Cystic Fibrosis in the Kidney: Monitoring the Effectiveness of Elexacaftor/tezacaftor/ivacaftor in Urine After a Short Pause of Therapy
University of Aarhus
30 participants
Jun 1, 2023
INTERVENTIONAL
Conditions
Summary
In cystic fibrosis (CF) renal base excretion is impaired, due to mutations in the Cystic Fibrosis Transmembrane Regulator (CFTR) gene, since CFTR function is crucial in regulation of the kidney's HCO3- excretion. The investigators suggest that challenged urine HCO3- excretion is a biomarker of CFTR function, which can be used to evaluate the extent of CFTR dysfunction and the possible correcting effects of CFTR modulating therapy. This study aims to evaluate changes in challenged urine HCO3- excretion in CF patients, who are currently in treatment with the triple CFTR modulator combination therapy, Elexacaftor/tezacaftor/ivacaftor (ETI), before, during, and after a short treatment pause.
Eligibility
Inclusion Criteria3
- Adult (age >17 years) CF patients.
- Normal kidney function estimated by eGFR>90.
- Adults capable of understanding and voluntarily consenting.
Exclusion Criteria3
- Critical acute illness.
- Severe lung disease (ppFEV1<40%).
- Adults not capable of understanding and voluntarily consenting.
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Interventions
Patients with CF are randomly allocated to ETI pause lasting 12 hours.
Patients with CF are randomly allocated to ETI pause lasting either 36 hours.
Patients with CF are randomly allocated to ETI pause lasting either 60 hours.
Locations(1)
View Full Details on ClinicalTrials.gov
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NCT05818319