De Mattia D, Penza R, Giordano P, Del Vecchio G C, Aceto G, Altomare M, Schettini F
Dipartimento di Biomedicina dell'Età Evolutiva, University of Bari, Italy.
Pediatr Nephrol. 1995 Oct;9(5):603-5. doi: 10.1007/BF00860949.
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.
在16例初诊的过敏性紫癜（HSP）患儿（7例男性，9例女性，年龄3 - 11岁）中，研究了血管性血友病因子抗原（vWf:Ag）水平和因子ⅩⅢ活性（FⅩⅢ）与临床症状严重程度（评分从0至3）以及免疫参数[免疫球蛋白A（IgA）、补体C3、补体C4和循环免疫复合物（CIC）]之间的关系。7例患者vWf:Ag升高，6例FⅩⅢ活性降低。在所有患儿中，均发现IgA水平升高，而C3和C4水平正常；11例CIC升高。vWf:Ag与临床评分以及IgA和CIC相关，这可能是免疫介导的内皮细胞损伤的结果。在HSP中观察到的止血改变对于理解该疾病的病理生理学很重要。
