MRI Based Biomarkers in Pediatric Autoimmune Liver Disease
|The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.|
|ClinicalTrials.gov Identifier: NCT03175471|
Recruitment Status : Recruiting
First Posted : June 5, 2017
Last Update Posted : July 2, 2020
|Condition or disease|
|Autoimmune Liver Disease Primary Sclerosing Cholangitis Autoimmune Hepatitis|
|Study Type :||Observational|
|Estimated Enrollment :||115 participants|
|Official Title:||Cross-sectional Study for Assessment of MRI Based Biomarkers of Bile Duct Injury and Hepatic Fibrosis in Pediatric Onset Autoimmune Liver Disease|
|Actual Study Start Date :||January 17, 2017|
|Estimated Primary Completion Date :||January 30, 2021|
|Estimated Study Completion Date :||January 30, 2027|
Patients with autoimmune liver disease
Patients (6-23 y.o.) with established clinical diagnosis of AIH or suspected diagnosis of AIH based on elevated serum AST or ALT, elevated IgG level >1.1 ULN, elevated titer of autoantibodies, including ANA, SMA, LKM, LC-1 or SLA, which is consistent with the simplified criteria for the diagnosis of AIH in children will be enrolled.
Patients (6-23 y.o.) with established clinical diagnosis of PSC or Suspected diagnosis of PSC supported by abnormal cholangiogram (ERCP or MRCP) or elevated GGT>1.5 ULN and dilated bile ducts by liver ultrasound will be enrolled.
- MRI based outcomes [ Time Frame: 36 months ]
MRCP based assessment of intrahepatic and extrahepatic duct irregularities by Majoie classification (on 4 and 5 point scale of 0-3 and 0-4 respectively; 0: No visible abnormalities, 1: minimal dilatation/irregularities, 2: saccular dilatations/segmental stricture, 3: severe pruning, 4: Extremely irregular margin).
MREL based quantification of mean shear stiffness (kPa) of liver.
- Liver histopathology based assessment of bile duct injury by ISHAK Score [ Time Frame: 36 months ]Assessment of bile duct injury by ISHAK Score (Confluent necrosis: on the 7 point scale of 0-6; Focal necrosis on the 4 point scale of 0-4 and portal inflammation on the 4 point scale of 0-4).
- Liver histopathology based assessment of bile duct injury by Ludwig score [ Time Frame: 36 months ]Assessment of bile duct injury by Ludwig score (on five point scale of 0-4; 0: No ductal injury, 1: portal inflammation, 2: periportal inflammation, 3: Portal bridging, 4: Nodular cirrhosis).
- Liver histopathology based assessment of liver fibrosis by Nakanuma score [ Time Frame: 36 months ]Assessment of liver fibrosis by Nakanuma score for on the 4 point scale of 0-3 (0; No portal fibrosis, 1; Portal fibrosis; 2; Bridging fibrosis, 3; Liver cirrhosis) .
- Liver histopathology based assessment of liver fibrosis by Ishak score [ Time Frame: 36 months ]Assessment of liver fibrosis by Ishak score on the 7 point scale of 0-6 (0; Absent, 1; confluent necrosis, 2; necrosis in some areas, 3; necrosis in most areas, 4; necrosis with occasional portal-central bridging necrosis, 5; necrosis with multiple portal-central bridging necrosis, 6; Panacinar or multiacinar necrosis).
- Liver histopathology based assessment of cholangitis and hepatic activity [ Time Frame: 36 months ]Cholangitis and hepatic activity by Nakanuma score for on the 4 point scale of 0-3 (0; No bile duct loss, 1; Bile duct loss in <1/3 of portal tracts; 2; Bile duct loss in 1/3-2/3 of portal tracts, 3; Bile duct loss in >2/3 of portal tracts).
- Serum based outcome [ Time Frame: 36 months ]Quantification of serum alkaline phosphatase (ALP in U/L) and Gamma-glutamyl transpeptidase (GGT in U/L).
- Enhanced Liver Fibrosis (ELF) score [ Time Frame: 36 months ]Assesment of Enhanced Liver Fibrosis (ELF) score on continuous scale of 1-10; <7.7 none -mild. ≥7.7 -<9.8 moderate, >9.8 sever).
- MR T1rho, T1, T2 Imaging [ Time Frame: 36 Months ]Mean of MR T1rho, T1, T2 signal in msec to measure the inflammation.
- Liver Morphometry [ Time Frame: 36 Months ]Collagen deposition in percent area fibrosis by image analysis
- Liver histopathology based outcomes [ Time Frame: 36 Months ]Liver histopathology based grade of inflammation by Scheuer score on 5 point scale of 0-4; (0: No ductal injury, 1: portal inflammation, 2: periportal inflammation, 3: Portal to portal bridging, 4: Nodular cirrhosis).
- Serum based outcomes [ Time Frame: 36 Months ]Quantification of serum fractionated ALP (U/L)
- Serum MMP7 [ Time Frame: 36 Months ]Quantification of serum MMP7 (pg/mL)
Biospecimen Retention: Samples With DNA
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03175471
|Contact: Alexander Miethke, MDemail@example.com|
|Contact: Ruchi Singh, PhDfirstname.lastname@example.org|
|United States, Ohio|
|Cincinnati Childrens Hospital Medical Center||Recruiting|
|Cincinnati, Ohio, United States, 45229|
|Contact: Alexander Miethke, MD 513-636-5581 email@example.com|
|Principal Investigator: Alexander Miethke, MD|
|Principal Investigator:||Alexander Miethke, MD||Cincinnati Childrens Hospital Medical Center|