{"id":"https://openalex.org/W85840762","doi":null,"title":"[Pathophysiology of thrombotic microangiopathies: current understanding].","display_name":"[Pathophysiology of thrombotic microangiopathies: current understanding].","publication_year":2002,"publication_date":"2002-05-01","ids":{"openalex":"https://openalex.org/W85840762","mag":"85840762","pmid":"https://pubmed.ncbi.nlm.nih.gov/12218898"},"language":"fr","primary_location":{"id":"pmid:12218898","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/12218898","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":"Annales de medecine interne","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":"https://openalex.org/A5041549015","display_name":"Paul Coppo","orcid":"https://orcid.org/0000-0002-4618-2095","observed_orcids":["https://orcid.org/0000-0002-4618-2095"]},"institutions":[{"id":"https://openalex.org/I2801843802","display_name":"Institut Gustave Roussy","ror":"https://ror.org/0321g0743","country_code":"FR","type":"facility","lineage":["https://openalex.org/I2801843802"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Paul Coppo","raw_affiliation_strings":["Laboratoire H\u00e9matopo\u00ef\u00e8se et Cellules souches (U362), Institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif. paulcoppo@aol.com"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Laboratoire H\u00e9matopo\u00ef\u00e8se et Cellules souches (U362), Institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif. paulcoppo@aol.com","institution_ids":["https://openalex.org/I2801843802"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5037817902","display_name":"Agn\u00e8s Veyradier","orcid":"https://orcid.org/0000-0001-9551-3076","observed_orcids":["https://orcid.org/0000-0001-9551-3076","https://orcid.org/0000-0001-7773-5003"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Agn\u00e8s Veyradier","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5076339052","display_name":"Marie\u2010Agn\u00e8s Dragon\u2010Durey","orcid":"https://orcid.org/0000-0002-5809-8122","observed_orcids":["https://orcid.org/0000-0002-5809-8122"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Marie-Agn\u00e8s Durey","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5025683973","display_name":"V\u00e9ronique Fr\u00e9meaux\u2010Bacchi","orcid":"https://orcid.org/0000-0002-4865-8528","observed_orcids":["https://orcid.org/0000-0002-4865-8528"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"V\u00e9ronique Fremeaux-Bacchi","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111865281","display_name":"Marie\u2010Lorraine Scrobohaci","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Marie-Lorraine Scrobohaci","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5110416495","display_name":"Fran\u00e7oise Amesland","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Fran\u00e7oise Amesland","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5001432138","display_name":"ANNETTE BUSSEL","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Annette Bussel","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":0.2108,"has_fulltext":false,"cited_by_count":15,"citation_normalized_percentile":{"value":0.47972447,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":87,"max":93},"biblio":{"volume":"153","issue":"3","first_page":"153","last_page":"66"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10993","display_name":"Complement system in diseases","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/T10993","display_name":"Complement system in diseases","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/T10881","display_name":"Blood Coagulation and Thrombosis Mechanisms","score":0.0,"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/T10746","display_name":"Platelet Disorders and Treatments","score":0.0,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/thrombotic-microangiopathy","display_name":"thrombotic microangiopathy","score":0.9819999933242798},{"id":"https://openalex.org/keywords/thrombotic-thrombocytopenic-purpura","display_name":"thrombotic thrombocytopenic purpura","score":0.9990000128746033},{"id":"https://openalex.org/keywords/hemolytic-uremic-syndrome","display_name":"hemolytic uremic syndrome","score":0.9990000128746033},{"id":"https://openalex.org/keywords/endothelial-injury","display_name":"endothelial injury","score":0.8970000147819519},{"id":"https://openalex.org/keywords/von-willebrand-factor-multimers","display_name":"von Willebrand factor multimers","score":0.8849999904632568},{"id":"https://openalex.org/keywords/autoantibodies","display_name":"autoantibodies","score":0.6990000009536743},{"id":"https://openalex.org/keywords/shiga-toxin","display_name":"Shiga toxin","score":0.8970000147819519},{"id":"https://openalex.org/keywords/atypical-hemolytic-uremic-syndrome","display_name":"atypical hemolytic uremic syndrome","score":0.7839999794960022},{"id":"https://openalex.org/keywords/complement-factor-h","display_name":"complement factor H","score":0.5849999785423279},{"id":"https://openalex.org/keywords/hypocomplementemia","display_name":"hypocomplementemia","score":0.9399999976158142},{"id":"https://openalex.org/keywords/microvascular-thrombosis","display_name":"microvascular thrombosis","score":0.8429999947547913},{"id":"https://openalex.org/keywords/precipitating-factors","display_name":"precipitating factors","score":0.4740000069141388}],"concepts":[{"id":"https://openalex.org/C2778585876","wikidata":"https://www.wikidata.org/wiki/Q1426491","display_name":"Thrombotic thrombocytopenic purpura","level":3,"score":0.9371145367622375},{"id":"https://openalex.org/C2779394231","wikidata":"https://www.wikidata.org/wiki/Q412310","display_name":"Von Willebrand factor","level":3,"score":0.7770612239837646},{"id":"https://openalex.org/C2781127562","wikidata":"https://www.wikidata.org/wiki/Q2063651","display_name":"Microangiopathic hemolytic anemia","level":4,"score":0.7424964904785156},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.6768973469734192},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.6112428307533264},{"id":"https://openalex.org/C2780921031","wikidata":"https://www.wikidata.org/wiki/Q14873945","display_name":"ADAMTS13","level":4,"score":0.6075244545936584},{"id":"https://openalex.org/C2776175824","wikidata":"https://www.wikidata.org/wiki/Q1145668","display_name":"Hemolytic anemia","level":2,"score":0.5286695957183838},{"id":"https://openalex.org/C89560881","wikidata":"https://www.wikidata.org/wiki/Q101026","display_name":"Platelet","level":2,"score":0.46664977073669434},{"id":"https://openalex.org/C65245452","wikidata":"https://www.wikidata.org/wiki/Q623225","display_name":"Schistocyte","level":4,"score":0.44274380803108215},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3300132751464844}],"mesh":[{"descriptor_ui":"D000071120","descriptor_name":"ADAMTS13 Protein","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006463","descriptor_name":"Hemolytic-Uremic Syndrome","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":false},{"descriptor_ui":"D006463","descriptor_name":"Hemolytic-Uremic Syndrome","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D006463","descriptor_name":"Hemolytic-Uremic Syndrome","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":true},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008666","descriptor_name":"Metalloendopeptidases","qualifier_ui":"Q000172","qualifier_name":"deficiency","is_major_topic":true},{"descriptor_ui":"D008833","descriptor_name":"Microcirculation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D010974","descriptor_name":"Platelet Aggregation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011697","descriptor_name":"Purpura, Thrombotic Thrombocytopenic","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":false},{"descriptor_ui":"D011697","descriptor_name":"Purpura, Thrombotic Thrombocytopenic","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D011697","descriptor_name":"Purpura, Thrombotic Thrombocytopenic","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":true},{"descriptor_ui":"D012307","descriptor_name":"Risk Factors","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D014841","descriptor_name":"von Willebrand Factor","qualifier_ui":"Q000502","qualifier_name":"physiology","is_major_topic":true},{"descriptor_ui":"D015551","descriptor_name":"Autoimmunity","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D017242","descriptor_name":"Complement Factor H","qualifier_ui":"Q000172","qualifier_name":"deficiency","is_major_topic":false},{"descriptor_ui":"D051722","descriptor_name":"ADAM Proteins","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"pmid:12218898","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/12218898","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":"Annales de medecine interne","raw_type":"Journal Article"},{"id":"mag:85840762","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/12218898/","pdf_url":null,"source":{"id":"https://openalex.org/S66188833","display_name":"Annales De Medecine Interne","issn_l":"0003-410X","issn":["0003-410X"],"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/P4310320175","host_organization_name":"Elsevier Masson","host_organization_lineage":["https://openalex.org/P4310320175","https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier Masson","Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":"Annales De Medecine Interne","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.9940000176429749}],"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/W2010543298","https://openalex.org/W2031360287","https://openalex.org/W2477646499","https://openalex.org/W1550047936","https://openalex.org/W4230901927","https://openalex.org/W54399843","https://openalex.org/W303308400","https://openalex.org/W4286373062","https://openalex.org/W2406414113","https://openalex.org/W1547629510"],"abstract_inverted_index":{"Thrombotic":[0,34],"microangiopathies":[1],"(TMA)":[2],"encompass":[3],"various":[4],"severe":[5],"diseases":[6],"characterized":[7,312],"by":[8,313],"microangiopathic":[9],"hemolytic":[10,39],"anemia":[11],"and":[12,20,29,38,54,75,77,98,101,119,128,153,181,193,263,279,297,316],"peripheral":[13],"thrombocytopenia,":[14],"associated":[15,161,234,273],"with":[16,162,235,277],"fever,":[17],"neurological":[18],"signs":[19],"renal":[21],"involvement.":[22],"Microvascular":[23],"thrombosis":[24],"is":[25,59,69,160,221,233,258],"the":[26,44,70,107,135,207,265,283,293],"typical":[27],"lesion,":[28],"results":[30],"in":[31,73,165,173,213,256,274],"tissue":[32,94],"ischemia.":[33],"thrombocytopenic":[35],"purpura":[36],"(TTP)":[37],"uremic":[40],"syndrome":[41],"(HUS)":[42],"are":[43,52,243],"two":[45,50,226],"most":[46],"classical":[47],"forms.":[48],"These":[49,241],"entities":[51],"clinically":[53],"histopathologically":[55],"closely":[56,259],"related.":[57],"There":[58],"a":[60,84,139,163],"body":[61],"of":[62,87,109,113,137,142,175,210,286,292,307],"evidence":[63],"suggesting":[64],"that":[65,78,151,309],"endothelial":[66,247],"cell":[67,104,248],"injury":[68,105],"initial":[71],"event":[72],"TTP":[74,152,159,214],"HUS,":[76,217,232],"it":[79,133,147],"may":[80,187,204,300,310],"be":[81,188,301,311],"related":[82,195,260],"to":[83,196,261,303],"large":[85,111],"number":[86],"triggering":[88,267,295],"factors,":[89],"such":[90,123],"as":[91,124],"infection,":[92],"connective":[93],"disease,":[95],"drugs,":[96],"cancer":[97],"chemotherapy,":[99],"transplantation,":[100],"pregnancy.":[102],"Endothelial":[103],"enhances":[106],"release":[108,136],"ultra":[110],"forms":[112],"von":[114,166],"Willebrand":[115,167],"factor":[116,280],"(ULvWF)":[117],"multimers":[118],"other":[120],"prothrombotic":[121],"agents,":[122],"plasminogen":[125],"activator":[126],"inhibitor":[127,141],"platelet":[129,143],"activating":[130],"factor,":[131],"whereas":[132],"decreases":[134],"prostaglandin-I2,":[138],"strong":[140],"aggregation.":[144],"Recently":[145],"however,":[146],"has":[148,271],"been":[149,272],"shown":[150],"HUS":[154,224,251,255,270],"were":[155],"pathophysiologically":[156],"distinct.":[157],"Actually,":[158],"deficiency":[164,186,203],"factor-cleaving":[168],"protease,":[169],"an":[170,197],"enzyme":[171],"involved":[172],"cleavage":[174],"ULvWF":[176,212],"into":[177],"circulating":[178],"200":[179],"kDa":[180,183],"350":[182],"fragments.":[184],"This":[185,201],"either":[189],"congenital":[190],"or":[191,230,237],"acquired,":[192],"then":[194],"IgG":[198],"inhibitory":[199],"autoantibody.":[200],"protease":[202,219],"account":[205],"for":[206,246],"high":[208],"amounts":[209],"plasmatic":[211],"patients.":[215],"In":[216],"vWF-cleaving":[218],"activity":[220],"found":[222],"normal.":[223],"encompasses":[225],"distinct":[227],"entities.":[228],"Epidemic,":[229],"diarrhea-associated":[231],"verotoxin":[236],"Shiga":[238],"toxin-associated":[239],"enterobacteriaceae.":[240],"toxins":[242],"directly":[244],"responsible":[245],"injury.":[249],"Sporadic":[250],"(also":[252],"termed":[253],"atypical":[254],"children)":[257],"TTP,":[262],"shares":[264],"same":[266],"factors.":[268],"Familial":[269],"some":[275],"cases":[276],"hypocomplementemia":[278],"H":[281],"dysfunction,":[282],"pathophysiological":[284],"role":[285],"which":[287],"remains":[288],"unclear.":[289],"The":[290],"study":[291],"different":[294,305],"factors":[296,299],"predisposing":[298],"useful":[302],"define":[304],"subsets":[306],"TMA,":[308],"their":[314],"course":[315],"prognosis.":[317]},"counts_by_year":[{"year":2024,"cited_by_count":1},{"year":2023,"cited_by_count":1},{"year":2021,"cited_by_count":1},{"year":2020,"cited_by_count":1},{"year":2014,"cited_by_count":1},{"year":2012,"cited_by_count":1}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2016-06-24T00:00:00"}
