Clinical trial NCT04970225: Cystic Fibrosis Blood Neutrophils
Official title: Functional and Phenotypic Characteristics of Blood Neutrophils in Cystic Fibrosis
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: NA
Conditions: Cystic Fibrosis
Keywords: Blood neutrophils
Pseudomonas aeruginosa
Cystic fibrosis
CFTR modulators
respiratory exacerbation
airway chronic infection
Lead sponsor: Assistance Publique - Hôpitaux de Paris
Sponsor class: OTHER
Collaborators: name: Institut National de la Santé Et de la Recherche Médicale, France
First posted: 2021-07-21
Enrollment: 130
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Primary purpose: BASIC_SCIENCE

Brief 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.


Eligibility criteria: 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.

Primary outcomes:
measure: Quantification of blood low density neutrophils in cystic fibrosis (CF)
timeFrame: Through study completion, an average of 1 year
measure: Transcriptomic analysis of blood neutrophils in cystic fibrosis (CF)
timeFrame: Through study completion, an average of 1 year
measure: Effector function analysis of blood neutrophils in CF.
timeFrame: Through study completion, an average of 1 year
measure: Phagocytosis potential of blood neutrophils in CF.
timeFrame: Through study completion, an average of 1 year
measure: Survival/apoptosis balance analysis in CF
timeFrame: Through study completion, an average of 1 year
Secondary outcomes:
measure: Quantification of blood low density neutrophils in CF vs other chronic inflammatory disorders.
timeFrame: Through study completion, an average of 1 year
measure: Quantification of blood low density neutrophils in CF according to different clinical situations.
timeFrame: Through study completion, an average of 1 year
measure: Transcriptomic analysis of blood neutrophils in CF according to different clinical situations.
timeFrame: Through study completion, an average of 1 year
measure: Effector function analysis of blood neutrophils in CF according to different clinical situations.
timeFrame: Through study completion, an average of 1 year
measure: Phagocytosis potential of blood neutrophils in CF according to different clinical situations.
timeFrame: Through study completion, an average of 1 year
measure: Survival/apoptosis balance analysis in CF according to different clinical situations.
timeFrame: Through study completion, an average of 1 year
Other outcomes:
Locations:
city: Paris
country: France
facility: Cochin hospital, AP-HP
Study officials:
affiliation: APHP
name: Pierre-Régis BURGEL, MD PHD
role: PRINCIPAL_INVESTIGATOR
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