{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T14:07:16Z","timestamp":1784297236173,"version":"3.55.0"},"reference-count":25,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T00:00:00Z","timestamp":1778457600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T00:00:00Z","timestamp":1778457600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Autosomal recessive interferon alpha and beta receptor subunit 1 (IFNAR1) deficiency is a rare and heritable inborn error of immunity (IEI) predisposing individuals to severe and life\u2010threatening viral infections. It is more common in people of Western Polynesian ancestry, with estimates of around one in six thousand live births affected, due to being homozygous for or having two copies of the regionally relevant pathogenic\n                    <jats:italic>IFNAR1<\/jats:italic>\n                    variant c.1156G&gt;T, p.Glu386*. IFNAR1 deficiency confers an increased risk of severe and life\u2010threatening infections caused by naturally circulating viruses including influenza, SARS\u2010CoV\u20102, herpes simplex virus, respiratory syncytial virus (RSV), arboviruses and viruses in live attenuated vaccines (LAVs) including measles\u2010mumps\u2010rubella (MMR) and yellow fever. Complications including virus induced systemic hyperinflammation (VISH) are associated with significant mortality. This document outlines expert consensus regarding early identification, diagnostic workup and management of IFNAR1 deficiency in Australia, Aotearoa New Zealand and Western Pacific nations.\n                  <\/jats:p>","DOI":"10.1111\/jpc.70421","type":"journal-article","created":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T05:50:56Z","timestamp":1778565056000},"page":"1113-1120","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Guideline for the Diagnosis and Management of Heritable\n                    <scp>IFNAR1<\/scp>\n                    Deficiency in Oceania"],"prefix":"10.1111","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-8188-232X","authenticated-orcid":false,"given":"Cecilia","family":"Verryt","sequence":"first","affiliation":[{"name":"Department of Clinical Immunology &amp; Allergy Starship Children's Hospital  Auckland New Zealand"},{"name":"Department of Paediatrics: Child and Youth Health University of Auckland  Auckland New Zealand"},{"name":"Department of Paediatrics Tauranga Hospital  Tauranga New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Gray","sequence":"additional","affiliation":[{"name":"Sydney Children's Hospital  Randwick New South Wales Australia"},{"name":"University of Western Sydney  Sydney Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"McNaughton","sequence":"additional","affiliation":[{"name":"Queensland Paediatric Immunology and Allergy Service Queensland Children's Hospital  South Brisbane Queensland Australia"},{"name":"University of Queensland  St Lucia Queensland Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane","family":"Peake","sequence":"additional","affiliation":[{"name":"Queensland Paediatric Immunology and Allergy Service Queensland Children's Hospital  South Brisbane Queensland Australia"},{"name":"University of Queensland  St Lucia Queensland Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Wong","sequence":"additional","affiliation":[{"name":"The Children's Hospital at Westmead  Westmead New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"George","family":"Aho","sequence":"additional","affiliation":[{"name":"Paediatric Department Vaiola Hospital, Ministry of Health  Nukualofa Tonga"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Best","sequence":"additional","affiliation":[{"name":"Department of Paediatrics: Child and Youth Health University of Auckland  Auckland New Zealand"},{"name":"Department of Paediatric Infectious Diseases Starship Children's Hospital  Auckland New Zealand"},{"name":"Immunisation Advisory Centre The University of Auckland  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maia","family":"Brewerton","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology &amp; Allergy Auckland City Hospital  Auckland New Zealand"},{"name":"Department of Microbiology and Immunology LabPLUS, Health New Zealand  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flora","family":"Lutui","sequence":"additional","affiliation":[{"name":"Paediatric Department Vaiola Hospital, Ministry of Health  Nukualofa Tonga"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Litara Esera","family":"Tulifau","sequence":"additional","affiliation":[{"name":"Tupua Tamasese Meaole Hospital  Apia Samoa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Qin","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology Women's and Children's Hospital  North Adelaide Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satupa\u2018itea","family":"Viali","sequence":"additional","affiliation":[{"name":"Oceania University of Medicine  Apia Samoa"},{"name":"Department of Molecular Medicine and Pathology University of Auckland  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petine","family":"White","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology &amp; Allergy Starship Children's Hospital  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Wood","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Pathology University of Auckland  Auckland New Zealand"},{"name":"Department of Oncology and Haematology Starship Children's Hospital  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"See\u2010Tarn","family":"Woon","sequence":"additional","affiliation":[{"name":"Department of Microbiology and Immunology LabPLUS, Health New Zealand  Auckland New Zealand"},{"name":"Department of Molecular Medicine and Pathology University of Auckland  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theresa","family":"Cole","sequence":"additional","affiliation":[{"name":"Department of Immunology and Allergy Royal Children's Hospital  Parkville Victoria Australia"},{"name":"Murdoch Children's Research Institute  Parkville Victoria Australia"},{"name":"Department of Paediatrics University of Melbourne  Parkville Melbourne Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto Pinzon","family":"Charry","sequence":"additional","affiliation":[{"name":"Queensland Paediatric Immunology and Allergy Service Queensland Children's Hospital  South Brisbane Queensland Australia"},{"name":"University of Queensland  St Lucia Queensland Australia"},{"name":"Griffith University  Nathan Queensland Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7412-9761","authenticated-orcid":false,"given":"Kuang\u2010Chih","family":"Hsiao","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology &amp; 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