Impact of CFTR-modulation with elexacaftor/tezacaftor/ivacaftor on the exercise capacity, intestinal and respiratory microbiome, microbial and inflammatory metabolites in cystic fibrosis

Impact of CFTR-modulation with elexacaftor/tezacaftor/ivacaftor on the exercise capacity, intestinal and respiratory microbiome, microbial and inflammatory metabolites in cystic fibrosis - IMMProveCFInflammation, Microbiome, Metabolome and physical Performance in Cystic Fibrosis

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

DRKS

Registry ID

DRKS00023862

Enrollment

Registered

2021-06-25

Start date

2021-05-04

Completion date

Unknown

Last updated

2025-12-08

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

E84.0

Interventions

Group 1: observation under medication with Kaftrio Group 2: Control group, healthy children and adults without any medical history

Eligibility

Sex/Gender

All

Age

6 Years to 100 Years

Inclusion criteria

Inclusion criteria: - Age 6 and older - Confirmed diagnosis and mutation - Planned to start medication with elexacaftor/tezacaftor/ivacaftor - Written informed consent (from parent or legal guardian if underaged) Or sex- and aged matched healthy control

Exclusion criteria

Exclusion criteria: - Comorbidity which exclude therapy with elexacaftor/tezacaftor/ivacaftor (e.g liver or renal insufficiency) - Respiratory infection at the start of treatment - Acute therapy changes 21 days before starting therapy with elexacaftor/tezacaftor/ivacaftor - Antibiotic treatment up to 3 weeks prior to study start

Design outcomes

Primary

Measure	Time frame
Longitudinal changes in diversity, species richness and in microbial composition of respiratory and intestinal microbiome and in exercise capacity (peak oxygen uptake and maximal work rate) during the therapy with CFTR-modulators.	—

Secondary

Measure	Time frame
Secondary objectives include - Evaluation of systemic inflammatory parameter (CRP, IgG, IL-8, TNF-alpha, TGF-beta), intestinal and airway inflammation (faecal calprotectin, Il-8 and NE in sputum) and urine cathepsine. - Lung function measurement with spirometry and MBW will be performed to assess absolute changes in ppFEV1, ppFVC, ppFEV75-25 and in LCI. - Changes in concentration of sweat chloride. - Cystic Fibrosis Questionnaire-Revised (CFQ-R) will be completed at all visits. - Assessment of CF metabolome by mass spectroscopy for CF-specific biomarkers.	—

Measure

Time frame

Secondary objectives include - Evaluation of systemic inflammatory parameter (CRP, IgG, IL-8, TNF-alpha, TGF-beta), intestinal and airway inflammation (faecal calprotectin, Il-8 and NE in sputum) and urine cathepsine. - Lung function measurement with spirometry and MBW will be performed to assess absolute changes in ppFEV1, ppFVC, ppFEV75-25 and in LCI. - Changes in concentration of sweat chloride. - Cystic Fibrosis Questionnaire-Revised (CFQ-R) will be completed at all visits. - Assessment of CF metabolome by mass spectroscopy for CF-specific biomarkers.

—

Countries

Germany

Contacts

Public ContactKrystyna Poplawska

Zentrum für Kinder- und Jugendmedizin der Universitätsmedizin Mainz

krystyna.poplawska@unimedizin-mainz.de06131172602

Outcome results

None listed

Source: DRKS (via WHO ICTRP) · Data processed: Feb 4, 2026