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

被引：14

作者：

Porta, Gilda ^{[1
]}

de Carvalho, Elisa ^{[2
]}

Santos, Jorge L. ^{[3
]}

Gama, Jorge ^{[4
]}

Borgesa, Cristian, V ^{[1
]}

Seixas, Renata B. P. M. ^{[5
]}

Ferreira, Alexandre R. ^{[6
]}

Miura, Irene K. ^{[1
]}

Silveira, Themis R. ^{[7
]}

Silva, Luciana R. ^{[8
]}

Fagundes, Eleonora D. T. ^{[6
]}

Bellomo-Brandao, Maria A. ^{[9
]}

Sawamura, Regina ^{[10
]}

Vieira, Sandra M. ^{[11
]}

Melere, Melina U. ^{[7
]}

Marques, Cibele D. F. ^{[8
]}

Pugliese, Renata P. ^{[1
]}

Danesi, Vera L. ^{[1
]}

Porta, Adriana ^{[1
]}

Marsillac, Marise E. ^{[12
,13
]}

Valladares, Marcia A. ^{[14
]}

Menezes, Daniela G. ^{[15
]}

Kieling, Carlos ^{[11
]}

de Paula, Mariana N. ^{[16
]}

Vasconcelos, Juliana R. ^{[17
]}

Ferreira, Cristina T. ^{[7
]}

Perin, Nilza ^{[18
]}

Resende, Leonardo R. ^{[19
]}

Maia, Jussara ^{[20
]}

De Tommaso, Adriana M. A. ^{[9
]}

Hessel, Gabriel ^{[9
]}

机构：

[1] Hosp Sirio Libanes, Hosp Menino Jesus, Grp Hepatol & Transplante Pediat, Sao Paulo, SP, Brazil

[2] Hosp Base Dist Fed, Hosp Crianca Brasilia, Brasilia, DF, Brazil

[3] Univ Beira Interior, Fac Ciencias Saude, Ctr Pesquisa Ciencias Saude CICS, Covilha, Portugal

[4] Univ Beira Interior, Ctr Matemat & Aplicacoes, Dept Matemat, Covilha, Portugal

[5] Hosp Base Dist Fed, Hosp Crianco Brasilia, Dept Gastroenterol Pediat, Brasilia, DF, Brazil

[6] Univ Fed Minas Gerais, Dept Gastroenterol & Hepatol Pediat, Belo Horizonte, MG, Brazil

[7] Hosp Santo Antonio, Dept Gastroenterol & Hepatol Pediat, Porto Alegre, RS, Brazil

[8] Univ Fed Bahia UFBA, Dept Gastroenterol & Hepatol Pediat, Salvador, BA, Brazil

[9] Univ Estadual Campinas, UNICAMP, Dept Gastroenterol & Hepatol Pediat, Campinas, SP, Brazil

[10] Univ Sao Paulo, FMRP, Dept Gastroenterol & Hepatol Pediat, Ribeirao Preto, SP, Brazil

[11] Univ Fed Rio Grande do Sul, Dept Gastroenterol Pediat, Porto Alegre, RS, Brazil

[12] Univ Estado Rio De Janeiro, Dept Gastroenterol Pediat, Rio De Janeiro, RJ, Brazil

[13] Hosp Fed Servidores Estado, Rio De Janeiro, RJ, Brazil

[14] Univ Fed Rio de Janeiro, Dept Gastroenterol & Hepatol Pediat, Rio de Janeiro, Brazil

[15] Univ Fed Sergipe, Dept Gastroenterol & Hepatol Pediat, Sao Cristovao, SE, Brazil

[16] Irmandade Santa Casa Misericordia Sao Paulo, Dept Gastroenterol & Hepatol Pediat, Sao Paulo, SP, Brazil

[17] Univ Fed Paraiba UFPB, Dept Gastroenterol & Hepatol Pediat, Joao Pessoa, Paraiba, Brazil

[18] Hosp Infantil Joana Gusmao, Dept Gastroenterol & Hepatol Pediat, Florianopolis, SC, Brazil

[19] Univ Fed Mato Grosso do Sul UFMS, Dept Gastroenterol & Hepatol Pediat, Campo Grande, MS, Brazil

[20] Univ Fed Rio Grande do Norte UFRN, Dept Gastroenterol & Hepatol Pediat, Natal, RN, Brazil

来源：

JORNAL DE PEDIATRIA | 2019年 / 95卷 / 04期

关键词：

Autoimmune hepatitis; Clinical; Laboratory; Treatments; Outcomes; Brazil; PRIMARY SCLEROSING CHOLANGITIS; DIAGNOSTIC-CRITERIA; LIVER-TRANSPLANTATION; MANAGEMENT; SPECTRUM;

D O I：

10.1016/j.jped.2018.04.007

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Objective: This large study with a tong-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. Methods: The 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. Results: Of 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%). Conclusions: In 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. (C) 2018 Published by Elsevier Editora Ltda. on behalf of Sociedade Brasiteira de Pediatria.

引用

页码：419 / 427

页数：9

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