Characteristics and outcome of autoimmune liver disease in Asian children

Hepatology International
Way Seah LeeJayalakshmi Pailoor

Abstract

Little is known about autoimmune liver disease (AILD) in Asian children. We studied the clinical features and predictors of outcome in childhood AILD in an Asian population. Retrospective review of AILD [autoimmune hepatitis type 1 and 2 (AIH1, AIH2), primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC)] seen at two pediatric liver units in Malaysia. At presentation, 17 (56%) of the 32 children [19 females, 59%; median (range) age 7.7 (1.8-15.5) years] with AILD (AIH1 = 18, AIH2 = 5, PSC = 0, ASC = 9) had liver cirrhosis. At final review [median (range) duration of follow-up 4.8 (0.4-12) years], 24 patients (75%) survived with a native liver. Twenty-one (66%) were in remission; 19 (AIH1 = 11; AIH2 = 4, ASC = 4) were on prednisolone and/or azathioprine, one on cyclosporine and another on mycophenolate mofetil. Three (AIH1 = 3) were in partial remission. Of the two who underwent liver transplantation (LT; 6.5%; both ASC), one died of primary graft failure after LT. Six patients (19%) died without LT (acute liver failure, n = 1; end-stage liver disease, n = 5). The overall survival rate (native liver and survival post-LT) was 78%. A delay in seeking treatment adversely affected the final outcome [surviv...Continue Reading

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Citations

Mar 20, 2015·Hepatology International·Giorgina Mieli-Vergani, Diego Vergani
Nov 8, 2017·Journal of Pediatric Gastroenterology and Nutrition·Giorgina Mieli-VerganiNedim Hadzic
Aug 14, 2019·Hepatology Research : the Official Journal of the Japan Society of Hepatology·Shuichiro UmetsuTomoo Fujisawa
Jan 17, 2020·Journal of Tropical Pediatrics·Sawsan YassinElizabeth Goddard
Jun 3, 2016·European Journal of Gastroenterology & Hepatology·Miray KarakoyunFunda Ozgenc
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Jan 23, 2018·Journal of Pediatric Gastroenterology and Nutrition·Giorgina Mieli-VerganiNedim Hadžić
Oct 4, 2017·World Journal of Gastroenterology : WJG·Benedetta Terziroli Beretta-PiccoliDiego Vergani
Jun 24, 2017·Journal of Pediatric Gastroenterology and Nutrition·Andréanne N ZizzoBinita M Kamath
Aug 17, 2020·Journal of Pediatric Gastroenterology and Nutrition·Isaiah G RoepeTamir Miloh

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