{"id":"https://openalex.org/W2896973634","doi":"https://doi.org/10.1093/rheumatology/key273.029","title":"P27 Life-threatening macrophage activation syndrome with neurological involvement responding to very high dose IV anakinra","display_name":"P27 Life-threatening macrophage activation syndrome with neurological involvement responding to very high dose IV anakinra","publication_year":2018,"publication_date":"2018-10-01","ids":{"openalex":"https://openalex.org/W2896973634","doi":"https://doi.org/10.1093/rheumatology/key273.029","mag":"2896973634"},"language":"en","primary_location":{"id":"doi:10.1093/rheumatology/key273.029","doi":"https://doi.org/10.1093/rheumatology/key273.029","is_oa":false,"landing_page_url":"https://doi.org/10.1093/rheumatology/key273.029","pdf_url":null,"source":{"id":"https://openalex.org/S183989336","display_name":"Rheumatology","issn_l":"1462-0324","issn":["1462-0324","1462-0332"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311648","host_organization_name":"Oxford University Press","host_organization_lineage":["https://openalex.org/P4310311648","https://openalex.org/P4310311647"],"host_organization_lineage_names":["Oxford University Press","University of Oxford"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology","raw_type":"journal-article"},"volume":null,"type":"conference-abstract","is_traditional":false,"indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5031649123","display_name":"Akhila Kavirayani","orcid":"https://orcid.org/0000-0002-7447-5514","observed_orcids":["https://orcid.org/0000-0002-7447-5514"]},"institutions":[{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Akhila Kavirayani","raw_affiliation_strings":["Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I4210147381"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5063152811","display_name":"Robindra Basu Roy","orcid":"https://orcid.org/0000-0001-5648-3628","observed_orcids":["https://orcid.org/0000-0001-5648-3628"]},"institutions":[{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Robin Basu-Roy","raw_affiliation_strings":["Paediatric Infectious Diseases, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Infectious Diseases, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I4210147381"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5090004519","display_name":"Seilesh Kadambari","orcid":"https://orcid.org/0000-0003-3658-7635","observed_orcids":["https://orcid.org/0000-0003-3658-7635","https://orcid.org/0000-0001-8940-3284"]},"institutions":[{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Seilesh Kadambari","raw_affiliation_strings":["Paediatric Infectious Diseases, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Infectious Diseases, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I4210147381"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5009455397","display_name":"Shelley Segal","orcid":"https://orcid.org/0000-0002-0697-1719","observed_orcids":["https://orcid.org/0000-0002-0697-1719"]},"institutions":[{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Shelley Segal","raw_affiliation_strings":["Paediatric Infectious Diseases, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Infectious Diseases, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I4210147381"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5060917596","display_name":"Amrana Qureshi","orcid":"https://orcid.org/0000-0003-0870-9594","observed_orcids":["https://orcid.org/0000-0003-0870-9594"]},"institutions":[{"id":"https://openalex.org/I40120149","display_name":"University of Oxford","ror":"https://ror.org/052gg0110","country_code":"GB","type":"education","lineage":["https://openalex.org/I40120149"]},{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Amrana Qureshi","raw_affiliation_strings":["Paediatric Haematology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Haematology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I40120149","https://openalex.org/I4210147381"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5055676786","display_name":"Ruth Finch","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Ruth Finch","raw_affiliation_strings":["Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I4210147381"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5089313452","display_name":"Elaine Parsons","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Elaine Parsons","raw_affiliation_strings":["Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I4210147381"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5057594145","display_name":"Kathryn M. Bailey","orcid":"https://orcid.org/0000-0003-4525-8673","observed_orcids":["https://orcid.org/0000-0003-4525-8673"]},"institutions":[{"id":"https://openalex.org/I4210147381","display_name":"Oxford University Hospitals NHS Trust","ror":"https://ror.org/03h2bh287","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210147381"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Kathryn Bailey","raw_affiliation_strings":["Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UNITED KINGDOM","institution_ids":["https://openalex.org/I4210147381"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":{"value":3486,"currency":"GBP","value_usd":4597},"apc_paid":null,"fwci":null,"has_fulltext":true,"cited_by_count":1,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":"57","issue":"suppl_8","first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11892","display_name":"Kawasaki Disease and Coronary Complications","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2735","display_name":"Pediatrics, Perinatology and Child Health"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12845","display_name":"Methemoglobinemia and Tumor Lysis Syndrome","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/macrophage-activation-syndrome","display_name":"macrophage activation syndrome","score":0.9990000128746033},{"id":"https://openalex.org/keywords/hemophagocytic-lymphohistiocytosis","display_name":"hemophagocytic lymphohistiocytosis","score":0.9929999709129333},{"id":"https://openalex.org/keywords/anakinra","display_name":"anakinra","score":0.9359999895095825},{"id":"https://openalex.org/keywords/neurological-complications","display_name":"neurological complications","score":0.5929999947547913},{"id":"https://openalex.org/keywords/kidney-failure","display_name":"renal failure","score":0.48500001430511475},{"id":"https://openalex.org/keywords/multiple-organ-failure","display_name":"multiple organ failure","score":0.6790000200271606},{"id":"https://openalex.org/keywords/hemophagocytosis","display_name":"hemophagocytosis","score":0.8289999961853027},{"id":"https://openalex.org/keywords/pediatric-patients","display_name":"pediatric patients","score":0.5979999899864197},{"id":"https://openalex.org/keywords/hyperferritinemia","display_name":"hyperferritinemia","score":0.6830000281333923},{"id":"https://openalex.org/keywords/hemofiltration","display_name":"hemofiltration","score":0.6660000085830688}],"concepts":[{"id":"https://openalex.org/C2781228260","wikidata":"https://www.wikidata.org/wiki/Q415411","display_name":"Anakinra","level":3,"score":0.9745018482208252},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9645718932151794},{"id":"https://openalex.org/C2779613421","wikidata":"https://www.wikidata.org/wiki/Q6725504","display_name":"Macrophage activation syndrome","level":3,"score":0.8185705542564392},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.41932886838912964},{"id":"https://openalex.org/C2779244956","wikidata":"https://www.wikidata.org/wiki/Q184204","display_name":"Macrophage","level":3,"score":0.4115103781223297},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.08509469032287598},{"id":"https://openalex.org/C202751555","wikidata":"https://www.wikidata.org/wiki/Q221681","display_name":"In vitro","level":2,"score":0.0},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.0},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1093/rheumatology/key273.029","doi":"https://doi.org/10.1093/rheumatology/key273.029","is_oa":false,"landing_page_url":"https://doi.org/10.1093/rheumatology/key273.029","pdf_url":null,"source":{"id":"https://openalex.org/S183989336","display_name":"Rheumatology","issn_l":"1462-0324","issn":["1462-0324","1462-0332"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311648","host_organization_name":"Oxford University Press","host_organization_lineage":["https://openalex.org/P4310311648","https://openalex.org/P4310311647"],"host_organization_lineage_names":["Oxford University Press","University of Oxford"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"score":1.0,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W3121629075","https://openalex.org/W2081414355","https://openalex.org/W2890948474","https://openalex.org/W3124256490","https://openalex.org/W4392957867","https://openalex.org/W4415893839","https://openalex.org/W4385567413","https://openalex.org/W3172783092","https://openalex.org/W2530025411","https://openalex.org/W2009248078"],"abstract_inverted_index":{"Background:":[0],"Severe":[1,115],"MAS":[2,24],"(macrophage":[3],"activation":[4],"syndrome)":[5],"with":[6,39,50,74,92],"neurological,":[7],"renal":[8,116],"&":[9,53,69,79,90,95],"bleeding":[10],"complications":[11],"is":[12],"challenging":[13],"to":[14,18,26,84],"manage.":[15],"We":[16],"aim":[17],"describe":[19],"a":[20],"case":[21],"of":[22,148],"life-threatening":[23],"responding":[25],"high":[27,40,75],"dose":[28],"IV":[29],"anakinra.":[30],"Methods:":[31],"A":[32],"nine":[33],"year":[34],"old":[35],"Caucasian":[36],"girl":[37],"presented":[38],"fevers":[41],"for":[42,103],"three":[43],"weeks,":[44],"severe":[45],"abdominal":[46,70],"and":[47,58,142],"leg":[48],"pains,":[49],"no":[51,54],"rashes":[52],"infectious":[55],"focus.":[56],"Past":[57],"family":[59],"history":[60],"were":[61],"non-contributory.":[62],"She":[63,99],"underwent":[64],"an":[65],"appendicectomy":[66],"(normal":[67],"appendix":[68],"MRI).":[71],"Fevers":[72],"continued":[73],"inflammatory":[76],"markers(ferritin":[77],"5000)":[78],"rising":[80],"creatinine.":[81],"Upon":[82],"transfer":[83],"our":[85],"centre,":[86],"she":[87],"was":[88,100,122],"febrile/delirious":[89],"collapsed":[91],"rapid":[93],"deterioration":[94],"multi":[96],"organ":[97],"dysfunction.":[98],"ventilated,needing":[101],"inotropes":[102],"profound":[104],"hypotension/poor":[105],"cardiac":[106],"output":[107],"(with":[108],"resultant":[109],"ischaemic":[110],"necrotic":[111],"patches":[112],"on":[113],"limbs).":[114],"failure":[117],"needed":[118],"haemofiltration.":[119],"Concurrent":[120],"ferritin":[121],"192549,":[123],"CRP":[124],"180,":[125],"ESR<1,":[126],"creatinine":[127],"257,":[128],"falling":[129],"Haemoglobin/platelets":[130],"(necessitating":[131],"transfusions),":[132],"fibrinogen<0.3,":[133],"triglycerides":[134],"3.9,":[135],"ALT":[136],"242,":[137],"LDH":[138],"3218,":[139],"prolonged":[140],"PT/aPTT":[141],"bone":[143],"marrow":[144],"showed":[145],"haemophagocytosis,":[146],"all":[147],"these":[149],"confirming":[150],"MAS/HLH.":[151]},"counts_by_year":[{"year":2023,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2018-10-26T00:00:00"}
