MRI to Characterize and Predict CF Liver Disease in PUSH Cohort

NCT02979340 | Completed DMC

• 2 other identifiers: CFLD-MREU01DK062456
• Study Type: observational
• Target: 115
• Locations: 1 country
• Sites: 8

Brief Summary

Specific Aim 1. Determine if valid results of non-sedated MRI based assessments of liver stiffness and lipid content can be obtained in more than 90% of children and young adults with cystic fibrosis. Specific Aim 2. Determine hepatic lipid content using the HepaFat sequence and liver stiffness using MRE. Investigators will compare the results obtained by MRI with PUSH study grayscale ultrasounds in CF patients with normal, heterogeneous, homogeneous or nodular (cirrhotic) pattern on ultrasound. Specific Aim 3. Creation of an imaging core lab to centralize evaluation of MR imaging data, allow for remote image upload, electronic data storage, and remote image viewing/interpretation. This infrastructure will be utilized to standardize image post processing. Specific Aim 4. Using the longitudinal PUSH study, determine if MRI based imaging improves discrimination of subjects at risk for progression to advanced CF liver disease (development of cirrhosis) compared to using US imaging alone

Conditions

Cystic Fibrosis Liver Disease; Pancreatic Insufficiency

Keywords

Cystic Fibrosis Liver Disease; Pancreatic Insufficiency

Outcome Measures

Primary Outcomes (1)

• Change over Time in Liver stiffness and Lipid content over time using MRE

  Analysis of variance (ANOVA) will be used to compare the distribution of liver stiffness (in kPa) and lipid content measures between all groups defined by PUSH study grayscale ultrasounds results over time.

  BSL, Year 1, Year 2

Interventions

MRI PROCEDURE

non-invasive assessment of hepatic fibrosis and steatosis

Eligibility Criteria

• Age: 6 Years - 26 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Non-Probability Sample

Study Population

Children aged 3 through 12 years of age at time of enrollment diagnosed with Cystic Fibrosis and pancreatic insufficiency and enrolled in the CFLD PUSH longitudinal study.

You may qualify if:

• \. Currently enrolled in longitudinal follow up in PUSH study at a site with MR elastography.

You may not qualify if:

• Age under 6 years (all current patients at time of opening of this study will be greater than 6 years of age).
• Internal appliance or hardware that is not compatible with MR.
• Inability to obtain MRI within 6 months of US.
• Inability to cooperate with MRI.

Sponsors & Collaborators

• National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): lead
• Cystic Fibrosis Foundation: collaborator

Study Sites (8)

Children's Hospital Colorado

Aurora, Colorado, 80045, United States

Location

Children's Healthcare of Atlanta

Atlanta, Georgia, 30322, United States

Location

Riley Hospital for Children

Indianapolis, Indiana, 46202, United States

Location

Johns Hopkins School of Medicine

Baltimore, Maryland, 21217, United States

Location

Washington University School of Medicine

St Louis, Missouri, 63110, United States

Location

Cincinnati Children's Hospital Medical

Cincinnati, Ohio, 45229, United States

Location

Texas Children's Hospital

Houston, Texas, 77030, United States

Location

Seattle Children's Hospital

Seattle, Washington, 98105, United States

Location

Related Publications (1)

• Towbin AJ, Ye W, Huang S, Karmazyn BW, Molleston JP, Masand P, Leung DH, Chang S, Narkewicz MR, Alazraki AL, Freeman AJ, Otto RK, Green N, Kamel IR, Karnsakul WW, Magee JC, Tkach J, Palermo JJ. Prospective study of quantitative liver MRI in cystic fibrosis: feasibility and comparison to PUSH cohort ultrasound. Pediatr Radiol. 2023 Oct;53(11):2210-2220. doi: 10.1007/s00247-023-05706-6. Epub 2023 Jul 28.

  PMID: 37500799 BACKGROUND

Related Links

• MRE is a study in the ChiLDReN Network

MeSH Terms

Conditions

Exocrine Pancreatic Insufficiency

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases

Study Officials

• Ed Doo, MD

  National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

  STUDY DIRECTOR

• Averell Sherker, MD

  National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

  STUDY DIRECTOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: November 18, 2016
• First Posted: December 1, 2016
• Study Start: January 27, 2017
• Primary Completion: December 27, 2019
• Study Completion: December 27, 2019
• Last Updated: April 11, 2025

Record last verified: 2025-04

Data Sharing

• IPD Sharing: Will not share

Locations

US (8)