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| ID | Type | Description | Link |
|---|---|---|---|
| U01DK062456 | U.S. NIH Grant/Contract | View source |
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| Name | Class |
|---|---|
| Cystic Fibrosis Foundation | OTHER |
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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.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Transient Elastography (TE) | Procedure | 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) |
| Measure | Description | Time Frame |
|---|---|---|
| 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). | 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). | 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 | Year 2 |
| Measure | Description | Time Frame |
|---|---|---|
| Liver steatosis obtained via transient elastography | Controlled Attenuation Parameter (CAP)" quantifies liver steatosis and is measured in dB | Baseline, Year 1, Year 2 |
| Comparison of liver stiffness and liver steatosis measurements at each time point to grayscale ultrasound grades |
| Measure | Description | Time Frame |
|---|---|---|
| Invalid measurements and success rate on liver stiffness and liver steatosis measurements | Baseline, Year 1 and Year 2 | |
| Inter quartile range (IQR) (kPa or dB/m) of all valid measurements within the examination | To reflect the dispersion of stiffness and steatosis |
Inclusion Criteria:
Participants enrolled in CFLD NET PUSH study in longitudinal follow up at centers with Fibroscan available (currently 8/11 centers)
Entry criteria for that study were:
CF as determined by sweat chloride >60 meq/l
Pancreatic insufficiency
Age 3-12 years old at entry
For entry into the longitudinal follow up subjects were in one of two groups
Exclusion Criteria:
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Participants enrolled in CFLD NET PUSH study in longitudinal follow up at centers with Fibroscan available (currently 8/11 centers) All racial and ethnic groups will be included.
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| Name | Affiliation | Role |
|---|---|---|
| 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 |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital Colorado | Aurora | Colorado | 80045 | United States | ||
| Children's Healthcare of Atlanta |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 34430781 | Background | Ye W, Leung DH, Molleston JP, Ling SC, Murray KF, Nicholas JL, Huang S, Karmazyn BW, Harned RK, Masand P, Alazraki AL, Navarro OM, Otto RK, Palermo JJ, Towbin AJ, Alonso EM, Karnsakul WW, Jane Schwarzenberg S, Seidel GF, Siegel M, Magee JC, Narkewicz MR, Jay Freeman A. Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis. Hepatol Commun. 2021 May 13;5(8):1362-1372. doi: 10.1002/hep4.1719. eCollection 2021 Aug. |
| Label | URL |
|---|---|
| ChiLDReN Website | View source |
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Grayscale ultrasound grades will be obtained from the parent study (PUSH: Clinical Trials: NCT01144507) |
| 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) | Baseline, Year 1 and Year 2 |
| Baseline, Year 1 and Year 2 |
| IQR/median ratio of all valid measurements within the examination | This quantity should remain as low as possible to ensure reliable results (goal < 30%) | Baseline, Year 1 and Year 2 |
| Atlanta |
| Georgia |
| 30322 |
| United States |
| Ann & Robert H. Lurie Children's | Chicago | Illinois | 60611 | United States |
| Riley Hospital for Children | Indianapolis | Indiana | 46202 | United States |
| Cincinnati Children's Hospital Medical | Cincinnati | Ohio | 45229 | United States |
| Texas Children's Hospital | Houston | Texas | 77030 | United States |
| Seattle Children's Hospital | Seattle | Washington | 98105 | United States |
| The Hospital for Sick Children | Toronto | Ontario | M5G 1X8 | Canada |
| ID | Term |
|---|---|
| D010188 | Exocrine Pancreatic Insufficiency |
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |
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