{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T12:49:15Z","timestamp":1776257355885,"version":"3.50.1"},"reference-count":43,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2016,9,20]],"date-time":"2016-09-20T00:00:00Z","timestamp":1474329600000},"content-version":"vor","delay-in-days":19,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2016,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Recurrent fever is a common presentation in paediatric practice and can be caused by a wide variety of diseases including autoinflammatory conditions. The innate immune system plays an essential role in the \u2018first line\u2019 response to infection through mediation of inflammatory responses. Inflammasomes are part of the regulatory process for this system and result in the production of the powerful pro\u2010inflammatory cytokine interleukin\u2010<jats:styled-content style=\"fixed-case\">1B<\/jats:styled-content>. Dysregulation of inflammasomes, and Interleukin 1 production, contributes to the pathogenesis of autoinflammatory diseases. This review focuses on described periodic fever syndromes (<jats:styled-content style=\"fixed-case\">PFS<\/jats:styled-content>) which are now collectively referred to as autoinflammatory syndromes. Conditions discussed include periodic fever aphthous stomatitis pharyngitis and cervical adenopathy, familial Mediterranean fever, tumour necrosis factor receptor\u2010associated periodic syndromes, hyperimmunoglobulinaemia D and the cryopyrin\u2010associated periodic syndromes. Presenting features, complications, diagnostic and treatment approaches for these conditions are discussed. Nonetheless, as most of these conditions are rare and may have significant long\u2010term complications, it is recommended that they be managed in consultations with a physician experienced in managing <jats:styled-content style=\"fixed-case\">PFS<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/jpc.13326","type":"journal-article","created":{"date-parts":[[2016,9,21]],"date-time":"2016-09-21T00:35:28Z","timestamp":1474418128000},"page":"865-871","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Periodic fevers and autoinflammatory syndromes in childhood"],"prefix":"10.1111","volume":"52","author":[{"given":"Genevieve T","family":"Ostring","sequence":"first","affiliation":[{"name":"Rheumatology Department Starship Children's Hospital  Auckland New Zealand"},{"name":"Paediatrics Child and Youth Health University of Auckland  Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Davinder","family":"Singh\u2010Grewal","sequence":"additional","affiliation":[{"name":"General Medicine The Sydney Children's Hospitals Network  New South Wales Australia"},{"name":"Department of Paediatrics and Child Health University of Sydney  Sydney New South Wales Australia"},{"name":"Department of Child and Maternal Health University of New South Wales  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,9,20]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2012.03.005"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbspin.2014.02.009"},{"key":"e_1_2_14_4_1","volume-title":"Illness in the Home","author":"Dingle J","year":"1964"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006454-200211000-00020"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199372030-00004"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.1053\/berh.2002.0239"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/70544"},{"key":"e_1_2_14_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa070265"},{"key":"e_1_2_14_10_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1103681108"},{"key":"e_1_2_14_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70316-1"},{"key":"e_1_2_14_12_1","first-page":"90","article-title":"Cytokine profile in PFAPA syndrome suggests continuous inflammation and reduced anti\u2010inflammatory response","volume":"17","author":"Stojanov S","year":"2006","journal-title":"Eur. Cytokine Netw."},{"key":"e_1_2_14_13_1","first-page":"358","article-title":"Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults","volume":"10","author":"Padeh S","year":"2008","journal-title":"Isr. Med. Assoc. J."},{"key":"e_1_2_14_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(87)80285-8"},{"key":"e_1_2_14_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70321-5"},{"key":"e_1_2_14_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70335-5"},{"key":"e_1_2_14_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2009.01554.x"},{"key":"e_1_2_14_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2010.12.014"},{"key":"e_1_2_14_19_1","doi-asserted-by":"publisher","DOI":"10.1001\/archoto.2007.7"},{"key":"e_1_2_14_20_1","first-page":"CD008669","article-title":"Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)","volume":"9","author":"Burton MJ","year":"2014","journal-title":"Cochrane Database Syst. Rev."},{"key":"e_1_2_14_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2010.08.014"},{"key":"e_1_2_14_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006454-199204000-00011"},{"key":"e_1_2_14_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(97)09408-7"},{"key":"e_1_2_14_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kei138"},{"key":"e_1_2_14_25_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2015-208690"},{"key":"e_1_2_14_26_1","first-page":"925","article-title":"Glucocorticoids but not NSAID abort attacks in hyper\u2010IgD and periodic fever syndrome","volume":"28","author":"Dios G\u2010DJ","year":"2001","journal-title":"J. Rheumatol."},{"key":"e_1_2_14_27_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.rhu.0000255772.25658.7c"},{"key":"e_1_2_14_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbspin.2008.04.001"},{"key":"e_1_2_14_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80721-7"},{"key":"e_1_2_14_30_1","doi-asserted-by":"publisher","DOI":"10.1186\/1824-7288-36-57"},{"key":"e_1_2_14_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2013.04.008"},{"key":"e_1_2_14_32_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.30149"},{"key":"e_1_2_14_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjp.0706695"},{"key":"e_1_2_14_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinthera.2012.09.009"},{"key":"e_1_2_14_35_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0810787"},{"key":"e_1_2_14_36_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ket415"},{"key":"e_1_2_14_37_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e318190cfb7"},{"key":"e_1_2_14_38_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kes097"},{"key":"e_1_2_14_39_1","doi-asserted-by":"publisher","DOI":"10.2174\/138920210793175921"},{"key":"e_1_2_14_40_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1013068"},{"key":"e_1_2_14_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2011.07.022"},{"key":"e_1_2_14_42_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0807865"},{"key":"e_1_2_14_43_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0809568"},{"key":"e_1_2_14_44_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2012.01725.x"}],"container-title":["Journal of Paediatrics and Child Health"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjpc.13326","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjpc.13326","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jpc.13326","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T12:41:33Z","timestamp":1695559293000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jpc.13326"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9]]},"references-count":43,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["10.1111\/jpc.13326"],"URL":"https:\/\/doi.org\/10.1111\/jpc.13326","archive":["Portico"],"relation":{},"ISSN":["1034-4810","1440-1754"],"issn-type":[{"value":"1034-4810","type":"print"},{"value":"1440-1754","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9]]},"assertion":[{"value":"2015-08-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2016-05-06","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2016-09-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}