TAAI Erasmus Research Initiative to Fight CF: Monitoring Inflammation in CF Lung disease into a new Era

Recruiting

Conditions: cystic fibrosis
mucoviscidosis
10038686
10017943
10010613

Registration Number: NL-OMON54176

Lead Sponsor: Erasmus MC, Universitair Medisch Centrum Rotterdam

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 103

Inclusion Criteria

In order to be eligible to participate in this study, a subject must meet all
of the following criteria:

Diagnosed with cystic fibrosis (CF), either by abnormal sweat test and/or
confirmed with 2 CF causing mutations found by genetic analysis, either from
heel-prick screening or diagnosed later in life. Furthermore, they have to be
older than 6 years (i.e. children and adults). Age appropriate written informed
consent is required.

In addition, patients need to meet the criteria of one of the following study
groups:

Group 1: CF patients, whom are eligible to start
elexacaftor/tezacaftor/ivacaftor treatment or who are already using it.
Group 2: CF patients, whom are not eligible to receive
elexacaftor/tezacaftor/ivacaftor treatment. This group will function as
controls.

Exclusion Criteria

- Patients who cannot follow instructions

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Primary endpoint is the comparison of VOCs, measured by GC-MS, during ETI<br /><br>treatment compared to control group over time during 3 different study visits.</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Secondary endpoints:<br /><br>- The correlation of VOCs by GC-MS breath profiles/VOCs, measured by eNose,<br /><br>inflammatory markers in induced sputum (IL-8, free neutrophilic elastase (NE),<br /><br>calprotectin and myeloperoxidase, plus a predetermined cytokine panel), blood<br /><br>(IL-18, IL-1β, TNF, hsCRP, sCD14, calprotectin, HGMB-1, amyloid and miRNA),<br /><br>urine and, lung function, quality of life and symptom scores at baseline (if<br /><br>available) and overtime during 3 consecutive study visits.<br /><br>- Change of VOCs by GC-MS and eNose from baseline till 3 months of ETI<br /><br>treatment.</p><br>