MRI to Characterize and Predict CF Liver Disease in PUSH Cohort

Status:	Enrolling by invitation
Conditions:	Gastrointestinal, Pulmonary
Therapuetic Areas:	Gastroenterology, Pulmonary / Respiratory Diseases
Healthy:	No
Age Range:	6 - 26
Updated:	4/17/2018
Start Date:	December 2016
End Date:	December 2019

Analysis of Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis (PUSH) Cohort Using Advanced MRI to Characterize and Predict CF Liver Disease

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

This proposal will be a cross sectional study of the current well-characterized PUSH cohort.
Patients will receive an MRI at the time of their scheduled PUSH ultrasound. Ideally, the MRI
will be performed the same day or a similar time point near liver ultrasound (+/-90 days, but
Investigators will allow a larger window due to the slow progression of fibrosis).

Blinded radiology review of imaging sequences will be performed by a local study radiologist
with central review performed by the Imaging Core. Analysis by the Imaging Core will include
calculation of the liver stiffness, liver and spleen volume, and hepatic and lipid content
for all MRI studies.

Inclusion Criteria:

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

Exclusion Criteria:

1. Age under 6 years (all current patients at time of opening of this study will be
greater than 6 years of age).

2. Internal appliance or hardware that is not compatible with MR.

3. Inability to obtain MRI within 6 months of US.

4. Inability to cooperate with MRI.

We found this trial at

sites

Children's Healthcare of Atlanta

1405 Clifton Road NE
Atlanta, Georgia 30322

404-785-6000