Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral Hepatitis

Untreated autoimmune hepatitis (AIH) can develop into liver cirrhosis, with potentially fatal outcomes. Viral hepatitis in children was endemic in Taiwan before universal hepatitis B vaccination, but AIH has rarely been reported in Taiwanese children. We performed this retrospective study to charact...

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Main Authors: Sheng-Hsiung Yeh, Yen-Hsuan Ni, Yung-Ming Jeng, Huey-Ling Chen, Jia-Feng Wu, Mei-Hwei Chang
Format: Article
Language:English
Published: Elsevier 2009-04-01
Series:Pediatrics and Neonatology
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S1875957209600358
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author Sheng-Hsiung Yeh
Yen-Hsuan Ni
Yung-Ming Jeng
Huey-Ling Chen
Jia-Feng Wu
Mei-Hwei Chang
author_facet Sheng-Hsiung Yeh
Yen-Hsuan Ni
Yung-Ming Jeng
Huey-Ling Chen
Jia-Feng Wu
Mei-Hwei Chang
author_sort Sheng-Hsiung Yeh
collection DOAJ
description Untreated autoimmune hepatitis (AIH) can develop into liver cirrhosis, with potentially fatal outcomes. Viral hepatitis in children was endemic in Taiwan before universal hepatitis B vaccination, but AIH has rarely been reported in Taiwanese children. We performed this retrospective study to characterize the clinical features of AIH in Taiwanese children. Methods: We enrolled children with AIH, based on the revised scoring system of the International Autoimmune Hepatitis Group (IAIHG) from 383 children hospitalized with hepatitis from January 2000 to April 2008. Other etiologies of hepatitis were excluded. Results: There were three definite and six probable AIH cases. The incidence of AIH among children hospitalized with hepatitis was 2.3%. Eight children had other autoimmune diseases, including systemic lupus erythematosus (SLE) (6), discoid lupus erythematosus (DLE) (1), and autoimmune polyendocrinopathy syndrome type 1 (1). Another had biliary atresia, and AIH developed after cadaveric liver transplantation. Antinuclear antibodies ranged from 1:160-1:2560. Peak alanine aminotransferase (ALT) values were 546 ± 188 U/L (mean ± SD). Jaundice occurred in four patients. Liver histology in the three definite AIH patients showed chronic hepatitis with predominantly lymphoplasmacytic infiltrates in portal areas, with prominent interface activity. Treatment included prednisolone, azathioprine, and/or cyclosporine. All patients survived. ALT fell to < 60 U/L after treatment. Hepatitis relapse occurred in one patient. Conclusion: AIH in Taiwanese children is commoner than previously thought. It is associated with other autoimmune diseases and may occur before, simultaneously with, or after other autoimmune diseases. Children with liver transplants are also at risk of AIH.
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spelling doaj.art-51c5633c9d8c41048b319df7115f30742022-12-21T19:05:46ZengElsevierPediatrics and Neonatology1875-95722009-04-01502656910.1016/S1875-9572(09)60035-8Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral HepatitisSheng-Hsiung Yeh0Yen-Hsuan Ni1Yung-Ming Jeng2Huey-Ling Chen3Jia-Feng Wu4Mei-Hwei Chang5Department of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanDepartment of Pathology, National Taiwan University Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanUntreated autoimmune hepatitis (AIH) can develop into liver cirrhosis, with potentially fatal outcomes. Viral hepatitis in children was endemic in Taiwan before universal hepatitis B vaccination, but AIH has rarely been reported in Taiwanese children. We performed this retrospective study to characterize the clinical features of AIH in Taiwanese children. Methods: We enrolled children with AIH, based on the revised scoring system of the International Autoimmune Hepatitis Group (IAIHG) from 383 children hospitalized with hepatitis from January 2000 to April 2008. Other etiologies of hepatitis were excluded. Results: There were three definite and six probable AIH cases. The incidence of AIH among children hospitalized with hepatitis was 2.3%. Eight children had other autoimmune diseases, including systemic lupus erythematosus (SLE) (6), discoid lupus erythematosus (DLE) (1), and autoimmune polyendocrinopathy syndrome type 1 (1). Another had biliary atresia, and AIH developed after cadaveric liver transplantation. Antinuclear antibodies ranged from 1:160-1:2560. Peak alanine aminotransferase (ALT) values were 546 ± 188 U/L (mean ± SD). Jaundice occurred in four patients. Liver histology in the three definite AIH patients showed chronic hepatitis with predominantly lymphoplasmacytic infiltrates in portal areas, with prominent interface activity. Treatment included prednisolone, azathioprine, and/or cyclosporine. All patients survived. ALT fell to < 60 U/L after treatment. Hepatitis relapse occurred in one patient. Conclusion: AIH in Taiwanese children is commoner than previously thought. It is associated with other autoimmune diseases and may occur before, simultaneously with, or after other autoimmune diseases. Children with liver transplants are also at risk of AIH.http://www.sciencedirect.com/science/article/pii/S1875957209600358autoimmune hepatitischildrenliver transplantationTaiwan
spellingShingle Sheng-Hsiung Yeh
Yen-Hsuan Ni
Yung-Ming Jeng
Huey-Ling Chen
Jia-Feng Wu
Mei-Hwei Chang
Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral Hepatitis
Pediatrics and Neonatology
autoimmune hepatitis
children
liver transplantation
Taiwan
title Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral Hepatitis
title_full Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral Hepatitis
title_fullStr Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral Hepatitis
title_full_unstemmed Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral Hepatitis
title_short Emerging Importance of Autoimmune Hepatitis in Children in Taiwan, an Endemic Area for Viral Hepatitis
title_sort emerging importance of autoimmune hepatitis in children in taiwan an endemic area for viral hepatitis
topic autoimmune hepatitis
children
liver transplantation
Taiwan
url http://www.sciencedirect.com/science/article/pii/S1875957209600358
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