ArticlePloS one2013
Coagulation activation in children with sickle cell disease is associated with cerebral small vessel vasculopathy.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 47 citations in OpenAlex.
- Trial
- Cerebrovascular involvement in hereditary spherocytosis: observational cohort and case-control MRI study.Orphanet journal of rare diseases · 2025Observational
- Abnormalities of hemostasis in sickle cell patients and predisposition to thrombotic risk: a systematic review and meta-analysis.Thrombosis journal · 2025Review
- The Highs and Lows of ADAMTS13 Activity.Journal of clinical medicine · 2024Review
- VWF-ADAMTS13 axis dysfunction in children with sickle cell disease treated with hydroxycarbamide vs blood transfusion.Blood advances · 2023Article
- Evidence of protective effects of recombinant ADAMTS13 in a humanized model of sickle cell disease.Haematologica · 2022Article
- Sickle cell disease and COVID-19: Susceptibility and severity.Pediatric blood & cancer · 2021Review
- Association Between Patent Foramen Ovale and Overt Ischemic Stroke in Children With Sickle Cell Disease.Frontiers in neurology · 2021Article
- Current challenges in the management of patients with sickle cell disease - A report of the Italian experience.Orphanet journal of rare diseases · 2019Review
- Serum homocysteine and disease severity in sickle cell anemia patients in Lagos.Journal of blood medicine · 2019Article
- Methylene tetrahydrofolate reductase gene mutation in sickle cell anaemia patients in Lagos, Nigeria.The Pan African medical journal · 2019Article
- Proteomic analysis of plasma from children with sickle cell anemia and silent cerebral infarction.Haematologica · 2018Article
- Biochemical markers in vascular cognitive impairment associated with subcortical small vessel disease - A consensus report.BMC neurology · 2017Review
- Evaluation of von Willebrand factor and ADAMTS-13 antigen and activity levels in sickle cell disease patients in Kuwait.Journal of thrombosis and thrombolysis · 2017Article
- Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke.British journal of haematology · 2017Article
- Coagulation abnormalities of sickle cell disease: Relationship with clinical outcomes and the effect of disease modifying therapies.Blood reviews · 2016Review
- Contribution of Sickle Cell Disease to the Pediatric Stroke Burden Among Hospital Discharges of African-Americans-United States, 1997-2012.Pediatric blood & cancer · 2015Article
- What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.Hematology. American Society of Hematology. Education Program · 2015Article
- Extracellular Vesicle Size Reveals Cargo Specific to Coagulation and Inflammation in Pediatric and Adult Sickle Cell Disease.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisObservational
Corrections and comments
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Authors and funding
17 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThrombotic 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.
methodsMarkers 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.
resultsSS-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.
conclusionsSS-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.
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