PMID- 37250880 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240502 IS - 0973-6883 (Print) IS - 2213-3453 (Electronic) IS - 0973-6883 (Linking) VI - 13 IP - 3 DP - 2023 May-Jun TI - Association of HLA DRB1 Allele Profile with Pediatric Autoimmune Liver Disease in India. PG - 397-403 LID - 10.1016/j.jceh.2023.01.001 [doi] AB - OBJECTIVES: The aim of this study is to study the association of human leukocyte antigen (HLA) DRB1 alleles with treatment response in Indian children with autoimmune liver disease (AILD). METHODS: HLA DRB1 alleles of 71 Indian children with pediatric AILD (pAILD) were analyzed along with 25 genetically confirmed patients with Wilson disease as controls. After 1 year of therapy, all those who failed to normalize aspartate & alanine transferase (AST/ ALT) (below 1.5 times of upper limit of normal) and/or failed to normalize IgG levels, or who had >2 relapses (AST/ALT levels >1.5 times of upper limit of normal) while on treatment, were labeled as difficult to treat (DTT). RESULTS: HLA DRB1 *3 was found to be significantly associated with AIH type 1 (46.2% vs. 4% in controls; P corrected = 0.011). Majority of the patients [55 (77.5%)] had chronic liver disease at presentation, with 42 (59.2%) having portal hypertension and 17 (23.9%) having ascites. Out of the 71 with pAILD, 19 (26.8%) were DTT. HLA DRB1 *14 was found to be independently associated with DTT cases (36.8% vs. 9.6%, OR 5.87, 95% CI 1.07-32.09, P = 0.041). Other factors independently associated with DTT were presence of autoimmune sclerosing cholangitis (OR 8.57, P = 0.008) and high-risk varices (OR 7.55, P = 0.016), improving the correctness of classification of the model from 73.2% to 84.5%. CONCLUSION: HLA DRB1 *14 is independently associated with treatment response in pAILD and HLA DRB1 *3 is associated with AIH type 1. HLA DRB1 alleles may thus provide supportive information for diagnosis and prognosis of AILD. CI - (c) 2023 Indian National Association for Study of the Liver. Published by Elsevier B.V. All rights reserved. FAU - Maria, Arjun AU - Maria A AD - Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. FAU - Sood, Vikrant AU - Sood V AD - Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. FAU - Khanna, Rajeev AU - Khanna R AD - Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. FAU - Lal, Bikrant B AU - Lal BB AD - Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. FAU - Trehanpati, Nirupama AU - Trehanpati N AD - Department of Molecular and Cellular Medicine, Institute of Liver and Biliary Sciences, New Delhi, India. FAU - Alam, Seema AU - Alam S AD - Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. LA - eng PT - Journal Article DEP - 20230105 PL - India TA - J Clin Exp Hepatol JT - Journal of clinical and experimental hepatology JID - 101574137 PMC - PMC10213850 OTO - NOTNLM OT - HLA DRB1 allele OT - human leukocyte antigen OT - pediatric autoimmune liver disease EDAT- 2023/05/30 13:07 MHDA- 2023/05/30 13:08 PMCR- 2024/05/01 CRDT- 2023/05/30 11:29 PHST- 2022/09/17 00:00 [received] PHST- 2023/01/02 00:00 [accepted] PHST- 2023/05/30 13:08 [medline] PHST- 2023/05/30 13:07 [pubmed] PHST- 2023/05/30 11:29 [entrez] PHST- 2024/05/01 00:00 [pmc-release] AID - S0973-6883(23)00001-4 [pii] AID - 10.1016/j.jceh.2023.01.001 [doi] PST - ppublish SO - J Clin Exp Hepatol. 2023 May-Jun;13(3):397-403. doi: 10.1016/j.jceh.2023.01.001. Epub 2023 Jan 5.