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

Porta, Gilda; Carvalho, Elisa de; Santos, Jorge Luiz dos; Gama, Jorge; Borges, Cristian V.; Seixas, Renata Belém Pessôa de Melo; Ferreira, Alexandre Rodrígues; Miura, Irene Kazue; Silveira, Themis Reverbel da; Silva, Luciana Rodrígues; Fagundes, Eleonora Druve Tavares; Bellomo-Brandão, Maria Ângela; Sawamura, Regina; Vieira, S; Melere, Melina Utz; Marques, Cibele D.F.; Pugliese, Renata; Danesi, Vera Lúcia Baggio; Porta, Adriana; Marsillac, M. E. De; Valladares, Marcia A.; Menezes, Daniela G.; Kieling, Carlos Oscar; Paula, Mariana Nascimento de; Vasconcelos, Juliana R.; Ferreira, Cristina Targa; Perin, Nilza Medeiros; Resende, Leonardo R.; Maia, Jussara Melo de Cerqueira; Tommaso, Adriana María Alves De; Hessel, Gabriel

doi:10.1016/j.jped.2018.04.007

Cited by 29 publications

(30 citation statements)

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“…In this study, the MD was significantly lower in children with overlap syndrome. This may be attributed to the presence of associated pathological changes of the bile duct and the presence of cirrhotic changes [4][5][6][7]. This finding indicates that patients associated with the overlap syndrome have a more aggressive form of the disease.…”

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confidence: 93%

“…Autoimmune hepatitis (AIH) is a chronic autoimmune disease with progressive necro-inflammatory liver disease that presents with clinical presentation varying from minor symptoms to acute liver failure. AIH is characterized biochemically by high level of transaminase, histologically by the presence of interface hepatitis, and serologically by a high level of immunoglobulin G (IgG) [1][2][3][4]. AIH is further subdivided into type I and type II, depending on the autoantibody profile: AIH type I is characte-rized by positivity for the anti-nuclear antibody (ANA) and/or anti-smooth muscle antibody (ASMA), and AIH type II is characterized by the presence of anti-liver kidney microsomal antibody type I (anti-LKM1) and/or anti-liver cytosol type 1 [5][6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

“…procedure with risk of complications [3][4][5][6][7]. Limited studies discuss the role of shear-wave elastography, magnetic resonance (MR) elastography, and MR spectroscopy in the diagnosis and monitoring of patients with AIH, but these results are preliminary and overlapping [10][11][12][13].…”

Section: Introductionmentioning

confidence: 99%

“…interface hepatitis and rosette formation, and clinically ranging from asymptomatic to having liver failure. AIH is characterized by positive autoantibodies including ANA, ASMA, and anti-LKM1 and increased immunoglobulin G (IgG) [2][3][4][5][6][7].…”

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confidence: 99%

“…This may be due to the presence of interface hepatitis at the portal-parenchymal interface, with dense plasma cell-rich lymphoplasmacytic, infiltrates, hepatocellular regeneration with rosette formation, emperipolesis (active penetration by one cell into and through a larger cell), and hepatocyte swelling and/or pycnotic necrosis piecemeal necrosis with periportal/periseptal lymphocyte infiltrating the portal plasma cells. Fibrosis is present in all children with AIH apart from those with the mildest forms of the disease [2][3][4][5][6][7].…”

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confidence: 99%

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The role of diffusion tensor imaging of the liver in children with autoimmune hepatitis

et al. 2021

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Purpose:To evaluate the role of diffusion tensor imaging (DTI) of the liver in children with autoimmune hepatitis (AIH). Material and methods:A prospective study was done on 42 children with AIH (30 girls and 12 boys, with a mean age of 13 years) and 20 age-and sex-matched healthy control children. They underwent DTI of the liver and laboratory tests. Liver biopsy was done for the patients. The mean diffusivity (MD) and fractional anisotropy (FA) of the liver were calculated and correlated with the pathological results. Results:The mean MD and FA of the liver in children with AIH were 1.42 ± 0.06 × 10 -3 mm²/s and 0.37 ± 0.11; and in the control children they were 1.55 ± 0.07 × 10 -3 mm²/s and 0.25 ± 0.03, respectively. The MD and FA were significantly different in the children with AIH compared to the control children (p = 0.001). The cutoff MD and FA used to differentiate patients from controls were 1.50 × 10 -3 mm²/s, 0.31 with AUC of 0.919 and 0.813, sensitivity of 97.6% and 66.7%, a specificity of 80% and 70%, an accuracy of 94.2% and 67.3%, PPV of 95.3 and 90.3, and NPV of 88.9 and 33.3, respectively. There was significantly lower MD and higher FA of the liver in children with AIH type I (n = 31) than type II (n = 11) (p = 0.001), and patients with (n = 9) and without (n = 33) overlap syndrome (p = 0.005). Conclusions:We concluded that DTI parameters can help to diagnose AIH, detect its phenotyping, and give clues as to the presence of associated overlap syndrome.

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confidence: 93%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

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confidence: 99%

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The role of diffusion tensor imaging of the liver in children with autoimmune hepatitis

et al. 2021

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Particularities of Autoimmune Hepatitis in Latin America

Porta

Cançado

2020

Clin. Liver Dis.

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Current practice in the management of paediatric autoimmune liver disease in Europe

Cananzi,

Jørgensen,

Buescher

et al. 2024

J. pediatr. gastroenterol. nutr.

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ObjectivePaediatric autoimmune liver disease (pAILD) is a rare condition with serious health implications. Notwithstanding treatment advancements, areas of uncertainty and knowledge gaps still exist. We here investigated the real‐life approach to pAILD management in Europe.MethodsA survey was distributed to members of the RARE‐LIVER ERN and the ESPGHAN Hepatology Interest Group. Information was gathered regarding clinical activity, medications used, and access to paediatric drug formulations at each site.ResultsThirty‐six centres from 22 European countries responded to the survey. The majority are exclusively paediatric units (86%). Among participants, 80% follow <50 children with pAILD, of which 25%–50% are <10 years old in 44% of centres. All centres use predniso(lo)ne as first‐line therapy, alone (15/36) or with azathioprine (21/36). Azathioprine and mycophenolate are the preferred second‐line options in centres using first‐line steroid monotherapy (11/15) or combined steroid‐azathioprine (19/21), respectively. Tacrolimus is used as third‐line agent in 15/36 centres. Proactive measurement of drug metabolites and target levels vary widely among centres. Paediatric predniso(lo)ne formulations are commercially available in 7/22 European countries, azathioprine in 3, mycophenolate in 14, tacrolimus in 15 and ursodeoxycholic acid in 14. When paediatric formulations are unavailable, children are treated with magisterial preparations or ‘solid’ formulations (crushed or intact).ConclusionsTreatment of pAILD in Europe varies widely in terms of medications used and treatment monitoring. Availability of paediatric drug formulations across Europe is limited. Collaborative initiatives are needed to define evidence‐based strategies for management of pAILD and to promote an equal, age‐appropriate treatment for affected children.

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

Cited by 29 publications

References 24 publications

The role of diffusion tensor imaging of the liver in children with autoimmune hepatitis

The role of diffusion tensor imaging of the liver in children with autoimmune hepatitis

Particularities of Autoimmune Hepatitis in Latin America

Current practice in the management of paediatric autoimmune liver disease in Europe

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