{"id":"https://openalex.org/W2590903998","doi":"https://doi.org/10.1182/blood.v114.22.1293.1293","title":"The Power of a Standardized Bleeding Score in Diagnosing Pediatric Type 1 Von Willebrand Disease.","display_name":"The Power of a Standardized Bleeding Score in Diagnosing Pediatric Type 1 Von Willebrand Disease.","publication_year":2009,"publication_date":"2009-11-20","ids":{"openalex":"https://openalex.org/W2590903998","doi":"https://doi.org/10.1182/blood.v114.22.1293.1293","mag":"2590903998"},"language":"en","primary_location":{"id":"doi:10.1182/blood.v114.22.1293.1293","doi":"https://doi.org/10.1182/blood.v114.22.1293.1293","is_oa":false,"landing_page_url":"https://doi.org/10.1182/blood.v114.22.1293.1293","pdf_url":null,"source":{"id":"https://openalex.org/S200071133","display_name":"Blood","issn_l":"0006-4971","issn":["0006-4971","1528-0020"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Blood","raw_type":"journal-article"},"volume":null,"type":"article","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/A5079866262","display_name":"Paul Marcus","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I52357470","display_name":"The Ohio State University","ror":"https://ror.org/00rs6vg23","country_code":"US","type":"education","lineage":["https://openalex.org/I52357470"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Paul D Marcus","raw_affiliation_strings":["College of Medicine, The Ohio State University, Columbus, OH, USA,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"College of Medicine, The Ohio State University, Columbus, OH, USA,","institution_ids":["https://openalex.org/I52357470"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5008344204","display_name":"Kidan G. Nire","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1314135232","display_name":"Nationwide Children's Hospital","ror":"https://ror.org/003rfsp33","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1314135232"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Kidan G Nire","raw_affiliation_strings":["Center for Innovation in Pediatric Practice, The Research Institute, Nationwide Children's Hospital, Columbus, OH,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Center for Innovation in Pediatric Practice, The Research Institute, Nationwide Children's Hospital, Columbus, OH,","institution_ids":["https://openalex.org/I1314135232"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111776763","display_name":"Linda P. Grooms","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1314135232","display_name":"Nationwide Children's Hospital","ror":"https://ror.org/003rfsp33","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1314135232"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Linda Grooms","raw_affiliation_strings":["Division of Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH, USA","institution_ids":["https://openalex.org/I1314135232"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5033556548","display_name":"Jennifer Klima","orcid":"https://orcid.org/0000-0002-4971-7614","observed_orcids":["https://orcid.org/0000-0002-4971-7614"]},"institutions":[{"id":"https://openalex.org/I1314135232","display_name":"Nationwide Children's Hospital","ror":"https://ror.org/003rfsp33","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1314135232"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Jennifer Klima","raw_affiliation_strings":["Center for Innovation in Pediatric Practice, The Research Institute, Nationwide Children's Hospital, Columbus, OH,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Center for Innovation in Pediatric Practice, The Research Institute, Nationwide Children's Hospital, Columbus, OH,","institution_ids":["https://openalex.org/I1314135232"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5050829820","display_name":"Sarah H. O\u2019Brien","orcid":"https://orcid.org/0000-0001-8855-9746","observed_orcids":["https://orcid.org/0000-0001-8855-9746","https://orcid.org/0000-0001-9939-6292"]},"institutions":[{"id":"https://openalex.org/I1314135232","display_name":"Nationwide Children's Hospital","ror":"https://ror.org/003rfsp33","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1314135232"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sarah O'Brien","raw_affiliation_strings":["Division of Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH, USA","institution_ids":["https://openalex.org/I1314135232"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5008344204","https://openalex.org/A5033556548","https://openalex.org/A5111776763"],"corresponding_institution_ids":["https://openalex.org/I1314135232"],"apc_list":{"value":5500,"currency":"USD","value_usd":5500},"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.41270186,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"114","issue":"22","first_page":"1293","last_page":"1293"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10746","display_name":"Platelet Disorders and Treatments","score":0.9897000193595886,"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/T10746","display_name":"Platelet Disorders and Treatments","score":0.9897000193595886,"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/T11280","display_name":"Hemophilia Treatment and Research","score":0.007699999958276749,"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/T10881","display_name":"Blood Coagulation and Thrombosis Mechanisms","score":0.00019999999494757503,"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/type-1-von-willebrand-disease","display_name":"type 1 von Willebrand disease","score":0.9890000224113464},{"id":"https://openalex.org/keywords/pediatric-bleeding-disorders","display_name":"pediatric bleeding disorders","score":0.7440000176429749},{"id":"https://openalex.org/keywords/vwf-ag","display_name":"VWF:Ag","score":0.6679999828338623},{"id":"https://openalex.org/keywords/vwf-rco","display_name":"VWF:RCo","score":0.9800000190734863},{"id":"https://openalex.org/keywords/bleeding-manifestations","display_name":"bleeding manifestations","score":0.3930000066757202},{"id":"https://openalex.org/keywords/diagnostic-workup","display_name":"diagnostic workup","score":0.37700000405311584},{"id":"https://openalex.org/keywords/pfa-100","display_name":"PFA-100","score":0.7960000038146973},{"id":"https://openalex.org/keywords/platelet-aggregation","display_name":"platelet aggregation","score":0.5809999704360962},{"id":"https://openalex.org/keywords/inherited-bleeding-disorders","display_name":"inherited bleeding disorders","score":0.578000009059906},{"id":"https://openalex.org/keywords/questionnaire-validation","display_name":"questionnaire validation","score":0.5170000195503235},{"id":"https://openalex.org/keywords/preoperative-testing","display_name":"preoperative screening","score":0.5260000228881836},{"id":"https://openalex.org/keywords/hemostasis","display_name":"hemostasis","score":0.4359999895095825}],"concepts":[{"id":"https://openalex.org/C2778780528","wikidata":"https://www.wikidata.org/wiki/Q709360","display_name":"Von Willebrand disease","level":4,"score":0.8602895736694336},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8146010041236877},{"id":"https://openalex.org/C2779394231","wikidata":"https://www.wikidata.org/wiki/Q412310","display_name":"Von Willebrand factor","level":3,"score":0.6608322858810425},{"id":"https://openalex.org/C187212893","wikidata":"https://www.wikidata.org/wiki/Q123028","display_name":"Pediatrics","level":1,"score":0.5589893460273743},{"id":"https://openalex.org/C2776259030","wikidata":"https://www.wikidata.org/wiki/Q769508","display_name":"Bleed","level":2,"score":0.5552965402603149},{"id":"https://openalex.org/C2908647359","wikidata":"https://www.wikidata.org/wiki/Q2625603","display_name":"Population","level":2,"score":0.5385223031044006},{"id":"https://openalex.org/C2778589496","wikidata":"https://www.wikidata.org/wiki/Q487937","display_name":"Hemostasis","level":2,"score":0.48076245188713074},{"id":"https://openalex.org/C2776217217","wikidata":"https://www.wikidata.org/wiki/Q611571","display_name":"Bleeding diathesis","level":3,"score":0.42770320177078247},{"id":"https://openalex.org/C2780868729","wikidata":"https://www.wikidata.org/wiki/Q261327","display_name":"Thrombosis","level":2,"score":0.42577287554740906},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.40252238512039185},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.28640973567962646},{"id":"https://openalex.org/C89560881","wikidata":"https://www.wikidata.org/wiki/Q101026","display_name":"Platelet","level":2,"score":0.16567149758338928},{"id":"https://openalex.org/C99454951","wikidata":"https://www.wikidata.org/wiki/Q932068","display_name":"Environmental health","level":1,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1182/blood.v114.22.1293.1293","doi":"https://doi.org/10.1182/blood.v114.22.1293.1293","is_oa":false,"landing_page_url":"https://doi.org/10.1182/blood.v114.22.1293.1293","pdf_url":null,"source":{"id":"https://openalex.org/S200071133","display_name":"Blood","issn_l":"0006-4971","issn":["0006-4971","1528-0020"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Blood","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"score":0.9922999739646912,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W1547206336","https://openalex.org/W3007799710","https://openalex.org/W2578863070","https://openalex.org/W2416422923","https://openalex.org/W2012197767","https://openalex.org/W2027991810","https://openalex.org/W2166751674","https://openalex.org/W1533889259","https://openalex.org/W1965653365","https://openalex.org/W4293454585"],"abstract_inverted_index":{"Abstract":[0,1],"1293":[2],"Poster":[3],"Board":[4],"I-315":[5],"INTRODUCTION":[6],"Type":[7,222,359,448,517],"I":[8],"von":[9,23],"Willebrand":[10,24],"disease":[11],"(VWD)":[12],"is":[13,28,69,526],"the":[14,31,114,168,180,189,195,198,202,275,324,329,351,410,427,439,507,520],"most":[15],"common":[16],"inherited":[17],"bleeding":[18,51,132,138,157,186,380,400,428,461],"disorder.":[19],"Repetitive":[20],"testing":[21,62,68],"of":[22,73,120,131,136,184,201,291,303,323,332,398,426,441,522,533],"factor":[25],"(VWF)":[26],"levels":[27,40,226],"necessary":[29],"before":[30],"diagnosis":[32,181],"can":[33],"be":[34,72],"safely":[35],"ruled":[36],"out,":[37],"as":[38,128,220,232,404],"VWF":[39,504],"fluctuate":[41],"in":[42,97,188,321,434,444,474,477,489],"response":[43],"to":[44,71,84,109,113,179,193,309,338,348,382,484,514,535],"genetic":[45],"and":[46,63,116,174,274,296,376,421,463,476],"environmental":[47],"factors.":[48],"A":[49,211,289],"predictive":[50,414,418,432,487],"score":[52,204,343,509],"(BS)":[53],"could":[54],"reveal":[55],"individuals":[56],"that":[57],"may":[58],"benefit":[59],"from":[60,227,259,264],"repetitive":[61,67],"those":[64],"for":[65,93,124,241,357,365],"whom":[66],"unlikely":[70],"benefit.":[74],"While":[75,506],"a":[76,94,98,125,129,137,154,185,300,372,399,478,490,511],"standardized":[77],"questionnaire":[78,196,429],"(the":[79],"Vicenza":[80,203,342,470,508],"score)":[81],"was":[82,89,218,278,319,344,454],"developed":[83],"evaluate":[85],"hemorrhagic":[86,317],"symptoms,":[87,133],"it":[88],"never":[90],"prospectively":[91],"validated":[92,473],"pediatric":[95,479,491],"population":[96,437],"tertiary":[99,492],"care":[100,481,493],"setting.":[101],"SUBJECTS":[102],"The":[103,340,452,469],"study":[104],"targeted":[105],"children,":[106,353],"ages":[107],"0":[108,337],"17":[110,310],"years,":[111],"referred":[112],"Hemostasis":[115],"Thrombosis":[117],"Center":[118],"(HTC)":[119],"Nationwide":[121],"Children's":[122],"Hospital":[123],"coagulation":[126,142],"evaluation":[127],"result":[130],"family":[134],"history":[135],"disorder":[139,187,401],"and/or":[140,253],"abnormal":[141],"labs":[143],"found":[144],"during":[145],"pre-operative":[146],"screening.":[147],"Children":[148,386],"were":[149,230,262,269,312,369,402],"excluded":[150,263],"if":[151,159,167],"they":[152],"had":[153,379],"previously":[155,472],"diagnosed":[156,370],"disorder,":[158],"their":[160],"caregiver":[161],"did":[162,170],"not":[163,171],"speak":[164],"English":[165],"or":[166,182,214,394,502],"child":[169],"undergo":[172],"VWF:Ag":[173,213],"VWF:RCo":[175,215],"testing.":[176],"METHODS":[177],"Prior":[178],"exclusion":[183],"child,":[190],"caregivers":[191],"consented":[192],"answer":[194],"over":[197],"telephone.":[199],"Descriptions":[200],"are":[205],"available":[206],"online":[207],"(http://www.euvwd.group.shef.ac.uk/bleed_score.htm).":[208],"LABORATORY":[209],"TESTING":[210],"single":[212],"<30":[216],"IU/dL":[217,229],"classified":[219,231,403],"\u201cDefinite":[221,358,447,516],"1":[223,307,360,449,518],"VWD\u201d":[224,450],"while":[225],"30-50":[228],"\u201cLow":[233,366],"VWF\u201d":[234],"(http://www.nhlbi.nih.gov/guidelines/vwd).":[235],"Platelet":[236],"function":[237,243,500],"analysis":[238],"(PFA-100)":[239],"screened":[240],"platelet":[242,250,254,499],"defects,":[244],"with":[245,280,299,328,371,387,438,457,498],"some":[246],"patients":[247,497],"undergoing":[248],"follow-up":[249],"aggregation":[251],"studies":[252,258],"electron":[255],"microscopy.":[256],"Laboratory":[257],"other":[260],"institutions":[261],"analysis.":[265],"Patients'":[266],"medical":[267],"records":[268],"reviewed":[270],"after":[271],"hematologic":[272],"evaluation,":[273],"resultant":[276],"data":[277],"analyzed":[279],"STATA":[281],"10.1":[282],"(Stata":[283],"Corp.,":[284],"College":[285],"Station,":[286],"TX).":[287],"RESULTS":[288],"total":[290],"104":[292,325,352],"children":[293,326,378],"(52":[294],"females":[295],"52":[297],"males)":[298],"mean":[301,330,341],"age":[302],"7.53":[304],"years":[305],"(range":[306,336,346],"month":[308],"years)":[311],"included.":[313],"At":[314],"least":[315],"one":[316],"symptom":[318],"present":[320],"99":[322],"(95%)":[327],"number":[331],"symptoms":[333],"being":[334],"2.87":[335],"7).":[339],"3.24":[345],"-1":[347],"13).":[349],"Of":[350],"8":[354],"met":[355,363],"criteria":[356,364,525],"VWD,\u201d":[361,519],"23":[362],"VWF,\u201d":[367],"14":[368],"\u201cPlatelet":[373],"Function":[374],"Defect,\u201d":[375],"2":[377],"secondary":[381],"Ehlers":[383],"Danlos":[384],"syndrome.":[385],"non-bleeding":[388],"disorders":[389],"(e.g.":[390],"Factor":[391],"XII":[392],"deficiency)":[393],"no":[395],"laboratory":[396],"evidence":[397],"\u201cNo":[405],"Bleeding":[406],"Disorder.\u201d":[407],"In":[408],"general,":[409],"sensitivity,":[411],"specificity,":[412],"positive":[413,422],"value":[415,419,433,488],"(PPV),":[416],"negative":[417],"(NPV)":[420],"diagnostic":[423],"likelihood":[424],"ratio":[425],"demonstrated":[430],"poor":[431],"our":[435],"patient":[436],"exception":[440],"high":[442,456,512],"specificity":[443],"ruling":[445],"out":[446],"(Table).":[451],"NPV":[453,513],"comparably":[455],"both":[458],"qualitative":[459,524],"(>2":[460],"symptoms)":[462],"quantitative":[464],"(BS":[465],"\u22652)":[466],"criteria.":[467],"CONCLUSIONS":[468],"score,":[471],"adults":[475],"primary":[480],"setting,":[482],"appears":[483],"have":[485],"limited":[486],"setting":[494],"when":[495],"evaluating":[496],"defects":[501],"low":[503],"levels.":[505],"has":[510],"exclude":[515],"use":[521],"simpler":[523],"similarly":[527],"predictive.":[528],"Disclosures":[529],"No":[530],"relevant":[531],"conflicts":[532],"interest":[534],"declare.":[536]},"counts_by_year":[],"updated_date":"2026-10-01T08:54:57.152042","created_date":"2017-03-03T00:00:00"}