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Background: There is limited evidence focusing on the use of non-invasive markers to assess liver fibrosis in pediatric chronic liver diseases., Objective: The aim of the study was to evaluate the correlation between liver stiffness measurement (LSM) using transient liver elastography (TLE) and serum non-invasive markers in pediatric autoimmune hepatitis (AIH)., Methods: Cross-sectional study between May 2016 and February 2020. Twenty-eight children with AIH (age range between 3 and 20 years old) who had undergone TLE and had routine serum tests were enrolled. Patients were divided into 2 groups: (1) Liver fibrosis degree ≥F2 (TLE ≥6.45 kPa) and (2)

Objective: This 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., 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., (Copyright © 2018. Published by Elsevier Editora Ltda.)

Context: Zinc deficiency in children and adolescents impairs their growing, development and immune system., Objectives: To verify the existence of plasma and leukocyte zinc deficiency in adolescents with autoimmune hepatitis., Methods: The study comprised 23 patients with autoimmune hepatitis, aged 10-18 years, assisted at the Ambulatory Service of Pediatric Hepatology of the University of Campinas Teaching Hospital, Campinas, SP, Brazil, and adolescents with ages compatible with the patients' ages comprised the control group. Sample of blood in both groups was collected for the analyses of plasma zinc and leukocyte zinc by atomic absorption spectrophotometry, beyond the nutritional status was evaluated in each adolescent. The following statistical tests were used: Mann-Whitney, Spearman's correlation and interclass concordance analysis., Results: The significance level adopted was 5%. The average zinc level in plasma in patients was 71.91 ± 11.79 µg/dL and, in the control group, it was 80.74 ± 10.92 µg/dL, showing a significant difference (P = 0.04). The leukocyte zinc level in patients was 222.33 ± 166.13 pmol/10⁶ cells and, in the control group, it was 226.64 ± 217.81 pmol/10⁶ cells; there was no statistical significance between them (P = 0.45)., Conclusion: The evaluation of the nutritional status showed that eutrophy is prevalent in patients, and they presented a higher body fat value than the control group, with a significant difference. More research is needed with adolescents with autoimmune hepatitis regarding levels of essential micronutrients, such as zinc, because a good nutritional status can improve the prognostic of liver disease.

Context: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status., Objectives: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity., Methods: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients underwent anthropometric evaluation, alimentary inquiry and determination of serum levels of vitamins A, D and E by high performance liquid chromatography. The Mann-Whitney test, Spearman correlation coefficients and variance analysis (ANOVA) were utilized for data treatment, regarding significant difference if P<0.05., Results: The highest nutritional deficit was observed in patients with biliary atresia, mainly with cholestasis. The serum levels of vitamins A and E for the reference group changed as a function of age. The serum levels of vitamins A, D and E were higher in reference group than in patients with biliary atresia and autoimmune hepatitis together or separately. There were not difference in the serum levels of vitamins A, D and E between biliary atresia groups with cholestasis and without cholestasis. It was verified correlation between weight/age, triceps skinfold thickness, subscapular skinfold thickness, midarm circumference, midarm fat area values and vitamin A serum levels, as well as between all anthropometric indicators and vitamin E in patients with autoimmune hepatitis and biliary atresia., Conclusion: The patients with biliary atresia and cholestasis presented the highest nutritional injury. The patients with biliary atresia and autoimmune hepatitis presented lower serum levels of vitamins A, D and E that in control group. There is a directly proportional correlation between vitamin serum levels, mainly vitamin E, and all anthropometric variables of biliary atresia and autoimmune hepatitis groups.

Background: Few studies on autoimmune hepatitis have enrolled non-Caucasian groups., Aims: To evaluate Brazilian children with type 1 and 2 autoimmune hepatitis regarding outcome and clinical and biochemical parameters., Patients and Methods: Thirty-six patients were submitted to a protocol that evaluated the clinical history, physical and biochemical data, and the course of the disease. Twenty-four children had type 1 autoimmune hepatitis, seven had type 2 and five had unclassified autoimmune hepatitis. Most patients were females (77%), with a median age at diagnosis of 11 years, and the median duration of symptoms was 5.5 and 8 months for types 1 and 2, respectively. Jaundice and choluria were the most common clinical manifestations., Results: Treatment with azathioprine and prednisone was successful in patients with type 1 and 2 autoimmune hepatitis. AST and ALT decreased after 4 to 8 weeks of treatment compared to pretreatment levels in type 1 autoimmune hepatitis. Increased GGT values returned to pretreatment levels after 1 year in the two types. Three patients died and three other patients underwent liver transplantation., Conclusions: Non-Caucasian children had a similar disease when compared to Caucasian ones with autoimmune hepatitis. Increased levels of GGT during the first year of treatment should not be the only parameter for the indication of cholangiopathy.

Background: Posterior subcapsular cataract (PSC) has been reported in patients on corticosteroids therapy for various pathologies. Autoimmune hepatitis (AIH) is a progressive inflammatory hepatic disease for which corticosteroids are recommended but PSC has not yet been reported in AIH patients on corticosteroids therapy., Methods: The AIH patients at our service underwent an ophthalmological assessment and their files were analyzed establish the dosage and duration of medication, the presence of visual complaints and the type of AIH, in order to correlate these with the ophthalmological findings. Patients were examined with a slit lamp following pupil dilation., Results: 17 AIH patients evaluated, only one patient had PSC., Conclusion: Although the frequency of PSC was low (1/17) in AIH patients on costicosteroid therapy, these individuals should undergo occasional ophthalmological assessment for the presence of cataracts since their visual acuity and quality of life may be adversely affected.