Abstract
Levels of von Willebrand factor antigen (vWf: Ag) and factor XIII activity (F XIII) were studied in relation to the severity of clinical symptoms (scored from 0 to 3) and to immunological parameters [IgA, C3, C4, and circulating immune complexes (CIC) in 16 children (7 males, 9 females, aged 3–11 years) with Henoch-Schonlein purpura (HSP) at presentation. vWf: Ag was increased in 7 patients, F XIII activity was decreased in 6. In all children we found high levels of IgA, while C3 and C4 levels were normal; CIC were elevated in 11. vWf: Ag correlated with clinical score and with IgA and CIC, probably as a result of immune-mediated endothelial cell damage. The haemostatic alterations observed in HSP are important for understanding the pathophysiology of the disease.
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De Mattia, D., Penza, R., Giordano, P. et al. Von Willebrand factor and factor XIII in children with Henoch-Schonlein purpura. Pediatr Nephrol 9, 603–605 (1995). https://doi.org/10.1007/BF00860949
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DOI: https://doi.org/10.1007/BF00860949