{"id":"https://openalex.org/W1535206124","doi":"https://doi.org/10.1542/peds.47.5.927","title":"PRIMARY IMMUNODEFICIENCIES","display_name":"PRIMARY IMMUNODEFICIENCIES","publication_year":1971,"publication_date":"1971-05-01","ids":{"openalex":"https://openalex.org/W1535206124","doi":"https://doi.org/10.1542/peds.47.5.927","mag":"1535206124","pmid":"https://pubmed.ncbi.nlm.nih.gov/4102352"},"language":"en","primary_location":{"id":"doi:10.1542/peds.47.5.927","doi":"https://doi.org/10.1542/peds.47.5.927","is_oa":false,"landing_page_url":"https://doi.org/10.1542/peds.47.5.927","pdf_url":null,"source":{"id":"https://openalex.org/S77494981","display_name":"PEDIATRICS","issn_l":"0031-4005","issn":["0031-4005","1098-4275"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","dongbi-a","doyens","erih-plus","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"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","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/A5010695214","display_name":"H. Hugh Fudenberg","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"H. Fudenberg","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5112156868","display_name":"R. A. Good","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"R. A. Good","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5110400956","display_name":"Howard C. Goodman","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"H. C. Goodman","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5110842174","display_name":"Walter Hermann Hitzig","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"W. Hitzig","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5113491670","display_name":"HERBERT G. KUNKEL","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"H. G. Kunkel","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5110430694","display_name":"Ivan M. Roitt","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"I. M. Roitt","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111433586","display_name":"Fred S. Rosen","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"F. S. Rosen","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5023788420","display_name":"DAVID S. ROWE","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"D. S. Rowe","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5113113414","display_name":"Maxime Seligmann","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"M. Seligmann","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5108521463","display_name":"J F Soothill","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"J. R. Soothill","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":20.7118,"has_fulltext":false,"cited_by_count":338,"citation_normalized_percentile":{"value":0.99682327,"is_in_top_1_percent":true,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":88,"max":98},"biblio":{"volume":"47","issue":"5","first_page":"927","last_page":"946"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10031","display_name":"T-cell and B-cell Immunology","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T12919","display_name":"Blood disorders and treatments","score":0.0,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"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/primary-immunodeficiency","display_name":"primary immunodeficiency","score":0.9890000224113464},{"id":"https://openalex.org/keywords/antibody-standardization","display_name":"antibody standardization","score":0.7039999961853027},{"id":"https://openalex.org/keywords/serum-antibody-measurement","display_name":"serum antibody measurement","score":0.7269999980926514},{"id":"https://openalex.org/keywords/immunoglobulin-therapy","display_name":"immunoglobulin therapy","score":0.8640000224113464},{"id":"https://openalex.org/keywords/plasma-therapy","display_name":"plasma therapy","score":0.7070000171661377},{"id":"https://openalex.org/keywords/cohn-fractionation","display_name":"Cohn fractionation","score":0.6299999952316284},{"id":"https://openalex.org/keywords/lymphocyte-destruction","display_name":"lymphocyte destruction","score":0.5090000033378601}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8870737552642822},{"id":"https://openalex.org/C159654299","wikidata":"https://www.wikidata.org/wiki/Q79460","display_name":"Antibody","level":2,"score":0.5778133869171143},{"id":"https://openalex.org/C2910905871","wikidata":"https://www.wikidata.org/wiki/Q641307","display_name":"Immunodeficiency Syndrome","level":4,"score":0.5728004574775696},{"id":"https://openalex.org/C2777607303","wikidata":"https://www.wikidata.org/wiki/Q641307","display_name":"Immunodeficiency","level":3,"score":0.5557309985160828},{"id":"https://openalex.org/C2779019163","wikidata":"https://www.wikidata.org/wiki/Q3043160","display_name":"Primary immunodeficiency","level":3,"score":0.505530834197998},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.5014095306396484},{"id":"https://openalex.org/C2779468541","wikidata":"https://www.wikidata.org/wiki/Q1334408","display_name":"Severe combined immunodeficiency","level":3,"score":0.4916990399360657},{"id":"https://openalex.org/C207001950","wikidata":"https://www.wikidata.org/wiki/Q141124","display_name":"In vivo","level":2,"score":0.48668867349624634},{"id":"https://openalex.org/C147483822","wikidata":"https://www.wikidata.org/wiki/Q103537","display_name":"Antigen","level":2,"score":0.44349050521850586},{"id":"https://openalex.org/C2779341262","wikidata":"https://www.wikidata.org/wiki/Q182581","display_name":"Immunity","level":3,"score":0.4366964101791382},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.22128957509994507},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.0},{"id":"https://openalex.org/C150903083","wikidata":"https://www.wikidata.org/wiki/Q7108","display_name":"Biotechnology","level":1,"score":0.0}],"mesh":[{"descriptor_ui":"D000906","descriptor_name":"Antibodies","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000921","descriptor_name":"Antibody-Producing Cells","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001803","descriptor_name":"Blood Transfusion","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005719","descriptor_name":"gamma-Globulins","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005779","descriptor_name":"Immunodiffusion","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005796","descriptor_name":"Genes","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005809","descriptor_name":"Genes, Regulator","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006412","descriptor_name":"Hematopoietic Stem Cells","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007111","descriptor_name":"Immunity, Cellular","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007114","descriptor_name":"Immunization","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007122","descriptor_name":"Immunoelectrophoresis","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007125","descriptor_name":"Immunogenetics","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007136","descriptor_name":"Immunoglobulins","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007153","descriptor_name":"Immunologic Deficiency Syndromes","qualifier_ui":"Q000145","qualifier_name":"classification","is_major_topic":true},{"descriptor_ui":"D007153","descriptor_name":"Immunologic Deficiency Syndromes","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D007153","descriptor_name":"Immunologic Deficiency Syndromes","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D008213","descriptor_name":"Lymphocyte Activation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008214","descriptor_name":"Lymphocytes","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D012042","descriptor_name":"Registries","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D013950","descriptor_name":"Thymus Gland","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D014184","descriptor_name":"Transplantation, Homologous","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D014944","descriptor_name":"World Health Organization","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.1542/peds.47.5.927","doi":"https://doi.org/10.1542/peds.47.5.927","is_oa":false,"landing_page_url":"https://doi.org/10.1542/peds.47.5.927","pdf_url":null,"source":{"id":"https://openalex.org/S77494981","display_name":"PEDIATRICS","issn_l":"0031-4005","issn":["0031-4005","1098-4275"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","dongbi-a","doyens","erih-plus","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"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","raw_type":"journal-article"},{"id":"pmid:4102352","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/4102352","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","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"score":0.9763000011444092,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W2010100927","https://openalex.org/W2128907698","https://openalex.org/W2777493923","https://openalex.org/W2331632898","https://openalex.org/W2125520756","https://openalex.org/W4251871634","https://openalex.org/W2460490200","https://openalex.org/W7038617349","https://openalex.org/W142982900","https://openalex.org/W2249429552"],"abstract_inverted_index":{"1.":[0],"Two":[1],"registries":[2],"are":[3,128],"to":[4,95,125,176,200,267,313],"be":[5,58,82,96,154,159,177,201,208,223,264,282,299,344],"established":[6],"in":[7,75,113,180,214,251,324],"association":[8],"with":[9,11,77,226,248,259,274,277,305,337,351],"WHO,":[10],"the":[12,26,111,184,255,269,319,347],"object":[13],"of":[14,21,33,40,71,118,138,150,186,257,265,272,293,303,317,322,329,339,349],"gathering":[15],"and":[16,63,107,121,156,179,194,210,247,276],"recording":[17],"information":[18],"on":[19,110,161],"cases":[20,32,70],"immunodeficiency":[22,35,352],"from":[23,335],"all":[24],"over":[25],"world.":[27],"One":[28,340],"registry":[29],"will":[30,57,68,81,89,105],"comprise":[31,69],"primary":[34,78],"that":[36],"have":[37,354],"received":[38],"transplants":[39],"any":[41],"immunologically":[42],"competent":[43],"organ,":[44],"cell,":[45],"or":[46,53,219],"cell":[47],"product,":[48],"e.g.,":[49],"bone":[50],"marrow,":[51],"thymus,":[52],"transfer":[54],"factor.":[55],"It":[56,262],"organized":[59,83],"by":[60,84,100,165],"W.":[61],"Hitzig":[62],"M.":[64],"Seligmann.":[65],"The":[66,103],"other":[67],"malignant":[72],"tumour":[73],"found":[74],"patients":[76,258,304,350],"immunodeficiency;":[79],"it":[80],"R.":[85],"A.":[86],"Good,":[87],"who":[88,353],"also":[90],"arrange":[91],"for":[92,98,135,140,163,171,183,192,217,225,236,254,284,301,346],"pathological":[93],"material":[94,112],"submitted":[97],"study":[99],"selected":[101],"pathologists.":[102],"organizers":[104],"collate":[106],"periodically":[108],"report":[109],"both":[114],"registries.":[115],"2.":[116],"Standardization":[117],"diagnostic":[119,126],"procedures":[120,206],"additional":[122],"studies":[123],"related":[124],"tests":[127,182,191,227],"required.":[129],"a.":[130,212],"There":[131],"is":[132,311],"a":[133],"need":[134,175,199],"standard":[136],"preparations":[137,216,289],"antigens":[139,152],"assessing":[141],"antibody":[142],"formation":[143],"as":[144,190,229,332],"described.":[145],"A":[146],"sufficiently":[147],"large":[148],"batch":[149],"such":[151,189,228],"should":[153,158,207,222,239],"prepared,":[155],"aliquots":[157],"sent,":[160],"request,":[162],"use":[164],"clinicians":[166],"investigating":[167],"immunodeficiency.":[168,260],"b.":[169,232],"Methods":[170],"measuring":[172],"serum":[173],"antibodies":[174,295],"standardized,":[178],"vitro":[181],"measurement":[185],"cell-mediated":[187],"immunity,":[188],"cytotoxicity":[193],"macrophage":[195],"migration":[196],"inhibition":[197],"factors,":[198],"further":[202],"developed.":[203],"3.":[204],"Therapeutic":[205],"standardized":[209],"assessed.":[211],"Aggregates":[213],"immunoglobulin":[215,237,275],"intramuscular":[218],"intravenous":[220],"injection":[221],"looked":[224],"those":[230],"recommended.":[231],"National":[233],"agencies":[234],"responsible":[235],"production":[238],"develop":[240,314],"stable":[241],"aggregate-free":[242],"intact":[243],"IgG,":[244],"without":[245],"fragments":[246],"relatively":[249],"normal":[250],"vivo":[252],"survival,":[253],"treatment":[256,302,348],"c.":[261],"would":[263],"value":[266],"determine":[268],"relative":[270],"effectiveness":[271],"therapy":[273],"plasma.":[278],"Clinical":[279],"trials":[280],"might":[281],"designed":[283],"this":[285],"purpose.":[286],"d.":[287],"Immunoglobulin":[288],"having":[290],"high":[291],"contents":[292],"specific":[294,306],"would,":[296],"if":[297],"available,":[298],"valuable":[300,345],"infections.":[307],"e.":[308],"Further":[309],"research":[310],"required":[312],"new":[315],"methods":[316],"destroying":[318],"biological":[320],"activity":[321],"lymphocytes":[323],"whole":[325],"blood.":[326],"f.":[327],"Pools":[328],"immunoglobulin,":[330],"prepared":[331],"Cohn":[333],"FII":[334],"donors":[336],"absence":[338],"genetic":[341],"allotype,":[342],"may":[343],"anti-allotype":[355],"antibodies.":[356]},"counts_by_year":[{"year":2026,"cited_by_count":3},{"year":2025,"cited_by_count":4},{"year":2024,"cited_by_count":6},{"year":2023,"cited_by_count":4},{"year":2022,"cited_by_count":2},{"year":2021,"cited_by_count":3},{"year":2020,"cited_by_count":1},{"year":2019,"cited_by_count":3},{"year":2018,"cited_by_count":3},{"year":2017,"cited_by_count":2},{"year":2016,"cited_by_count":1},{"year":2015,"cited_by_count":1},{"year":2014,"cited_by_count":2},{"year":2013,"cited_by_count":1}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2016-06-24T00:00:00"}