{"id":"https://openalex.org/W2936968561","doi":null,"title":"Two related cases of primary complement deficiencies.","display_name":"Two related cases of primary complement deficiencies.","publication_year":2003,"publication_date":"2003-06-01","ids":{"openalex":"https://openalex.org/W2936968561","mag":"2936968561","pmid":"https://pubmed.ncbi.nlm.nih.gov/17301359"},"language":"en","primary_location":{"id":"pmid:17301359","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/17301359","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":"Iranian journal of allergy, asthma, and immunology","raw_type":"Journal Article"},"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":null,"display_name":"A Farhoudi","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I70640408","display_name":"Tehran University of Medical Sciences","ror":"https://ror.org/01c4pz451","country_code":"IR","type":"education","lineage":["https://openalex.org/I70640408"]}],"countries":["IR"],"is_corresponding":false,"raw_author_name":"A Farhoudi","raw_affiliation_strings":["Immunology, Asthma and Allergy Research Institute; Children Hospital Medical Center; Tehran University of Medical Sciences, Tehran, Iran"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Immunology, Asthma and Allergy Research Institute; Children Hospital Medical Center; Tehran University of Medical Sciences, Tehran, Iran","institution_ids":["https://openalex.org/I70640408"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5016360540","display_name":"Nasrin Bazargan","orcid":"https://orcid.org/0000-0002-3100-9388","observed_orcids":["https://orcid.org/0000-0002-3100-9388"]},"institutions":[{"id":"https://openalex.org/I70640408","display_name":"Tehran University of Medical Sciences","ror":"https://ror.org/01c4pz451","country_code":"IR","type":"education","lineage":["https://openalex.org/I70640408"]}],"countries":["IR"],"is_corresponding":false,"raw_author_name":"Nasrin Bazargan","raw_affiliation_strings":["Tehran University of Medical Sciences, Tehran, Iran"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Tehran University of Medical Sciences, Tehran, Iran","institution_ids":["https://openalex.org/I70640408"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Zabra Pourpak","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I70640408","display_name":"Tehran University of Medical Sciences","ror":"https://ror.org/01c4pz451","country_code":"IR","type":"education","lineage":["https://openalex.org/I70640408"]}],"countries":["IR"],"is_corresponding":false,"raw_author_name":"Zabra Pourpak","raw_affiliation_strings":["Tehran University of Medical Sciences, Tehran, Iran"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Tehran University of Medical Sciences, Tehran, Iran","institution_ids":["https://openalex.org/I70640408"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5101864302","display_name":"Maryam Mahmoudi","orcid":"https://orcid.org/0000-0003-3656-2390","observed_orcids":["https://orcid.org/0000-0003-3656-2390"]},"institutions":[{"id":"https://openalex.org/I70640408","display_name":"Tehran University of Medical Sciences","ror":"https://ror.org/01c4pz451","country_code":"IR","type":"education","lineage":["https://openalex.org/I70640408"]}],"countries":["IR"],"is_corresponding":false,"raw_author_name":"Maryam Mahmoudi","raw_affiliation_strings":["Tehran University of Medical Sciences, Tehran, Iran"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Tehran University of Medical Sciences, Tehran, Iran","institution_ids":["https://openalex.org/I70640408"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":["https://openalex.org/I70640408"],"apc_list":null,"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":3,"citation_normalized_percentile":{"value":0.0,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":93},"biblio":{"volume":"2","issue":"2","first_page":"69","last_page":"74"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10993","display_name":"Complement system in diseases","score":0.948199987411499,"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/T10993","display_name":"Complement system in diseases","score":0.948199987411499,"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/T11917","display_name":"Coagulation, Bradykinin, Polyphosphates, and Angioedema","score":0.045899998396635056,"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/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":0.005400000140070915,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/c3-deficiency","display_name":"C3 deficiency","score":0.9309999942779541},{"id":"https://openalex.org/keywords/c1-inhibitor-deficiency","display_name":"C1 inhibitor deficiency","score":0.8100000023841858},{"id":"https://openalex.org/keywords/hereditary-angioedema","display_name":"hereditary angioedema","score":0.9470000267028809},{"id":"https://openalex.org/keywords/angioedema","display_name":"angioedema","score":0.800000011920929},{"id":"https://openalex.org/keywords/pneumococcal-meningitis","display_name":"pneumococcal meningitis","score":0.828000009059906},{"id":"https://openalex.org/keywords/glomerulonephritis","display_name":"glomerulonephritis","score":0.921999990940094},{"id":"https://openalex.org/keywords/c4-deficiency","display_name":"C4 deficiency","score":0.6790000200271606},{"id":"https://openalex.org/keywords/danazol","display_name":"danazol","score":0.7120000123977661},{"id":"https://openalex.org/keywords/stanozolol","display_name":"stanozolol","score":0.597000002861023},{"id":"https://openalex.org/keywords/antibiotic-prophylaxis","display_name":"antibiotic prophylaxis","score":0.6940000057220459}],"concepts":[{"id":"https://openalex.org/C2777788586","wikidata":"https://www.wikidata.org/wiki/Q2096745","display_name":"Hereditary angioedema","level":2,"score":0.8747225403785706},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8110366463661194},{"id":"https://openalex.org/C2777416354","wikidata":"https://www.wikidata.org/wiki/Q419652","display_name":"Danazol","level":3,"score":0.7119269371032715},{"id":"https://openalex.org/C2777995640","wikidata":"https://www.wikidata.org/wiki/Q1129007","display_name":"Angioedema","level":2,"score":0.6388267278671265},{"id":"https://openalex.org/C2779019163","wikidata":"https://www.wikidata.org/wiki/Q3043160","display_name":"Primary immunodeficiency","level":3,"score":0.581223726272583},{"id":"https://openalex.org/C2779931649","wikidata":"https://www.wikidata.org/wiki/Q416510","display_name":"C1-inhibitor","level":3,"score":0.49060001969337463},{"id":"https://openalex.org/C2780886150","wikidata":"https://www.wikidata.org/wiki/Q152234","display_name":"Edema","level":2,"score":0.4878096878528595},{"id":"https://openalex.org/C2776995267","wikidata":"https://www.wikidata.org/wiki/Q4165484","display_name":"Lymphoproliferative disorders","level":3,"score":0.458700567483902},{"id":"https://openalex.org/C16005928","wikidata":"https://www.wikidata.org/wiki/Q171171","display_name":"Dermatology","level":1,"score":0.4229079484939575},{"id":"https://openalex.org/C2781026528","wikidata":"https://www.wikidata.org/wiki/Q48143","display_name":"Meningitis","level":2,"score":0.41963934898376465},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3908863067626953},{"id":"https://openalex.org/C187212893","wikidata":"https://www.wikidata.org/wiki/Q123028","display_name":"Pediatrics","level":1,"score":0.37424203753471375},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.36499616503715515},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.3580172061920166},{"id":"https://openalex.org/C2779338263","wikidata":"https://www.wikidata.org/wiki/Q208414","display_name":"Lymphoma","level":2,"score":0.10504928231239319},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.07854166626930237},{"id":"https://openalex.org/C2779522080","wikidata":"https://www.wikidata.org/wiki/Q205764","display_name":"Endometriosis","level":2,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"pmid:17301359","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/17301359","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":"Iranian journal of allergy, asthma, and immunology","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"score":0.98580002784729,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"study_designs":[{"display_name":"Case Report","id":"https://openalex.org/study-designs/case-report"}],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":41,"referenced_works":["https://openalex.org/W41516964","https://openalex.org/W58690323","https://openalex.org/W93053989","https://openalex.org/W1501473386","https://openalex.org/W1547733031","https://openalex.org/W1594144334","https://openalex.org/W2008492357","https://openalex.org/W2014707628","https://openalex.org/W2045562495","https://openalex.org/W2066613071","https://openalex.org/W2098511755","https://openalex.org/W2100604852","https://openalex.org/W2101503666","https://openalex.org/W2105894548","https://openalex.org/W2142099365","https://openalex.org/W2144930991","https://openalex.org/W2150891147","https://openalex.org/W2172421250","https://openalex.org/W2337485820","https://openalex.org/W2409360547","https://openalex.org/W2409679560","https://openalex.org/W2414044484","https://openalex.org/W2508997451","https://openalex.org/W2520211681","https://openalex.org/W2542822064","https://openalex.org/W2769671204","https://openalex.org/W2791309620","https://openalex.org/W2895903821","https://openalex.org/W2909894833","https://openalex.org/W2981617674","https://openalex.org/W2990840371","https://openalex.org/W3016852213","https://openalex.org/W3016940380","https://openalex.org/W3025259451","https://openalex.org/W3028330817","https://openalex.org/W3034225365","https://openalex.org/W3035467903","https://openalex.org/W3036451283","https://openalex.org/W3092541624","https://openalex.org/W3147479802","https://openalex.org/W6776928405"],"related_works":["https://openalex.org/W1965261400","https://openalex.org/W2052754778","https://openalex.org/W2035749563","https://openalex.org/W2130248271","https://openalex.org/W4210698956","https://openalex.org/W4239811646","https://openalex.org/W3128981560","https://openalex.org/W4323361892","https://openalex.org/W3014035220","https://openalex.org/W2483592580"],"abstract_inverted_index":{"Primary":[0],"complement":[1],"deficiencies":[2],"are":[3,9,167],"rare":[4],"and":[5,27,62,87,90,118,162],"two":[6,177],"related":[7],"patients":[8,166],"reported":[10],"here.":[11],"The":[12,31,73,92,148,156],"first":[13],"patient":[14,57],"is":[15,76],"a":[16,45,77,145,172],"41-":[17],"year-":[18],"old":[19],"man":[20],"with":[21,82,106,160],"eighteen":[22],"episodes":[23,84],"of":[24,85,132],"pneumococcal":[25],"meningitis":[26,64],"other":[28,50,125],"purulent":[29],"infections.":[30],"serum":[32,93],"C3":[33,47],"level":[34],"was":[35,44,111,120,158],"checked":[36],"at":[37],"three":[38],"separate":[39],"times,":[40],"showing":[41],"that":[42],"this":[43,169],"primary":[46],"deficient":[48],"case;":[49],"immunological":[51,126],"tests":[52,127],"were":[53,96,102,128,138],"however":[54],"normal.":[55,129],"This":[56],"now":[58],"takes":[59],"prophylactic":[60],"antibiotics":[61],"the":[63],"has":[65],"not":[66],"recurred,":[67],"but":[68,124],"he":[69],"does":[70],"have":[71],"glomerulonephritis.":[72],"second":[74],"case":[75],"40":[78],"-":[79],"year-old":[80],"woman":[81],"repeated":[83],"orofacial":[86],"laryngeal":[88],"edema":[89],"dyspnea.":[91],"C1INH":[94],"levels":[95],"4.3":[97],"to":[98,153],"7":[99],"mgldL":[100],"which":[101],"very":[103],"low":[104],"compared":[105],"normal":[107,116,123],"healthy":[108],"subjects":[109],"(C1INH":[110],"40-50":[112],"mg/dL":[113],"in":[114],"ten":[115],"controls)":[117],"C4":[119],"lower":[121],"than":[122],"Other":[130],"causes":[131],"angioederna":[133],"such":[134],"as":[135],"lymphoproliferative":[136],"disorders":[137],"excluded.":[139],"She":[140],"had":[141],"hereditary":[142],"angioedema":[143,157],"without":[144],"family":[146],"background.":[147],"condition":[149],"may":[150,170],"be":[151],"due":[152],"genetic":[154,173],"mutation.":[155],"controlled":[159],"Danazol":[161],"Stanasol.":[163],"As":[164],"our":[165],"related,":[168],"suggest":[171],"relationship":[174],"between":[175],"these":[176],"disorders.":[178]},"counts_by_year":[{"year":2014,"cited_by_count":1},{"year":2012,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2019-04-25T00:00:00"}