic process for cystic fibrosis?
Topics: Chlorides; Cystic Fibrosis; Humans; Infant; Practice Guidelines as Topic; Sweat | 2018 |
No sweat, no genes: A diagnostic dilemma.
Topics: Biomarkers; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Delayed | 2018 |
Considering exercise-associated hyponatraemia as a continuum.
Topics: Adult; Aftercare; Anticonvulsants; Antidiuretic Agents; Confusion; Cystic Fibrosis; Deamino Arginine | 2018 |
Sweat test for cystic fibrosis: Wearable sweat sensor vs. standard laboratory test.
Topics: Adult; Chlorides; Cystic Fibrosis; Dimensional Measurement Accuracy; Equipment Design; Female; Human | 2018 |
Intra-individual biological variation in sweat chloride concentrations in CF, CFTR dysfunction, and healthy pediatric subjects.
Topics: Adolescent; Biological Variation, Individual; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Tra | 2018 |
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.
Topics: Adult; Aminophenols; Animals; Bicarbonates; Biological Transport, Active; Bronchoalveolar Lavage Flu | 2018 |
Effect of topiramate on sweat chloride level while screening for cystic fibrosis.
Topics: Adolescent; Anticonvulsants; Asthma; Chlorides; Cystic Fibrosis; False Positive Reactions; Female; H | 2018 |
Evaluation of continuous constant current and continuous pulsed current in sweat induction for cystic fibrosis diagnosis.
Topics: Adolescent; Adult; Aged; Biomarkers; Child; Child, Preschool; Chlorides; Cross-Sectional Studies; Cy | 2018 |
Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV
Topics: Adult; Aminophenols; Biomarkers; Body Mass Index; Child; Chloride Channel Agonists; Cystic Fibrosis; | 2019 |
Positive clinical response to ivacaftor treatment in an individual with the CFTR genotype F508del/V456A.
Topics: Adult; Aminophenols; Chloride Channel Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 2019 |
[Not Available].
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Germany; Humans; Infant; Infan | 2016 |
Devil in the detail of newborn screening for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant, New | 2019 |
The Incidence of Cystic Fibrosis in the Central Region of Anatolia in Turkey Between 2015 and 2016
Topics: Cross-Sectional Studies; Cystic Fibrosis; Female; Genetic Testing; Humans; Incidence; Infant; Infant | 2019 |
Predictive factors for lumacaftor/ivacaftor clinical response.
Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biomarkers, Pharmacological; Child; Chloride Channel Ag | 2019 |
Comparison of two sweat test systems for the diagnosis of cystic fibrosis in newborns.
Topics: Chlorides; Cystic Fibrosis; Diagnostic Tests, Routine; Electric Conductivity; Female; Humans; Infant | 2019 |
Sweat Chloride Testing.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; F | 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.
Topics: Alleles; Aminophenols; Aminopyridines; Antiporters; Base Sequence; Benzodioxoles; Body Mass Index; C | 2019 |
Aquagenic Wrinkling of Skin: A Screening Test for Cystic Fibrosis.
Topics: Adolescent; Case-Control Studies; Child; Child, Preschool; Cystic Fibrosis; Diagnostic Techniques an | 2019 |
Inconclusive diagnosis after a positive newborn bloodspot screening result for cystic fibrosis; clarification of the harmonised international definition.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, Differential; False | 2019 |
Iontophoresis burns: a peculiar cutaneous injury from a diagnostic sweat test for cystic fibrosis.
Topics: Burns; Conservative Treatment; Cystic Fibrosis; Female; Humans; Infant; Iontophoresis; Skin; Sweat | 2019 |
Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations.
Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Chlorides; Compassionate Use Tria | 2019 |
Nasal potential difference in suspected cystic fibrosis patients with 5T polymorphism.
Topics: Adult; Chlorides; Correlation of Data; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Re | 2020 |
Adult cystic fibrosis--a rare diagnosis from India.
Topics: Adult; Bronchiectasis; Cystic Fibrosis; Female; Humans; India; Sexual Infantilism; Sweat | 2012 |
The impact of personalised therapies on respiratory medicine.
Topics: Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genet | 2013 |
CFTR2: How will it help care?
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Anal | 2013 |
Evaluation of an inductively coupled plasma mass spectrometry method for the analysis of sweat chloride and sodium for use in the diagnosis of cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Humans; Mass Spectrometry; Sodium; Sweat | 2013 |
The predictive potential of the sweat chloride test in cystic fibrosis patients with the G551D mutation.
Topics: Administration, Oral; Adolescent; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis T | 2013 |
50 years ago in the Journal of Pediatrics: A note on studies of salt excretion in sweat: relationships between rate, conductivity, and electrolyte composition of sweat from patients with cystic fibrosis and from control subjects. Gibson, LE, di Sant’Agnes
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; History, 20th Century; Humans; | 2013 |
Newborn screening for cystic fibrosis in Switzerland--consequences after analysis of a 4 months pilot study.
Topics: Algorithms; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Ana | 2013 |
Electrochemical detection of chloride levels in sweat using silver nanoparticles: a basis for the preliminary screening for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Electrochemical Techniques; Humans; Metal Nanoparticles; Silver; Sweat | 2013 |
The need for salt: does a relationship exist between cystic fibrosis and exercise-associated hyponatremia?
Topics: Body Weight; Case-Control Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regula | 2014 |
Relationship between sweat chloride, sodium, and age in clinically obtained samples.
Topics: Adolescent; Adult; Age Factors; Aged; Chemistry, Clinical; Child; Child, Preschool; Chlorides; Cysti | 2014 |
Evaluating the predictive ability of sweat chloride.
Topics: Aminophenols; Chlorides; Cystic Fibrosis; Female; Humans; Male; Quinolones; Sweat | 2014 |
[Cystic fibrosis - a childhood illness grows up].
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Chlorides; Combined Modality Therapy; Cysti | 2013 |
Relevance of nasal potential difference in diagnosis of cystic fibrosis among children.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regu | 2013 |
A patient nicknamed 'saline': atypical course with cystic fibrosis.
Topics: Adolescent; Adult; Alleles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regula | 2013 |
Ivacaftor in a G551D homozygote with cystic fibrosis.
Topics: Adolescent; Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regu | 2013 |
Normalization of sweat chloride concentration and clinical improvement with ivacaftor in a patient with cystic fibrosis with mutation S549N.
Topics: Aminophenols; Child; Chlorides; Cystic Fibrosis; Female; Humans; Mutation; Quinolones; Sweat | 2013 |
Response.
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.
Topics: Aminophenols; Chlorides; Cystic Fibrosis; Humans; Outcome Assessment, Health Care; Quinolones; Sweat | 2013 |
Does extensive genotyping and nasal potential difference testing clarify the diagnosis of cystic fibrosis among patients with single-organ manifestations of cystic fibrosis?
Topics: Adult; Alleles; Biomarkers; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 2014 |
In vivo readout of CFTR function: ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glands.
Topics: Adrenergic beta-Agonists; Adult; Aminophylline; Atropine; Cystic Fibrosis; Cystic Fibrosis Transmemb | 2013 |
[Aquagenic palmoplantar keratoderma in children with cystic fibrosis].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 2013 |
Sweat chloride is not a useful marker of clinical response to Ivacaftor.
Topics: Aminophenols; Biomarkers; Body Height; Body Weight; Chlorides; Cohort Studies; Cystic Fibrosis; Forc | 2014 |
Aquagenic wrinkling of the palms in a patient with cystic fibrosis.
Topics: Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator | 2013 |
False-negative sweat chloride testing in a child with cystic fibrosis and undiagnosed hypohidrotic ectodermal dysplasia.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Ectodermal Dysplasia 1, Anhidrotic; False Negative Rea | 2014 |
Aquagenic wrinkling of the palms and cystic fibrosis: an Italian study with controls and genotype-phenotype correlations.
Topics: Adolescent; Adult; Case-Control Studies; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis T | 2014 |
Sweat conductivity: an accurate diagnostic test for cystic fibrosis?
Topics: Child, Preschool; Chlorides; Cross-Sectional Studies; Cystic Fibrosis; Electric Conductivity; Female | 2014 |
A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor.
Topics: Amino Acid Substitution; Aminophenols; Case-Control Studies; Cystic Fibrosis; Cystic Fibrosis Transm | 2014 |
Diagnosis of cystic fibrosis in the kindred of an infant with CFTR-related metabolic syndrome: importance of follow-up that includes monitoring sweat chloride concentrations over time.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 2014 |
Implementation of a quality improvement program to improve sweat test performance in a pediatric hospital.
Topics: Chlorides; Cystic Fibrosis; Hospitals, Pediatric; Humans; Infant; Infant, Newborn; Iontophoresis; Ma | 2014 |
CFTR genotype and clinical outcomes of adult patients carried as cystic fibrosis disease.
Topics: Adolescent; Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; | 2014 |
Does integration of various ion channel measurements improve diagnostic performance in cystic fibrosis?
Topics: Adolescent; Adult; Azoospermia; Case-Control Studies; Child; Cohort Studies; Cystic Fibrosis; Cystic | 2014 |
Higher sweat chloride levels in patients with asthma: a case-control study.
Topics: Adolescent; Asthma; Case-Control Studies; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagn | 2015 |
A synopsis of methods of sweat tests in pathology.
Topics: Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Humans; Sweat; Sweating | 2014 |
A new method of sweat testing: the CF Quantum®sweat test.
Topics: Chlorides; Cystic Fibrosis; Humans; Sensitivity and Specificity; Sweat | 2014 |
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.
Topics: Adolescent; Adult; Aminophenols; Biomarkers; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2014 |
Double opposite end injection capillary electrophoresis with contactless conductometric detection for simultaneous determination of chloride, sodium and potassium in cystic fibrosis diagnosis.
Topics: Case-Control Studies; Chlorides; Cystic Fibrosis; Electrophoresis, Capillary; False Positive Reactio | 2014 |
Ion chromatography for the precise analysis of chloride and sodium in sweat for the diagnosis of cystic fibrosis.
Topics: Chlorides; Chromatography, High Pressure Liquid; Chromatography, Ion Exchange; Cystic Fibrosis; Huma | 2015 |
Rectal prolapse and cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Incidence; Infant; | 2015 |
Lack of harmonization in sweat testing for cystic fibrosis - a national survey.
Topics: Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Denmark; Health Care Surveys; Humans; Referenc | 2014 |
Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes.
Topics: Adult; Case-Control Studies; Chlorides; Chronic Disease; Cystic Fibrosis; Cystic Fibrosis Transmembr | 2014 |
Intestinal current measurement versus nasal potential difference measurements for diagnosis of cystic fibrosis: a case-control study.
Topics: Adolescent; Adult; Case-Control Studies; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmem | 2014 |
Pulsed direct and constant direct currents in the pilocarpine iontophoresis sweat chloride test.
Topics: Adult; Biopsy; Chlorides; Cystic Fibrosis; Electric Impedance; Electrodiagnosis; Female; Humans; Ion | 2014 |
Sweating the small stuff: adequacy and accuracy in sweat chloride determination.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Infant | 2015 |
Sweat chloride levels in asthma.
Topics: Asthma; Child; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Sweat | 2015 |
Characterization of gene mutations and phenotypes of cystic fibrosis in Chinese patients.
Topics: Adolescent; Adult; Age of Onset; Asian People; Azo Compounds; Child; Child, Preschool; China; Chlori | 2015 |
From lip to lab: salty tasting skin is the main clue that raises clinical suspicion of cystic fibrosis in young infants.
Topics: Adult; Clinical Decision-Making; Cystic Fibrosis; Epidemiologic Studies; Female; Humans; Infant; Inf | 2015 |
Elevated sweat chloride levels due to arsenic toxicity.
Topics: Adult; Aged; Arsenic; Chlorides; Cross-Sectional Studies; Cystic Fibrosis; Cystic Fibrosis Transmemb | 2015 |
Association of sweat chloride concentration at time of diagnosis and CFTR genotype with mortality and cystic fibrosis phenotype.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 2015 |
Clinical evaluation of the Nanoduct sweat test system in the diagnosis of cystic fibrosis after newborn screening.
Topics: Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Female; Humans; Infant, Newborn; Male; Nanotec | 2015 |
Next generation sequencing to determine the cystic fibrosis mutation spectrum in Palestinian population.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; High-Throughput Nucl | 2015 |
Misdiagnosis of cystic fibrosis based on transient pancreatic insufficiency and elevated sweat chloride.
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regul | 2015 |
A paper-based skin patch for the diagnostic screening of cystic fibrosis.
Topics: Colorimetry; Cystic Fibrosis; Humans; Mass Screening; Paper; Skin; Sweat | 2015 |
Long-term outcomes of children with intermediate sweat chloride values in infancy.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Male; Neonata | 2015 |
Genotype-phenotype relationship in Iranian patients with cystic fibrosis.
Topics: Age of Onset; Asian People; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Re | 2015 |
New challenges in the diagnosis and management of cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Female; Humans; Male; Neonatal Screening; Sweat | 2015 |
Sweat test practice in pediatric pulmonology after introduction of cystic fibrosis newborn screening.
Topics: Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Femal | 2015 |
A candidate reference method using ICP-MS for sweat chloride quantification.
Topics: Calibration; Chlorides; Cystic Fibrosis; Humans; Mass Spectrometry; Sweat | 2016 |
Non-allergic asthma as a CFTR-related disorder.
Topics: Adolescent; Adult; Asthma; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conducta | 2016 |
Analysis of Cystic Fibrosis in Federation of Bosnia and Herzegovina.
Topics: Adolescent; Bosnia and Herzegovina; Cetylpyridinium; Child; Child, Preschool; Cystic Fibrosis; Femal | 2015 |
Refining the continuum of CFTR-associated disorders in the era of newborn screening.
Topics: Adolescent; Child; Chlorides; Cohort Studies; Cross-Sectional Studies; Cystic Fibrosis; Cystic Fibro | 2016 |
Sweat chloride and immunoreactive trypsinogen in infants carrying two
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regul | 2017 |
Clinical Phenotypes and Genotypic Spectrum of Cystic Fibrosis in Chinese Children.
Topics: 3' Untranslated Regions; Adolescent; Asian People; Aspergillosis, Allergic Bronchopulmonary; Bronchi | 2016 |
Measurement of fecal elastase improves performance of newborn screening for cystic fibrosis.
Topics: Algorithms; Clinical Protocols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator | 2016 |
The Italian pilot external quality assessment program for cystic fibrosis sweat test.
Topics: Chlorides; Clinical Laboratory Techniques; Cystic Fibrosis; Diagnostic Tests, Routine; Follow-Up Stu | 2016 |
Loss of carbonic anhydrase XII function in individuals with elevated sweat chloride concentration and pulmonary airway disease.
Topics: Adolescent; Adult; Animals; Carbonic Anhydrases; Child; Child, Preschool; Chlorides; Cystic Fibrosis | 2016 |
A false positive newborn screening result due to a complex allele carrying two frequent CF-causing variants.
Topics: Alleles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, Differenti | 2016 |
Validation of a Nutrition Screening Tool for Pediatric Patients with Cystic Fibrosis.
Topics: Adolescent; Body Mass Index; Body Weight; Child; Cross-Sectional Studies; Cystic Fibrosis; Energy In | 2016 |
Sources of Variation in Sweat Chloride Measurements in Cystic Fibrosis.
Topics: Adult; Biomarkers; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; | 2016 |
Nasal potential difference outcomes support diagnostic decisions in cystic fibrosis.
Topics: Adrenergic beta-Agonists; Adult; Amiloride; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 2016 |
50 Years Ago in TheJournal ofPediatrics: The Sweat Test in Cystic Fibrosis: A Comparison of Overnight Sweat Collection versus the Pilocarpine Iontophoresis Method.
Topics: Chlorides; Cystic Fibrosis; Diagnostic Tests, Routine; History, 20th Century; Humans; Iontophoresis; | 2016 |
Sweat chloride testing: controversies and issues.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; D | 2016 |
Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.
Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cysti | 2017 |
Correlation of sweat chloride and percent predicted FEV
Topics: Aminophenols; Biomarkers; Chloride Channel Agonists; Chlorides; Clinical Trials as Topic; Cystic Fib | 2017 |
Quality of sweat test (ST) based on the proportion of sweat sodium (Na) and sweat chloride (Cl) as diagnostic parameter of cystic fibrosis: are we on the right way?
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Fem | 2016 |
Diagnosis of cystic fibrosis in a patient of Egyptian background.
Topics: Arabs; Asthma; Bronchiectasis; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Cond | 2016 |
Sweat Chloride: The Critical Biomarker for Cystic Fibrosis Trials.
Topics: Biomarkers; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; | 2016 |
Diagnosis of cystic fibrosis with chloride meter (Sherwood M926S chloride analyzer®) and sweat test analysis system (CFΔ collection system®) compared to the Gibson Cooke method.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electric Conductivity; Femal | 2016 |
Variability of sweat chloride - A never ending story.
Topics: Chlorides; Cystic Fibrosis; Humans; Sweat | 2017 |
Does newborn screening influence the young cystic fibrosis cohort included in national registries?
Topics: Age Factors; Australia; Belgium; Body Mass Index; Child; Child, Preschool; Cohort Studies; Cystic Fi | 2017 |
Wearable Potentiometric Chloride Sweat Sensor: The Critical Role of the Salt Bridge.
Topics: Adult; Chlorides; Cystic Fibrosis; Electrolytes; Equipment Design; Exercise; Female; Humans; Male; P | 2016 |
Role of CFTR mutation analysis in the diagnostic algorithm for cystic fibrosis.
Topics: Adolescent; Adult; Aged; Algorithms; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembran | 2017 |
Individual pharmacokinetic variation leads to underdosing of ciprofloxacin in some cystic fibrosis patients.
Topics: Adult; Anti-Bacterial Agents; Breath Tests; Ciprofloxacin; Cystic Fibrosis; Cytochrome P-450 CYP3A; | 2017 |
Ethnicity impacts the cystic fibrosis diagnosis: A note of caution.
Topics: Adolescent; Adult; Asian People; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Trans | 2017 |
[Management of infants whose diagnosis is inconclusive at neonatal screening for cystic fibrosis].
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, Differen | 2017 |
Sweat potassium concentration may help to identify falsification of sweat test: a case report.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Male; Munchausen Syndrome by Proxy; Potassium; | 2008 |
Shwachman-Diamond syndrome in a child presenting with cystic fibrosis-type symptoms and a false-positive sweat test.
Topics: Bone Marrow Diseases; Chronic Disease; Cystic Fibrosis; Diagnosis, Differential; False Positive Reac | 2008 |
Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report.
Topics: Adult; Age Factors; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; | 2008 |
[Standardized sweat chloride analysis for the diagnosis of cystic fibrosis in Korea].
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Infant; Iont | 2008 |
Early determination of cystic fibrosis by electrochemical chloride quantification in sweat.
Topics: Adult; Biomarkers; Biosensing Techniques; Chlorides; Cystic Fibrosis; Disposable Equipment; Electroc | 2009 |
Uncertainty of sweat chloride testing: does the right hand know what the left hand is doing?
Topics: Analysis of Variance; Child; Child, Preschool; Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; | 2008 |
A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Testing; Humans; Infan | 2009 |
By the sweat of our brows: how salty should a person be?
Topics: Aging; Chlorides; Cystic Fibrosis; Databases, Factual; Humans; Infant, Newborn; Reference Values; Sw | 2008 |
Sweat chloride testing in infants identified as heterozygote carriers by newborn screening.
Topics: Carrier State; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gene | 2008 |
Transient correction of the basic defect in sweat glands in an individual with cystic fibrosis carrying the complex CFTR allele F508del-R553Q.
Topics: Adult; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; False Negative Reaction | 2009 |
[The diagnosis of cystic fibrosis in adults: lessons from a family story].
Topics: Adult; Age Factors; Aged; Bronchiectasis; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transme | 2009 |
[Clinical characteristics and outcome of cystic fibrosis: report of 16 cases].
Topics: Adolescent; Bronchiectasis; Child; Child, Preschool; Chronic Disease; Consanguinity; Cystic Fibrosis | 2008 |
Borderline sweat test: Utility and limits of genetic analysis for the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 2009 |
Phenotypic characterisation of patients with intermediate sweat chloride values: towards validation of the European diagnostic algorithm for cystic fibrosis.
Topics: Adolescent; Adult; Algorithms; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Cond | 2009 |
Does the Macroduct collection system reliably define sweat chloride concentration in subjects with intermediate results?
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Clinical Laboratory Techniques; Cystic Fibros | 2009 |
Evaluation of the SenseWear activity monitor during exercise in cystic fibrosis and in health.
Topics: Adolescent; Adult; Algorithms; Analysis of Variance; Calorimetry, Indirect; Cystic Fibrosis; Energy | 2009 |
Sweat testing for cystic fibrosis: standards of performance in Australasia.
Topics: Adolescent; Adult; Australia; Clinical Chemistry Tests; Cystic Fibrosis; Humans; Surveys and Questio | 2009 |
Cystic fibrosis.
Topics: Anti-Bacterial Agents; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Digesti | 2009 |
Sweat chloride concentration in cystic fibrosis patients with cystic fibrosis trans-membrane conductance regulator I1234V mutation.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 2009 |
Sweat gland bioelectrics differ in cystic fibrosis: a new concept for potential diagnosis and assessment of CFTR function in cystic fibrosis.
Topics: Adrenergic beta-Agonists; Adult; Biomarkers; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembra | 2009 |
Clinical and molecular characterization of S1118F-CFTR.
Topics: Blotting, Western; Cells, Cultured; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conduc | 2009 |
Non-classic cystic fibrosis associated with D1152H CFTR mutation.
Topics: Adolescent; Adult; Aged; Amino Acid Substitution; Child; Child, Preschool; Chlorides; Cohort Studies | 2010 |
The use of sweat chloride test for screening cystic fibrosis among malnourished children suffering from frequent respiratory infections.
Topics: Child; Chlorides; Cross-Sectional Studies; Cystic Fibrosis; Humans; Malnutrition; Respiratory Tract | 2009 |
Classic respiratory disease but atypical diagnostic testing distinguishes adult presentation of cystic fibrosis.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Chlorides; Cross-Sectional Stud | 2010 |
Does sweat volume influence the sweat test result?
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electric Conductivity; | 2010 |
[Ascites and extreme lipid abnormalities as initial symptoms of cystic fibrosis in a 5-years-old girl--case report].
Topics: Ascites; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Fem | 2009 |
Detection of large rearrangements in the cystic fibrosis transmembrane conductance regulator gene by multiplex ligation-dependent probe amplification assay when sequencing fails to detect two disease-causing mutations.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Anal | 2010 |
False negative cystic fibrosis newborn screen.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; False Negative Reactions; Huma | 2010 |
Cystic fibrosis transmembrane conductance regulator channel dysfunction in non-cystic fibrosis bronchiectasis.
Topics: Adult; Aged; Bronchiectasis; Case-Control Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Co | 2010 |
Psychological effects of false-positive results in cystic fibrosis newborn screening: a two-year follow-up.
Topics: Anxiety; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; False Positive Reacti | 2010 |
Sweat chloride concentration in cystic fibrosis patients with cystic fibrosis trans-membrane conductance regulator 11234V mutation.
Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infa | 2010 |
Use of sweat conductivity measurements.
Topics: Cystic Fibrosis; Electric Conductivity; Humans; Sodium Chloride; Sweat | 2010 |
Cystic fibrosis newborn screening: using experience to optimize the screening algorithm.
Topics: Algorithms; Biomarkers; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regula | 2010 |
Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; F | 2010 |
Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport.
Topics: Adolescent; Biomarkers; Case-Control Studies; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Tra | 2010 |
Cystic fibrosis papers of the year 2009.
Topics: Amitriptyline; Antidepressive Agents, Tricyclic; Aspergillosis, Allergic Bronchopulmonary; Child; Ch | 2010 |
Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembran | 2010 |
Initial evaluation of a biochemical cystic fibrosis newborn screening by sequential analysis of immunoreactive trypsinogen and pancreatitis-associated protein (IRT/PAP) as a strategy that does not involve DNA testing in a Northern European population.
Topics: Antigens, Neoplasm; Biomarkers; Biomarkers, Tumor; Chlorides; Cystic Fibrosis; Cystic Fibrosis Trans | 2010 |
[French guidelines for sweat test practice and interpretation for cystic fibrosis neonatal screening].
Topics: Administration, Cutaneous; Chlorides; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Iontophoresi | 2010 |
When to suspect atypical cystic fibrosis.
Topics: Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Deoxyribonuc | 2010 |
The need for quality improvement in sweat testing infants after newborn screening for cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Infant, Newborn; Neonatal Screening; Prospective Studies; Quality of Health | 2010 |
Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator function.
Topics: Adolescent; Adult; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Hum | 2011 |
Abnormal electrochemical skin conductance in cystic fibrosis.
Topics: Adult; Chloride Channels; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regu | 2011 |
Type of CFTR mutation determines risk of pancreatitis in patients with cystic fibrosis.
Topics: Adult; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrin | 2011 |
The use of sweat chloride test for screening cystic fibrosis among malnourished children suffering from frequent respiratory infections.
Topics: Child; Chlorides; Cystic Fibrosis; Humans; Malnutrition; Respiratory Tract Infections; Sweat | 2010 |
Bilateral sweat tests with two different methods as a part of cystic fibrosis newborn screening (CF NBS) protocol and additional quality control.
Topics: Case-Control Studies; Chlorides; Cystic Fibrosis; Electric Conductivity; False Positive Reactions; H | 2010 |
Parental information seeking following a positive newborn screening for cystic fibrosis.
Topics: Adult; Cystic Fibrosis; Female; Genetic Counseling; Health Knowledge, Attitudes, Practice; Humans; I | 2010 |
Parental understanding of newborn screening for cystic fibrosis after a negative sweat-test.
Topics: Adult; Comprehension; Cystic Fibrosis; Female; Genetic Carrier Screening; Genetic Counseling; Health | 2011 |
Low abundance of sweat duct Cl- channel CFTR in both healthy and cystic fibrosis athletes with exceptionally salty sweat during exercise.
Topics: Adult; Aldosterone; Aquaporin 5; Bicycling; Biopsy; Case-Control Studies; Cystic Fibrosis; Cystic Fi | 2011 |
Clinical outcomes in infants with cystic fibrosis transmembrane conductance regulator (CFTR) related metabolic syndrome.
Topics: Anti-Bacterial Agents; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2011 |
An infant with alternating metabolic acidosis and alkalosis: question.
Topics: Acidosis; Acute Kidney Injury; Alkalosis; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane | 2012 |
Sweat analysis using indirect ion-selective electrode on the routine chemistry analyser meets UK guidelines.
Topics: Chlorides; Cystic Fibrosis; Humans; Ion-Selective Electrodes; Reference Standards; Sodium; Sweat | 2011 |
High-sweat Na+ in cystic fibrosis and healthy individuals does not diminish thirst during exercise in the heat.
Topics: Adult; Aldosterone; Angiotensin II; Body Weight; Case-Control Studies; Cystic Fibrosis; Dehydration; | 2011 |
Clinical practices for intermediate sweat tests following abnormal cystic fibrosis newborn screens.
Topics: Cystic Fibrosis; Diagnostic Tests, Routine; Humans; Infant, Newborn; Neonatal Screening; Practice Pa | 2011 |
Cystic fibrosis mutations for p.F508del compound heterozygotes predict sweat chloride levels and pancreatic sufficiency.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Heterozyg | 2012 |
A continuing role for sweat testing in an era of newborn screening for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivi | 2011 |
Elevated sweat chloride concentration in children without cystic fibrosis who are receiving topiramate therapy.
Topics: Adolescent; Analgesics; Anticonvulsants; Bronchi; Cells, Cultured; Child; Chlorides; Cystic Fibrosis | 2012 |
Factors accounting for a missed diagnosis of cystic fibrosis after newborn screening.
Topics: Chlorides; Cystic Fibrosis; Diagnostic Errors; Female; Humans; Infant; Infant, Newborn; Male; Neonat | 2011 |
Comparing age of cystic fibrosis diagnosis and treatment initiation after newborn screening with two common strategies.
Topics: Cystic Fibrosis; DNA Mutational Analysis; Genetic Carrier Screening; Genotyping Techniques; Humans; | 2012 |
Impact of IVS8-(TG)m(T)n on IRT and sweat chloride levels in newborns identified by California CF newborn screening.
Topics: California; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance | 2012 |
Lessons learned from 20 years of newborn screening for cystic fibrosis.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; DNA Mutational Analysis; | 2012 |
Improving test properties for neonatal cystic fibrosis screening in the Netherlands before the nationwide start by May 1st 2011.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Testing; Humans; Infan | 2012 |
Diagnosing cystic fibrosis: what are we sweating about?
Topics: Adolescent; Adult; Child; Cystic Fibrosis; Diagnosis, Differential; Humans; Sweat; Sweating; Young A | 2012 |
Detection of a large heterozygous deletion and a splicing defect in the CFTR transcripts from nasal swab of a Japanese case of cystic fibrosis.
Topics: Alleles; Asian People; Base Sequence; Chlorides; Cloning, Molecular; Cystic Fibrosis; Cystic Fibrosi | 2012 |
Comparison of quantitative sweat chloride methods after positive newborn screen for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Iontophoresis; Male; Muscarinic | 2012 |
β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis.
Topics: Adolescent; Adrenergic beta-Agonists; Adult; Aged; Biomarkers; Case-Control Studies; Chlorides; Cyst | 2012 |
Cystic fibrosis infant care challenges in diagnosis and management in the era of newborn screening.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Deoxyribonuclease I; Disease P | 2012 |
To sweat or not to sweat: is that enough to diagnose cystic fibrosis?
Topics: Adrenergic beta-Agonists; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Fema | 2012 |
Effects of immediate telephone follow-up with providers on sweat chloride test timing after cystic fibrosis newborn screening identifies a single mutation.
Topics: Chlorides; Cohort Studies; Cystic Fibrosis; Female; Health Communication; Humans; Infant; Infant, Ne | 2013 |
Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience.
Topics: Aminophenols; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Co | 2013 |
CFTR p.Arg117His associated with CBAVD and other CFTR-related disorders.
Topics: Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Femal | 2013 |
Response to "Use of nasal potential differences and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis".
Topics: Chloride Channels; Clinical Trials as Topic; Cystic Fibrosis; Humans; Membrane Potentials; Multicent | 2002 |
Screening for cystic fibrosis in newborn infants: results of a pilot programme based on a two tier protocol (IRT/DNA/IRT) in the Italian population.
Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Genet | 2002 |
Variant cystic fibrosis phenotypes in the absence of CFTR mutations.
Topics: Adolescent; Adult; Chi-Square Distribution; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembran | 2002 |
[A food loving child with rhinitis].
Topics: Appetite; Child, Preschool; Cystic Fibrosis; Diarrhea; Female; Humans; Rectal Prolapse; Rhinitis; Sw | 2001 |
CFTR gene mutations in patients suffering from acute pancreatitis.
Topics: Adult; Aged; Alleles; Chlorine; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator | 2002 |
[Clinical and molecular genetic study of cystic fibrosis in the 5th Region of Chile].
Topics: Adolescent; Adult; Child; Child, Preschool; Chile; Cystic Fibrosis; Cystic Fibrosis Transmembrane Co | 2002 |
An indigenously developed method for sweat collection and estimation of chloride for diagnosis of cystic fibrosis.
Topics: Cystic Fibrosis; Iontophoresis; Observer Variation; Reproducibility of Results; Specimen Handling; S | 2002 |
Modified method to measure nasal potential difference.
Topics: Adolescent; Adult; Amiloride; Biological Transport, Active; Case-Control Studies; Child; Chlorides; | 2003 |
Molecular diagnosis of cystic fibrosis in Indian patients--a preliminary report.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembran | 2003 |
Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis.
Topics: Action Potentials; Adult; Aged; Case-Control Studies; Chloride Channels; Chlorides; Cholangitis, Scl | 2003 |
Analysis of the costs of diagnosing cystic fibrosis with a newborn screening program.
Topics: Chlorides; Cost Savings; Cystic Fibrosis; Health Care Costs; Humans; Infant, Newborn; Monte Carlo Me | 2003 |
An indigenously developed method for sweat collection.
Topics: Cystic Fibrosis; Humans; Iontophoresis; Sweat | 2003 |
A girl with cystic fibrosis and failure to thrive.
Topics: Amebicides; Biopsy; Celiac Disease; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Dientamoebi | 2003 |
Mutations located in exon 24 of the CFTR gene are associated with a mild cystic fibrosis phenotype.
Topics: Adolescent; Adult; Age of Onset; Amino Acid Sequence; Amino Acid Substitution; Chlorides; Codon, Ter | 2003 |
CFTR genotypes in patients with normal or borderline sweat chloride levels.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembran | 2003 |
Sweat test results in children with primary protein energy malnutrition.
Topics: Case-Control Studies; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Electri | 2003 |
Electrolyte abnormalities of the sweat in fibrocystic disease of the pancreas.
Topics: Cystic Fibrosis; Disease; Electrolytes; Pancreas; Pancreatic Diseases; Sweat; Sweating | 1953 |
Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas.
Topics: Cystic Fibrosis; Disease; Electrolytes; Humans; Pancreas; Pancreatic Diseases; Sweat | 1953 |
Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas; clinical significance and relationship to the disease.
Topics: Cystic Fibrosis; Disease; Electrolytes; Humans; Pancreas; Pancreatic Diseases; Sweat | 1953 |
ELECTROLYTE losses from sweat in fibrocystic disease.
Topics: Cystic Fibrosis; Disease; Electrolytes; Humans; Pancreas; Pancreatic Diseases; Sweat; Sweating | 1956 |
Measurement of the electrical conductivity of sweat; its application to the study of cystic fibrosis of the pancreas.
Topics: Cystic Fibrosis; Electric Conductivity; Humans; Sweat | 1957 |
The electrolyte content of the sweat in fibrocystic disease of the pancreas.
Topics: Cystic Fibrosis; Electrolytes; Humans; Sweat | 1957 |
The sweat test in cystic fibrosis of the pancreas.
Topics: Cystic Fibrosis; Humans; Pancreas; Sweat | 1957 |
[A simple method for detecting increased salt excretion in sweat].
Topics: Cystic Fibrosis; Humans; Pancreas; Sodium Chloride; Sodium Chloride, Dietary; Sweat; Sweating | 1957 |
Fingerprint sweat test in fibrocystic disease of pancreas; preliminary communication.
Topics: Communication; Cystic Fibrosis; Electrolytes; Female; Fibrocystic Breast Disease; Humans; Pancreas; | 1957 |
The sweat test in the diagnosis of fibrocystic disease of the pancreas.
Topics: Cystic Fibrosis; Female; Fibrocystic Breast Disease; Humans; Pancreas; Sweat | 1957 |
[Practical importance of analysis of electrolytes in sweat in children].
Topics: Anemia; Child; Cystic Fibrosis; Electrolytes; Humans; Infant; Nephrosis; Pancreas; Pneumonia; Sweat | 1957 |
[Value of the sweat test in pancreatic fibrosis].
Topics: Cystic Fibrosis; Humans; Pancreas; Sweat | 1957 |
Sweat analysis in fibrocystic disease, chronic pulmonary disease and controls.
Topics: Child; Chronic Disease; Cystic Fibrosis; Humans; Infant; Lung Diseases; Sweat | 1958 |
Sweat test for the diagnosis of fibrocystic disease of the pancreas; report of a fatality.
Topics: Cystic Fibrosis; Humans; Sweat | 1958 |
A simple method of sweat collection with analysis of electrolytes in patients with fibrocystic disease of the pancreas, and their families.
Topics: Cystic Fibrosis; Electrolytes; Humans; Pancreas; Sweat | 1958 |
The finger-print sweat test.
Topics: Cystic Fibrosis; Dermatoglyphics; Humans; Sweat | 1958 |
[Sweat test by intradermal injection of a synthetic parasympathomimetic: Alvin, M. Mauer & Clark D. West method].
Topics: Cystic Fibrosis; Humans; Injections, Intradermal; Pancreas; Parasympathomimetics; Sweat; Sweating | 1958 |
[Electrolyte content of sweat as a diagnostic sign in cystic fibrosis of the pancreas].
Topics: Cystic Fibrosis; Electrolytes; Humans; Pancreas; Physical Examination; Sweat | 1958 |
Saliva, tears and duodenal contents in cystic fibrosis of the pancreas.
Topics: Cystic Fibrosis; Duodenum; Humans; Lacrimal Apparatus; Saliva; Sweat; Tears | 1958 |
Performance of the sweat test under standardized conditions.
Topics: Cystic Fibrosis; Humans; Pancreas; Reference Standards; Sweat | 1958 |
Sweat tests for diagnosis of cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Pancreas; Sweat | 1958 |
[Diagnostic value of the sweat test in incomplete types of mucoviscidosis].
Topics: Cystic Fibrosis; Humans; Pancreas; Sweat | 1958 |
A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis.
Topics: Chlorides; Cystic Fibrosis; Electrolytes; Humans; Iontophoresis; Pilocarpine; Sodium; Sodium, Dietar | 1959 |
A patch test for chloride in sweat as a simple screening method for detecting cystic fibrosis of the pancreas; preliminary report.
Topics: Chlorides; Cystic Fibrosis; Humans; Patch Tests; Sweat | 1959 |
A comparison of sweat chlorides and intestinal fat absorption in chronic obstructive pulmonary emphysema and fibrocystic disease of the pancreas.
Topics: Chlorides; Cystic Fibrosis; Emphysema; Fats; Humans; Intestinal Absorption; Intestines; Pancreas; Pu | 1959 |
Evaluation of the finger imprint method for sweat electrolyte estimation in fibrocystic disease of the pancreas.
Topics: Cystic Fibrosis; Electrolytes; Fingers; Humans; Pancreas; Regression Analysis; Sweat | 1959 |
'Sweat test' results in normal persons of different ages compared with families with fibrocystic disease of the pancreas.
Topics: Cystic Fibrosis; Humans; Sweat | 1960 |
[The sweat test in pancreatic cystic fibrosis].
Topics: Cystic Fibrosis; Humans; Sweat | 1960 |
Components of sweat. Cystic fibrosis of the pancreas compared with controls.
Topics: Cystic Fibrosis; Humans; Sweat | 1961 |
[Electrolyte concentrations in the sweat of children with pancreatic cystic fibrosis, Addison's disease and hypophyseal dwarfism].
Topics: Addison Disease; Adrenal Insufficiency; Child; Cystic Fibrosis; Dwarfism; Electrolytes; Humans; Hypo | 1961 |
Thiocyanate secretion in sweat in cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Sweat; Thiocyanates | 1961 |
[The composition of sweat in patients with pancreatic fibrosis].
Topics: Cystic Fibrosis; Humans; Sweat | 1960 |
[On mucoviscidosis in adults and diabetes mellitus].
Topics: Anions; Chlorides; Cystic Fibrosis; Diabetes Mellitus; Humans; Ions; Sodium; Sodium, Dietary; Sweat | 1961 |
[Technic and interpretation of the sweat test in the adult].
Topics: Adult; Cystic Fibrosis; Humans; Sweat | 1961 |
[Determination of sweat electrolytes after pilocarpin iontophoresis].
Topics: Cystic Fibrosis; Electrolytes; Humans; Iontophoresis; Pilocarpine; Sweat | 1961 |
[Mucoviscidosis-role of sweat examination].
Topics: Cystic Fibrosis; Humans; Sweat | 1960 |
[Sweat production with the aid of iontophoresis in the diagnosis of hereditary mucoviscidosis in children].
Topics: Child; Cystic Fibrosis; Humans; Iontophoresis; Sweat; Sweating | 1961 |
The electrical conductivity of electrolytes found in the sweat of patients with fibrocystic disease of the pancreas.
Topics: Cystic Fibrosis; Electric Conductivity; Electrolytes; Humans; Sweat | 1959 |
Sweat iodide excretion in patients with cystic fibrosis of the pancreas.
Topics: Biological Transport; Cystic Fibrosis; Humans; Iodides; Sweat | 1960 |
A study of the free amino acids in sweat from patients with cystic fibrosis.
Topics: Amino Acids; Antifibrinolytic Agents; Biochemical Phenomena; Cystic Fibrosis; Humans; Sweat | 1960 |
Observations on (i) sweat sodium levels in relation to chronic respiratory disease in adults and (ii) the incidence of respiratory and other disease in parents and siblings of patients with fibrocystic disease of the pancreas.
Topics: Adult; Chronic Disease; Cystic Fibrosis; Disease; Humans; Incidence; Pancreas; Parents; Respiration | 1962 |
Sweat chloride concentration in children with allergy and with cystic fibrosis of the pancreas.
Topics: Child; Chlorides; Cystic Fibrosis; Humans; Hypersensitivity; Infant; Sweat | 1962 |
Observations on skin resistance to electricity and sweat chloride content. A preliminary report.
Topics: Central Nervous System Diseases; Chlorides; Cystic Fibrosis; Electricity; Humans; Skin Physiological | 1962 |
[The palm print test according to Knights' method for the routine investigation of pancreatic cystic fibrosis].
Topics: Cystic Fibrosis; Hand; Humans; Sweat | 1961 |
[Apropos of the attempted detection of incomplete forms of mucoviscidosis in adults. New contribution to the study of the sweat test].
Topics: Cystic Fibrosis; Humans; Sweat; Sweat Glands | 1961 |
[The respiratory forms of mucoviscidosis. Critical study of the sweat test].
Topics: Bronchopneumonia; Cystic Fibrosis; Humans; Sweat | 1961 |
Sweat collection in cystic fibrosis. Report of a simplified method employing pilocarpine iontophoresis and cellulose sponge.
Topics: Animals; Cellulose; Cystic Fibrosis; Humans; Iontophoresis; Pilocarpine; Porifera; Sweat; Sweating | 1962 |
Some practical simplifications of perspiration electrolyte analysis ("sweat test").
Topics: Cystic Fibrosis; Electrolytes; Sweat | 1961 |
Pilocarpine iontophoresis as a method of obtaining sweat for electrolyte determinations; a preliminary report.
Topics: Cystic Fibrosis; Electrolytes; Humans; Iontophoresis; Pilocarpine; Sweat | 1962 |
Acetylcholine in sweat in fibrocystic disease of the pancreas.
Topics: Acetylcholine; Cystic Fibrosis; Humans; Pancreas; Sweat | 1961 |
[Considerations on the modified Shwachman test for diagnosis of mucoviscidosis].
Topics: Cystic Fibrosis; Humans; Sweat | 1961 |
The sweat test.
Topics: Cystic Fibrosis; Humans; Sweat | 1962 |
[Sweat test. Physiological basis, methodology and clinical evaluation].
Topics: Cystic Fibrosis; Humans; Sweat | 1962 |
[The importance of sweat electrolyte concentration in the differential diagnosis of chronic nonspecific lungs diseases in childhood].
Topics: Child; Chronic Disease; Cystic Fibrosis; Diagnosis, Differential; Electrolytes; Humans; Infant; Lung | 1961 |
[A method for the determination of chlorides in the diagnosis of mucoviscidosis].
Topics: Chlorides; Cystic Fibrosis; Sweat | 1962 |
[Significance of the Na-plus and Cl-minus concentration in the sweat for the diagnosis of mucoviscidosis in adults].
Topics: Cystic Fibrosis; Humans; Sodium; Sweat | 1962 |
Response of sweat electrolyte concentrations to 9 alpha--fluorohydrocortisone in patients with cystic fibrosis and their families.
Topics: Cystic Fibrosis; Electrolytes; Fludrocortisone; Humans; Sweat | 1963 |
[Aldosterone blocks the sweat test in mucoviscidosis patients].
Topics: Aldosterone; Cystic Fibrosis; Humans; Sweat | 1962 |
[Determination of sweat electrolytes].
Topics: Asthma; Chlorides; Cystic Fibrosis; Deficiency Diseases; Electrolytes; Humans; Lung Diseases; Sweat | 1963 |
Electrical conductivity of sweat: a simple, home-assembled apparatus.
Topics: Cystic Fibrosis; Electric Conductivity; Humans; Sweat | 1963 |
Studies of salt excretion in sweat. Relationships between rate, conductivity, and electrolyte composition of sweat from patients with cystic fibrosis and from control subjects.
Topics: Cystic Fibrosis; Electrolytes; Humans; Potassium; Sodium Chloride; Sweat; Sweating | 1963 |
[Critical study of the sweat test for detection of mucoviscidosis heterozygotes].
Topics: Cystic Fibrosis; Heterozygote; Humans; Sweat | 1962 |
Electrical conductivity of sweat: a simple diagnostic test in children.
Topics: Child; Cystic Fibrosis; Diagnostic Tests, Routine; Electric Conductivity; Humans; Sweat | 1963 |
[Electrolyte concentrations in the sweat of newborn infants].
Topics: Cystic Fibrosis; Electrolysis; Electrolytes; Humans; Infant; Infant, Newborn; Sweat | 1961 |
The excretion of Na-22 in sweat.
Topics: Biological Transport; Cystic Fibrosis; Lung Diseases; Sodium; Sweat | 1963 |
[Mucoviscidosis and chronic broncho-pulmonary diseases. Results of the sweat test performed on 55 patients].
Topics: Bronchial Diseases; Chronic Disease; Cystic Fibrosis; Humans; Lung Diseases; Sweat; Sweating | 1962 |
Sweat chloride in patients with chronic bronchial disease and its relation to mucoviscidosis (cystic fibrosis).
Topics: Bronchial Diseases; Bronchitis; Chlorides; Chronic Disease; Cystic Fibrosis; Humans; Sweat | 1963 |
[Mucoviscidosis of the adult. Clinico-biologic findings].
Topics: Adult; Biological Products; Bronchiectasis; Bronchitis; Cystic Fibrosis; Diabetes Mellitus; Humans; | 1962 |
[Sweat levels of K in mucoviscidosis of the adult].
Topics: Adult; Bronchitis; Cystic Fibrosis; Diabetes Mellitus; Humans; Peptic Ulcer; Potassium; Sweat | 1962 |
[ON METHODS OF SWEAT PRODUCTION FOR THE DIAGNOSIS OF MUCOVISCIDOSIS].
Topics: Baths; Child; Cystic Fibrosis; Humans; Iontophoresis; Pilocarpine; Sweat; Sweating | 1963 |
THE MECHANISM OF ECCRINE SWEAT-GLAND FUNCTION IN NORMAL SUBJECTS AND IN PATIENTS WITH MUCOVISCOIDOSIS.
Topics: Cystic Fibrosis; Eccrine Glands; Electrolytes; Histocytochemistry; Humans; Physiology; Sweat; Sweat | 1963 |
[ON THE ASSOCIATION OF A CHRONIC BRONCHOPNEUMOPATHY, PANCREATIC INSUFFICIENCY AND A NORMAL SWEAT TEST. INTERPRETATIVE CONSIDERATIONS].
Topics: Bronchiectasis; Bronchitis; Bronchopneumonia; Child; Cystic Fibrosis; Diagnosis; Electrolytes; Exocr | 1963 |
ELEVATED SWEAT SODIUM AND CHLORIDE IN ADULT WITHOUT CYSTIC FIBROSIS.
Topics: Adolescent; Adult; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Genetics, Medical; Heat Exha | 1963 |
[MUCOVISCIDOSIS IN AN INTERNAL DISEASE CLINIC].
Topics: Cystic Fibrosis; Humans; Photometry; Sodium Chloride; Sweat | 1963 |
SALT EXCRETION IN SWEAT IN CYSTIC FIBROSIS.
Topics: Biological Transport; Cystic Fibrosis; Diagnosis; Humans; Sodium Chloride; Sweat | 1963 |
CYSTIC FIBROSIS OF THE PANCREAS. DIAGNOSIS BY APPLICATION OF A SODIUM ELECTRODE TO THE SKIN.
Topics: Cystic Fibrosis; Electrodes; Electrodiagnosis; Humans; Skin; Sodium; Sodium, Dietary; Sweat; Sweatin | 1963 |
STUDY OF THE GALVANIC SKIN RESPONSE IN CYSTIC FIBROSIS.
Topics: Adolescent; Child; Chlorides; Cystic Fibrosis; Galvanic Skin Response; Humans; Infant; Potassium; So | 1963 |
SWEAT SODIUM CONTENT AND FLOW RATE IN CYSTIC FIBROSIS OF THE PANCREAS.
Topics: Adolescent; Child; Cystic Fibrosis; Humans; Injections; Ions; Iontophoresis; Metabolism; Methacholin | 1963 |
EVALUATION OF THE SWEAT CHLORIDE ASSAY IN ADULTS: USE OF PILOCARPINE IONTOPHORESIS.
Topics: Adolescent; Adult; Aging; Child; Chlorides; Cystic Fibrosis; Humans; Infant; Iontophoresis; Lung Dis | 1963 |
MUCOVISCIDOSIS AND CHRONIC LUNG DISEASE IN ADULTS.
Topics: Adult; Bronchiectasis; Bronchitis; Chlorides; Cystic Fibrosis; Genetics; Humans; Lung Diseases; Sodi | 1963 |
[STUDY OF THE SWEAT TEST IN DIABETIC CHILDREN].
Topics: Adolescent; Child; Cystic Fibrosis; Diabetes Mellitus; Diabetes Mellitus, Type 1; Diagnosis; Humans; | 1963 |
[THE PROBLEM OF MUCOVISCIDOSIS IN ADULTS BASED ON SWEAT ELECTROLYTE TESTS].
Topics: Adult; Bronchitis; Colitis; Colitis, Ulcerative; Cystic Fibrosis; Diabetes Mellitus; Duodenal Ulcer; | 1963 |
[MUCOVISCIDOSIS IN ADULTS, A RARE DISEASE].
Topics: Adult; Anions; Chlorides; Cystic Fibrosis; Humans; Ions; Rare Diseases; Sodium; Sodium, Dietary; Swe | 1963 |
[MUCOVISCIDOSIS].
Topics: Adolescent; Anti-Bacterial Agents; Antibiotics, Antitubercular; Blood Protein Disorders; Bronchopneu | 1963 |
THE EFFECT OF ACETAZOLAMIDE ON SWEAT ELECTROLYTES IN MUCOVISCIDOSIS.
Topics: Acetazolamide; Carbonic Anhydrases; Child; Chlorides; Cystic Fibrosis; Electrolytes; Humans; Iontoph | 1963 |
[A CASE OF PURE BRONCHOPULMONARY MUCOVISCIDOSIS IN AN 11-YEAR-OLD BOY].
Topics: Bronchiectasis; Bronchography; Bronchopneumonia; Bronchospirometry; Cardiac Catheterization; Cystic | 1963 |
[STATISTICAL STUDY OF RESULTS OF THE SWEAT TEST IN 595 ADULTS (200 CONTROLS, 190 BRONCHO-EMPHYSEMA PATIENTS, 160 DIABETICS, 45 SPONTANEOUS PNEUMOTHORAX PATIENTS)].
Topics: Bronchitis; Cystic Fibrosis; Diabetes Mellitus; Emphysema; Pneumothorax; Pulmonary Emphysema; Sweat; | 1963 |
[A RECENT CASE OF LYMPHOCYTO-PHTHISIS WITH HYPOGAMMAGLOBULINEMIA, HAVING PREDOMINANTLY RESPIRATORY MANIFESTATIONS].
Topics: Agammaglobulinemia; Cystic Fibrosis; Dermatomycoses; Diagnosis, Differential; Hematology; Humans; Ly | 1963 |
ALBUMIN IN THE MECONIUM OF INFANTS WITH CYSTIC FIBROSIS: A PRELIMINARY REPORT.
Topics: Albumins; Chlorides; Cystic Fibrosis; Diagnosis; Humans; Immunoelectrophoresis; Infant; Infant, Newb | 1964 |
[MUCOVISCIDOSIS AND CHRONIC LUNG DISEASES IN ADULTS].
Topics: Chlorides; Chronic Disease; Cystic Fibrosis; Humans; Lung Diseases; Sweat | 1963 |
[DETERMINATION OF SODIUM AND CHLORIDE IN THE SWEAT OF ADULT CHRONIC BRONCHITICS].
Topics: Adult; Bronchitis; Chlorides; Colorimetry; Cystic Fibrosis; Geriatrics; Humans; Ions; Photometry; So | 1964 |
[CHRONIC BRONCHITIS AND MUCOVISCIDOSIS].
Topics: Adolescent; Bronchitis; Bronchitis, Chronic; Cystic Fibrosis; Diagnosis; Geriatrics; Humans; Sweat | 1964 |
[MUCOVISCIDOSIS, WITH SPECIAL REFERENCE TO 3 CLINICAL CASES].
Topics: Adolescent; Bronchitis; Cystic Fibrosis; Electrolytes; Humans; Lung Neoplasms; Pulmonary Atelectasis | 1964 |
[EVALUATION OF THE SHWACHMAN-GAHM TEST IN VARIOUS CHILDHOOD DISEASES].
Topics: Adolescent; Asthma; Biomedical Research; Child; Chlorides; Clinical Laboratory Techniques; Cystic Fi | 1964 |
[IMMUNOELECTROPHORESIS OF PROTEINS IN SWEAT AND ON THE PROBLEM OF ITS USE IN THE LABORATORY DIAGNOSIS OF MUCOVISCIDOSIS].
Topics: Antigens; Clinical Laboratory Techniques; Cystic Fibrosis; Humans; Immunoelectrophoresis; Pilocarpin | 1964 |
ELECTROENCEPHALOGRAMS OF PATIENTS WITH CYSTIC FIBROSIS.
Topics: Adolescent; Black People; Blood Chemical Analysis; Blood Gas Analysis; Child; Chlorides; Cystic Fibr | 1964 |
EFFECT OF EXPERIMENTAL SALT DEPLETION AND ALDOSTERONE LOAD ON SODIUM AND CHLORIDE CONCENTRATION IN THE SWEAT OF PATIENTS WITH CYSTIC FIBROSIS OF THE PANCREAS AND OF NORMAL CHILDREN.
Topics: Adolescent; Aldosterone; Biochemical Phenomena; Biochemistry; Body Weight; Child; Chlorides; Chlorth | 1964 |
[SIGNIFICANCE AND LIMITS OF THE SWEAT TEST AS AN ORIENTATION TO THE DIAGNOSIS OF MUCOVISCIDOSIS. POSSIBILITY OF A SEMIQUANTITATIVE EVALUATION OF CHLORIDES IN SWEAT WITH KNIGHTS' TEST].
Topics: Biomedical Research; Child; Chlorides; Cystic Fibrosis; Diagnosis; Genetics, Medical; Humans; Infant | 1963 |
[BRONCHOPULMONARY MUCOVISCIDOSIS IN THE CHILD].
Topics: Aerosols; Anti-Bacterial Agents; Antibiotics, Antitubercular; Bronchopneumonia; Child; Cystic Fibros | 1964 |
SPECTROSCOPIC ANALYSIS OF TISSUES FROM PATIENTS WITH CYSTIC FIBROSIS AND CONTROLS.
Topics: Aluminum; Blood; Boron; Cobalt; Copper; Cystic Fibrosis; Duodenum; Exudates and Transudates; Hair; H | 1964 |
[MUCOVISCIDOSIS AND OTORHINOLARYNGOLOGICAL MANIFESTATIONS].
Topics: Child; Cystic Fibrosis; Diagnosis; Electrolytes; Humans; Nasal Mucosa; Nasal Polyps; Paranasal Sinus | 1963 |
[A CASE OF PULMONARY MUCOVISCIDOSIS IN AN ADULT].
Topics: Adult; Cystic Fibrosis; Diagnosis, Differential; Humans; Lung Diseases; Sweat; Tuberculosis; Tubercu | 1964 |
[MUCOVISCIDOSIS OF THE ADULT: A NOT TOO RARE DISEASE].
Topics: Adult; Bronchitis; Cystic Fibrosis; Diagnosis; Duodenal Ulcer; Humans; Radiography, Thoracic; Rare D | 1964 |
A NEW METHOD OF IONTOPHORESIS AND ANALYSIS OF SWEAT ELECTROLYTE.
Topics: Chemistry Techniques, Analytical; Cystic Fibrosis; Electrolytes; Genetics, Medical; Humans; Iontopho | 1964 |
SWEAT CHLORIDE CONCENTRATION; A COMPARATIVE STUDY IN CHILDREN WITH BRONCHIAL ASTHMA AND WITH CYSTIC FIBROSIS.
Topics: Adolescent; Asthma; Biomedical Research; Black People; Chemistry Techniques, Analytical; Child; Chlo | 1965 |
THE ELECTROLYTE ABNORMALITY IN CYSTIC FIBROSIS.
Topics: Acidosis; Anti-Bacterial Agents; Bronchi; Bronchiectasis; Child; Chlorides; Cystic Fibrosis; Drug Th | 1964 |
SWEAT CHLORIDE BY IONTOPHORESIS.
Topics: Chlorides; Clinical Laboratory Techniques; Cystic Fibrosis; Humans; Infant; Iontophoresis; Pilocarpi | 1964 |
CYSTIC FIBROSIS IN NEGRO CHILDREN: RESULTS OF A SEARCH.
Topics: Adolescent; Black People; Child; Chlorides; Cystic Fibrosis; District of Columbia; Humans; Infant; I | 1964 |
STUDIES IN CYSTIC FIBROSIS: ANALYSIS OF NAIL CLIPPINGS FOR SODIUM AND POTASSIUM.
Topics: Cystic Fibrosis; Diagnosis; Genetics, Medical; Humans; Ions; Nails; Potassium; Sodium; Sodium, Dieta | 1965 |
A STANDARD PROCEDURE FOR MEASURING CONDUCTIVITY OF SWEAT AS A DIAGNOSTIC TEST FOR CYSTIC FIBROSIS.
Topics: Adolescent; Child; Cystic Fibrosis; Diagnosis; Diagnostic Tests, Routine; Humans; Infant; Sweat | 1965 |
CONTINUOUS RECORDING OF SWEATING BY RESISTANCE HYGROMETRY.
Topics: Arrhythmias, Cardiac; Biochemical Phenomena; Biochemistry; Child; Chlorides; Cystic Fibrosis; Electr | 1965 |
[RESULTS OF MASS TESTS FOR MINIMAL OR RESIDUAL CASES OF MUCOVISCIDOSIS].
Topics: Biometry; Child; Cystic Fibrosis; Humans; Infant; Mass Screening; Poland; Statistics as Topic; Sweat | 1964 |
A NEW METHOD OF IONTOPHORESIS AND ANALYSIS OF SWEAT ELECTROLYTE: A SURVEY OF SEVERAL KINDRED WITH CYSTIC FIBROSIS.
Topics: Child; Cystic Fibrosis; Data Collection; Electrolytes; Genetics, Medical; Geriatrics; Humans; Iontop | 1965 |
SWEAT CHLORIDES IN SALT-DEPRIVED CYSTIC FIBROSIS HETEROZYGOTES.
Topics: Aldosterone; Carrier State; Child; Chlorides; Cystic Fibrosis; Diagnosis; Diet, Sodium-Restricted; H | 1965 |
[THE IMPRINT TEST IN SCREENING FOR MUCOVISCIDOSIS].
Topics: Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Sweat | 1965 |
STUDIES OF SWEAT ELECTROLYTES IN JAPANESE CHILDREN.
Topics: Adolescent; Asian People; Child; Cystic Fibrosis; Electrolytes; Humans; Infant; Infant, Newborn; Jap | 1964 |
THE SILVER ELECTRODE METHOD FOR RAPID ANALYSIS OF SWEAT CHLORIDE.
Topics: Chlorides; Cystic Fibrosis; Diagnosis; Electrodes; Humans; Silver; Sweat | 1965 |
[GENE CARRIERS OF MUCOVISICIDOSIS AND THE DETERMINATION OF SUDORAL CHLORIDE. (PRELIMINARY RESULTS)].
Topics: Chlorides; Cystic Fibrosis; Genetics, Medical; Heterozygote; Humans; Infant; Sweat | 1965 |
[SODIUM AND POTASSIUM DETERMINATION IN THE SWEAT OF CHILDREN WITH CHRONIC OR RECURRENT LUNG INFECTIONS].
Topics: Child; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Potassium; Respiratory Tract Infections; So | 1965 |
[A CASE OF MUCOVISCIDOSIS IN AN ADULT TREATED AS PULMONARY TUBERCULOSIS].
Topics: Adult; Cystic Fibrosis; Diagnosis, Differential; Humans; Sweat; Tuberculosis; Tuberculosis, Pulmonar | 1965 |
[SODIUM AND POTASSIUM CONTENT IN THE SWEAT OF PATIENTS WITH GASTRIC ULCER AND CHRONIC BRONCHITIS].
Topics: Adolescent; Bronchitis; Bronchitis, Chronic; Child; Cystic Fibrosis; Geriatrics; Humans; Peptic Ulce | 1965 |
[A simplified "sweat test"].
Topics: Cystic Fibrosis; Humans; Sweat; Sweating | 1959 |
Consideration of cystic fibrosis in adults, with a study of sweat electrolyte values.
Topics: Adult; Cystic Fibrosis; Electrolytes; Humans; Sweat | 1959 |
[Cystic fibrosis in adults. Study of electrolyte values of sweat].
Topics: Adult; Cystic Fibrosis; Electrolytes; Humans; Sweat | 1960 |
[Shwachmann's test in mucoviscidosis and in other diseases].
Topics: Chlorides; Cystic Fibrosis; Humans; Sweat | 1960 |
Fibrocystic disease and sweat.
Topics: Cystic Fibrosis; Humans; Pancreas; Sweat | 1961 |
[Experiences with the fingerprint test by Shwachman and Gahm in adults with chronic emphysema bronchitis and ulcer with reference to mucoviscidosis].
Topics: Bronchitis; Bronchitis, Chronic; Cystic Fibrosis; Emphysema; Lung; Peptic Ulcer; Pulmonary Emphysema | 1962 |
[Sweat electrolyte values in adults and their relation to mucoviscoidosis].
Topics: Cystic Fibrosis; Electrolytes; Humans; Sweat | 1961 |
[Diabetes and mucoviscidosis].
Topics: Chlorides; Cystic Fibrosis; Diabetes Mellitus; Humans; Sweat | 1962 |
Effect of age, sex, and cystic fibrosis on the sodium and potassium content of human sweat.
Topics: Aging; Cystic Fibrosis; Humans; Potassium; Sex; Sodium; Sodium, Dietary; Sweat | 1962 |
[Role of the sweat test in diseases in children with special reference to mucoviscidosis].
Topics: Child; Cystic Fibrosis; Humans; Sweat | 1962 |
Effect of sodium intake upon sweat electrolyte concentrations in children.
Topics: Child; Cystic Fibrosis; Diet; Electrolytes; Humans; Nutrition Assessment; Nutritional Status; Sodium | 1962 |
[Determination of excessive chlorine excretion by the skin in pancreatic fibrosis].
Topics: Body Fluids; Chlorine; Cystic Fibrosis; Humans; Pancreatic Diseases; Skin; Sweat | 1962 |
[The sweat test with intradermal injection of pilocarpine nitrate].
Topics: Cystic Fibrosis; Humans; Injections, Intradermal; Pilocarpine; Sweat; Sweating | 1961 |
Serum CA 19-9 levels as a diagnostic marker in cystic fibrosis patients with borderline sweat tests.
Topics: Adolescent; Adult; CA-19-9 Antigen; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductanc | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis.
Topics: Adolescent; Child; Cystic Fibrosis; Depressive Disorder; Female; Humans; Male; Predictive Value of T | 2003 |
Sweat test results in children with primary protein energy malnutrition.
Topics: Chlorides; Cystic Fibrosis; Humans; Protein-Energy Malnutrition; Sweat | 2004 |
Sweat test results in children with primary protein energy malnutrition.
Topics: Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Prevalence; Protein-Energy Malnutrition | 2004 |
An analysis of communication following newborn screening for cystic fibrosis.
Topics: Adult; Alleles; Communication; Cystic Fibrosis; Female; Genetic Counseling; Genetic Testing; Humans; | 2004 |
Heat wave and acute pancreatitis: very unusual cystic fibrosis presentation.
Topics: Acute Disease; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnostic Errors; Exocrine Panc | 2004 |
Mutations in CFTR gene and clinical correlation in Argentine patients with congenital bilateral absence of the vas deferens.
Topics: Adult; Argentina; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Gene | 2004 |
Relation of sweat chloride concentration to severity of lung disease in cystic fibrosis.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Female; Humans; Male; Severity of Illness Inde | 2004 |
Sweat testing in newborns positive to neonatal screening for cystic fibrosis.
Topics: Aging; Chlorides; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Infant, Newborn; Male; Neonata | 2004 |
Disease severity associated with cystic fibrosis mutations deltaF508 and S549R(T-->G).
Topics: Amino Acid Substitution; Arabs; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis | 2001 |
CF or not CF? That is the question.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 2002 |
Sweat conductivity and chloride titration for cystic fibrosis diagnosis in 3834 subjects.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electric Conductivity; Femal | 2003 |
Diagnosis of cystic fibrosis in adults with diffuse bronchiectasis.
Topics: Adolescent; Adult; Bronchiectasis; Chlorides; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Trans | 2004 |
Sweat conductivity for the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Electric Conductivity; Humans; Infant; | 2004 |
Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood.
Topics: Adult; Cystic Fibrosis; DNA Mutational Analysis; Female; Forced Expiratory Volume; Humans; Male; Ret | 2004 |
Clinical problem-solving. Unfashionably late.
Topics: Adolescent; Biopsy; Burkholderia cepacia; Burkholderia Infections; Chest Pain; Chlorides; Cough; Cys | 2005 |
Misdiagnosing cystic fibrosis in the era of gene analysis: case reports.
Topics: Amino Acid Substitution; Child; Cystic Fibrosis; Female; Humans; Infant, Newborn; Mutation; Penetran | 2005 |
Testing diagnostic tests: why size matters.
Topics: Chlorides; Cystic Fibrosis; Diagnostic Tests, Routine; Humans; Reproducibility of Results; Sample Si | 2005 |
Cystic fibrosis in Korean children:a case report identified by a quantitative pilocarpine iontophoresis sweat test and genetic analysis.
Topics: Blood Pressure; Bronchiectasis; Child; Cough; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conduct | 2005 |
Sweat-testing in preterm and full-term infants less than 6 weeks of age.
Topics: Black or African American; Body Weight; Chlorides; Cystic Fibrosis; Female; Gestational Age; Humans; | 2005 |
Cystic fibrosis.
Topics: Absorption; Body Fluids; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Human | 2005 |
Cystic fibrosis.
Topics: Absorption; Body Fluids; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Human | 2005 |
Cystic fibrosis.
Topics: Absorption; Body Fluids; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Human | 2005 |
Cystic fibrosis.
Topics: Absorption; Body Fluids; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Human | 2005 |
Psychosocial risk associated with newborn screening for cystic fibrosis: parents' experience while awaiting the sweat-test appointment.
Topics: Adaptation, Psychological; Adult; Anxiety; Appointments and Schedules; Attitude to Health; Chlorides | 2005 |
A CFTR mutation (D1152H) in a family with mild lung disease and normal sweat chlorides.
Topics: Aged; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Family; Femal | 2005 |
Sweat collection for testing in Canadian Cystic Fibrosis Centers, is it optimal?
Topics: Adult; Canada; Child; Clinical Chemistry Tests; Cystic Fibrosis; Diagnostic Services; Humans; Infant | 2005 |
The sweat test: effect of elution time on chloride and sodium concentrations.
Topics: Chlorides; Cystic Fibrosis; Guidelines as Topic; Humans; Quality Control; Sodium; Sweat | 2005 |
Sweat testing infants detected by cystic fibrosis newborn screening.
Topics: Age Factors; Algorithms; Chlorides; Cystic Fibrosis; Decision Trees; DNA Mutational Analysis; Early | 2005 |
Newborn screening for cystic fibrosis in Wisconsin: nine-year experience with routine trypsinogen/DNA testing.
Topics: Age Factors; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mu | 2005 |
Diagnostic dilemmas resulting from the immunoreactive trypsinogen/DNA cystic fibrosis newborn screening algorithm.
Topics: Algorithms; Chlorides; Clinical Protocols; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductanc | 2005 |
Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes.
Topics: Chlorides; Colorado; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutat | 2005 |
Mild cystic fibrosis revealed by persistent hyponatremia during the French 2003 heat wave, associated with the S1455X C-terminus CFTR mutation.
Topics: Blood Chemical Analysis; Child; Chlorides; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transme | 2005 |
Sweat testing for cystic fibrosis: How good is your laboratory?
Topics: Australia; Clinical Chemistry Tests; Cystic Fibrosis; Humans; New Zealand; Surveys and Questionnaire | 2006 |
Sweat testing for cystic fibrosis: A review of New Zealand laboratories.
Topics: Chemistry, Clinical; Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Humans; Infant; Infant, N | 2006 |
Newborn screening for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; DNA Mutational Analysis; Genetic Testing; Humans; Infant, Newborn; Neona | 2006 |
Cystic fibrosis in 65- and 67-year-old siblings. Clinical feature and nasal potential difference measurement in patients with genotypes F508del and 2789+5G-->A.
Topics: Aged; Amiloride; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Ge | 2006 |
Diagnosing CF: sweat, blood and years.
Topics: Algorithms; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; | 2006 |
Cystic fibrosis detection in high-risk Egyptian children and CFTR mutation analysis.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; D | 2007 |
Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials.
Topics: Alleles; Amiloride; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; | 2006 |
Newborn screening for cystic fibrosis: parents' preferences regarding counseling at the time of infants' sweat test.
Topics: Adaptation, Psychological; Adolescent; Adult; Choice Behavior; Consumer Behavior; Cystic Fibrosis; C | 2006 |
The CF-CIRC study: a French collaborative study to assess the accuracy of cystic fibrosis diagnosis in neonatal screening.
Topics: Amiloride; Catheterization; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembr | 2006 |
The evaluation of a novel conductometric device for the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Case-Control Studies; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagno | 2006 |
Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.
Topics: Adult; Aged; Aged, 80 and over; Body Mass Index; Bronchiectasis; Chloride Channels; Chlorides; Cysti | 2006 |
Genetic and physiologic correlates of longitudinal immunoreactive trypsinogen decline in infants with cystic fibrosis identified through newborn screening.
Topics: Biomarkers; Body Height; Body Weight; Chlorides; Cystic Fibrosis; DNA Mutational Analysis; Fats; Fec | 2006 |
Cystic fibrosis presenting with haematological abnormalities.
Topics: Anemia; Bone Marrow Examination; Chlorides; Cystic Fibrosis; Diagnosis, Differential; DNA Mutational | 2006 |
Hyponatremic dehydration as a presentation of cystic fibrosis.
Topics: Alkalosis; Chlorides; Cystic Fibrosis; Dehydration; Diagnostic Tests, Routine; Female; Humans; Hypon | 2006 |
N1303K and IVS8-5T, clinical presentation within a family with atypical cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Fingerprinting; | 2007 |
The sweat test for the diagnosis of cystic fibrosis--a personal experience of guideline production.
Topics: Cystic Fibrosis; Evidence-Based Medicine; Humans; Infant, Newborn; Neonatal Screening; Practice Guid | 2007 |
Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report.
Topics: Algorithms; Cystic Fibrosis; Humans; Infant, Newborn; Neonatal Screening; Outcome Assessment, Health | 2007 |
Is early identification of asymptomatic infants with 'mild' CFTR genotypes clinically useful?
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; F | 2007 |
Analysis of CFTR gene mutations and cystic fibrosis incidence in the Ecuadorian population.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 2007 |
Nanoduct sweat testing for rapid diagnosis in newborns, infants and children with cystic fibrosis.
Topics: Child; Child, Preschool; Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Female; Humans; Infan | 2008 |
Sweat testing in CF.
Topics: Chlorides; Cystic Fibrosis; Humans; Sweat | 2007 |
Sweat testing practice in Swiss hospitals.
Topics: Adult; Child; Chlorides; Clinical Chemistry Tests; Cystic Fibrosis; Electrolytes; Humans; Osmolar Co | 2007 |
Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines.
Topics: Cystic Fibrosis; Diagnostic Tests, Routine; Female; Guideline Adherence; Humans; Infant, Newborn; Ma | 2007 |
[Genotype and phenotype of gastrointestinal symptoms analysis in children with cystic fibrosis].
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 2005 |
Acute intestinal obstruction as a presentation of cystic fibrosis in infancy.
Topics: Cystic Fibrosis; Diseases in Twins; Female; Humans; Infant; Intestinal Obstruction; Sweat; Syndrome; | 2008 |
Diagnosing cystic fibrosis--asthma and failure to thrive as indications for a sweat test.
Topics: Adolescent; Asthma; Child; Child, Preschool; Cystic Fibrosis; Failure to Thrive; Female; Humans; Inf | 2004 |
Diversity of the basic defect of homozygous CFTR mutation genotypes in humans.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regu | 2008 |
The changing face of the exocrine pancreas in cystic fibrosis: the correlation between pancreatic status, pancreatitis and cystic fibrosis genotype.
Topics: Acute Disease; Adolescent; Adult; Child; Child, Preschool; Chlorides; Cohort Studies; Cystic Fibrosi | 2008 |
Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis.
Topics: Acute Disease; Adolescent; Adult; Child; Chloride Channels; Chlorides; Cystic Fibrosis; Cystic Fibro | 2008 |
Total intra-individual variation in sweat sodium and chloride concentrations for the diagnosis of cystic fibrosis.
Topics: Adult; Chlorides; Cystic Fibrosis; Female; Humans; Male; Middle Aged; Reproducibility of Results; So | 2008 |
Audit of sweat testing: a first report from Italian Cystic Fibrosis Centres.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regu | 2008 |
Sweat test in patients with glucose-6-phosphate-1-dehydrogenase deficiency.
Topics: Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Electric Conduc | 2008 |
Cystic fibrosis of the pancreas.
Topics: Acetylcysteine; Anti-Bacterial Agents; Bronchi; Bronchial Diseases; Child; Cystic Fibrosis; Digestiv | 1966 |
[Apropos of mucoviscidosis in the adult].
Topics: Adult; Cystic Fibrosis; Electrolytes; Humans; Sweat | 1966 |
The effects of exogenous aldosterone on sweat electrolytes. II. Patients with cystic fibrosis of the pancreas.
Topics: Adolescent; Adult; Aldosterone; Body Weight; Child; Child, Preschool; Cystic Fibrosis; Female; Human | 1967 |
Iontophoretic sweat test for cystic fibrosis: technical details.
Topics: Cystic Fibrosis; Iontophoresis; Sweat | 1967 |
Nasal mucosa in pancreatic cystic fibrosis.
Topics: Chlorides; Cilia; Culture Techniques; Cystic Fibrosis; Epithelium; Humans; Nasal Mucosa; Nasal Polyp | 1967 |
[Sodium level in the sweat of patients with gastric and duodenal ulcer, and the value of sweat electrolyte determination in detecting of mucoviscidosis in adults].
Topics: Adolescent; Adult; Aged; Cystic Fibrosis; Female; Humans; Iontophoresis; Male; Middle Aged; Peptic U | 1967 |
Some genetic considerations in cystic fibrosis. A study of nail and sweat sodium in two sibships.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Europe; Humans; Infant; Nails; Potassiu | 1967 |
Clinical findings and research in cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Exocrine Glands; Lung Diseases; Mucus; Pancreas; Saliva; Sodium; Sweat | 1967 |
Pilocarpine iontophoresis sweat testing results of seven years' experience.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn | 1967 |
Systematic research of the taint of mucoviscidosis in chronic bronchitis in adults.
Topics: Adult; Aged; Bronchitis; Chlorides; Chronic Disease; Cystic Fibrosis; Female; Humans; Male; Middle A | 1967 |
Crystal forms in sweat from patients with cystic fibrosis.
Topics: Crystallography; Cystic Fibrosis; Humans; Sweat | 1967 |
The ionic composition of pilocarpine induced sweat in relation to gland output during aging and in cystic fibrosis.
Topics: Adolescent; Adult; Aging; Calcium; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Huma | 1967 |
Excretion of different substances in the sweat of children with cystic fibrosis and controls.
Topics: Aldosterone; Calcium; Child; Chlorides; Creatinine; Cystic Fibrosis; Humans; Lactates; Male; Sodium; | 1967 |
Sodium transport: inhibitory factor in sweat of patients with cystic fibrosis.
Topics: Absorption; Animals; Antimetabolites; Biological Transport, Active; Cystic Fibrosis; Humans; Parotid | 1967 |
Sweat chloride assay for cystic fibrosis. Using pilocarpine iontophoresis stimulation, filter paper collection and cotlove chloridometer analysis.
Topics: Chlorides; Cystic Fibrosis; Humans; Pilocarpine; Sweat | 1967 |
[Estimation of sweat bromide content following oral administration of bromide preparations].
Topics: Bromides; Child, Preschool; Cystic Fibrosis; Humans; Methods; Sweat | 1967 |
[Excretion of chlorides and bromides in children's sweat after oral administration of sodium bromide].
Topics: Bromides; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Respiratory Tract Inf | 1967 |
[Cases of mucoviscidosis in the Regional Allergologic Outpatient Hospital for Children in Zabrze].
Topics: Asthma; Child; Cystic Fibrosis; Humans; Outpatient Clinics, Hospital; Poland; Sweat | 1967 |
[Sweat composition changes in mucoviscidosis following pilocarpine and adrenalin iontophoresis].
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Epinephrine; Female; Humans; Iontophoresis; Ma | 1967 |
Obstructive azoospermia as a diagnostic criterion for the cystic fibrosis syndrome.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Lung Diseases | 1982 |
Diagnosis of cystic fibrosis.
Topics: Age Factors; Child, Preschool; Cystic Fibrosis; Diagnostic Errors; Humans; Infant; Infant, Newborn; | 1982 |
Overdiagnosis of cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Diagnostic Errors; Diagnostic Tests, Routine; | 1982 |
Sweat tests for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Humans; Sodium; Sweat | 1983 |
Sweat tests for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Diagnostic Errors; Humans; Sodium; Sweat | 1983 |
Cystic fibrosis: a case report.
Topics: Anti-Bacterial Agents; Biopsy; Chronic Disease; Cystic Fibrosis; Electrolytes; Fatal Outcome; Humans | 1981 |
Cystic fibrosis in Jordan: a pilot study.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Intestinal Ob | 1984 |
Diagnostic testing in cystic fibrosis.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Humans; Iontophoresis; Pilocarpine; Sweat | 1983 |
Decreased Cl permeability as the basis for increased bioelectrical potentials in Cystic Fibrosis.
Topics: Adolescent; Adult; Cell Membrane Permeability; Chlorides; Cystic Fibrosis; Humans; Ion Channels; Mem | 1983 |
Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro.
Topics: Atropine; Child; Cystic Fibrosis; Humans; Isoproterenol; Methacholine Chloride; Methacholine Compoun | 1984 |
Influence of abnormal Cl- impermeability on sweating in cystic fibrosis.
Topics: Adolescent; Adult; Cell Membrane Permeability; Chlorides; Cystic Fibrosis; Female; Humans; Hydrogen- | 1984 |
Exercise and heat stress in cystic fibrosis patients.
Topics: Adolescent; Adult; Aldosterone; Child; Cystic Fibrosis; Female; Hot Temperature; Humans; Male; Physi | 1983 |
Cystic fibrosis mimicking Bartter's syndrome.
Topics: Aldosterone; Bartter Syndrome; Cystic Fibrosis; Diagnosis, Differential; Electrolytes; Female; Human | 1983 |
Heat acclimation in cystic fibrosis.
Topics: Acclimatization; Adult; Aldosterone; Body Temperature; Body Weight; Chlorides; Cystic Fibrosis; Fema | 1984 |
Diagnosis of maternal cystic fibrosis during pregnancy.
Topics: Adolescent; Adult; Ampicillin; Chlorides; Cystic Fibrosis; Female; Humans; Maternal Mortality; Obste | 1983 |
Sweat tests to diagnose cystic fibrosis in adults.
Topics: Adolescent; Adult; Cystic Fibrosis; Female; Fludrocortisone; Humans; Male; Reference Values; Sodium; | 1983 |
Sweat tests to diagnose cystic fibrosis in adults.
Topics: Adult; Age Factors; Cystic Fibrosis; Humans; Sodium; Sweat | 1983 |
Sweat tests to diagnose cystic fibrosis in adults.
Topics: Adolescent; Adult; Cystic Fibrosis; Electrolytes; Humans; Sweat | 1983 |
Prospective study of fatty acid supplementation over 3 years in patients with cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Fat Emulsions, Intravenous; Female; Humans; In | 1983 |
Electrophoretic patterns of proteins in cystic fibrosis sweat.
Topics: Child; Cystic Fibrosis; Electrophoresis, Polyacrylamide Gel; Female; Heterozygote; Humans; Male; Pro | 1984 |
Evaluation of a paper-patch test for sweat chloride determination.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Evaluation Studies as Topic; | 1984 |
Improved sweat test method for the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Middle | 1984 |
The role of the laboratory in the diagnosis of cystic fibrosis.
Topics: Adolescent; Blood Proteins; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newbor | 1984 |
Improved prognosis in CF patients with normal fat absorption.
Topics: Adolescent; Adult; Age Factors; Body Weight; Celiac Disease; Child; Child, Preschool; Cystic Fibrosi | 1984 |
Apparent absence of cystic fibrosis sweat factor on ion-selective and transport properties of the perfused human sweat duct.
Topics: Adult; Biological Transport, Active; Calcium; Chlorides; Cystic Fibrosis; Electrolytes; Humans; Perf | 1984 |
Diagnosis and treatment of cystic fibrosis in adults.
Topics: Adrenal Cortex Hormones; Adult; Age Factors; Anti-Bacterial Agents; Cholelithiasis; Cystic Fibrosis; | 1984 |
The use of sweat osmolality in the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Osmolar Concentration; | 1984 |
A puzzling triad: anorexia nervosa, high sweat electrolytes and indication to partial exocrine pancreatic insufficiency.
Topics: 4-Aminobenzoic Acid; Adolescent; Anorexia Nervosa; Cystic Fibrosis; Diagnosis, Differential; Electro | 1983 |
Pancreatitis as initial presentation of cystic fibrosis in young adults. A report of two cases.
Topics: Adult; Cholangiopancreatography, Endoscopic Retrograde; Cystic Fibrosis; Diagnosis, Differential; Hu | 1983 |
Measurement of osmolality and sodium concentration in heated-cup sweat collections for the investigation of cystic fibrosis.
Topics: Adult; Child; Cystic Fibrosis; Female; Hot Temperature; Humans; Iontophoresis; Male; Osmolar Concent | 1983 |
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 6-1984. A 20-year-old woman with cough, hemoptysis, and a pulmonary cavitation.
Topics: Adult; Bronchi; Bronchitis; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Female; Hemoptysis; | 1984 |
Early diagnosis of cystic fibrosis by means of sweat microosmometry.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Evaluation Studies as Topic; Humans; In | 1984 |
Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis.
Topics: Absorption; Adolescent; Adult; Chlorides; Cystic Fibrosis; Electrophysiology; Humans; Permeability; | 1983 |
Hypogammaglobulinemia and elevated sweat chloride values.
Topics: Adolescent; Agammaglobulinemia; Cystic Fibrosis; Humans; Male; Sodium; Sweat | 1983 |
[Laboratory diagnosis possibilities for the early detection and confirmation of cystic fibrosis (mucoviscidosis). Overview and personal studies].
Topics: Amniocentesis; Child; Child, Preschool; Cystic Fibrosis; Female; Germany, West; Humans; Infant; Infa | 1983 |
Sweat chloride concentration in adults with pulmonary diseases.
Topics: Adolescent; Adult; Aged; Aging; Chlorides; Cystic Fibrosis; Female; Humans; Lung Diseases; Male; Mid | 1983 |
Prognostic value of sweat-chloride levels in cystic fibrosis: a negative report.
Topics: Chlorides; Cystic Fibrosis; Humans; Infant, Newborn; Prognosis; Sweat | 1983 |
Treatment and prognosis of rectal prolapse in cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Male; Prognosis | 1982 |
Hypogammaglobulinemia and antibody deficiency in patients with elevated sweat chloride concentrations.
Topics: Adolescent; Adult; Agammaglobulinemia; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Female; | 1982 |
[Simple method of sweat chloride determination (author's transl)].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Methods; Sweat | 1982 |
Cystic fibrosis mistaken for gastroesophageal reflux.
Topics: Cystic Fibrosis; Diagnosis, Differential; Gastroesophageal Reflux; Humans; Infant; Sweat | 1982 |
Case study: cystic fibrosis.
Topics: Child; Cystic Fibrosis; Electrolytes; Female; Humans; Pseudomonas Infections; Respiratory Tract Infe | 1982 |
Sweat sodium and chloride values.
Topics: Chlorides; Cystic Fibrosis; Humans; Sodium; Sweat | 1982 |
Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Dietary Fats; Female; Humans | 1982 |
Commentary: the clinical forms of cystic fibrosis.
Topics: Cystic Fibrosis; Electrolytes; Humans; Sweat | 1982 |
Suggestion of an abnormal anion exchange mechanism in sweat glands of cystic fibrosis patients.
Topics: Adult; Anions; Chlorides; Cystic Fibrosis; Humans; Hydrogen; Potassium; Sodium; Sweat; Sweat Glands | 1982 |
Effect of temperature on response of the sweat chloride ion-selective electrode.
Topics: Body Temperature; Chlorides; Cystic Fibrosis; Electrodes; Humans; Quality Control; Sweat | 1982 |
Cystic fibrosis presenting with chronic electrolyte depletion, metabolic alkalosis and hyperaldosteronism.
Topics: Alkalosis; Chlorides; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Humans; Hyperaldosteronism | 1982 |
The sweat test: sodium and chloride values.
Topics: Child; Chlorides; Cystic Fibrosis; Humans; Potassium; Sodium; Sweat | 1981 |
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 14-1981.
Topics: Adult; Chlorides; Cystic Fibrosis; Humans; Lung Diseases; Male; Oligospermia; Sweat | 1981 |
A 69-year-old man with chronic obstructive pulmonary disease, pancreatic insufficiency and elevated sweat electrolytes.
Topics: Age Factors; Aged; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Exocrine Pancreatic Insuffic | 1981 |
[Rational diagnosis of mucoviscidosis by a modified method of sweat chloride determination].
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Methods; Sweat | 1981 |
[Sweat electrolyte analysis by pilocarpine iontophoresis].
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn | 1981 |
False-positive sweat tests.
Topics: Chlorides; Cystic Fibrosis; False Positive Reactions; Humans; Sodium; Sweat | 1981 |
An unusual case of XXY Klinefelter's syndrome associated with cystic fibrosis.
Topics: Cystic Fibrosis; Follow-Up Studies; Humans; Infant; Klinefelter Syndrome; Male; Sweat | 1981 |
[Neonatal screening program for cystic fibrosis of the pancreas].
Topics: Albumins; Chlorides; Cystic Fibrosis; Humans; Infant, Newborn; Italy; Mass Screening; Meconium; Sodi | 1981 |
Incidence of meconium abnormalities in newborn infants with cystic fibrosis.
Topics: Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Diseases; Intestinal | 1980 |
[Organoleptic method in the diagnosis of mycoviscidosis in young infants].
Topics: Cystic Fibrosis; Female; Forehead; Humans; Infant; Sweat; Taste | 1980 |
Cystic fibrosis in South India.
Topics: Cystic Fibrosis; Female; Humans; India; Infant; Male; Pedigree; Sodium; Sweat | 1980 |
[The dilemma of borderline sweat tests].
Topics: Cystic Fibrosis; Humans; Sweat | 1980 |
The diagnosis of cystic fibrosis.
Topics: Child; Cystic Fibrosis; Diagnostic Errors; Humans; Infant; Infant, Newborn; Pancreas; Radiography; S | 1980 |
[Sweat test with the use of Tc-99m in the diagnosis of mucoviscidosis. Preliminary report].
Topics: Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Isotope Labeling; Radioactivity; Sweat; Te | 1980 |
Low sweat electrolytes in a patient with cystic fibrosis.
Topics: Adult; Chlorides; Cystic Fibrosis; Electrolytes; Exocrine Pancreatic Insufficiency; Female; Humans; | 1980 |
Undiagnosed patients with cystic fibrosis.
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Ne | 1980 |
[Normal sweat electrolyte concentrations in patients with mucoviscidosis in infancy].
Topics: Age Factors; Chlorides; Cystic Fibrosis; Humans; Infant; Sweat | 1980 |
Cooperative study comparing three methods of performing sweat tests to diagnose cystic fibrosis.
Topics: Child; Cystic Fibrosis; Electrodes; False Negative Reactions; False Positive Reactions; Humans; Iont | 1980 |
Reappraisal of the chloride plate test as screening test for cystic fibrosis.
Topics: Agar; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Methods; Sweat | 1981 |
Screening for cystic fibrosis by a stool trypsin method.
Topics: Cystic Fibrosis; Feces; Humans; Infant, Newborn; Mass Screening; Sweat; Trypsin | 1981 |
New approach to cystic fibrosis diagnosis by use of an improved sweat-induction/collection system and osmometry.
Topics: Chlorides; Cystic Fibrosis; Humans; Iontophoresis; Osmolar Concentration; Pilocarpine; Specimen Hand | 1981 |
Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations.
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regul | 1995 |
A cystic fibrosis mutation associated with mild lung disease.
Topics: Chlorides; Cystic Fibrosis; Humans; Mutation; Sodium; Sweat | 1995 |
Nasal epithelial ion transport and genetic analysis of infertile men with congenital bilateral absence of the vas deferens.
Topics: Biological Transport; Chloride Channels; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 1993 |
Screening for CF mutations in adult cystic fibrosis patients with a directed and optimized SSCP strategy.
Topics: Adolescent; Adult; Base Sequence; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conducta | 1994 |
A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations.
Topics: Adolescent; Adult; Base Sequence; Child; Child, Preschool; Chloride Channels; Chlorides; Chromosomes | 1994 |
Normal sweat chloride values do not exclude the diagnosis of cystic fibrosis.
Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Hete | 1995 |
Sweat sodium and chloride analysis using BM/Hitachi 911 ion-selective electrodes.
Topics: Chlorides; Cystic Fibrosis; Humans; Ion-Selective Electrodes; Sodium; Sweat | 1995 |
Delayed diagnosis of cystic fibrosis due to normal sweat electrolytes.
Topics: Chlorides; Cystic Fibrosis; Electrolytes; False Negative Reactions; Follow-Up Studies; Humans; Infan | 1995 |
Normal sweat chloride values do not exclude the diagnosis of cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Humans; Mutation; Sweat | 1995 |
Application of DNA analysis in a population-screening program for neonatal diagnosis of cystic fibrosis (CF): comparison of screening protocols.
Topics: Analysis of Variance; Australia; Cohort Studies; Colorado; Cystic Fibrosis; Cystic Fibrosis Transmem | 1993 |
Congenital bilateral absence of vas deferens in absence of cystic fibrosis.
Topics: Abnormalities, Multiple; Adult; Chlorides; Cystic Fibrosis; Humans; Infertility, Male; Kidney; Male; | 1995 |
Diagnostic criteria for cystic fibrosis in men with congenital absence of the vas deferens.
Topics: Adult; Chlorides; Cystic Fibrosis; Genetic Carrier Screening; Genotype; Humans; Infertility, Male; M | 1995 |
Increased Na+/Mg2+ antiport in erythrocytes of patients with cystic fibrosis.
Topics: Adolescent; Adult; Antiporters; Child; Cystic Fibrosis; Erythrocytes; Female; Genotype; Humans; Magn | 1994 |
Cystic fibrosis screening in neonates--measurement of immunoreactive trypsin and direct genotype analysis for delta F508 mutation.
Topics: Cystic Fibrosis; DNA Mutational Analysis; Genetic Testing; Genotype; Humans; Infant, Newborn; Neonat | 1994 |
Pseudohypoaldosteronism with increased sweat and saliva electrolyte values and frequent lower respiratory tract infections mimicking cystic fibrosis.
Topics: Aldosterone; Child; Cystic Fibrosis; Diagnosis, Differential; Diseases in Twins; Female; Humans; Inf | 1994 |
Current status of sweat testing in North America. Results of the College of American Pathologists needs assessment survey.
Topics: Chemistry, Clinical; Chlorides; Cystic Fibrosis; Data Collection; Humans; North America; Sodium; Swe | 1994 |
Mild cystic fibrosis and normal or borderline sweat test in patients with the 3849 + 10 kb C-->T mutation.
Topics: Age Factors; Child; Chlorides; Cystic Fibrosis; Female; Humans; Male; Phenotype; Point Mutation; Swe | 1993 |
Sweat chloride and delta F508 mutation in chronic bronchitis or bronchiectasis.
Topics: Adult; Aged; Bronchiectasis; Bronchitis; Chlorides; Chronic Disease; Cystic Fibrosis; Humans; Male; | 1993 |
Screening for cystic fibrosis in dried blood spots of newborns.
Topics: Algorithms; Chlorides; Cystic Fibrosis; DNA Mutational Analysis; Electrophoresis, Polyacrylamide Gel | 1993 |
Sweat osmolality in some of the congenital diseases.
Topics: Adolescent; Adult; Age Factors; Child; Cystic Fibrosis; Down Syndrome; Female; Humans; Intellectual | 1993 |
Clinical evaluation of the macroduct sweat collection system and conductivity analyzer in the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Aged; Case-Control Studies; Child; Child, Preschool; Chlorides; Cystic Fibrosis; | 1994 |
Improved correlation of sweat chloride quantification by the CF Indicator System and the Gibson-Cooke Sweat Test.
Topics: Chlorides; Cystic Fibrosis; Humans; Sweat | 1993 |
Clinical quiz. Sweat test indicates cystic fibrosis in a child with sweat sodium of 99 mmol/l and sweat chloride of 139 mmol/l.
Topics: Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Infant; Sodium; Sweat | 1993 |
Clinical spectrum in homozygotes and compound heterozygotes inheriting cystic fibrosis mutation 3849 + 10kbC > T: significance for geneticists.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Forced Expiratory Volume; Ge | 1995 |
Sweat chloride concentrations in infants homozygous or heterozygous for F508 cystic fibrosis.
Topics: Age Factors; Alleles; Base Composition; Chlorides; Codon; Cystic Fibrosis; DNA; Follow-Up Studies; G | 1996 |
The significance of sweat Cl/Na ratio in patients with borderline sweat test.
Topics: Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Muta | 1995 |
[The usefulness of the conduction sweat test in diagnosis of cystic fibrosis].
Topics: Adolescent; Adult; Biomarkers; Child; Child, Preschool; Cystic Fibrosis; Electric Conductivity; Fema | 1995 |
[A study evaluating the correlation between the phenotype and genotype among 65 cystic fibrosis patients].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; | 1995 |
Pulmonary function and clinical observations in men with congenital bilateral absence of the vas deferens.
Topics: Adult; Body Height; Body Weight; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulato | 1996 |
Pancreatitis and an abnormal sweat test at presentation in a child with non-Hodgkin's lymphoma.
Topics: Child; Chlorides; Cystic Fibrosis; Humans; Lymphoma, Non-Hodgkin; Male; Pancreatitis; Sweat | 1996 |
[Increase of chlorine in sweat in the absence of cystic fibrosis].
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Male; Sweat | 1996 |
Analysis of sweat chloride by reversed phase high-performance liquid chromatography.
Topics: Chlorides; Chromatography, High Pressure Liquid; Cystic Fibrosis; Evaluation Studies as Topic; Human | 1995 |
A baby with eczema and an abnormal sweat test.
Topics: Cystic Fibrosis; Dermatitis, Atopic; Diagnostic Errors; False Positive Reactions; Humans; Infant; Ma | 1996 |
Measurement of sweat sodium using a modified enzymatic assay.
Topics: Child; Clinical Enzyme Tests; Cystic Fibrosis; Humans; Nitrophenols; Reagent Kits, Diagnostic; Sodiu | 1996 |
Enzymatic determination of sodium and chloride in sweat.
Topics: alpha-Amylases; beta-Galactosidase; Bridged Bicyclo Compounds, Heterocyclic; Chelating Agents; Chlor | 1996 |
Eczema and an abnormal sweat test.
Topics: Cystic Fibrosis; Eczema; False Positive Reactions; Humans; Infant; Sweat | 1996 |
Sweat testing for the diagnosis of cystic fibrosis: practical considerations.
Topics: Arm; Chlorides; Cystic Fibrosis; Electric Conductivity; False Negative Reactions; False Positive Rea | 1996 |
Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent.
Topics: Acidosis; Adolescent; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype | 1997 |
[Male infertility caused by bilateral agenesis of the vas deferens: a new clinical form of cystic fibrosis?].
Topics: Adult; Chlorine; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Het | 1997 |
Identification of a splice site mutation (2789 +5 G > A) associated with small amounts of normal CFTR mRNA and mild cystic fibrosis.
Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutation | 1997 |
Nasal potential difference in cystic fibrosis patients presenting borderline sweat test.
Topics: Adolescent; Adult; Child; Cystic Fibrosis; DNA; Female; Humans; Male; Membrane Potentials; Nasal Muc | 1997 |
[A method of sampling material for the determination of chlorides in sweat].
Topics: Chlorides; Cystic Fibrosis; Electrophoresis, Paper; Forearm; Humans; Pilocarpine; Specimen Handling; | 1997 |
A missense cystic fibrosis transmembrane conductance regulator mutation with variable phenotype.
Topics: Age of Onset; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator | 1997 |
Urticaria associated with the pilocarpine iontophoresis sweat test.
Topics: Child; Chlorides; Cystic Fibrosis; Female; Histamine H1 Antagonists; Humans; Iontophoresis; Pilocarp | 1997 |
Genotype-phenotype correlation in cystic fibrosis patients compound heterozygous for the A455E mutation.
Topics: Adolescent; Adult; Canada; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 1997 |
Sweat sodium is not sweat chloride.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Humans; Sodium; Sweat | 1997 |
[Cystic fibrosis in adults--clinical aspects].
Topics: Adolescent; Adult; Bronchiectasis; Chlorides; Cystic Fibrosis; DNA; Female; Humans; Male; Mutation; | 1997 |
A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis.
Topics: Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Huma | 1998 |
P67L: a cystic fibrosis allele with mild effects found at high frequency in the Scottish population.
Topics: Adolescent; Adult; Alleles; Child; Cystic Fibrosis; DNA; Exocrine Pancreatic Insufficiency; Female; | 1998 |
Sweat electrolyte and cystic fibrosis mutation analysis allows early diagnosis in Brazilian children with clinical signs compatible with cystic fibrosis.
Topics: Age Factors; Brazil; Child; Child, Preschool; Chlorides; Cystic Fibrosis; DNA Mutational Analysis; F | 1998 |
Correlation of sweat chloride concentration with genotypes in cystic fibrosis patients in Saguenay Lac-Saint-Jean, Quebec, Canada.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Testing; Ge | 1998 |
Making the diagnosis of cystic fibrosis.
Topics: Consensus Development Conferences as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductan | 1998 |
Uncertainty in the diagnosis of cystic fibrosis: possible role of in vivo nasal potential difference measurements.
Topics: Adolescent; Adult; Case-Control Studies; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 1998 |
Test for the concentration of electrolytes in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis, by Lewis E. Gibson and Robert E. Cooke, Pediatrics; 1959;24:545-549.
Topics: Cystic Fibrosis; Electrolytes; History, 20th Century; Humans; Iontophoresis; Pilocarpine; Sweat | 1998 |
Mutations of the cystic fibrosis gene in patients with chronic pancreatitis.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Chlorides; Chronic Disease; Cystic Fibrosis; Cystic Fibr | 1998 |
Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
Topics: Adolescent; Adult; Aged; Child; Chlorides; Chronic Disease; Cystic Fibrosis; Cystic Fibrosis Transme | 1998 |
Nasal potential difference in congenital bilateral absence of the vas deferens.
Topics: Adrenergic beta-Agonists; Adult; Amiloride; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembran | 1998 |
Cystic fibrosis, Young's syndrome, and normal sweat chloride.
Topics: Adult; Asthma; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Male; Mutation; Oligospe | 1998 |
Cystic fibrosis mutations in heterozygous newborns with hypertrypsinemia and low sweat chloride.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Heterozygote; Human | 1999 |
Mutations of the cystic fibrosis gene and pancreatitis.
Topics: Chlorides; Chronic Disease; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Hu | 1999 |
Pitfall in the use of genotype analysis as the sole diagnostic criterion for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Diagnostic Errors; Female; Genetic Testing; Genotype; Humans; Infant; Mu | 1999 |
Sunshine, sweating, and main d'accoucheur.
Topics: Adult; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrolytes; Hand; Hum | 1999 |
Cystic fibrosis: the twilight zone.
Topics: Adolescent; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Hum | 1999 |
Increased sweat chloride concentrations in adult patients with pancreatic insufficiency: cystic fibrosis phenotype or lack of specificity of sweat test?
Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Pan | 1999 |
Evidence of mild respiratory disease in men with congenital absence of the vas deferens.
Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Hu | 1999 |
Triple A syndrome mimicking cystic fibrosis.
Topics: Adrenal Glands; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Esophageal Ac | 2000 |
Cystic fibrosis diagnosed in adult patients.
Topics: Adolescent; Adult; Aged; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Follo | 2000 |
Inconsistencies in sweat testing in UK laboratories.
Topics: Age Factors; Clinical Laboratory Techniques; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Labor | 2000 |
Indirect measurements of sweat electrolyte concentration in the laboratory diagnosis of cystic fibrosis.
Topics: Adolescent; Age Factors; Case-Control Studies; Child; Child, Preschool; Chlorides; Cystic Fibrosis; | 2000 |
Micro-flowcell conductometric sweat analysis for cystic fibrosis diagnosis.
Topics: Adult; Automation; Calibration; Conductometry; Cystic Fibrosis; Humans; Iontophoresis; Sweat; Temper | 2000 |
Sweat testing following newborn screening for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Diagnosis, Differential; DNA Mutational Analysis; Female; Genetic Testin | 2000 |
Protracted neonatal hypertrypsinogenaemia, normal sweat chloride, and cystic fibrosis.
Topics: Adolescent; Chlorides; Cystic Fibrosis; Humans; Male; Neonatal Screening; Sputum; Sweat; Trypsinogen | 2000 |
[A normal sweat test does not exclude the diagnosis of cystic fibrosis].
Topics: Cystic Fibrosis; False Negative Reactions; Humans; Predictive Value of Tests; Sweat | 2000 |
Newborn screening for cystic fibrosis in Victoria: 10 years' experience (1989-1998).
Topics: Chlorides; Cystic Fibrosis; Humans; Neonatal Screening; Retrospective Studies; Sweat; Trypsinogen; V | 2000 |
Time to think again: cystic fibrosis is not an "all or none" disease.
Topics: Cystic Fibrosis; Diagnosis, Differential; DNA Mutational Analysis; Female; Humans; Infant; Male; Phe | 2000 |
Clinical features associated with a delayed diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Burkholderia cepacia; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2000 |
Reliability of sweat-testing by the Macroduct collection method combined with conductivity analysis in comparison with the classic Gibson and Cooke technique.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Electrolytes; Humans; Infant; Infant, N | 2000 |
Serum lipase levels as a diagnostic marker in cystic fibrosis patients with normal or borderline sweat tests.
Topics: Adult; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocr | 2000 |
Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population.
Topics: Adult; Aged; Case-Control Studies; Chronic Disease; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 2000 |
Lung disease associated with the IVS8 5T allele of the CFTR gene.
Topics: Age of Onset; Alleles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithel | 2000 |
Recommendations for quality improvement in genetic testing for cystic fibrosis. European Concerted Action on Cystic Fibrosis.
Topics: Accreditation; Adolescent; Adult; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance | 2000 |
Sweat analysis proficiency testing for cystic fibrosis.
Topics: Chlorides; Clinical Laboratory Techniques; Cystic Fibrosis; Health Care Surveys; Humans; Infant; Inf | 2000 |
A simplified cyclic adenosine monophosphate-mediated sweat rate test for quantitative measure of cystic fibrosis transmembrane regulator (CFTR) function.
Topics: Adolescent; Adrenergic beta-Agonists; Adult; Aminophylline; Atropine; Child; Cyclic AMP; Cystic Fibr | 2000 |
Genotype analysis and phenotypic manifestations of children with intermediate sweat chloride test results.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 2000 |
Point-of-need diagnosis of cystic fibrosis using a potentiometric ion-selective electrode array.
Topics: Chlorine; Cluster Analysis; Cystic Fibrosis; Humans; Point-of-Care Systems; Potassium; Potentiometry | 2000 |
Cystic fibrosis and Down's syndrome: not always a poor prognosis.
Topics: Bronchiolitis; Child; Cystic Fibrosis; Diagnosis, Differential; Down Syndrome; Female; Humans; Mosai | 2001 |
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in neonatal hypertrypsinaemia with normal sweat test.
Topics: Alleles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; DNA Mutational A | 2001 |
Genetic counseling and neonatal screening for cystic fibrosis: an assessment of the communication process.
Topics: Adult; Attitude to Health; Child, Preschool; Chlorides; Communication; Cystic Fibrosis; Electrolytes | 2001 |
Sweat Chloride and conductivity 1.
Topics: Chlorides; Cystic Fibrosis; Electric Conductivity; Humans; Sensitivity and Specificity; Sweat | 2001 |
Sweat chloride and conductivity 2.
Topics: Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Electric Conductivity; Humans; Sensitivity and | 2001 |
Cystic fibrosis: the 'bicarbonate before chloride' hypothesis.
Topics: Bicarbonates; Biological Transport; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conduc | 2001 |
Sweat chloride: quantitative patch for collection and measurement.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Iontop | 2001 |
[Cystic fibrosis and normal sweat chloride values: a case-report].
Topics: Adolescent; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; | 2001 |
Assessment of sweat-testing practices for the diagnosis of cystic fibrosis.
Topics: Chemistry, Clinical; Chlorides; Cystic Fibrosis; Humans; Laboratories; Quality Control; Surveys and | 2001 |
Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations.
Topics: Adolescent; Adult; Alleles; alpha-Amylases; Child; Chlorides; Cystic Fibrosis; Epithelium; Female; G | 2001 |
Cystic fibrosis diagnosis: new dilemmas for an old disorder.
Topics: Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diagnosis, Di | 2002 |
Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis.
Topics: Adolescent; Adult; Analysis of Variance; Chlorides; Clinical Trials, Phase I as Topic; Cystic Fibros | 2002 |
Cystic fibrosis: keeping it in the family.
Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Fert | 2002 |
Biological variation in sweat sodium chloride conductivity.
Topics: Adolescent; Adult; Case-Control Studies; Child; Child, Preschool; Cystic Fibrosis; Electric Conducti | 2002 |
Mutations of the cystic fibrosis gene and intermediate sweat chloride levels in children.
Topics: Adolescent; Belgium; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembr | 2002 |
Mercurimetric determination of chloride in sweat.
Topics: Chlorides; Cystic Fibrosis; Humans; Linear Models; Mercury; Sensitivity and Specificity; Sweat; Titr | 2002 |
Negative sweat test in hypertrypsinaemic infants with cystic fibrosis carrying rare CFTR mutations.
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regul | 2002 |
Positive sweat test following meningococcal septicaemia.
Topics: Bacteremia; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Diagnostic Tests, Routine; E | 2002 |
pH of sweat of patients with cystic fibrosis.
Topics: Bicarbonates; Cystic Fibrosis; Humans; Hydrogen-Ion Concentration; Sweat; Sweating | 1976 |
[Pathogenesis and early diagnosis of mucoviscidosis in children (literature survey)].
Topics: Adrenal Cortex Hormones; Albumins; Bronchi; Calcium; Calcium Metabolism Disorders; Child; Child, Pre | 1976 |
Sweat testing for cystic fibrosis: errors associated with the in-situ sweat test using chloride ion selective electrodes.
Topics: Chlorides; Cystic Fibrosis; Electrodes; Humans; Hydrogen-Ion Concentration; Methods; Skin Absorption | 1977 |
Ultramicro-electrophoresis of protein in sweat from patients with cystic fibrosis of the pancreas and controls.
Topics: Adolescent; Binding Sites; Cell Membrane; Child; Child, Preschool; Cystic Fibrosis; Electrophoresis; | 1978 |
Serum alpha - fetoprotein levels in patients with cystic fibrosis and their parents and siblings.
Topics: alpha-Fetoproteins; Bronchiectasis; Celiac Disease; Chlorides; Cystic Fibrosis; Fetal Proteins; Gene | 1975 |
[Sweat test for diagnosis of mucoviscidosis].
Topics: Child; Chlorine; Cystic Fibrosis; Humans; Iontophoresis; Silver Nitrate; Sodium; Sweat | 1975 |
Letter: Screening for cystic fibrosis.
Topics: Albumins; Cystic Fibrosis; False Positive Reactions; Humans; Infant, Newborn; Infant, Newborn, Disea | 1976 |
Cystic fibrosis: normal volumes of red cells. Model experiments on the pathomechanism of the disease.
Topics: Cystic Fibrosis; Erythrocytes; Gramicidin; Humans; Ion Exchange; Permeability; Potassium; Sodium; Sw | 1976 |
Cystic-fibrosis heterozygosity in childhood bronchial asthma.
Topics: Asthma; Bromides; Child; Chlorides; Cystic Fibrosis; Heterozygote; Humans; Sodium; Sweat | 1977 |
[Comparative evaluation of various tests in the diagnosis of mucoviscidosis in children].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Methods; Potassium; | 1977 |
Does that child really have cystic fibrosis?
Topics: Amylases; Bicarbonates; Child; Child, Preschool; Chlorides; Cholecystokinin; Cystic Fibrosis; Evalua | 1978 |
Cystic-fibrosis variants--or variations?
Topics: Bronchitis; Chlorides; Chronic Disease; Cystic Fibrosis; Diagnosis, Differential; Humans; Pancreas; | 1978 |
Sweat tests in cystic fibrosis.
Topics: Child; Chlorides; Cystic Fibrosis; Eczema; False Positive Reactions; Humans; Sodium; Sweat | 1978 |
'Rusters'. The corrosive action of palmar sweat: I. Sodium chloride in sweat.
Topics: Administration, Topical; Adolescent; Adult; Aluminum; Chlorides; Corrosion; Cystic Fibrosis; Dermato | 1979 |
Prostaglandins and cystic fibrosis.
Topics: Adult; Child; Cyclic AMP; Cystic Fibrosis; Humans; Platelet Aggregation; Prostaglandins E; Sweat | 1979 |
[A new type of sweat test and its use in children].
Topics: Adolescent; Age Factors; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Humans; In | 1979 |
Improvement on the safety of sweat-test equipment.
Topics: Accident Prevention; Burns, Electric; Cystic Fibrosis; Humans; Iontophoresis; Safety; Sweat | 1978 |
Diagnosing cystic fibrosis in adults.
Topics: Adult; Age Factors; Cystic Fibrosis; Electrolytes; Humans; Sweat | 1979 |
Sweat tests.
Topics: Cystic Fibrosis; Humans; Sweat | 1979 |
[Chlorine determination method in the screening of mucoviscidosis].
Topics: Adolescent; Child; Child, Preschool; Chlorine; Cystic Fibrosis; Humans; Infant; Skin; Sweat | 1979 |
Reliability of sweat test in cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; False Positive Reactions; Humans; Sodium; Sweat | 1979 |
The sweat test for cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Sweat | 1979 |
Metabolic alkalosis in infants with cystic fibrosis.
Topics: Alkalosis; Carbon Dioxide; Chlorides; Cystic Fibrosis; Electrolytes; Female; Humans; Infant; Male; S | 1979 |
[Improvement of the sweat test in diagnosis of mucoviscidosis].
Topics: Child; Cystic Fibrosis; Humans; Mass Screening; Pilocarpine; Secretory Rate; Sweat | 1979 |
[Problems in the early diagnosis of mucoviscidosis (cystic fibrosis].
Topics: Adolescent; Albumins; Bronchitis; Child; Child, Preschool; Cystic Fibrosis; Female; Genes, Recessive | 1979 |
Limitations of diagnostic value of the sweat test.
Topics: Adolescent; Asthma; Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Humans; Sodiu | 1977 |
Bronchiectasis and mucoviscidosis.
Topics: Adolescent; Adult; Aged; Bronchiectasis; Chlorides; Cystic Fibrosis; Humans; Male; Middle Aged; Swea | 1977 |
Meconium screening for cystic fibrosis.
Topics: Albumins; Cystic Fibrosis; False Negative Reactions; Humans; Infant, Newborn; Mass Screening; Meconi | 1978 |
[A simple test for the screening of mucoviscidosis (cystic fibrosis)].
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Hungary; Male; Mass Screening; | 1978 |
Edema as the presenting symptom of cystic fibrosis: difficulties in diagnosis.
Topics: Chlorides; Cystic Fibrosis; Edema; Female; Humans; Hypoproteinemia; Infant; Male; Sweat | 1978 |
[Simultaneous occurrence of mucoviscidosis and bronchial asthma and its significance from the viewpoint of therapy and care].
Topics: Asthma; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Male; Sweat | 1978 |
Limitations of the sweat test.
Topics: Child; Chlorides; Cystic Fibrosis; Humans; Pancreas; Sodium; Sweat | 1978 |
Intermediate-range sweat chloride concentration and Pseudomonas bronchitis. A cystic fibrosis variant with preservation of exocrine pancreatic function.
Topics: Adolescent; Bronchitis; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential | 1978 |
[Family studies in mucoviscidosis with special emphasis on the elucidation of the heterozygote gene carrier state and heredity].
Topics: Bromides; Chlorides; Cystic Fibrosis; Female; Heterozygote; Humans; Male; Pedigree; Sodium; Sweat | 1978 |
A modified ion-selective electrode method for measurement of chloride in sweat.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Humans; Infant; | 1978 |
The significance of the sweat test in chronic pancreatitis.
Topics: Adolescent; Adult; Age Factors; Aged; Child; Chronic Disease; Cystic Fibrosis; Eating; Female; Human | 1978 |
Sweat testing for cystic fibrosis. Diagnostic screening with a combination chloride ion-selective electrode.
Topics: Age Factors; Chlorides; Cystic Fibrosis; Electrodes; Female; Humans; Infant, Newborn; Infant, Newbor | 1978 |
False negative sweat tests with apparently adequate sweat collections.
Topics: Child; Chlorides; Cystic Fibrosis; False Negative Reactions; Humans; Sodium; Sweat | 1978 |
Cystic fibrosis. Problems encountered with sweat testing.
Topics: Chlorides; Cystic Fibrosis; False Negative Reactions; False Positive Reactions; Humans; Iontophoresi | 1978 |
Measurement of chloride in sweat with the chloride-selective electrode.
Topics: Chlorides; Cystic Fibrosis; Electrodes; Humans; Infant, Newborn; Methods; Pilocarpine; Sweat | 1978 |
Sweat testing for cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; False Negative Reactions; Humans; Infant, Newborn; Mass Screening; Sweat | 1978 |
Quality of sweat test performance in the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Pilocarpine; Sweat; Swe | 1979 |
Atypical cystic fibrosis of the pancreas with normal levels of sweat chloride and minimal pancreatic lesions.
Topics: Child; Chlorides; Cystic Fibrosis; Humans; Infant; Male; Pancreas; Sweat | 1979 |
False positive sweat test, malnutrition, and the Mauriac syndrome.
Topics: Child; Chlorides; Cystic Fibrosis; Diabetes Mellitus, Type 1; Dwarfism; False Positive Reactions; Fe | 1979 |
Cystic fibrosis in adolescents and adults.
Topics: Adolescent; Adult; Aspergillus; Cystic Fibrosis; Female; Gastrointestinal Diseases; Growth; Humans; | 1976 |
Test strip meconium screening for cystic fibrosis.
Topics: Albumins; Cystic Fibrosis; False Negative Reactions; False Positive Reactions; Female; Humans; Indic | 1977 |
[Sweat bromide test in infants with dystrepsia].
Topics: Age Factors; Bromides; Cystic Fibrosis; Heterozygote; Homozygote; Humans; Infant; Sweat | 1977 |
Dietary essential fatty acids in cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Dietary Fats; Fatty Acids; Fatty Acids, Essent | 1977 |
Renin-like (angiotensinogenase) activity in sweat of patients with cystic fibrosis and controls.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Humans; Renin; Sweat; Sweat Glands | 1977 |
Cystic fibrosis: a new outlook. 70 patients above 25 years of age.
Topics: Adult; Anti-Bacterial Agents; Child; Clubfoot; Cystic Fibrosis; Educational Status; Family Character | 1977 |
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 26-1977.
Topics: Age Factors; Cystic Fibrosis; Diagnosis, Differential; Epididymis; Humans; Intestines; Liver; Lung; | 1977 |
[Sweat-electrolyte studies under intravenous load with sodium chloride in patients with mucoviscidosis and chronic bronchitis].
Topics: Adult; Bronchitis; Child; Chronic Disease; Cystic Fibrosis; Humans; Injections, Intravenous; Middle | 1977 |
[The results of bromide tests in patients with cystic fibrosis, in their parents and control subjects].
Topics: Adolescent; Adult; Bromides; Child, Preschool; Cystic Fibrosis; Heterozygote; Humans; Parents; Sweat | 1977 |
Sweat testing for cystic fibrosis: characteristics of a combination chloride ion-selective electrode.
Topics: Chemistry, Clinical; Chlorides; Cystic Fibrosis; Drug Stability; Electrodes; Humans; Methods; Sweat; | 1977 |
Mother and daughter with cystic fibrosis.
Topics: Adult; Chlorides; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Pregn | 1977 |
[Neonatal screening for cystic fibrosis with the BM test on meconium. Preliminary results].
Topics: Cystic Fibrosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Meconium; Sweat | 1977 |
Cystic fibrosis screening by sweat analysis: a critical review of techniques.
Topics: Child; Child, Preschool; Cystic Fibrosis; Electric Conductivity; Humans; Infant; Infant, Newborn; Os | 1977 |
Collecting sweat in patients with cystic fibrosis.
Topics: Child; Cystic Fibrosis; Humans; Specimen Handling; Sweat | 1977 |
[Current use of the sweat test (author's transl)].
Topics: Cystic Fibrosis; Humans; Infant, Newborn; Iontophoresis; Methods; Pilocarpine; Sweat | 1977 |
[Usefulness of the bromide sweat test in early diagnosis of mucoviscidosis].
Topics: Age Factors; Bromides; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Sweat | 1977 |
ALPHA-Amylase activity in sweat and serum of patients with cystic fibrosis of the pancreas.
Topics: Adolescent; Amylases; Child; Child, Preschool; Cystic Fibrosis; Humans; Sweat | 1976 |
[Diagnosis of cystic fibrosis].
Topics: Albumins; Chlorides; Cystic Fibrosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Meconium; | 1976 |
The use of electrolyte measurements in the detection of cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Heterozygote; Humans | 1976 |
[Sweat conductometry in cellulose acetate foil--a simplified method for diagnosis of cystic fibrosis].
Topics: Conductometry; Cystic Fibrosis; Humans; Sweat | 1976 |
Electrolyte values of sweat obtained by local and whole body collection methods in cystic fibrosis patients.
Topics: Adult; Chlorides; Cystic Fibrosis; Humans; Male; Sodium; Sweat | 1976 |
Letter: Intralipid and cystic fibrosis.
Topics: Cystic Fibrosis; Electrolytes; Humans; Infant Food; Infant, Newborn; Sweat | 1976 |
Laboratory confirmation of the diagnosis of cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Clinical Laboratory Techniques; Cystic Fibros | 1976 |
[Concentration values of sodium, potassium and chlorine electrolytes and lactic acid concentrations in the sweat of healthy children, in children with chronic infections of the respiratory tract (perinasal sinusitis, recurrent bronchitis, sinal-and-bronch
Topics: Age Factors; Bronchitis; Child; Chlorine; Chronic Disease; Cystic Fibrosis; Humans; Lactates; Potass | 1975 |
[Determination of lastic acid concentration in the blood, sweat and faeces of children with mucoviscidosis and in healthy children (author's transl)].
Topics: Child; Cystic Fibrosis; Feces; Humans; Lactates; Photometry; Sweat | 1975 |
[BM-test Meconium. A new screening method for cystic fibrosis].
Topics: Albumins; Cystic Fibrosis; Electrolytes; Humans; Infant, Newborn; Mass Screening; Meconium; Sodium; | 1975 |
Insulin iontophoresis in cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Heterozygote; Humans; Insulin; Iontophoresis; Pilocarpine; Sweat | 1975 |
Negative sweat tests and cystic fibrosis.
Topics: Amylases; Bicarbonates; Child; Child, Preschool; Chronic Disease; Cystic Fibrosis; Female; Humans; L | 1975 |
The source of Na+ and Cl- activities on the skin surface. Experiences with electrode measurements in children.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Humans; Infant; | 1975 |
Cystic fibrosis: present status and future prospects in detection of patients and carriers.
Topics: Biological Assay; Blood Proteins; Carrier State; Cystic Fibrosis; Female; Heterozygote; Homozygote; | 1975 |
[Appendicitis and mucoviscidosis].
Topics: Adolescent; Appendicitis; Appendix; Cystic Fibrosis; Humans; Sweat | 1975 |
Unusual clinical course in a child with cystic fibrosis treated with fat emulsion.
Topics: Cystic Fibrosis; Emulsions; Female; Glycine max; Humans; Ileostomy; Infant; Infant, Newborn; Infusio | 1975 |
Polyamines in cystic fibrosis.
Topics: Bile; Cystic Fibrosis; Duodenum; Humans; Intestinal Secretions; Polyamines; Saliva; Spermidine; Sper | 1975 |
[Determination of sweat chloride with an ion-selective electrode].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Humans; Infant; Infant, | 1976 |
A therapeutic trial of fatty acid supplementation in cystic fibrosis.
Topics: Body Weight; Chymotrypsin; Cystic Fibrosis; Fatty Acids; Feces; Humans; Infant; Linoleic Acids; Sodi | 1976 |
[Cystic fibrosis screening using a simplified night sweat test--results of a pilot study].
Topics: Child; Child, Preschool; Cystic Fibrosis; Humans; Mass Screening; Sweat; Time Factors | 1976 |
Cystic fibrosis screening in the newborn.
Topics: Chymotrypsin; Clinical Enzyme Tests; Cystic Fibrosis; Feces; Humans; Infant, Newborn; Sweat; Trypsin | 1976 |
The kallikrein-kinin system of sweat in normal and cystic fibrosis subjects.
Topics: Child; Cystic Fibrosis; Female; Humans; Isoproterenol; Kallikreins; Male; Pilocarpine; Prekallikrein | 1992 |
Cystic fibrosis in a 70 year old woman.
Topics: Aged; Bronchiectasis; Celiac Disease; Cystic Fibrosis; Family; Female; Genotype; Humans; Respiratory | 1992 |
Lithium treatment of a bipolar patient with cystic fibrosis.
Topics: Adult; Bipolar Disorder; Chlorides; Cystic Fibrosis; Female; Humans; Lithium Carbonate; Sweat | 1992 |
Variation of sweat sodium and chloride with age in cystic fibrosis and normal populations: further investigations in equivocal cases.
Topics: Adolescent; Adult; Age Factors; Child; Chlorides; Cystic Fibrosis; DNA; Female; Humans; Male; Osmola | 1992 |
[Determination of nasal transepithelial potential difference (DDPTE) in cystic fibrosis. Analysis of a simplified measurement technique].
Topics: Child; Child, Preschool; Cystic Fibrosis; Epithelium; Humans; Infant; Ion Channels; Membrane Potenti | 1991 |
[Comparison of the classical Gibson-Cooke methods and the chloride-sensitive electrode in sweat testing for diagnosis of cystic fibrosis].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Female; Humans; Infant; | 1991 |
A simple and disposable sweat collector.
Topics: Adolescent; Adult; Child; Cystic Fibrosis; Electrolytes; Exercise Test; Humans; Lactates; Male; Spec | 1991 |
Efficacy of statewide neonatal screening for cystic fibrosis by assay of trypsinogen concentrations.
Topics: Clinical Protocols; Cystic Fibrosis; False Negative Reactions; False Positive Reactions; Humans; Inc | 1991 |
Cystic fibrosis gene mutation in two sisters with mild disease and normal sweat electrolyte levels.
Topics: Base Sequence; Cystic Fibrosis; DNA; Electrolytes; Exons; Female; Humans; Middle Aged; Molecular Seq | 1991 |
Benign missense variations in the cystic fibrosis gene.
Topics: Adult; Alleles; Base Sequence; Chlorides; Chromosome Deletion; Cystic Fibrosis; DNA Mutational Analy | 1990 |
Sweat sodium or chloride?
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Infant, Newborn; So | 1991 |
Colonic transepithelial potential difference in infants with cystic fibrosis.
Topics: Amiloride; Chlorides; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Absorption; Intes | 1991 |
Correlation of phenotypic and genetic heterogeneity in cystic fibrosis: variability in sweat electrolyte levels contributes to heterogeneity and is increased with the XV-2c/KM19 B haplotype.
Topics: Child; Cystic Fibrosis; DNA; Electrolytes; Genetic Markers; Haplotypes; HLA-DR Antigens; Humans; Phe | 1991 |
Sweat chloride concentration in cystic fibrosis patients varies with KM.19 genotype but not with the presence of the common F508 deletion.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Chromosome Deletion; Cystic Fibrosis; Female; | 1991 |
Lung perfusion scintigrams in children with intermittently increased sweat electrolytes.
Topics: Child; Cystic Fibrosis; Electrolytes; Humans; Lung; Radionuclide Imaging; Sweat | 1990 |
The effect of nicardipine, a calcium channel blocker, on the sweat test in adult patients with cystic fibrosis.
Topics: Adult; Cystic Fibrosis; Humans; Nicardipine; Sodium; Sweat | 1990 |
Study of the forms of sweat crystallisation in cystic fibrosis patients.
Topics: Crystallization; Cystic Fibrosis; Diagnostic Errors; Humans; Sweat | 1990 |
Na+, K+, H+, Cl-, and Ca2+ concentrations in cystic fibrosis eccrine sweat in vivo and in vitro.
Topics: Adolescent; Adult; Calcium; Chlorides; Cystic Fibrosis; Hot Temperature; Humans; Hydrogen-Ion Concen | 1990 |
[The form of crystallization of perspiration in pancreatic cystic fibrosis].
Topics: Crystallization; Cystic Fibrosis; Electrolytes; Humans; Iontophoresis; Pilocarpine; Sweat | 1990 |
The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508).
Topics: Adolescent; Adult; Alleles; Child; Child, Preschool; Chlorides; Chromosomes, Human, Pair 7; Cystic F | 1990 |
[Reference values of Na(+) and Cl(-) concentrations in adult sweat].
Topics: Adult; Bronchiectasis; Bronchitis; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Inf | 1990 |
Assay of a urinary CF-lectin factor as a second diagnostic test for cystic fibrosis.
Topics: Adolescent; Adult; Chlorides; Chromatography, Gel; Chromatography, Ion Exchange; Cystic Fibrosis; Fe | 1990 |
DNA analysis of cystic fibrosis genotypes in relatives with equivocal sweat test results.
Topics: Chromosomes, Human, Pair 7; Cystic Fibrosis; DNA; Electrolytes; Family Health; Female; Genetic Marke | 1990 |
The sweat crystallization test in the diagnosis of cystic fibrosis.
Topics: Crystallization; Cystic Fibrosis; Diagnostic Errors; Humans; Sweat | 1990 |
Depressive illness and abnormal sweat electrolytes. A case report.
Topics: Adolescent; Chlorides; Cystic Fibrosis; Depression; Diagnosis, Differential; False Positive Reaction | 1990 |
The sweat test for cystic fibrosis.
Topics: Cystic Fibrosis; False Negative Reactions; False Positive Reactions; Humans; Sweat | 1990 |
Sweat sodium and chloride concentrations--essential criteria for the diagnosis of cystic fibrosis in adults.
Topics: Adolescent; Adult; Chlorides; Cystic Fibrosis; Female; Humans; Male; Predictive Value of Tests; Repr | 1990 |
Quantification of chloride in sweat with the Cystic Fibrosis Indicator System.
Topics: Chlorides; Cystic Fibrosis; Diagnosis, Computer-Assisted; Humans; Patch Tests; Skin Tests; Sweat | 1990 |
Pathophysiology of cystic fibrosis with emphasis on salivary gland involvement.
Topics: Animals; Chlorides; Cystic Fibrosis; Exocrine Glands; Humans; Ion Channels; Mucus; Saliva; Salivary | 1987 |
Prenatal diagnosis of cystic fibrosis by using linked DNA markers in 138 pregnancies at 1-in-4 risk.
Topics: Cystic Fibrosis; Evaluation Studies as Topic; Female; Genetic Linkage; Genetic Markers; Humans; Pedi | 1989 |
Results of second trimester prenatal diagnosis of cystic fibrosis in risk families.
Topics: Adult; Amniotic Fluid; Chlorides; Clinical Enzyme Tests; Cystic Fibrosis; Female; gamma-Glutamyltran | 1989 |
[Are there mucoviscidosis specific humoral factors? 1: Properties, prevalence, preparation, formation].
Topics: alpha-Macroglobulins; Blood Proteins; Calgranulin A; Ciliary Motility Disorders; Complement C3; Comp | 1985 |
[Diagnosis of mucoviscidosis by examination of sweat: value of conductometry].
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Flame Ionization; Galvanic Skin Response; Huma | 1989 |
Fractional measurements of sweat osmolality in patients with cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Iontophoresis; Male; Osmolar C | 1989 |
Elevated sweat potassium, hyperaldosteronism and pseudo-Bartter's syndrome: a spectrum of disorders associated with cystic fibrosis.
Topics: Bartter Syndrome; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Hyperaldosteronism; | 1989 |
"Typical" cystic fibrosis in an elderly woman.
Topics: Aged; Chlorides; Chronic Disease; Cystic Fibrosis; Female; Humans; Lung; Radiography; Sweat | 1989 |
Interpretation of sweat sodium results--the effect of patient age.
Topics: Age Factors; Child; Child, Preschool; Cystic Fibrosis; False Negative Reactions; Humans; Infant; Inf | 1989 |
Neonatal screening for cystic fibrosis.
Topics: Age Factors; Child; Child, Preschool; Cystic Fibrosis; Female; Growth; Humans; Infant; Infant, Newbo | 1986 |
Reduced morbidity in cystic fibrosis.
Topics: Australia; Cystic Fibrosis; Humans; Infant, Newborn; Length of Stay; Sweat | 1986 |
Neonatal screening for cystic fibrosis.
Topics: Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Sweat | 1986 |
Neonatal screening for cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Nether | 1986 |
Diagnosis of cystic fibrosis in premature infants.
Topics: Adult; Child; Cystic Fibrosis; Electrolytes; Humans; Infant, Newborn; Infant, Premature, Diseases; S | 1987 |
Use of DNA markers linked to cystic fibrosis gene to resolve equivocal sweat test results.
Topics: Child; Cystic Fibrosis; DNA; Genetic Markers; Humans; Infant; Male; Polymorphism, Restriction Fragme | 1987 |
Diagnosis of cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Humans; Sodium; Sweat | 1987 |
Diagnosis of cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Infant; Infant, Newborn; Sweat | 1987 |
Variable reduction in beta-adrenergic sweat secretion in cystic fibrosis heterozygotes.
Topics: Acclimatization; Adrenergic beta-Agonists; Adult; Cystic Fibrosis; Dose-Response Relationship, Drug; | 1988 |
DNA typing of a cystic fibrosis family with borderline sweat tests.
Topics: Child; Child, Preschool; Cystic Fibrosis; DNA; Female; Humans; Male; Polymorphism, Genetic; Polymorp | 1988 |
Sweat tests and flucloxacillin.
Topics: Cloxacillin; Cystic Fibrosis; Floxacillin; Humans; Infant; Male; Sweat | 1989 |
Abnormally high sweat osmolality in children with Down's syndrome.
Topics: Child, Preschool; Cystic Fibrosis; Down Syndrome; Humans; Iontophoresis; Osmolar Concentration; Swea | 1985 |
Sweat osmolality in Down's syndrome and cystic fibrosis in an Indian population.
Topics: Adolescent; Adult; Age Factors; Child; Cystic Fibrosis; Down Syndrome; Electrolytes; Female; Humans; | 1987 |
Diagnosis of cystic fibrosis.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Genetic Techniques; Humans; Infant; Infant, Newborn; M | 1988 |
Abnormal sweat electrolytes in symptomatic human immunodeficiency virus infection in a child.
Topics: Acquired Immunodeficiency Syndrome; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Fema | 1987 |
An evaluation of screening for cystic fibrosis.
Topics: Attitude of Health Personnel; Cost-Benefit Analysis; Cystic Fibrosis; False Negative Reactions; Fals | 1987 |
Diagnosis of cystic fibrosis.
Topics: Cystic Fibrosis; Female; Humans; Sodium Chloride; Sweat | 1988 |
Borderline sweat test: criteria for cystic fibrosis diagnosis.
Topics: Adult; Child; Cystic Fibrosis; Diet, Sodium-Restricted; False Negative Reactions; False Positive Rea | 1988 |
Cystic fibrosis: chromosome 7 DNA genotyping. An aide in resolving ambiguous diagnoses in siblings of known patients.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Chromosomes, Human, Pair 7; Cystic Fibrosis; Female; | 1988 |
Screening for cystic fibrosis: a four year regional experience.
Topics: Age Factors; Child; Child, Preschool; Chlorides; Cystic Fibrosis; False Negative Reactions; False Po | 1988 |
The sweat chloride concentration and prolactin activity in cystic fibrosis.
Topics: Animals; Chlorides; Cystic Fibrosis; Humans; Mice; Mice, Nude; Prolactin; Skin Transplantation; Swea | 1988 |
Automated Ektachem method for measuring sodium in sweat.
Topics: Autoanalysis; Cystic Fibrosis; Reference Values; Sodium; Sweat | 1988 |
Biochemical changes and endocrine responses in cystic fibrosis in relation to a marathon race.
Topics: Adolescent; Adult; Chlorides; Cystic Fibrosis; Hormones; Humans; Male; Physical Exertion; Running; S | 1988 |
[Decentralized screening for mucoviscidosis with a chloride-sensitive electrode].
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Female; Germany, East; Humans; Infa | 1988 |
Incidence and implications of false-negative sweat test reports in patients with cystic fibrosis.
Topics: Cystic Fibrosis; False Negative Reactions; Humans; Retrospective Studies; Sweat; Time Factors | 1988 |
Sweat test in children with cystic fibrosis.
Topics: Cystic Fibrosis; False Negative Reactions; Female; Humans; Infant; Infant, Newborn; Sodium; Sweat | 1988 |
[Sweat chloride concentration and pancreatic function tests in patients with diffuse panbronchiolitis].
Topics: Adult; Aged; Bronchiolitis; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Female; Humans; Mal | 1988 |
Sweat tests and flucloxacillin.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cloxacillin; Cystic Fibrosis; Floxacillin; Humans; I | 1988 |
Sweat testing by capillary collection and osmometry: suitability of the Wescor Macroduct System for screening suspected cystic fibrosis patients.
Topics: Adolescent; Capillary Permeability; Child; Child, Preschool; Cystic Fibrosis; Electrodes; Equipment | 1988 |
Cystic fibrosis--clinical viewpoint--a disease that doesn't make sense.
Topics: Chlorides; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Humans; Infant, Newborn; Intestinal O | 1987 |
The sweat test.
Topics: Cystic Fibrosis; Humans; Sodium Chloride; Sweat | 1987 |
Sweat sodium levels in adults with nasal polyps.
Topics: Adult; Cystic Fibrosis; Female; Humans; Immunotherapy; Male; Nasal Polyps; Skin Tests; Sodium; Sweat | 1987 |
[Sweat collection and osmometry in the diagnosis of cystic fibrosis: a new method].
Topics: Cystic Fibrosis; Electrolytes; Humans; Infant; Osmolar Concentration; Specimen Handling; Sweat | 1987 |
Chloride measurement by microelectrode in cystic fibrosis and normal sweat.
Topics: Chlorides; Chromatography, High Pressure Liquid; Cystic Fibrosis; False Negative Reactions; False Po | 1987 |
[Diagnosis of cystic fibrosis].
Topics: Child; Cystic Fibrosis; Humans; Methods; Sweat | 1987 |
[A simple and economical method of screening diagnosis of cystic fibrosis].
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Mass Screening; Sweat | 1987 |
Cystic fibrosis: report of a Chinese case with suggestive family history.
Topics: Asian People; Child; Cystic Fibrosis; Humans; Male; Pedigree; Radiography; Sweat; Taiwan | 1987 |
Sweat sulfate concentrations are decreased in cystic fibrosis.
Topics: Adolescent; Adult; Biological Transport; Child; Chlorides; Chromatography, Ion Exchange; Cystic Fibr | 1986 |
[Incidence of cystic fibrosis in children with diseases of the respiratory system].
Topics: Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; False Positive Reactions; Humans; | 1986 |
Malnutrition: a cause of elevated sweat chloride concentration.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Deficiency Diseases; Female; Humans | 1986 |
Sweat sodium related to amount of sweat after sweat test in children with and without cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Sodium; Sweat | 1986 |
Munchausen syndrome by proxy simulating cystic fibrosis.
Topics: Body Weight; Child Abuse; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Fat | 1986 |
Interpretation of sweat sodium and chloride concentrations.
Topics: Chlorides; Cystic Fibrosis; Humans; Sodium; Sweat | 1986 |
The sweat test.
Topics: Chlorides; Cystic Fibrosis; Diagnostic Errors; Humans; Sodium; Specimen Handling; Sweat | 1986 |
Lactate and bicarbonate uptake in the sweat duct of cystic fibrosis and normal subjects.
Topics: Bicarbonates; Chlorides; Cystic Fibrosis; Humans; Hydrogen-Ion Concentration; Lactates; Lactic Acid; | 1987 |
Misdiagnosis of cystic fibrosis. Need for continuing follow-up and reevaluation.
Topics: Asthma; Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Electrolytes; False Posit | 1987 |
Abnormal sweat electrolytes in a case of celiac disease and a case of psychosocial failure to thrive. Review of other reported causes.
Topics: Celiac Disease; Cystic Fibrosis; Diagnosis, Differential; Electrolytes; Failure to Thrive; False Pos | 1987 |
[Results of the sweat test, carried out by 2 methods, for the diagnosis of cystic fibrosis].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electric Conductivity; Humans; Infa | 1987 |
[Determination of electrolytes in sweat in the diagnosis of cystic fibrosis. A 23-year experience].
Topics: Chlorine; Cystic Fibrosis; Electrolytes; Humans; Iontophoresis; Potassium; Sodium; Sweat | 1985 |
Increased sweat chloride levels associated with prostaglandin E1 infusion.
Topics: Abnormalities, Multiple; Alprostadil; Chlorides; Cystic Fibrosis; Humans; Infant, Newborn; Long-Term | 1985 |
Diagnosis of cystic fibrosis during adolescence.
Topics: Adolescent; Adult; Body Height; Body Weight; Child; Chlorides; Cystic Fibrosis; Female; Forced Expir | 1986 |
[Cystic fibrosis].
Topics: Child, Preschool; Cystic Fibrosis; Finland; Humans; Infant; Infant, Newborn; Sweat | 1986 |
Advances in cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Pilocarpine; Sodium Chloride; Sweat | 1986 |
Increased nasal potential difference and amiloride sensitivity in neonates with cystic fibrosis.
Topics: Adult; Amiloride; Biological Transport; Child; Chlorides; Cystic Fibrosis; Electron Transport; Human | 1986 |
Plasma and urinary catecholamines in patients with cystic fibrosis.
Topics: Adolescent; Adult; Blood Pressure; Catecholamines; Ceruloplasmin; Child; Copper; Cystic Fibrosis; El | 1985 |
Screening for cystic fibrosis.
Topics: Child; Cystic Fibrosis; Evaluation Studies as Topic; Humans; Infant; Reagent Kits, Diagnostic; Sweat | 1985 |
Detection of cystic fibrosis heterozygotes using a modified loading with bromide.
Topics: Administration, Oral; Adolescent; Adult; Bromides; Child; Child, Preschool; Chlorides; Cystic Fibros | 1985 |
Coulometric titration of sweat collected with the Webster Collection System.
Topics: Chlorides; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Male; Specimen Handling; Sweat | 1985 |
Is the sweat test infallible in cystic fibrosis?
Topics: Cystic Fibrosis; Female; Humans; Infant; Male; Sodium; Sweat | 1985 |
A study of sweat sodium and chloride; criteria for the diagnosis of cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnostic Errors; Humans; Infant; | 1985 |
Liquid-chromatographic determination of chloride in sweat from cystic fibrosis patients and normal persons.
Topics: Child, Preschool; Chlorides; Chromatography, Ion Exchange; Cystic Fibrosis; Female; Humans; Infant; | 1985 |
Environmental deprivation and transient elevation of sweat electrolytes.
Topics: Child, Preschool; Cystic Fibrosis; Electrolytes; Failure to Thrive; Humans; Male; Psychosocial Depri | 1985 |
[Mucoviscidosis in the adult].
Topics: Adolescent; Chlorine; Cystic Fibrosis; Humans; Male; Pneumonia; Sweat | 1985 |
Anion-exchange chromatography to determine the concentration of chloride in sweat for diagnosis of cystic fibrosis.
Topics: Adult; Child; Child, Preschool; Chlorides; Chromatography, Ion Exchange; Cystic Fibrosis; Female; Hu | 1985 |
Recurrent respiratory disease, azoospermia, and nasal polyposis. A syndrome that mimics cystic fibrosis and immotile cilia syndrome.
Topics: Adult; Chronic Disease; Ciliary Motility Disorders; Cystic Fibrosis; Diagnosis, Differential; Humans | 1985 |
Thermal sweat lactate in cystic fibrosis and in normal children.
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Energy Metabolism; Hot Temperature; Humans; Inf | 1985 |
Mucoid Pseudomonas aeruginosa in patients with chronic illnesses.
Topics: Adolescent; Adult; Bacteriuria; Bronchiectasis; Calcium; Child; Child, Preschool; Chronic Disease; C | 1971 |
Screening for cystic fibrosis.
Topics: Anti-Bacterial Agents; Child Health Services; Costs and Cost Analysis; Cystic Fibrosis; England; Fem | 1973 |
Letter: Skin wrinkling in cystic fibrosis.
Topics: Cystic Fibrosis; Hand; Humans; Hydrogen-Ion Concentration; Immersion; Keratins; Protein Binding; Ski | 1974 |
Plasma proteins in meconium from normal infants and from babies with cystic fibrosis.
Topics: Antigens; Beta-Globulins; Blood Proteins; Chymotrypsin; Cystic Fibrosis; gamma-Globulins; Humans; Im | 1974 |
Mechanism of water and electrolyte secretion by the eccrine sweat gland.
Topics: Chemical Phenomena; Chemistry; Chlorides; Cystic Fibrosis; Humans; Lactates; Potassium; Sodium; Swea | 1966 |
Elevated sweat chlorides and hypothyroidism.
Topics: Chlorides; Cystic Fibrosis; Diagnosis, Differential; Electrolytes; Humans; Hypothyroidism; Infant; M | 1968 |
Effect of cystic-fibrosis sweat on sodium reabsorption by the normao sweat-gland.
Topics: Cystic Fibrosis; Humans; Potassium; Sodium; Sweat; Sweat Glands | 1970 |
Down's syndrome and cystic fibrosis.
Topics: Bone Marrow Diseases; Chlorides; Cystic Fibrosis; Down Syndrome; Humans; Infant; Pancreatic Diseases | 1969 |
[Current views on the etiopathogenesis of mucoviscidosis and on the characterization of the defect].
Topics: Calcium; Cystic Fibrosis; Electrolytes; Glucosyltransferases; Glycoproteins; Glycosaminoglycans; Hum | 1971 |
[Mucoviscidosis in adults].
Topics: Acetylcysteine; Adult; Bronchitis; Cystic Fibrosis; Duodenal Ulcer; Humans; Male; Middle Aged; Oxyte | 1968 |
Diminished excretion of bicarbonate from the single sweat gland of patients with cystic fibrosis of the pancreas.
Topics: Adult; Bicarbonates; Biological Transport; Body Water; Cell Membrane Permeability; Child; Child, Pre | 1974 |
Proceedings: Experience with an ion-specific electrode to measure sweat chloride.
Topics: Child; Chlorides; Cystic Fibrosis; Electrodes; Humans; Infant, Newborn; Sweat | 1974 |
Screening for cystic fibrosis with chloride electrode. An investigation of sweat chloride with chloride electrode orion 417 in normal persons and in patients with cystic fibrosis.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electric Stimulation; Female | 1974 |
[Mucoviscidosis and nasal polyposis in children].
Topics: Child; Chlorine; Cystic Fibrosis; Humans; Nasal Polyps; Nose Neoplasms; Sweat | 1974 |
[Nightsweat collection for simple diagnosis of mucoviscidosis (author's transl)].
Topics: Child; Chlorides; Cystic Fibrosis; Humans; Iontophoresis; Methods; Pilocarpine; Specimen Handling; S | 1974 |
Cystic fibrosis and coeliac disease: coexistence in two children.
Topics: Biopsy; Body Height; Body Weight; Celiac Disease; Child; Child, Preschool; Chlorides; Cystic Fibrosi | 1973 |
Cystic fibrosis in a patient with Kartagener syndrome.
Topics: Chlorides; Cystic Fibrosis; Humans; Infant; Kartagener Syndrome; Lung; Male; Radiography; Situs Inve | 1974 |
Cystic fibrosis--survival could depend on you.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diet Therapy; Feces; Humans; | 1972 |
Viscosity of exocrine secretions in cystic fibrosis: sweat, duodenal fluid and submaxillary saliva.
Topics: Cystic Fibrosis; Duodenum; Humans; Intestinal Secretions; Saliva; Submandibular Gland; Surface Prope | 1972 |
[Pathogenesis of mucoviscidosis].
Topics: Cystic Fibrosis; Heart; Humans; Liver; Lung; Metabolism, Inborn Errors; Pancreas; Sweat; Water-Elect | 1972 |
Sweat bromide test--a diagnostic tool for mucoviscidosis. A micromethod of bromide determination.
Topics: Adolescent; Adult; Bromides; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Methods; Micr | 1972 |
Cystic fibrosis: an improved diagnostic method utilizing constant current iontophoresis.
Topics: Chlorides; Cystic Fibrosis; Humans; Iontophoresis; Methods; Pilocarpine; Sodium; Sweat | 1972 |
[Risk of error in the determination of sweat chlorine with the Orion iontophoresis apparatus].
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; False Positive Reactions; Female; Humans; | 1973 |
[Laboratory studies of children with asthma and mucoviscidosis].
Topics: Asthma; Child; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Sweat | 1973 |
Cystic fibrosis and the role of the physical therapist in its management.
Topics: Aerosols; Breathing Exercises; Chlorides; Cystic Fibrosis; Drainage; Exercise Therapy; Family; Healt | 1973 |
Cystic fibrosis (mucoviscidosis).
Topics: Cystic Fibrosis; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; | 1973 |
Effects of vasopressin on sweat rate and composition in patients with diabetes insipidus and normal controls.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Diabetes Insipidus; Female; Humans; Iontophore | 1973 |
Metabolic alkalosis and salt depletion in cystic fibrosis.
Topics: Alkalosis; Cystic Fibrosis; Humans; Hyponatremia; Infant; Male; Potassium; Sodium; Sweat | 1973 |
Sweat tests in the newborn period.
Topics: Age Factors; Bethanechol Compounds; Chlorides; Cystic Fibrosis; Female; Humans; Infant; Infant, Newb | 1973 |
Newborn screening for cystic fibrosis with the chloride electrode and neutron activation analysis.
Topics: Activation Analysis; Chlorides; Cystic Fibrosis; Electrodes; Female; Humans; Male; Mass Screening; M | 1973 |
Direct measurement of chloride in sweat with an ion-selective electrode.
Topics: Adolescent; Bicarbonates; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differenti | 1973 |
Calcium and magnesium concentration in sweat of normal children and patients with cystic fibrosis.
Topics: Adolescent; Calcium; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Magnesium; Sweat | 1973 |
[Screening of cystic fibrosis by means of a simplified night sweat test].
Topics: Child; Cystic Fibrosis; Humans; Methods; Sweat; Sweating | 1973 |
Cystic fibrosis and coeliac disease. Report of two cases.
Topics: Biopsy; Body Height; Body Weight; Celiac Disease; Cystic Fibrosis; Diet Therapy; Disaccharides; Duod | 1973 |
[Sweat secretion tests in diabetic children].
Topics: Adolescent; Bromides; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diabetes Mellitus, Type 1 | 1973 |
Possible genetic links between cystic fibrosis of the pancreas and aspirin sensitive asthma.
Topics: Amino Acids; Animals; Aspirin; Asthma; Binding Sites; Cystic Fibrosis; Drug Hypersensitivity; Hetero | 1973 |
The decline of the sweat test. Comments on pitfalls and reliability.
Topics: Cystic Fibrosis; Humans; Iontophoresis; Methods; Sodium Chloride; Sweat; Sweating | 1973 |
Survey of electrolytes of unstimulated sweat from the hand in normal and diseased adults.
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Hand; H | 1973 |
Studies on ciliary dyskinesia factor in cystic fibrosis. 3. Skin fibroblasts and cultured amniotic fluid cells.
Topics: Amniotic Fluid; Biological Assay; Blood; Cell Division; Cells, Cultured; Cilia; Culture Media; Cysti | 1973 |
Letter: Screening for cystic fibrosis by specific ion electrode.
Topics: Chlorides; Cystic Fibrosis; Evaluation Studies as Topic; Humans; Mass Screening; Methods; Pilocarpin | 1973 |
[Follow-up studies in 10 cases of mucoviscidosis diagnosed in children].
Topics: Adolescent; Age Factors; Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Diet, So | 1973 |
Cystic fibrosis with edema and falsely negative sweat test.
Topics: Chlorides; Cystic Fibrosis; Edema; False Negative Reactions; Humans; Hypoproteinemia; Infant, Newbor | 1973 |
[Necessity and possibilities of mass screening in mucoviscidosis].
Topics: Adolescent; Age Factors; Bronchitis; Child; Child, Preschool; Chlorides; Chronic Disease; Cystic Fib | 1973 |
[Experiences in the standardisation of sweat analysis].
Topics: Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Methods; Sweat | 1973 |
[Sweat calcium concentration in children with cystic fibrosis of the pancreas and in normal children].
Topics: Calcium; Child; Child, Preschool; Cystic Fibrosis; Humans; Sweat | 1973 |
[Sodium content in the sweat and ungual laminae in healthy children and in those with mucoviscoidosis, as well as in relatives of the patients].
Topics: Adult; Child; Child, Preschool; Chlorine; Chronic Disease; Cystic Fibrosis; Female; Foot; Hand; Huma | 1973 |
[Diabetes mellitus and mucoviscidosis].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diabetes Complications; Glucose Tol | 1973 |
[Perspiration test for chronic recidive bronchitis in children (author's transl)].
Topics: Adolescent; Bronchitis; Child; Chronic Disease; Cystic Fibrosis; Female; Histamine; Humans; Infant, | 1973 |
Microperfusion study of the sweat gland abnormality in cystic fibrosis.
Topics: Adult; Animals; Carbon Radioisotopes; Cattle; Chlorides; Cystic Fibrosis; Female; Humans; In Vitro T | 1973 |
Sweat electrolytes as a clinical and genetic test in mucoviscidosis. Study of variability and genetic conditioning.
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Environment; Fe | 1973 |
Letter: Effect of antibiotics on sweat chloride levels in cystic fibrosis.
Topics: Chlorides; Cloxacillin; Cystic Fibrosis; Humans; Infant; Sweat | 1974 |
[Electrolyte transport in sweat glands of cystic fibrosis].
Topics: Adolescent; Adult; Biological Transport; Child; Child, Preschool; Cystic Fibrosis; Electrolytes; Hum | 1974 |
Letter: Rates and calcium concentrations of sweat in relation to cystic fibrosis.
Topics: Calcium; Cystic Fibrosis; Humans; Secretory Rate; Sweat | 1974 |
Letter: Cystic fibrosis with edema and falsely negative sweat test.
Topics: Chlorides; Cystic Fibrosis; Edema; False Negative Reactions; Female; Humans; Infant; Sodium; Sweat | 1974 |
[Bromide sweat test in diagnosis of mycoviscidosis. A micromethod for the determination of bromide].
Topics: Adolescent; Adult; Bromides; Child; Child, Preschool; Cystic Fibrosis; Heterozygote; Humans; Infant; | 1974 |
[Impression tests in the diagnosis of mucoviscidosis].
Topics: Agar; Child; Chlorine; Cystic Fibrosis; Humans; Iontophoresis; Methods; Sweat | 1974 |
[Test with desoxycorticosterone acetate in the diagnosis of mucoviscidosis in children].
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Desoxycorticosterone; Diet, Sodium-Rest | 1974 |
A methodological study of the diagnosis of cystic fibrosis by instrumental neutron activation analysis of sodium in nail clippings.
Topics: Activation Analysis; Child, Preschool; Cystic Fibrosis; Humans; Methods; Nails; Sodium; Sweat; Time | 1974 |
[Bromide test using ion-selective membrane electrodes in the demonstration of heterozygotes among patients with mucoviscidosis].
Topics: Bromides; Cystic Fibrosis; Heterozygote; Homozygote; Humans; Ions; Sweat | 1974 |
[Determination of the bromidine index in mucoviscidosis using an ionselective membrane-electrode].
Topics: Bromides; Cell Membrane Permeability; Chlorides; Cystic Fibrosis; Electrodes; Filtration; Heterozygo | 1974 |
Chronic suppurative lung disease in sisters mimicking cystic fibrosis.
Topics: Autopsy; Child; Chronic Disease; Cystic Fibrosis; Diagnosis, Differential; Female; Humans; Lung; Lun | 1974 |
[Determination of DNase activity in the sweat and the urine. Micro disc-electrophoretic assay in health and mucoviscidosis disease (author's transl)].
Topics: Adolescent; Calcium; Child; Child, Preschool; Cystic Fibrosis; Densitometry; Deoxyribonucleases; Ele | 1974 |
Cystic fibrosis cases found by re-examination of histology of pancreas and postmortem protocol in Japanese children and sweat test on the siblings.
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; In Vitro Techniques; Infant; I | 1965 |
Sweat proteins in cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Immunodiffusion; Immunoelectrophoresis; Proteins; Sweat | 1967 |
Micro-isoelectric focusing of proteins in pilocarpine-induced sweat.
Topics: Adult; Animals; Child; Child, Preschool; Cystic Fibrosis; Glycoproteins; Horses; Humans; Immune Sera | 1971 |
[Personal experience and general considerations concerning the determination of sweat electrolyte levels by means of pilocarpine iontophoresis].
Topics: Child; Child, Preschool; Cystic Fibrosis; Electrolytes; Female; Humans; Infant; Iontophoresis; Male; | 1972 |
[Diagnosis of mucoviscidosis. Gibson's and Cook's apparatus without batteries for sweat collection using pilocarpine iontophoresis].
Topics: Cystic Fibrosis; Humans; Iontophoresis; Pilocarpine; Sodium Chloride; Sweat | 1972 |
Anomalous sweat chloride levels in cystic fibrosis during antibiotic therapy.
Topics: Child, Preschool; Chlorides; Cloxacillin; Cystic Fibrosis; Electrodes; Female; Humans; Infant, Newbo | 1972 |
[Mucoviscidosis in childhood. Optimal sweat production and analysis].
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Ionto | 1972 |
The diagnosis of cystic fibrosis in the practice of allergy.
Topics: Child; Cystic Fibrosis; Diagnosis, Differential; Humans; Hypersensitivity; Male; Sweat | 1972 |
Reliability of sweat tests in diagnosis of cystic fibrosis.
Topics: Chlorides; Cystic Fibrosis; Diseases in Twins; False Negative Reactions; False Positive Reactions; H | 1972 |
[Development of chloride losses by sweating in children].
Topics: Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Male; Sweat; Sweating; W | 1972 |
Mucus, calcium, sweat, water, and permeability in cystic fibrosis.
Topics: Animals; Calcium; Cell Membrane Permeability; Cystic Fibrosis; Humans; Mucus; Rats; Sweat; Water-Ele | 1972 |
[Results concerning the state of children with mucoviscidosis treated in a specialized center. (4 years of experience at the Heliomarin Centre in roscoff)].
Topics: Breathing Exercises; Celiac Disease; Child; Child, Preschool; Cystic Fibrosis; Female; France; Hospi | 1972 |
Elevated sweat chlorides in a child with malnutrition.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Follow-Up Studies; Humans; In | 1971 |
[Liver changes in mucoviscidosis heterozygotes].
Topics: Biopsy; Bronchitis; Bronchography; Child; Chronic Disease; Connective Tissue; Cystic Fibrosis; Diagn | 1971 |
Some observations on sweat test by Pilocarpine Iontophoresis.
Topics: Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Humans; Infant; Iontophoresis; Nu | 1971 |
Effect of mineralocorticoids on sweat sodium in cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Mineralocorticoids; Sodium; Sweat | 1971 |
Metabolic alkalosis in cystic fibrosis.
Topics: Alkalosis; Bicarbonates; Birth Weight; Cystic Fibrosis; Diet Therapy; Diseases in Twins; Female; Hum | 1971 |
[Sweat analysis following pilocarpine iontophoresis. Normal values of chloride in sweat, saliva and tears in relation to the child's age].
Topics: Adult; Age Factors; Child; Cystic Fibrosis; Diagnosis, Differential; Humans; Iontophoresis; Methods; | 1971 |
[Sweating in the newborn infant. Studies on the pilocarpine stimulated single sweat gland].
Topics: Cystic Fibrosis; Female; Humans; Infant, Newborn; Male; Pilocarpine; Potassium; Sodium; Sweat; Sweat | 1971 |
[Screening for mucoviscidosis (cystic fibrosis-CF). Systematic review and results].
Topics: Child, Preschool; Cystic Fibrosis; Electrodes; Electrodiagnosis; Electrolytes; False Negative Reacti | 1971 |
Sweat tests in cystic fibrosis: a comparison of the thermal test with pilocarpine stimulation at two dose levels.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Evaluation Studies as | 1971 |
[Electrolytic disorders in patients with mucoviscidosis].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrolytes; Humans; Infant; Infan | 1968 |
Meconium ileus and fibrocystic disease of the pancreas in Japan.
Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; In Vitro Techniques; Infant; Infant, N | 1966 |
An analysis of 46 autopsy cases of Japanese children, showing fibrocystic changes of the pancreas and one case of fibrocystic disease of the pancreas.
Topics: Age Factors; Birth Weight; Chlorides; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Kidn | 1967 |
Fibrocystic disease of the pancreas (mucoviscidosis) in New Zealand.
Topics: Child, Preschool; Cystic Fibrosis; Electrolytes; Health Surveys; Humans; Infant; Infant, Newborn; Ne | 1968 |
Sweat electrolyte concentrations in Puerto Rican children.
Topics: Child; Child, Preschool; Cystic Fibrosis; Electrolytes; Female; Humans; Infant; Infant, Newborn; Mal | 1969 |
[Determination of electrolytes in sweat. Generalities].
Topics: Chlorides; Cystic Fibrosis; Humans; Iontophoresis; Methods; Sodium; Sweat | 1965 |
[Dependency on age and diagnostic evidence of sweat electrolytes].
Topics: Adolescent; Age Factors; Child; Child, Preschool; Cystic Fibrosis; Diabetes Mellitus; Humans; Infant | 1967 |
[The sweat test].
Topics: Cystic Fibrosis; Humans; Iontophoresis; Pilocarpine; Sweat | 1969 |
[Diagnostic difficulties in mucoviscidosis].
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Humans; Lung Diseases; | 1969 |
[Clinical findings in adult mucoviscidosis].
Topics: Adolescent; Chlorine; Cystic Fibrosis; Humans; Male; Radiography, Thoracic; Skull; Sodium; Sweat | 1969 |
Cystic fibrosis of pancreas in adults. A report of 4 cases.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Female; Humans; Iontophoresis; Male; Pilocarpi | 1969 |
[Measurement of sweat resistance in mucoviscodosis using a simple capillary probe].
Topics: Cystic Fibrosis; Electric Conductivity; Electrolytes; Humans; Infant, Newborn; Methods; Sodium Chlor | 1969 |
[Hair sodium, potassium and chloride content in normal conditions and in mucoviscidosis].
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Hair; Humans; I | 1970 |
Detection of heterozygocity for mucoviscidosis by the sweat bromide test.
Topics: Bromides; Chlorides; Cystic Fibrosis; Eugenics; Gastrointestinal Diseases; Heterozygote; Respiratory | 1970 |
[Amylasemia, heterogeneity of serum amylase and activity of the sweat amylolytic enzyme in members of families afflicted with mucoviscidosis].
Topics: Adolescent; Adult; Amylases; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Isoenzymes; M | 1970 |
Studies in cystic fibrosis. Report of 130 patients diagnosed under 3 months of age over a 20-year period.
Topics: Anemia; Body Weight; Chloramphenicol; Chlortetracycline; Cough; Cystic Fibrosis; Diabetes Mellitus, | 1970 |
[Problem of mucoviscidosis in children with diseases of the respiratory organs and diagnostic value of several tests].
Topics: Child; Child, Preschool; Clinical Enzyme Tests; Cystic Fibrosis; Electrolytes; Humans; Infant; Respi | 1970 |
[Factors in interpretation of sweat test in children. Determination of electrolytes using the neutron activation technic].
Topics: Activation Analysis; Adolescent; Child; Child, Preschool; Chlorine; Cystic Fibrosis; Female; Humans; | 1970 |
[Bronchiectasis in mucoviscidosis (cystic fibrosis)].
Topics: Bronchiectasis; Cystic Fibrosis; Humans; Infant, Newborn; Prognosis; Respiratory Therapy; Sweat | 1970 |
[The significance of the sweat test in adults].
Topics: Adolescent; Adult; Age Factors; Aged; Child; Child, Preschool; Chlorine; Cystic Fibrosis; Humans; In | 1970 |
[The sweat test: a simple method of specimen collection with a rubber cap].
Topics: Cystic Fibrosis; Electrolytes; Humans; Infant; Infant, Newborn; Specimen Handling; Sweat | 1970 |
[Aldosterone effect on the sodium chloride and potassium excretion in the sweat of cystic fibrosis patients and healthy persons].
Topics: Adolescent; Aldosterone; Biological Transport; Child; Cystic Fibrosis; Female; Humans; Male; Photome | 1970 |
Neutron activation analysis technique for nail sodium concentration in cystic fibrosis patients.
Topics: Activation Analysis; Adult; Age Factors; Child; Cystic Fibrosis; Female; Humans; Infant, Newborn; Ma | 1971 |
Distribution of pulmonary blood flow in children with cystic fibrosis.
Topics: Adult; Body Height; Body Weight; Carbon Dioxide; Child; Child, Preschool; Chlorides; Cystic Fibrosis | 1971 |
[Value of the systematic sweat test in chronic bronchitis for detection of mucoviscidosis with delayed manifestation].
Topics: Activation Analysis; Bronchitis; Chlorides; Chronic Disease; Cystic Fibrosis; Humans; Radioisotopes; | 1971 |
[Significance of the Shvakhman-Gam test and determination of sweat electrolyte levels in the diagnosis of mucoviscidosis in children with bronchial asthma].
Topics: Adolescent; Asthma; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Sodium; Swe | 1971 |
[Modification of a test for recognition of heterozygotic genecarriers of mucoviscidosis].
Topics: Child; Child, Preschool; Chromosome Aberrations; Chromosome Disorders; Cystic Fibrosis; Genes; Heter | 1971 |
Effect of exogenous mineralocorticoids on sweat sodium in cystic fibrosis.
Topics: Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Hydrocortisone; Male; Sodium; Sweat | 1971 |
[Sweat electrolytes in a selected group of children with suspected muscoviscidosis].
Topics: Body Weight; Child; Child, Preschool; Chlorine; Cystic Fibrosis; Humans; Infant; Sweat | 1971 |
[Salt loss as the initial symptom in mucoviscidosis].
Topics: Adrenal Gland Diseases; Cystic Fibrosis; Diagnosis, Differential; Humans; Infant; Male; Pancreatic D | 1971 |
The sweat chloride content in various diseases.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Sweat | 1967 |
Determination of bromide in the sweat after oral administration of bromide preparations.
Topics: Bromides; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Sweat | 1967 |
Chloride and bromide excretion in the sweat of children after oral administration of sodium bromide.
Topics: Bromides; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Dyspepsia; H | 1967 |
[Value of a simple method for collecting perspiration].
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Humans; Infant; Iontoph | 1967 |
[Functions of sweat glands].
Topics: Cystic Fibrosis; Humans; Sweat; Sweat Glands | 1967 |
Biochemical studies of sweat secretion in cystic fibrosis.
Topics: Calcium; Child; Cystic Fibrosis; Humans; Iontophoresis; Potassium; Sodium; Sweat; Sweat Glands; Swea | 1968 |
Sodium-potassium measurement interferences in various biological fluids.
Topics: Body Fluids; Cystic Fibrosis; Intestinal Secretions; Methods; Mucus; Photometry; Potassium; Saliva; | 1968 |
Sweat chloride analysis by chloride ion-specific electrode method using heat stimulation.
Topics: Chemistry, Clinical; Chlorides; Cystic Fibrosis; Electrodiagnosis; Hot Temperature; Humans; Iontopho | 1968 |
Presumptive tests for cystic fibrosis based on serum protein in meconium.
Topics: Agglutination Tests; Blood Proteins; Cystic Fibrosis; Electrolytes; Humans; Infant, Newborn; Infant, | 1968 |
[Studies directed to mucoviscidosis in the etiology of respiratory diseases and in systemic diseases of the connective tissue].
Topics: Arthritis, Rheumatoid; Child; Chlorides; Cystic Fibrosis; Humans; Iontophoresis; Respiratory Tract D | 1968 |
Meconium ileus equivalent.
Topics: Child; Cystic Fibrosis; Fecal Impaction; Female; Humans; Sodium; Sweat | 1968 |
Cases of cystic fibrosis of the pancreas in the District Allergological Outpatient Department for Children in Zabrze.
Topics: Asthma; Child; Chlorides; Cystic Fibrosis; Humans; Outpatient Clinics, Hospital; Sweat | 1968 |
[Contribution to comparative studies of mucoviscidosis and bronchial asthma in children].
Topics: Adolescent; Asthma; Child; Child, Preschool; Cystic Fibrosis; Electrolytes; Humans; Sweat | 1968 |
The effect of adrenergic stimulation upon sweating in normal children and cystic fibrosis patients.
Topics: Adolescent; Adult; Atropine; Child; Child, Preschool; Cystic Fibrosis; Epinephrine; Humans; Iontopho | 1968 |
[Study of the sweating function in 2 cases of adult mucoviscidosis].
Topics: Absorption; Adult; Age Factors; Aldosterone; Bronchitis; Cystic Fibrosis; Female; Humans; Male; Pedi | 1968 |
Protein in meconium from meconium ileus.
Topics: Albumins; Child; Child, Preschool; Cystic Fibrosis; Electrophoresis; Humans; Infant; Infant, Newborn | 1968 |
Cystic fibrosis and pregnancy.
Topics: Adult; Cystic Fibrosis; Diagnosis, Differential; Female; Humans; Infant, Newborn; Iontophoresis; Pil | 1968 |
Some hazards of the sweat test.
Topics: Blister; Burns, Chemical; Burns, Electric; Child; Cystic Fibrosis; Electrodiagnosis; Humans; Hydroch | 1968 |
[Diagnosis and differential diagnosis of mucoviscidosis (short report)].
Topics: Child; Cystic Fibrosis; Diagnosis, Differential; Duodenum; Electrolytes; Humans; Pancreas; Sweat | 1968 |
[False pathologic findings in sweat test].
Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Electrolytes; Humans; | 1968 |
Sweat composition in relation to rate of sweating in patients with cystic fibrosis of the pancreas.
Topics: Calcium; Chlorides; Creatinine; Cystic Fibrosis; Glucose; Humans; Hydrogen-Ion Concentration; Lactat | 1968 |
[Study of the mucoviscidosis factor and familiar factors in chronic bronchitis of the miners].
Topics: Adult; Aged; Bronchitis; Chronic Disease; Cystic Fibrosis; Environment; Humans; Male; Middle Aged; M | 1968 |
[Behavior of extracellular fluid volume and volume of the chlorine metabolic pool in mucoviscidosis].
Topics: Chlorine; Cystic Fibrosis; Electrolytes; Endocrine Glands; Extracellular Space; Humans; Infant; Swea | 1968 |
Sweat electrolytes in mature and premature neonates after normal and pathological gestations and births.
Topics: Calcium; Cystic Fibrosis; Female; Humans; Infant, Newborn; Infant, Premature; Iontophoresis; Pilocar | 1968 |
The values of polarographically active proteinaceous substances in the sweat of children.
Topics: Adolescent; Age Factors; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Polarography; Pro | 1968 |
General discussion. Sweat--"First sample phenomenon".
Topics: Adult; Aldosterone; Animals; Binding Sites; Biological Transport; Calcium; Cats; Cell Membrane Perme | 1968 |
Studies in cystic fibrosis: determination of sweat electrolytes in situ with direct reading electrodes.
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Electrodes; Hot Temperature; Humans; Infant; In | 1969 |
Cystic fibrosis of the pancreas. Diagnosis by sodium electrode sweat tests.
Topics: Adult; Child, Preschool; Cystic Fibrosis; Humans; Sodium; Sweat | 1969 |
Investigation of the child with asthma.
Topics: Allergens; Asthma; Blood Gas Analysis; Body Height; Body Weight; Child; Child, Preschool; Cystic Fib | 1969 |
Cystic fibrosis of the pancreas.
Topics: Child; Cystic Fibrosis; Humans; Saliva; Sweat | 1969 |
[Results of studies for mucoviscidosis in school children in Cieszyn].
Topics: Adolescent; Child; Cystic Fibrosis; Humans; Mass Screening; Poland; School Health Services; Sodium C | 1969 |
[On the problem of mucoviscidosis in adults].
Topics: Adult; Age Factors; Aged; Bronchitis; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Female; H | 1969 |
Micropuncture studies of the sweat formation in cystic fibrosis patients.
Topics: Adolescent; Adult; Child; Chlorides; Cystic Fibrosis; Female; Humans; Male; Methods; Osmolar Concent | 1969 |
[On the diagnosis of mucoviscidosis (cystic fibrosis)].
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Indicators and Reagents; Infant; Iontop | 1969 |
Excretion of 4-14C-cortisol and 1,2-3H-D-aldosterone in human thermal sweat.
Topics: Adult; Aldosterone; Carbon Isotopes; Chromatography, Paper; Cortisone; Cystic Fibrosis; Humans; Hydr | 1969 |
An infant with both cystic fibrosis and coeliac disease.
Topics: Biopsy; Celiac Disease; Cystic Fibrosis; Diet Therapy; Duodenum; Glutens; Humans; Infant; Intestinal | 1969 |
[Detection of mucoviscidosis heterozygotes by the bromide sweat test].
Topics: Adult; Bromides; Child; Cystic Fibrosis; Heterozygote; Homozygote; Humans; Sweat | 1969 |
A new screening test for cystic fibrosis.
Topics: Adolescent; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Mass Screening; Sod | 1965 |
Accuracy of the sweat test: advantage of a micromethod.
Topics: Cystic Fibrosis; Humans; In Vitro Techniques; Iontophoresis; Pilocarpine; Sweat | 1965 |
Absence of detrimental effect of the carrier state for the cystic fibrosis gene.
Topics: Adult; Carrier State; Chlorides; Communicable Diseases; Cystic Fibrosis; Ethnology; Female; Humans; | 1965 |
[Pancreatic cystic fibrosis. Its diagnosis by means of the determination of the electric conductivity of the sweat. Our experience in 216 determinations].
Topics: Adolescent; Chemical Phenomena; Chemistry, Physical; Child; Child, Preschool; Cystic Fibrosis; Elect | 1965 |
[Contribution to the study of mucoviscidosis in the adult].
Topics: Adult; Cystic Fibrosis; Desoxycorticosterone; Electrolytes; Humans; Male; Sweat | 1964 |
[Influence of aldosterone on the concentration of sodium and chloride in sweat in mucoviscidosis and in the normal subject].
Topics: Adolescent; Aldosterone; Child; Chlorides; Cystic Fibrosis; Diet, Sodium-Restricted; Electrolytes; H | 1963 |
The electrolyte composition of normal adult sweat.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; In Vitro Techn | 1966 |
Cystic fibrosis of the pancreas: a study of sweat electrolyte levels in thirty-six families using pilocarpine iontophoresis.
Topics: Adult; Child; Cystic Fibrosis; Electrolytes; Humans; Iontophoresis; Pilocarpine; Sweat | 1966 |
Quantitative estimation of sweat chloride in cases of recurrent bronchitis in children.
Topics: Adult; Aged; Bronchitis; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Middle Aged; S | 1966 |
Characteristics of cystic fibrosis in adults: a report of seven patients.
Topics: Adolescent; Adult; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Female; Follow-Up Studies; H | 1966 |
Diagnosis of heterozygosity for cystic fibrosis by discriminatory analysis of sweat chloride distribution.
Topics: Adolescent; Adult; Child; Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Male; Statis | 1966 |
The sweat test in cystic fibrosis. A comparison of overnight sweat collection versus the pilocarpine iontophoresis method.
Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Electrolytes; Humans; Infant; Infant, N | 1966 |
Cystic fibrosis sweat test for newborns.
Topics: Cystic Fibrosis; Humans; Infant, Newborn; Mass Screening; Sweat | 1966 |
The influx and outflux of sodium in the sweat-gland.
Topics: Cystic Fibrosis; Humans; Secretory Rate; Sodium; Sweat; Sweat Glands; Urea | 1966 |
The surgical treatment of bronchiectasis and the possibility of mucoviscidosis.
Topics: Adolescent; Adult; Bronchiectasis; Chlorides; Cystic Fibrosis; Humans; Middle Aged; Sweat | 1966 |
[Total osmolarity and principal osmoactive constituents of sweat in subjects with mucoviscidosis and their families].
Topics: Adult; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infant; Lactates; Middle Aged; Potassiu | 1966 |
[Total osmolarity of sweat in normal subjects of the 1st, 2nd and 3rd pediatric ages].
Topics: Chemical Phenomena; Chemistry, Physical; Child, Preschool; Chlorides; Cystic Fibrosis; Humans; Infan | 1966 |
[Mercurimetric determination of chlorides in sweat].
Topics: Child; Child, Preschool; Chlorides; Cystic Fibrosis; Diagnosis, Differential; Female; Humans; Infant | 1966 |