Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings,histological profile,treatments, and outcomes |
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| Authors: | Gilda Porta Elisa de Carvalho Jorge L. Santos Jorge Gama Cristian V. Borges Renata B.P.M. Seixas Alexandre R. Ferreira Irene K. Miura Themis R. Silveira Luciana R. Silva Eleonora D.T. Fagundes Maria A. Bellomo-Brandao Regina Sawamura Sandra M. Vieira Melina U. Melere Cibele D.F. Marques Renata P. Pugliese Vera L. Danesi Gabriel Hessel |
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| Affiliation: | 1. Hospital Sírio Libanês, Hospital Menino Jesus, Grupo de Hepatologia e Transplante Pediátrico, São Paulo, SP, Brazil;2. Hospital de Base do Distrito Federal, Hospital da Criança de Brasília, Departamento de Gastroenterologia e Hepatologia, Brasília, DF, Brazil;3. Universidade da Beira Interior, Faculdade de Ciências da Saúde, Centro de Pesquisa em Ciências da Saúde (CICS-UBI), Covilhã, Portugal;4. Universidade da Beira Interior, Centro de Matemática e Aplicações, Departamento de Matemática, Covilhã, Portugal;5. Hospital de Base do Distrito Federal, Hospital da Criança de Brasília, Departamento de Gastroenterologia Pediátrica, Brasília, DF, Brazil;6. Universidade Federal de Minas Gerais (UFMG), Departamento de Gastroenterologia e Hepatologia Pediátrica, Belo Horizonte, MG, Brazil;7. Hospital Santo Antônio, Departamento de Gastroenterologia e Hepatologia Pediátrica, Porto Alegre, RS, Brazil;8. Universidade Federal da Bahia (UFBA), Departamento de Gastroenterologia e Hepatologia Pediátrica, Salvador, BA, Brazil;9. Universidade Estadual de Campinas (Unicamp), Departamento de Gastroenterologia e Hepatologia Pediátrica, Campinas, SP, Brazil;10. Universidade de São Paulo (USP), Faculdade de Medicina de Ribeirão Preto (FMRP), Departamento de Gastroenterologia e Hepatologia Pediátrica, Ribeirão Preto, SP, Brazil;11. Universidade Federal do Rio Grande do Sul (UFRGS), Unidade de Transplante de Fígado, Porto Alegre, RS, Brazil;12. Universidade do Estado do Rio de Janeiro (UERJ), Departamento de Gastroenterologia Pediátrica, Rio de Janeiro, RJ, Brazil;13. Hospital Federal dos Servidores do Estado, Rio de Janeiro, RJ, Brazil;14. Universidade Federal do Rio de Janeiro (UFRJ), Departamento de Gastroenterologia e Hepatologia Pediátrica, Rio de Janeiro, RJ, Brazil;15. Universidade Federal de Sergipe (UFS), Departamento de Gastroenterologia e Hepatologia Pediátrica, São Cristóvão, SE, Brazil;p. Irmandade da Santa Casa Misericórdia de São Paulo, Departamento de Gastroenterologia e Hepatologia Pediátrica, São Paulo, SP, Brazil;q. Universidade Federal da Paraíba (UFPB), Departamento de Gastroenterologia e Hepatologia Pediátrica, João Pessoa, PB, Brazil;r. Hospital Infantil Joana de Gusmão, Departamento de Gastroenterologia e Hepatologia Pediátrica, Florianópolis, SC, Brazil;s. Universidade Federal de Mato Grosso do Sul (UFMS), Departamento de Gastroenterologia Pediátrica, Campo Grande, MS, Brazil;t. Universidade Federal do Rio Grande do Norte (UFRN), Departamento de Gastroenterologia e Hepatologia Pediátrica, Natal, RN, Brazil |
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| Abstract: | ObjectiveThis large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis.MethodsThe medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments.ResultsOf all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical 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%; autoimmune hepatitis-2: 2.4%).ConclusionsIn this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death. |
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| Keywords: | Corresponding author. Autoimmune hepatitis Clinical Laboratory Treatments Outcomes Brazil Hepatite autoimune Clínico Laboratorial Tratamentos Resultados Brasil |
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