{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T01:36:10Z","timestamp":1788226570494,"version":"build-2803163510"},"reference-count":10,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2017,12,1]],"date-time":"2017-12-01T00:00:00Z","timestamp":1512086400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2017,12,1]],"date-time":"2017-12-01T00:00:00Z","timestamp":1512086400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jacionline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Allergy and Clinical Immunology"],"published-print":{"date-parts":[[2017,12]]},"DOI":"10.1016\/j.jaci.2017.05.039","type":"journal-article","created":{"date-parts":[[2017,6,20]],"date-time":"2017-06-20T04:17:22Z","timestamp":1497932242000},"page":"1721-1724","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":26,"title":["Hemophagocytic lymphohistiocytosis as presenting manifestation of profound combined immunodeficiency due to an ORAI1 mutation"],"prefix":"10.1016","volume":"140","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5639-8690","authenticated-orcid":false,"given":"Christian","family":"Klemann","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Ammann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Heizmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9191-7970","authenticated-orcid":false,"given":"Sebastian","family":"Fuchs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian F.","family":"Bode","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maximilian","family":"Heeg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"Fuchs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai","family":"Lehmberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Udo","family":"zur Stadt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Roll","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Vraetz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten","family":"Speckmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myriam Ricarda","family":"Lorenz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Schwarz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Rohr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Feske","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephan","family":"Ehl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2017.05.039_bib1","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/j.blre.2014.03.002","article-title":"Hemophagocytic syndromes\u2013an update","volume":"28","author":"Janka","year":"2014","journal-title":"Blood Rev"},{"key":"10.1016\/j.jaci.2017.05.039_bib2","doi-asserted-by":"crossref","first-page":"978","DOI":"10.3324\/haematol.2014.121608","article-title":"The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis","volume":"100","author":"Bode","year":"2015","journal-title":"Haematologica"},{"key":"10.1016\/j.jaci.2017.05.039_bib3","doi-asserted-by":"crossref","first-page":"3324","DOI":"10.1073\/pnas.1013285108","article-title":"ORAI1-mediated calcium influx is required for human cytotoxic lymphocyte degranulation and target cell lysis","volume":"108","author":"Maul-Pavicic","year":"2011","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2017.05.039_bib4","doi-asserted-by":"crossref","first-page":"1523","DOI":"10.4049\/jimmunol.1102507","article-title":"Antiviral and regulatory T cell immunity in a patient with STIM1 deficiency","volume":"188","author":"Fuchs","year":"2012","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2017.05.039_bib5","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1002\/eji.1830240319","article-title":"Involvement of p21ras activation in T cell CD69 expression","volume":"24","author":"D'Ambrosio","year":"1994","journal-title":"Eur J Immunol"},{"key":"10.1016\/j.jaci.2017.05.039_bib6","first-page":"1","article-title":"Store-operated Ca2+ entry regulates Ca2+-activated chloride channels and eccrine sweat gland function","volume":"1","author":"Concepcion","year":"2016","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2017.05.039_bib7","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/978-3-319-26974-0_2","article-title":"The STIM1: Orai interaction","volume":"898","author":"Frischauf","year":"2016","journal-title":"Adv Exp Med Biol"},{"key":"10.1016\/j.jaci.2017.05.039_bib8","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1111\/nyas.12938","article-title":"Diseases caused by mutations in ORAI1 and STIM1","volume":"1356","author":"Lacruz","year":"2015","journal-title":"Ann N Y Acad Sci"},{"key":"10.1016\/j.jaci.2017.05.039_bib9","doi-asserted-by":"crossref","first-page":"5066","DOI":"10.1074\/jbc.M109.072736","article-title":"The intracellular loop of Orai1 plays a central role in fast inactivation of Ca2+ release-activated Ca2+ channels","volume":"285","author":"Srikanth","year":"2010","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2017.05.039_bib10","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1002\/eji.201646686","article-title":"Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T-cell activation, differentiation and repertoire","volume":"47","author":"Ammann","year":"2017","journal-title":"Eur J Immunol"}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674917309995?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674917309995?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T09:10:11Z","timestamp":1758791411000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674917309995"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12]]},"references-count":10,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2017,12]]}},"alternative-id":["S0091674917309995"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2017.05.039","relation":{},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2017,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Hemophagocytic lymphohistiocytosis as presenting manifestation of profound combined immunodeficiency due to an ORAI1 mutation","name":"articletitle","label":"Article Title"},{"value":"Journal of Allergy and Clinical Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaci.2017.05.039","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 American Academy of Allergy, Asthma & Immunology","name":"copyright","label":"Copyright"}]}}