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Venous Malformations and Blood Coagulation in Children

Introduction: Venous malformations (VMs) are congenital low-flow lesions with a wide spectrum of clinical manifestations. An increasing number of studies link VMs to coagulation abnormalities, especially to elevated D-dimer and decreased fibrinogen. This condition, termed localized intravascular coa...

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Autores principales: Aronniemi, Johanna, Långström, Satu, Mattila, Katariina A., Mäkipernaa, Anne, Salminen, Päivi, Pitkäranta, Anne, Pekkola, Johanna, Lassila, Riitta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8074292/
https://www.ncbi.nlm.nih.gov/pubmed/33924092
http://dx.doi.org/10.3390/children8040312
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author Aronniemi, Johanna
Långström, Satu
Mattila, Katariina A.
Mäkipernaa, Anne
Salminen, Päivi
Pitkäranta, Anne
Pekkola, Johanna
Lassila, Riitta
author_facet Aronniemi, Johanna
Långström, Satu
Mattila, Katariina A.
Mäkipernaa, Anne
Salminen, Päivi
Pitkäranta, Anne
Pekkola, Johanna
Lassila, Riitta
author_sort Aronniemi, Johanna
collection PubMed
description Introduction: Venous malformations (VMs) are congenital low-flow lesions with a wide spectrum of clinical manifestations. An increasing number of studies link VMs to coagulation abnormalities, especially to elevated D-dimer and decreased fibrinogen. This condition, termed localized intravascular coagulopathy (LIC), may pose a risk for hemostatic complications. However, detailed data on the laboratory variables for coagulation and fibrinolytic activity in VM patients are limited. We addressed this question by systematically analyzing the coagulation parameters in pediatric VM patients. Methods: We included 62 patients (median age 11.9 years) with detailed laboratory tests for coagulation and fibrinolytic activity at a clinically steady phase. We assessed clinical and imaging features of VMs and their correlations with coagulation and fibrinolysis variables using patient records and MRI. Results: D-dimer was elevated in 39% and FXIII decreased in 20% of the patients, as a sign of LIC. Elevated D-dimer and decreased FXIII were associated with large size, deep location, and diffuse and multifocal VMs. FVIII was elevated in 17% of the patients and was associated with small VM size, superficial and confined location, discrete morphology, and less pain. Surprisingly, antithrombin was elevated in 55% of the patients but without associations with clinical or other laboratory variables. Conclusions: LIC was common in pediatric patients with VMs. Our results provide a basis for when evaluating the risks of hemostatic complications in children with VMs. Further research is warranted to explore the mechanisms behind coagulation disturbances and their relation to clinical complications.
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spelling pubmed-80742922021-04-27 Venous Malformations and Blood Coagulation in Children Aronniemi, Johanna Långström, Satu Mattila, Katariina A. Mäkipernaa, Anne Salminen, Päivi Pitkäranta, Anne Pekkola, Johanna Lassila, Riitta Children (Basel) Article Introduction: Venous malformations (VMs) are congenital low-flow lesions with a wide spectrum of clinical manifestations. An increasing number of studies link VMs to coagulation abnormalities, especially to elevated D-dimer and decreased fibrinogen. This condition, termed localized intravascular coagulopathy (LIC), may pose a risk for hemostatic complications. However, detailed data on the laboratory variables for coagulation and fibrinolytic activity in VM patients are limited. We addressed this question by systematically analyzing the coagulation parameters in pediatric VM patients. Methods: We included 62 patients (median age 11.9 years) with detailed laboratory tests for coagulation and fibrinolytic activity at a clinically steady phase. We assessed clinical and imaging features of VMs and their correlations with coagulation and fibrinolysis variables using patient records and MRI. Results: D-dimer was elevated in 39% and FXIII decreased in 20% of the patients, as a sign of LIC. Elevated D-dimer and decreased FXIII were associated with large size, deep location, and diffuse and multifocal VMs. FVIII was elevated in 17% of the patients and was associated with small VM size, superficial and confined location, discrete morphology, and less pain. Surprisingly, antithrombin was elevated in 55% of the patients but without associations with clinical or other laboratory variables. Conclusions: LIC was common in pediatric patients with VMs. Our results provide a basis for when evaluating the risks of hemostatic complications in children with VMs. Further research is warranted to explore the mechanisms behind coagulation disturbances and their relation to clinical complications. MDPI 2021-04-20 /pmc/articles/PMC8074292/ /pubmed/33924092 http://dx.doi.org/10.3390/children8040312 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Aronniemi, Johanna
Långström, Satu
Mattila, Katariina A.
Mäkipernaa, Anne
Salminen, Päivi
Pitkäranta, Anne
Pekkola, Johanna
Lassila, Riitta
Venous Malformations and Blood Coagulation in Children
title Venous Malformations and Blood Coagulation in Children
title_full Venous Malformations and Blood Coagulation in Children
title_fullStr Venous Malformations and Blood Coagulation in Children
title_full_unstemmed Venous Malformations and Blood Coagulation in Children
title_short Venous Malformations and Blood Coagulation in Children
title_sort venous malformations and blood coagulation in children
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8074292/
https://www.ncbi.nlm.nih.gov/pubmed/33924092
http://dx.doi.org/10.3390/children8040312
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