A-LiNK: Improving Outcomes in Autoimmune Liver Disease

Study Overview
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Brief Summary
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The Autoimmune Liver disease Network for Kids (A-LiNK) is a multi-institutional group with the mission to deliver the best care to kids with pediatric autoimmune liver disease (AILD). This study will establish a shared clinical registry and a learning health network for the participating sites focusing on collecting and transmitting clinical measurement data, information about processes, and participation in an improvement collaborative. Pediatric Autoimmune Hepatitis (AIH) and Primary Sclerosing Cholangitis (PSC), represent a spectrum of AILD which present unique diagnostic and therapeutic challenges.A lack of accepted guidelines for disease monitoring or symptom management results in wide treatment variation with liver transplants indicated in refractory, progressive disease. The aims of A-LiNK are to: 1.) Create a learning health network focused on patient-centered outcomes research characterized by transparent sharing among centers, common priorities, and feasible plans for implementing new practices; 2) shift from traditional investigator-driven study to a patient and family-centered approach, and 3.) improve clinical outcomes and quality of life for pediatric AILD patients.

Official Title
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Autoimmune Liver Disease Network for Kids (A-LiNK): Using Patient Data to Transform Care and Improve Outcomes for Children, Adolescents, and Young Adults With Autoimmune Liver Disease

Conditions
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Autoimmune Hepatitis, Primary Sclerosing Cholangitis

Intervention / Treatment
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* Other: No interventions


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Clinical diagnosis of autoimmune hepatitis (AIH) Clinical diagnosis of primary sclerosing cholangitis (PSC) Clinical diagnosis of autoimmune sclerosing cholangitis (ASC) Exclusion Criteria: • History of liver transplant
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Children and adolescents with Autoimmune Liver Disease<br>Pediatric patients with a diagnosis of autoimmune hepatitis, or primary sclerosing cholangitis. | Other: No interventions<br>* There are no interventions<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Baseline disease characteristics in the population |  | 2034 | 
| Disease relapse and biochemical remission rates | Biochenical remission = transaminases (ALT/AST) within the upper limit of normal for age | 2034 | 
| Complications of disease | Including ascites, esophageal variceal bleeding, cholangitis, hepatic encephalopathy, listed for liver transplant, hepatocellular carcinoma, or cholangiocarcinoma | 2034 | 
| Side-effects of steroids | Including hypertension, diabetes mellitus, low bone mineral density, glaucoma, cataract, overweight, obesity | 2034 | 
| Pediatric Quality of Life Inventory (PedsQL 4.0) Generic Core Scales Short Form 15 | Health-related quality of life | 2034 | 
| Racial and ethnic disparities |  | 2034 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pediatrics, Autoimmune Hepatitis, Primary Sclerosing Cholangitis, Autoimmune Sclerosing Cholangitis, Chronic Liver Disease, End-stage Liver Disease, Clinical Registry, Research Registry, Quality Improvement, Outcomes Research, Comparative Effectiveness Research, Patient Reported Outcomes, Health Services Research