关键词: Alport综合征, 可溶性尿激酶型纤溶酶原激活物受体, 慢性肾脏病, 蛋白尿

Abstract:

Ｏｂｊｅｃｔｉｖｅ：To investigated the role of serum levels of soluble urokinase plasminogen activator receptor (suPAR) in children with Alport syndrome.
Ｍｅｔｈｏｄｏｌｏｇｙ：34 children with Alport syndrome were enrolled into this study. 11 children with primary FSGS were as disease controls and 13 healthy children were as normal controls. The clinical and pathological data were collected at the time of take the serum samples. The serum suPAR were measured using commercially available kits. The relationship between  levels and  protienuria, serum creatinine, creatinine clearance rate, and other clinical and pathological data were analyzed.
Ｒｅｓｕｌｔｓ：The average age of children with Alport syndrome was 956±465 ys. Male and female rate was 28∶6. There were 24 children with Xlinked Alport syndrome, and 10 with autosomal recessive Alport syndrome. 12 children were combined with hearing loss. The mean level of serum suPAR was significantly elevated in children with Alport syndrome compared to normal controls [(3 89665±97934) pg/ml vs (2 74762±27759) pg/ml, P<0000 1]. The mean level of serum suPAR in children with primary FSGS was (4 61927±1 04356) pg/ml. Furthermore, the serum suPAR levels in children with Alport syndrome were positively correlated with the 24 hours proteinuria (r=0400 0, P=0021 1) and serum creatinine levels (r=0410 1, P=0016 0). The serum suPAR level was significantly higher in Alport children with Ccr<90 (ml/min·173m2) than in Alport children with Ccr≥90 (ml/min·173m2) (P<0000 1). The serum suPAR levels in children with primary FSGS were negatively correlated with Ccr (r=-0833 3，P=0008 3).
Ｃｏｎｃｌｕｓｉｏｎ：An elevated level of suPAR was detected in children with Alport syndrome. The serum suPAR level may serve as a new marker associated with renal damage.