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Assessing coagulopathy and endothelial dysfunction in paediatric venous malformation: A thromboelastometry and syndecan-1 study
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  • Francisco Nava y Hurtado,
  • Elena Monzon Manzano,
  • Vanesa Viana-Huete,
  • Paloma Triana,
  • Maria Teresa Alvarez-Roman,
  • Elena G. Arias-Salgado,
  • Nora Butta,
  • Juan Carlos Lopez-Gutierrez
Francisco Nava y Hurtado
Hospital Universitario La Paz

Corresponding Author:[email protected]

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Elena Monzon Manzano
Hospital Universitario La Paz
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Vanesa Viana-Huete
Universidad Europea Facultad de Ciencias Biomedicas y de la Salud
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Paloma Triana
Hospital Universitario La Paz
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Maria Teresa Alvarez-Roman
Hospital Universitario La Paz
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Elena G. Arias-Salgado
Hospital Universitario La Paz
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Nora Butta
Hospital Universitario La Paz
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Juan Carlos Lopez-Gutierrez
Hospital Universitario La Paz
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Abstract

Objective (69) 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 (42) 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 (80) In the ROTEM ® analyses, the A5, A10, and 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<0.026) and controls (p<0.03), suggesting differences in the state of endothelium. Conclusions (39) 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.
17 Nov 2023Submission Checks Completed
17 Nov 2023Assigned to Editor
17 Nov 2023Submitted to Pediatric Blood & Cancer
17 Nov 2023Review(s) Completed, Editorial Evaluation Pending
18 Nov 2023Reviewer(s) Assigned
28 Jan 2024Review(s) Completed, Editorial Evaluation Pending
28 Jan 2024Editorial Decision: Accept