Coagulation activation in children with sickle cell disease is associated with cerebral small vessel vasculopathy
Thrombotic complications in Sickle Cell Disease (SCD) arise since infancy, but the role of the coagulation system in children has been poorly explored. To determine its role in the development of clinical complications in childhood we measured coagulation and endothelial parameters in children with...
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| creator | Colombatti, Raffaella De Bon, Emiliano Bertomoro, Antonella Casonato, Alessandra Pontara, Elena Omenetto, Elisabetta Saggiorato, Graziella Steffan, Agostino Damian, Tamara Cella, Giuseppe Teso, Simone Manara, Renzo Rampazzo, Patrizia Meneghetti, Giorgio Basso, Giuseppe Sartori, Maria Teresa Sainati, Laura |
| description | Thrombotic complications in Sickle Cell Disease (SCD) arise since infancy, but the role of the coagulation system in children has been poorly explored. To determine its role in the development of clinical complications in childhood we measured coagulation and endothelial parameters in children with SCD at steady state.
Markers of thrombin generation, fibrin dissolution and endothelial activation were evaluated in 38 children with SS-Sβ°, 6 with SC disease and 50 age and blood group matched controls. Coagulation variables were correlated with markers of hemolysis and inflammation, with the presence of cerebral and lung vasculopathy and with the frequency of clinical complications.
SS-Sβ° patients presented higher levels of factor VIII, von Willebrand factor antigen (VWF:Ag) and collagen binding activity, tissue plasminogen activator antigen (t-PA:Ag), D-dimer, p-selectin, prothrombin fragment1+2 (F1+2) and lower ADAMTS-13:activity/VWF:Ag (p |
| doi_str_mv | 10.1371/journal.pone.0078801 |
| format | Article |
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Markers of thrombin generation, fibrin dissolution and endothelial activation were evaluated in 38 children with SS-Sβ°, 6 with SC disease and 50 age and blood group matched controls. Coagulation variables were correlated with markers of hemolysis and inflammation, with the presence of cerebral and lung vasculopathy and with the frequency of clinical complications.
SS-Sβ° patients presented higher levels of factor VIII, von Willebrand factor antigen (VWF:Ag) and collagen binding activity, tissue plasminogen activator antigen (t-PA:Ag), D-dimer, p-selectin, prothrombin fragment1+2 (F1+2) and lower ADAMTS-13:activity/VWF:Ag (p<0.05) compared to controls and SC patients. In SS-Sβ° patients coagulation variables correlated positively with markers of inflammation, hemolysis, and negatively with HbF (p<0.05). Patients with cerebral silent infarcts showed significant decrease in t-PA:Ag and ADAMTS-13 Antigen and a tendency toward higher D-dimer, F1+2, TAT compared to patients without them. D-dimer was associated with a six fold increased risk of cerebral silent infarcts. No correlation was found between coagulation activation and large vessel vasculopathy or other clinical events except for decreased t-PA:Ag in patients with tricuspid Rigurgitant Velocity >2.5m/sec.
SS-Sβ° disease is associated with extensive activation of the coagulation system at steady state since young age. ADAMTS-13 and t-PA:Ag are involved in the development of cerebral silent infarcts.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0078801</identifier><identifier>PMID: 24205317</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Activation ; Adolescent ; Age ; Anemia, Sickle Cell - blood ; Anemia, Sickle Cell - metabolism ; Blood ; Blood Coagulation ; Blood groups ; Blood Vessels - physiopathology ; Brain - blood supply ; Child ; Child, Preschool ; Children ; Coagulation ; Coagulation factors ; Collagen ; Complications ; Correlation ; Disease control ; Female ; Fibrin ; Hematology ; Hemolysis ; Humans ; Infant ; Laboratories ; Lung - blood supply ; Lungs ; Male ; Markers ; Medical imaging ; NMR ; Nuclear magnetic resonance ; Oncology ; P-selectin ; Patients ; Pediatrics ; Prothrombin ; Pulmonary hypertension ; Sickle cell anemia ; Sickle cell disease ; Steady state ; Stroke ; t-Plasminogen activator ; Thrombin ; Thrombin - biosynthesis ; Vascular diseases ; Von Willebrand factor</subject><ispartof>PloS one, 2013-10, Vol.8 (10), p.e78801-e78801</ispartof><rights>2013 Colombatti et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License: https://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2013 Colombatti et al 2013 Colombatti et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c526t-306ffe2ce44ce618c694c4723e46154129eef1d0b1faf8904e564bf841b49b323</citedby><cites>FETCH-LOGICAL-c526t-306ffe2ce44ce618c694c4723e46154129eef1d0b1faf8904e564bf841b49b323</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3808283/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3808283/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,860,881,2096,2915,23845,27901,27902,53766,53768,79569,79570</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/24205317$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Colombatti, Raffaella</creatorcontrib><creatorcontrib>De Bon, Emiliano</creatorcontrib><creatorcontrib>Bertomoro, Antonella</creatorcontrib><creatorcontrib>Casonato, Alessandra</creatorcontrib><creatorcontrib>Pontara, Elena</creatorcontrib><creatorcontrib>Omenetto, Elisabetta</creatorcontrib><creatorcontrib>Saggiorato, Graziella</creatorcontrib><creatorcontrib>Steffan, Agostino</creatorcontrib><creatorcontrib>Damian, Tamara</creatorcontrib><creatorcontrib>Cella, Giuseppe</creatorcontrib><creatorcontrib>Teso, Simone</creatorcontrib><creatorcontrib>Manara, Renzo</creatorcontrib><creatorcontrib>Rampazzo, Patrizia</creatorcontrib><creatorcontrib>Meneghetti, Giorgio</creatorcontrib><creatorcontrib>Basso, Giuseppe</creatorcontrib><creatorcontrib>Sartori, Maria Teresa</creatorcontrib><creatorcontrib>Sainati, Laura</creatorcontrib><title>Coagulation activation in children with sickle cell disease is associated with cerebral small vessel vasculopathy</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>Thrombotic complications in Sickle Cell Disease (SCD) arise since infancy, but the role of the coagulation system in children has been poorly explored. To determine its role in the development of clinical complications in childhood we measured coagulation and endothelial parameters in children with SCD at steady state.
Markers of thrombin generation, fibrin dissolution and endothelial activation were evaluated in 38 children with SS-Sβ°, 6 with SC disease and 50 age and blood group matched controls. Coagulation variables were correlated with markers of hemolysis and inflammation, with the presence of cerebral and lung vasculopathy and with the frequency of clinical complications.
SS-Sβ° patients presented higher levels of factor VIII, von Willebrand factor antigen (VWF:Ag) and collagen binding activity, tissue plasminogen activator antigen (t-PA:Ag), D-dimer, p-selectin, prothrombin fragment1+2 (F1+2) and lower ADAMTS-13:activity/VWF:Ag (p<0.05) compared to controls and SC patients. In SS-Sβ° patients coagulation variables correlated positively with markers of inflammation, hemolysis, and negatively with HbF (p<0.05). Patients with cerebral silent infarcts showed significant decrease in t-PA:Ag and ADAMTS-13 Antigen and a tendency toward higher D-dimer, F1+2, TAT compared to patients without them. D-dimer was associated with a six fold increased risk of cerebral silent infarcts. No correlation was found between coagulation activation and large vessel vasculopathy or other clinical events except for decreased t-PA:Ag in patients with tricuspid Rigurgitant Velocity >2.5m/sec.
SS-Sβ° disease is associated with extensive activation of the coagulation system at steady state since young age. ADAMTS-13 and t-PA:Ag are involved in the development of cerebral silent infarcts.</description><subject>Activation</subject><subject>Adolescent</subject><subject>Age</subject><subject>Anemia, Sickle Cell - blood</subject><subject>Anemia, Sickle Cell - metabolism</subject><subject>Blood</subject><subject>Blood Coagulation</subject><subject>Blood groups</subject><subject>Blood Vessels - physiopathology</subject><subject>Brain - blood supply</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>Coagulation</subject><subject>Coagulation factors</subject><subject>Collagen</subject><subject>Complications</subject><subject>Correlation</subject><subject>Disease control</subject><subject>Female</subject><subject>Fibrin</subject><subject>Hematology</subject><subject>Hemolysis</subject><subject>Humans</subject><subject>Infant</subject><subject>Laboratories</subject><subject>Lung - blood supply</subject><subject>Lungs</subject><subject>Male</subject><subject>Markers</subject><subject>Medical imaging</subject><subject>NMR</subject><subject>Nuclear magnetic resonance</subject><subject>Oncology</subject><subject>P-selectin</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Prothrombin</subject><subject>Pulmonary hypertension</subject><subject>Sickle cell anemia</subject><subject>Sickle cell disease</subject><subject>Steady state</subject><subject>Stroke</subject><subject>t-Plasminogen activator</subject><subject>Thrombin</subject><subject>Thrombin - 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Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PloS one</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Colombatti, Raffaella</au><au>De Bon, Emiliano</au><au>Bertomoro, Antonella</au><au>Casonato, Alessandra</au><au>Pontara, Elena</au><au>Omenetto, Elisabetta</au><au>Saggiorato, Graziella</au><au>Steffan, Agostino</au><au>Damian, Tamara</au><au>Cella, Giuseppe</au><au>Teso, Simone</au><au>Manara, Renzo</au><au>Rampazzo, Patrizia</au><au>Meneghetti, Giorgio</au><au>Basso, Giuseppe</au><au>Sartori, Maria Teresa</au><au>Sainati, Laura</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Coagulation activation in children with sickle cell disease is associated with cerebral small vessel vasculopathy</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2013-10-25</date><risdate>2013</risdate><volume>8</volume><issue>10</issue><spage>e78801</spage><epage>e78801</epage><pages>e78801-e78801</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>Thrombotic complications in Sickle Cell Disease (SCD) arise since infancy, but the role of the coagulation system in children has been poorly explored. To determine its role in the development of clinical complications in childhood we measured coagulation and endothelial parameters in children with SCD at steady state.
Markers of thrombin generation, fibrin dissolution and endothelial activation were evaluated in 38 children with SS-Sβ°, 6 with SC disease and 50 age and blood group matched controls. Coagulation variables were correlated with markers of hemolysis and inflammation, with the presence of cerebral and lung vasculopathy and with the frequency of clinical complications.
SS-Sβ° patients presented higher levels of factor VIII, von Willebrand factor antigen (VWF:Ag) and collagen binding activity, tissue plasminogen activator antigen (t-PA:Ag), D-dimer, p-selectin, prothrombin fragment1+2 (F1+2) and lower ADAMTS-13:activity/VWF:Ag (p<0.05) compared to controls and SC patients. In SS-Sβ° patients coagulation variables correlated positively with markers of inflammation, hemolysis, and negatively with HbF (p<0.05). Patients with cerebral silent infarcts showed significant decrease in t-PA:Ag and ADAMTS-13 Antigen and a tendency toward higher D-dimer, F1+2, TAT compared to patients without them. D-dimer was associated with a six fold increased risk of cerebral silent infarcts. No correlation was found between coagulation activation and large vessel vasculopathy or other clinical events except for decreased t-PA:Ag in patients with tricuspid Rigurgitant Velocity >2.5m/sec.
SS-Sβ° disease is associated with extensive activation of the coagulation system at steady state since young age. ADAMTS-13 and t-PA:Ag are involved in the development of cerebral silent infarcts.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>24205317</pmid><doi>10.1371/journal.pone.0078801</doi><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1932-6203 |
| ispartof | PloS one, 2013-10, Vol.8 (10), p.e78801-e78801 |
| issn | 1932-6203 1932-6203 |
| language | eng |
| recordid | cdi_plos_journals_1445923245 |
| source | MEDLINE; DOAJ Directory of Open Access Journals; EZB-FREE-00999 freely available EZB journals; PubMed Central; Free Full-Text Journals in Chemistry; Public Library of Science (PLoS) |
| subjects | Activation Adolescent Age Anemia, Sickle Cell - blood Anemia, Sickle Cell - metabolism Blood Blood Coagulation Blood groups Blood Vessels - physiopathology Brain - blood supply Child Child, Preschool Children Coagulation Coagulation factors Collagen Complications Correlation Disease control Female Fibrin Hematology Hemolysis Humans Infant Laboratories Lung - blood supply Lungs Male Markers Medical imaging NMR Nuclear magnetic resonance Oncology P-selectin Patients Pediatrics Prothrombin Pulmonary hypertension Sickle cell anemia Sickle cell disease Steady state Stroke t-Plasminogen activator Thrombin Thrombin - biosynthesis Vascular diseases Von Willebrand factor |
| title | Coagulation activation in children with sickle cell disease is associated with cerebral small vessel vasculopathy |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-15T15%3A00%3A18IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Coagulation%20activation%20in%20children%20with%20sickle%20cell%20disease%20is%20associated%20with%20cerebral%20small%20vessel%20vasculopathy&rft.jtitle=PloS%20one&rft.au=Colombatti,%20Raffaella&rft.date=2013-10-25&rft.volume=8&rft.issue=10&rft.spage=e78801&rft.epage=e78801&rft.pages=e78801-e78801&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0078801&rft_dat=%3Cproquest_plos_%3E3110575491%3C/proquest_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1445923245&rft_id=info:pmid/24205317&rft_doaj_id=oai_doaj_org_article_404213950a79495e80b5829c16e14454&rfr_iscdi=true |