{"id":"https://openalex.org/W106313530","doi":null,"title":"Macrophage activation syndrome and rheumatic disease in childhood: a report of four new cases.","display_name":"Macrophage activation syndrome and rheumatic disease in childhood: a report of four new cases.","publication_year":1993,"publication_date":"1993-07-01","ids":{"openalex":"https://openalex.org/W106313530","mag":"106313530","pmid":"https://pubmed.ncbi.nlm.nih.gov/8403593"},"language":"en","primary_location":{"id":"pmid:8403593","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/8403593","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Clinical and experimental rheumatology","raw_type":"Case Reports"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["pubmed"],"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/A5109320857","display_name":"JL St\u00e9phan","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1288880153","display_name":"H\u00f4pital Necker-Enfants Malades","ror":"https://ror.org/05tr67282","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I1288880153","https://openalex.org/I4210097159","https://openalex.org/I4210120235"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"J L St\u00e9phan","raw_affiliation_strings":["Pediatric Rheumatology Unit, H\u00f4pital des Enfants Malades, Paris, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pediatric Rheumatology Unit, H\u00f4pital des Enfants Malades, Paris, France","institution_ids":["https://openalex.org/I1288880153"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5112238156","display_name":"Jason Zeller","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"J Zeller","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5038063185","display_name":"P. Hubert","orcid":"https://orcid.org/0000-0002-4535-3593","observed_orcids":["https://orcid.org/0000-0002-4535-3593"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"P Hubert","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5069628486","display_name":"Catherine Herbelin","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"C Herbelin","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5109308776","display_name":"J.-M. Dayer","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"J M Dayer","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5108519163","display_name":"Anne\u2010Marie Prieur","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"A M Prieur","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":1.4345,"has_fulltext":false,"cited_by_count":185,"citation_normalized_percentile":{"value":0.80158855,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":99},"biblio":{"volume":"11","issue":"4","first_page":"451","last_page":"6"},"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":0.0,"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/T11078","display_name":"Inflammasome and immune disorders","score":0.0,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/macrophage-activation-syndrome","display_name":"macrophage activation syndrome","score":1.0},{"id":"https://openalex.org/keywords/hemophagocytic-lymphohistiocytosis","display_name":"hemophagocytic lymphohistiocytosis","score":0.8579999804496765},{"id":"https://openalex.org/keywords/juvenile-idiopathic-arthritis","display_name":"juvenile idiopathic arthritis","score":0.7450000047683716},{"id":"https://openalex.org/keywords/pediatric-rheumatic-diseases","display_name":"pediatric rheumatic diseases","score":0.7049999833106995},{"id":"https://openalex.org/keywords/hemophagocytosis","display_name":"hemophagocytosis","score":0.8050000071525574},{"id":"https://openalex.org/keywords/monokines","display_name":"monokines","score":0.734000027179718},{"id":"https://openalex.org/keywords/cyclosporine","display_name":"cyclosporine","score":0.9800000190734863},{"id":"https://openalex.org/keywords/corticosteroid-therapy","display_name":"corticosteroid therapy","score":0.8360000252723694},{"id":"https://openalex.org/keywords/t-cell-dysfunction","display_name":"T cell dysfunction","score":0.7689999938011169},{"id":"https://openalex.org/keywords/hypofibrinogenemia","display_name":"hypofibrinogenemia","score":0.8970000147819519}],"concepts":[{"id":"https://openalex.org/C2779613421","wikidata":"https://www.wikidata.org/wiki/Q6725504","display_name":"Macrophage activation syndrome","level":3,"score":0.9752016067504883},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9472474455833435},{"id":"https://openalex.org/C2779244956","wikidata":"https://www.wikidata.org/wiki/Q184204","display_name":"Macrophage","level":3,"score":0.663447916507721},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.5588659644126892},{"id":"https://openalex.org/C109159458","wikidata":"https://www.wikidata.org/wiki/Q919283","display_name":"Haematopoiesis","level":3,"score":0.4708530306816101},{"id":"https://openalex.org/C2777077863","wikidata":"https://www.wikidata.org/wiki/Q170990","display_name":"Arthritis","level":2,"score":0.46682044863700867},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3711947500705719},{"id":"https://openalex.org/C28328180","wikidata":"https://www.wikidata.org/wiki/Q48196","display_name":"Stem cell","level":2,"score":0.12744173407554626},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"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/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.0},{"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/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.0}],"mesh":[{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001171","descriptor_name":"Arthritis, Juvenile","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":false},{"descriptor_ui":"D001171","descriptor_name":"Arthritis, Juvenile","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002675","descriptor_name":"Child, Preschool","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007154","descriptor_name":"Immune System Diseases","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":false},{"descriptor_ui":"D007154","descriptor_name":"Immune System Diseases","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D007154","descriptor_name":"Immune System Diseases","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D008099","descriptor_name":"Liver","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D008262","descriptor_name":"Macrophage Activation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D010179","descriptor_name":"Pancreas","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D012216","descriptor_name":"Rheumatic Diseases","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":false},{"descriptor_ui":"D012216","descriptor_name":"Rheumatic Diseases","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":true},{"descriptor_ui":"D013577","descriptor_name":"Syndrome","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D016572","descriptor_name":"Cyclosporine","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false}],"locations_count":2,"locations":[{"id":"pmid:8403593","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/8403593","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Clinical and experimental rheumatology","raw_type":"Case Reports"},{"id":"mag:106313530","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/8403593","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":1.0}],"study_designs":[{"display_name":"Case Report","id":"https://openalex.org/study-designs/case-report"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W2165127856","https://openalex.org/W2416658947","https://openalex.org/W2127002308","https://openalex.org/W1975349722","https://openalex.org/W2324569383","https://openalex.org/W4232460139","https://openalex.org/W2769315834","https://openalex.org/W3181017624","https://openalex.org/W2913279027","https://openalex.org/W2117673822"],"abstract_inverted_index":{"A":[0,125],"macrophage":[1,64,177],"activation":[2],"syndrome":[3],"(MAS)":[4],"developed":[5],"in":[6,43,58,73,128,136,157],"four":[7],"children":[8,160],"with":[9,55,96,115,161],"chronic":[10,170],"rheumatic":[11,162],"diseases.":[12],"The":[13,34],"presentation":[14],"included":[15],"fever,":[16],"hepatic":[17],"and":[18,32,94,131,195],"splenic":[19],"enlargement,":[20],"profound":[21],"depression":[22],"of":[23,27,30,38,48,62,71,76,82,112,117,134,139,149,168,191],"blood":[24],"counts,":[25],"lowering":[26],"ESR,":[28],"elevation":[29],"SGOT/PT":[31],"hypofibrinogenemia.":[33],"most":[35],"characteristic":[36],"sign":[37],"MAS":[39],"was":[40,65,126],"the":[41,44,63,74,137,140,146,165],"presence":[42],"bone":[45],"marrow":[46],"aspirate":[47],"well":[49],"differentiated":[50],"macrophages":[51],"showing":[52],"active":[53],"haemophagocytosis":[54],"haematopoietic":[56],"elements":[57],"their":[59],"cytoplasm.":[60],"Activation":[61],"also":[66],"illustrated":[67],"by":[68,88],"high":[69],"levels":[70],"monokines":[72],"serum":[75],"2":[77],"patients.":[78],"This":[79],"immuno-hematological":[80],"process":[81],"unknown":[83],"etiology":[84],"can":[85,121],"be":[86,107,133,155,182],"triggered":[87],"ubiquitous":[89],"events":[90],"such":[91],"as":[92],"infections":[93,198],"treatment":[95],"anti-inflammatory":[97],"drugs.":[98],"It":[99,153],"is":[100,199],"a":[101,150],"potentially":[102,118],"lethal":[103],"complication":[104],"which":[105,179],"should":[106],"diagnosed":[108],"rapidly,":[109],"since":[110],"administration":[111],"high-dose":[113],"steroids":[114],"discontinuation":[116],"toxic":[119],"drugs":[120,194],"induce":[122],"remission.":[123],"Cyclosporin":[124],"effective":[127],"two":[129],"patients":[130],"may":[132],"value":[135],"management":[138],"macrophage-activation":[141],"syndrome.":[142],"Its":[143],"efficacy":[144],"supports":[145],"central":[147],"involvement":[148],"T-cell":[151],"dysfunction.":[152],"must":[154],"borne":[156],"mind":[158],"that":[159],"diseases,":[163],"especially":[164],"systemic":[166],"form":[167],"juvenile":[169],"arthritis,":[171],"are":[172],"highly":[173],"vulnerable":[174],"to":[175,181],"life-threatening":[176],"activation,":[178],"appears":[180],"more":[183],"frequent":[184],"than":[185],"previously":[186],"recognized.":[187],"Very":[188],"careful":[189],"monitoring":[190],"apparently":[192],"\"innocent\"":[193],"intercurrent":[196],"viral":[197],"thus":[200],"required.":[201]},"counts_by_year":[{"year":2026,"cited_by_count":1},{"year":2025,"cited_by_count":4},{"year":2024,"cited_by_count":7},{"year":2023,"cited_by_count":2},{"year":2022,"cited_by_count":1},{"year":2021,"cited_by_count":3},{"year":2020,"cited_by_count":5},{"year":2019,"cited_by_count":7},{"year":2018,"cited_by_count":5},{"year":2017,"cited_by_count":8},{"year":2016,"cited_by_count":9},{"year":2015,"cited_by_count":9},{"year":2014,"cited_by_count":8},{"year":2013,"cited_by_count":6},{"year":2012,"cited_by_count":10}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}
