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  • McGarry, Meghan E.  (5)
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  • 1
    Online Resource
    Online Resource
    In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N‐of‐1 studies
    Wiley ; 2017
    In:  Pediatric Pulmonology Vol. 52, No. 4 ( 2017-04), p. 472-479
    Details
    In: Pediatric Pulmonology, Wiley, Vol. 52, No. 4 ( 2017-04), p. 472-479
    Abstract: Ivacaftor, a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, decreases sweat chloride concentration, and improves pulmonary function in 6% of cystic fibrosis (CF) patients with specific CFTR mutations. Ivacaftor increases chloride transport in many other CFTR mutations in non‐human cells, if CFTR is in the epithelium. Some CF patients have CFTR in the epithelium with residual CFTR function. The effect of ivacaftor in these patients is unknown. Methods This was a series of randomized, crossover N‐of‐1 trials of ivacaftor and placebo in CF patients ≥8 years old with potential residual CFTR function (intermediate sweat chloride concentration, pancreatic sufficient, or mild bronchiectasis on chest CT). Human nasal epithelium (HNE) was obtained via nasal brushing and cultured. Sweat chloride concentration change was the in vivo outcome. Chloride current change in HNE cultures with ivacaftor was the in vitro outcome. Results Three subjects had decreased sweat chloride concentration (−14.8 to −40.8 mmol/L, P   〈  0.01). Two subjects had unchanged sweat chloride concentration. Two subjects had increased sweat chloride concentration (+23.8 and +27.3 mmol/L, P   〈  0.001); both were heterozygous for A455E and pancreatic sufficient. Only subjects with decreased sweat chloride concentration had increased chloride current in HNE cultures. Conclusions Some CF patients with residual CFTR function have decreased sweat chloride concentration with ivacaftor. Increased chloride current in HNE cultures among subjects with decreased sweat chloride concentrations may predict clinical response to ivacaftor. Ivacaftor can increase sweat chloride concentration in certain mutations with unclear clinical effect. Pediatr Pulmonol. 2017;52:472–479. © 2017 Wiley Periodicals, Inc.
    Type of Medium: Online Resource
    ISSN: 8755-6863 , 1099-0496
    URL: Issue
    URL: Article
    DOI: 10.1002/ppul.v52.4
    DOI: 10.1002/ppul.23659
    Language: English
    Publisher: Wiley
    Publication Date: 2017
    detail.hit.zdb_id: 1491904-7
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  • 2
    Online Resource
    Online Resource
    Regional variations in longitudinal pulmonary function: A comparison of Hispanic and non‐Hispanic subjects with cystic fibrosis in the United States
    Wiley ; 2019
    In:  Pediatric Pulmonology Vol. 54, No. 9 ( 2019-09), p. 1382-1390
    Details
    In: Pediatric Pulmonology, Wiley, Vol. 54, No. 9 ( 2019-09), p. 1382-1390
    Abstract: Hispanic subjects with cystic fibrosis (CF) have increased morbidity and mortality than non‐Hispanic white subjects. The ethnic disparity in mortality varies by region. Factors influencing pulmonary function vary by both ethnicity and region. Objective To determine if the ethnic difference in pulmonary function varies by region. Methods This retrospective cohort study compared differences in longitudinal pulmonary function (percent‐predicted forced vital capacity [FVC], forced expiratory volume in 1 second [FEV 1 ], forced expiratory flow at 25% to 75% [FEF 25‐75 ], FEV 1 /FVC, and FEV 1 decline) between Hispanic and non‐Hispanic white subjects with CF by Census region of the United States (West, South, Midwest, and Northeast). Subjects were of ages 6 to 25 years and in the CF Foundation Patient Registry from 2008 to 2013. We used linear mixed effects models with subject‐specific slopes and intercepts, adjusting for 14 demographic and clinical variables. Results Of 14 932 subjects, 1433 (9.6%) were Hispanic and 13 499 (90.4%) were non‐Hispanic white. Hispanic subjects’ FEV 1 was 9.0% (8.3%‐9.8%) lower than non‐Hispanic white subjects in the West, while Hispanic subjects’ FEV 1 was only 4.0% (3.0%‐5.0%) lower in the Midwest, 4.4% (3.1%‐5.7%) lower in the Northeast, and 4.4% (3.2%‐5.5%) lower in the South. Similarly, FVC and FEF 25‐75 were lower among Hispanic subjects compared to non‐Hispanic white subjects in all US regions, with the biggest differences in the West. Only in the West was FEV 1 /FVC significantly lower in Hispanic subjects (−0.019; −0.022 to −0.015). FEV 1 decline was not significantly different between ethnicities in any region. Conclusions In CF, Hispanic subjects have lower pulmonary function than non‐Hispanic white subjects in all geographic regions with the largest difference in occurring in the West.
    Type of Medium: Online Resource
    ISSN: 8755-6863 , 1099-0496
    URL: Issue
    URL: Article
    DOI: 10.1002/ppul.v54.9
    DOI: 10.1002/ppul.24377
    Language: English
    Publisher: Wiley
    Publication Date: 2019
    detail.hit.zdb_id: 1491904-7
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  • 3
    Online Resource
    Online Resource
    Normalization of Sweat Chloride Concentration and Clinical Improvement With Ivacaftor in a Patient With Cystic Fibrosis With Mutation S549N
    Elsevier BV ; 2013
    In:  Chest Vol. 144, No. 4 ( 2013-10), p. e1-e4
    Details
    In: Chest, Elsevier BV, Vol. 144, No. 4 ( 2013-10), p. e1-e4
    Type of Medium: Online Resource
    ISSN: 0012-3692
    URL: Article
    DOI: 10.1378/chest.13-0239
    RVK:
    XA 36340
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2013
    detail.hit.zdb_id: 2007244-2
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  • 4
    Online Resource
    Online Resource
    Pulmonary function disparities exist and persist in Hispanic patients with cystic fibrosis: A longitudinal analysis
    Wiley ; 2017
    In:  Pediatric Pulmonology Vol. 52, No. 12 ( 2017-12), p. 1550-1557
    Details
    In: Pediatric Pulmonology, Wiley, Vol. 52, No. 12 ( 2017-12), p. 1550-1557
    Abstract: Hispanic patients with cystic fibrosis (CF) have decreased life expectancy compared to non‐Hispanic white patients. Pulmonary function is a main predictor of life expectancy in CF. Ethnic differences in pulmonary function in CF have been understudied. The objective was to compare longitudinal pulmonary function between Hispanic and non‐Hispanic white patients with CF. Methods This cohort study of 15 018 6‐25 years old patients in the CF Foundation Patient Registry from 2008 to 2013 compared FEV 1 percent predicted and longitudinal change in FEV 1 percent predicted in Hispanic to non‐Hispanic white patients. We used linear mixed effects models with patient‐specific slopes and intercepts, adjusting for 14 demographic and clinical variables. We did sub‐analyses by CFTR class, F508del copies, and PERT use. Results Hispanic patients had lower FEV 1 percent predicted (79.9%) compared with non‐Hispanic white patients (85.6%); (−5.8%, 95%CI −6.7% to −4.8%, P   〈  0.001), however, there was no difference in FEV 1 decline over time. Patients on PERT had a larger difference between Hispanic and non‐Hispanic white patients in FEV 1 percent predicted than patients not on PERT (−6.0% vs −4.1%, P  = 0.02). The ethnic difference in FEV 1 percent predicted was not statistically significant between CFTR classes (Class I‐III: −6.1%, Class IV‐V: −5.9%, Unclassified: −5.7%, P  〉  0.05) or between F508del copies (None: −7.6%, Heterozygotes: −5.6%, Homozygotes: −5.3%, P  〉  0.05). Conclusions Disparities in pulmonary function exist in Hispanic patients with CF early in life and then persist without improving or worsening over time. It is valuable to investigate the factors contributing to pulmonary function in Hispanic patients with CF.
    Type of Medium: Online Resource
    ISSN: 8755-6863 , 1099-0496
    URL: Issue
    URL: Article
    DOI: 10.1002/ppul.v52.12
    DOI: 10.1002/ppul.23884
    Language: English
    Publisher: Wiley
    Publication Date: 2017
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  • 5
    Online Resource
    Online Resource
    Early acquisition and conversion of Pseudomonas aeruginosa in Hispanic youth with cystic fibrosis in the United States
    Elsevier BV ; 2021
    In:  Journal of Cystic Fibrosis Vol. 20, No. 3 ( 2021-05), p. 424-431
    Details
    In: Journal of Cystic Fibrosis, Elsevier BV, Vol. 20, No. 3 ( 2021-05), p. 424-431
    Type of Medium: Online Resource
    ISSN: 1569-1993
    URL: Article
    DOI: 10.1016/j.jcf.2020.10.002
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2021
    detail.hit.zdb_id: 2091075-7
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