Cystic Fibrosis Blood Neutrophils (MUCO-PNN)

Assistance Publique - Hôpitaux de Paris

Status: Completed

Conditions: Cystic Fibrosis

Treatments: Other: 1 blood sample; Other: 2 blood samples

Study type: Interventional

Funder types: Other

Identifiers

NCT04970225

2018-A03017-48 (Other Identifier)

APHP180430

Details and patient eligibility

About

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.

Enrollment: 47 patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

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.

Trial design

Primary purpose: Basic Science

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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47 participants in 3 patient groups

Stable state

Experimental group

Description:

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

Treatment:

Other: 1 blood sample

Starting Ivacaftor-Tezacaftor-Elexacaftor

Experimental group

Description:

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

Treatment:

Other: 2 blood samples

Exacerbation

Experimental group

Description:

15 adult cystic fibrosis patients followed in the respiratory medicine department of Cochin hospital, paris, France and hospitalized for respiratory exacerbation

Treatment:

Other: 2 blood samples