{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,13]],"date-time":"2026-11-13T17:17:28Z","timestamp":1794590248913,"version":"4.9.1"},"reference-count":19,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,4,22]],"date-time":"2024-04-22T00:00:00Z","timestamp":1713744000000},"content-version":"vor","delay-in-days":112,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010610","name":"SSM Health Cardinal Glennon Children&apos;s Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010610","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001448","name":"Alpha-1 Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001448","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/NO24842\/1"],"award-info":[{"award-number":["MR\/NO24842\/1"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/V034243\/1"],"award-info":[{"award-number":["MR\/V034243\/1"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010346","name":"University of Colorado Denver","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010346","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012317","name":"UCLH Biomedical Research Centre","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012317","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007180","name":"Children&apos;s Hospital Colorado","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007180","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100023392","name":"NIHR Barts Biomedical Research Centre","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100023392","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007270","name":"University of Michigan","doi-asserted-by":"publisher","award":["NIH-DK062456"],"award-info":[{"award-number":["NIH-DK062456"]}],"id":[{"id":"10.13039\/100007270","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010390","name":"Saint Louis University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010390","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011620","name":"Liver Center, Saint Louis University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011620","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["DK 62453"],"award-info":[{"award-number":["DK 62453"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","ghadvances.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Gastro Hep Advances"],"published-print":{"date-parts":[[2024]]},"DOI":"10.1016\/j.gastha.2024.04.010","type":"journal-article","created":{"date-parts":[[2024,4,26]],"date-time":"2024-04-26T12:53:24Z","timestamp":1714136004000},"page":"842-850","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":7,"title":["Biomarkers Associated With Future Severe Liver Disease in Children With Alpha-1-Antitrypsin Deficiency"],"prefix":"10.1016","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4870-2621","authenticated-orcid":false,"given":"Jeffrey H.","family":"Teckman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Buchanan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0849-9497","authenticated-orcid":false,"given":"Keith Steven","family":"Blomenkamp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nina","family":"Heyer-Chauhan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keith","family":"Burling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Lomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.gastha.2024.04.010_bib1","doi-asserted-by":"crossref","first-page":"23","DOI":"10.21037\/tgh.2020.02.23","article-title":"Alpha-1 antitrypsin deficiency liver disease","volume":"6","author":"Patel","year":"2021","journal-title":"Transl Gastroenterol Hepatol"},{"issue":"15","key":"10.1016\/j.gastha.2024.04.010_bib2","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1056\/NEJMra1910234","article-title":"Alpha(1)-Antitrypsin deficiency","volume":"382","author":"Strnad","year":"2020","journal-title":"N\u00a0Engl J Med"},{"issue":"6379","key":"10.1016\/j.gastha.2024.04.010_bib3","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1038\/357605a0","article-title":"The mechanism of Z alpha 1-antitrypsin accumulation in the liver","volume":"357","author":"Lomas","year":"1992","journal-title":"Nature"},{"issue":"24","key":"10.1016\/j.gastha.2024.04.010_bib4","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1056\/NEJM197606102942404","article-title":"Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants","volume":"294","author":"Sveger","year":"1976","journal-title":"N\u00a0Engl J Med"},{"issue":"3","key":"10.1016\/j.gastha.2024.04.010_bib5","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/j.cld.2022.03.004","article-title":"Alpha-1 antitrypsin deficiency liver disease","volume":"26","author":"Suri","year":"2022","journal-title":"Clin Liver Dis"},{"key":"10.1016\/j.gastha.2024.04.010_bib6","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.jpeds.2020.07.031","article-title":"Longitudinal outcomes in young patients with alpha-1-antitrypsin deficiency with native liver reveal that neonatal cholestasis is a poor predictor of future portal hypertension","volume":"227","author":"Teckman","year":"2020","journal-title":"J\u00a0Pediatr"},{"issue":"11","key":"10.1016\/j.gastha.2024.04.010_bib7","doi-asserted-by":"crossref","first-page":"1608","DOI":"10.1111\/liv.13586","article-title":"SERPINA1 and MAN1B1 polymorphisms are not linked to severe liver disease in a French cohort of alpha-1 antitrypsin deficiency children","volume":"37","author":"Joly","year":"2017","journal-title":"Liver Int"},{"issue":"5","key":"10.1016\/j.gastha.2024.04.010_bib8","doi-asserted-by":"crossref","first-page":"1501","DOI":"10.1183\/09031936.00111213","article-title":"Circulating polymers in alpha1-antitrypsin deficiency","volume":"43","author":"Tan","year":"2014","journal-title":"Eur Respir J"},{"issue":"3","key":"10.1016\/j.gastha.2024.04.010_bib9","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1183\/13993003.00940-2015","article-title":"Polymers of Z alpha1-antitrypsin are secreted in cell models of disease","volume":"47","author":"Fra","year":"2016","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.gastha.2024.04.010_bib10","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1016\/j.jhep.2018.08.005","article-title":"Clinical and histologic features of adults with alpha-1 antitrypsin deficiency in a non-cirrhotic cohort","volume":"69","author":"Clark","year":"2018","journal-title":"J\u00a0Hepatol"},{"issue":"4","key":"10.1016\/j.gastha.2024.04.010_bib11","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1002\/hep.21822","article-title":"Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model","volume":"46","author":"Lindblad","year":"2007","journal-title":"Hepatology"},{"key":"10.1016\/j.gastha.2024.04.010_bib12","article-title":"Alpha1-antitrypsin deficiency","volume":"2","author":"Greene","year":"2016","journal-title":"Nat Rev Dis Prim"},{"issue":"1","key":"10.1016\/j.gastha.2024.04.010_bib13","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1097\/MPG.0000000000000753","article-title":"Baseline analysis of a young alpha-1-antitrypsin deficiency liver disease cohort reveals frequent portal hypertension","volume":"61","author":"Teckman","year":"2015","journal-title":"J\u00a0Pediatr Gastroenterol Nutr"},{"issue":"37","key":"10.1016\/j.gastha.2024.04.010_bib14","doi-asserted-by":"crossref","first-page":"22791","DOI":"10.1074\/jbc.271.37.22791","article-title":"Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity","volume":"271","author":"Qu","year":"1996","journal-title":"J\u00a0Biol Chem"},{"issue":"19","key":"10.1016\/j.gastha.2024.04.010_bib15","doi-asserted-by":"crossref","first-page":"9014","DOI":"10.1073\/pnas.91.19.9014","article-title":"A\u00a0lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency","volume":"91","author":"Wu","year":"1994","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"22","key":"10.1016\/j.gastha.2024.04.010_bib16","doi-asserted-by":"crossref","first-page":"13215","DOI":"10.1074\/jbc.271.22.13215","article-title":"The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein","volume":"271","author":"Teckman","year":"1996","journal-title":"J\u00a0Biol Chem"},{"issue":"6","key":"10.1016\/j.gastha.2024.04.010_bib17","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1056\/NEJMoa2205416","article-title":"Fazirsiran for liver disease associated with alpha(1)-antitrypsin deficiency","volume":"387","author":"Strnad","year":"2022","journal-title":"N\u00a0Engl J Med"},{"issue":"1","key":"10.1016\/j.gastha.2024.04.010_bib18","doi-asserted-by":"crossref","first-page":"6371","DOI":"10.1038\/s41467-020-20147-7","article-title":"High-resolution ex\u00a0vivo NMR spectroscopy of human Z alpha(1)-antitrypsin","volume":"11","author":"Jagger","year":"2020","journal-title":"Nat Commun"},{"issue":"6","key":"10.1016\/j.gastha.2024.04.010_bib19","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1165\/ajrcmb.26.6.4739","article-title":"Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema","volume":"26","author":"Parmar","year":"2002","journal-title":"Am J Respir Cell Mol Biol"}],"container-title":["Gastro Hep Advances"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2772572324000608?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2772572324000608?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T12:04:45Z","timestamp":1778933085000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2772572324000608"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024]]},"references-count":19,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024]]}},"alternative-id":["S2772572324000608"],"URL":"https:\/\/doi.org\/10.1016\/j.gastha.2024.04.010","relation":{},"ISSN":["2772-5723"],"issn-type":[{"value":"2772-5723","type":"print"}],"subject":[],"published":{"date-parts":[[2024]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Biomarkers Associated With Future Severe Liver Disease in Children With Alpha-1-Antitrypsin Deficiency","name":"articletitle","label":"Article Title"},{"value":"Gastro Hep Advances","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.gastha.2024.04.010","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Authors. Published by Elsevier Inc. on behalf of the AGA Institute.","name":"copyright","label":"Copyright"}]}}