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  1. Article ; Online: Corrigendum to FEV1 Variability Helps Identify Patients with Cystic Fibrosis at Risk of Greater Loss of Lung Function [The Journal of Pediatrics (2016) 116-121].

    Morgan, Wayne J / VanDevanter, Donald R / Pasta, David J / Foreman, Aimee J / Wagener, Jeffrey S / Konstan, Michael W

    The Journal of pediatrics

    2023  Volume 255, Page(s) 265

    Language English
    Publishing date 2023-01-17
    Publishing country United States
    Document type Published Erratum
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2022.12.017
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  2. Article ; Online: Recombinant Human Deoxyribonuclease I

    Lazarus, Robert A. / Wagener†, Jeffrey S.

    Pharmaceutical Biotechnology

    Abstract: Human deoxyribonuclease I (DNase I) is an endonuclease that catalyzes the hydrolysis of extracellular DNA and is just one of the numerous types of nucleases found in nature. The enzymatic mechanism for a single turnover is reasonably well understood ... ...

    Abstract Human deoxyribonuclease I (DNase I) is an endonuclease that catalyzes the hydrolysis of extracellular DNA and is just one of the numerous types of nucleases found in nature. The enzymatic mechanism for a single turnover is reasonably well understood based on biochemical and structural studies that are consistent with divalent metal ion dependent nonspecific nicking of a phosphodiester bond in one of the strands of double stranded DNA. Recombinant human DNase I (rhDNase I, rhDNase, Pulmozyme(®), dornase alfa) has been expressed in mammalian cell culture in Chinese hamster ovary cells and developed clinically where it is aerosolized into the airways for treatment of pulmonary disease in patients with cystic fibrosis (CF). rhDNase I hydrolyzes the DNA in purulent sputum of CF patients and reduces sputum viscoelasticity. Reduction of high molecular weight DNA into smaller fragments by treatment with aerosolized rhDNase I has been proposed as the mechanism to reduce the mucus viscosity and improve mucus clearability from obstructed airways in patients. The improved clearance of the purulent mucus enhances pulmonary function and reduces recurrent exacerbations of respiratory symptoms. rhDNase I was approved for clinical use in 1993 and has been widely used as a safe and effective therapy for CF patients. The use of rhDNase I has also been investigated in other diseases where exogenous DNA has been implicated in the disease pathology.
    Keywords covid19
    Publisher PMC
    Document type Article ; Online
    DOI 10.1007/978-3-030-00710-2_22
    Database COVID19

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  3. Article ; Online: Epidemiologic Study of Cystic Fibrosis: 25 years of observational research.

    Konstan, Michael W / Pasta, David J / VanDevanter, Donald R / Wagener, Jeffrey S / Morgan, Wayne J

    Pediatric pulmonology

    2021  Volume 56, Issue 5, Page(s) 823–836

    Abstract: ... Although ESCF data collection concluded in 2005, through a collaboration with the U.S. Cystic Fibrosis ...

    Abstract The Epidemiologic Study of Cystic Fibrosis (ESCF) was a prospective observational study of over 32,000 people with cystic fibrosis (CF) from 250 clinical care sites in North America from 1994 to 2005. Begun as a pharmacovigilance study in connection with the approval of dornase alfa in 1993, ESCF was open to all people with CF treated at any participating site in the United States or Canada. In addition to obtaining safety and effectiveness data on dornase alfa, ESCF collected encounter-based data to characterize the natural history and management of CF with a special focus on lung disease. During the study, 32,178 patients reported at least one encounter, contributing 869,136 encounters, 622,592 pulmonary function tests, 432,896 cultures, and 118,563 pulmonary exacerbations treated with intravenous antibiotics. Although ESCF data collection concluded in 2005, through a collaboration with the U.S. Cystic Fibrosis Foundation Patient Registry, additional follow-up data through 2017 was available for two-thirds of patients. This allowed for updating of CF genotype and survival information. Fifty-six peer-reviewed publications (cited over 3600 times) resulted from this study. In this manuscript we summarize the published ESCF manuscripts in thematic groups with key study findings and brief comments, and speculate on how ESCF findings will inform future data registries and patient care practices.
    MeSH term(s) Administration, Intravenous ; Cystic Fibrosis/drug therapy ; Cystic Fibrosis/epidemiology ; Deoxyribonuclease I/therapeutic use ; Humans ; Lung ; Observational Studies as Topic ; Prospective Studies ; Respiratory Function Tests ; United States/epidemiology
    Chemical Substances Deoxyribonuclease I (EC 3.1.21.1)
    Language English
    Publishing date 2021-01-12
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.25248
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  4. Article: Strengthening care teams to improve adherence in cystic fibrosis: a qualitative practice assessment and quality improvement initiative.

    Gardner, Allison J / Gray, Alice L / Self, Staci / Wagener, Jeffrey S

    Patient preference and adherence

    2017  Volume 11, Page(s) 761–767

    Abstract: Background: Treatment regimens for patients with cystic fibrosis (CF) are complex, time consuming, and burdensome, and adherence to CF treatment is suboptimal. CF care teams play a critical role in supporting patients' chronic self-management skills, ... ...

    Abstract Background: Treatment regimens for patients with cystic fibrosis (CF) are complex, time consuming, and burdensome, and adherence to CF treatment is suboptimal. CF care teams play a critical role in supporting patients' chronic self-management skills, but there is no uniform method for assessing patients' adherence to treatment or standard interventions to help patients improve when necessary.
    Methods: Between May 2015 and March 2016, care team members from 10 CF centers in the USA participated in a practice assessment and quality improvement (QI) initiative. The intervention included a baseline practice assessment survey, personalized continuing medical education (CME)-certified Webconferences with expert study faculty, targeted reinforcement of key practice points, and follow-up online survey and telephone interviews to evaluate the benefits and limitations of the intervention.
    Results: Responses to the baseline practice assessment survey were received from 50 multidisciplinary care team members representing 10 CF centers. Primary barriers to adherence-related aspects of care in their clinics were motivating patients and caregivers to improve adherence and obtaining accurate information about adherence from patients. At the conclusion of the initiative, participants reported improvements in communication within their care team, implementation of new approaches to asking about adherence, and a renewed commitment to asking patients and caregivers about adherence at each clinic visit.
    Conclusion: Structured QI interventions that bring multidisciplinary care teams together to reflect on clinic processes and elicit objective insights from outside faculty have the potential to improve practice patterns related to the assessment and improvement of patient adherence in CF.
    Language English
    Publishing date 2017-04-10
    Publishing country New Zealand
    Document type Journal Article
    ZDB-ID 2455848-5
    ISSN 1177-889X
    ISSN 1177-889X
    DOI 10.2147/PPA.S130439
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  5. Article ; Online: Dornase alfa (Pulmozyme).

    Wagener, Jeffrey S / Kupfer, Oren

    Current opinion in pulmonary medicine

    2012  Volume 18, Issue 6, Page(s) 609–614

    Abstract: Purpose of review: Dornase alfa (Pulmozyme) is one of the most commonly used medications to treat the lung disease of cystic fibrosis (CF). As other respiratory medications have entered the clinical market, understanding the proper use and indication ... ...

    Abstract Purpose of review: Dornase alfa (Pulmozyme) is one of the most commonly used medications to treat the lung disease of cystic fibrosis (CF). As other respiratory medications have entered the clinical market, understanding the proper use and indication for dornase alfa is increasingly important. In addition, dornase alfa is being used to treat other medical conditions. This review covers recent publications and expanding indications.
    Recent findings: Understanding dornase alfa's mechanism of action and impact on the pathophysiology of CF leads to an improved understanding of optimal therapy, ways to improve adherence and use with other medications. Most importantly, routine use of dornase alfa is associated with improved lung function and survival in patients with CF. Outside of CF, potential uses include treating patients with empyema or on mechanical ventilation.
    Summary: Dornase alfa has been available for clinical use for nearly 20 years and is one of the most commonly used medications in patients with CF. Routine use is associated with a reduced rate of pulmonary deterioration and improved survival. Recent clinical reports suggest that dornase alfa may have clinical value with other medical problems such as complicated pneumonia and mechanically ventilated patients with atelectasis.
    MeSH term(s) Cystic Fibrosis/drug therapy ; Deoxyribonuclease I/pharmacology ; Deoxyribonuclease I/therapeutic use ; Humans ; Recombinant Proteins/pharmacology ; Recombinant Proteins/therapeutic use
    Chemical Substances Recombinant Proteins ; Deoxyribonuclease I (EC 3.1.21.1) ; dornase alfa (EC 3.1.21.1)
    Language English
    Publishing date 2012-11
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1285505-4
    ISSN 1531-6971 ; 1070-5287 ; 1078-1641
    ISSN (online) 1531-6971
    ISSN 1070-5287 ; 1078-1641
    DOI 10.1097/MCP.0b013e328358d51f
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4900: Show issues Location:
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  6. Article ; Online: Lung function changes before and after pulmonary exacerbation antimicrobial treatment in cystic fibrosis.

    Wagener, Jeffrey S / VanDevanter, Donald R / Konstan, Michael W / Pasta, David J / Millar, Stefanie J / Morgan, Wayne J

    Pediatric pulmonology

    2019  Volume 55, Issue 3, Page(s) 828–834

    Abstract: ... forced expiratory volume in 1 s [FEV: Methods: We analyzed pre- and posttreatment ppFEV: Results: Of 15 147 PEx, 10 ...

    Abstract Background: In cystic fibrosis, observation of a lung function drop (as percent predicted forced expiratory volume in 1 s [FEV
    Methods: We analyzed pre- and posttreatment ppFEV
    Results: Of 15 147 PEx, 10 166 (67.1%), 3479 (23.0%), and 1502 (9.9%) presented with a ≥10%, <10%, or no ppFEV
    Conclusions: Among the 10% of PEx presenting with no apparent ppFEV
    MeSH term(s) Adolescent ; Adult ; Anti-Infective Agents/therapeutic use ; Child ; Cystic Fibrosis/drug therapy ; Cystic Fibrosis/physiopathology ; Disease Progression ; Female ; Forced Expiratory Volume ; Humans ; Lung/physiopathology ; Male ; Young Adult
    Chemical Substances Anti-Infective Agents
    Language English
    Publishing date 2019-11-20
    Publishing country United States
    Document type Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.24577
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    Zs.A 2143: Show issues Location:
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  7. Article ; Online: Frequency and costs of pulmonary exacerbations in patients with cystic fibrosis in the United States.

    Rubin, Jaime L / Thayer, Sarah / Watkins, Angela / Wagener, Jeffrey S / Hodgkins, Paul S / Schechter, Michael S

    Current medical research and opinion

    2017  Volume 33, Issue 4, Page(s) 667–674

    Abstract: Background: Information is limited regarding the cost of pulmonary exacerbations (PEx) among patients with cystic fibrosis in the United States.: Methods: To examine PEx costs, medical chart data were linked to insurance claims for patients aged ≥6 ... ...

    Abstract Background: Information is limited regarding the cost of pulmonary exacerbations (PEx) among patients with cystic fibrosis in the United States.
    Methods: To examine PEx costs, medical chart data were linked to insurance claims for patients aged ≥6 years who had commercial coverage from a large US health insurer affiliated with Optum during 2008-2013. A PEx was categorized as an episode requiring newly started (1) oral antibiotics (PEx-O) or (2) intravenous (IV) antibiotics and/or inpatient stay (PEx-IV).
    Results: Among 241 patients, 88.0% had ≥1 PEx (2.9/year) of any type, and 48.1% had ≥1 PEx-IV. Prior PEx-IV was the strongest risk factor for subsequent PEx-IV. The mean cost per episode was $12,784 for PEx of any type and $36,319 for PEx-IV. Patients with worse lung function were more likely to experience a PEx and incurred higher annual PEx-related costs.
    Limitations: This was an observational study using a convenience sample of patients with commercial coverage from a large US health insurer whose medical charts were available for abstraction. Results of the study may not be generalizable to individuals with Medicaid coverage and other types of insurance, or to the uninsured.
    Conclusions: Most patients experience ≥1 PEx annually, and nearly half require IV antibiotics and/or inpatient stay at considerable cost.
    MeSH term(s) Adolescent ; Adult ; Anti-Bacterial Agents/administration & dosage ; Anti-Bacterial Agents/economics ; Anti-Bacterial Agents/therapeutic use ; Child ; Cystic Fibrosis/complications ; Cystic Fibrosis/economics ; Cystic Fibrosis/epidemiology ; Female ; Humans ; Male ; Respiratory Tract Infections/complications ; Respiratory Tract Infections/drug therapy ; Risk Factors ; United States/epidemiology ; Young Adult
    Chemical Substances Anti-Bacterial Agents
    Language English
    Publishing date 2017-04
    Publishing country England
    Document type Journal Article ; Observational Study
    ZDB-ID 80296-7
    ISSN 1473-4877 ; 0300-7995
    ISSN (online) 1473-4877
    ISSN 0300-7995
    DOI 10.1080/03007995.2016.1277196
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  8. Article ; Online: Patients with Cystic Fibrosis and a G551D or Homozygous F508del Mutation: Similar Lung Function Decline.

    Sawicki, Gregory S / McKone, Edward F / Millar, Stefanie J / Pasta, David J / Konstan, Michael W / Lubarsky, Barry / Wagener, Jeffrey S

    American journal of respiratory and critical care medicine

    2017  Volume 195, Issue 12, Page(s) 1673–1676

    Language English
    Publishing date 2017-06-15
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1180953-x
    ISSN 1535-4970 ; 0003-0805 ; 1073-449X
    ISSN (online) 1535-4970
    ISSN 0003-0805 ; 1073-449X
    DOI 10.1164/rccm.201608-1678LE
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  9. Article ; Online: BMI fails to identify poor nutritional status in stunted children with CF.

    Konstan, Michael W / Pasta, David J / Wagener, Jeffrey S / VanDevanter, Donald R / Morgan, Wayne J

    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

    2016  Volume 16, Issue 1, Page(s) 158–160

    Abstract: Background: Body mass index (BMI) is currently emphasized for evaluating nutritional status in children with cystic fibrosis (CF). Weight for age (WFA) and height for age (HFA) may get less attention.: Methods: Data from the Epidemiologic Study of ... ...

    Abstract Background: Body mass index (BMI) is currently emphasized for evaluating nutritional status in children with cystic fibrosis (CF). Weight for age (WFA) and height for age (HFA) may get less attention.
    Methods: Data from the Epidemiologic Study of Cystic Fibrosis were used to compare patient WFA, HFA, and BMI percentiles for children age 2 to 18years.
    Results: For children with BMI between the 25th and 50th percentiles, 16.8% had WFA <10th percentile and 26.6% had HFA <10th percentile.
    Conclusions: BMI fails to identify a substantial proportion of children with CF who have stunting or potentially poor nutritional status as measured by WFA and/or HFA.
    MeSH term(s) Adolescent ; Age Factors ; Body Height ; Body Mass Index ; Body Weight ; Child ; Child, Preschool ; Cystic Fibrosis/complications ; Cystic Fibrosis/physiopathology ; Female ; Growth Disorders/diagnosis ; Growth Disorders/etiology ; Growth Disorders/physiopathology ; Humans ; Male ; Nutritional Status ; Reproducibility of Results
    Language English
    Publishing date 2016-12-01
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2091075-7
    ISSN 1873-5010 ; 1569-1993
    ISSN (online) 1873-5010
    ISSN 1569-1993
    DOI 10.1016/j.jcf.2016.11.005
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  10. Article ; Online: Newborn screening for cystic fibrosis.

    Wagener, Jeffrey S / Zemanick, Edith T / Sontag, Marci K

    Current opinion in pediatrics

    2012  Volume 24, Issue 3, Page(s) 329–335

    Abstract: Purpose of review: Newborn screening for cystic fibrosis (CF) is now universal in the US and many other countries. The rapid expansion of screening has resulted in numerous publications identifying new challenges for healthcare providers. This review ... ...

    Abstract Purpose of review: Newborn screening for cystic fibrosis (CF) is now universal in the US and many other countries. The rapid expansion of screening has resulted in numerous publications identifying new challenges for healthcare providers. This review provides an overview of these publications and includes ideas on managing these challenges.
    Recent findings: Most CF newborn screening algorithms involve DNA mutation analysis. As screening has expanded, new challenges have been identified related to carrier detection and inconclusive diagnoses. Early descriptions of infants with CF-related metabolic syndrome (CRMS) indicate that the natural history of this condition cannot be predicted. Early identification has also provided an opportunity to better understand the pathophysiology of CF. However, few studies have been conducted in infants with CF to determine optimal therapy and recommendations are largely anecdotal.
    Summary: Newborn screening provides an opportunity to identify and begin treatment early in individuals with CF. Whereas a single, optimal approach to screening does not exist, all programs can benefit from new findings regarding sweat testing, carrier detection, early pathophysiology, and clinical outcomes.
    MeSH term(s) Biomarkers/blood ; Cystic Fibrosis/diagnosis ; Cystic Fibrosis/therapy ; False Positive Reactions ; Genetic Carrier Screening ; Humans ; Infant, Newborn ; Neonatal Screening/methods ; Sweat/chemistry ; Trypsinogen/blood
    Chemical Substances Biomarkers ; Trypsinogen (9002-08-8)
    Language English
    Publishing date 2012-06
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1049374-8
    ISSN 1531-698X ; 1040-8703
    ISSN (online) 1531-698X
    ISSN 1040-8703
    DOI 10.1097/MOP.0b013e328353489a
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