Cystic Fibrosis
Conditions
Keywords
Blood neutrophils, Pseudomonas aeruginosa, Cystic fibrosis, CFTR modulators, respiratory exacerbation, airway chronic infection
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.
Interventions
OTHER1 blood sample
4 tubes of 7 ml per sample (a single sample)
OTHER2 blood samples
4 tubes of 7 ml per sample / 2 samples : before / after antibiotic treatment
Sponsors
Institut National de la Santé Et de la Recherche Médicale, France
URC-CIC Paris Descartes Necker Cochin
Assistance Publique - Hôpitaux de Paris
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
BASIC_SCIENCE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Quantification of blood low density neutrophils in cystic fibrosis (CF) | 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. |
| Transcriptomic analysis of blood neutrophils in cystic fibrosis (CF) | 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. |
| Effector function analysis of blood neutrophils in CF. | Through study completion, an average of 1 year | Comparison of effector function analysis of blood neutrophils of CF patients versus control blood-donors. |
| Phagocytosis potential of blood neutrophils in CF. | Through study completion, an average of 1 year | Comparison of phagocytosis potential of blood neutrophils of CF patients versus control blood-donors. |
| Survival/apoptosis balance analysis in CF | Through study completion, an average of 1 year | Survival / apoptosis balance analysis : (PCNA localization) in CF patients blood neutrophils versus control blood-donors. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Quantification of blood low density neutrophils in CF vs other chronic inflammatory disorders. | 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). |
| Survival/apoptosis balance analysis in CF according to different clinical situations. | 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. |
| Quantification of blood low density neutrophils in CF according to different clinical situations. | 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 |
| Transcriptomic analysis of blood neutrophils in CF according to different clinical situations. | 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 |
| Effector function analysis of blood neutrophils in CF according to different clinical situations. | 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 |
| Phagocytosis potential of blood neutrophils in CF according to different clinical situations. | 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 |
Countries
France
Outcome results
None listed