{"id":"https://openalex.org/W4298111110","doi":"https://doi.org/10.1542/pir.2021-005367","title":"Histiocytic Disorders of Childhood","display_name":"Histiocytic Disorders of Childhood","publication_year":2022,"publication_date":"2022-10-01","ids":{"openalex":"https://openalex.org/W4298111110","doi":"https://doi.org/10.1542/pir.2021-005367","pmid":"https://pubmed.ncbi.nlm.nih.gov/36180546"},"language":"en","primary_location":{"id":"doi:10.1542/pir.2021-005367","doi":"https://doi.org/10.1542/pir.2021-005367","is_oa":false,"landing_page_url":"https://doi.org/10.1542/pir.2021-005367","pdf_url":null,"source":{"id":"https://openalex.org/S118940750","display_name":"Pediatrics in Review","issn_l":"0191-9601","issn":["0191-9601","1526-3347","1529-7233"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-c","jufo-1","medline","norway-1","poland-40","russia-white-list-2"],"host_organization":"https://openalex.org/P4310315734","host_organization_name":"American Academy of Pediatrics","host_organization_lineage":["https://openalex.org/P4310315734"],"host_organization_lineage_names":["American Academy of Pediatrics"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pediatrics In Review","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","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/A5078648308","display_name":"Olive S. Eckstein","orcid":"https://orcid.org/0000-0002-1895-8810","observed_orcids":["https://orcid.org/0000-0002-1895-8810"]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]},{"id":"https://openalex.org/I4210147586","display_name":"Texas Children's Hospital","ror":"https://ror.org/05cz92x43","country_code":"US","type":"funder","lineage":["https://openalex.org/I4210147586"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Olive S. Eckstein","raw_affiliation_strings":["*Texas Children\u2019s Hospital Cancer and Hematology Centers, Section of Pediatric Hematology and Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"*Texas Children\u2019s Hospital Cancer and Hematology Centers, Section of Pediatric Hematology and Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX","institution_ids":["https://openalex.org/I181547552","https://openalex.org/I4210147586"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5066407702","display_name":"Jennifer Lee Picarsic","orcid":"https://orcid.org/0000-0002-3718-6422","observed_orcids":["https://orcid.org/0000-0002-3718-6422"]},"institutions":[{"id":"https://openalex.org/I1285204247","display_name":"Cincinnati Children's Hospital Medical Center","ror":"https://ror.org/01hcyya48","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1285204247"]},{"id":"https://openalex.org/I63135867","display_name":"University of Cincinnati","ror":"https://ror.org/01e3m7079","country_code":"US","type":"education","lineage":["https://openalex.org/I63135867"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Jennifer Picarsic","raw_affiliation_strings":["\u2020Cincinnati Children\u2019s Hospital, Department of Pathology and Laboratory Medicine, University of Cincinnati, Cincinnati, OH"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"\u2020Cincinnati Children\u2019s Hospital, Department of Pathology and Laboratory Medicine, University of Cincinnati, Cincinnati, OH","institution_ids":["https://openalex.org/I1285204247","https://openalex.org/I63135867"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5006499960","display_name":"Carl E. Allen","orcid":"https://orcid.org/0000-0002-6625-739X","observed_orcids":["https://orcid.org/0000-0002-6625-739X","https://orcid.org/0000-0002-2241-5712"]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]},{"id":"https://openalex.org/I4210147586","display_name":"Texas Children's Hospital","ror":"https://ror.org/05cz92x43","country_code":"US","type":"funder","lineage":["https://openalex.org/I4210147586"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Carl E. Allen","raw_affiliation_strings":["*Texas Children\u2019s Hospital Cancer and Hematology Centers, Section of Pediatric Hematology and Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"*Texas Children\u2019s Hospital Cancer and Hematology Centers, Section of Pediatric Hematology and Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX","institution_ids":["https://openalex.org/I181547552","https://openalex.org/I4210147586"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":4,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":1.2072,"has_fulltext":false,"cited_by_count":8,"citation_normalized_percentile":{"value":0.77023111,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":93,"max":97},"biblio":{"volume":"43","issue":"10","first_page":"561","last_page":"571"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11909","display_name":"Histiocytic Disorders and Treatments","score":0.9994999766349792,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology (medical)"},"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/T11909","display_name":"Histiocytic Disorders and Treatments","score":0.9994999766349792,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology (medical)"},"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/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","score":0.00039999998989515007,"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/T10980","display_name":"Vascular Malformations and Hemangiomas","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"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/langerhans-cell-histiocytosis","display_name":"Langerhans cell histiocytosis","score":1.0},{"id":"https://openalex.org/keywords/juvenile-xanthogranuloma","display_name":"juvenile xanthogranuloma","score":0.9990000128746033},{"id":"https://openalex.org/keywords/rosai-dorfman-disease","display_name":"Rosai-Dorfman disease","score":0.9980000257492065},{"id":"https://openalex.org/keywords/hemophagocytic-lymphohistiocytosis","display_name":"hemophagocytic lymphohistiocytosis","score":0.9959999918937683},{"id":"https://openalex.org/keywords/mapk-pathway-mutations","display_name":"MAPK pathway mutations","score":0.9020000100135803},{"id":"https://openalex.org/keywords/braf-v600e-mutation","display_name":"BRAF V600E mutation","score":0.9800000190734863},{"id":"https://openalex.org/keywords/targeted-therapy","display_name":"targeted therapy","score":0.7170000076293945},{"id":"https://openalex.org/keywords/emapalumab","display_name":"emapalumab","score":0.8569999933242798},{"id":"https://openalex.org/keywords/interferon-gamma-signaling","display_name":"interferon-gamma signaling","score":0.9399999976158142},{"id":"https://openalex.org/keywords/myeloid-neoplasms","display_name":"myeloid neoplasms","score":0.8949999809265137},{"id":"https://openalex.org/keywords/immune-dysregulation","display_name":"immune dysregulation","score":0.9179999828338623},{"id":"https://openalex.org/keywords/long-term-sequelae","display_name":"long-term sequelae","score":0.6600000262260437}],"concepts":[{"id":"https://openalex.org/C43100952","wikidata":"https://www.wikidata.org/wiki/Q654612","display_name":"Histiocyte","level":2,"score":0.7773206233978271},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.7080840468406677},{"id":"https://openalex.org/C2779079688","wikidata":"https://www.wikidata.org/wiki/Q1642170","display_name":"Hemophagocytic lymphohistiocytosis","level":3,"score":0.7052290439605713},{"id":"https://openalex.org/C2780194698","wikidata":"https://www.wikidata.org/wiki/Q6005429","display_name":"Immune dysregulation","level":3,"score":0.6730821132659912},{"id":"https://openalex.org/C2777484599","wikidata":"https://www.wikidata.org/wiki/Q374036","display_name":"Langerhans cell histiocytosis","level":3,"score":0.6702108383178711},{"id":"https://openalex.org/C2780174970","wikidata":"https://www.wikidata.org/wiki/Q1411740","display_name":"Histiocytosis","level":3,"score":0.6212851405143738},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.5334563255310059},{"id":"https://openalex.org/C2778175462","wikidata":"https://www.wikidata.org/wiki/Q2165663","display_name":"Chronic granulomatous disease","level":2,"score":0.48453181982040405},{"id":"https://openalex.org/C2779282312","wikidata":"https://www.wikidata.org/wiki/Q1956561","display_name":"Myeloid","level":2,"score":0.4772191643714905},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.45138248801231384},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.4467353820800781},{"id":"https://openalex.org/C2777060389","wikidata":"https://www.wikidata.org/wiki/Q3570558","display_name":"Juvenile xanthogranuloma","level":3,"score":0.4435196816921234},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.24530580639839172}],"mesh":[{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006644","descriptor_name":"Histiocytes","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D006646","descriptor_name":"Histiocytosis, Langerhans-Cell","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D006646","descriptor_name":"Histiocytosis, Langerhans-Cell","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D006646","descriptor_name":"Histiocytosis, Langerhans-Cell","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007371","descriptor_name":"Interferon-gamma","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D014972","descriptor_name":"Xanthogranuloma, Juvenile","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":true},{"descriptor_ui":"D014972","descriptor_name":"Xanthogranuloma, Juvenile","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D014972","descriptor_name":"Xanthogranuloma, Juvenile","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D015618","descriptor_name":"Histiocytosis, Sinus","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":true},{"descriptor_ui":"D020928","descriptor_name":"Mitogen-Activated Protein Kinases","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.1542/pir.2021-005367","doi":"https://doi.org/10.1542/pir.2021-005367","is_oa":false,"landing_page_url":"https://doi.org/10.1542/pir.2021-005367","pdf_url":null,"source":{"id":"https://openalex.org/S118940750","display_name":"Pediatrics in Review","issn_l":"0191-9601","issn":["0191-9601","1526-3347","1529-7233"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-c","jufo-1","medline","norway-1","poland-40","russia-white-list-2"],"host_organization":"https://openalex.org/P4310315734","host_organization_name":"American Academy of Pediatrics","host_organization_lineage":["https://openalex.org/P4310315734"],"host_organization_lineage_names":["American Academy of Pediatrics"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pediatrics In Review","raw_type":"journal-article"},{"id":"pmid:36180546","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/36180546","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":"Pediatrics in review","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":0.9922999739646912}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":61,"referenced_works":["https://openalex.org/W16222215","https://openalex.org/W1530342027","https://openalex.org/W1836851208","https://openalex.org/W1914761215","https://openalex.org/W1967246357","https://openalex.org/W1973260302","https://openalex.org/W1974779668","https://openalex.org/W1983067071","https://openalex.org/W1992672718","https://openalex.org/W2000454164","https://openalex.org/W2006268804","https://openalex.org/W2009347177","https://openalex.org/W2023594410","https://openalex.org/W2041650858","https://openalex.org/W2045372770","https://openalex.org/W2048765718","https://openalex.org/W2050404580","https://openalex.org/W2051898696","https://openalex.org/W2055389755","https://openalex.org/W2057410184","https://openalex.org/W2072020294","https://openalex.org/W2077098367","https://openalex.org/W2081669045","https://openalex.org/W2106451155","https://openalex.org/W2107166045","https://openalex.org/W2110914459","https://openalex.org/W2122175476","https://openalex.org/W2145327925","https://openalex.org/W2155477306","https://openalex.org/W2157171689","https://openalex.org/W2161282095","https://openalex.org/W2161616892","https://openalex.org/W2162251899","https://openalex.org/W2166173433","https://openalex.org/W2172043322","https://openalex.org/W2234683072","https://openalex.org/W2295097092","https://openalex.org/W2395217751","https://openalex.org/W2411184154","https://openalex.org/W2587847258","https://openalex.org/W2724414938","https://openalex.org/W2756862815","https://openalex.org/W2757190301","https://openalex.org/W2770975168","https://openalex.org/W2780808014","https://openalex.org/W2796087719","https://openalex.org/W2797160725","https://openalex.org/W2802582740","https://openalex.org/W2905376779","https://openalex.org/W2912707964","https://openalex.org/W2921153272","https://openalex.org/W2963476527","https://openalex.org/W2972707815","https://openalex.org/W2984662023","https://openalex.org/W2989688132","https://openalex.org/W3021363743","https://openalex.org/W3033907671","https://openalex.org/W3092735206","https://openalex.org/W3124631467","https://openalex.org/W3196325957","https://openalex.org/W7010203083"],"related_works":["https://openalex.org/W4245550761","https://openalex.org/W2938550938","https://openalex.org/W3168739199","https://openalex.org/W4377093299","https://openalex.org/W4404967241","https://openalex.org/W2284067977","https://openalex.org/W2083353372","https://openalex.org/W2910138223","https://openalex.org/W4386425088","https://openalex.org/W4200432403"],"abstract_inverted_index":{"Histiocytic":[0],"disorders":[1],"of":[2,8,16,32,48,63,76,165,191,226],"childhood":[3],"represent":[4],"a":[5,189],"wide":[6],"spectrum":[7],"conditions":[9,147,167],"that":[10],"share":[11],"the":[12,26,163],"common":[13,119],"histologic":[14,127],"feature":[15],"activated":[17],"or":[18,53,247],"transformed":[19],"\"histiocytes.\"":[20],"Langerhans":[21],"cell":[22],"histiocytosis":[23],"(LCH)":[24],"is":[25,71,187,195,215],"most":[27],"common,":[28],"with":[29,65,73,98,104,231,245],"an":[30],"incidence":[31],"approximately":[33,209,232],"5":[34],"per":[35,212],"million":[36,213],"children.":[37],"LCH":[38,121],"may":[39],"be":[40,154],"difficult":[41],"to":[42,58,101],"distinguish":[43],"from":[44],"more":[45,60],"ubiquitous":[46],"causes":[47],"skin":[49],"rashes,":[50],"bone":[51],"pain,":[52],"fever.":[54],"Current":[55],"chemotherapy":[56],"fails":[57],"cure":[59],"than":[61,120],"50%":[62],"children":[64,228],"multifocal":[66],"disease,":[67],"and":[68,113,122,128,141,174,214,222,250],"treatment":[69],"failure":[70],"associated":[72],"increased":[74],"risks":[75],"long-term":[77],"sequelae.":[78],"Somatic":[79],"activating":[80],"mitogen-activated":[81],"protein":[82],"kinase":[83],"(MAPK)":[84],"pathway-activating":[85],"mutations":[86,135],"(most":[87],"often":[88],"BRAFV600E)":[89],"have":[90],"been":[91,159],"identified":[92,138],"in":[93,96,139,208],"hematopoietic":[94],"precursors":[95],"patients":[97,244],"LCH.":[99],"Opportunities":[100],"improve":[102],"outcomes":[103],"targeted":[105],"therapies":[106,253],"are":[107,117,123,136,176,229,254],"under":[108,255],"investigation.":[109,256],"Juvenile":[110],"xanthogranuloma":[111],"(JXG)":[112],"Rosai-Dorfman":[114],"disease":[115,152],"(RDD)":[116],"less":[118],"distinguished":[124],"by":[125,197,202],"specific":[126],"clinical":[129],"features.":[130],"Recurrent":[131],"MAPK":[132],"pathway":[133],"gene":[134],"also":[137],"JXG":[140],"RDD.":[142],"In":[143,182],"many":[144],"cases,":[145],"these":[146,166],"spontaneously":[148],"resolve,":[149],"but":[150],"disseminated":[151],"can":[153],"fatal.":[155],"Although":[156],"there":[157],"has":[158],"historic":[160],"debate":[161],"regarding":[162],"nature":[164],"as":[168],"inflammatory":[169],"versus":[170],"neoplastic,":[171],"LCH,":[172],"JXG,":[173],"RDD":[175],"now":[177],"considered":[178],"myeloid":[179],"neoplastic":[180],"disorders.":[181],"contrast,":[183],"hemophagocytic":[184],"lymphohistiocytosis":[185],"(HLH)":[186],"clearly":[188],"disorder":[190],"immune":[192,199,223],"dysregulation.":[193],"HLH":[194,206],"characterized":[196],"extreme":[198],"activation":[200],"driven":[201],"hyperactivated":[203],"T":[204],"cells.":[205],"arises":[207],"1":[210],"child":[211],"nearly":[216],"universally":[217],"fatal":[218],"without":[219],"prompt":[220],"recognition":[221],"suppression.":[224],"Outcomes":[225],"treated":[227],"poor,":[230],"60%":[233],"survival.":[234],"Emapalumab,":[235],"which":[236],"targets":[237],"interferon-\u03b3":[238],"signaling,":[239],"was":[240],"recently":[241],"approved":[242],"for":[243],"recurrent":[246],"refractory":[248],"HLH,":[249],"additional":[251],"cytokine-directed":[252]},"counts_by_year":[{"year":2025,"cited_by_count":3},{"year":2024,"cited_by_count":3},{"year":2023,"cited_by_count":2}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2022-10-01T00:00:00"}