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Cited by 101Cystic Fibrosis Research AdvancesInhalation and Respiratory Drug DeliveryChronic Obstructive Pulmonary Disease (COPD) Research

Dornase alfa for cystic fibrosis

Open access
Yang, Connie, Montgomery, Mark·2018·Cochrane Database of Systematic Reviews
DOI 10.1002/14651858.cd001127.pub4PMC PMC6513278
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Abstract

BACKGROUND: Dornase alfa is currently used as a mucolytic to treat pulmonary disease (the major cause of morbidity and mortality) in cystic fibrosis. It reduces mucus viscosity in the lungs, promoting improved clearance of secretions. This is an update of a previously published review. OBJECTIVES: To determine whether the use of dornase alfa in cystic fibrosis is associated with improved mortality and morbidity compared to placebo or other medications that improve airway clearance, and to identify any adverse events associated with its use. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and abstracts from conferences. Date of the most recent search of the Group's Cystic Fibrosis Register: 23 April 2018.Clinicaltrials.gov and the International Clinical Trials Registry Platform were also searched to identify unpublished or ongoing trials. Date of most recent search: 07 June 2018. SELECTION CRITERIA: All randomised and quasi-randomised controlled trials comparing dornase alfa to placebo, standard therapy or other medications that improve airway clearance. DATA COLLECTION AND ANALYSIS: Authors independently assessed trials against the inclusion criteria; two authors carried out analysis of methodological quality and data extraction. GRADE was used to assess the level of evidence. MAIN RESULTS: The searches identified 69 trials, of which 19 (2565 participants) met our inclusion criteria. Fifteen trials compared dornase alfa to placebo or no dornase alfa (2447 participants); two compared daily dornase to hypertonic saline (32 participants); one compared daily dornase alfa to hypertonic saline and alternate day dornase alfa (48 participants); one compared dornase alfa to mannitol and the combination of both drugs (38 participants). Trial duration varied from six days to three years.Dornase alfa compared to placebo or no treatmentDornase alfa improved forced expiratory volume at one second at one month (four trials, 248 participants), three months (one trial, 320 participants; moderate-quality evidence), six months (one trial, 647 participants; high-quality evidence) and two years (one trial, 410 participants). Limited low-quality evidence showed no difference between groups for changes in quality of life. There was a decrease in pulmonary exacerbations with dornase alfa in trials of up to two years (moderate-quality evidence). One trial that examined the cost of care, including the cost of dornase alfa, found that the cost savings from dornase alfa offset 18% to 38% of the medication costs.Dornase alfa: daily versus alternate dayOne cross-over trial (43 children) found no differences between treatment regimens for lung function, quality of life or pulmonary exacerbations (low-quality evidence).Dornase alfa compared to other medications that improve airway clearanceResults for these comparisons were mixed. One trial (43 children) showed a greater improvement in forced expiratory volume at one second for dornase alfa compared to hypertonic saline (low-quality evidence), and one trial (23 participants) reported no difference in lung function between dornase alfa and mannitol or dornase alfa and dornase alfa plus mannitol (low-quality evidence). One trial (23 participants) found a difference in quality of life favouring dornase alfa when compared to dornase alfa plus mannitol (low-quality evidence); other comparisons found no difference in this outcome (low-quality evidence). No trials in any comparison reported any difference between groups in the number of pulmonary exacerbations (low-quality evidence).When all comparisons are assessed, dornase alfa did not cause significantly more adverse effects than other treatments, except voice alteration and rash. AUTHORS' CONCLUSIONS: There is evidence to show that, compared with placebo, therapy with dornase alfa improves lung function in people with cystic fibrosis in trials lasting from one month to two years. There was a decrease in pulmonary exacerbations in trials of six months or longer. Voice alteration and rash appear to be the only adverse events reported with increased frequency in randomised controlled trials. There is not enough evidence to firmly conclude if dornase alfa is superior to other hyperosmolar agents in improving lung function.

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  • As lung disease is the primary cause of morbidity and mortality in people with CF, nebulised medications (or inhaled therapies) are usually prescribed, with multiple trials demonstrating their efficacy.

    “As lung disease is the primary cause of morbidity and mortality in people with CF, nebulised medications (or inhaled therapies) are usually prescribed (ECFS Patient Registry 2017), with multiple trials demonstrating their efficacy (Smith 2018; Yang 2018).”

    From Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · cites this paper as Yang 2018

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  1. Two additional cross-over trials compared dornase alfa and hypertonic saline, no significant differences were found between the treatments for % change in FEV 1 and other primary outcomes of the review were not recorded in these trials (Adde 2004; Ballmann 2002).
  2. Higgins JPT, Altman DG on behalf of the Cochrane Statistical Methods Group and the Cochrane Bias Methods Group (editors). Chapter 8: Assessing risk of bias in included studies. In: Higgins JPT, Green S (editors). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 [updated March 2011]. The Cochrane Collaboration, 2011. Available from www.cochrane-handbook.org.
  3. Kearney CE, Wallis CE. Deoxyribonuclease for cystic fibrosis. Cochrane Database of Systematic Reviews 1998, Issue 4. [DOI: 10.1002/14651858.CD001127] * Indicates the major publication for the study · DOI

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Authors
Yang, C., & Montgomery, M.
Affiliations
  • BC Children's Hospital (CA)
  • Alberta Children's Hospital (CA)
ORCID
Yang, Connie
Venue
Cochrane Database of Systematic Reviews
Publisher
Wiley
Published
2018-09-06
Volume
9
Pages
CD001127-CD001127
ISSN
  • 1361-6137 (ISSN-L)
  • 1465-1858
DOI
10.1002/14651858.cd001127.pub4
PMC
PMC6513278
OpenAlex
W4211109516
Cited by
101
References
79
Citations by year
2026: 2 · 2025: 4 · 2024: 2 · 2023: 15 · 2022: 12 · 2021: 22 · 2020: 28 · 2019: 12 · 2018: 3 · 2016: 1
FWCI
2.90
Type
journal article
Language
en
Update notices
new_version (2021-03-18)
Open access
Locations
  • Cochrane Database of Systematic Reviews (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Inhalation and Respiratory Drug Delivery
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
Keywords
dornase alfa, cystic fibrosis, mucolytic therapy, airway clearance, pulmonary exacerbations, lung function, FEV1, quality of life, adverse events, hypertonic saline, mannitol, morbidity, drug safety, combination therapy, placebo comparison, alternate-day therapy, bronchiectasis
MeSH terms
Adolescent, Child, Child, Preschool, Cystic Fibrosis, Deoxyribonuclease I, Expectorants, Forced Expiratory Volume, Humans, Infant, Mannitol, Recombinant Proteins, Saline Solution, Hypertonic, Vital Capacity, Randomized Controlled Trials as Topic

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