Cystic Fibrosis in the Kidney: Monitoring the Effectiveness of Elexacaftor/tezacaftor/ivacaftor in Urine After a Short Pause of Therapy

Last updated: February 17, 2025
Sponsor: University of Aarhus
Overall Status: Active - Recruiting

Phase

N/A

Condition

Cystic Fibrosis

Lung Disease

Scar Tissue

Treatment

12 hours ETI pause

36 hours ETI pause

60 hours ETI pause

Clinical Study ID

NCT05818319
CFPT29092022
  • Ages > 18
  • All Genders

Study 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 Criteria

Inclusion

Inclusion Criteria:

  • Adult (age >17 years) CF patients.

  • Normal kidney function estimated by eGFR>90.

  • Adults capable of understanding and voluntarily consenting.

Exclusion

Exclusion Criteria:

  • Critical acute illness.

  • Severe lung disease (ppFEV1<40%).

  • Adults not capable of understanding and voluntarily consenting.

Study Design

Total Participants: 30
Treatment Group(s): 3
Primary Treatment: 12 hours ETI pause
Phase:
Study Start date:
June 01, 2023
Estimated Completion Date:
December 31, 2025

Connect with a study center

  • Department of Infectious Diseases, Aarhus University Hospital

    Aarhus C, Midtjylland 8000
    Denmark

    Active - Recruiting

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