Cystic Fibrosis Transmembrane Regulator (CFTR) Biomarker Study to Evaluate the Rescue of Mutant CFTR in Patients With Cystic Fibrosis Treated With CFTR-modulators

NCT ID: NCT04732910

Last Updated: 2024-03-04

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Total Enrollment

200 participants

Study Classification

OBSERVATIONAL

Study Start Date

2018-07-01

Study Completion Date

2027-12-31

Brief Summary

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This observational study evaluates the effect of therapy with cystic fibrosis transmembrane regulator (CFTR) modulators on CFTR function measured by the CFTR biomarker intestinal current measurement (ICM), nasal potential difference (NPD) and sweat chloride in a post-approval setting in patients with cystic fibrosis (CF).

Detailed Description

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Cystic fibrosis transmembrane regulator (CFTR) biomarker (intestinal current measurement (ICM), nasal potential difference (NPD), sweat chloride) before the start of therapy and 12 and 52 weeks after initiation of therapy Clinical parameters (anthropometry, lung function, lung magnetic resonance imaging (MRI), lung computer tomography (CT)) before the start of therapy and after initiation of therapy Assessment of airway secretion specimens before the start of therapy and after initiation of therapy

Conditions

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Cystic Fibrosis

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Interventions

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Treatment with cystic fibrosis transmembrane regulator (CFTR) modualtors Ivacaftor, Lumacaftor-Ivacaftor, Tezacaftor-Ivacaftor, Elexacaftor-Tezacaftor-Ivacaftor

observational

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Decision for cystic fibrosis (CF) transmembrane regulator (CFTR)-modulator therapy by the patient and the caring CF physician
* Signed informed consent form (ICF) and, where appropriate, signed assent form.

Exclusion Criteria

* Ongoing participation in an investigational drug study (including studies investigating lumacaftor, tezacaftor or ivacaftor)
Minimum Eligible Age

6 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Hannover Medical School

OTHER

Sponsor Role collaborator

Heidelberg University

OTHER

Sponsor Role collaborator

University of Giessen

OTHER

Sponsor Role collaborator

Charite University, Berlin, Germany

OTHER

Sponsor Role lead

Responsible Party

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Simon Graeber

BIH Clinician Scientist

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Simon Y Graeber, MD

Role: PRINCIPAL_INVESTIGATOR

Charite University, Berlin, Germany

Locations

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Charité - Universitätsmedizin Berlin

Berlin, , Germany

Site Status RECRUITING

Justus-Liebig-University Giessen

Giessen, , Germany

Site Status RECRUITING

Hannover Medical School

Hanover, , Germany

Site Status RECRUITING

University of Heidelberg

Heidelberg, , Germany

Site Status RECRUITING

Countries

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Germany

Central Contacts

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Simon Y Graeber, MD

Role: CONTACT

+4930 450 566 587

Facility Contacts

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Simon Graeber, MD

Role: primary

+4930 450 566 587

Lutz Nährlich, MD

Role: primary

Burkhard Tümmler, MD

Role: primary

Olaf Sommerburg, MD

Role: primary

References

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Schaupp L, Addante A, Voller M, Fentker K, Kuppe A, Bardua M, Duerr J, Piehler L, Rohmel J, Thee S, Kirchner M, Ziehm M, Lauster D, Haag R, Gradzielski M, Stahl M, Mertins P, Boutin S, Graeber SY, Mall MA. Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis. Eur Respir J. 2023 Aug 3;62(2):2202153. doi: 10.1183/13993003.02153-2022. Print 2023 Aug.

Reference Type DERIVED
PMID: 37414422 (View on PubMed)

Graeber SY, Renz DM, Stahl M, Pallenberg ST, Sommerburg O, Naehrlich L, Berges J, Dohna M, Ringshausen FC, Doellinger F, Vitzthum C, Rohmel J, Allomba C, Hammerling S, Barth S, Ruckes-Nilges C, Wielputz MO, Hansen G, Vogel-Claussen J, Tummler B, Mall MA, Dittrich AM. Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles. Am J Respir Crit Care Med. 2022 Aug 1;206(3):311-320. doi: 10.1164/rccm.202201-0219OC.

Reference Type DERIVED
PMID: 35536314 (View on PubMed)

Graeber SY, Vitzthum C, Pallenberg ST, Naehrlich L, Stahl M, Rohrbach A, Drescher M, Minso R, Ringshausen FC, Rueckes-Nilges C, Klajda J, Berges J, Yu Y, Scheuermann H, Hirtz S, Sommerburg O, Dittrich AM, Tummler B, Mall MA. Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles. Am J Respir Crit Care Med. 2022 Mar 1;205(5):540-549. doi: 10.1164/rccm.202110-2249OC.

Reference Type DERIVED
PMID: 34936849 (View on PubMed)

Other Identifiers

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20012746

Identifier Type: -

Identifier Source: org_study_id

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