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  1. Article ; Online: Clinical effectiveness of elexacaftor/tezacaftor/ivacaftor in a man with A559T and 3120+1G>CFTR variants.

    Mitchell, Emily / Albon, Dana

    Pediatric pulmonology

    2024  

    Language English
    Publishing date 2024-04-12
    Publishing country United States
    Document type Journal Article
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.26997
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    Zs.A 2143: Show issues Location:
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  2. Article ; Online: Real world study on elexacaftor-tezacaftor-ivacaftor impact on cholesterol levels in adults with cystic fibrosis.

    Lonabaugh, Kevin / Li, Galvin / List, Rhonda / Huang, Reyna / James, Amber / Barros, Andrew / Somerville, Lindsay / Albon, Dana

    Pharmacotherapy

    2024  Volume 44, Issue 3, Page(s) 231–240

    Abstract: Introduction: The introduction of the highly effective modulator therapy elexacaftor-tezacaftor-ivacaftor (ETI) has revolutionized the care of persons with cystic fibrosis (PwCF) with major improvements seen in lung function and body mass index. The ... ...

    Abstract Introduction: The introduction of the highly effective modulator therapy elexacaftor-tezacaftor-ivacaftor (ETI) has revolutionized the care of persons with cystic fibrosis (PwCF) with major improvements seen in lung function and body mass index. The effects of ETI therapy in real-world cohorts on other parameters such as cholesterol levels are largely unknown.
    Methods: A single-center, retrospective chart review study was conducted to assess the change in lipid panels before and after ETI initiation. The study investigated total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), and triglyceride levels using both a univariate and multivariate mixed-effects model to evaluate the change after initiation of ETI in a cohort of PwCF.
    Results: There were 128 adult PwCF included in the analysis. Statistically significant changes were seen in both univariate and multivariate analyses for TC, LDL-C, and HDL-C. On multivariate analysis, TC increased by an average of 15.0 mg/dL after ETI initiation (p < 0.0001), LDL-C increased by an average of 9.3 mg/dL (p < 0.001), and HDL-C increased by an average of 3.8 mg/dL (p < 0.001) after ETI initiation.
    Conclusion: In this real-world cohort of PwCF, cholesterol parameters increased after initiation with ETI therapy. Further consideration may need to be given for PwCF in regards to screening for cardiometabolic risk factors as PwCF age as well as the potential need for cholesterol-lowering therapies.
    MeSH term(s) Adult ; Humans ; Cholesterol, LDL ; Cystic Fibrosis/drug therapy ; Retrospective Studies ; Mutation ; Benzodioxoles ; Indoles ; Aminophenols ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
    Chemical Substances elexacaftor (RRN67GMB0V) ; ivacaftor (1Y740ILL1Z) ; tezacaftor ; Cholesterol, LDL ; Benzodioxoles ; Indoles ; Aminophenols ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
    Language English
    Publishing date 2024-01-12
    Publishing country United States
    Document type Journal Article
    ZDB-ID 603158-4
    ISSN 1875-9114 ; 0277-0008
    ISSN (online) 1875-9114
    ISSN 0277-0008
    DOI 10.1002/phar.2903
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    Zs.A 1738: Show issues Location:
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    Zs.MO 585: Show issues

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  3. Article ; Online: Potential Implications of the COVID-19 Pandemic on the Homeless Population.

    Albon, Dana / Soper, Morgan / Haro, Anthony

    Chest

    2020  Volume 158, Issue 2, Page(s) 477–478

    MeSH term(s) Betacoronavirus ; COVID-19 ; Coronavirus Infections/epidemiology ; Delivery of Health Care/methods ; Homeless Persons/statistics & numerical data ; Humans ; Pandemics ; Pneumonia, Viral/epidemiology ; SARS-CoV-2 ; United States/epidemiology
    Keywords covid19
    Language English
    Publishing date 2020-05-08
    Publishing country United States
    Document type Editorial
    ZDB-ID 1032552-9
    ISSN 1931-3543 ; 0012-3692
    ISSN (online) 1931-3543
    ISSN 0012-3692
    DOI 10.1016/j.chest.2020.03.057
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  5. Article: The Impact of CFTR Modulator Triple Therapy on Type 2 Inflammatory Response in Patients with Cystic Fibrosis.

    Mehta, Ajay / Lee, Irene / Li, Galvin / Jones, Marieke / Hanson, Lydia / Lonabaugh, Kevin / List, Rhonda / Borish, Larry / Albon, Dana

    Research square

    2023  

    Abstract: Background Treatment of cystic fibrosis (CF) has been revolutionized by the use of cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators such as elexacaftor/tezacaftor/ivacaftor (ETI) triple therapy. Prior studies support a role ... ...

    Abstract Background Treatment of cystic fibrosis (CF) has been revolutionized by the use of cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators such as elexacaftor/tezacaftor/ivacaftor (ETI) triple therapy. Prior studies support a role for type 2 (T2) inflammation in many people with CF (PwCF) and CF-asthma overlap syndrome (CFAOS) is considered a separate clinical entity. It is unknown whether initiation of ETI therapy impacts T2 inflammation in PwCF. We hypothesized that ETI initiation decreases T2 inflammation in PwCF. Methods A single center retrospective chart review was conducted for adult PwCF. As markers of T2 inflammation, absolute eosinophil count (AEC) and total immunoglobulin E (IgE) data were collected longitudinally 12 months prior to ETI therapy initiation and 12 months following therapy initiation. Multivariable analyses adjusted for the age, gender, CFTR mutation, disease severity, inhaled steroid use, and microbiological colonization. Results There was a statistically significant reduction (20.10%, p < 0.001) in 12-month mean IgE following ETI initiation; this change remained statistically significant in the multivariate model. The longitudinal analysis demonstrated no change in AEC following therapy initiation. Conclusion This study shows reduction in IgE but no change in AEC after ETI therapy initiation. We think that the lack of influence on AEC argues against an impact on previously established T2 inflammation and that the reduction in IgE is likely related to antigen load reduction post ETI. Further studies are warranted to determine the underlying mechanism of ETI impact on T2 inflammation and possible role for asthma immunomodulator therapy post ETI initiation in CFAOS.
    Language English
    Publishing date 2023-05-10
    Publishing country United States
    Document type Preprint
    DOI 10.21203/rs.3.rs-2846739/v1
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Analysis of iron status after initiation of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis.

    James, Amber / Li, Galvin / List, Rhonda / Lonabaugh, Kevin / Smith, Aaron D / Barros, Andrew / Somerville, Lindsay / Albon, Dana

    Pediatric pulmonology

    2023  Volume 59, Issue 3, Page(s) 669–678

    Abstract: Background: Iron deficiency is highly prevalent in people with cystic fibrosis (PwCF). While elexacaftor/tezacaftor/ivacaftor (ETI) has shown remarkable improvements in respiratory symptoms in PwCF, the effect of ETI on iron status remains unknown. This ...

    Abstract Background: Iron deficiency is highly prevalent in people with cystic fibrosis (PwCF). While elexacaftor/tezacaftor/ivacaftor (ETI) has shown remarkable improvements in respiratory symptoms in PwCF, the effect of ETI on iron status remains unknown. This study aims to identify the effect of ETI on iron status in PwCF.
    Methods: A single-center retrospective cohort study of 127 adult PwCF was conducted to assess the impact of ETI on iron, ferritin, transferrin levels, and percent saturation of transferrin (PSAT). Data were collected from the electronic medical record from January 2017 to September 2022, encompassing 2 years before and after ETI initiation. The primary outcome was serum iron parameters: iron, ferritin, transferrin, and PSAT levels following ETI treatment. Secondary outcomes analyzed iron supplementation. Univariate and multivariate mixed-effects models were used for the analysis of ETI.
    Results: After adjusting for covariates, following ETI initiation, the mean iron level increased by 20.24 μg/dL (p < .001), ferritin levels were 31.4% (p < .001) higher, PSAT showed a 5.09 percentage point increase (p < .001), and transferrin levels increased by 2.71 mg/dL (p = .439). Patients with and without iron supplementation experienced a significant increase in iron after ETI (p < .001).
    Conclusions: ETI is associated with a significant increase in iron, ferritin, and PSAT levels. Patients with and without iron supplementation demonstrated a significant increase in iron. This study shows the benefits of ETI on iron status in PwCF. However, further translational studies are required to understand the impact of ETI on iron absorption and metabolism in PwCF.
    MeSH term(s) Adult ; Humans ; Iron ; Cystic Fibrosis/complications ; Cystic Fibrosis/drug therapy ; Retrospective Studies ; Ferritins ; Transferrins ; Cystic Fibrosis Transmembrane Conductance Regulator ; Mutation ; Aminophenols/therapeutic use ; Benzodioxoles/therapeutic use ; Indoles ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
    Chemical Substances Iron (E1UOL152H7) ; elexacaftor (RRN67GMB0V) ; ivacaftor (1Y740ILL1Z) ; tezacaftor ; Ferritins (9007-73-2) ; Transferrins ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) ; Aminophenols ; Benzodioxoles ; Indoles ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
    Language English
    Publishing date 2023-12-13
    Publishing country United States
    Document type Journal Article
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.26805
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    Zs.A 2143: Show issues Location:
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  7. Article: Association between Cystic Fibrosis exacerbations, lung function, T2 inflammation and microbiological colonization.

    Albon, Dana / Zhang, Lijia / Patrie, James / Jones, Marieke / Li, Z Galvin / Noonan, Emily / Borish, Larry

    Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology

    2023  Volume 19, Issue 1, Page(s) 15

    Abstract: Background: The Cystic Fibrosis Foundation Patient Registry (CFFPR) reports a high prevalence of asthma (34.6%) in people with Cystic Fibrosis (PwCF). While our current understanding of this relationship is limited, a type 2 inflammatory (T2) phenotype ... ...

    Abstract Background: The Cystic Fibrosis Foundation Patient Registry (CFFPR) reports a high prevalence of asthma (34.6%) in people with Cystic Fibrosis (PwCF). While our current understanding of this relationship is limited, a type 2 inflammatory (T2) phenotype has often been identified in CF patients.
    Research question: This study aimed to evaluate the relationship between the eosinophilic CF T2 inflammatory phenotype and CF-related pulmonary outcomes and microbiological data.
    Study design and methods: We conducted a retrospective chart review of adult patients with CF (18 and older; n = 93) receiving their care at University of Virginia Medical Center adult program from January, 2013 through December, 2018. Data collected included demographic data, CFTR (CF transmembrane conductance regulator) mutation, CF comorbidities, medications, Absolute Eosinophil Counts (AEC) in cells/µL and Immunoglobulin E (IgE) levels in IU/mL.
    Results: Of 93 patients screened for study eligibility, 74 were included in the final analysis; 19 patients were excluded due to lack of longitudinal data across the study timeline. Lung function decline correlated with increased AEC (p < 0.001) and IgE (p < 0.001) even when adjusting for covariates: age, gender, presence of Pseudomonas spp., MRSA, other bacterial spp., Aspergillus spp., and other fungi (p < 0.001). Univariate analysis demonstrated that people with CF who experienced more than 2 exacerbations requiring hospitalizations and/or intravenous antibiotics a year were more likely to have high AEC (p = 0.018). Logistic regression showed that as AEC increases, the probability that the measurement was taken during a CF exacerbation increases (p = 0.0039). A linear mixed model showed that each additional annual exacerbation event increased on average the log IgE by 0.04. (p = 0.015). This finding remained stable in a multivariate model (p = 0.0145). When adjusted for atopy, log IgE increases as the number of exacerbation events increases (p = 0.022). There was no association between AEC and IgE and microbiological colonization.
    Interpretation: This study has shown that in CF patients, T2 inflammation based on serum AEC and IgE correlated with pulmonary exacerbations requiring hospitalizations and/or intravenous antibiotics, independent of bacterial airway colonization. In addition, lung function decline correlated with increased IgE and AEC. Further studies are needed to explore these correlations and potential impact on treatment.
    Language English
    Publishing date 2023-02-27
    Publishing country England
    Document type Journal Article
    ZDB-ID 2434973-2
    ISSN 1710-1492 ; 1710-1484
    ISSN (online) 1710-1492
    ISSN 1710-1484
    DOI 10.1186/s13223-023-00760-z
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  8. Article ; Online: Potential Implications of the COVID-19 Pandemic on the Homeless Population

    Albon, Dana / Soper, Morgan / Haro, Anthony

    Chest

    2020  Volume 158, Issue 2, Page(s) 477–478

    Keywords Critical Care and Intensive Care Medicine ; Pulmonary and Respiratory Medicine ; Cardiology and Cardiovascular Medicine ; covid19
    Language English
    Publisher Elsevier BV
    Publishing country us
    Document type Article ; Online
    ZDB-ID 1032552-9
    ISSN 1931-3543 ; 0012-3692
    ISSN (online) 1931-3543
    ISSN 0012-3692
    DOI 10.1016/j.chest.2020.03.057
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  9. Article: Use of mepolizumab in adult patients with cystic fibrosis and an eosinophilic phenotype: case series.

    Zhang, Lijia / Borish, Larry / Smith, Anna / Somerville, Lindsay / Albon, Dana

    Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology

    2020  Volume 16, Page(s) 3

    Abstract: Background: Cystic fibrosis (CF) is characterized by inflammation, progressive lung disease, and respiratory failure. Although the relationship is not well understood, patients with CF are thought to have a higher prevalence of asthma than the general ... ...

    Abstract Background: Cystic fibrosis (CF) is characterized by inflammation, progressive lung disease, and respiratory failure. Although the relationship is not well understood, patients with CF are thought to have a higher prevalence of asthma than the general population. CF Foundation (CFF) annual registry data in 2017 reported a prevalence of asthma in CF of 32%. It is difficult to differentiate asthma from CF given similarities in symptoms and reversible obstructive lung function in both diseases. However, a specific asthma phenotype (type 2 inflammatory signature), is often identified in CF patients and this would suggest potential responsiveness to biologics targeting this asthma phenotype. A type 2 inflammatory condition is defined by the presence of an interleukin (IL)-4
    Results: We present three patients with CF (63, 34 and 24 year of age) and personal history of asthma, who displayed significant eosinophilic inflammation and high total serum IgE concentrations (type 2 inflammation) who were treated with mepolizumab. All three patients were colonized with multiple organisms including
    Conclusions: Mepolizumab appears to have a positive effect on clinical course in patients with CF presenting with a type 2 phenotype characterized by allergic sensitization and hyper-eosinophilia.
    Language English
    Publishing date 2020-01-06
    Publishing country England
    Document type Case Reports
    ZDB-ID 2434973-2
    ISSN 1710-1492 ; 1710-1484
    ISSN (online) 1710-1492
    ISSN 1710-1484
    DOI 10.1186/s13223-019-0397-3
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  10. Article ; Online: Prevalence and Risk Factors for Iron Deficiency in Adults With Cystic Fibrosis.

    Gettle, Lucy S / Harden, Adrienne / Bridges, Meagan / Albon, Dana

    Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition

    2020  Volume 35, Issue 6, Page(s) 1101–1109

    Abstract: Background: Iron deficiency is common in cystic fibrosis (CF), but previous prevalence studies often reported results confounded by acute exacerbations. This single-center retrospective study aimed to identify the prevalence of iron deficiency in a ... ...

    Abstract Background: Iron deficiency is common in cystic fibrosis (CF), but previous prevalence studies often reported results confounded by acute exacerbations. This single-center retrospective study aimed to identify the prevalence of iron deficiency in a stable adult CF population, identify the risk factors associated with iron deficiency, and compare common laboratory indicators of iron status.
    Methods: Medical charts of 105 patients aged 18-67 were reviewed to determine the prevalence of anemia. Of these patients, a subgroup of 67 were included in analyses of iron deficiency, defined as serum ferritin < 12 ng/mL and/or percent transferrin saturation (TSAT) < 16%. Data on sex, age, body mass index, anemia status, vitamin deficiencies, presence of comorbidities, colonization with Pseudomonas aeruginosa, and use of acid blockers and CF transmembrane conductance regulator modulators were collected to evaluate relationship of iron deficiency with these clinical factors. κ agreements between serum iron, ferritin, transferrin, and TSAT were compared.
    Results: In this stable CF population, the prevalence of iron deficiency was 41.8% (n = 67), and the prevalence of anemia was 33.3% (n = 105). Iron deficiency was associated with presence of anemia (P < .001), vitamin A deficiency (P = .012), and moderate (P = .047) and severe lung disease (P = .045) compared with mild lung disease. Transferrin agreed poorly with other iron status indicators.
    Conclusion: Iron deficiency is common in CF, although prevalence rates can vary widely depending on the laboratory parameters used. CF centers should consider routine screening for iron deficiency.
    MeSH term(s) Adult ; Anemia, Iron-Deficiency/complications ; Anemia, Iron-Deficiency/epidemiology ; Cystic Fibrosis/complications ; Hemoglobins/analysis ; Humans ; Prevalence ; Retrospective Studies ; Risk Factors
    Chemical Substances Hemoglobins
    Language English
    Publishing date 2020-01-29
    Publishing country United States
    Document type Journal Article
    ZDB-ID 645074-x
    ISSN 1941-2452 ; 0884-5336
    ISSN (online) 1941-2452
    ISSN 0884-5336
    DOI 10.1002/ncp.10454
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