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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Bibliographic Details
Published in:PloS one 2013-10, Vol.8 (10), p.e78801-e78801
Main Authors: 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
Format: Article
Language:English
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
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Summary: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
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0078801