Autoimmune Hepatitis in Pediatric Patients

Study Overview
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Brief Summary
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Autoimmune hepatitis (AIH) is an immune-mediated and inflammatory liver disorder. It is currently divided into types 1 and 2, differentiated and defined by the presence of specific autoantibodies. The objectives are to describe the prevalence and incidence of type 1 and 2 autoimmune hepatitis and to analyze the clinics, biochemical and histopathological profiles at diagnosis and follow-up, initial therapy, response to therapy and long-term follow-up in three Italian centers of patients with type 1 and type 2 AIH.

Detailed Description
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Background: Autoimmune hepatitis (AIH) is an immune-mediated and inflammatory liver disorder with typical onset in pediatric age. It is currently divided into types 1 and 2, differentiated and defined by the presence of specific autoantibodies. AIH type 1 (AIH-1) is characterised by smooth muscle and/or antinuclear autoantibodies (SMA/ANA) while anti-liver kidney microsomal (anti-LKM) or liver cytosol type 1 (anti-LC1) identified AIH type 2 (AIH-2). The conventional treatment is based on corticosteroids and azathioprine as first line therapy. The optimal duration is not clear definied, however it should be extended for at least five years due to the frequent relapses. Aims of the study: The primary objective is to describe the prevalence and incidence of type 1 and 2 autoimmune hepatitis on a selected italian pediatric population. Secondary objectives are to analyze the clinical, biochemical and histopathological profiles at diagnosis and follow-up, type of treatment received, response to therapy and long-term follow-up. Methods: The investigators conducted a retrospective and prospective cohort study on patients aged ≤ 18 years who the diagnosis of AIH 1 and 2 between 01/01/1989 and 31/12/2019 and were followed in three Italian pediatric centers.

Official Title
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Multicentric Study on the Long-term Follow-up of Autoimmune Hepatitis in the Pediatric Patients

Conditions
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Autoimmune Hepatitis

Intervention / Treatment
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* Diagnostic Test: pediatric AIH


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age ≤ 18 years at the time of diagnosis Diagnosis of autoimmune hepatitis type 1 or 2 according to the Mieli-Vergani score: a pre-treatment score> 15 or post-treatment> 17 indicates a definite diagnosis of Autoimmune Hepatitis; a pre-treatment score between 10 and 15 or post-treatment between 12 and 17 indicates a probable diagnosis. Exclusion Criteria: Age> 18 years at the time of diagnosis Presence of other causes of liver disease Presence of overlap syndrome (hepatito-cholangitis)

Ages Eligible for Study
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Maximum Age: 18 Years

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 |
| --- | --- |
| Pediatric AIH<br>Patients diagnosed with hepatitis type 1 and type 2 formulated between the ages of 0 and 18. | Diagnostic Test: pediatric AIH<br>* pediatric patients with clinical, laboratory (transaminase and IgG increase), instrumental (ultrasound, liver biopsy) characteristics<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Prevalence and incidence | The primary objective was to calculate the proportion of AIH type 1 and 2 patients present in the Italian pediatric population at a given time (prevalence) and the proportion of new AIH type 1 and 2 patients in the Italian pediatric population (0-18 years) in a given period of time (incidence). | from 01/01/1989 to 31/12/2019 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Characteristics of AIH in pediatric patients | Describe the baseline characteristics (age of onset, clinical-laboratory, radiological and histological characteristics), the response to therapy (medical and / or the possible use of liver transplantation) and the follow-up of patients with autoimmune hepatitis type 1 and 2. | from 01/01/1989 to 31/12/2019 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Autoimmune hepatitis, incidence, prevalence, immunosuppressive treatmen, liver transplantation