Evaluation of Lung T1-MRI in Pediatric Cystic Fibrosis Patients

Lung T1 MRI Assessments of Children With CF Initiating Trikafta Therapy

Status

Completed

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT04994301

Enrollment

Registered

2021-08-06

Start date

2020-12-15

Completion date

2023-12-15

Last updated

2026-09-14

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

Conditions

Cystic Fibrosis

Keywords

Cystic Fibrosis, Transmembrane, Conductance, Regulator

Brief summary

In this observational study, the investigators evaluate the sensitivity of T1-MRI to identify lung perfusion changes in pediatric patients with CF (age = 6-11) before and after initiating FDA-approved Trikafta therapy. The investigators compare these Lung T1 MRI assessments (% Normal lung perfusion) to currently best-available assessments of lung function in CF patients (i.e., MBW (LCI( and Spirometry (FEV1 % Predicted).

Detailed description

This is a prospective study with 3 study visits to evaluate the utility of Magnetic Resonance Imaging (MRI) and clinical lung function assessments to detect changes in Cystic Fibrosis (CF) patients before and after administration of the FDA-approved Trikafta therapy. The 3 study visits include: Visit 1: Before starting Trikafta Visit 2: 3 months from start of Trikafta Visit 3: 6 months from start of Trikafta Along with the clinical assessments (MBW and Spirometry), all participants will undergo an MRI scan of the lungs to generate quantitative lung T1 maps. The investigators will compare the lung T1 MRI (% Normal Lung Perfusion) to Multiple Breath Washout (LCI) and spirometry (FEV1 % Predicted) as methods to assess lung changes with administration of Trikafta. The investigators will obtain additional clinical assessments from participant's medical records. This is a multi-site study involving 3 sites.

Interventions

DIAGNOSTIC_TESTLung T1 MRI

Evaluation of lung T1 MRI to Assess Lung Disease

Study design

Observational model

COHORT

Time perspective

PROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

5 Years to 11 Years

Healthy volunteers

Inclusion criteria

• Male or female individuals with a diagnosis of cystic fibrosis and have at least one copy of the F508del mutation.

Exclusion criteria

* Subject who cannot hold their breath for up to 15 seconds. * Subjects who are pregnant. * Subjects with MRI contraindication (e.g., heart pacemaker, heart defibrillator, metal in within the body.

Design outcomes

Primary

Measure	Time frame	Description
Lung T1-MRI	Visit 1: Before starting Trikafta, Visit 2: 3 months (+/- 15 days) from start of Trikafta, Visit 3: 6 months (+/- 30 days) from start of Trikafta	Evaluate lung T1-MRI (% Normal Lung Perfusion) to assess lung perfusion changes associated with FDA-approved Trikafta therapy in pediatric cystic fibrosis patients.

Secondary

Measure	Time frame	Description
Spirometry	Visit 1: Before starting Trikafta, Visit 2: 3 months (+/- 15 days) from start of Trikafta, Visit 3: 6 months (+/- 30 days) from start of Trikafta	Clinical standard pulmonary function test (FEV1 % Predicted).
Multiple breath washout (MBW)	Visit 1: Before starting Trikafta, Visit 2: 3 months (+/- 15 days) after start of Trikafta, Visit 3: 6 months (+/- 30 days) after start of Trikafta	Multiple breath washout to assess lung clearance index (LCI) applied per CF clinical standard.

Countries

United States

Contacts

PRINCIPAL_INVESTIGATORChris Flask, PhD

Case Western Reserve University

Outcome results

None listed

Source: ClinicalTrials.gov · Data processed: Sep 15, 2026