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Cystic FibrosisMUCO-PNNNCT04970225

Cystic Fibrosis Blood Neutrophils

A study testing 1 blood sample and 2 blood samples in people with Cystic Fibrosis, run by Assistance Publique - Hôpitaux de Paris at 1 site in France.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
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Trial summary

The purpose of this prospective study is to analyze function and phenotype of blood neutrophils in cystic fibrosis patients and the impact of Pseudomonas aeruginosa chronic infection, treatment with CFTR modulators and acute exacerbation on blood neutrophils phenotype and function. Read more about this study

Who is it for?
Ages 18 years and older
Conditions the study is for
Cystic Fibrosis
What is being tested?
1 blood sample and 2 blood samples
Could I receive a placebo?
No, everyone receives an active treatment
How many people
47 participants
Where
1 siteFrance
Study dates
8 Jul 2021to 8 Jan 2025
Phase
Interventional
Are there results?
None posted in the registry

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 18 years and older
  • All sexes
  • No healthy volunteers
  • Adult, Older adult

Who may be able to join

  • Patient over the age of 18 who is not under legal protection
  • Patients with CF according to the diagnostic criteria of the Cystic Fibrosis Foundation including:
  • 15 patients with severe mutation but not chronically infected with PA and not treated with lumacaftor / ivacaftor
  • 15 patients homozygous phe508del, chronically infected with PA and not treated with lumacaftor / ivacaftor
  • 15 patients homozygous phe508del, chronically infected with PA and treated with lumacaftor / ivacaftor
  • 15 hospitalized patients for respiratory exacerbation
Show all 10Show fewer
  • 40 patients initiating Ivacaftor-Tezacaftor-Elexacaftor treatment.
  • No change in baseline treatment for 15 days (including antibiotic treatment).
  • Patient affiliated to a social security system
  • Free, informed and written consent, dated and signed by the patient and the investigator, at the latest on the day of inclusion and before any action required by the study.

Who cannot join

  • Informed consent impossible to obtain
  • Involvement in an interventional research protocol in the previous 3 months if exclusion directive was given in this protocol.

Trial locations and contacts

1 site in 1 country.

France1 site
  • Cochin hospital, AP-HP

    Paris, 75014

Other trials in France

About this study

Official title: Functional and Phenotypic Characteristics of Blood Neutrophils in Cystic Fibrosis

The purpose of this prospective study is to analyze function and phenotype of blood neutrophils in cystic fibrosis patients and the impact of Pseudomonas aeruginosa chronic infection, treatment with CFTR modulators and acute exacerbation on blood neutrophils phenotype and function.

Conditions
Cystic Fibrosis
Being tested
1 blood sample, 2 blood samples
Keywords
Blood neutrophils, Pseudomonas aeruginosa, Cystic fibrosis, CFTR modulators, respiratory exacerbation, airway chronic infection
Medical subject headings
Cystic Fibrosis, Pseudomonas Infections

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • 1 blood sampleOther

    4 tubes of 7 ml per sample (a single sample)

  • 2 blood samplesOther

    4 tubes of 7 ml per sample / 2 samples : before / after antibiotic treatment

Study groups in NCT04970225
Study groupRoleWhat they receive
Stable stateExperimental45 adult cystic fibrosis patients followed in the respiratory medicine department of Cochin hospital, paris, France: * With severe cftr mutations * With or without PA chronic infection * Treated or not with Ivacaftor-Lumacaftor
Receives: Other: 1 blood sample
Starting Ivacaftor-Tezacaftor-ElexacaftorExperimental40 adult cystic fibrosis patients followed in the respiratory medicine department of Cochin hospital, paris, France: * With at least one severe cftr mutation * With or without PA chronic infection * Initiating Ivacaftor-Tezacaftor-Elexacaftor
Receives: Other: 2 blood samples
ExacerbationExperimental15 adult cystic fibrosis patients followed in the respiratory medicine department of Cochin hospital, paris, France and hospitalized for respiratory exacerbation
Receives: Other: 2 blood samples

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Quantification of blood low density neutrophils in cystic fibrosis (CF)

    Quantitative flow cytometric evaluation of neutrophil membrane markers that differ according to whether they are :

    • mature neutrophils : CD16high, CD15high, CD33high, CD10high
    • or Low density neutrophils (LDG) : CD16low, CD15neg, CD33 neg, CD10 neg. Comparison of neutrophil membrane markers profile in CF patients versus control blood-donors.

    Measured: Through study completion, an average of 1 year

  2. Transcriptomic analysis of blood neutrophils in cystic fibrosis (CF)

    Transcriptomic analysis of blood neutrophil proinflammatory or immunomodulatory potential.

    Comparison of transcriptomic blood neutrophil profile in CF patients versus control blood-donors.

    Measured: Through study completion, an average of 1 year

  3. Effector function analysis of blood neutrophils in CF.

    Comparison of effector function analysis of blood neutrophils of CF patients versus control blood-donors.

    Measured: Through study completion, an average of 1 year

  4. Phagocytosis potential of blood neutrophils in CF.

    Comparison of phagocytosis potential of blood neutrophils of CF patients versus control blood-donors.

    Measured: Through study completion, an average of 1 year

  5. Survival/apoptosis balance analysis in CF

    Survival / apoptosis balance analysis : (PCNA localization) in CF patients blood neutrophils versus control blood-donors.

    Measured: Through study completion, an average of 1 year

Secondary outcomes (6)
  1. Quantification of blood low density neutrophils in CF vs other chronic inflammatory disorders.

    Quantitative flow cytometric evaluation of neutrophil membrane markers that differ according to whether they are:

    • mature neutrophils : CD16high, CD15high, CD33high, CD10high
    • Low density neutrophils (LDG) : CD16low, CD15neg, CD33 neg, CD10 neg. Comparison of neutrophil membrane markers profile in CF patients versus patients with other chronic inflammatory disease (eg. Rheumatoid arthritis, inflammatory bowel disease).

    Measured: Through study completion, an average of 1 year

  2. Quantification of blood low density neutrophils in CF according to different clinical situations.

    Quantitative flow cytometric evaluation of neutrophil membrane markers.

    • At stable state:
    • presence or absence of airway chronic infection with PA
    • whether or not patients are treated Ivacaftor-Lumacaftor
    • At beginning of exacerbation or after antibiotic treatment.
    • Before or after Ivacaftor-Tezacaftor-Elexacaftor treatment

    Measured: Through study completion, an average of 1 year

  3. Transcriptomic analysis of blood neutrophils in CF according to different clinical situations.

    Transcriptomic analysis of blood neutrophil proinflammatory or immunomodulatory potential.

    • At stable state:
    • presence or absence of airway chronic infection with PA
    • whether or not patients are treated Ivacaftor-Lumacaftor
    • At beginning of exacerbation or after antibiotic treatment.
    • Before or after Ivacaftor-Tezacaftor-Elexacaftor treatment

    Measured: Through study completion, an average of 1 year

  4. Effector function analysis of blood neutrophils in CF according to different clinical situations.

    Effector function analysis of blood neutrophils:

    • At stable state:
    • presence or absence of airway chronic infection with PA
    • whether or not patients are treated Ivacaftor-Lumacaftor
    • At beginning of exacerbation or after antibiotic treatment.
    • Before or after Ivacaftor-Tezacaftor-Elexacaftor treatment

    Measured: Through study completion, an average of 1 year

  5. Phagocytosis potential of blood neutrophils in CF according to different clinical situations.

    Phagocytosis potential analysis of blood neutrophils in CF:

    • At stable state:
    • presence or absence of airway chronic infection with PA
    • whether or not patients are treated Ivacaftor-Lumacaftor
    • At beginning of exacerbation or after antibiotic treatment.
    • Before or after Ivacaftor-Tezacaftor-Elexacaftor treatment

    Measured: Through study completion, an average of 1 year

  6. Survival/apoptosis balance analysis in CF according to different clinical situations.

    Survival / apoptosis balance analysis (PCNA localization):

    • At stable state:
    • presence or absence of airway chronic infection with PA
    • whether or not patients are treated Ivacaftor-Lumacaftor
    • At beginning of exacerbation or after antibiotic treatment.
    • Before or after Ivacaftor-Tezacaftor-Elexacaftor treatment.

    Measured: Through study completion, an average of 1 year

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted21 Jul 2021
  2. Study start8 Jul 2021
  3. Primary completion6 Apr 2023
  4. Study completion8 Jan 2025

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase N/A
Primary purpose
Basic science
Allocation
Non-randomised
Intervention model
Parallel groups
Masking
None (open label)
Enrolment
47 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT04970225
Sponsor's protocol ID
APHP180430
Other IDs
2018-A03017-48
Acronym
MUCO-PNN
Status last verified
Sep 2025
First submitted
17 Jan 2019
Last updated
12 Sep 2025
Results posted
No
Data monitoring committee
No
FDA-regulated drug
No
FDA-regulated device
No
Individual data sharing
No

Publications

  • Adib-Conquy M, Pedron T, Petit-Bertron AF, Tabary O, Corvol H, Jacquot J, Clement A, Cavaillon JM. Neutrophils in cystic fibrosis display a distinct gene expression pattern. Mol Med. 2008 Jan-Feb;14(1-2):36-44. doi: 10.2119/2007-00081.Adib-Conquy.

    PubMed 18026571
  • Bonvillain RW, Painter RG, Adams DE, Viswanathan A, Lanson NA Jr, Wang G. RNA interference against CFTR affects HL60-derived neutrophil microbicidal function. Free Radic Biol Med. 2010 Dec 15;49(12):1872-80. doi: 10.1016/j.freeradbiomed.2010.09.012. Epub 2010 Sep 24.

    PubMed 20870018
  • Bouayad D, Pederzoli-Ribeil M, Mocek J, Candalh C, Arlet JB, Hermine O, Reuter N, Davezac N, Witko-Sarsat V. Nuclear-to-cytoplasmic relocalization of the proliferating cell nuclear antigen (PCNA) during differentiation involves a chromosome region maintenance 1 (CRM1)-dependent export and is a prerequisite for PCNA antiapoptotic activity in mature neutrophils. J Biol Chem. 2012 Sep 28;287(40):33812-25. doi: 10.1074/jbc.M112.367839. Epub 2012 Jul 30.

    PubMed 22846997
  • Bruijnzeel PL, Uddin M, Koenderman L. Targeting neutrophilic inflammation in severe neutrophilic asthma: can we target the disease-relevant neutrophil phenotype? J Leukoc Biol. 2015 Oct;98(4):549-56. doi: 10.1189/jlb.3VMR1214-600RR. Epub 2015 May 14.

    PubMed 25977288
  • Bulloch MN, Hanna C, Giovane R. Lumacaftor/ivacaftor, a novel agent for the treatment of cystic fibrosis patients who are homozygous for the F580del CFTR mutation. Expert Rev Clin Pharmacol. 2017 Oct;10(10):1055-1072. doi: 10.1080/17512433.2017.1378094. Epub 2017 Sep 22.

    PubMed 28891346
  • Chiara AD, Pederzoli-Ribeil M, Burgel PR, Danel C, Witko-Sarsat V. Targeting cytosolic proliferating cell nuclear antigen in neutrophil-dominated inflammation. Front Immunol. 2012 Oct 9;3:311. doi: 10.3389/fimmu.2012.00311. eCollection 2012.

    PubMed 23181059
  • Cantin A. Cystic fibrosis lung inflammation: early, sustained, and severe. Am J Respir Crit Care Med. 1995 Apr;151(4):939-41. doi: 10.1164/ajrccm.151.4.7697269. No abstract available.

    PubMed 7697269
  • Dibbert B, Weber M, Nikolaizik WH, Vogt P, Schoni MH, Blaser K, Simon HU. Cytokine-mediated Bax deficiency and consequent delayed neutrophil apoptosis: a general mechanism to accumulate effector cells in inflammation. Proc Natl Acad Sci U S A. 1999 Nov 9;96(23):13330-5. doi: 10.1073/pnas.96.23.13330.

    PubMed 10557320
  • Downey DG, Bell SC, Elborn JS. Neutrophils in cystic fibrosis. Thorax. 2009 Jan;64(1):81-8. doi: 10.1136/thx.2007.082388.

    PubMed 19103874
  • Fadok VA, Bratton DL, Henson PM. Phagocyte receptors for apoptotic cells: recognition, uptake, and consequences. J Clin Invest. 2001 Oct;108(7):957-62. doi: 10.1172/JCI14122. No abstract available.

    PubMed 11581295
  • Futosi K, Mocsai A. Tyrosine kinase signaling pathways in neutrophils. Immunol Rev. 2016 Sep;273(1):121-39. doi: 10.1111/imr.12455.

    PubMed 27558332
  • Gilroy DW, Lawrence T, Perretti M, Rossi AG. Inflammatory resolution: new opportunities for drug discovery. Nat Rev Drug Discov. 2004 May;3(5):401-16. doi: 10.1038/nrd1383. No abstract available.

    PubMed 15136788
  • Hayes E, Pohl K, McElvaney NG, Reeves EP. The cystic fibrosis neutrophil: a specialized yet potentially defective cell. Arch Immunol Ther Exp (Warsz). 2011 Apr;59(2):97-112. doi: 10.1007/s00005-011-0113-6. Epub 2011 Feb 11.

    PubMed 21311988
  • Khan TZ, Wagener JS, Bost T, Martinez J, Accurso FJ, Riches DW. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 1995 Apr;151(4):1075-82. doi: 10.1164/ajrccm/151.4.1075.

    PubMed 7697234
  • Koenderman L, Chilvers ER. Future treatment in patients with chronic obstructive pulmonary disease: to reverse or not to reverse steroid resistance-that is the question. J Allergy Clin Immunol. 2014 Aug;134(2):323-4. doi: 10.1016/j.jaci.2014.04.030. Epub 2014 Jun 13. No abstract available.

    PubMed 24934274

Questions about this trial

What is this clinical trial studying?

A study testing 1 blood sample and 2 blood samples in people with Cystic Fibrosis, run by Assistance Publique - Hôpitaux de Paris at 1 site in France.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 10 inclusion and 2 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 12 Sep 2025. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 1 site in France. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

Are results available for this study?

The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Quantification of blood low density neutrophils in cystic fibrosis (CF) (time frame: Through study completion, an average of 1 year). The study lists 5 primary outcomes in all. It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT04970225, which also lists 44 linked publications.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 12 Sep 2025.

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