Journal: Advances in Medicine and Engineering Interdisciplinary Research DOI: 10.32629/ameir.v4i3.5464
Abstract
Introduction. Autoimmune hepatitis is a progressive inflammatory liver disease that is rare in children, characterized by inflammation in liver histology, the presence of auto-antibodies and high levels of immunoglobulin G. There are few data in children and in some studies performed in Latin America, but limited in Colombia. The aim of this study is to describe the demographic, clinical, paraclinical and therapeutic data as well as, the outcome of paediatric patients diagnosed with autoimmune hepatitis in a tertiary care centre in south-western Colombia. Methods. This is a descriptive study of a retrospective cohort of paediatric patients (≤ 18 years) with a diagnosis of autoimmune hepatitis, attended in a third-level hospital in Colombia between 2011 and 2019. Statistical analyses were performed using the program Stata 14.0. Results. A total of 40 patients were included, 57.5% were female, with a median age of 10.5 years (IQR 4-13). The 62.5% presented with acute hepatitis, 20% had liver failure, a 27.5% with cirrhosis at diagnosis; 75% presented compatible liver biopsy, and 82.5% were classified as type 1 autoimmune hepatitis. All patients received a steroid plus immunomodulator; presenting biochemical remission in 75% relapse in the 22.5%. Four of the patients required liver transplantation. Conclusions. Autoimmune hepatitis is an infrequent entity, although not negligible, in children in our environment. A higher prevalence of type I autoimmune hepatitis was found. Most patients were female and presented with symptoms of acute hepatitis at the initial diagnosis. The characteristics and clinical responses, in this cohort, are similar to those described in different series in the world population.
Keywords
autoimmune hepatitis; children; liver
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[2] Heneghan MA, Yeoman AD, Verma S, et al. Autoimmune hepatitis. The Lancet. Elsevier BV. 2013;382:1433-44.
[3] Mieli-Vergani G, Vergani D, Baumann U, et al. Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESP-GHAN Hepatology Committee Position Statement. J Pediatr Gastroenterol Nutr. 2018;66:345-60.
[4] Roberts EA. Autoimmune hepatitis from the paediatric perspective. Liver Int. 2011;31:1424-31.
[5] Mieli-Vergani G, Vergani D. Paediatric autoimmune liver disease. Arch Dis Child. 2013;98:1012-7.
[6] Wang Q, Yang F, Miao Q, et al. The clinical phenotypes of autoimmune hepatitis: A comprehensive review. J Autoimmun. 2016;66:98-107.
[7] Della Corte C, Sartorelli MR, Sindoni CD, et al. Autoimmune hepatitis in children: An overview of the disease focusing on current therapies. Eur J Gastroenterol Hepatol. 2012;24:739-46.
[8] Wong RJ, Gish R, Frederick T, et al. The Impact of Race/Ethnicity on the Clinical Epidemiology of Autoimmune Hepatitis. J Clin Gastroenterol. 2012;46:155-61.
[9] Radhakrishnan KR, Alkhouri N, Worley S, et al. Autoimmune hepatitis in children.
[10] Palle SK, Naik KB, McCracken CE, et al. Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis. Liver Int. 2019;39:976-84.
[11] Maldonado Gómez H, Sepúlveda CE, Subdirector R, et al. República De Colombia Departamento Administrativo Nacional De Estadística. DANE. 2009:28-38. 1997;40:283-6.
[12] Nares-Cisneros J, Jaramillo-Rodríguez Y. Hepatitis autoinmune en niños: Evolución de 20 casos del norte de México. Rev Gastroenterol Mex. 2014;79:238-43.
[13] Costaguta A, González A, Pochettino S, et al. Incidence and Clinical Features of Autoimmune Hepatitis in the Province of Santa Fe (Argentina). J Pediatr Gastroenterol Nutr. 2018;67:e107-e110.
[14] Goldberg AC, Bittencourt PL, Oliveira LC, et al. Autoimmune Hepatitis in Brazil: An Overview. Scand J Immunol. 2007;66:208-16.
[15] Porta G, Carvalho E de, Santos JL, et al. Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes. J Pediatr, Rio J. 2019;95:419-27.
[16] Suárez D, Andrade R, Vera JF, et al. Clinical and histopathological characterization of children with autoimmune hepatitis at a referral center in Bogotá, Colombia. Rev Colomb Gastroenterol. 2019;34:364-9.
[17] Mileti E, Rosenthal P, Peters MG. Validation and Modification of Simplified Diagnostic Criteria for Autoimmune Hepatitis in Children. Clin Gastroenterol Hepatol. 2012;10:417-21.e2.
[18] Lee WS, Lum SH, Lim CB, et al. Characteristics and outcome of autoimmune liver disease in Asian children. Hepatol Int. 2015;9:292-302.
[19] Al-Chalabi T, Underhill JA, Portmann BC, et al. Impact of gender on the long-term outcome and survival of patients with autoimmune hepatitis. J Hepatol. 2008;48:140-7.
[20] Jossen J, Annunziato R, Kim HS, et al. Liver transplantation for children with primary sclerosing cholangitis and autoimmune hepatitis: UNOS database analysis. J Pediatr Gastroenterol Nutr. 2017;64:e83-e87.
[21] Perkins JD. Techniques to ensure adequate portal flow in the presence of splenorenal shunts. Liver Transplant. 2007;13:767-8.
[22] Van Gerven NMF, Verwer BJ, Witte BI, et al. Relapse is almost universal after withdrawal of immunosuppressive medication in patients with autoimmune hepatitis in remission. J Hepatol. 2013;58:141-7.
[23] Saadah OI, Smith AL, Hardikar W. Long-term outcome of autoimmune hepatitis in children. J Gastroenterol Hepatol. 2001;16:1297-302.
[24] Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: A 20-year experience. Hepatology. 1997;25:541-7.
[25] Ferreira AR, Roquete MLV, Toppa NH, et al. Effect of treatment of hepatic Histopathology.
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