Chronic active autoimmune hepatitis in children

Fainboim, Leonardo; Marcos, Y.; Pando, Marcelo J.; Capucchio, M.; Reyes, Gloria B.; Galoppo, Cristina; Badia, I.; Remondino, Graciela; Ciocca, Mirta; Ramonet, Margarita; Fainboim, Hugo; Satz, M. L.

doi:10.1016/0198-8859(94)90008-6

Cited by 107 publications

(13 citation statements)

References 11 publications

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“…Though most were of pediatric age group, disease had already been very advanced. This trend is similar to what reported in South American children with AIH (35, 36). One third of our patients had decompensated liver disease.…”

Section: Discussionsupporting

confidence: 90%

Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan

et al. 2013

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BackgroundHuman leukocyte antigen (HLA) typing in autoimmune hepatitis (AIH) has been investigated in different populations and ethnic groups, but no such data is available from Pakistan.ObjectivesThe aim of this study was to evaluate the clinical profile of autoimmune hepatitis (AIH), and determine the associated antigens and alleles by performing HLA typing.Patients and MethodsA total of 58 patients, diagnosed and treated as AIH in the last 10 years were reviewed. Diagnosis was based on International AIH Group criteria. Forty one patients underwent liver biopsy. HLA typing was performed in 44 patients and 912 controls by serological method for HLA A and B, and by PCR technique using sequence specific primers for DR alleles.ResultsOf 58 cases, 35 were females (60.3%). The median age was 14.5 (range 4-70 years), and AIH score was 14 (10-22). Thirty-six (62.0%) patients had type 1 AIH, 10 (17.2%) type 2, and the remaining 12 were seronegative with biopsy proven AIH. Forty-nine patients (84.4%) had cirrhosis. Twenty-four (41.4%) patients had ascites at the time of presentation. Among 41 patients who underwent liver biopsy, thirty-two had advance stages III and IV disease, and twenty had severe grade of inflammation. Fifteen patients had other associated autoimmune diseases and one developed hepatocellular carcinoma. HLA A2 (P = 0.036), HLA A9 (23) (P = 0.018), HLA A10 (25) (P = 0.000), HLA A19 (33) (P = 0.000), HLA B15 (63) (P = 0.007), HLA B40 (61) ( P = 0.002), HLA DR6 (P = 0.001) with its subtypes HLA-DRB1*13 (P = 0.032) and HLA-DRB1*14 (p = 0.017) were more prevalent in AIH with statistical significance than controls.ConclusionsAIH in our region presents with advanced disease affecting predominantly children and adolescents. There is a genetic association of HLA DR6 along with other alleles and antigens in our patients with AIH.

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Section: Discussionsupporting

confidence: 90%

Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan

et al. 2013

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“…In addition, studies in human papillomavirus (HPV) infection have associated DRB1*13 with protection against the HPV-16 serotype, and negative correlation of DRB1*13 with the occurrence of cervical carcinoma (334). By contrast, DRB1*13 enrichment has been reported in subjects with autoimmune diseases (335, 336). DRB1*1303 might, therefore, share some similarities with the HLA-Ia allele B*2705, which is associated with protection in HIV and HCV infection but is linked to autoimmune disorders (337).…”

Section: Diversity Of Hla-class II Genes and Their Influence On Viremmentioning

confidence: 85%

Human Leukocyte Antigen (HLA) and Immune Regulation: How Do Classical and Non-Classical HLA Alleles Modulate Immune Response to Human Immunodeficiency Virus and Hepatitis C Virus Infections?

2017

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The genetic factors associated with susceptibility or resistance to viral infections are likely to involve a sophisticated array of immune response. These genetic elements may modulate other biological factors that account for significant influence on the gene expression and/or protein function in the host. Among them, the role of the major histocompatibility complex in viral pathogenesis in particular human immunodeficiency virus (HIV) and hepatitis C virus (HCV), is very well documented. We, recently, added a novel insight into the field by identifying the molecular mechanism associated with the protective role of human leukocyte antigen (HLA)-B27/B57 CD8+ T cells in the context of HIV-1 infection and why these alleles act as a double-edged sword protecting against viral infections but predisposing the host to autoimmune diseases. The focus of this review will be reexamining the role of classical and non-classical HLA alleles, including class Ia (HLA-A, -B, -C), class Ib (HLA-E, -F, -G, -H), and class II (HLA-DR, -DQ, -DM, and -DP) in immune regulation and viral pathogenesis (e.g., HIV and HCV). To our knowledge, this is the very first review of its kind to comprehensively analyze the role of these molecules in immune regulation associated with chronic viral infections.

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“…However, distinct differences in the pattern of association between genetic susceptibility and type 1 AIH are seen in populations from various geographical regions. For example, type 1 AIH is associated with HLA-DRB1*04:05 in Japan and Argentina (Seki et al 1992; Pando et al 1999), with HLA-DRB1*13:01 in Brazil and Venezuela (Fainboim et al 1994; Czaja et al 2002; Fortes Mdel et al 2007) and with HLA-DRB1*04:04 in Mexico (Vazquez-Garcia et al 1998). Polymorphisms in genes outside the HLA locus have also been associated with type 1 AIH.…”

Section: Introductionmentioning

confidence: 99%

Association of the HLA locus and TNF with type I autoimmune hepatitis susceptibility in New Zealand Caucasians

et al. 2013

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PurposeThe precise etiology of autoimmune hepatitis (AIH) remains unknown, although a number of genetic loci have been implicated in the susceptibility of type 1 AIH. The purpose of this study was to test for association of these loci with type 1 AIH in New Zealand Caucasians.Methods77 AIH patients and 485 healthy controls were genotyped for the SNPs rs2187668 (HLA-DRB*03:01), rs660895 (HLA-DRB*04:01), rs3749971 (HLA-A1-B8-DR3), rs231775 (CLTLA4), rs1800629 (TNF), and rs1800682 (FAS) using predesigned TaqMan SNP genotyping assays. Chi square analysis was used to test for association of allele and genotype with overall AIH, and with severe fibrosis and ALT levels at 6 months.ResultsSignificant risk of AIH was conferred by the minor alleles of rs2187668 (OR = 2.45, 95% CI 1.65-3.61, p < 0.0001), rs3749971 (OR = 1.89, 95% CI 1.21-2.94, p = 0.004) and rs1800629 (OR = 2.06, 95% CI 1.41-3.01, p = 0.0001). Multivariate analysis showed that rs2187668 was independently associated with type 1 AIH susceptibility (OR = 2.40, 95% CI 1.46-3.93, p = 0.001). The C allele of FAS SNP rs1800682 was associated with increased risk of severe fibrosis at diagnosis (OR = 2.03, 95% CI 1.05-3.93, p = 0.035) and with incomplete normalization of ALT levels at 6 months post-diagnosis (OR = 3.94, 95% CI 1.62-9.54, p = 0.0015).ConclusionsThis is the first population-based study to investigate genetic risk loci for type 1 AIH in New Zealand Caucasians. We report significant independent association of HLA-DRB1*03:01 with overall susceptibility to type 1 AIH, as well as FAS with a more aggressive disease phenotype.

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Chronic active autoimmune hepatitis in children

Cited by 107 publications

References 11 publications

Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan

Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan

Human Leukocyte Antigen (HLA) and Immune Regulation: How Do Classical and Non-Classical HLA Alleles Modulate Immune Response to Human Immunodeficiency Virus and Hepatitis C Virus Infections?

Association of the HLA locus and TNF with type I autoimmune hepatitis susceptibility in New Zealand Caucasians

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