Acute onset of autoimmune hepatitis in children and adolescents

作     者：Vratislav Smolka Oksana Tkachyk Jiri Ehrmann Eva Karaskova Martin Zapalka Jana Volejnikova Vratislav Smolka;Oksana Tkachyk;Jiri Ehrmann;Eva Karaskova;Martin Zapalka;Jana Volejnikova

作者机构：Department of PediatricsFaculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 185/6Olomouc 77900Czech Republic Department of Clinical and Molecular PathologyFaculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 185/6Olomouc 77900Czech Republic 

出 版 物：《Hepatobiliary & Pancreatic Diseases International》 (国际肝胆胰疾病杂志（英文版）)

年 卷 期：2020年第19卷第1期

页      面：17-21页

核心收录：

学科分类：1002[医学-临床医学] 100202[医学-儿科学] 10[医学] 

基　　金：supported by grants from the European Re-gional Development Fund-Project ENOCH(CZ.02.1.01/0.0/0.0/16_019/0000868) the Ministry of Health,Czech Republic–conceptual development of research organization(MH DRO grant FNOL,0098892) 

主　　题：Acute liver failure Autoimmune hepatitis Children Onset 

摘      要：Background: Autoimmune hepatitis (AIH) is a rare progressive liver disease, which manifests as acute hepatitis in 40%-50% of pediatric cases. This refers predominantly to spontaneous exacerbations of previ- ously unrecognized subclinical AIH with laboratory and histological signs of chronic hepatitis, or to acute exacerbations of known chronic disease. Only a few of these patients ful ll criteria for acute liver failure (ALF). Methods: Forty children diagnosed with AIH in our center between 2000 and 2018 were included in this study. All of them ful lled revised diagnostic criteria of the International Autoimmune Hepatitis Group (IAIHG) for probable or con rmed AIH, and other etiologies of liver diseases were excluded. Patients were divided into two groups: acute AIH (A-AIH) or chronic AIH (C-AIH). Results: Acute onset of AIH occurred in 19/40 children (48%). Six of them ful lled the criteria of ALF with coagulopathy and encephalopathy. Five of 6 children with ALF suffered from exacerbation of previ- ously undiagnosed chronic AIH, among which 4 children were histologically con rmed as micronodular cirrhosis. The remaining one patient had fulminant AIH with centrilobular necrosis, but no histological signs of previous chronic liver damage. We observed signi cantly lower levels of albumin, higher levels of aminotransferases, bilirubin, INR, IgG, higher IAIHG score and more severe histological ndings in A- AIH than in C-AIH. No differences in patient age and presence of autoantibodies were observed between A-AIH and C-AIH. All children, including those with ALF and cirrhosis, were treated with corticosteroids, and are alive and achieved AIH remission. Liver transplant was not indicated in any patient. Conclusion: Rapid and accurate diagnosis of A-AIH may be di cult. However, timely start of immunosup-pressive therapy improves prognosis and decreases number of indicated liver transplantations in children with AIH.