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Meta-Analysis
. 2021 Mar 18;3(3):CD001127.
doi: 10.1002/14651858.CD001127.pub5.

Dornase alfa for cystic fibrosis

Connie Yang  1 , Mark Montgomery  2
Affiliations
Meta-Analysis

Dornase alfa for cystic fibrosis

Connie Yang et al. Cochrane Database Syst Rev. .

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: 12 October 2020. Clinicaltrials.gov and the International Clinical Trials Registry Platform were also searched to identify unpublished or ongoing trials. Date of most recent search: 08 February 2021.

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 74 trials, of which 19 (2565 participants) met our inclusion criteria. 15 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 treatment Dornase alfa probably improved forced expiratory volume at one second (FEV1) 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 treatment may make little or no difference in quality of life. Dornase alfa probably reduced the number of pulmonary exacerbations 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 day One cross-over trial (43 children) found little or no difference between treatment regimens for lung function, quality of life or pulmonary exacerbations (low-quality evidence). Dornase alfa compared to other medications that improve airway clearance Results for these comparisons were mixed. One trial (43 children) showed dornase alfa may lead to a greater improvement in FEV1 compared to hypertonic saline (low-quality evidence), and one trial (23 participants) reported little or no differences in lung function between dornase alfa and mannitol or dornase alfa and dornase alfa plus mannitol (low-quality evidence). One trial (23 participants) found dornase alfa may improve quality of life compared to dornase alfa plus mannitol (low-quality evidence); other comparisons found little or 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 may improve 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, probably due to treatment. 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.

Trial registration: ClinicalTrials.gov NCT00557089 NCT00117208 NCT00446680 NCT00416182 NCT04320381 NCT01712334 NCT04378153.

PubMed Disclaimer

Conflict of interest statement

Dr Connie Yang has no conflicts of interest.

Dr. Montgomery has no conflicts of interest.

Figures

1
1
Study flow diagram.
2
2
Risk of bias summary: review authors' judgements about each risk of bias item for each included study.
1.1
1.1. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 1: Relative mean % change in FEV1 (% predicted)
1.2
1.2. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 2: Relative mean % change in FEV1 (% predicted) at one month ‐ subgroup analysis by disease severity
1.3
1.3. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 3: Absolute mean % change in FEV1 (% predicted)
1.4
1.4. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 4: Absolute mean % change in FEV1 (% predicted)
1.5
1.5. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 5: Relative mean % change in FEV1 (in participants with acute exacerbations)
1.6
1.6. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 6: Relative mean % change in FVC (% predicted)
1.7
1.7. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 7: Relative mean % change in FVC (% predicted)
1.8
1.8. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 8: Relative mean % change in FVC at one month ‐ subgroup analysis by disease severity
1.9
1.9. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 9: Absolute mean % change in FVC (% predicted)
1.10
1.10. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 10: Absolute mean % change in FVC (% predicted)
1.11
1.11. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 11: Absolute mean change in LCI
1.12
1.12. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 12: Absolute change in FEV0.5 (z score)
1.13
1.13. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 13: Quality of life ‐ CFQ‐R respiratory
1.14
1.14. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 14: Quality of life ‐ CFQ‐R Parent respiratory
1.15
1.15. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 15: Number of people experiencing exacerbations
1.16
1.16. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 16: Number of deaths
1.17
1.17. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 17: Mean number of days of antibiotics (inhaled, oral or IV)
1.18
1.18. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 18: Mean number of days IV antibiotics used
1.19
1.19. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 19: Mean number of days inpatient treatment
1.20
1.20. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 20: Mean change in weight from baseline
1.21
1.21. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 21: Adverse event ‐ haemoptysis (blood‐stained sputum)
1.22
1.22. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 22: Adverse event ‐ dyspnoea (shortness of breath)
1.23
1.23. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 23: Adverse event ‐ pneumothorax
1.24
1.24. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 24: Adverse event ‐ pneumothorax (in participants with acute exacerbations)
1.25
1.25. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 25: Adverse event ‐ voice alteration
1.26
1.26. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 26: Adverse event ‐ voice alteration (1x versus 2x daily treatment)
1.27
1.27. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 27: Adverse event ‐ voice alteration (in participants with acute exacerbations)
1.28
1.28. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 28: Adverse event ‐ rash
1.29
1.29. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 29: Adverse event ‐ chest pain
1.30
1.30. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 30: Adverse event ‐ cough (new or increased)
1.31
1.31. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 31: Adverse event ‐ increased sputum production
1.32
1.32. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 32: Adverse event ‐ dry throat
1.33
1.33. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 33: Adverse event ‐ pharyngitis
1.34
1.34. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 34: Adverse event ‐ laryngitis
1.35
1.35. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 35: Adverse event ‐ conjunctivitis
1.36
1.36. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 36: Adverse event ‐ wheeze
1.37
1.37. Analysis
Comparison 1: Dornase alfa versus placebo, Outcome 37: Adverse event ‐ facial oedema
2.1
2.1. Analysis
Comparison 2: Dornase alfa once daily versus dornase alfa on alternate days, Outcome 1: Mean % change in FEV1
2.2
2.2. Analysis
Comparison 2: Dornase alfa once daily versus dornase alfa on alternate days, Outcome 2: Mean % change in FVC
2.3
2.3. Analysis
Comparison 2: Dornase alfa once daily versus dornase alfa on alternate days, Outcome 3: Mean % change in quality of life score
2.4
2.4. Analysis
Comparison 2: Dornase alfa once daily versus dornase alfa on alternate days, Outcome 4: Mean number of days inpatient treatment
2.5
2.5. Analysis
Comparison 2: Dornase alfa once daily versus dornase alfa on alternate days, Outcome 5: Mean change in weight (kg) from baseline
3.1
3.1. Analysis
Comparison 3: Dornase alfa daily versus hypertonic saline, Outcome 1: Mean % change in FEV1
3.2
3.2. Analysis
Comparison 3: Dornase alfa daily versus hypertonic saline, Outcome 2: Mean % change in FVC
3.3
3.3. Analysis
Comparison 3: Dornase alfa daily versus hypertonic saline, Outcome 3: Mean % change in quality of life score
3.4
3.4. Analysis
Comparison 3: Dornase alfa daily versus hypertonic saline, Outcome 4: Mean number of days inpatient treatment
3.5
3.5. Analysis
Comparison 3: Dornase alfa daily versus hypertonic saline, Outcome 5: Mean change in weight (kg) from baseline
4.1
4.1. Analysis
Comparison 4: Dornase alfa versus mannitol, Outcome 1: Mean absolute change in FEV1 (L)
4.2
4.2. Analysis
Comparison 4: Dornase alfa versus mannitol, Outcome 2: Mean absolute change in FVC (L)
4.3
4.3. Analysis
Comparison 4: Dornase alfa versus mannitol, Outcome 3: Quality of life ‐ CFQ‐R
4.4
4.4. Analysis
Comparison 4: Dornase alfa versus mannitol, Outcome 4: Number of people experiencing exacerbations
4.5
4.5. Analysis
Comparison 4: Dornase alfa versus mannitol, Outcome 5: Adverse events at 3 months
5.1
5.1. Analysis
Comparison 5: Dornase alfa versus dornase alfa and mannitol, Outcome 1: Mean absolute change in FEV1 (L)
5.2
5.2. Analysis
Comparison 5: Dornase alfa versus dornase alfa and mannitol, Outcome 2: Mean absolute change in FVC (L)
5.3
5.3. Analysis
Comparison 5: Dornase alfa versus dornase alfa and mannitol, Outcome 3: Quality of life ‐ CFQ‐R
5.4
5.4. Analysis
Comparison 5: Dornase alfa versus dornase alfa and mannitol, Outcome 4: Number of people experiencing exacerbations
5.5
5.5. Analysis
Comparison 5: Dornase alfa versus dornase alfa and mannitol, Outcome 5: Adverse events at 3 months
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References

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References to studies excluded from this review

Amelina 2019 {published and unpublished data}
    1. Amelina EL, Krasovskiy SA, Abdulganieva DI, Asherova IK, Zil'ber IE, Trishina SV, et al. Efficacy and safety of the biosimilar medicinal product Tigerase® (dornase alfa) in long-term symptomatic treatment of patients with cystic fibrosis: results of a phase III clinical trial. Pulmonologiya 2019;29(6):695-706. [CENTRAL: CN-02130241] [CFGD REGISTER: BD278] [CLINICALTRIALS.GOV: NCT04468100] [EMBASE: 2005791265]
Anderson 2009 {published data only}
    1. Anderson P, Morton J. Evaluation of two different timings of Pulmozyme nebulisation in relation to chest physiotherapy in children with cystic fibrosis. Journal of Cystic Fibrosis 2009;8 Suppl 2:S74. [ABSTRACT NO.: 299] [CFGD REGISTER: BD162]
Bakker 2010 {published data only}
    1. Bakker EM, Volpi S, Salonini E, Mullinger B, Kroneberg P, Hop WCJ, et al. Efficacy of peripheral deposition of inhaled rhDNase in CF patients during a respiratory tract infection. Journal of Cystic Fibrosis 2010;9 Supplement 1:S62. [ABSTRACT NO.: 239] [CFGD REGISTER: BD155a]
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Bilton 2011 {published data only}
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Bollert 1999 {published data only}
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    1. Bollert FG, Paton JY, Marshall TG, Calvert J, Greening AP, Innes JA. Recombinant DNase in cystic fibrosis: a protocol for targeted introduction through n-of-1 trials. Scottish Cystic Fibrosis Group. European Respiratory Journal 1999;13(1):107-13. - PubMed
Cimmino 2005 {published data only}
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    1. Malfroot A, Dab I, Baran D, App EM, Coffiner M, Nagy AM. Randomised multicentric double blind study of tolerability and efficacy of a DPI Nacystelyn versus placebo in cystic fibrosis patients treated by rhDNase for at least 3 months. In: 13th International Cystic Fibrosis Congress; 2000 June 4-8; Stockholm, Sweden. 2000:146.
    1. Malfroot A, Dab I, Baran D, App EM, Coffiner M, Nagy AM. Randomized multicentric double blind study of tolerability and efficacy of a DPI Nacystelyn versus placebo in cystic fibrosis patients treated by rhDNase for at least 3 months. Pediatric Pulmonology 1999;28 Suppl 19:244.
Elkins 2006 {published data only}
    1. Elkins MR, Bye PTP. Comparison of Pari LC-Star and -Plus nebulisers delivering 2.5mg recombinant human deoxyribonuclease (rhDNase). Journal of Cystic Fibrosis 2006;5 Suppl 1:S42.
EUCTR2006‐002098‐30‐NL {published data only}
    1. EUCTR2006-002098-30-NL. Efficacy of inhaled rhDNase in mechanically ventilated pediatric patients with an atelectasis - rhDNase in ventilated pediatric patients. apps.who.int/trialsearch/Trial2.aspx?TrialID=EUCTR2006-002098-30-NL (first received 01 June 2006).
EUCTR2007‐000935‐25‐NL {published data only}
    1. EUCTR2007-000935-25-NL. Peripheral targeting of inhaled rhDNase in stable CF patients. apps.who.int/trialsearch/Trial2.aspx?TrialID=EUCTR2007-000935-25-NL (first received 02 April 2007).
Fitzgerald 2005 {published data only}
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Furuya 2001 {published data only}
    1. Furuya ME, Lezana-Fernandez JL, Vargas MH, Hernandez-Sierra JF, Ramirez-Figueroa JL. Efficacy of human recombinant DNase in pediatric patients with cystic fibrosis. Archives of Medical Research 2001;32(1):30-4. - PubMed
Griese 1997 {published data only}
    1. Griese M, App EM, Derouix A, Burkert A, Schams A. Recombinant human DNase (rhDNase) influences phospholipid composition, surface activity, rheology and consecutively clearance indices of cystic fibrosis sputum. Pulmonary Pharmacology and Therapeutics 1997;10(1):21-7. - PubMed
Hagelberg 2008 {published data only}
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Heijerman 1995 {published data only}
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Hubbard 1992 {published data only}
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Johnson 2006 {published data only}
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Kelijo 2001 {published data only}
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King 1997 {published data only}
    1. King M, Dasgupta B, Tomkiewicz RP, Brown NE, Pulmonary Research Group. Rheology of cystic fibrosis sputum after in vitro treatment with hypertonic saline alone and in combination with recombinant human deoxyribonuclease I. American Journal of Respiratory and Critical Care Medicine 1997;156(1):173-7. - PubMed
Lahiri 2012 {published data only}
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Laube 2005 {published data only}
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Mainz 2011 {published data only}
    1. Mainz J, Mentzel H, Scheider G, Riethmuller J, Schiller I, Ritschel C, et al. Double-blind, placebo-controlled pilot trial on sinonasal inhalation of dornase alfa in CF. Pediatric Pulmonology 2008;43(Suppl 31):305. [CFGD REGISTER: BD131b] - PubMed
    1. Mainz J, Mentzel HJ, Schneider G, Riethmuller J, Schiller I, Ritschel C, et al. Sinu-nasal inhalation of Dornase alfa in CF. Results of a double-blind placebo-controlled pilot trial. Journal of Cystic Fibrosis 2008;7(Suppl 2):S27. [CFGD REGISTER: BD131a] - PubMed
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Mainz 2014 {published data only}
    1. EUCTR2007-001548-36-DE. Nasal inhalation of dornase alfa (pulmozyme) in patients with cystic fibrosis and chronic rhinosinusitis - pulmozyme-nasal-cf [Nasale Inhalation von Pulmozyme bei Patienten mit Mukoviszidose und chronischer Rhinosinusitis mit dem Pari Sinus-Vernebler. - bizentrische, randomisierte, doppel-blinde, placebo-kontrollierte, prospektive klinische Studie]. www.who.int/trialsearch/Trial2.aspx?TrialID=EUCTR2007-001548-36-DE (first received 27 July 2009). [CENTRAL: CN-01887259] [CFGD REGISTER: BD154e]
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    1. Mainz JG, Schiller I, Koitschev A, Koitschev C, Riethmuller J, Wiedemann B, et al. Sinonasal inhalation of dornase alfa reduces rhinosinusitis symptoms in CF. Results of a DBPC-cross-over-study. Journal of Cystic Fibrosis 2010;9 Suppl 1:S23. [ABSTRACT NO.: 88] [CENTRAL: CN-00766447] [CFGD REGISTER: BD154a]
    1. NCT00534079. Nasal inhalation of pulmozyme in patients with cystic fibrosis and chronic rhinosinusitis [Nasal inhalation of dornase alfa (pulmozyme) in patients with cystic fibrosis and chronic rhinosinusitis - a double blind placebo-controlled cross-over, bicenter, prospective clinical study]. clinicaltrials.gov/show/nct00534079 (first received 24 September 2007). [CENTRAL: CN-00757315] [CFGD REGISTER: BD154d]
Majaesic 1996 {published data only}
    1. Majaesic CM, Montgomery M, Jones R, King M. Reduction in sputum viscosity using high frequency chest compressions (HFCC) compared to conventional chest physiotherapy (CCP). Pediatric Pulmonology 1996;22 Suppl 13:308.
Nasr 2001 {published data only}
    1. Nasr SZ, Kuhns LR, Brown RW, Hurwitz ME, Sanders GM, Strouse PJ. Use of computerized tomography and chest-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than 5 years: A preliminary study. Pediatric Pulmology 2001;31(5):377-82. - PubMed
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NCT00311506 {published data only}
    1. NCT00311506. A study to determine the variability of a 6-minute walk test in cystic fibrosis subjects with advanced lung disease. clinicaltrials.gov/ct2/show/NCT00311506 (first posted 06 April 2006).
NCT00434278 {published data only}
    1. NCT00434278. A trial of pulmozyme withdrawal on exercise tolerance in cystic fibrosis subjects with severe lung disease (TOPIC). clinicaltrials.gov/ct2/show/NCT00434278 (first posted 13 February 2007).
NCT00680316 {published data only}
    1. NCT00680316. A study of Pulmozyme® (dornase alpha) in 3- to 5-year-old patients with cystic fibrosis. clinicaltrials.gov/ct2/show/NCT00680316 (first posted 20 May 2008).
NCT00843817 {published data only}
    1. NCT00843817. RhDNase and biodistribution of PMN serine proteases in cystic fibrosis sputum (BioDNase). clinicaltrials.gov/ct2/show/NCT00843817 (first posted 13 February 2009).
NCT01025258 {published data only}
    1. NCT01025258. Improving adherence and clinical outcome in cystic fibrosis (CF) patients. clinicaltrials.gov/ct2/show/NCT01025258 (first posted 03 December 2009).
NCT01155752 {published data only}
    1. NCT01155752. Pulmozyme in cystic fibrosis with sinusitis [Z4770s, Use of Recombinant Human DNASE in Cystic Fibrosis Patients With Chronic Sinusitis to Prevent Acute Sinusitis Exacerbations and Improve Symptoms and Outcomes - A Pilot Study]. clinicaltrials.gov/ct2/show/NCT01155752 (first posted 02 July 2010).
NCT01232478 {published data only}
    1. NCT01232478. I change adherence and raise expectations (iCARE). clinicaltrials.gov/ct2/show/NCT01232478 (first posted 02 November 2010).
NCT02301377 {published data only}
    1. NCT02301377. Exploring novel interventions to improve adherence in children with cystic fibrosis. clinicaltrials.gov/ct2/show/NCT02301377 (first posted 25 November 2014).
NCT02682290 {published data only}
    1. NCT02682290. Assessment of rheological parameters of human sputum (RHEOMUCO). clinicaltrials.gov/ct2/show/NCT02682290 (first posted 15 February 2016).
NCT02722122 {published data only}
    1. NCT02722122. Study to evaluate the safety,tolerability, pharmacokinetics and exploratory efficacy parameters of AIR DNase™ in patients with cystic fibrosis previously treated with Pulmozyme®. clinicaltrials.gov/ct2/show/NCT02722122 (first posted 29 March 2016).
Potter 2008 {published data only}
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QUEST {published data only}
    1. Qualitative understanding of experiences with the SIMPLIFY Trial (QUEST). clinicaltrials.gov/ct2/show/NCT04320381 first posted 25 March 2020.
Riethmueller 2006 {published data only}
    1. Riethmueller J, Borth-Bruhns T, Kumpf M, Vonthein R, Wiskirchen J, Stern M, et al. Recombinant human deoxyribonuclease shortens ventilation time in young, mechanically ventilated children. Pediatric Pulmonology 2006;41(1):61-6. - PubMed
Robinson 2002 {published data only}
    1. Robinson T, Goris ML, Bhise P, Sathi A, Zhu JH, Moss RB. Quantitative HRCT air trapping analysis in CF subjects with mild lung disease during a pulmozyme intervention study. Pediatric Pulmonology 2002;34 Suppl 24:298. [CFGD REGISTER: MU94]
Sawicki 2014 {published data only}
    1. Konstan MW, Chou W, Trzaskoma B, Xu Y. A study to evaluate the comparable efficacy and safety of Pulmozyme® delivered by the Erapid™ nebulizer system. Pediatric Pulmonology 2013;48 Suppl 36:452. [ABSTRACT NO.: 668] [CENTRAL: 999883] [CFGD REGISTER: BD219c]
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    1. Sawicki GS, Konstan M, Chou W, Trzaskoma B, Raimundo K. Impact of dornase alfa delivered by the eRapid™ vs. Pari LC® plus jet nebulizer system on cystic fibrosis-related quality of life - results from the IMPART Study. Pediatric Pulmonology 2014;49 Suppl 38:445-6. [ABSTRACT NO.: 621] [CENTRAL: 1012526] [CFGD REGISTER: BD219a]
    1. Sawicki GS, Konston M, Chou W, Trzaskoma B, Raimundo K. Treatment preferences and satisfaction with dornase alfa delivered by the eRapid™ vs. Pari LC® plus jet nebulizer system to patients with cystic fibrosis - results from the IMPART Study. Pediatric Pulmonology 2014;49 Suppl 38:444. [ABSTRACT NO.: 617] [CENTRAL: 1012527] [CFGD REGISTER: BD219b]
Shah 1995b {published data only}
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Shah 1995c {published data only}
    1. Shah PL, Scott SF, Fuchs HJ, Geddes DM, Hodson ME. Medium term treatment of stable stage cystic fibrosis with recombinant human DNase I. Thorax 1995;50(4):333-8. [CFGD REGISTER: BD127] - PMC - PubMed
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    1. Shah PL, Scott SF, Geddes DM, Conway S, Carr S, Wallis C, et al. An evaluation of two aerosol delivery systems for rhDNase [abstract]. Israel Journal of Medical Sciences 1996;32(Suppl):S222. [CFGD REGISTER: DT11a] - PubMed
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Tarrant 2019 {published data only}
    1. Tarrant B, Snell G, Ivulich S, Button B, Thompson B, Holland A. Dornase alfa during lower respiratory tract infection post lung transplantation. Respirology 2018;23(Supplement 1):50. [CENTRAL: CN-01607181] [CFGD REGISTER: BD279a] [EMBASE: 622091701] - PubMed
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ten Berge 2003 {published data only}
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van der Giessen 2007b {published data only}
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Wilson 2007 {published data only}
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References to ongoing studies

SIMPLIFY {published data only}
    1. Impact of discontinuing chronic therapies in people with cystic fibrosis on highly effective CFTR modulator therapy (SIMPLIFY). clinicaltrials.gov/ct2/show/NCT04378153 first posted 07 May 2020.
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