To determine if transient elastography (TE), when combined with ultrasound (US) pattern characterization can improve the prediction of progression to a nodular pattern on US. To confirm the feasibility of obtaining TE measurements in children with Cystic Fibrosis (CF) To prospectively assess whether TE data are associated with conventional laboratory markers of hepatic fibrosis To determine the variability of TE measurements taken at different sites in the same patient
A noninvasive assessment of hepatic fibrosis is desperately needed to advance the care of children with CF significant liver disease and to provide for measurements during clinical trials. That global assessment might serve as both a predictor/descriptor of disease course but also as a critical biomarker for clinical research. FibroScan® measurement of liver stiffness has great potential to fill this void. The underlying hypothesis of this proposal is that elastography in addition to US can improved the prediction of the development of a nodular liver on US and development of portal hypertension over time in children and young adults with CF.
Study Type
OBSERVATIONAL
Enrollment
141
Participants enrolled in Cystic Fibrosis Liver Disease Network (CFLD NET)Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis (PUSH) study in longitudinal follow up at centers with Fibroscan available (currently 8/11 centers)
Children's Hospital Colorado
Aurora, Colorado, United States
Children's Healthcare of Atlanta
Atlanta, Georgia, United States
Ann & Robert H. Lurie Children's
Chicago, Illinois, United States
Riley Hospital for Children
Indianapolis, Indiana, United States
Liver Stiffness Measurement (LSM) obtained via transient elastography using FibroScanTM
Liver stiffness" quantifies liver fibrosis and is measured in kPa (median of 10 subsequent valid measurements and are deemed acceptable if the ratio of interquartile range and median is \<30% and success rate is \>60%, meaning 10 valid measurements are obtained within 16 attempts).
Time frame: Baseline
Liver Stiffness Measurement (LSM) obtained via transient elastography using FibroScanTM
Liver stiffness" quantifies liver fibrosis and is measured in kPa (median of 10 subsequent valid measurements and are deemed acceptable if the ratio of interquartile range and median is \<30% and success rate is \>60%, meaning 10 valid measurements are obtained within 16 attempts).
Time frame: Year 1
Liver Stiffness Measurement (LSM) obtained via transient elastography using FibroScanTM
Liver stiffness" quantifies liver fibrosis and is measured in kPa (median of 10 subsequent
Time frame: Year 2
Liver steatosis obtained via transient elastography
Controlled Attenuation Parameter (CAP)" quantifies liver steatosis and is measured in dB
Time frame: Baseline, Year 1, Year 2
Comparison of liver stiffness and liver steatosis measurements at each time point to grayscale ultrasound grades
Grayscale ultrasound grades will be obtained from the parent study (PUSH: Clinical Trials: NCT01144507)
Time frame: Baseline, Year 1 and Year 2
Comparison of liver stiffness and liver steatosis measurements at each time point to clinical findings of portal hypertension
Grayscale ultrasound grades will be obtained from the parent study (PUSH: Clinical Trials: NCT01144507)
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Cincinnati Children's Hospital Medical
Cincinnati, Ohio, United States
Texas Children's Hospital
Houston, Texas, United States
Seattle Children's Hospital
Seattle, Washington, United States
The Hospital for Sick Children
Toronto, Ontario, Canada
Time frame: Baseline, Year 1 and Year 2