Autoimmune Hepatitis in Pediatric Patients

NCT ID: NCT04247490

Last Updated: 2020-01-30

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

109 participants

Study Classification

OBSERVATIONAL

Study Start Date

2019-12-17

Study Completion Date

2019-12-31

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.

Conditions

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Autoimmune Hepatitis

Study Design

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Observational Model Type

COHORT

Study Time Perspective

RETROSPECTIVE

Study Groups

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Pediatric AIH

Patients diagnosed with hepatitis type 1 and type 2 formulated between the ages of 0 and 18.

pediatric AIH

Intervention Type DIAGNOSTIC_TEST

pediatric patients with clinical, laboratory (transaminase and IgG increase), instrumental (ultrasound, liver biopsy) characteristics

Interventions

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pediatric AIH

pediatric patients with clinical, laboratory (transaminase and IgG increase), instrumental (ultrasound, liver biopsy) characteristics

Intervention Type DIAGNOSTIC_TEST

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)
Maximum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University Hospital of Ferrara

OTHER

Sponsor Role lead

Responsible Party

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Giuseppe Maggiore

Full Professor

Responsibility Role PRINCIPAL_INVESTIGATOR

Locations

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University Hospital of Ferrara

Cona, Ferrara, Italy

Site Status

Countries

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Italy

References

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Jimenez-Rivera C, Ling SC, Ahmed N, Yap J, Aglipay M, Barrowman N, Graitson S, Critch J, Rashid M, Ng VL, Roberts EA, Brill H, Dowhaniuk JK, Bruce G, Bax K, Deneau M, Guttman OR, Schreiber RA, Martin S, Alvarez F. Incidence and Characteristics of Autoimmune Hepatitis. Pediatrics. 2015 Nov;136(5):e1237-48. doi: 10.1542/peds.2015-0578. Epub 2015 Oct 19.

Reference Type BACKGROUND
PMID: 26482664 (View on PubMed)

Verma S, Torbenson M, Thuluvath PJ. The impact of ethnicity on the natural history of autoimmune hepatitis. Hepatology. 2007 Dec;46(6):1828-35. doi: 10.1002/hep.21884.

Reference Type RESULT
PMID: 17705297 (View on PubMed)

Nastasio S, Sciveres M, Matarazzo L, Maggiore G. Old and New Treatments for Pediatric Autoimmune Hepatitis. Curr Pediatr Rev. 2018;14(3):187-195. doi: 10.2174/1573396314666180516130314.

Reference Type RESULT
PMID: 29766815 (View on PubMed)

Maggiore G, Porta G, Bernard O, Hadchouel M, Alvarez F, Homberg JC, Alagille D. Autoimmune hepatitis with initial presentation as acute hepatic failure in young children. J Pediatr. 1990 Feb;116(2):280-2. doi: 10.1016/s0022-3476(05)82892-6. No abstract available.

Reference Type RESULT
PMID: 2299503 (View on PubMed)

Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, Cooksley WG, Czaja AJ, Desmet VJ, Donaldson PT, Eddleston AL, Fainboim L, Heathcote J, Homberg JC, Hoofnagle JH, Kakumu S, Krawitt EL, Mackay IR, MacSween RN, Maddrey WC, Manns MP, McFarlane IG, Meyer zum Buschenfelde KH, Zeniya M, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999 Nov;31(5):929-38. doi: 10.1016/s0168-8278(99)80297-9. No abstract available.

Reference Type RESULT
PMID: 10580593 (View on PubMed)

Mieli-Vergani G, Vergani D, Baumann U, Czubkowski P, Debray D, Dezsofi A, Fischler B, Gupte G, Hierro L, Indolfi G, Jahnel J, Smets F, Verkade HJ, Hadzic N. Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement. J Pediatr Gastroenterol Nutr. 2018 Feb;66(2):345-360. doi: 10.1097/MPG.0000000000001801.

Reference Type RESULT
PMID: 29356770 (View on PubMed)

Other Identifiers

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637/2019/Oss/AOUFe

Identifier Type: -

Identifier Source: org_study_id

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