Cystic Fibrosis Blood Neutrophils (MUCO-PNN)

September 18, 2023 updated by: Assistance Publique - Hôpitaux de Paris

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.

Study Overview

Status

Recruiting

Conditions

Intervention / Treatment

Study Type

Interventional

Enrollment (Estimated)

130

Phase

  • Not Applicable

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years and older (Adult, Older Adult)

Accepts Healthy Volunteers

No

Description

Inclusion Criteria:

  • 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
    • 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.

Exclusion Criteria:

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Primary Purpose: Basic Science
  • Allocation: Non-Randomized
  • Interventional Model: Parallel Assignment
  • Masking: None (Open Label)

Arms and Interventions

Participant Group / Arm
Intervention / Treatment
Experimental: Stable state

45 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
Other: 1 blood sample
4 tubes of 7 ml per sample (a single sample)
Experimental: Starting Ivacaftor-Tezacaftor-Elexacaftor

40 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
Other: 2 blood samples
4 tubes of 7 ml per sample / 2 samples : before / after antibiotic treatment
Experimental: Exacerbation
15 adult cystic fibrosis patients followed in the respiratory medicine department of Cochin hospital, paris, France and hospitalized for respiratory exacerbation
Other: 2 blood samples
4 tubes of 7 ml per sample / 2 samples : before / after antibiotic treatment

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Quantification of blood low density neutrophils in cystic fibrosis (CF)
Time Frame: Through study completion, an average of 1 year

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.
Through study completion, an average of 1 year
Transcriptomic analysis of blood neutrophils in cystic fibrosis (CF)
Time Frame: Through study completion, an average of 1 year

Transcriptomic analysis of blood neutrophil proinflammatory or immunomodulatory potential.

Comparison of transcriptomic blood neutrophil profile in CF patients versus control blood-donors.
Through study completion, an average of 1 year
Effector function analysis of blood neutrophils in CF.
Time Frame: Through study completion, an average of 1 year
Comparison of effector function analysis of blood neutrophils of CF patients versus control blood-donors.
Through study completion, an average of 1 year
Phagocytosis potential of blood neutrophils in CF.
Time Frame: Through study completion, an average of 1 year
Comparison of phagocytosis potential of blood neutrophils of CF patients versus control blood-donors.
Through study completion, an average of 1 year
Survival/apoptosis balance analysis in CF
Time Frame: Through study completion, an average of 1 year
Survival / apoptosis balance analysis : (PCNA localization) in CF patients blood neutrophils versus control blood-donors.
Through study completion, an average of 1 year

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Quantification of blood low density neutrophils in CF vs other chronic inflammatory disorders.
Time Frame: Through study completion, an average of 1 year

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).
Through study completion, an average of 1 year
Quantification of blood low density neutrophils in CF according to different clinical situations.
Time Frame: Through study completion, an average of 1 year

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
Through study completion, an average of 1 year
Transcriptomic analysis of blood neutrophils in CF according to different clinical situations.
Time Frame: Through study completion, an average of 1 year

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
Through study completion, an average of 1 year
Effector function analysis of blood neutrophils in CF according to different clinical situations.
Time Frame: Through study completion, an average of 1 year

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
Through study completion, an average of 1 year
Phagocytosis potential of blood neutrophils in CF according to different clinical situations.
Time Frame: Through study completion, an average of 1 year

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
Through study completion, an average of 1 year
Survival/apoptosis balance analysis in CF according to different clinical situations.
Time Frame: Through study completion, an average of 1 year

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.
Through study completion, an average of 1 year

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Assistance Publique - Hôpitaux de Paris

Collaborators

Institut National de la Santé Et de la Recherche Médicale, France

Investigators

  • Principal Investigator: Pierre-Régis BURGEL, MD PHD, APHP

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

July 8, 2021

Primary Completion (Estimated)

July 1, 2024

Study Completion (Estimated)

October 1, 2024

Study Registration Dates

First Submitted

January 17, 2019

First Submitted That Met QC Criteria

July 9, 2021

First Posted (Actual)

July 21, 2021

Study Record Updates

Last Update Posted (Actual)

September 21, 2023

Last Update Submitted That Met QC Criteria

September 18, 2023

Last Verified

September 1, 2023

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • APHP180430
  • 2018-A03017-48 (Other Identifier: ID-RCB Number)

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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