Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes

• Main Author: Porta, Gilda
• Publication Date: 2019
• Other Authors: Carvalho, Elisa, Santos, Jorge Luiz Dos, Gama, Jorge, Borges, Cristian V., Seixas, Renata, Ferreira, Alexandre, Miura, Irene, Silveira, Themis Reverbel, Silva, Luciana, Fagundes, Eleonora, Brandao, MA Bellomo, Sawamura, Regina, Vieira, Sandra, Melere, Melina, Marques, Cibele D F, Pugliese, Renata P., Danesi, Vera, Porta, Adriana, Marsillac, Marise, Valladares, Marcia Angélica, Menezes, Daniela G., Kieling, Carlos, Paula, Mariana N. de, Vasconcelos, Juliana R., Ferreira, Cristina T., Perin, Nilza, Resende, Leonardo R., Maia, Jussara, Tommaso, Adriana M.A. De, Hessel, Gabriel
• Format: Article
• Language: eng
• Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
• Download full: http://hdl.handle.net/10400.6/9045
• topic: Autoimmune hepatitis; Clinical; Laboratory; Treatments; Outcomes; Brazil

sentation, laboratory findings, histological profile, treatments, and outcomes of children andadolescents with autoimmune hepatitis.Methods: The medical records of 828 children and adolescents with autoimmune hepatitiswere reviewed. A questionnaire was used to collect anonymous data on clinical presentation,biochemical and histological findings, and treatments.Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmunehepatitis-2. The female sex was predominant in both groups. The median age at symptomonset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher forautoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patientswith autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-foldhigher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were sig-nificantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels werelower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochem-ical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6%underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmunehepatitis-2: 2.4%).Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmunehepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higherdisease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.