Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study
Objective The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D‐dimer levels and the presence of phleboliths within the malformation, has been...
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| Veröffentlicht in: | Pediatric blood & cancer 2024-05, Vol.71 (5), p.e30915-n/a |
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| creator | Nava y Hurtado, Francisco Monzon Manzano, Elena Viana‐Huete, Vanesa Triana Junco, Paloma Alvarez‐Roman, Maria Teresa Arias‐Salgado, Elena G. Butta, Nora Lopez Gutierrez, Juan Carlos |
| description | Objective
The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D‐dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation.
Methods
With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan‐1 was determined by ELISA.
Results
In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan‐1 showed significant differences between extralesional and intralesional levels (p |
| doi_str_mv | 10.1002/pbc.30915 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2928584246</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2928584246</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3135-9e934eff2946c0b142ab31b62716db6bb99bb6f8049fe836dcd26a3ddfc8b9173</originalsourceid><addsrcrecordid>eNp10ctu1TAQBmALgegFFrwAssSGLk5rx4kTszscQYtUCRawjnyZ9Lhy7GA7Rdn1EXjGPgkuabtAYjWz-PRrRj9Cbyg5pYRUZ5PSp4wI2jxDh7Spm01DaPv8aSfiAB2ldF0oJ033Eh2wjnHBO3GIbrcpQUrWX2Ed5NXswiTzfsHSGwzehLwHZ6XDZknD7HW2wWPr8QTGyhytxjfgw5zwKN0Q4ijvwQe8xXkfw6gCOJlyGCHHNTIt3oCW_u72N8Upz2Z5hV4M0iV4_TCP0Y_Pn77vLjaXX8-_7LaXG80oazYCBKthGCpRc00UrSupGFW8aik3iislhFJ86EgtBijfGW0qLpkxg-6UoC07Ru_X3CmGnzOk3I82aXBOeigP9JWouqarq5oX-u4feh3m6Mt1RbWMtC2vu6JOVqVjSCnC0E_RjjIuPSX9fS19qaX_W0uxbx8SZzWCeZKPPRRwtoJf1sHy_6T-28fdGvkHBeCafw</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2973077648</pqid></control><display><type>article</type><title>Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study</title><source>Wiley Online Library Journals Frontfile Complete</source><creator>Nava y Hurtado, Francisco ; Monzon Manzano, Elena ; Viana‐Huete, Vanesa ; Triana Junco, Paloma ; Alvarez‐Roman, Maria Teresa ; Arias‐Salgado, Elena G. ; Butta, Nora ; Lopez Gutierrez, Juan Carlos</creator><creatorcontrib>Nava y Hurtado, Francisco ; Monzon Manzano, Elena ; Viana‐Huete, Vanesa ; Triana Junco, Paloma ; Alvarez‐Roman, Maria Teresa ; Arias‐Salgado, Elena G. ; Butta, Nora ; Lopez Gutierrez, Juan Carlos</creatorcontrib><description>Objective
The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D‐dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation.
Methods
With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan‐1 was determined by ELISA.
Results
In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan‐1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium.
Conclusions
For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.</description><identifier>ISSN: 1545-5009</identifier><identifier>EISSN: 1545-5017</identifier><identifier>DOI: 10.1002/pbc.30915</identifier><identifier>PMID: 38369689</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Coagulation ; coagulopathy ; Endothelium ; Enzyme-linked immunosorbent assay ; Fibrinolysis ; glycocalyx ; intralesional ; Pediatrics ; Quality of life ; ROTEM ; Syndecan ; syndecan‐1 ; t-Plasminogen activator ; venous malformation</subject><ispartof>Pediatric blood & cancer, 2024-05, Vol.71 (5), p.e30915-n/a</ispartof><rights>2024 Wiley Periodicals LLC.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3135-9e934eff2946c0b142ab31b62716db6bb99bb6f8049fe836dcd26a3ddfc8b9173</cites><orcidid>0000-0003-1167-7757 ; 0000-0001-8390-1346 ; 0000-0002-8176-6010 ; 0000-0002-7967-6358</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fpbc.30915$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fpbc.30915$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38369689$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Nava y Hurtado, Francisco</creatorcontrib><creatorcontrib>Monzon Manzano, Elena</creatorcontrib><creatorcontrib>Viana‐Huete, Vanesa</creatorcontrib><creatorcontrib>Triana Junco, Paloma</creatorcontrib><creatorcontrib>Alvarez‐Roman, Maria Teresa</creatorcontrib><creatorcontrib>Arias‐Salgado, Elena G.</creatorcontrib><creatorcontrib>Butta, Nora</creatorcontrib><creatorcontrib>Lopez Gutierrez, Juan Carlos</creatorcontrib><title>Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study</title><title>Pediatric blood & cancer</title><addtitle>Pediatr Blood Cancer</addtitle><description>Objective
The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D‐dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation.
Methods
With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan‐1 was determined by ELISA.
Results
In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan‐1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium.
Conclusions
For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.</description><subject>Coagulation</subject><subject>coagulopathy</subject><subject>Endothelium</subject><subject>Enzyme-linked immunosorbent assay</subject><subject>Fibrinolysis</subject><subject>glycocalyx</subject><subject>intralesional</subject><subject>Pediatrics</subject><subject>Quality of life</subject><subject>ROTEM</subject><subject>Syndecan</subject><subject>syndecan‐1</subject><subject>t-Plasminogen activator</subject><subject>venous malformation</subject><issn>1545-5009</issn><issn>1545-5017</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><recordid>eNp10ctu1TAQBmALgegFFrwAssSGLk5rx4kTszscQYtUCRawjnyZ9Lhy7GA7Rdn1EXjGPgkuabtAYjWz-PRrRj9Cbyg5pYRUZ5PSp4wI2jxDh7Spm01DaPv8aSfiAB2ldF0oJ033Eh2wjnHBO3GIbrcpQUrWX2Ed5NXswiTzfsHSGwzehLwHZ6XDZknD7HW2wWPr8QTGyhytxjfgw5zwKN0Q4ijvwQe8xXkfw6gCOJlyGCHHNTIt3oCW_u72N8Upz2Z5hV4M0iV4_TCP0Y_Pn77vLjaXX8-_7LaXG80oazYCBKthGCpRc00UrSupGFW8aik3iislhFJ86EgtBijfGW0qLpkxg-6UoC07Ru_X3CmGnzOk3I82aXBOeigP9JWouqarq5oX-u4feh3m6Mt1RbWMtC2vu6JOVqVjSCnC0E_RjjIuPSX9fS19qaX_W0uxbx8SZzWCeZKPPRRwtoJf1sHy_6T-28fdGvkHBeCafw</recordid><startdate>202405</startdate><enddate>202405</enddate><creator>Nava y Hurtado, Francisco</creator><creator>Monzon Manzano, Elena</creator><creator>Viana‐Huete, Vanesa</creator><creator>Triana Junco, Paloma</creator><creator>Alvarez‐Roman, Maria Teresa</creator><creator>Arias‐Salgado, Elena G.</creator><creator>Butta, Nora</creator><creator>Lopez Gutierrez, Juan Carlos</creator><general>Wiley Subscription Services, Inc</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>7TO</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>K9.</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0003-1167-7757</orcidid><orcidid>https://orcid.org/0000-0001-8390-1346</orcidid><orcidid>https://orcid.org/0000-0002-8176-6010</orcidid><orcidid>https://orcid.org/0000-0002-7967-6358</orcidid></search><sort><creationdate>202405</creationdate><title>Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study</title><author>Nava y Hurtado, Francisco ; Monzon Manzano, Elena ; Viana‐Huete, Vanesa ; Triana Junco, Paloma ; Alvarez‐Roman, Maria Teresa ; Arias‐Salgado, Elena G. ; Butta, Nora ; Lopez Gutierrez, Juan Carlos</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3135-9e934eff2946c0b142ab31b62716db6bb99bb6f8049fe836dcd26a3ddfc8b9173</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Coagulation</topic><topic>coagulopathy</topic><topic>Endothelium</topic><topic>Enzyme-linked immunosorbent assay</topic><topic>Fibrinolysis</topic><topic>glycocalyx</topic><topic>intralesional</topic><topic>Pediatrics</topic><topic>Quality of life</topic><topic>ROTEM</topic><topic>Syndecan</topic><topic>syndecan‐1</topic><topic>t-Plasminogen activator</topic><topic>venous malformation</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Nava y Hurtado, Francisco</creatorcontrib><creatorcontrib>Monzon Manzano, Elena</creatorcontrib><creatorcontrib>Viana‐Huete, Vanesa</creatorcontrib><creatorcontrib>Triana Junco, Paloma</creatorcontrib><creatorcontrib>Alvarez‐Roman, Maria Teresa</creatorcontrib><creatorcontrib>Arias‐Salgado, Elena G.</creatorcontrib><creatorcontrib>Butta, Nora</creatorcontrib><creatorcontrib>Lopez Gutierrez, Juan Carlos</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric blood & cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Nava y Hurtado, Francisco</au><au>Monzon Manzano, Elena</au><au>Viana‐Huete, Vanesa</au><au>Triana Junco, Paloma</au><au>Alvarez‐Roman, Maria Teresa</au><au>Arias‐Salgado, Elena G.</au><au>Butta, Nora</au><au>Lopez Gutierrez, Juan Carlos</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study</atitle><jtitle>Pediatric blood & cancer</jtitle><addtitle>Pediatr Blood Cancer</addtitle><date>2024-05</date><risdate>2024</risdate><volume>71</volume><issue>5</issue><spage>e30915</spage><epage>n/a</epage><pages>e30915-n/a</pages><issn>1545-5009</issn><eissn>1545-5017</eissn><abstract>Objective
The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D‐dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation.
Methods
With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan‐1 was determined by ELISA.
Results
In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan‐1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium.
Conclusions
For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>38369689</pmid><doi>10.1002/pbc.30915</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0003-1167-7757</orcidid><orcidid>https://orcid.org/0000-0001-8390-1346</orcidid><orcidid>https://orcid.org/0000-0002-8176-6010</orcidid><orcidid>https://orcid.org/0000-0002-7967-6358</orcidid></addata></record> |
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| subjects | Coagulation coagulopathy Endothelium Enzyme-linked immunosorbent assay Fibrinolysis glycocalyx intralesional Pediatrics Quality of life ROTEM Syndecan syndecan‐1 t-Plasminogen activator venous malformation |
| title | Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study |
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