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Autoimmune hepatitis in Egyptian children: A single center experience

BACKGROUND AND AIM: Autoimmune hepatitis (AIH) has variable clinical manifestations and should be considered in the diagnostic work-up of any patient with cryptogenic liver disease. The aim of the study was to determine the clinical, biochemical, histopathological characteristics and treatment outco...

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Autores principales: Mogahed, Engy, El-Karaksy, Hanaa, Zaki, Heba, Abdullatif, Hala
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8894955/
https://www.ncbi.nlm.nih.gov/pubmed/35231187
http://dx.doi.org/10.1177/20587384211073265
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author Mogahed, Engy
El-Karaksy, Hanaa
Zaki, Heba
Abdullatif, Hala
author_facet Mogahed, Engy
El-Karaksy, Hanaa
Zaki, Heba
Abdullatif, Hala
author_sort Mogahed, Engy
collection PubMed
description BACKGROUND AND AIM: Autoimmune hepatitis (AIH) has variable clinical manifestations and should be considered in the diagnostic work-up of any patient with cryptogenic liver disease. The aim of the study was to determine the clinical, biochemical, histopathological characteristics and treatment outcome of AIH in Egyptian children. PATIENTS AND METHODS: This observational study was conducted at the Pediatric Hepatology Unit at Cairo University Pediatric Hospital, Egypt. All children (<18 years of age) presenting from 2009 to 2016 with established diagnosis of AIH were included. Medical history, clinical examination, and results of investigations were retrieved from patients’ files. The main outcome measures included the rate of remission, relapses, and mortality. RESULTS: The study included 34 children with AIH. Twenty patients (58%) presented with chronic liver disease. There was a history of concomitant autoimmune diseases in 5 patients. Transaminases were elevated in all patients. There was synthetic dysfunction in 58%. Twenty-four patients (70.5%) had AIH-1, while nine patients (26.4%) had AIH-2 and one patient (2.9%) had autoantibody negative AIH. Piecemeal necrosis was observed in the liver biopsy of 79% of our cohort. Approximately 80% achieved biochemical remission (88% received combined therapy of prednisolone and azathioprine). About half of the patients developed relapses. One patient died of liver cell failure. CONCLUSION: In children with liver disease, a diagnosis of AIH should be considered. In those patients, AIH-1 is more common than AIH-2. Prednisolone monotherapy or combined with azathioprine could achieve remission, but relapse is still common. Treatment non-adherence is the main risk factor for relapse.
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spelling pubmed-88949552022-03-05 Autoimmune hepatitis in Egyptian children: A single center experience Mogahed, Engy El-Karaksy, Hanaa Zaki, Heba Abdullatif, Hala Int J Immunopathol Pharmacol Original Research Article BACKGROUND AND AIM: Autoimmune hepatitis (AIH) has variable clinical manifestations and should be considered in the diagnostic work-up of any patient with cryptogenic liver disease. The aim of the study was to determine the clinical, biochemical, histopathological characteristics and treatment outcome of AIH in Egyptian children. PATIENTS AND METHODS: This observational study was conducted at the Pediatric Hepatology Unit at Cairo University Pediatric Hospital, Egypt. All children (<18 years of age) presenting from 2009 to 2016 with established diagnosis of AIH were included. Medical history, clinical examination, and results of investigations were retrieved from patients’ files. The main outcome measures included the rate of remission, relapses, and mortality. RESULTS: The study included 34 children with AIH. Twenty patients (58%) presented with chronic liver disease. There was a history of concomitant autoimmune diseases in 5 patients. Transaminases were elevated in all patients. There was synthetic dysfunction in 58%. Twenty-four patients (70.5%) had AIH-1, while nine patients (26.4%) had AIH-2 and one patient (2.9%) had autoantibody negative AIH. Piecemeal necrosis was observed in the liver biopsy of 79% of our cohort. Approximately 80% achieved biochemical remission (88% received combined therapy of prednisolone and azathioprine). About half of the patients developed relapses. One patient died of liver cell failure. CONCLUSION: In children with liver disease, a diagnosis of AIH should be considered. In those patients, AIH-1 is more common than AIH-2. Prednisolone monotherapy or combined with azathioprine could achieve remission, but relapse is still common. Treatment non-adherence is the main risk factor for relapse. SAGE Publications 2022-03-01 /pmc/articles/PMC8894955/ /pubmed/35231187 http://dx.doi.org/10.1177/20587384211073265 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Original Research Article
Mogahed, Engy
El-Karaksy, Hanaa
Zaki, Heba
Abdullatif, Hala
Autoimmune hepatitis in Egyptian children: A single center experience
title Autoimmune hepatitis in Egyptian children: A single center experience
title_full Autoimmune hepatitis in Egyptian children: A single center experience
title_fullStr Autoimmune hepatitis in Egyptian children: A single center experience
title_full_unstemmed Autoimmune hepatitis in Egyptian children: A single center experience
title_short Autoimmune hepatitis in Egyptian children: A single center experience
title_sort autoimmune hepatitis in egyptian children: a single center experience
topic Original Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8894955/
https://www.ncbi.nlm.nih.gov/pubmed/35231187
http://dx.doi.org/10.1177/20587384211073265
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