von Willebrand factor and factor XIII in children with Henoch-Schonlein purpura.
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.
De Mattia
D
D
Dipartimento di Biomedicina dell'Età Evolutiva, University of Bari, Italy.
Penza
R
R
Giordano
P
P
Del Vecchio
G C
GC
Aceto
G
G
Altomare
M
M
Schettini
F
F
D000208:Acute Disease; D001324:Autoantigens / Q000097:blood; D001780:Blood Coagulation Tests; D002648:Child; D002675:Child, Preschool; D004797:Enzyme-Linked Immunosorbent Assay; D005176:Factor XIII / Q000378:metabolism*; D005260:Female; D005340:Fibrinogen / Q000378:metabolism; D006801:Humans; D011695:IgA Vasculitis / Q000097:blood* / Q000276:immunology; D007136:Immunoglobulins / Q000097:blood; D008297:Male; D012720:Severity of Illness Index; D014841:von Willebrand Factor / Q000276:immunology*
D001324:Autoantigens; D007136:Immunoglobulins; D014841:von Willebrand Factor; D005340:Fibrinogen; D005176:Factor XIII